Classification and Current Management of Inner Ear Malformations.

PubMed

Sennaroğlu, Levent; Bajin, Münir Demir

2017-09-29

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

[Pre-operation evaluation and intra-operation management of cochlear implantation].

PubMed

Zhang, Dao-xing; Hu, Bao-hua; Xiao, Yu-li; Shi, Bo-ning

2004-10-01

To summarize pre-operation evaluation experiences in cochlear implantation. Performing auditory evaluation and image analysis seriously in 158 severe hearing loss or total deaf cases before cochlear implantation, comparing their performance with the findings during and post operation. Among the total 158 cases, 116 cases with normal structure, 42 cases with the abnormal findings of the inner or middle ear. Stapedial gusher happened in 6 cases, 1 case was not predicted before operation. Except 1 case with serious malformation, the findings of other 157 cases in operation were consistent with the pre-operation evaluation. We helped all patients reconstruct auditory conduction with cochlear implantation, and the average hearing level up to 37.6 dB SPL. Performing image analysis seriously before operation and planning for operation according to HRCT can do great help to cochlear implantation. The operation under the HRCT instruction has less complications.

[Inspecting the cochlear scala tympanic with flexible and semi-flexible micro-endoscope].

PubMed

Zhang, Daoxcing; Zhang, Yankun

2006-02-01

Flexible and semi-flexible micro-endoscopes were used in cochlear scala tympani inspection , to explore their application in inner ear examination. Fifteen profound hearing loss patients preparing for cochlear implant were included in this study. During the operation, micro-endoscopy was performed after opening the cochlear scala tympani. And 1 mm diameter semi-flexible micro-endoscope could go as deep as 9 mm into the cochlear scala tympani, while 0. 5 mm diameter flexible micro-endoscope could go as deep as 25 mm. The inspecting results were compared with video recording. Using 0.5 mm flexible micro-endoscope, we canould check cochlear scala tympani with depth range of 15-25 mm, but the video imaging was not clear enough to examine the microstructure in the cochlear. With 1 mm diameter semi-flexible micro-endoscope, we could reach 9 mm deep into the cochlear. During the examination, we found 3 cases with calcification deposit in osseous spiral lamina, l case with granulation tissue in the lateral wall of scala tympani, no abnormal findings in the other 11 cases. Inspecting the cochlear scala tympani with 0.5 mm flexible micro-endoscope, even though we can reach the second circuit of the cochlear, it is difficult to find the pathology in the cochlear because of the poor video imaging. With 1 mm semi-flexible micro-endoscope, we can identify the microstructure of the cochlear clearly and find the pathologic changes, but the inserting depth was limited to 9 mm with limitation to examine the whole cochlear.

Evolution of crossmodal reorganization of the voice area in cochlear-implanted deaf patients.

PubMed

Rouger, Julien; Lagleyre, Sébastien; Démonet, Jean-François; Fraysse, Bernard; Deguine, Olivier; Barone, Pascal

2012-08-01

Psychophysical and neuroimaging studies in both animal and human subjects have clearly demonstrated that cortical plasticity following sensory deprivation leads to a brain functional reorganization that favors the spared modalities. In postlingually deaf patients, the use of a cochlear implant (CI) allows a recovery of the auditory function, which will probably counteract the cortical crossmodal reorganization induced by hearing loss. To study the dynamics of such reversed crossmodal plasticity, we designed a longitudinal neuroimaging study involving the follow-up of 10 postlingually deaf adult CI users engaged in a visual speechreading task. While speechreading activates Broca's area in normally hearing subjects (NHS), the activity level elicited in this region in CI patients is abnormally low and increases progressively with post-implantation time. Furthermore, speechreading in CI patients induces abnormal crossmodal activations in right anterior regions of the superior temporal cortex normally devoted to processing human voice stimuli (temporal voice-sensitive areas-TVA). These abnormal activity levels diminish with post-implantation time and tend towards the levels observed in NHS. First, our study revealed that the neuroplasticity after cochlear implantation involves not only auditory but also visual and audiovisual speech processing networks. Second, our results suggest that during deafness, the functional links between cortical regions specialized in face and voice processing are reallocated to support speech-related visual processing through cross-modal reorganization. Such reorganization allows a more efficient audiovisual integration of speech after cochlear implantation. These compensatory sensory strategies are later completed by the progressive restoration of the visuo-audio-motor speech processing loop, including Broca's area. Copyright © 2011 Wiley Periodicals, Inc.

Imaging Characteristics of Children with Auditory Neuropathy Spectrum Disorder

PubMed Central

Roche, Joseph P.; Huang, Benjamin Y.; Castillo, Mauricio; Bassim, Marc K.; Adunka, Oliver F.; Buchman, Craig A.

2013-01-01

Objective To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD). Design Retrospective medical records review and analysis of both temporal bone computed tomography (CT) and magnetic resonance images (MRI) in from children with the diagnosis of ANSD. Setting Tertiary referral center. Patients 118 children with the electrophysiological characteristics of ANSD with available imaging studies for review. Interventions Two neuroradiologists and a neurotologist reviewed each study and consensus descriptions were established. Main outcome measures The type and number of imaging findings were tabulated. Results Sixty-eight (64%) MRIs revealed at least one imaging abnormality while selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n=51; 28% of 183 nerves), brain abnormalities (n=42; 40% of 106 brains) and prominent temporal horns (n=33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n=13; 31%). Conclusions MRI will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Since many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we believe that MRI should be the initial imaging study of choice for children with ANSD. PMID:20593543

Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

PubMed

Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

2001-01-01

The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

Cadaveric feasibility study of da Vinci Si-assisted cochlear implant with augmented visual navigation for otologic surgery.

PubMed

Liu, Wen P; Azizian, Mahdi; Sorger, Jonathan; Taylor, Russell H; Reilly, Brian K; Cleary, Kevin; Preciado, Diego

2014-03-01

To our knowledge, this is the first reported cadaveric feasibility study of a master-slave-assisted cochlear implant procedure in the otolaryngology-head and neck surgery field using the da Vinci Si system (da Vinci Surgical System; Intuitive Surgical, Inc). We describe the surgical workflow adaptations using a minimally invasive system and image guidance integrating intraoperative cone beam computed tomography through augmented reality. To test the feasibility of da Vinci Si-assisted cochlear implant surgery with augmented reality, with visualization of critical structures and facilitation with precise cochleostomy for electrode insertion. Cadaveric case study of bilateral cochlear implant approaches conducted at Intuitive Surgical Inc, Sunnyvale, California. Bilateral cadaveric mastoidectomies, posterior tympanostomies, and cochleostomies were performed using the da Vinci Si system on a single adult human donor cadaveric specimen. Radiographic confirmation of successful cochleostomies, placement of a phantom cochlear implant wire, and visual confirmation of critical anatomic structures (facial nerve, cochlea, and round window) in augmented stereoendoscopy. With a surgical mean time of 160 minutes per side, complete bilateral cochlear implant procedures were successfully performed with no violation of critical structures, notably the facial nerve, chorda tympani, sigmoid sinus, dura, or ossicles. Augmented reality image overlay of the facial nerve, round window position, and basal turn of the cochlea was precise. Postoperative cone beam computed tomography scans confirmed successful placement of the phantom implant electrode array into the basal turn of the cochlea. To our knowledge, this is the first study in the otolaryngology-head and neck surgery literature examining the use of master-slave-assisted cochleostomy with augmented reality for cochlear implants using the da Vinci Si system. The described system for cochleostomy has the potential to improve the surgeon's confidence, as well as surgical safety, efficiency, and precision by filtering tremor. The integration of augmented reality may be valuable for surgeons dealing with complex cases of congenital anatomic abnormality, for revision cochlear implant with distorted anatomy and poorly pneumatized mastoids, and as a method of interactive teaching. Further research into the cost-benefit ratio of da Vinci Si-assisted otologic surgery, as well as refinements of the proposed workflow, are required before considering clinical studies.

Stimulation from Cochlear Implant Electrodes Assists with Recovery from Asymmetric Perceptual Tilt: Evidence from the Subjective Visual Vertical Test

PubMed Central

Gnanasegaram, Joshua J.; Parkes, William J.; Cushing, Sharon L.; McKnight, Carmen L.; Papsin, Blake C.; Gordon, Karen A.

2016-01-01

Vestibular end organ impairment is highly prevalent in children who have sensorineural hearing loss (SNHL) rehabilitated with cochlear implants (CIs). As a result, spatial perception is likely to be impacted in this population. Of particular interest is the perception of visual vertical because it reflects a perceptual tilt in the roll axis and is sensitive to an imbalance in otolith function. The objectives of the present study were thus to identify abnormalities in perception of the vertical plane in children with SNHL and determine whether such abnormalities could be resolved with stimulation from the CI. Participants included 53 children (15.2 ± 4.0 years of age) with SNHL and vestibular loss, confirmed with vestibular evoked myogenic potential (VEMP) testing. Testing protocol was validated in a sample of nine young adults with normal hearing (28.8 ± 7.7 years). Perception of visual vertical was assessed using the static Subjective Visual Vertical (SVV) test performed with and without stimulation in the participants with cochleovestibular loss. Trains of electrical pulses were delivered by an electrode in the left and/or right ear. Asymmetric spatial orientation deficits were found in nearly half of the participants with CIs (24/53 [45%]). The abnormal perception in this cohort was exacerbated by visual tilts in the direction of their deficit. Electric pulse trains delivered using the CI shifted this abnormal perception towards center (i.e., normal; p = 0.007). Importantly, this benefit was realized regardless of which ear was stimulated. These results suggest a role for CI stimulation beyond the auditory system, in particular, for improving vestibular/balance function. PMID:27679562

[The experimental research of inner ear metabolism and electrical physiology of autoimmune sensorineural hearing loss].

PubMed

Tan, C; Cao, Y; Hu, P

1998-09-01

Inquire into the mechanism of inner ear pathological physiology in autoimmune sensorineural hearing loss (ASHL). With the auditory electric-physiological techniques and enzyme-histochemical method, the change of inner ear hearing function and enzyme activity were observed. These animals, which threshold of auditory nerve compound active potential (CAP) and cochlear microphonic potential(CM) heightening evidently, showed that the amplitude of endolymphatic potential(EP) (include-EP) bring down in various degrees, which was related to the change of the active of Na(+)-K(+)-ATPase and SDH in vascularis stria and endolymphatic sac. The abnormality of enzymes metabolism in inner ear tissues, which following autoimmune inflammation damage, is the pathological foundation of hearing dysfunction.

Cochlear pathology in chronic suppurative otitis media.

PubMed

Walby, A P; Barrera, A; Schuknecht, H F

1983-01-01

Chronic suppurative otitis media (COM) is reported to cause elevation of bone-conduction thresholds either by damage to cochlear sensorineural structures or by alteration in the mechanics of sound transmission in the ear. A retrospective study was made of the medical records of 87 patients with unilateral uncomplicated COM to document that abnormality in bone conduction does exist. In a separate study the cochlear pathology in 12 pairs of temporal bones with unilateral COM was studied by light microscopy. Infected ears showed higher than normal mean bone-conduction thresholds by amounts ranging from 1 dB at 500 Hz to 9.5 dB at 4,000 Hz. The temporal bones showed no greater loss of specialized sensorineural structures in infected ears than in normal control ears. Because there is no evidence that COM caused destruction of hair cells or cochlear neurons, alteration in the mechanics of sound transmission becomes a more plausible explanation for the hearing losses.

Chest MRI

MedlinePlus

... heart valves Heart defibrillator or pacemaker Inner ear (cochlear) implants Kidney disease or are on dialysis (you ... Diagnose abnormal growths in the chest Evaluate blood flow Show lymph nodes and blood vessels Show the ...

Experience Changes How Emotion in Music Is Judged: Evidence from Children Listening with Bilateral Cochlear Implants, Bimodal Devices, and Normal Hearing

PubMed Central

Papsin, Blake C.; Paludetti, Gaetano; Gordon, Karen A.

2015-01-01

Children using unilateral cochlear implants abnormally rely on tempo rather than mode cues to distinguish whether a musical piece is happy or sad. This led us to question how this judgment is affected by the type of experience in early auditory development. We hypothesized that judgments of the emotional content of music would vary by the type and duration of access to sound in early life due to deafness, altered perception of musical cues through new ways of using auditory prostheses bilaterally, and formal music training during childhood. Seventy-five participants completed the Montreal Emotion Identification Test. Thirty-three had normal hearing (aged 6.6 to 40.0 years) and 42 children had hearing loss and used bilateral auditory prostheses (31 bilaterally implanted and 11 unilaterally implanted with contralateral hearing aid use). Reaction time and accuracy were measured. Accurate judgment of emotion in music was achieved across ages and musical experience. Musical training accentuated the reliance on mode cues which developed with age in the normal hearing group. Degrading pitch cues through cochlear implant-mediated hearing induced greater reliance on tempo cues, but mode cues grew in salience when at least partial acoustic information was available through some residual hearing in the contralateral ear. Finally, when pitch cues were experimentally distorted to represent cochlear implant hearing, individuals with normal hearing (including those with musical training) switched to an abnormal dependence on tempo cues. The data indicate that, in a western culture, access to acoustic hearing in early life promotes a preference for mode rather than tempo cues which is enhanced by musical training. The challenge to these preferred strategies during cochlear implant hearing (simulated and real), regardless of musical training, suggests that access to pitch cues for children with hearing loss must be improved by preservation of residual hearing and improvements in cochlear implant technology. PMID:26317976

Experience Changes How Emotion in Music Is Judged: Evidence from Children Listening with Bilateral Cochlear Implants, Bimodal Devices, and Normal Hearing.

PubMed

Giannantonio, Sara; Polonenko, Melissa J; Papsin, Blake C; Paludetti, Gaetano; Gordon, Karen A

2015-01-01

Children using unilateral cochlear implants abnormally rely on tempo rather than mode cues to distinguish whether a musical piece is happy or sad. This led us to question how this judgment is affected by the type of experience in early auditory development. We hypothesized that judgments of the emotional content of music would vary by the type and duration of access to sound in early life due to deafness, altered perception of musical cues through new ways of using auditory prostheses bilaterally, and formal music training during childhood. Seventy-five participants completed the Montreal Emotion Identification Test. Thirty-three had normal hearing (aged 6.6 to 40.0 years) and 42 children had hearing loss and used bilateral auditory prostheses (31 bilaterally implanted and 11 unilaterally implanted with contralateral hearing aid use). Reaction time and accuracy were measured. Accurate judgment of emotion in music was achieved across ages and musical experience. Musical training accentuated the reliance on mode cues which developed with age in the normal hearing group. Degrading pitch cues through cochlear implant-mediated hearing induced greater reliance on tempo cues, but mode cues grew in salience when at least partial acoustic information was available through some residual hearing in the contralateral ear. Finally, when pitch cues were experimentally distorted to represent cochlear implant hearing, individuals with normal hearing (including those with musical training) switched to an abnormal dependence on tempo cues. The data indicate that, in a western culture, access to acoustic hearing in early life promotes a preference for mode rather than tempo cues which is enhanced by musical training. The challenge to these preferred strategies during cochlear implant hearing (simulated and real), regardless of musical training, suggests that access to pitch cues for children with hearing loss must be improved by preservation of residual hearing and improvements in cochlear implant technology.

Comprehensive analysis of cochlear implant failure: usefulness of clinical symptom-based algorithm combined with in situ integrity testing.

PubMed

Yamazaki, Hiroshi; O'Leary, Stephen; Moran, Michelle; Briggs, Robert

2014-04-01

Accurate diagnosis of cochlear implant failures is important for management; however, appropriate strategies to assess possible device failures are not always clear. The purpose of this study is to understand correlation between causes of device failure and the presenting clinical symptoms as well as results of in situ integrity testing and to propose effective strategies for diagnosis of device failure. Retrospective case review. Cochlear implant center at a tertiary referral hospital. Twenty-seven cases with suspected device failure of Cochlear Nucleus systems (excluding CI512 failures) on the basis of deterioration in auditory perception from January 2000 to September 2012 in the Melbourne cochlear implant clinic. Clinical presentations and types of abnormalities on in situ integrity testing were compared with modes of device failure detected by returned device analysis. Sudden deterioration in auditory perception was always observed in cases with "critical damage": either fracture of the integrated circuit or most or all of the electrode wires. Subacute or gradually progressive deterioration in auditory perception was significantly associated with a more limited number of broken electrode wires. Cochlear implant mediated auditory and nonauditory symptoms were significantly associated with an insulation problem. An algorithm based on the time course of deterioration in auditory perception and cochlear implant-mediated auditory and nonauditory symptoms was developed on the basis of these retrospective analyses, to help predict the mode of device failure. In situ integrity testing, which included close monitoring of device function in routine programming sessions as well as repeating the manufacturer's integrity test battery, was sensitive enough to detect malfunction in all suspected device failures, and each mode of device failure showed a characteristic abnormality on in situ integrity testing. Our clinical manifestation-based algorithm combined with in situ integrity testing may be useful for accurate diagnosis and appropriate management of device failure. Close monitoring of device function in routine programming sessions as well as repeating the manufacturer's integrity test battery is important if the initial in situ integrity testing is inconclusive because objective evidence of failure in the implanted device is essential to recommend explantation/reimplantation.

The Modified Rambo Transcanal Approach for Cochlear Implantation in CHARGE Syndrome.

PubMed

Wick, Cameron C; Moore, Amy M; Killeen, Daniel E; Isaacson, Brandon

2017-10-01

CHARGE syndrome is associated with a variety of temporal bone anomalies and deafness. The lack of surgical landmarks and facial nerve irregularities make cochlear implantation in this population a challenging endeavor. This study aims to describe a safe and efficacious transcanal approach for cochlear implantation that obviates the need to perform a mastoidectomy and facial recess. Three children with profound hearing loss secondary to CHARGE syndrome. Transcanal cochlear implantation with closure of the ear canal via a modified Rambo meatoplasty. Retrospective chart review of temporal bone anomalies associated with CHARGE syndrome, technical nuances of this transcanal approach, and cochlear implant outcomes. The mean patient age was 2.5 years (range 1.5-3.8 yr). Two were male and two were left ears. All patients had a hypoplastic mastoid, semicircular canal aplasia, and had some degree of cochlear dysplasia. A full cochlear implant insertion was achieved in all cases, even in the presence of grossly abnormal middle ear and facial nerve anatomy. There were no intraoperative or postoperative complications. The mean follow-up was 12.4 months (range, 3.9-25.2 mo). All three patients use their device daily. Their guardians report improved vocalization and environmental awareness. The modified Rambo transcanal approach provides a safe corridor for cochlear implantation in patients with CHARGE syndrome. This approach minimizes the anatomical variations associated with the syndrome and may reduce the risk of electrode extrusion. Implant outcomes in this patient population remain highly variable based on the patient's global cognitive capacity.

Perceptual consequences of normal and abnormal peripheral compression: Potential links between psychoacoustics and speech perception

NASA Astrophysics Data System (ADS)

Oxenham, Andrew J.; Rosengard, Peninah S.; Braida, Louis D.

2004-05-01

Cochlear damage can lead to a reduction in the overall amount of peripheral auditory compression, presumably due to outer hair cell (OHC) loss or dysfunction. The perceptual consequences of functional OHC loss include loudness recruitment and reduced dynamic range, poorer frequency selectivity, and poorer effective temporal resolution. These in turn may lead to a reduced ability to make use of spectral and temporal fluctuations in background noise when listening to a target sound, such as speech. We tested the effect of OHC function on speech reception in hearing-impaired listeners by comparing psychoacoustic measures of cochlear compression and sentence recognition in a variety of noise backgrounds. In line with earlier studies, we found weak (nonsignificant) correlations between the psychoacoustic tasks and speech reception thresholds in quiet or in steady-state noise. However, when spectral and temporal fluctuations were introduced in the masker, speech reception improved to an extent that was well predicted by the psychoacoustic measures. Thus, our initial results suggest a strong relationship between measures of cochlear compression and the ability of listeners to take advantage of spectral and temporal masker fluctuations in recognizing speech. [Work supported by NIH Grants Nos. R01DC03909, T32DC00038, and R01DC00117.

Large vestibular aqueduct syndrome: Impedance changes over time with different cochlear implant electrode arrays.

PubMed

Powell, Harry R F; Birman, Catherine S

2015-01-01

The aim of this study was to assess if large vestibular aqueduct syndrome (LVAS), with the increase in perilymphatic pressure, affects impedance changes over time with different types of Cochlear(®) implant electrode arrays Contour, Straight, and CI 422. To report speech perception outcomes for these cochlear implant recipients. Retrospective case review of impedance levels and categories of auditory performance. Impedance data were collected at switch on, 1 month, 3, 6, 12, and 24 months after cochlear implantation and compared with control (non-LVAS cochlear implant recipient) data for each array type. Forty-seven patients with exclusive LVAS and no other vestibulocochlear abnormalities or other identifiable cause of deafness were eligible for inclusion in the study. In LVAS patients, there was a significant difference in impedance between the three types of device (P < 0.0001). Time since switch on was associated with a decrease in impedance for all three devices (P < 0.0001). The mean impedance reduced between switch on and 1 month and remained relatively constant thereafter. Sound variation with softening of sounds was seen in four CI 422 (Straight Research Array) recipients due to ongoing fluctuations in electrode compliance. For all three array types, there was no significant difference in the mean impedance between the LVAS patients and controls over the first 12 months. In keeping with previous studies cochlear implant recipients with LVAS hear very well through the cochlear implant.

Ion flow in cochlear hair cells and the regulation of hearing sensitivity.

PubMed

Patuzzi, Robert

2011-10-01

This paper discusses how ion transport proteins in the hair cells of the mammalian cochlea work to produce a sensitive but stable hearing organ. The transport proteins in the inner and outer hair cells are summarized (including their current voltage characteristics), and the roles of these proteins in determining intracellular Ca(2+), membrane potential, and ultimately cochlear sensitivity are discussed. The paper also discusses the role of the Ca(2+) sequestration sacs in outer hair cells in the autoregulation of hair cell membrane potential and cochlear gain, and how the underdamped control of Ca(2+) within these sacs may produce the observed slow oscillations in cochlear sensitivity and otoacoustic emissions after cochlear perturbations, including perilymphatic perfusions and prolonged low-frequency tones. The relative insensitivity of cochlear gain to short-term changes in the endocochlear potential is also discussed. Copyright © 2011 Elsevier B.V. All rights reserved.

Electric charge requirements of pediatric cochlear implant recipients enrolled in the Childhood Development After Cochlear Implantation study.

PubMed

Zwolan, Teresa A; O'Sullivan, Mary Beth; Fink, Nancy E; Niparko, John K

2008-02-01

To evaluate mapping characteristics of children with cochlear implants who are enrolled in the Childhood Development After Cochlear Implantation (CDACI) multicenter study. Longitudinal evaluation during 24 months of speech processor maps of children with cochlear implants prospectively enrolled in the study. Six tertiary referral centers. One hundred eighty-eight children enrolled in the CDACI study who were 5 years old or younger at the time of enrollment. Of these children, 184 received unilateral implants, and 4 received simultaneous bilateral implants. Children attended regular mapping sessions at their implant clinic as part of the study protocol. Maps were examined for each subject at 4 different time intervals: at device activation and 6, 12, and 24 months postactivation. Mean C/M levels (in charge per phase) were compared for 4 different time intervals, for 3 different devices, for 6 different implant centers, and for children with normal and abnormal cochleae. All 3 types of implant devices demonstrate significant increases in C/M levels between device activation and the 24-month appointment. Significant differences in mean C/M levels were noted between devices. Children with cochlear anomalies demonstrate significantly greater C/M levels than children with normal cochleae. The CDACI study has enabled us to evaluate the mapping characteristics of pediatric patients who use 3 different devices and were implanted at a variety of implant centers. Analysis of such data enables us to better understand the mapping characteristics of children with cochlear implants.

Multichannel cochlear implantation in the scala vestibuli.

PubMed

Lin, Karen; Marrinan, Michelle S; Waltzman, Susan B; Roland, J Thomas

2006-08-01

Sensorineural hearing loss resulting from otosclerosis, meningitis, chronic otitis media, autoimmune ear disease, and trauma can be associated with partial or total obstruction of the cochlear scalae. Multichannel cochlear implantation may be difficult in a cochlea with an obstructed scala tympani. The purpose of this study is to determine the safety and efficacy of scala tympani electrode insertion. Retrospective chart review. Academic medical center. Eight children and adults with profound sensorineural hearing loss who underwent cochlear implantation with known scala vestibuli electrode array insertion were subjects for this study. Eight study subjects underwent implantation: five with the Nucleus 24RCS (Contour) device and three with the Nucleus 24M device. Imaging findings, operative findings, and age-appropriate speech perception testing. All patients had full electrode insertion. Various obstructive patterns on computed tomography and magnetic resonance imaging were found, and there was a range of speech perception results. All but one patient improved based on age-appropriate monosyllabic word and sentence tests. Scala vestibuli multielectrode insertion is a viable alternative when scala tympani insertion is not possible because of abnormal anatomy or anatomical changes secondary to disease or previous implantation. We will also present an algorithm of options for decision making for implantation when encountering cochlear obstruction and difficult electrode insertion.

Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.

PubMed

Liberman, M Charles; Kujawa, Sharon G

2017-06-01

Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans. Copyright © 2017 Elsevier B.V. All rights reserved.

Effect of simulated bilateral cochlear distortion on speech discrimination in normal subjects.

PubMed

Hood, J D; Prasher, D K

1990-01-01

Bilateral sensorineural hearing loss may introduce grossly dissimilar cochlear distortion at the two ears, causing abnormal demands to be made upon the cortical analytical centres which normally receive congruent information. As a result, the prescription of binaural hearing aids may be a handicap rather than a help. In order to explore this possibility, 10 normal subjects were presented with simulated, dissimilar cochlear distortion at the two ears. Discrimination scores with binaural presentation were poorer than the best monaural score and there were clear indications that in the former, subjects selectively attended to one ear and neglected the other. In contrast, binaural presentation of the same simulated distortion resulted in a significant improvement, compared with the monaural discrimination score. Inability of the cortex to contend with discongruent speech input from the two ears may be a factor contributing to the rejection of binaural hearing aids in some individuals.

Cochlear dysfunction in hyperuricemia: otoacoustic emission analysis.

PubMed

Hamed, Sherifa A; El-Attar, Amal M

2010-01-01

The objective of this study is to provide evidence that primary hyperuricemia is associated with cochlear dysfunction as other metabolic diseases that interfere with cell metabolism. Cochlear function was evaluated in 25 subjects with asymptomatic hyperuricemia using routine diagnostic audiometry along with transient evoked and distortion product otoacoustic emissions (TEOAE and DPOAE, respectively). To support the notion that vascular compromise was a significant underlying factor for such cochlear dysfunction, we assessed vascular anatomical and functional states through measuring the common carotid artery intima-media thickness and flow velocity of the basal intracranial vessels. Compared with control subjects, reduced response levels of TEOAEs (P < .01) and amplitudes of DPOAEs (P < .001) were detected at higher frequencies. The reduced DPOAE levels at 5 kHz and TEOAEs at 4 kHz correlated significantly with uric acid (P < .05; P < .01), patients' age (P < .06; P < .05), duration since diagnosis of hyperuricemia (P < .05; P < .001), common carotid artery intima-media thickness (P < .05), mean flow velocities of middle cerebral arteries (P < .05), and vertebral arteries (P < .01). Multivariate analysis showed that the abnormalities at higher frequencies were significantly correlated with the duration and degree of hyperuricemia. These data suggest that subclinical changes in cochlear function are associated with hyperuricemia. They support the usefulness of otoacoustic emissions in early detection of cochlear dysfunction. It is possible that hyperuricemia could be accompanied by increased stiffness and/or compromise of blood supply of the outer hair cells, which will impair their electromotile response. Copyright (c) 2010 Elsevier Inc. All rights reserved.

Audiological manifestations in mitochondrial encephalomyopathy lactic acidosis and stroke like episodes (MELAS) syndrome.

PubMed

Vandana, V P; Bindu, Parayil Sankaran; Sonam, Kothari; Govindaraj, Periyasamy; Taly, Arun B; Gayathri, Narayanappa; Chiplunkar, Shwetha; Govindaraju, Chikkanna; Arvinda, H R; Nagappa, Madhu; Sinha, Sanjib; Thangaraj, Kumarasamy

2016-09-01

Reports of audiological manifestations in specific subgroups of mitochondrial disorders are limited. This study aims to describe the audiological findings in patients with MELAS syndrome and m.3243A>G mutation. Audiological evaluation was carried out in eight patients with confirmed MELAS syndrome and m.3243A>G mutation. The evaluation included a complete neurological evaluation, pure tone audiometry (n=8), otoacoustic emissions (n=8) and brainstem evoked response audiometry (n=6), magnetic resonance imaging (n=8) and muscle biospy (n=6). Eight patients (Age range: 5-45 years; M:F-1:3) including six children and two adults underwent formal audiological evaluation. Five patients had hearing loss; of these two had "subclinical hearing loss", one had moderate and two had severe hearing loss. The abnormalities included abnormal audiometry (n=5), otoacoustic emission testing (n=7) and absent brainstem auditory evoked responses (n=1). The findings were suggestive of cochlear involvement in four and retrocochlear in one. This study shows that hearing loss of both cochlear and retrocochlear origin occurs in patients with MELAS and may be subclinical. Early referrals for audiological evaluation is warranted to recognize the subclinical hearing loss in these patients. The therapeutic implications include early interventions in the form of hearing aids, cochlear implants and cautioning the physicians for avoidance of aminoglycosides. Copyright © 2016 Elsevier B.V. All rights reserved.

Fingolimod (FTY-720) is Capable of Reversing Tumor Necrosis Factor Induced Decreases in Cochlear Blood Flow.

PubMed

Bertlich, Mattis; Ihler, Friedrich; Weiss, Bernhard G; Freytag, Saskia; Jakob, Mark; Strupp, Michael; Pellkofer, Hannah; Canis, Martin

2017-09-01

The potential of Fingolimod (FTY-720), a sphingosine-1-phosphate analogue, to revoke the changes in cochlear blood flow induced by tumor necrosis factor (TNF) was investigated. Impairment of cochlear blood flow has often been considered as the common final pathway of various inner ear pathologies. TNF, an ubiquitous cytokine, plays a major role in these pathologies, reducing cochlear blood flow via sphingosine-1-phosphate-signaling. Fifteen Dunkin-Hartley guinea pigs were randomly assigned to one of three groups (placebo/placebo, TNF/placebo, TNF/FTY-720). Cochlear microcirculation was quantified over 60 minutes by in vivo fluorescence microscopy before and after topical application of placebo or TNF (5 ng/ml) and after subsequent application of placebo or FTY-720 (200 μg/ml). Treatment with TNF led to a significant decrease of cochlear blood flow.Following this, application of placebo caused no significant changes while application of FTY-720 caused a significant rise in cochlear blood flow. FTY-720 is capable of reversing changes in cochlear blood flow induced by application of TNF. This makes FTY-720 a valid candidate for potential treatment of numerous inner ear pathologies.

Otosclerosis: Temporal Bone Pathology.

PubMed

Quesnel, Alicia M; Ishai, Reuven; McKenna, Michael J

2018-04-01

Otosclerosis is pathologically characterized by abnormal bony remodeling, which includes bone resorption, new bone deposition, and vascular proliferation in the temporal bone. Sensorineural hearing loss in otosclerosis is associated with extension of otosclerosis to the cochlear endosteum and deposition of collagen throughout the spiral ligament. Persistent or recurrent conductive hearing loss after stapedectomy has been associated with incomplete footplate fenestration, poor incus-prosthesis connection, and incus resorption in temporal bone specimens. Human temporal bone pathology has helped to define the role of computed tomography imaging for otosclerosis, confirming that computed tomography is highly sensitive for diagnosis, yet limited in assessing cochlear endosteal involvement. Copyright © 2017 Elsevier Inc. All rights reserved.

Anaesthesia Management in a Patient with Waardenburg Syndrome and Review of the Literature.

PubMed

Peker, Kevser; Ergil, Julide; Öztürk, İbrahim

2015-10-01

Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%-3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation.

Analysis of electrically evoked compound action potential of the auditory nerve in children with bilateral cochlear implants.

PubMed

Caldas, Fernanda Ferreira; Cardoso, Carolina Costa; Barreto, Monique Antunes de Souza Chelminski; Teixeira, Marina Santos; Hilgenberg, Anacléia Melo da Silva; Serra, Lucieny Silva Martins; Bahmad Junior, Fayez

2016-01-01

The cochlear implant device has the capacity to measure the electrically evoked compound action potential of the auditory nerve. The neural response telemetry is used in order to measure the electrically evoked compound action potential of the auditory nerve. To analyze the electrically evoked compound action potential, through the neural response telemetry, in children with bilateral cochlear implants. This is an analytical, prospective, longitudinal, historical cohort study. Six children, aged 1-4 years, with bilateral cochlear implant were assessed at five different intervals during their first year of cochlear implant use. There were significant differences in follow-up time (p=0.0082) and electrode position (p=0.0019) in the T-NRT measure. There was a significant difference in the interaction between time of follow-up and electrode position (p=0.0143) when measuring the N1-P1 wave amplitude between the three electrodes at each time of follow-up. The electrically evoked compound action potential measurement using neural response telemetry in children with bilateral cochlear implants during the first year of follow-up was effective in demonstrating the synchronized bilateral development of the peripheral auditory pathways in the studied population. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Long latency auditory evoked potentials in children with cochlear implants: systematic review.

PubMed

Silva, Liliane Aparecida Fagundes; Couto, Maria Inês Vieira; Matas, Carla Gentile; Carvalho, Ana Claudia Martinho de

2013-11-25

The aim of this study was to analyze the findings on Cortical Auditory Evoked Potentials in children with cochlear implant through a systematic literature review. After formulation of research question and search of studies in four data bases with the following descriptors: electrophysiology (eletrofisiologia), cochlear implantation (implante coclear), child (criança), neuronal plasticity (plasticidade neuronal) and audiology (audiologia), were selected articles (original and complete) published between 2002 and 2013 in Brazilian Portuguese or English. A total of 208 studies were found; however, only 13 contemplated the established criteria and were further analyzed; was made data extraction for analysis of methodology and content of the studies. The results described suggest rapid changes in P1 component of Cortical Auditory Evoked Potentials in children with cochlear implants. Although there are few studies on the theme, cochlear implant has been shown to produce effective changes in central auditory path ways especially in children implanted before 3 years and 6 months of age.

Verbal Working Memory in Children With Cochlear Implants

PubMed Central

Caldwell-Tarr, Amanda; Low, Keri E.; Lowenstein, Joanna H.

2017-01-01

Purpose Verbal working memory in children with cochlear implants and children with normal hearing was examined. Participants Ninety-three fourth graders (47 with normal hearing, 46 with cochlear implants) participated, all of whom were in a longitudinal study and had working memory assessed 2 years earlier. Method A dual-component model of working memory was adopted, and a serial recall task measured storage and processing. Potential predictor variables were phonological awareness, vocabulary knowledge, nonverbal IQ, and several treatment variables. Potential dependent functions were literacy, expressive language, and speech-in-noise recognition. Results Children with cochlear implants showed deficits in storage and processing, similar in size to those at second grade. Predictors of verbal working memory differed across groups: Phonological awareness explained the most variance in children with normal hearing; vocabulary explained the most variance in children with cochlear implants. Treatment variables explained little of the variance. Where potentially dependent functions were concerned, verbal working memory accounted for little variance once the variance explained by other predictors was removed. Conclusions The verbal working memory deficits of children with cochlear implants arise due to signal degradation, which limits their abilities to acquire phonological awareness. That hinders their abilities to store items using a phonological code. PMID:29075747

Cochlear implantation in patient with Dandy-walker syndrome.

PubMed

de Oliveira, Adriana Kosma Pires; Hamerschmidt, Rogerio; Mocelin, Marcos; Rezende, Rodrigo K

2012-07-01

 Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment.  To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation.  CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of "Dandy-Walker syndrome" for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality.

Noise alters hair-bundle mechanics at the cochlear apex

NASA Astrophysics Data System (ADS)

Strimbu, C. Elliott; Fridberger, Anders

2015-12-01

Exposure to loud sounds can lead to both permanent and short term changes in auditory sensitivity. Permanent hearing loss is often associated with gross changes in cochlear morphology including the loss of hair cells and auditory nerve fibers while the mechanisms of short term threshold shifts are much less well understood and may vary at different locations across the cochlea. Previous reports suggest that exposure to loud sounds leads to a decrease in the cochlear microphonic potential and in the stiffness of the organ of Corti. Because the cochlear microphonic reflects changes in the membrane potential of the hair cells, this suggests that hair-bundle motion should be reversibly altered following exposure to loud sounds. Using an in vitro preparation of the guinea pig temporal bone we investigate changes in the micro-mechanical response near the cochlear apex following a brief (up to 10 - 20 minutes) exposure to loud (˜ 120 dB) tones near the best frequency at this location. We use time-resolved confocal imaging to record the motion of outer hair cell bundles before and after acoustic overstimulation. We have also recorded larger-scale structural views of the organ of Corti before and after exposure to the loud sound. Conventional electrophysiological techniques are used measure the cochlear microphonic potential. As has been previously reported, following acoustic overexposure the cochlear microphonic declines in value and typically recovers on the order of 30 - 60 minutes. Hair-bundle trajectories are affected following the loud sound and typically recover on a somewhat faster time scale than the microphonic potential, although the results vary considerably across preparations. Preliminary results also suggest reversible changes in the hair cell's resting potential following the loud sound.

Beneficial auditory and cognitive effects of auditory brainstem implantation in children.

PubMed

Colletti, Liliana

2007-09-01

This preliminary study demonstrates the development of hearing ability and shows that there is a significant improvement in some cognitive parameters related to selective visual/spatial attention and to fluid or multisensory reasoning, in children fitted with auditory brainstem implantation (ABI). The improvement in cognitive paramenters is due to several factors, among which there is certainly, as demonstrated in the literature on a cochlear implants (CIs), the activation of the auditory sensory canal, which was previously absent. The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities with associated cognitive deficits should not be excluded from ABI implantation. The indications for ABI have been extended over the last 10 years to adults with non-tumoral (NT) cochlear or cochlear nerve abnormalities that cannot benefit from CI. We demonstrated that the ABI with surface electrodes may provide sufficient stimulation of the central auditory system in adults for open set speech recognition. These favourable results motivated us to extend ABI indications to children with profound hearing loss who were not candidates for a CI. This study investigated the performances of young deaf children undergoing ABI, in terms of their auditory perceptual development and their non-verbal cognitive abilities. In our department from 2000 to 2006, 24 children aged 14 months to 16 years received an ABI for different tumour and non-tumour diseases. Two children had NF2 tumours. Eighteen children had bilateral cochlear nerve aplasia. In this group, nine children had associated cochlear malformations, two had unilateral facial nerve agenesia and two had combined microtia, aural atresia and middle ear malformations. Four of these children had previously been fitted elsewhere with a CI with no auditory results. One child had bilateral incomplete cochlear partition (type II); one child, who had previously been fitted unsuccessfully elsewhere with a CI, had auditory neuropathy; one child showed total cochlear ossification bilaterally due to meningitis; and one child had profound hearing loss with cochlear fractures after a head injury. Twelve of these children had multiple associated psychomotor handicaps. The retrosigmoid approach was used in all children. Intraoperative electrical auditory brainstem responses (EABRs) and postoperative EABRs and electrical middle latency responses (EMLRs) were performed. Perceptual auditory abilities were evaluated with the Evaluation of Auditory Responses to Speech (EARS) battery - the Listening Progress Profile (LIP), the Meaningful Auditory Integration Scale (MAIS), the Meaningful Use of Speech Scale (MUSS) - and the Category of Auditory Performance (CAP). Cognitive evaluation was performed on seven children using the Leiter International Performance Scale - Revised (LIPS-R) test with the following subtests: Figure ground, Form completion, Sequential order and Repeated pattern. No postoperative complications were observed. All children consistently used their devices for >75% of waking hours and had environmental sound awareness and utterance of words and simple sentences. Their CAP scores ranged from 1 to 7 (average =4); with MAIS they scored 2-97.5% (average =38%); MUSS scores ranged from 5 to 100% (average =49%) and LIP scores from 5 to 100% (average =45%). Owing to associated disabilities, 12 children were given other therapies (e.g. physical therapy and counselling) in addition to speech and aural rehabilitation therapy. Scores for two of the four subtests of LIPS-R in this study increased significantly during the first year of auditory brainstem implant use in all seven children selected for cognitive evaluation.

Preventing Ototoxic Synergy of Prior Noise Trauma During Aminoglycoside Therapy

DTIC Science & Technology

2015-12-01

cochlear blood flow . Hearing Research 313, 38-46 (2014). 12. Koo, J.-W. et al. Endotoxemia-mediated inflammation potentiates cochlear uptake of...event in response to a need for higher cochlear blood flow . A 3.7% dilation was adequate for a 20% increase of blood flow 10. On the contrary...summation of the two insults. We have found that prior sound exposure enhances cochlear uptake of aminoglycosides, providing a mechanistic basis for the

Isolated cochlear neuritis from varicella reactivation mimicking a vestibular schwannoma.

PubMed

Goodale, Adam D; Golub, Justin S; Cornelius, Rebecca S; Samy, Ravi N

2016-09-01

We present a case of a patient with progressive unilateral sensorineural hearing loss and tinnitus with internal auditory canal enhancement on magnetic resonance imaging (MRI) secondary to isolated cochlear neuritis from varicella reactivation. MRI following antiviral treatment showed resolution of enhancement. Varicella reactivation is commonly seen in the form of Ramsay Hunt syndrome, which is known to produce abnormal MRI enhancement from facial and vestibulocochlear neuritis; however, its characteristic clinical signs aid the diagnosis. This case is unique in that the only manifestation of varicella infection was unilateral hearing loss. This case outlines the importance of maintaining a broad differential diagnosis in the evaluation of unilateral hearing loss as well as recognizing the limited specificity of MRI.

Anaesthesia Management in a Patient with Waardenburg Syndrome and Review of the Literature

PubMed Central

Peker, Kevser; Ergil, Julide; Öztürk, İbrahim

2015-01-01

Waardenburg syndrome is a rare autosomal dominant disease that may cause hearing loss, pigmentary abnormalities, neurocristopathy and partial albinism. Incidence is estimated as 2%–3% among the cases of congenital deafness and 1/42,000 of the general population. Children with Waardenburg syndrome usually require anaesthesia for the cochlear implant operation in early age. The features of the syndrome that may bear importance for anaesthetic management are laryngomalacia, multiple muscle contractures, limited neck movements, cyanotic cardiopathy and electrolyte imbalance. Patients with Waardenburg syndrome stand for difficult airway. We aimed to report anaesthetic management of a child with Waardenburg syndrome who underwent surgery for cochlear implantation. PMID:27366529

Cochlear potential difference between endolymph fluid and the hair cell's interior: a retold interpretation based on the Goldman equation.

PubMed

Kurbel, Sven; Borzan, Vladimir; Golem, Hilda; Dinjar, Kristijan

2017-02-01

Reported cochlear potential values of near 150 mV are often attributed to endolymph itself, although membrane potentials result from ion fluxes across the adjacent semipermeable membranes due to concentration gradients. Since any two fluids separated by a semipermeable membrane develop potential due to differences in solute concentrations, a proposed interpretation here is that positive potential emanates from the Reissner membrane due to small influx of sodium from perilymph to endolymph. Basolateral hair cell membranes leak potassium into the interstitial fluid and this negative potential inside hair cells further augments the electric gradient of cochlear potential. Taken together as a sum, these two potentials are near the reported values of cochlear potential. This is based on reported data for cochlear fluids used for the calculation of Nernst and Goldman potentials. The reported positive potential of Reissner membrane can be explained almost entirely by the traffic of Na+ that enters endolymph through this membrane. At the apical membrane of hair cells, acoustic stimulation modulates stereocillia permeability to potassium. Potassium concentration gradients on the apical membrane are low (the calculated Nernst value is <+3 mV), suggesting that the potassium current is not caused by the local potassium concentration gradient, but an electric field between the positive sodium generated potential on the Reissner membrane and negative inside hair cells. Potassium is forced by this overall electric field to enter hair cells when stereocilia are permeable due to mechanical bending. Copyright© by the Medical Assotiation of Zenica-Doboj Canton.

Electrophysiological evidence for altered visual, but not auditory, selective attention in adolescent cochlear implant users.

PubMed

Harris, Jill; Kamke, Marc R

2014-11-01

Selective attention fundamentally alters sensory perception, but little is known about the functioning of attention in individuals who use a cochlear implant. This study aimed to investigate visual and auditory attention in adolescent cochlear implant users. Event related potentials were used to investigate the influence of attention on visual and auditory evoked potentials in six cochlear implant users and age-matched normally-hearing children. Participants were presented with streams of alternating visual and auditory stimuli in an oddball paradigm: each modality contained frequently presented 'standard' and infrequent 'deviant' stimuli. Across different blocks attention was directed to either the visual or auditory modality. For the visual stimuli attention boosted the early N1 potential, but this effect was larger for cochlear implant users. Attention was also associated with a later P3 component for the visual deviant stimulus, but there was no difference between groups in the later attention effects. For the auditory stimuli, attention was associated with a decrease in N1 latency as well as a robust P3 for the deviant tone. Importantly, there was no difference between groups in these auditory attention effects. The results suggest that basic mechanisms of auditory attention are largely normal in children who are proficient cochlear implant users, but that visual attention may be altered. Ultimately, a better understanding of how selective attention influences sensory perception in cochlear implant users will be important for optimising habilitation strategies. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Cortical auditory evoked potentials in the assessment of auditory neuropathy: two case studies.

PubMed

Pearce, Wendy; Golding, Maryanne; Dillon, Harvey

2007-05-01

Infants with auditory neuropathy and possible hearing impairment are being identified at very young ages through the implementation of hearing screening programs. The diagnosis is commonly based on evidence of normal cochlear function but abnormal brainstem function. This lack of normal brainstem function is highly problematic when prescribing amplification in young infants because prescriptive formulae require the input of hearing thresholds that are normally estimated from auditory brainstem responses to tonal stimuli. Without this information, there is great uncertainty surrounding the final fitting. Cortical auditory evoked potentials may, however, still be evident and reliably recorded to speech stimuli presented at conversational levels. The case studies of two infants are presented that demonstrate how these higher order electrophysiological responses may be utilized in the audiological management of some infants with auditory neuropathy.

Perception of Binaural Cues Develops in Children Who Are Deaf through Bilateral Cochlear Implantation

PubMed Central

Gordon, Karen A.; Deighton, Michael R.; Abbasalipour, Parvaneh; Papsin, Blake C.

2014-01-01

There are significant challenges to restoring binaural hearing to children who have been deaf from an early age. The uncoordinated and poor temporal information available from cochlear implants distorts perception of interaural timing differences normally important for sound localization and listening in noise. Moreover, binaural development can be compromised by bilateral and unilateral auditory deprivation. Here, we studied perception of both interaural level and timing differences in 79 children/adolescents using bilateral cochlear implants and 16 peers with normal hearing. They were asked on which side of their head they heard unilaterally or bilaterally presented click- or electrical pulse- trains. Interaural level cues were identified by most participants including adolescents with long periods of unilateral cochlear implant use and little bilateral implant experience. Interaural timing cues were not detected by new bilateral adolescent users, consistent with previous evidence. Evidence of binaural timing detection was, for the first time, found in children who had much longer implant experience but it was marked by poorer than normal sensitivity and abnormally strong dependence on current level differences between implants. In addition, children with prior unilateral implant use showed a higher proportion of responses to their first implanted sides than children implanted simultaneously. These data indicate that there are functional repercussions of developing binaural hearing through bilateral cochlear implants, particularly when provided sequentially; nonetheless, children have an opportunity to use these devices to hear better in noise and gain spatial hearing. PMID:25531107

Progress in Cochlear Physiology after Békésy

PubMed Central

Guinan, John J.; Salt, Alec; Cheatham, Mary Ann

2012-01-01

In the fifty years since Békésy was awarded the Nobel Prize, cochlear physiology has blossomed. Many topics that are now current are things Békésy could not have imagined. In this review we start by describing progress in understanding the origin of cochlear gross potentials, particularly the cochlear microphonic, an area in which Békésy had extensive experience. We then review progress in areas of cochlear physiology that were mostly unknown to Békésy, including: (1) stereocilia mechano-electrical transduction, force production, and response amplification, (2) outer hair cell (OHC) somatic motility and its molecular basis in prestin, (3) cochlear amplification and related micromechanics, including the evidence that prestin is the main motor for cochlear amplification, (4) the influence of the tectorial membrane, (5) cochlear micromechanics and the mechanical drives to inner hair cell stereocilia, (6) otoacoustic emissions, and (7) olivocochlear efferents and their influence on cochlear physiology. We then return to a subject that Békésy knew well: cochlear fluids and standing currents, as well as our present understanding of energy dependence on the lateral wall of the cochlea. Finally, we touch on cochlear pathologies including noise damage and aging, with an emphasis on where the field might go in the future. PMID:22633944

Visualization of spiral ganglion neurites within the scala tympani with a cochlear implant in situ

PubMed Central

Chikar, Jennifer A.; Batts, Shelley A.; Pfingst, Bryan E.; Raphael, Yehoash

2009-01-01

Current cochlear histology methods do not allow in situ processing of cochlear implants. The metal components of the implant preclude standard embedding and mid-modiolar sectioning, and whole mounts do not have the spatial resolution needed to view the implant within the scala tympani. One focus of recent auditory research is the regeneration of structures within the cochlea, particularly the ganglion cells and their processes, and there are multiple potential benefits to cochlear implant users from this work. To facilitate experimental investigations of auditory nerve regeneration performed in conjunction with cochlear implantation, it is critical to visualize the cochlear tissue and the implant together to determine if the nerve has made contact with the implant. This paper presents a novel histological technique that enables simultaneous visualization of the in situ cochlear implant and neurofilament – labeled nerve processes within the scala tympani, and the spatial relationship between them. PMID:19428528

Visualization of spiral ganglion neurites within the scala tympani with a cochlear implant in situ.

PubMed

Chikar, Jennifer A; Batts, Shelley A; Pfingst, Bryan E; Raphael, Yehoash

2009-05-15

Current cochlear histology methods do not allow in situ processing of cochlear implants. The metal components of the implant preclude standard embedding and mid-modiolar sectioning, and whole mounts do not have the spatial resolution needed to view the implant within the scala tympani. One focus of recent auditory research is the regeneration of structures within the cochlea, particularly the ganglion cells and their processes, and there are multiple potential benefits to cochlear implant users from this work. To facilitate experimental investigations of auditory nerve regeneration performed in conjunction with cochlear implantation, it is critical to visualize the cochlear tissue and the implant together to determine if the nerve has made contact with the implant. This paper presents a novel histological technique that enables simultaneous visualization of the in situ cochlear implant and neurofilament-labeled nerve processes within the scala tympani, and the spatial relationship between them.

Cochlear implantation in patient with Dandy-walker syndrome

PubMed Central

de Oliveira, Adriana Kosma Pires; Hamerschmidt, Rogerio; Mocelin, Marcos; Rezende, Rodrigo K.

2012-01-01

Summary Introduction: Dandy Walker Syndrome is a congenital abnormality in the central nervous system, characterized by a deficiency in the development of middle cerebelar structures, cystic dilatation of the posterior pit communicating with the fourth ventricle and upward shift of the transverse sinuses, tentorium and dyes. Among the clinical signs are occipital protuberances, a progressive increase of the skull, bowing before the fontanels, papilledema, ataxia, gait disturbances, nystagmus, and intellectual impairment. Objectives: To describe a case of female patient, 13 years old with a diagnosis of this syndrome and bilateral hearing loss underwent cochlear implant surgery under local anesthesia and sedation. Case Report: CGS, 13 years old female was referred to the Otolaryngological Department of Otolaryngology Institute of Parana with a diagnosis of “Dandy-Walker syndrome” for Otolaryngological evaluation for bilateral hearing loss with no response to the use of hearing aids. Final Comments: The field of cochlear implants is growing rapidly. We believe that the presence of Dandy-Walker syndrome cannot be considered a contraindication to the performance of cochlear implant surgery, and there were no surgical complications due to neurological disorders with very favorable results for the patient who exhibits excellent discrimination. It has less need for lip reading with improvement in speech quality. PMID:25991966

CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery.

PubMed

Vesseur, A C; Verbist, B M; Westerlaan, H E; Kloostra, F J J; Admiraal, R J C; van Ravenswaaij-Arts, C M A; Free, R H; Mylanus, E A M

2016-12-01

To provide an overview of anomalies of the temporal bone in CHARGE syndrome relevant to cochlear implantation (CI), anatomical structures of the temporal bone and the respective genotypes were analysed. In this retrospective study, 42 CTs of the temporal bone of 42 patients with CHARGE syndrome were reviewed in consensus by two head-and-neck radiologists and two otological surgeons. Anatomical structures of the temporal bone were evaluated and correlated with genetic data. Abnormalities that might affect CI surgery were seen, such as a vascular structure, a petrosquamosal sinus (13 %), an underdeveloped mastoid (8 %) and an aberrant course of the facial nerve crossing the round window (9 %) and/or the promontory (18 %). The appearance of the inner ear varied widely: in 77 % of patients all semicircular canals were absent and the cochlea varied from normal to hypoplastic. A stenotic cochlear aperture was observed in 37 %. The middle ear was often affected with a stenotic round (14 %) or oval window (71 %). More anomalies were observed in patients with truncating mutations than with non-truncating mutations. Temporal bone findings in CHARGE syndrome vary widely. Vascular variants, aberrant route of the facial nerve, an underdeveloped mastoid, aplasia of the semicircular canals, and stenotic round window may complicate cochlear implantation.

Bilateral cochlear implantation in the ferret: A novel animal model for behavioral studies

PubMed Central

Hartley, Douglas E.H.; Vongpaisal, Tara; Xu, Jin; Shepherd, Robert K.; King, Andrew J.; Isaiah, Amal

2010-01-01

Bilateral cochlear implantation has recently been introduced with the aim of improving both speech perception in background noise and sound localization. Although evidence suggests that binaural perception is possible with two cochlear implants, results in humans are variable. To explore potential contributing factors to these variable outcomes, we have developed a behavioral animal model of bilateral cochlear implantation in a novel species, the ferret. Although ferrets are ideally suited to psychophysical and physiological assessments of binaural hearing, cochlear implantation has not been previously described in this species. This paper describes the techniques of deafening with aminoglycoside administration, surgical implantation of an intracochlear array and chronic intracochlear electrical stimulation with monitoring for electrode integrity and efficacy of stimulation. Experiments have been presented elsewhere to show that the model can be used to study behavioral and electrophysiological measures of binaural hearing in chronically implanted animals. This paper demonstrates that cochlear implantation and chronic intracochlear electrical stimulation are both safe and effective in ferrets, opening up the possibility of using this model to study potential protective effects of bilateral cochlear implantation on the developing central auditory pathway. Since ferrets can be used to assess psychophysical and physiological aspects of hearing along with the structure of the auditory pathway in the same animals, we anticipate that this model will help develop novel neuroprosthetic therapies for use in humans. PMID:20576507

The diagnostic value of earlier and later components of Vestibular Evoked Myogenic Potentials (VEMP) in multiple sclerosis.

PubMed

Eleftheriadou, A; Deftereos, S N; Zarikas, V; Panagopoulos, G; Sfetsos, S; Karageorgiou, C L; Ferekidou, E; Kandiloros, D; Korres, S

2009-01-01

To evaluate the ability of VEMP to disclose spatial dissemination of Multiple Sclerosis. Forty-six MS patients with auditory and/or vestibular symptoms were studied. Patients were divided in two groups. Group 1 included 24 patients with brainstem MRI findings, and Group 2 included 22 patients without MRI findings. VEMP and BAEP have been recorded and assessed. Abnormal p13n23 wave was observed in 50%, while unilateral absence or bilateral delay of the n34p44 in 43% of the patients. The overall diagnostic value considering abnormal cases suggested by both first and second VEMP waves was increased to 71%. Statistically significant differences revealed between patients and controls for p13 latency (p=0.018). The p13n23 was abnormal in 7 patients, although MRI scanning did not reveal brainstem lesions. In 9 out of 18 MS patients suffering from unilateral hearing loss, n34p44 was present in the unaffected ears and absent in the affected side, although p13n23 was normal. Abnormal VEMP imply the presence of lesions undetected by MRI neuroimaging, which verifies the diagnostic value of the method. Unilateral absence of n34p44 complex was related with sensorineural hearing loss, supporting the hypothesis that n34p44 is of cochlear origin.

Cochlear implantation in chronic demyelinating inflammatory polyneuropathy.

PubMed

Mowry, Sarah E; King, Sarah

2017-03-01

To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss. Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.

Ischemia-reperfusion injury of the cochlea: pharmacological strategies for cochlear protection and implications of glutamate and reactive oxygen species.

PubMed

Tabuchi, Keiji; Nishimura, Bungo; Tanaka, Shuho; Hayashi, Kentaro; Hirose, Yuki; Hara, Akira

2010-06-01

A large amount of energy produced by active aerobic metabolism is necessary for the cochlea to maintain its function. This makes the cochlea vulnerable to blockade of cochlear blood flow and interruption of the oxygen supply. Although certain forms of human idiopathic sudden sensorineural hearing loss reportedly arise from ischemic injury, the pathological mechanism of cochlear ischemia-reperfusion injury has not been fully elucidated. Recent animal studies have shed light on the mechanisms of cochlear ischemia-reperfusion injury. It will help in the understanding of the pathology of cochlear ischemia-reperfusion injury to classify this injury into ischemic injury and reperfusion injury. Excitotoxicity, mainly observed during the ischemic period, aggravates the injury of primary auditory neurons. On the other hand, oxidative damage induced by hydroxyl radicals and nitric oxide enhances cochlear reperfusion injury. This article briefly summarizes the generation mechanisms of cochlear ischemia-reperfusion injury and potential therapeutic targets that could be developed for the effective management of this injury type.

Intraoperative cochlear nerve mapping with the mobile cochlear nerve compound action potential tracer in vestibular schwannoma surgery.

PubMed

Watanabe, Nobuyuki; Ishii, Takuya; Fujitsu, Kazuhiko; Kaku, Shogo; Ichikawa, Teruo; Miyahara, Kosuke; Okada, Tomu; Tanino, Shin; Uriu, Yasuhiro; Murayama, Yuichi

2018-05-18

OBJECTIVE The authors describe the usefulness and limitations of the cochlear nerve compound action potential (CNAP) mobile tracer (MCT) that they developed to aid in cochlear nerve mapping during vestibular schwannoma surgery (VSS) for hearing preservation. METHODS This MCT device requires no more than 2 seconds for stable placement on the nerve to obtain the CNAP and thus is able to trace the cochlear nerve instantaneously. Simultaneous bipolar and monopolar recording is possible. The authors present the outcomes of 18 consecutive patients who underwent preoperative useful hearing (defined as class I or II of the Gardner-Robertson classification system) and underwent hearing-preservation VSS with the use of the MCT. Mapping was considered successful when it was possible to detect and trace the cochlear nerve. RESULTS Mapping of the cochlear nerve was successful in 13 of 18 patients (72.2%), and useful hearing was preserved in 11 patients (61.1%). Among 8 patients with large tumors (Koos grade 3 or 4), the rate of successful mapping was 62.5% (5 patients). The rate of hearing preservation in patients with large tumors was 50% (4 patients). CONCLUSIONS In addition to microsurgical presumption of the arrangement of each nerve, frequent probing on and around an unidentified nerve and comparison of each waveform are advisable with the use of both more sensitive monopolar and more location-specific bipolar MCT. MCT proved to be useful in cochlear nerve mapping and may consequently be helpful in hearing preservation. The authors discuss some limitations and problems with this device.

Preventing hearing damage using topical dexamethasone during reversible cochlear ischemia: an animal model.

PubMed

Morawski, Krzysztof; Telischi, Fred F; Bohorquez, Jorge; Niemczyk, Kazimierz

2009-09-01

Local application of dexamethasone to the round window (RW) niche prevents cochlear damage caused by local reversible ischemia. Cochlear ischemia induced by internal auditory artery (IAA) compression/stretching is thought to cause postoperative sensory hearing loss after attempted hearing preservation removal of acoustic neuroma tumors. Dexamethasone administered to the RW niche traveling through the membrane to the cochlear fluids may prevent ischemic damage. Ten young albino rabbits were used for this study. Ischemic episodes were induced by compressing the IAA. Laser Doppler cochlear blood flow was measured using a probe positioned at the RW niche. Transtympanic electrocochleography was measured at 4, 8, and 12 kHz. In 5 test ears, dexamethasone was administered topically at the RW for approximately 50 minutes before the IAA compressions, whereas in 5 control ears, saline was applied in the same way. Each ear underwent one 10-minute IAA compression with a 60-minute postischemic period of transtympanic electrocochleography monitoring. In both control- and dexamethasone-treated ears, ischemic episodes measured by Laser Doppler cochlear blood flow were comparable. Fifty minutes after IAA decompression, in dexamethasone-pretreated ears, cochlear microphonic and compound action potential amplitudes at all test frequencies were 10 to 15% less reduced than those in control ears. Compound action potential latencies in dexamethasone-pretreated ears resulted in shorter latency delay than in control ears. The RW seems to be an efficacious route for the administration of dexamethasone into the inner ear. Dexamethasone showed a protective effect on cochlear function after local ischemia. Transtympanic electrocochleography was found to be a sufficient and effective tool in monitoring hearing.

Across-site patterns of electrically evoked compound action potential amplitude-growth functions in multichannel cochlear implant recipients and the effects of the interphase gap.

PubMed

Schvartz-Leyzac, Kara C; Pfingst, Bryan E

2016-11-01

Electrically evoked compound action potential (ECAP) measures of peak amplitude, and amplitude-growth function (AGF) slope have been shown to reflect characteristics of cochlear health (primarily spiral ganglion density) in anesthetized cochlear-implanted guinea pigs. Likewise, the effect of increasing the interphase gap (IPG) in each of these measures also reflects SGN density in the implanted guinea pig. Based on these findings, we hypothesize that suprathreshold ECAP measures, and also how they change as the IPG is increased, have the potential to be clinically applicable in human subjects. However, further work is first needed in order to determine the characteristics of these measures in humans who use cochlear implants. The current study examined across-site patterns of suprathreshold ECAP measures in 10 bilaterally-implanted, adult cochlear implant users. Results showed that both peak amplitude and slope of the AGF varied significantly from electrode to electrode in ear-specific patterns across the subjects' electrode arrays. As expected, increasing the IPG on average increased the peak amplitude and slope. Across ears, there was a significant, negative correlation between the slope of the ECAP AGF and the duration of hearing loss. Across-site patterns of ECAP peak amplitude and AGF slopes were also compared with common ground impedance values and significant correlations were observed in some cases, depending on the subject and condition. The results of this study, coupled with previous studies in animals, suggest that it is feasible to measure the change in suprathreshold ECAP measures as the IPG increases on most electrodes. Further work is needed to investigate the relationship between these measures and cochlear implant outcomes, and determine how these measures might be used when programming a cochlear-implant processor. Published by Elsevier B.V.

Sensitivity in Interactions between Hearing Mothers and their Toddlers with Hearing Loss: The Effect of Cochlear Implantation

ERIC Educational Resources Information Center

Bakar, Zaharah Abu; Brown, P. Margaret; Remine, Maria D.

2010-01-01

This study investigated the potential effects of cochlear implantation and age at implantation on maternal interactional sensitivity. Three groups of dyads were studied at two points over 1 year. The hearing aid (HA) group wore hearing aids throughout the study, the early cochlear implanted (ECI) group were implanted prior to 22 months of age, and…

Microsurgical laser Doppler probe for simultaneous intraoperative monitoring of cochlear blood flow and electrocochleography from the round window

NASA Astrophysics Data System (ADS)

Abiy, Lidet; Telischi, Fred; Parel, Jean-Marie A.; Manns, Fabrice; Saettele, Ralph; Morawski, Krzysztof; Ozdamar, Ozcan; Borgos, John; Delgado, Rafael; Miskiel, Edward; Yavuz, Erdem

2003-06-01

The aim of this project is the development of a microsurgical laser Doppler (LD) probe that simultaneously monitors blood flow and Electrocochleography (ECochG) from the round window of the ear. The device will prevent neurosensory hearing loss during acoustic neuroma surgery by preventing damage to the internal auditory nerve and to the cochlear blood flow supply. A commercially available 0.5 mm diameter Laser-Doppler velocimetry probe (LaserFlo, Vasamedics) was modified to integrate an ECochG electrode. A tube for suction and irrigation was incorporated into a sheath of the probe shaft, to facilitate cleaning of the round window (RW) and allow drug delivery to the round window membrane. The prototype microprobe was calibrated on a single vessel model and tested in vivo in a rabbit model. Preliminary results indicate that the microprobe was able to measure changes in cochlear blood flow (CBF) and ECochG potentials from the round window of rabbits in vivo. The microprobe is suitable for monitoring cochlear blood flow and auditory cochlear potentials during human surgery.

Music mixing preferences of cochlear implant recipients: a pilot study.

PubMed

Buyens, Wim; van Dijk, Bas; Moonen, Marc; Wouters, Jan

2014-05-01

Music perception and appraisal are generally poor in cochlear implant recipients. Simple musical structures, lyrics that are easy to follow, and clear rhythm/beat have been reported among the top factors to enhance music enjoyment. The present study investigated the preference for modified relative instrument levels in music with normal-hearing and cochlear implant subjects. In experiment 1, test subjects were given a mixing console and multi-track recordings to determine their most enjoyable audio mix. In experiment 2, a preference rating experiment based on the preferred relative level settings in experiment 1 was performed. Experiment 1 was performed with four postlingually deafened cochlear implant subjects, experiment 2 with ten normal-hearing and ten cochlear implant subjects. A significant difference in preference rating was found between normal-hearing and cochlear implant subjects. The latter preferred an audio mix with larger vocals-to-instruments ratio. In addition, given an audio mix with clear vocals and attenuated instruments, cochlear implant subjects preferred the bass/drum track to be louder than the other instrument tracks. The original audio mix in real-world music might not be suitable for cochlear implant recipients. Modifying the relative instrument level settings potentially improves music enjoyment.

Histaminergic H3-Heteroreceptors as a Potential Mediator of Betahistine-Induced Increase in Cochlear Blood Flow.

PubMed

Bertlich, Mattis; Ihler, Friedrich; Freytag, Saskia; Weiss, Bernhard G; Strupp, Michael; Canis, Martin

2015-01-01

Betahistine is a histamine-like drug that is considered beneficial in Ménière's disease by increasing cochlear blood flow. Acting as an agonist at the histamine H1-receptor and as an inverse agonist at the H3-receptor, these receptors as well as the adrenergic α2-receptor were investigated for betahistine effects on cochlear blood flow. A total of 54 Dunkin-Hartley guinea pigs were randomly assigned to one of nine groups treated with a selection of H1-, H3- or α2-selective agonists and antagonists together with betahistine. Cochlear blood flow and mean arterial pressure were recorded for 3 min before and 15 min after infusion. Blockage of the H3- or α2-receptors caused a suppression of betahistine-mediated typical changes in cochlear blood flow or blood pressure. Activation of H3-receptors caused a drop in cochlear blood flow and blood pressure. H1-receptors showed no involvement in betahistine-mediated changes of cochlear blood flow. Betahistine most likely affects cochlear blood flow through histaminergic H3-heteroreceptors. © 2015 S. Karger AG, Basel.

Cochlear microphonic broad tuning curves

NASA Astrophysics Data System (ADS)

Ayat, Mohammad; Teal, Paul D.; Searchfield, Grant D.; Razali, Najwani

2015-12-01

It is known that the cochlear microphonic voltage exhibits much broader tuning than does the basilar membrane motion. The most commonly used explanation for this is that when an electrode is inserted at a particular point inside the scala media, the microphonic potentials of neighbouring hair cells have different phases, leading to cancelation at the electrodes location. In situ recording of functioning outer hair cells (OHCs) for investigating this hypothesis is exceptionally difficult. Therefore, to investigate the discrepancy between the tuning curves of the basilar membrane and those of the cochlear microphonic, and the effect of phase cancellation of adjacent hair cells on the broadness of the cochlear microphonic tuning curves, we use an electromechanical model of the cochlea to devise an experiment. We explore the effect of adjacent hair cells (i.e., longitudinal phase cancellation) on the broadness of the cochlear microphonic tuning curves in different locations. The results of the experiment indicate that active longitudinal coupling (i.e., coupling with active adjacent outer hair cells) only slightly changes the broadness of the CM tuning curves. The results also demonstrate that there is a π phase difference between the potentials produced by the hair bundle and the soma near the place associated with the characteristic frequency based on place-frequency maps (i.e., the best place). We suggest that the transversal phase cancellation (caused by the phase difference between the hair bundle and the soma) plays a far more important role than longitudinal phase cancellation in the broadness of the cochlear microphonic tuning curves. Moreover, by increasing the modelled longitudinal resistance resulting the cochlear microphonic curves exhibiting sharper tuning. The results of the simulations suggest that the passive network of the organ of Corti determines the phase difference between the hair bundle and soma, and hence determines the sharpness of the cochlear microphonic tuning curves.

Hearing dysfunction in heterozygous Mitf(Mi-wh) /+ mice, a model for Waardenburg syndrome type 2 and Tietz syndrome.

PubMed

Ni, Christina; Zhang, Deming; Beyer, Lisa A; Halsey, Karin E; Fukui, Hideto; Raphael, Yehoash; Dolan, David F; Hornyak, Thomas J

2013-01-01

The human deafness-pigmentation syndromes, Waardenburg syndrome (WS) type 2a, and Tietz syndrome are characterized by profound deafness but only partial cutaneous pigmentary abnormalities. Both syndromes are caused by mutations in MITF. To illuminate differences between cutaneous and otic melanocytes in these syndromes, their development and survival in heterozygous Microphthalmia-White (Mitf(Mi-wh) /+) mice were studied and hearing function of these mice characterized. Mitf(Mi-wh) /+ mice have a profound hearing deficit, characterized by elevated auditory brainstem response thresholds, reduced distortion product otoacoustic emissions, absent endocochlear potential, loss of outer hair cells, and stria vascularis abnormalities. Mitf(Mi-wh) /+ embryos have fewer melanoblasts during embryonic development than their wild-type littermates. Although cochlear melanocytes are present at birth, they disappear from the Mitf(Mi-wh) /+ cochlea between P1 and P7. These findings may provide insight into the mechanism of melanocyte and hearing loss in human deafness-pigmentation syndromes such as WS and Tietz syndrome and illustrate differences between otic and follicular melanocytes. © 2012 John Wiley & Sons A/S.

Stanford Center for Military Photomedicine

DTIC Science & Technology

2014-09-08

cochlear implants after blast injury. A.2. WOUND HEALING. We have used several in vivo and in vitro models of wound healing to study the basic cell and...clinical information we will obtain has the potential to fundamentally alter the diagnosis and treatment of human cochlear pathology. Our microscope...of live guinea pigs, and have shown that FME can resolve cochlear structures in live subjects in a manner far superior to that of any other existing

Evaluation of Jet Fuel and Noise-Induced Hearing Loss in Rats (Rattus norvegicus)

DTIC Science & Technology

2014-05-13

product otoacoustic emissions (DPOAE) and compound action potential (CAP) testing of the cochlear nerve. Inner ear hair cell loss was also not affected...conclusion by revealing that exposure to JP-8 combined with noise may result in the loss of pre-neural cochlear sensitivity as shown by suppression of...distortion product otoacoustic emissions (DPOAE) and depletion of cochlear sensory cells as evidenced by cytocochleograms that plot the percentage of

Hearing Loss After Radiotherapy for Pediatric Brain Tumors: Effect of Cochlear Dose

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hua, Chiaho; Bass, Johnnie K.; Khan, Raja

Purpose: To determine the effect of cochlear dose on sensorineural hearing loss in pediatric patients with brain tumor treated by using conformal radiation therapy (CRT). Patients and Methods: We studied 78 pediatric patients (155 ears) with localized brain tumors treated in 1997-2001 who had not received platinum-based chemotherapy and were followed up for at least 48 months. They were evaluated prospectively by means of serial pure-tone audiograms (250 Hz-8 kHz) and/or auditory brainstem response before and every 6 months after CRT. Results: Hearing loss occurred in 14% (11 of 78) of patients and 11% (17 of 155) of cochleae, withmore » onset most often at 3-5 years after CRT. The incidence of hearing loss was low for a cochlear mean dose of 30 Gy or less and increased at greater than 40-45 Gy. Risk was greater at high frequencies (6-8 kHz). In children who tested abnormal for hearing, average hearing thresholds increased from a less than 25 decibel (dB) hearing level (HL) at baseline to a mean of 46 {+-} 13 (SD) dB HL for high frequencies, 41 {+-} 7 dB HL for low frequencies, and 38 {+-} 6 dB HL for intermediate frequencies. Conclusions: Sensorineural hearing loss is a late effect of CRT. In the absence of other factors, including ototoxic chemotherapy, increase in cochlear dose correlates positively with hearing loss in pediatric patients with brain tumor. To minimize the risk of hearing loss for children treated with radiation therapy, a cumulative cochlear dose less than 35 Gy is recommended for patients planned to receive 54-59.4 Gy in 30-33 treatment fractions.« less

The Summating Potential Is a Reliable Marker of Electrode Position in Electrocochleography: Cochlear Implant as a Theragnostic Probe.

PubMed

Helmstaedter, Victor; Lenarz, Thomas; Erfurt, Peter; Kral, Andrej; Baumhoff, Peter

2017-12-14

For the increasing number of cochlear implantations in subjects with residual hearing, hearing preservation, and thus the prevention of implantation trauma, is crucial. A method for monitoring the intracochlear position of a cochlear implant (CI) and early indication of imminent cochlear trauma would help to assist the surgeon to achieve this goal. The aim of this study was to evaluate the reliability of the different electric components recorded by an intracochlear electrocochleography (ECochG) as markers for the cochleotopic position of a CI. The measurements were made directly from the CI, combining intrasurgical diagnostics with the therapeutical use of the CI, thus, turning the CI into a "theragnostic probe." Intracochlear ECochGs were measured in 10 Dunkin Hartley guinea pigs of either sex, with normal auditory brainstem response thresholds. All subjects were fully implanted (4 to 5 mm) with a custom six contact CI. The ECochG was recorded simultaneously from all six contacts with monopolar configuration (retroauricular reference electrode). The gross ECochG signal was filtered off-line to separate three of its main components: compound action potential, cochlear microphonic, and summating potential (SP). Additionally, five cochleae were harvested and histologically processed to access the spatial position of the CI contacts. Both ECochG data and histological reconstructions of the electrode position were fitted with the Greenwood function to verify the reliability of the deduced cochleotopic position of the CI. SPs could be used as suitable markers for the frequency position of the recording electrode with an accuracy of ±1/4 octave in the functioning cochlea, verified by histology. Cochlear microphonics showed a dependency on electrode position but were less reliable as positional markers. Compound action potentials were not suitable for CI position information but were sensitive to "cochlear health" (e.g., insertion trauma). SPs directly recorded from the contacts of a CI during surgery can be used to access the intracochlear frequency position of the CI. Using SP monitoring, implantation may be stopped before penetrating functioning cochlear regions. If the technique was similarly effective in humans, it could prevent implantation trauma and increase hearing preservation during CI surgery. Diagnostic hardware and software for recording biological signals with a CI without filter limitations might be a valuable add-on to the portfolios of CI manufacturers.

Ethical considerations in resource allocation in a cochlear implant program.

PubMed

Westerberg, Brian D; Pijl, Sipke; McDonald, Michael

2008-04-01

To review processes of resource allocation and the ethical considerations relevant to the fair allocation of a limited number of cochlear implants to increasing numbers of potential recipients. Review of relevant considerations. Tertiary referral hospital. Editorial discussion of the ethical issues of resource allocation. Heterogeneity of audiometric thresholds, self-reported disability of hearing loss, age of the potential cochlear implant recipient, cost-effectiveness, access to resources, compliance with follow-up, social support available to the recipient, social consequences of hearing impairment, and other recipient-related factors. In a publicly funded health care system, there will always be a need for decision-making processes for allocation of finite fiscal resources. All candidates for cochlear implantation deserve fair consideration. However, they are a heterogeneous group in terms of needs and expected outcomes consisting of traditional and marginal candidates, with a wide range of benefit from acoustic amplification. We argue that implant programs should thoughtfully prioritize treatment on the basis of need and potential benefit. We reject queuing on the basis of "first-come, first-served" or on the basis of perceived social worth.

Usher syndrome and cochlear implantation.

PubMed

Loundon, Natalie; Marlin, Sandrine; Busquet, Denise; Denoyelle, Françoise; Roger, Gilles; Renaud, Francis; Garabedian, Erea Noel

2003-03-01

To evaluate the symptoms leading to diagnosis and the quality of rehabilitation after cochlear implantation in Usher syndrome. Retrospective cohort study. ENT department of a tertiary referral hospital. Among 210 patients given an implantation in the Ear, Nose, and Throat department, 185 were congenitally deaf and 13 had Usher syndrome (7.0%). Five had a family history of Usher, and eight were sporadic cases. Eleven cases were Usher type I, one was Usher type III, and one was not classified. The age at implantation ranged from 18 months to 44 years (mean, 6 years 1 month). The mean follow-up was 52 months (range, 9 months to 9 years). All patients had audiophonological and clinical examination, computed tomography scan of the temporal bones, ophthalmologic examination with fundoscopy, and an electroretinogram. Cerebral magnetic resonance imaging and vestibular examination were performed in 9 of 13 and 10 of 13 cases, respectively. Logopedic outcome measured preimplant and postimplant closed- and open-set word recognition and oral expression at follow-up. The most frequent initial sign of Usher syndrome was delayed walking, with a mean age of 20 months. Among the 172 other congenitally deaf children with implants, when deafness was not associated with other neurologic disorders, the mean age at walking was 14 months (p < 0.001). The fundoscopy was always abnormal after the age of 5 years, and the electroretinogram was abnormal in all cases. Vestibular function was abnormal in all but one case (nonclassified). The computed tomography scan and the magnetic resonance imaging were always normal. Logopedic results with cochlear implants showed good perception skills in all but one case. The best perceptive results were obtained in children implanted before the age of 9 years. Oral language had significantly progressed in 9 of 13 at follow-up. There was no relation between the visual acuity and the logopedic results. The earliest clinical sign associated with deafness evoking Usher syndrome is late walking. The electroretinogram is the only reliable examination to enable the diagnosis. When severe profound deafness is associated with late walking, the electroretinogram should be systematically proposed. Logopedic results are linked to precocity of implantation, and early Usher's diagnosis contributes to optimize speech therapy.

Remote programming of cochlear implants: a telecommunications model.

PubMed

McElveen, John T; Blackburn, Erin L; Green, J Douglas; McLear, Patrick W; Thimsen, Donald J; Wilson, Blake S

2010-09-01

Evaluate the effectiveness of remote programming for cochlear implants. Retrospective review of the cochlear implant performance for patients who had undergone mapping and programming of their cochlear implant via remote connection through the Internet. Postoperative Hearing in Noise Test and Consonant/Nucleus/Consonant word scores for 7 patients who had undergone remote mapping and programming of their cochlear implant were compared with the mean scores of 7 patients who had been programmed by the same audiologist over a 12-month period. Times required for remote and direct programming were also compared. The quality of the Internet connection was assessed using standardized measures. Remote programming was performed via a virtual private network with a separate software program used for video and audio linkage. All 7 patients were programmed successfully via remote connectivity. No untoward patient experiences were encountered. No statistically significant differences could be found in comparing postoperative Hearing in Noise Test and Consonant/Nucleus/Consonant word scores for patients who had undergone remote programming versus a similar group of patients who had their cochlear implant programmed directly. Remote programming did not require a significantly longer programming time for the audiologist with these 7 patients. Remote programming of a cochlear implant can be performed safely without any deterioration in the quality of the programming. This ability to remotely program cochlear implant patients gives the potential to extend cochlear implantation to underserved areas in the United States and elsewhere.

Using Evoked Potentials to Match Interaural Electrode Pairs with Bilateral Cochlear Implants

PubMed Central

Delgutte, Bertrand

2007-01-01

Bilateral cochlear implantation seeks to restore the advantages of binaural hearing to the profoundly deaf by providing binaural cues normally important for accurate sound localization and speech reception in noise. Psychophysical observations suggest that a key issue for the implementation of a successful binaural prosthesis is the ability to match the cochlear positions of stimulation channels in each ear. We used a cat model of bilateral cochlear implants with eight-electrode arrays implanted in each cochlea to develop and test a noninvasive method based on evoked potentials for matching interaural electrodes. The arrays allowed the cochlear location of stimulation to be independently varied in each ear. The binaural interaction component (BIC) of the electrically evoked auditory brainstem response (EABR) was used as an assay of binaural processing. BIC amplitude peaked for interaural electrode pairs at the same relative cochlear position and dropped with increasing cochlear separation in either direction. To test the hypothesis that BIC amplitude peaks when electrodes from the two sides activate maximally overlapping neural populations, we measured multiunit neural activity along the tonotopic gradient of the inferior colliculus (IC) with 16-channel recording probes and determined the spatial pattern of IC activation for each stimulating electrode. We found that the interaural electrode pairings that produced the best aligned IC activation patterns were also those that yielded maximum BIC amplitude. These results suggest that EABR measurements may provide a method for assigning frequency–channel mappings in bilateral implant recipients, such as pediatric patients, for which psychophysical measures of pitch ranking or binaural fusion are unavailable. PMID:17225976

Using evoked potentials to match interaural electrode pairs with bilateral cochlear implants.

PubMed

Smith, Zachary M; Delgutte, Bertrand

2007-03-01

Bilateral cochlear implantation seeks to restore the advantages of binaural hearing to the profoundly deaf by providing binaural cues normally important for accurate sound localization and speech reception in noise. Psychophysical observations suggest that a key issue for the implementation of a successful binaural prosthesis is the ability to match the cochlear positions of stimulation channels in each ear. We used a cat model of bilateral cochlear implants with eight-electrode arrays implanted in each cochlea to develop and test a noninvasive method based on evoked potentials for matching interaural electrodes. The arrays allowed the cochlear location of stimulation to be independently varied in each ear. The binaural interaction component (BIC) of the electrically evoked auditory brainstem response (EABR) was used as an assay of binaural processing. BIC amplitude peaked for interaural electrode pairs at the same relative cochlear position and dropped with increasing cochlear separation in either direction. To test the hypothesis that BIC amplitude peaks when electrodes from the two sides activate maximally overlapping neural populations, we measured multiunit neural activity along the tonotopic gradient of the inferior colliculus (IC) with 16-channel recording probes and determined the spatial pattern of IC activation for each stimulating electrode. We found that the interaural electrode pairings that produced the best aligned IC activation patterns were also those that yielded maximum BIC amplitude. These results suggest that EABR measurements may provide a method for assigning frequency-channel mappings in bilateral implant recipients, such as pediatric patients, for which psychophysical measures of pitch ranking or binaural fusion are unavailable.

Persistence, Distribution, and Impact of Distinctly Segmented Microparticles on Cochlear Health following In Vivo Infusion3*

PubMed Central

Ross, Astin M.; Rahmani, Sahar; Prieskorn, Diane M.; Dishman, Acacia F; Miller, Josef M.; Lahann, Joerg; Altschuler, Richard A.

2016-01-01

Delivery of pharmaceuticals to the cochleae of patients with auditory dysfunction could potentially have many benefits from enhancing auditory nerve survival to protecting remaining sensory cells and their neuronal connections. Treatment would require platforms to enable drug delivery directly to the cochlea and increase the potential efficacy of intervention. Cochlear implant recipients are a specific patient subset that could benefit from local drug delivery as more candidates have residual hearing; and since residual hearing directly contributes to post-implantation hearing outcomes, it requires protection from implant insertion-induced trauma. This study assessed the feasibility of utilizing microparticles for drug delivery into cochlear fluids, testing persistence, distribution, biocompatibility, and drug release characteristics. To allow for delivery of multiple therapeutics, particles were composed of two distinct compartments; one containing polylactide-co-glycolide (PLGA), and one composed of acetal-modified dextran and PLGA. Following in vivo infusion, image analysis revealed microparticle persistence in the cochlea for at least 7 days post-infusion, primarily in the first and second turns. The majority of subjects maintained or had only slight elevation in auditory brainstem response thresholds at 7 days post-infusion compared to pre-infusion baselines. There was only minor to limited loss of cochlear hair cells and negligible immune response based on CD45+ immunolabling. When Piribedil-loaded microparticles were infused, Piribedil was detectable within the cochlear fluids at 7 days post-infusion. These results indicate that segmented microparticles are relatively inert, can persist, release their contents, and be functionally and biologically compatible with cochlear function and therefore are promising vehicles for cochlear drug delivery. PMID:26841263

Intrinsic regenerative potential of murine cochlear supporting cells.

PubMed

Sinkkonen, Saku T; Chai, Renjie; Jan, Taha A; Hartman, Byron H; Laske, Roman D; Gahlen, Felix; Sinkkonen, Wera; Cheng, Alan G; Oshima, Kazuo; Heller, Stefan

2011-01-01

The lack of cochlear regenerative potential is the main cause for the permanence of hearing loss. Albeit quiescent in vivo, dissociated non-sensory cells from the neonatal cochlea proliferate and show ability to generate hair cell-like cells in vitro. Only a few non-sensory cell-derived colonies, however, give rise to hair cell-like cells, suggesting that sensory progenitor cells are a subpopulation of proliferating non-sensory cells. Here we purify from the neonatal mouse cochlea four different non-sensory cell populations by fluorescence-activated cell sorting (FACS). All four populations displayed proliferative potential, but only lesser epithelial ridge and supporting cells robustly gave rise to hair cell marker-positive cells. These results suggest that cochlear supporting cells and cells of the lesser epithelial ridge show robust potential to de-differentiate into prosensory cells that proliferate and undergo differentiation in similar fashion to native prosensory cells of the developing inner ear.

Communication routes between intracranial spaces and inner ear: function, pathophysiologic importance and relations with inner ear diseases.

PubMed

Ciuman, Raphael R

2009-01-01

There exist 3 communication routes between the intracranial space and the inner ear, the vestibular aqueduct, the cochlear aqueduct, and the internal auditory canal. They possess a key role in inner ear pressure regulation and fluid homeostasis and are related to inner ear diseases. Relevant literature was reviewed, and the current knowledge of the anatomy, physiologic importance, and relations to inner ear diseases were described. Pathologic communication routes such as semicircular canal dehiscence syndrome were highlighted as well. Abnormalities in all 3 communication routes may predispose or be the cause of distinct inner ear pathologic condition and involved in other cochlear and vestibular syndromes, in which their role is not completely clear. The increasing knowledge of the underlying mechanisms encourages promising approaches for possible intervention in the future.

The benefits of remote microphone technology for adults with cochlear implants.

PubMed

Fitzpatrick, Elizabeth M; Séguin, Christiane; Schramm, David R; Armstrong, Shelly; Chénier, Josée

2009-10-01

Cochlear implantation has become a standard practice for adults with severe to profound hearing loss who demonstrate limited benefit from hearing aids. Despite the substantial auditory benefits provided by cochlear implants, many adults experience difficulty understanding speech in noisy environments and in other challenging listening conditions such as television. Remote microphone technology may provide some benefit in these situations; however, little is known about whether these systems are effective in improving speech understanding in difficult acoustic environments for this population. This study was undertaken with adult cochlear implant recipients to assess the potential benefits of remote microphone technology. The objectives were to examine the measurable and perceived benefit of remote microphone devices during television viewing and to assess the benefits of a frequency-modulated system for speech understanding in noise. Fifteen adult unilateral cochlear implant users were fit with remote microphone devices in a clinical environment. The study used a combination of direct measurements and patient perceptions to assess speech understanding with and without remote microphone technology. The direct measures involved a within-subject repeated-measures design. Direct measures of patients' speech understanding during television viewing were collected using their cochlear implant alone and with their implant device coupled to an assistive listening device. Questionnaires were administered to document patients' perceptions of benefits during the television-listening tasks. Speech recognition tests of open-set sentences in noise with and without remote microphone technology were also administered. Participants showed improved speech understanding for television listening when using remote microphone devices coupled to their cochlear implant compared with a cochlear implant alone. This benefit was documented both when listening to news and talk show recordings. Questionnaire results also showed statistically significant differences between listening with a cochlear implant alone and listening with a remote microphone device. Participants judged that remote microphone technology provided them with better comprehension, more confidence, and greater ease of listening. Use of a frequency-modulated system coupled to a cochlear implant also showed significant improvement over a cochlear implant alone for open-set sentence recognition in +10 and +5 dB signal to noise ratios. Benefits were measured during remote microphone use in focused-listening situations in a clinical setting, for both television viewing and speech understanding in noise in the audiometric sound suite. The results suggest that adult cochlear implant users should be counseled regarding the potential for enhanced speech understanding in difficult listening environments through the use of remote microphone technology.

Olivocochlear neuron central anatomy is normal in alpha 9 knockout mice.

PubMed

Brown, M Christian; Vetter, Douglas E

2009-03-01

Olivocochlear (OC) neurons were studied in a transgenic mouse with deletion of the alpha 9 nicotinic acetylcholine receptor subunit. In this alpha 9 knockout mouse, the peripheral effects of OC stimulation are lacking and the peripheral terminals of OC neurons under outer hair cells have abnormal morphology. To account for this mouse's apparently normal hearing, it has been proposed to have central compensation via collateral branches to the cochlear nucleus. We tested this idea by staining OC neurons for acetylcholinesterase and examining their morphology in knockout mice, wild-type mice of the same background strain, and CBA/CaJ mice. Knockout mice had normal OC systems in terms of numbers of OC neurons, dendritic patterns, and numbers of branches to the cochlear nucleus. The branch terminations were mainly to edge regions and to a lesser extent the core of the cochlear nucleus, and were similar among the strains in terms of the distribution and staining density. These data demonstrate that there are no obvious changes in the central morphology of the OC neurons in alpha 9 knockout mice and make less attractive the idea that there is central compensation for deletion of the peripheral receptor in these mice.

Children Using Cochlear Implants Capitalize on Acoustical Hearing for Music Perception

PubMed Central

Hopyan, Talar; Peretz, Isabelle; Chan, Lisa P.; Papsin, Blake C.; Gordon, Karen A.

2012-01-01

Cochlear implants (CIs) electrically stimulate the auditory nerve providing children who are deaf with access to speech and music. Because of device limitations, it was hypothesized that children using CIs develop abnormal perception of musical cues. Perception of pitch and rhythm as well as memory for music was measured by the children’s version of the Montreal Battery of Evaluation of Amusia (MBEA) in 23 unilateral CI users and 22 age-matched children with normal hearing. Children with CIs were less accurate than their normal hearing peers (p < 0.05). CI users were best able to discern rhythm changes (p < 0.01) and to remember musical pieces (p < 0.01). Contrary to expectations, abilities to hear cues in music improved as the age at implantation increased (p < 0.01). Because the children implanted at older ages also had better low frequency hearing prior to cochlear implantation and were able to use this hearing by wearing hearing aids. Access to early acoustical hearing in the lower frequency ranges appears to establish a base for music perception, which can be accessed with later electrical CI hearing. PMID:23133430

Loud Music Exposure and Cochlear Synaptopathy in Young Adults: Isolated Auditory Brainstem Response Effects but No Perceptual Consequences.

PubMed

Grose, John H; Buss, Emily; Hall, Joseph W

2017-01-01

The purpose of this study was to test the hypothesis that listeners with frequent exposure to loud music exhibit deficits in suprathreshold auditory performance consistent with cochlear synaptopathy. Young adults with normal audiograms were recruited who either did ( n = 31) or did not ( n = 30) have a history of frequent attendance at loud music venues where the typical sound levels could be expected to result in temporary threshold shifts. A test battery was administered that comprised three sets of procedures: (a) electrophysiological tests including distortion product otoacoustic emissions, auditory brainstem responses, envelope following responses, and the acoustic change complex evoked by an interaural phase inversion; (b) psychoacoustic tests including temporal modulation detection, spectral modulation detection, and sensitivity to interaural phase; and (c) speech tests including filtered phoneme recognition and speech-in-noise recognition. The results demonstrated that a history of loud music exposure can lead to a profile of peripheral auditory function that is consistent with an interpretation of cochlear synaptopathy in humans, namely, modestly abnormal auditory brainstem response Wave I/Wave V ratios in the presence of normal distortion product otoacoustic emissions and normal audiometric thresholds. However, there were no other electrophysiological, psychophysical, or speech perception effects. The absence of any behavioral effects in suprathreshold sound processing indicated that, even if cochlear synaptopathy is a valid pathophysiological condition in humans, its perceptual sequelae are either too diffuse or too inconsequential to permit a simple differential diagnosis of hidden hearing loss.

Cortical Auditory Evoked Potentials with Simple (Tone Burst) and Complex (Speech) Stimuli in Children with Cochlear Implant

PubMed Central

Martins, Kelly Vasconcelos Chaves; Gil, Daniela

2017-01-01

Introduction  The registry of the component P1 of the cortical auditory evoked potential has been widely used to analyze the behavior of auditory pathways in response to cochlear implant stimulation. Objective  To determine the influence of aural rehabilitation in the parameters of latency and amplitude of the P1 cortical auditory evoked potential component elicited by simple auditory stimuli (tone burst) and complex stimuli (speech) in children with cochlear implants. Method  The study included six individuals of both genders aged 5 to 10 years old who have been cochlear implant users for at least 12 months, and who attended auditory rehabilitation with an aural rehabilitation therapy approach. Participants were submitted to research of the cortical auditory evoked potential at the beginning of the study and after 3 months of aural rehabilitation. To elicit the responses, simple stimuli (tone burst) and complex stimuli (speech) were used and presented in free field at 70 dB HL. The results were statistically analyzed, and both evaluations were compared. Results  There was no significant difference between the type of eliciting stimulus of the cortical auditory evoked potential for the latency and the amplitude of P1. There was a statistically significant difference in the P1 latency between the evaluations for both stimuli, with reduction of the latency in the second evaluation after 3 months of auditory rehabilitation. There was no statistically significant difference regarding the amplitude of P1 under the two types of stimuli or in the two evaluations. Conclusion  A decrease in latency of the P1 component elicited by both simple and complex stimuli was observed within a three-month interval in children with cochlear implant undergoing aural rehabilitation. PMID:29018498

The rat cochlea in the absence of circulating adrenal hormones: an electrophysiological and morphological study.

PubMed

Lohuis, P J; Börjesson, P K; Klis, S F; Smoorenburg, G F

2000-05-01

Circulating adrenal hormones affect strial function. Removal of endogenous levels of adrenal steroids by bilateral adrenalectomy (ADX) in rats causes a decrease of Na(+)/K(+)-ATPase activity in the cochlear lateral wall [Rarey et al., 1989. Arch. Otolaryngol. Head Neck Surg. 115, 817-821] and a decrease of the volume of the marginal cells in the stria vascularis [Lohuis et al., 1990. Acta Otolaryngol. (Stockh.) 110, 348-356]. To study further the effect of absence of circulating adrenocorticosteroids on cochlear function, 18 male Long Evans rats underwent either an ADX or a SHAM operation. Electrocochleography was performed 1 week after surgery for tone bursts in a frequency range of 1-16 kHz. Thereafter, the cochleas were harvested and examined histologically. No significant changes in the amplitude growth curves of the summating potential (SP), the compound action potential (CAP) and the cochlear microphonics (CM) were detected after ADX. However, visually, there appeared to be a decrease of endolymphatic volume (tentatively called imdrops). Reissner's membrane (RM) extended less into scala vestibuli in ADX animals than in SHAM-operated animals. The ratio between the length of RM and the straight distance between the medial and lateral attachment points of RM were used as an objective measure to quantify this effect in each sub-apical half turn of the cochlea. The decrease in length of RM was statistically significant. Thus, circulating adrenal hormones appear to be necessary for normal cochlear fluid homeostasis. Absence of one or more of these hormones leads to shrinkage of the scala media (imdrops). However, the absence of adrenal hormones does not affect the gross cochlear potentials. Apparently, the cochlea is capable of compensating for the absence of circulating adrenal hormones to sustain the conditions necessary for proper cochlear transduction.

Simultaneous Communication Supports Learning in Noise by Cochlear Implant Users

PubMed Central

Blom, Helen C.; Marschark, Marc; Machmer, Elizabeth

2017-01-01

Objectives This study sought to evaluate the potential of using spoken language and signing together (simultaneous communication, SimCom, sign-supported speech) as a means of improving speech recognition, comprehension, and learning by cochlear implant users in noisy contexts. Methods Forty eight college students who were active cochlear implant users, watched videos of three short presentations, the text versions of which were standardized at the 8th grade reading level. One passage was presented in spoken language only, one was presented in spoken language with multi-talker babble background noise, and one was presented via simultaneous communication with the same background noise. Following each passage, participants responded to 10 (standardized) open-ended questions designed to assess comprehension. Indicators of participants’ spoken language and sign language skills were obtained via self-reports and objective assessments. Results When spoken materials were accompanied by signs, scores were significantly higher than when materials were spoken in noise without signs. Participants’ receptive spoken language skills significantly predicted scores in all three conditions; neither their receptive sign skills nor age of implantation predicted performance. Discussion Students who are cochlear implant users typically rely solely on spoken language in the classroom. The present results, however, suggest that there are potential benefits of simultaneous communication for such learners in noisy settings. For those cochlear implant users who know sign language, the redundancy of speech and signs potentially can offset the reduced fidelity of spoken language in noise. Conclusion Accompanying spoken language with signs can benefit learners who are cochlear implant users in noisy situations such as classroom settings. Factors associated with such benefits, such as receptive skills in signed and spoken modalities, classroom acoustics, and material difficulty need to be empirically examined. PMID:28010675

Acute hyperfibrinogenemia impairs cochlear blood flow and hearing function in guinea pigs in vivo.

PubMed

Ihler, Fritz; Strieth, Sebastian; Pieri, Nicos; Göhring, Peter; Canis, Martin

2012-03-01

Impairment of microcirculation is a possible cause of sudden sensorineural hearing loss (SSNHL). Fibrinogen is known as a risk factor for both microvascular dysfunction and SSNHL. Therefore, the aim of this study was to investigate the effect of elevated serum levels of fibrinogen on cochlear blood flow and hearing function in vivo. One group of guinea pigs received two consecutive injections of 100 mg fibrinogen while a control group received equimolar doses of albumin. Measurements of cochlear microcirculation by intravital microscopy and of hearing thresholds by auditory brainstem response (ABR) recordings were carried out before, after first and after second injection. Ten healthy guinea pigs were randomly assigned to a treatment group or a control group of five animals each. Serum fibrinogen levels were elevated after the first and second injections of fibrinogen compared to basal values and control group respectively. Increasing levels of fibrinogen were paralleled by decreasing cochlear blood flow as well as increasing hearing thresholds. Hearing threshold correlated negatively with cochlear blood flow. The effect of microcirculatory impairment on hearing function could be explained by a malfunction of the cochlear amplifier. Further investigation is needed to quantify cochlear potentials under elevated serum fibrinogen levels.

Auditory Cortex Basal Activity Modulates Cochlear Responses in Chinchillas

PubMed Central

León, Alex; Elgueda, Diego; Silva, María A.; Hamamé, Carlos M.; Delano, Paul H.

2012-01-01

Background The auditory efferent system has unique neuroanatomical pathways that connect the cerebral cortex with sensory receptor cells. Pyramidal neurons located in layers V and VI of the primary auditory cortex constitute descending projections to the thalamus, inferior colliculus, and even directly to the superior olivary complex and to the cochlear nucleus. Efferent pathways are connected to the cochlear receptor by the olivocochlear system, which innervates outer hair cells and auditory nerve fibers. The functional role of the cortico-olivocochlear efferent system remains debated. We hypothesized that auditory cortex basal activity modulates cochlear and auditory-nerve afferent responses through the efferent system. Methodology/Principal Findings Cochlear microphonics (CM), auditory-nerve compound action potentials (CAP) and auditory cortex evoked potentials (ACEP) were recorded in twenty anesthetized chinchillas, before, during and after auditory cortex deactivation by two methods: lidocaine microinjections or cortical cooling with cryoloops. Auditory cortex deactivation induced a transient reduction in ACEP amplitudes in fifteen animals (deactivation experiments) and a permanent reduction in five chinchillas (lesion experiments). We found significant changes in the amplitude of CM in both types of experiments, being the most common effect a CM decrease found in fifteen animals. Concomitantly to CM amplitude changes, we found CAP increases in seven chinchillas and CAP reductions in thirteen animals. Although ACEP amplitudes were completely recovered after ninety minutes in deactivation experiments, only partial recovery was observed in the magnitudes of cochlear responses. Conclusions/Significance These results show that blocking ongoing auditory cortex activity modulates CM and CAP responses, demonstrating that cortico-olivocochlear circuits regulate auditory nerve and cochlear responses through a basal efferent tone. The diversity of the obtained effects suggests that there are at least two functional pathways from the auditory cortex to the cochlea. PMID:22558383

Developmental and cross-modal plasticity in deafness: evidence from the P1 and N1 event related potentials in cochlear implanted children.

PubMed

Sharma, Anu; Campbell, Julia; Cardon, Garrett

2015-02-01

Cortical development is dependent on extrinsic stimulation. As such, sensory deprivation, as in congenital deafness, can dramatically alter functional connectivity and growth in the auditory system. Cochlear implants ameliorate deprivation-induced delays in maturation by directly stimulating the central nervous system, and thereby restoring auditory input. The scenario in which hearing is lost due to deafness and then reestablished via a cochlear implant provides a window into the development of the central auditory system. Converging evidence from electrophysiologic and brain imaging studies of deaf animals and children fitted with cochlear implants has allowed us to elucidate the details of the time course for auditory cortical maturation under conditions of deprivation. Here, we review how the P1 cortical auditory evoked potential (CAEP) provides useful insight into sensitive period cut-offs for development of the primary auditory cortex in deaf children fitted with cochlear implants. Additionally, we present new data on similar sensitive period dynamics in higher-order auditory cortices, as measured by the N1 CAEP in cochlear implant recipients. Furthermore, cortical re-organization, secondary to sensory deprivation, may take the form of compensatory cross-modal plasticity. We provide new case-study evidence that cross-modal re-organization, in which intact sensory modalities (i.e., vision and somatosensation) recruit cortical regions associated with deficient sensory modalities (i.e., auditory) in cochlear implanted children may influence their behavioral outcomes with the implant. Improvements in our understanding of developmental neuroplasticity in the auditory system should lead to harnessing central auditory plasticity for superior clinical technique. Copyright © 2014 Elsevier B.V. All rights reserved.

Neural and receptor cochlear potentials obtained by transtympanic electrocochleography in auditory neuropathy.

PubMed

Santarelli, Rosamaria; Starr, Arnold; Michalewski, Henry J; Arslan, Edoardo

2008-05-01

Transtympanic electrocochleography (ECochG) was recorded bilaterally in children and adults with auditory neuropathy (AN) to evaluate receptor and neural generators. Test stimuli were clicks from 60 to 120dB p.e. SPL. Measures obtained from eight AN subjects were compared to 16 normally hearing children. Receptor cochlear microphonics (CMs) in AN were of normal or enhanced amplitude. Neural compound action potentials (CAPs) and receptor summating potentials (SPs) were identified in five AN ears. ECochG potentials in those ears without CAPs were of negative polarity and of normal or prolonged duration. We used adaptation to rapid stimulus rates to distinguish whether the generators of the negative potentials were of neural or receptor origin. Adaptation in controls resulted in amplitude reduction of CAP twice that of SP without affecting the duration of ECochG potentials. In seven AN ears without CAP and with prolonged negative potential, adaptation was accompanied by reduction of both amplitude and duration of the negative potential to control values consistent with neural generation. In four ears without CAP and with normal duration potentials, adaptation was without effect consistent with receptor generation. In five AN ears with CAP, there was reduction in amplitude of CAP and SP as controls but with a significant decrease in response duration. Three patterns of cochlear potentials were identified in AN: (1) presence of receptor SP without CAP consistent with pre-synaptic disorder of inner hair cells; (2) presence of both SP and CAP consistent with post-synaptic disorder of proximal auditory nerve; (3) presence of prolonged neural potentials without a CAP consistent with post-synaptic disorder of nerve terminals. Cochlear potential measures may identify pre- and post-synaptic disorders of inner hair cells and auditory nerves in AN.

Hearing loss and enlarged internal auditory canal in children.

PubMed

Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael

2014-01-01

Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations. Copyright © 2013 Elsevier España, S.L. All rights reserved.

[Value of the study of cochlear microphonic recordings in deep and severe deafness].

PubMed

Moatti, L; Busquet, D; Cotin, G

1983-01-01

A study was conducted to assess the contribution of cochlear microphonic potential recordings during electrophysiologic audiometry examinations. Amplitude of microphonic recordings were correlated with the degree of deafness, its etiology, and the prosthetic prognosis in 38 electrocochleographic examinations. Preliminary results are analyzed.

An Electromechanical Model for the Cochlear Microphonic

NASA Astrophysics Data System (ADS)

Teal, Paul D.; Lineton, Ben; Elliott, Stephen J.

2011-11-01

The first of the many electrical signals generated in the ear, nerves and brain as a response to a sound incident on the ear is the cochlear microphonic (CM). The CM is generated by the hair cells of the cochlea, primarily the outer hairs cells. The potentials of this signal are a nonlinear filtered version of the acoustic pressure at the tympanic membrane. The CM signal has been used very little in recent years for clinical audiology and audiological research. This is because of uncertainty in interpreting the CM signal as a diagnostic measure, and also because of the difficulty of obtaining the signal, which has usually required the use of a transtympanic electrode. There are however, several potential clinical and research applications for acquisition of the CM. To promote understanding of the CM, and potential clinical application, a model is presented which can account for the generation of the cochlear microphonic signal. The model incorporates micro-mechanical and macro-mechanical aspects of previously published models of the basilar membrane and reticular lamina, as well as cochlear fluid mechanics, piezoelectric activity and capacitance of the outer hair cells. It also models the electrical coupling of signals along the scalae.

Pharmacokinetic Properties of Adenosine Amine Congener in Cochlear Perilymph after Systemic Administration.

PubMed

Chang, Hao; Telang, Ravindra S; Sreebhavan, Sreevalsan; Tingle, Malcolm; Thorne, Peter R; Vlajkovic, Srdjan M

2017-01-01

Noise-induced hearing loss (NIHL) is a global health problem affecting over 5% of the population worldwide. We have shown previously that acute noise-induced cochlear injury can be ameliorated by administration of drugs acting on adenosine receptors in the inner ear, and a selective A 1 adenosine receptor agonist adenosine amine congener (ADAC) has emerged as a potentially effective treatment for cochlear injury and resulting hearing loss. This study investigated pharmacokinetic properties of ADAC in rat perilymph after systemic (intravenous) administration using a newly developed liquid chromatography-tandem mass spectrometry detection method. The method was developed and validated in accordance with the USA FDA guidelines including accuracy, precision, specificity, and linearity. Perilymph was sampled from the apical turn of the cochlea to prevent contamination with the cerebrospinal fluid. ADAC was detected in cochlear perilymph within two minutes following intravenous administration and remained in perilymph above its minimal effective concentration for at least two hours. The pharmacokinetic pattern of ADAC was significantly altered by exposure to noise, suggesting transient changes in permeability of the blood-labyrinth barrier and/or cochlear blood flow. This study supports ADAC development as a potential clinical otological treatment for acute sensorineural hearing loss caused by exposure to traumatic noise.

Pharmacokinetic Properties of Adenosine Amine Congener in Cochlear Perilymph after Systemic Administration

PubMed Central

Sreebhavan, Sreevalsan; Thorne, Peter R.

2017-01-01

Noise-induced hearing loss (NIHL) is a global health problem affecting over 5% of the population worldwide. We have shown previously that acute noise-induced cochlear injury can be ameliorated by administration of drugs acting on adenosine receptors in the inner ear, and a selective A1 adenosine receptor agonist adenosine amine congener (ADAC) has emerged as a potentially effective treatment for cochlear injury and resulting hearing loss. This study investigated pharmacokinetic properties of ADAC in rat perilymph after systemic (intravenous) administration using a newly developed liquid chromatography-tandem mass spectrometry detection method. The method was developed and validated in accordance with the USA FDA guidelines including accuracy, precision, specificity, and linearity. Perilymph was sampled from the apical turn of the cochlea to prevent contamination with the cerebrospinal fluid. ADAC was detected in cochlear perilymph within two minutes following intravenous administration and remained in perilymph above its minimal effective concentration for at least two hours. The pharmacokinetic pattern of ADAC was significantly altered by exposure to noise, suggesting transient changes in permeability of the blood-labyrinth barrier and/or cochlear blood flow. This study supports ADAC development as a potential clinical otological treatment for acute sensorineural hearing loss caused by exposure to traumatic noise. PMID:28194422

Simultaneous Communication and Cochlear Implants in the Classroom?

ERIC Educational Resources Information Center

Blom, Helen C.; Marschark, Marc

2015-01-01

This study was designed to evaluate the potential of simultaneous communication (sign and speech together) to support classroom learning by college students who use cochlear implants (CIs). Metacognitive awareness of learning also was evaluated. A within-subjects design involving 40 implant users indicated that the student participants learned…

Relational Learning in Children with Deafness and Cochlear Implants

ERIC Educational Resources Information Center

Almeida-Verdu, Ana Claudia; Huziwara, Edson M.; de Souza, Deisy G.; de Rose, Julio C.; Bevilacqua, Maria Cecilia; Lopes, Jair, Jr.; Alves, Cristiane O.; McIlvane, William J.

2008-01-01

This four-experiment series sought to evaluate the potential of children with neurosensory deafness and cochlear implants to exhibit auditory-visual and visual-visual stimulus equivalence relations within a matching-to-sample format. Twelve children who became deaf prior to acquiring language (prelingual) and four who became deaf afterwards…

A New Acoustic Portal into the Odontocete Ear and Vibrational Analysis of the Tympanoperiotic Complex

DTIC Science & Technology

2010-08-01

are credited. Funding: Fleet Industrial Supply Center (FISC). Grant# N00244-08-1-0025. The funding flows from the Chief of Naval Operations...34, 35, 36 and 37). Middle Ear Function McCormick [22] studied hearing in the bottlenose dolphin (Tursiops truncatus) by measuring cochlear potentials...tympanic ligament attenuated the cochlear potentials by 18 dB with respect to the level obtained during preliminary tests, whereas removing the

Effects of central nervous system residua on cochlear implant results in children deafened by meningitis.

PubMed

Francis, Howard W; Pulsifer, Margaret B; Chinnici, Jill; Nutt, Robert; Venick, Holly S; Yeagle, Jennifer D; Niparko, John K

2004-05-01

This study explored factors associated with speech recognition outcomes in postmeningitic deafness (PMD). The results of cochlear implantation may vary in children with PMD because of sequelae that extend beyond the auditory periphery. To determine which factors might be most determinative of outcome of cochlear implantation in children with PMD. Retrospective chart review. A referral center for pediatric cochlear implantation and rehabilitation. Thirty children with cochlear implants who were deafened by meningitis were matched with subjects who were deafened by other causes based on the age at diagnosis, age at cochlear implantation, age at which hearing aids were first used, and method of communication used at home or in the classroom. Speech perception performance within the first 2 years after cochlear implantation and its relationship with presurgical cognitive measures and medical history. There was no difference in the overall cognitive or postoperative speech perception performance between the children with PMD and those deafened by other causes. The presence of postmeningitic hydrocephalus, however, posed greater challenges to the rehabilitation process, as indicated by significantly smaller gains in speech perception and a predilection for behavioral problems. By comparison, cochlear scarring and incomplete electrode insertion had no impact on speech perception results. Although the results demonstrated no significant delay in cognitive or speech perception performance in the PMD group, central nervous system residua, when present, can impede the acquisition of speech perception with a cochlear implant. Central effects associated with PMD may thus impact language learning potential; cognitive and behavioral therapy should be considered in rehabilitative planning and in establishing expectations of outcome.

Cochlear implantation for single-sided deafness and tinnitus suppression.

PubMed

Holder, Jourdan T; O'Connell, Brendan; Hedley-Williams, Andrea; Wanna, George

To quantify the potential effectiveness of cochlear implantation for tinnitus suppression in patients with single-sided deafness using the Tinnitus Handicap Inventory. The study included 12 patients with unilateral tinnitus who were undergoing cochlear implantation for single-sided deafness. The Tinnitus Handicap Inventory was administered at the patient's cochlear implant candidacy evaluation appointment prior to implantation and every cochlear implant follow-up appointment, except activation, following implantation. Patient demographics and speech recognition scores were also retrospectively recorded using the electronic medical record. A significant reduction was found when comparing Tinnitus Handicap Inventory score preoperatively (61.2±27.5) to the Tinnitus Handicap Inventory score after three months of cochlear implant use (24.6±28.2, p=0.004) and the Tinnitus Handicap Inventory score beyond 6months of CI use (13.3±18.9, p=0.008). Further, 45% of patients reported total tinnitus suppression. Mean CNC word recognition score improved from 2.9% (SD 9.4) pre-operatively to 40.8% (SD 31.7) by 6months post-activation, which was significantly improved from pre-operative scores (p=0.008). The present data is in agreement with previously published studies that have shown an improvement in tinnitus following cochlear implantation for the large majority of patients with single-sided deafness. Copyright © 2017 Elsevier Inc. All rights reserved.

Predicting the location of missing outer hair cells using the electrical signal recorded at the round window

PubMed Central

Chertoff, Mark E.; Earl, Brian R.; Diaz, Francisco J.; Sorensen, Janna L.; Thomas, Megan L. A.; Kamerer, Aryn M.; Peppi, Marcello

2014-01-01

The electrical signal recorded at the round window was used to estimate the location of missing outer hair cells. The cochlear response was recorded to a low frequency tone embedded in high-pass filtered noise conditions. Cochlear damage was created by either overexposure to frequency-specific tones or laser light. In animals with continuous damage along the partition, the amplitude of the cochlear response increased as the high-pass cutoff frequency increased, eventually reaching a plateau. The cochlear distance at the onset of the plateau correlated with the anatomical onset of outer hair cell loss. A mathematical model replicated the physiologic data but was limited to cases with continuous hair cell loss in the middle and basal turns. The neural contribution to the cochlear response was determined by recording the response before and after application of Ouabain. Application of Ouabain eliminated or reduced auditory neural activity from approximately two turns of the cochlea. The amplitude of the cochlear response was reduced for moderate signal levels with a limited effect at higher levels, indicating that the cochlear response was dominated by outer hair cell currents at high signal levels and neural potentials at low to moderate signal levels. PMID:25190395

Cortical Auditory Evoked Potentials to Evaluate Cochlear Implant Candidacy in an Ear With Long-standing Hearing Loss: A Case Report.

PubMed

Patel, Tirth R; Shahin, Antoine J; Bhat, Jyoti; Welling, D Bradley; Moberly, Aaron C

2016-10-01

We describe a novel use of cortical auditory evoked potentials in the preoperative workup to determine ear candidacy for cochlear implantation. A 71-year-old male was evaluated who had a long-deafened right ear, had never worn a hearing aid in that ear, and relied heavily on use of a left-sided hearing aid. Electroencephalographic testing was performed using free field auditory stimulation of each ear independently with pure tones at 1000 and 2000 Hz at approximately 10 dB above pure-tone thresholds for each frequency and for each ear. Mature cortical potentials were identified through auditory stimulation of the long-deafened ear. The patient underwent successful implantation of that ear. He experienced progressively improving aided pure-tone thresholds and binaural speech recognition benefit (AzBio score of 74%). Findings suggest that use of cortical auditory evoked potentials may serve a preoperative role in ear selection prior to cochlear implantation. © The Author(s) 2016.

Predictors of round window accessibility for adult cochlear implantation based on pre-operative CT scan: a prospective observational study.

PubMed

Park, Edward; Amoodi, Hosam; Kuthubutheen, Jafri; Chen, Joseph M; Nedzelski, Julian M; Lin, Vincent Y W

2015-05-28

Cochlear implantation has become a mainstream treatment option for patients with severe to profound sensorineural hearing loss. During cochlear implant, there are key surgical steps which are influenced by anatomical variations between each patient. The aim of this study is to determine if there are potential predictors of difficulties that may be encountered during the cortical mastoidectomy, facial recess approach and round window access in cochlear implant surgery based upon pre-operative temporal bone CT scan. Fifty seven patients undergoing unilateral cochlear implantation were analyzed. Difficulty with 1) cortical mastoidectomy, 2) facial recess approach, and 3) round window access were scored intra-operatively by the surgeon in a blinded fashion (1 = "easy", 2 = "moderate", 3 = "difficult"). Pre-operative temporal bone CT scans were analyzed for 1) degree of mastoid aeration; 2) location of the sigmoid sinus; 3) height of the tegmen; 4) the presence of air cells in the facial recess, and 5) degree of round window bony overhang. Poor mastoid aeration and lower tegmen position, but not the location of sigmoid sinus, are associated with greater difficulty with the cortical mastoidectomy. Presence of an air cell around the facial nerve was predictive of easier facial recess access. However, the degree of round window bony overhang was not predictive of difficulty associated with round window access. Certain parameters on the pre-operative temporal bone CT scan may be useful in predicting potential difficulties encountered during the key steps involved in cochlear implant surgery.

Deaf Culture and the Cochlear Implant Debate: Cyborg Politics and the Identity of People with Disabilities.

ERIC Educational Resources Information Center

Cherney, James L.

1999-01-01

Examines the potential benefits and limitations of cyborg politics. Explores depictions of cyborgs in science fiction stories and examines the deaf culture's arguments in the cochlear-implant debate. Investigates the current viability of cyborg politics as a mode of advocacy for people with disabilities. (SC)

Changing Realities in the Classroom for Hearing-Impaired Children with Cochlear Implant

ERIC Educational Resources Information Center

Vermeulen, Anneke; De Raeve, Leo; Langereis, Margreet; Snik, Ad

2012-01-01

Auditory perception with cochlear implants (CIs) enables the majority of deaf children with normal learning potential to develop (near) age-appropriate spoken language. As a consequence, a large proportion of children now attend mainstream education from an early stage. The acoustical environment in kindergartens and schools, however, might be…

The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

PubMed

Tranebjærg, Lisbeth; Strenzke, Nicola; Lindholm, Sture; Rendtorff, Nanna D; Poulsen, Hanne; Khandelia, Himanshu; Kopec, Wojciech; Lyngbye, Troels J Brünnich; Hamel, Christian; Delettre, Cecile; Bocquet, Beatrice; Bille, Michael; Owen, Hanne H; Bek, Toke; Jensen, Hanne; Østergaard, Karen; Möller, Claes; Luxon, Linda; Carr, Lucinda; Wilson, Louise; Rajput, Kaukab; Sirimanna, Tony; Harrop-Griffiths, Katherine; Rahman, Shamima; Vona, Barbara; Doll, Julia; Haaf, Thomas; Bartsch, Oliver; Rosewich, Hendrik; Moser, Tobias; Bitner-Glindzicz, Maria

2018-02-01

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing impairment (CAPOS) is a rare clinically distinct syndrome caused by a single dominant missense mutation, c.2452G>A, p.Glu818Lys, in ATP1A3, encoding the neuron-specific alpha subunit of the Na+/K+-ATPase α3. Allelic mutations cause the neurological diseases rapid dystonia Parkinsonism and alternating hemiplegia of childhood, disorders which do not encompass hearing or visual impairment. We present detailed clinical phenotypic information in 18 genetically confirmed patients from 11 families (10 previously unreported) from Denmark, Sweden, UK and Germany indicating a specific type of hearing impairment-auditory neuropathy (AN). All patients were clinically suspected of CAPOS and had hearing problems. In this retrospective analysis of audiological data, we show for the first time that cochlear outer hair cell activity was preserved as shown by the presence of otoacoustic emissions and cochlear microphonic potentials, but the auditory brainstem responses were grossly abnormal, likely reflecting neural dyssynchrony. Poor speech perception was observed, especially in noise, which was beyond the hearing level obtained in the pure tone audiograms in several of the patients presented here. Molecular modelling and in vitro electrophysiological studies of the specific CAPOS mutation were performed. Heterologous expression studies of α3 with the p.Glu818Lys mutation affects sodium binding to, and release from, the sodium-specific site in the pump, the third ion-binding site. Molecular dynamics simulations confirm that the structure of the C-terminal region is affected. In conclusion, we demonstrate for the first time evidence for auditory neuropathy in CAPOS syndrome, which may reflect impaired propagation of electrical impulses along the spiral ganglion neurons. This has implications for diagnosis and patient management. Auditory neuropathy is difficult to treat with conventional hearing aids, but preliminary improvement in speech perception in some patients suggests that cochlear implantation may be effective in CAPOS patients.

Corticofugal modulation of peripheral auditory responses

PubMed Central

Terreros, Gonzalo; Delano, Paul H.

2015-01-01

The auditory efferent system originates in the auditory cortex and projects to the medial geniculate body (MGB), inferior colliculus (IC), cochlear nucleus (CN) and superior olivary complex (SOC) reaching the cochlea through olivocochlear (OC) fibers. This unique neuronal network is organized in several afferent-efferent feedback loops including: the (i) colliculo-thalamic-cortico-collicular; (ii) cortico-(collicular)-OC; and (iii) cortico-(collicular)-CN pathways. Recent experiments demonstrate that blocking ongoing auditory-cortex activity with pharmacological and physical methods modulates the amplitude of cochlear potentials. In addition, auditory-cortex microstimulation independently modulates cochlear sensitivity and the strength of the OC reflex. In this mini-review, anatomical and physiological evidence supporting the presence of a functional efferent network from the auditory cortex to the cochlear receptor is presented. Special emphasis is given to the corticofugal effects on initial auditory processing, that is, on CN, auditory nerve and cochlear responses. A working model of three parallel pathways from the auditory cortex to the cochlea and auditory nerve is proposed. PMID:26483647

Improved outcomes in auditory brainstem implantation with the use of near-field electrical compound action potentials.

PubMed

Mandalà, Marco; Colletti, Liliana; Colletti, Giacomo; Colletti, Vittorio

2014-12-01

To compare the outcomes (auditory threshold and open-set speech perception at 48-month follow-up) of a new near-field monitoring procedure, electrical compound action potential, on positioning the auditory brainstem implant electrode array on the surface of the cochlear nuclei versus the traditional far-field electrical auditory brainstem response. Retrospective study. Tertiary referral center. Among the 202 patients with auditory brainstem implants fitted and monitored with electrical auditory brainstem response during implant fitting, 9 also underwent electrical compound action potential recording. These subjects were matched retrospectively with a control group of 9 patients in whom only the electrical auditory brainstem response was recorded. Electrical compound action potentials were obtained using a cotton-wick recording electrode located near the surface of the cochlear nuclei and on several cranial nerves. Significantly lower potential thresholds were observed with the recording electrode located on the cochlear nuclei surface compared with the electrical auditory brainstem response (104.4 ± 32.5 vs 158.9 ± 24.2, P = .0030). Electrical brainstem response and compound action potentials identified effects on the neighboring cranial nerves on 3.2 ± 2.4 and 7.8 ± 3.2 electrodes, respectively (P = .0034). Open-set speech perception outcomes at 48-month follow-up had improved significantly in the near- versus far-field recording groups (78.9% versus 56.7%; P = .0051). Electrical compound action potentials during auditory brainstem implantation significantly improved the definition of the potential threshold and the number of auditory and extra-auditory waves generated. It led to the best coupling between the electrode array and cochlear nuclei, significantly improving the overall open-set speech perception. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

Cochlear implants and spoken language processing abilities: review and assessment of the literature.

PubMed

Peterson, Nathaniel R; Pisoni, David B; Miyamoto, Richard T

2010-01-01

Cochlear implants (CIs) process sounds electronically and then transmit electric stimulation to the cochlea of individuals with sensorineural deafness, restoring some sensation of auditory perception. Many congenitally deaf CI recipients achieve a high degree of accuracy in speech perception and develop near-normal language skills. Post-lingually deafened implant recipients often regain the ability to understand and use spoken language with or without the aid of visual input (i.e. lip reading). However, there is wide variation in individual outcomes following cochlear implantation, and some CI recipients never develop useable speech and oral language skills. The causes of this enormous variation in outcomes are only partly understood at the present time. The variables most strongly associated with language outcomes are age at implantation and mode of communication in rehabilitation. Thus, some of the more important factors determining success of cochlear implantation are broadly related to neural plasticity that appears to be transiently present in deaf individuals. In this article we review the expected outcomes of cochlear implantation, potential predictors of those outcomes, the basic science regarding critical and sensitive periods, and several new research directions in the field of cochlear implantation.

[Assessment of the horizontal semicircular canal function after cochlear implantation by video head impulse test and caloric test].

PubMed

Zeng, Jie; Huang, Hong Ming; Wang, Xiao Qian; Zhong, Kai Bang; Wu, Pei Na

2018-01-20

Objective: To analyze the functional change of horizontal semicircular canals after cochlear implantation. Method: Eighteen patients were enrolled in this study.Their vestibular function was evaluated by using the caloric test and video head impulse test before and one week,one month after CI surgery,respectively.The unilateral weakness(UW),slow phase velocity(SPV)in caloric test and gain in video head impulse test(vHIT-G)were observed.Caloric test was abnormal when UW>25% or SPV mean<6°/s,while vHIT was abnormal when vHIT-G<0.8. Result: The SPV of the implanted ear were[(10.36±8.01)°/s;(14.77±14.24)°/s]pre-operatively,[(6.45±7.52)°/s;(5.14±4.67)°/s]1 week post-operatively and[(6.05±3.86)°/s;(6.27±4.17)°/s]1 month post-operatively.Statistically significant difference( P <0.05)was found between pre-and post-operative period.The vHIT-G of the implanted ear were(0.73±0.33)pre-operatively,(0.65±0.32)1 week post-operatively and(0.71±0.36)1 month post-operatively.There was no statistically significant difference of vHIT-G between preand post-operative period( P (pre-operative/1 week post-operative)=0.084, P (pre-operative/1 month post-operative)=0.679).Four patients presented with vertigo and one of them manifested slight unsteadiness post-operatively.All symptoms resolved within 7 days.These symptoms had no correlate with age,gender,implantedear and results of vestibular test. Conclusion: Cochlear implantation can affect the horizontal semicircular canal function,and the video head impulse test and caloric test should be used in a complementary fashion.

[Water regulation in the cochlea : Do molecular water channels facilitate potassium-dependent sound transduction?].

PubMed

Eckhard, A; Löwenheim, H

2014-06-01

Sound transduction in the cochlea critically depends on the circulation of potassium ions (K(+)) along so-called "K(+) recycling routes" between the endolymph and perilymph. These K(+) currents generate high ionic and osmotic gradients, which potentially impair the excitability of sensory hair cells and threaten cell survival in the entire cochlear duct. Molecular water channels-aquaporins (AQP)-are expressed in all cochlear supporting cells along the K(+) recycling routes; however, their significance for osmotic equilibration in cochlear duct cells is unknown. The diffusive and osmotic water permeabilies of Reissner's membrane, the organ of Corti and the entire cochlear duct epithelium were determined. Expression of the potassium channel Kir4.1 and the water channel AQP4 in the cochlear duct was investigated by immunohistochemistry. The calculated water permeability values indicate the extent of AQP-facilitated water flux across the cochlear duct epithelium. Immunohistochemically, Kir4.1 and AQP4 were found to colocalize in distinct membrane domains of supporting cells along the K(+)-recycling routes. These observations suggest the presence of a rapid AQP-mediated water exchange between the endolymph, the cells of the cochlear duct and the perilymph. The subcellular colocalization of Kir4.1 and AQP4 in epithelial supporting cells indicates functional coupling of potassium and water flow in the cochlea. Finally, this offers an explanation for the hearing impairment observed in individuals with mutations in the AQP4 gene.

Binaural Pitch Fusion in Bilateral Cochlear Implant Users.

PubMed

Reiss, Lina A J; Fowler, Jennifer R; Hartling, Curtis L; Oh, Yonghee

Binaural pitch fusion is the fusion of stimuli that evoke different pitches between the ears into a single auditory image. Individuals who use hearing aids or bimodal cochlear implants (CIs) experience abnormally broad binaural pitch fusion, such that sounds differing in pitch by as much as 3-4 octaves are fused across ears, leading to spectral averaging and speech perception interference. The goal of this study was to determine if adult bilateral CI users also experience broad binaural pitch fusion. Stimuli were pulse trains delivered to individual electrodes. Fusion ranges were measured using simultaneous, dichotic presentation of reference and comparison stimuli in opposite ears, and varying the comparison stimulus to find the range that fused with the reference stimulus. Bilateral CI listeners had binaural pitch fusion ranges varying from 0 to 12 mm (average 6.1 ± 3.9 mm), where 12 mm indicates fusion over all electrodes in the array. No significant correlations of fusion range were observed with any subject factors related to age, hearing loss history, or hearing device history, or with any electrode factors including interaural electrode pitch mismatch, pitch match bandwidth, or within-ear electrode discrimination abilities. Bilateral CI listeners have abnormally broad fusion, similar to hearing aid and bimodal CI listeners. This broad fusion may explain the variability of binaural benefits for speech perception in quiet and in noise in bilateral CI users.

Cerebellar interaction with the acoustic reflex.

PubMed

Jastreboff, P J

1981-01-01

The involvement of the cerebellar vermis in the acoustic reflex was analyzed in 12 cats, decerebrated or in pentobarbital anesthesia. Anatomical data suggested the existence of a connection of lobules VIII with the ventral cochlear nucleus. Single cell recording and evoked potential techniques demonstrated the existence of the acoustic projection to lobulus VIII. Electrical stimulation of this area changed the tension of the middle ear muscle and caused evoked potential responses in the caudal part of the ventral cochlear nucleus. Electrical stimulation of the motor nucleus of the facial nerve evoked a slow wave in the recording taken from the surrounding of the cochlear round window. A hypothesis is proposed which postulates the involvement of the acoustic reflex in space localization of acoustic stimuli and the action of cerebellar vermis in order to assure the stability and plasticity of the acoustic reflex arc.

The cochlear CRF signaling systems and their mechanisms of action in modulating cochlear sensitivity and protection against trauma

PubMed Central

Graham, Christine E.; Basappa, Johnvesly; Turcan, Sevin; Vetter, Douglas E.

2011-01-01

A key requirement for encoding the auditory environment is the ability to dynamically alter cochlear sensitivity. However, merely attaining a steady state of maximal sensitivity is not a viable solution since the sensory cells and ganglion cells of the cochlea are prone to damage following exposure to loud sound. Most often, such damage is via initial metabolic insult that can lead to cellular death. Thus, establishing the highest sensitivity must be balanced with protection against cellular metabolic damage that can lead to loss of hair cells and ganglion cells, resulting in loss of frequency representation. While feedback mechanisms are known to exist in the cochlea that alter sensitivity, they respond only after stimulus encoding, allowing potentially damaging sounds to impact the inner ear at times coincident with increased sensitivity. Thus, questions remain concerning the endogenous signaling systems involved in dynamic modulation of cochlear sensitivity and protection against metabolic stress. Understanding endogenous signaling systems involved in cochlear protection may lead to new strategies and therapies for prevention of cochlear damage and consequent hearing loss. We have recently discovered a novel cochlear signaling system that is molecularly equivalent to the classic hypothalamic-pituitary-adrenal (HPA) axis. This cochlear HPA-equivalent system functions to balance auditory sensitivity and susceptibility to noise-induced hearing loss, and also protects against cellular metabolic insults resulting from exposures to ototoxic drugs. We review the anatomy, physiology, and cellular signaling of this system, and compare it to similar signaling in other organs/tissues of the body. PMID:21909974

Cell-based neurotrophin treatment supports long-term auditory neuron survival in the deaf guinea pig.

PubMed

Gillespie, Lisa N; Zanin, Mark P; Shepherd, Robert K

2015-01-28

The cochlear implant provides auditory cues to profoundly deaf patients by electrically stimulating the primary auditory neurons (ANs) of the cochlea. However, ANs degenerate in deafness; the preservation of a robust AN target population, in combination with advances in cochlear implant technology, may provide improved hearing outcomes for cochlear implant patients. The exogenous delivery of neurotrophins such as brain-derived neurotrophic factor (BDNF) and neurotrophin-3 is well known to support AN survival in deafness, and cell-based therapies provide a potential clinically viable option for delivering neurotrophins into the deaf cochlea. This study utilized cells that were genetically modified to express BDNF and encapsulated in alginate microspheres, and investigated AN survival in the deaf guinea pig following (a) cell-based neurotrophin treatment in conjunction with chronic electrical stimulation from a cochlear implant, and (b) long-term cell-based neurotrophin delivery. In comparison to deafened controls, there was significantly greater AN survival following the cell-based neurotrophin treatment, and there were ongoing survival effects for at least six months. In addition, functional benefits were observed following cell-based neurotrophin treatment and chronic electrical stimulation, with a statistically significant decrease in electrically evoked auditory brainstem response thresholds observed during the experimental period. This study demonstrates that cell-based therapies, in conjunction with a cochlear implant, shows potential as a clinically transferable means of providing neurotrophin treatment to support AN survival in deafness. This technology also has the potential to deliver other therapeutic agents, and to be used in conjunction with other biomedical devices for the treatment of a variety of neurodegenerative conditions. Copyright © 2014 Elsevier B.V. All rights reserved.

Tinnitus and Sleep Difficulties After Cochlear Implantation.

PubMed

Pierzycki, Robert H; Edmondson-Jones, Mark; Dawes, Piers; Munro, Kevin J; Moore, David R; Kitterick, Pádraig T

To estimate and compare the prevalence of and associations between tinnitus and sleep difficulties in a sample of UK adult cochlear implant users and those identified as potential candidates for cochlear implantation. The study was conducted using the UK Biobank resource, a population-based cohort of 40- to 69-year olds. Self-report data on hearing, tinnitus, sleep difficulties, and demographic variables were collected from cochlear implant users (n = 194) and individuals identified as potential candidates for cochlear implantation (n = 211). These "candidates" were selected based on (i) impaired hearing sensitivity, inferred from self-reported hearing aid use and (ii) impaired hearing function, inferred from an inability to report words accurately at negative signal to noise ratios on an unaided closed-set test of speech perception. Data on tinnitus (presence, persistence, and related distress) and on sleep difficulties were analyzed using logistic regression models controlling for gender, age, deprivation, and neuroticism. The prevalence of tinnitus was similar among implant users (50%) and candidates (52%; p = 0.39). However, implant users were less likely to report that their tinnitus was distressing at its worst (41%) compared with candidates (63%; p = 0.02). The logistic regression model suggested that this difference between the two groups could be explained by the fact that tinnitus was less persistent in implant users (46%) compared with candidates (72%; p < 0.001). Self-reported difficulties with sleep were similar among implant users (75%) and candidates (82%; p = 0.28), but participants with tinnitus were more likely to report sleep difficulties than those without (p < 0.001). The prevalence of sleep difficulties was not related to tinnitus persistence (p = 0.28) or the extent to which tinnitus was distressing (p = 0.55). The lack of association between tinnitus persistence and sleep difficulties is compatible with the notion that tinnitus is suppressed in implant users primarily during active electrical stimulation and may return when the implant is switched off at night time. This explanation is supported by the similar prevalence of sleep problems among implant users and potential candidates for cochlear implantation, despite differences between the groups in tinnitus persistence and related emotional distress. Cochlear implantation may therefore not be an appropriate intervention where the primary aim is to alleviate sleep difficulties.

Use of Electrically Evoked Compound Action Potentials for Cochlear Implant Fitting: A Systematic Review.

PubMed

de Vos, Johan J; Biesheuvel, Jan Dirk; Briaire, Jeroen J; Boot, Pieter S; van Gendt, Margriet J; Dekkers, Olaf M; Fiocco, Marta; Frijns, Johan H M

The electrically evoked compound action potential (eCAP) is widely used in the clinic as an objective measure to assess cochlear implant functionality. During the past decade, there has been increasing interest in applying eCAPs for fitting of cochlear implants. Several studies have shown that eCAP-based fitting can potentially replace time-consuming behavioral fitting procedures, especially in young children. However, a closer look to all available literature revealed that there is no clear consensus on the validity of this fitting procedure. This study evaluated the validity of eCAP-based fitting of cochlear implant recipients based on a systematic review of the recent literature. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses were used to search the PubMed, Web of Science, and Cochrane Library databases. The term "eCAP" was combined with "cochlear implants," "thresholds," and "levels," in addition to a range of related terms. Finally, 32 studies met the inclusion criteria. These studies were evaluated on the risk of bias and, when possible, compared by meta-analysis. Almost all assessed studies suffered from some form of risk of bias. Twenty-nine of the studies based their conclusion on a group correlation instead of individual subject correlations (analytical bias); 14 studies were unclear about randomization or blinding (outcome assessment bias); 9 studies provided no clear description of the populations used, for example, prelingually or postlingually implanted subjects (selection bias); and 4 studies had a high rate of loss (>10%) for patients or electrodes (attrition bias). Meta-analysis of these studies revealed a weak pooled correlation between eCAP thresholds and both behavioral T- and C-levels (r = 0.58 and r = 0.61, respectively). This review shows that the majority of the assessed studies suffered from substantial shortcomings in study design and statistical analysis. Meta-analysis showed that there is only weak evidence to support the use of eCAP data for cochlear implant fitting purposes; eCAP thresholds are an equally weak predictor for both T- and C-levels. Based on this review, it can be concluded that research on eCAP-based fitting needs a profound reflection on study design and analysis to draw well-grounded conclusions about the validity of eCAP-based fitting of cochlear implant recipients.

Correlates of Psychosocial Adjustment in Deaf Adolescents with and without Cochlear Implants: A Preliminary Investigation

ERIC Educational Resources Information Center

Leigh, Irene W.; Maxwell-McCaw, Deborah; Bat-Chava, Yael; Christiansen, John B.

2009-01-01

The number of children who have received cochlear implants (CIs) has increased dramatically in the past two decades. In view of potential concerns about their psychosocial adjustment, our aim was to assess the effect of implants on the adolescents' psychosocial functioning among a group of 57 deaf adolescents with and without CIs, using published…

Intraoperative observation of changes in cochlear nerve action potentials during exposure to electromagnetic fields generated by mobile phones.

PubMed

Colletti, Vittorio; Mandalà, Marco; Manganotti, Paolo; Ramat, Stefano; Sacchetto, Luca; Colletti, Liliana

2011-07-01

The rapid spread of devices generating electromagnetic fields (EMF) has raised concerns as to the possible effects of this technology on humans. The auditory system is the neural organ most frequently and directly exposed to electromagnetic activity owing to the daily use of mobile phones. In recent publications, a possible correlation between mobile phone usage and central nervous system tumours has been detected. Very recently a deterioration in otoacoustic emissions and in the auditory middle latency responses after intensive and long-term magnetic field exposure in humans has been demonstrated. To determine with objective observations if exposure to mobile phone EMF affects acoustically evoked cochlear nerve compound action potentials, seven patients suffering from Ménière's disease and undergoing retrosigmoid vestibular neurectomy were exposed to the effects of mobile phone placed over the craniotomy for 5 min. All patients showed a substantial decrease in amplitude and a significant increase in latency of cochlear nerve compound action potentials during the 5 min of exposure to EMF. These changes lasted for a period of around 5 min after exposure. The possibility that EMF can produce relatively long-lasting effects on cochlear nerve conduction is discussed and analysed in light of contrasting previous literature obtained under non-surgical conditions. Limitations of this novel approach, including the effects of the anaesthetics, craniotomy and surgical procedure, are presented in detail.

Using Electrically-evoked Compound Action Potentials to Estimate Perceptive Levels in Experienced Adult Cochlear Implant Users.

PubMed

Joly, Charles-Alexandre; Péan, Vincent; Hermann, Ruben; Seldran, Fabien; Thai-Van, Hung; Truy, Eric

2017-10-01

The cochlear implant (CI) fitting level prediction accuracy of electrically-evoked compound action potential (ECAP) should be enhanced by the addition of demographic data in models. No accurate automated fitting of CI based on ECAP has yet been proposed. We recorded ECAP in 45 adults who had been using MED-EL CIs for more than 11 months and collected the most comfortable loudness level (MCL) used for CI fitting (prog-MCL), perception thresholds (meas-THR), and MCL (meas-MCL) measured with the stimulation used for ECAP recording. Linear mixed models taking into account cochlear site factors were computed to explain prog-MCL, meas-MCL, and meas-THR. Cochlear region and ECAP threshold were predictors of the three levels. In addition, significant predictors were the ECAP amplitude for the prog-MCL and the duration of deafness for the prog-MCL and the meas-THR. Estimations were more accurate for the meas-THR, then the meas-MCL, and finally the prog-MCL. These results show that 1) ECAP thresholds are more closely related to perception threshold than to comfort level, 2) predictions are more accurate when the inter-subject and cochlear regions variations are considered, and 3) differences between the stimulations used for ECAP recording and for CI fitting make it difficult to accurately predict the prog-MCL from the ECAP recording. Predicted prog-MCL could be used as bases for fitting but should be used with care to avoid any uncomfortable or painful stimulation.

Design and Evaluation of a Personal Digital Assistant-based Research Platform for Cochlear Implants

PubMed Central

Ali, Hussnain; Lobo, Arthur P.; Loizou, Philipos C.

2014-01-01

This paper discusses the design, development, features, and clinical evaluation of a personal digital assistant (PDA)-based platform for cochlear implant research. This highly versatile and portable research platform allows researchers to design and perform complex experiments with cochlear implants manufactured by Cochlear Corporation with great ease and flexibility. The research platform includes a portable processor for implementing and evaluating novel speech processing algorithms, a stimulator unit which can be used for electrical stimulation and neurophysio-logic studies with animals, and a recording unit for collecting electroencephalogram/evoked potentials from human subjects. The design of the platform for real time and offline stimulation modes is discussed for electric-only and electric plus acoustic stimulation followed by results from an acute study with implant users for speech intelligibility in quiet and noisy conditions. The results are comparable with users’ clinical processor and very promising for undertaking chronic studies. PMID:23674422

Vestibulo-ocular and vestibulospinal function before and after cochlear implant surgery

NASA Technical Reports Server (NTRS)

Black, F. O.; Lilly, D. J.; Peterka, R. J.; Fowler, L. P.; Simmons, F. B.

1987-01-01

Vestibular function in cochlear implant candidates varies from normal to total absence of function. In patients with intact vestibular function preoperatively, invasion of the otic capsule places residual vestibular function at risk. Speech-processing strategies that result in large amplitude electrical transients or strategies that employ high amplitude broad frequency carrier signals have the potential for disrupting vestibular function. Five patients were tested with and without electrical stimulation via cochlear electrodes. Two patients experienced subjective vestibular effects that were quickly resolved. No long-term vestibular effects were noted for the two types of second generation cochlear implants evaluated. Histopathological findings from another patient, who had electrically generated vestibular reflex responses to intramodiolar electrodes, indicated that responses elicited were a function of several variables including electrode location, stimulus intensity, stimulus amplitude, and stimulus frequency. Differential auditory, vestibulocolic, and vestibulospinal reflexes were demonstrated from the same electrode as a function of stimulus amplitude, frequency, and duration.

High frequency bone conduction auditory evoked potentials in the guinea pig: Assessing cochlear injury after ossicular chain manipulation.

PubMed

Bergin, M J; Bird, P A; Vlajkovic, S M; Thorne, P R

2015-12-01

Permanent high frequency (>4 kHz) sensorineural hearing loss following middle ear surgery occurs in up to 25% of patients. The aetiology of this loss is poorly understood and may involve transmission of supra-physiological forces down the ossicular chain to the cochlea. Investigating the mechanisms of this injury using animal models is challenging, as evaluating cochlear function with evoked potentials is confounded when ossicular manipulation disrupts the normal air conduction (AC) pathway. Bone conduction (BC) using clinical bone vibrators in small animals is limited by poor transducer output at high frequencies sensitive to trauma. The objectives of the present study were firstly to evaluate a novel high frequency bone conduction transducer with evoked auditory potentials in a guinea pig model, and secondly to use this model to investigate the impact of middle ear surgical manipulation on cochlear function. We modified a magnetostrictive device as a high frequency BC transducer and evaluated its performance by comparison with a calibrated AC transducer at frequencies up to 32 kHz using the auditory brainstem response (ABR), compound action potential (CAP) and summating potential (SP). To mimic a middle ear traumatising stimulus, a rotating bur was brought in to contact with the incudomalleal complex and the effect on evoked cochlear potentials was observed. BC-evoked potentials followed the same input-output function pattern as AC potentials for all ABR frequencies. Deterioration in CAP and SP thresholds was observed after ossicular manipulation. It is possible to use high frequency BC to evoke responses from the injury sensitive basal region of the cochlea and so not rely on AC with the potential confounder of conductive hearing loss. Ongoing research explores how these findings evolve over time, and ways in which injury may be reduced and the cochlea protected during middle ear surgery. Copyright © 2015 Elsevier B.V. All rights reserved.

The auditory nerve overlapped waveform (ANOW): A new objective measure of low-frequency hearing

NASA Astrophysics Data System (ADS)

Lichtenhan, Jeffery T.; Salt, Alec N.; Guinan, John J.

2015-12-01

One of the most pressing problems today in the mechanics of hearing is to understand the mechanical motions in the apical half of the cochlea. Almost all available measurements from the cochlear apex of basilar membrane or other organ-of-Corti transverse motion have been made from ears where the health, or sensitivity, in the apical half of the cochlea was not known. A key step in understanding the mechanics of the cochlear base was to trust mechanical measurements only when objective measures from auditory-nerve compound action potentials (CAPs) showed good preparation sensitivity. However, such traditional objective measures are not adequate monitors of cochlear health in the very low-frequency regions of the apex that are accessible for mechanical measurements. To address this problem, we developed the Auditory Nerve Overlapped Waveform (ANOW) that originates from auditory nerve output in the apex. When responses from the round window to alternating low-frequency tones are averaged, the cochlear microphonic is canceled and phase-locked neural firing interleaves in time (i.e., overlaps). The result is a waveform that oscillates at twice the probe frequency. We have demonstrated that this Auditory Nerve Overlapped Waveform - called ANOW - originates from auditory nerve fibers in the cochlear apex [8], relates well to single-auditory-nerve-fiber thresholds, and can provide an objective estimate of low-frequency sensitivity [7]. Our new experiments demonstrate that ANOW is a highly sensitive indicator of apical cochlear function. During four different manipulations to the scala media along the cochlear spiral, ANOW amplitude changed when either no, or only small, changes occurred in CAP thresholds. Overall, our results demonstrate that ANOW can be used to monitor cochlear sensitivity of low-frequency regions during experiments that make apical basilar membrane motion measurements.

Music training improves pitch perception in prelingually deafened children with cochlear implants.

PubMed

Chen, Joshua Kuang-Chao; Chuang, Ann Yi Chiun; McMahon, Catherine; Hsieh, Jen-Chuen; Tung, Tao-Hsin; Li, Lieber Po-Hung

2010-04-01

The comparatively poor music appreciation in patients with cochlear implants might be ascribed to an inadequate exposure to music; however, the effect of training on music perception in prelingually deafened children with cochlear implants remains unknown. This study aimed to investigate whether previous musical education improves pitch perception ability in these children. Twenty-seven children with congenital/prelingual deafness of profound degree were studied. Test stimuli consisted of 2 sequential piano tones, ranging from C (256 Hz) to B (495 Hz). Children were asked to identify the pitch relationship between the 2 tones (same, higher, or lower). Effects of musical training duration, pitch-interval size, current age, age of implantation, gender, and type of cochlear implant on accuracy of pitch perception were evaluated. The duration of musical training positively correlated with the correct rate of pitch perception. Pitch perception performance was better in children who had a cochlear implant and were older than 6 years than in those who were aged < or =6 years (ie, preschool). Effect of pitch-interval size was insignificant on pitch perception, and there was no correlation between pitch perception and the age of implantation, gender, or type of cochlear implant. Musical training seems to improve pitch perception ability in prelingually deafened children with a cochlear implant. Auditory plasticity might play an important role in such enhancement. This suggests that incorporation of a structured training program on music perception early in life and as part of the postoperative rehabilitation program for prelingually deafened children with cochlear implants would be beneficial. A longitudinal study is needed to show whether improvement of music performance in these children is measurable by use of auditory evoked potentials.

Effects of High-Rate Pulse Trains on Electrode Discrimination in Cochlear Implant Users

PubMed Central

Runge-Samuelson, Christina L.

2009-01-01

Overcoming issues related to abnormally high neural synchrony in response to electrical stimulation is one aspect in improving hearing with a cochlear implant. Desynchronization of electrical stimuli have shown benefits in neural encoding of electrical signals and improvements in psychophysical tasks. In the present study, 10 participants with either CII or HiRes 90k Advanced Bionics devices were tested for the effects of desynchronizing constant-amplitude high-rate (5,000 Hz) pulse trains on electrode discrimination of sinusoidal stimuli (1,000 Hz). When averaged across the sinusoidal dynamic range, overall improvements in electrode discrimination with high-rate pulses were found for 8 of 10 participants. This effect was significant for the group (p = .003). Nonmonotonic patterns of electrode discrimination as a function of sinusoidal stimulation level were observed. By providing additional spectral channels, it is possible that clinical implementation of constant-amplitude high-rate pulse trains in a signal processing strategy may improve performance with the device. PMID:19447763

Waardenburg Syndrome: An Unusual Indication of Cochlear Implantation Experienced in 11 Patients.

PubMed

Bayrak, Feda; Çatlı, Tolgahan; Atsal, Görkem; Tokat, Taşkın; Olgun, Levent

2017-08-01

The aim of this study was to present the surgical findings of children with Waardenburg syndrome (WS) and investigate speech development after cochlear implantation in this unique group of patients. A retrospective chart review of the patients diagnosed with WS and implanted between 1998 and 2015 was performed. Categories of auditory performance (CAP) test were used to assess the auditory skills of these patients. CAP is a nonlinear hierarchical scale used to rate a child's developing auditory abilities. Preoperative test results and intraoperative surgical findings of these patients have been presented. In total, 1835 cases were implanted a tour institution, and 1210 of these were children. Among these implantees, 11 were diagnosed with WS (0.59% of all implantees). Four of the 11 patients showed incomplete partition type 2bony labyrinth abnormality (Mondini deformity) and all patients showed intraoperative gusher during cochleostomy, which was subsided through routine interventions. No other complications occurred during surgery, and all patients showed satisfactory CAP results in the late postoperative period. Our experiences with cochlear implantation in patients with WS showed that the procedure is safe and effective in this group of patients. Surgeons should be aware of possible labyrinth malformations and intraoperative problems such as gusher in these patients. In long term, auditory performances may exhibit satisfactory results with optimal postoperative educational and supportive measures.

Altered vesicular glutamate transporter distributions in the mouse cochlear nucleus following cochlear insult

PubMed Central

Heeringa, Amarins N.; Stefanescu, Roxana A.; Raphael, Yehoash; Shore, Susan E.

2015-01-01

Vesicular glutamate transporters 1 and 2 (VGLUT1 and VGLUT2) have distinct distributions in the cochlear nucleus that correspond to the sources of the labeled terminals. VGLUT1 is mainly associated with terminals of auditory nerve fibers, whereas VGLUT2 is mainly associated with glutamatergic terminals deriving from other sources that project to the cochlear nucleus (CN), including somatosensory and vestibular terminals. Previous studies in guinea pig have shown that cochlear damage results in a decrease of VGLUT1-labeled puncta and an increase in VGLUT2-labeled puncta. This indicates cross-modal compensation that is of potential importance in somatic tinnitus. To examine whether this effect is consistent across species and to provide a background for future studies, using transgenesis, the current study examines VGLUT expression profiles upon cochlear insult by intracochlear kanamycin injections in the mouse. Intracochlear kanamycin injections abolished ipsilateral ABR responses in all animals and reduced ipsilateral spiral ganglion neuron densities in animals that were sacrificed after four weeks, but not in animals that were sacrificed after three weeks. In all unilaterally deafened animals, VGLUT1 density was decreased in CN regions that receive auditory nerve fiber terminals, i.e. in the deep layer of the dorsal cochlear nucleus (DCN), in the interstitial region where the auditory nerve enters the CN, and in the magnocellular region of the antero- and posteroventral CN. In contrast, density of VGLUT2 expression was upregulated in the fusiform cell layer of the DCN and in the granule cell lamina, which are known to receive somatosensory and vestibular terminals. These results show that a cochlear insult induces cross-modal compensation in the cochlear nucleus of the mouse, confirming previous findings in guinea pig, and that these changes are not dependent on the occurrence of spiral ganglion neuron degeneration. PMID:26705736

Altered vesicular glutamate transporter distributions in the mouse cochlear nucleus following cochlear insult.

PubMed

Heeringa, A N; Stefanescu, R A; Raphael, Y; Shore, S E

2016-02-19

Vesicular glutamate transporters 1 and 2 (VGLUT1 and VGLUT2) have distinct distributions in the cochlear nucleus that correspond to sources of the labeled terminals. VGLUT1 is mainly associated with terminals of auditory nerve fibers, whereas VGLUT2 is mainly associated with glutamatergic terminals deriving from other sources that project to the cochlear nucleus (CN), including somatosensory and vestibular terminals. Previous studies in guinea pig have shown that cochlear damage results in a decrease of VGLUT1-labeled puncta and an increase in VGLUT2-labeled puncta. This indicates cross-modal compensation that is of potential importance in somatic tinnitus. To examine whether this effect is consistent across species and to provide a background for future studies, using transgenesis, the current study examines VGLUT expression profiles upon cochlear insult by intracochlear kanamycin injections in the mouse. Intracochlear kanamycin injections abolished ipsilateral ABR responses in all animals and reduced ipsilateral spiral ganglion neuron densities in animals that were sacrificed after four weeks, but not in animals that were sacrificed after three weeks. In all unilaterally deafened animals, VGLUT1 density was decreased in CN regions that receive auditory nerve fiber terminals, i.e., in the deep layer of the dorsal cochlear nucleus (DCN), in the interstitial region where the auditory nerve enters the CN, and in the magnocellular region of the antero- and posteroventral CN. In contrast, density of VGLUT2 expression was upregulated in the fusiform cell layer of the DCN and in the granule cell lamina, which are known to receive somatosensory and vestibular terminals. These results show that a cochlear insult induces cross-modal compensation in the cochlear nucleus of the mouse, confirming previous findings in guinea pig, and that these changes are not dependent on the occurrence of spiral ganglion neuron degeneration. Copyright © 2015 IBRO. Published by Elsevier Ltd. All rights reserved.

Should children who use cochlear implants wear hearing aids in the opposite ear?

PubMed

Ching, T Y; Psarros, C; Hill, M; Dillon, H; Incerti, P

2001-10-01

The aim of this study was to investigate 1) whether a hearing aid needs to be adjusted differently depending on whether a child wears a cochlear implant or another hearing aid in the contralateral ear; 2) whether the use of a hearing aid and a cochlear implant in opposite ears leads to binaural interference; and 3) whether the use of a hearing aid and a cochlear implant in opposite ears leads to binaural benefits in speech perception, localization, and communicative functioning in real life. Sixteen children participated in this study. All children used a Nucleus 22 or Nucleus 24 cochlear implant system programmed with the SPEAK strategy in one ear. The hearing aid amplification requirements in the nonimplanted ear of these children were determined using two procedures. A paired comparison technique was used to identify the frequency response that was best for speech intelligibility in quiet, and a loudness balancing technique was used to match the loudness of speech in the ear with a hearing aid to that with a cochlear implant. Eleven of the 16 children participated in the investigation of binaural effects. Performance in speech perception, localization, and communicative functioning was assessed under four aided conditions: cochlear implant with hearing aid as worn, cochlear implant alone, hearing aid alone, and cochlear implant with hearing aid adjusted according to individual requirements. Fifteen of the 16 children whose amplification requirements were determined preferred a hearing aid frequency response that was within +/-6 dB/octave of the NAL-RP prescription. On average, the children required 6 dB more gain than prescribed to balance the loudness of the implanted ear for a speech signal presented at 65 dB SPL. For all 11 children whose performance was evaluated for investigating binaural effects, there was no indication of significantly poorer performance under bilaterally aided conditions compared with unilaterally aided conditions. On average, there were significant benefits in speech perception, localization, and aural/oral function when the children used cochlear implants with adjusted hearing aids than when they used cochlear implants alone. All individuals showed benefits in at least one of the measures. Hearing aids for children who also use cochlear implants can be selected using the NAL-RP prescription. Adjustment of hearing aid gain to match loudness in the implanted ear can facilitate integration of signals from both ears, leading to better speech perception. Given that there are binaural advantages from using cochlear implants with hearing aids in opposite ears, clinicians should advise parents and other professionals about these potential advantages, and facilitate bilateral amplification by adjusting hearing aids after stable cochlear implant MAPs are established.

Assessment of Spectral and Temporal Resolution in Cochlear Implant Users Using Psychoacoustic Discrimination and Speech Cue Categorization.

PubMed

Winn, Matthew B; Won, Jong Ho; Moon, Il Joon

This study was conducted to measure auditory perception by cochlear implant users in the spectral and temporal domains, using tests of either categorization (using speech-based cues) or discrimination (using conventional psychoacoustic tests). The authors hypothesized that traditional nonlinguistic tests assessing spectral and temporal auditory resolution would correspond to speech-based measures assessing specific aspects of phonetic categorization assumed to depend on spectral and temporal auditory resolution. The authors further hypothesized that speech-based categorization performance would ultimately be a superior predictor of speech recognition performance, because of the fundamental nature of speech recognition as categorization. Nineteen cochlear implant listeners and 10 listeners with normal hearing participated in a suite of tasks that included spectral ripple discrimination, temporal modulation detection, and syllable categorization, which was split into a spectral cue-based task (targeting the /ba/-/da/ contrast) and a timing cue-based task (targeting the /b/-/p/ and /d/-/t/ contrasts). Speech sounds were manipulated to contain specific spectral or temporal modulations (formant transitions or voice onset time, respectively) that could be categorized. Categorization responses were quantified using logistic regression to assess perceptual sensitivity to acoustic phonetic cues. Word recognition testing was also conducted for cochlear implant listeners. Cochlear implant users were generally less successful at utilizing both spectral and temporal cues for categorization compared with listeners with normal hearing. For the cochlear implant listener group, spectral ripple discrimination was significantly correlated with the categorization of formant transitions; both were correlated with better word recognition. Temporal modulation detection using 100- and 10-Hz-modulated noise was not correlated either with the cochlear implant subjects' categorization of voice onset time or with word recognition. Word recognition was correlated more closely with categorization of the controlled speech cues than with performance on the psychophysical discrimination tasks. When evaluating people with cochlear implants, controlled speech-based stimuli are feasible to use in tests of auditory cue categorization, to complement traditional measures of auditory discrimination. Stimuli based on specific speech cues correspond to counterpart nonlinguistic measures of discrimination, but potentially show better correspondence with speech perception more generally. The ubiquity of the spectral (formant transition) and temporal (voice onset time) stimulus dimensions across languages highlights the potential to use this testing approach even in cases where English is not the native language.

Where is the spike generator of the cochlear nerve? Voltage-gated sodium channels in the mouse cochlea.

PubMed

Hossain, Waheeda A; Antic, Srdjan D; Yang, Yang; Rasband, Matthew N; Morest, D Kent

2005-07-20

The origin of the action potential in the cochlea has been a long-standing puzzle. Because voltage-dependent Na+ (Nav) channels are essential for action potential generation, we investigated the detailed distribution of Nav1.6 and Nav1.2 in the cochlear ganglion, cochlear nerve, and organ of Corti, including the type I and type II ganglion cells. In most type I ganglion cells, Nav1.6 was present at the first nodes flanking the myelinated bipolar cell body and at subsequent nodes of Ranvier. In the other ganglion cells, including type II, Nav1.6 clustered in the initial segments of both of the axons that flank the unmyelinated bipolar ganglion cell bodies. In the organ of Corti, Nav1.6 was localized in the short segments of the afferent axons and their sensory endings beneath each inner hair cell. Surprisingly, the outer spiral fibers and their sensory endings were well labeled beneath the outer hair cells over their entire trajectory. In contrast, Nav1.2 in the organ of Corti was localized to the unmyelinated efferent axons and their endings on the inner and outer hair cells. We present a computational model illustrating the potential role of the Nav channel distribution described here. In the deaf mutant quivering mouse, the localization of Nav1.6 was disrupted in the sensory epithelium and ganglion. Together, these results suggest that distinct Nav channels generate and regenerate action potentials at multiple sites along the cochlear ganglion cells and nerve fibers, including the afferent endings, ganglionic initial segments, and nodes of Ranvier.

Hidden Hearing Injury: The Emerging Science and Military Relevance of Cochlear Synaptopathy.

PubMed

Tepe, Victoria; Smalt, Christopher; Nelson, Jeremy; Quatieri, Thomas; Pitts, Kenneth

2017-09-01

The phenomenon recently described as "hidden hearing loss" was the subject of a meeting co-hosted by the Department of Defense Hearing Center of Excellence and MIT Lincoln Laboratory to consider the potential relevance of noise-related synaptopathic injury to military settings and performance, service-related injury scenarios, and military medical priorities. Participants included approximately 50 researchers and subject matter experts from academic, federal, and military laboratories. Here we present a synthesis of discussion topics and concerns, as well as specific research objectives identified to develop militarily relevant knowledge. We consider findings from studies to date that have demonstrated cochlear synaptopathy and neurodegenerative processes apparently linked to noise exposure in animal models. We explore the potential relevance of these findings to the prediction and prevention of military hearing injuries, and to comorbid injuries in the neurological domain. Noise-induced cochlear synaptopathic injury is not detected by conventional audiometric assessment of threshold sensitivity. Animal studies suggest there may be a generous window of opportunity for intervention to mitigate or prevent cochlear neurodegenerative processes, e.g., by administration of neurotrophins or antioxidants. However, it is not yet known if the mechanisms that underlie "hidden hearing loss" also occur in human beings or, if so, how to identify them early, and how and when to intervene. Neurological injuries resulting from noise exposures via the auditory system have potentially significant implications for military Service Member performance, long-term Veteran health, and noise exposure standards. Mediated via auditory pathways, such injuries have possible relationship to clinical impairments including speech perception, and may be a largely overlooked contributor to cognitive symptoms associated with other military service-related injuries such as blast exposure and brain trauma. The potential health and performance consequences of noise-induced cochlear synaptopathic injury are easily overlooked, especially if it is assumed that hearing threshold sensitivity loss is the major concern. There should be a renewed impetus to further characterize and model synaptopathic mechanisms of auditory injury; study its potential impact on human auditory function, cognition, and performance metrics of military relevance; and develop solutions for auditory protection (including noise dosimetry) and treatment if appropriate following noise or blast exposure in military scenarios. We identify specific problems, solution objectives, and research objectives. Recommended research calls for a multidisciplinary approach to address cochlear nerve synaptopathy, central (brain) dysfunction, noise exposure measurement and metrics, and clinical assessment. Reprint & Copyright © 2017 Association of Military Surgeons of the U.S.

Functional expression of P2X4 receptor in capillary endothelial cells of the cochlear spiral ligament and its role in regulating the capillary diameter.

PubMed

Wu, T; Dai, M; Shi, X R; Jiang, Z G; Nuttall, A L

2011-07-01

The cochlear lateral wall generates the endocochlear potential (EP), which creates a driving force for the hair cell transduction current and is essential for normal hearing. Blood flow at the cochlear lateral wall is critically important for maintaining the EP. The vulnerability of the EP to hypoxia suggests that the blood flow in the cochlear lateral wall is dynamically and precisely regulated to meet the changing metabolic needs of the cochlear lateral wall. It has been reported that ATP, an important extracellular signaling molecule, plays an essential role in regulating cochlear blood flow. However, the cellular mechanism underlying ATP-induced regional blood flow changes has not been investigated. In the current study, we demonstrate that 1) the P2X4 receptor is expressed in endothelial cells (ECs) of spiral ligament (SL) capillaries. 2) ATP elicits a characteristic current through P2X4 on ECs in a dose-dependent manner (EC(50) = 0.16 mM). The ATP current has a reversal potential at ∼0 mV; is inhibited by 5-(3-bromophenyl)-1,3-dihydro-2H-benzofuro[3,2-e]-1,4-diazepin-2-one (5-BDBD), LaCl(3), pyridoxal phosphate-6-azo(benzene-2,4-disulfonic acid) tetrasodium salt hydrate (PPADS), and extracellular acidosis; and is less sensitive to α,β-methyleneadenosine 5'-triphosphate (α,β-MeATP) and 2'- and 3'-O-(4-benzoyl-benzoyl) adenosine 5'-triphosphate (BzATP). 3) ATP elicits a transient increase of intracellular Ca(2+) in ECs. 4) In accordance with the above in vitro findings, perilymphatic ATP (1 mM) caused dilation in SL capillaries in vivo by 11.5%. N(ω)-nitro-l-arginine methyl ester hydrochloride (l-NAME), a nonselective inhibitor of nitric oxide synthase, or 5-BDBD, the specific P2X4 inhibitor, significantly blocked the dilation. These findings support our hypothesis that extracellular ATP regulates cochlear lateral blood flow through P2X4 activation in ECs.

Musical experience sharpens human cochlear tuning.

PubMed

Bidelman, Gavin M; Nelms, Caitlin; Bhagat, Shaum P

2016-05-01

The mammalian cochlea functions as a filter bank that performs a spectral, Fourier-like decomposition on the acoustic signal. While tuning can be compromised (e.g., broadened with hearing impairment), whether or not human cochlear frequency resolution can be sharpened through experiential factors (e.g., training or learning) has not yet been established. Previous studies have demonstrated sharper psychophysical tuning curves in trained musicians compared to nonmusicians, implying superior peripheral tuning. However, these findings are based on perceptual masking paradigms, and reflect engagement of the entire auditory system rather than cochlear tuning, per se. Here, by directly mapping physiological tuning curves from stimulus frequency otoacoustic emissions (SFOAEs)-cochlear emitted sounds-we show that estimates of human cochlear tuning in a high-frequency cochlear region (4 kHz) is further sharpened (by a factor of 1.5×) in musicians and improves with the number of years of their auditory training. These findings were corroborated by measurements of psychophysical tuning curves (PTCs) derived via simultaneous masking, which similarly showed sharper tuning in musicians. Comparisons between SFOAE and PTCs revealed closer correspondence between physiological and behavioral curves in musicians, indicating that tuning is also more consistent between different levels of auditory processing in trained ears. Our findings demonstrate an experience-dependent enhancement in the resolving power of the cochlear sensory epithelium and the spectral resolution of human hearing and provide a peripheral account for the auditory perceptual benefits observed in musicians. Both local and feedback (e.g., medial olivocochlear efferent) mechanisms are discussed as potential mechanisms for experience-dependent tuning. Copyright © 2016 Elsevier B.V. All rights reserved.

Variation analysis of transcriptome changes reveals cochlear genes and their associated functions in cochlear susceptibility to acoustic overstimulation.

PubMed

Yang, Shuzhi; Cai, Qunfeng; Bard, Jonathan; Jamison, Jennifer; Wang, Jianmin; Yang, Weiping; Hu, Bo Hua

2015-12-01

Individual variation in the susceptibility of the auditory system to acoustic overstimulation has been well-documented at both the functional and structural levels. However, the molecular mechanism responsible for this variation is unclear. The current investigation was designed to examine the variation patterns of cochlear gene expression using RNA-seq data and to identify the genes with expression variation that increased following acoustic trauma. This study revealed that the constitutive expressions of cochlear genes displayed diverse levels of gene-specific variation. These variation patterns were altered by acoustic trauma; approximately one-third of the examined genes displayed marked increases in their expression variation. Bioinformatics analyses revealed that the genes that exhibited increased variation were functionally related to cell death, biomolecule metabolism, and membrane function. In contrast, the stable genes were primarily related to basic cellular processes, including protein and macromolecular syntheses and transport. There was no functional overlap between the stable and variable genes. Importantly, we demonstrated that glutamate metabolism is related to the variation in the functional response of the cochlea to acoustic overstimulation. Taken together, the results indicate that our analyses of the individual variations in transcriptome changes of cochlear genes provide important information for the identification of genes that potentially contribute to the generation of individual variation in cochlear responses to acoustic overstimulation. Copyright © 2015 Elsevier B.V. All rights reserved.

Contribution of Family Environment to Pediatric Cochlear Implant Users’ Speech and Language Outcomes: Some Preliminary Findings

PubMed Central

Holt, Rachael Frush; Beer, Jessica; Kronenberger, William G.; Pisoni, David B.; Lalonde, Kaylah

2012-01-01

Purpose To evaluate the family environments of children with cochlear implants and to examine relationships between family environment and post-implant language development and executive function. Method Forty-five families of children with cochlear implants completed a self-report family environment questionnaire (FES) and an inventory of executive function (BRIEF/BRIEF-P). Children’s receptive vocabulary (PPVT-4) and global language skills (PLS-4/CELF-4) were also evaluated. Results The family environments of children with cochlear implants differed from those of normal-hearing children, but not in clinically significant ways. Language development and executive function were found to be atypical, but not uncharacteristic of this clinical population. Families with higher levels of self-reported control had children with smaller vocabularies. Families reporting a higher emphasis on achievement had children with fewer executive function and working memory problems. Finally, families reporting a higher emphasis on organization had children with fewer problems related to inhibition. Conclusions Some of the variability in cochlear implantation outcomes that have protracted periods of development is related to family environment. Because family environment can be modified and enhanced by therapy or education, these preliminary findings hold promise for future work in helping families to create robust language-learning environments that can maximize their child’s potential with a cochlear implant. PMID:22232387

Questioning cochlear amplification

NASA Astrophysics Data System (ADS)

van der Heijden, Marcel; Versteegh, Corstiaen P. C.

2015-12-01

Thirty years ago it was hypothesized that motile processes inject mechanical energy into cochlear traveling waves. This mechanical amplification, alternatively described as negative damping, is invoked to explain both the sensitivity and the nonlinear compression of cochlear responses. There is a recent trend to present cochlear amplification as an established fact, even though the evidence is at most circumstantial and several thorny problems have remained unresolved. We analyze several of these issues, and present new basilar membrane recordings that allowed us to quantify cochlear energy flow. Specifically, we address the following questions: (1) Does auditory sensitivity require narrowband amplification? (2) Has the "RC problem" (lowpass filtering of outer hair cell receptor potential) been resolved? (3) Can OHC motility improve auditory sensitivity? (4) Is there a net power gain between neighboring locations on the basilar membrane? The analyses indicate that mechanical amplification in the cochlea is neither necessary nor useful, and that realizing it by known forms of motility would reduce sensitivity rather than enhance it. Finally, our experimental data show that the peaking of the traveling wave is realized by focusing the acoustic energy rather than amplifying it. (Abbreviations. BM: basilar membrane; CF: characteristic frequency; IHC: inner hair cell; ME: middle ear; MT; mechanotransducer; OHC: outer hair cell; SPL: sound pressure level.)

Evidence-based guidelines for recommending cochlear implantation for postlingually deafened adults.

PubMed

Leigh, Jaime R; Moran, Michelle; Hollow, Rodney; Dowell, Richard C

2016-01-01

Adult selection criteria for cochlear implantation have been developed based on analysis of the post-operative performance of a large group of postlingually deafened adults. Original criteria published in 2004 were reviewed and amended to reflect outcomes currently being achieved by implant recipients. Retrospective review of 12-month post-operative speech perception performance of adults implanted at the Eye and Ear Hospital, Melbourne, Australia. A total of 382 postlingually deafened adults, using a Freedom, Nucleus 5, or CI422 Slim Straight cochlear implant were used to create a comparative set of data. Revised guidelines suggest that adults with postlingual hearing loss can now be considered cochlear implant candidates if they obtain scores of up to 55% for open-set phonemes in quiet in the ear to be implanted. Functional benefit may vary depending on the recipients' contralateral hearing. This study supports the provision of cochlear implants to candidates with significant residual hearing when at least one ear meets the criterion outlined above. Patient-specific counseling is required to ensure the potential to benefit predicted by the current model is acceptable to the individual patient and their family. Counseling regarding functional benefit must take into consideration hearing in the contralateral ear.

Chronic lead exposure induces cochlear oxidative stress and potentiates noise-induced hearing loss.

PubMed

Jamesdaniel, Samson; Rosati, Rita; Westrick, Judy; Ruden, Douglas M

2018-08-01

Acquired hearing loss is caused by complex interactions of multiple environmental risk factors, such as elevated levels of lead and noise, which are prevalent in urban communities. This study delineates the mechanism underlying lead-induced auditory dysfunction and its potential interaction with noise exposure. Young-adult C57BL/6 mice were exposed to: 1) control conditions; 2) 2 mM lead acetate in drinking water for 28 days; 3) 90 dB broadband noise 2 h/day for two weeks; and 4) both lead and noise. Blood lead levels were measured by inductively coupled plasma mass spectrometry analysis (ICP-MS) lead-induced cochlear oxidative stress signaling was assessed using targeted gene arrays, and the hearing thresholds were assessed by recording auditory brainstem responses. Chronic lead exposure downregulated cochlear Sod1, Gpx1, and Gstk1, which encode critical antioxidant enzymes, and upregulated ApoE, Hspa1a, Ercc2, Prnp, Ccl5, and Sqstm1, which are indicative of cellular apoptosis. Isolated exposure to lead or noise induced 8-12 dB and 11-25 dB shifts in hearing thresholds, respectively. Combined exposure induced 18-30 dB shifts, which was significantly higher than that observed with isolated exposures. This study suggests that chronic exposure to lead induces cochlear oxidative stress and potentiates noise-induced hearing impairment, possibly through parallel pathways. Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.

Adenosine and the Auditory System

PubMed Central

Vlajkovic, Srdjan M; Housley, Gary D; Thorne, Peter R

2009-01-01

Adenosine is a signalling molecule that modulates cellular activity in the central nervous system and peripheral organs via four G protein-coupled receptors designated A1, A2A, A2B, and A3. This review surveys the literature on the role of adenosine in auditory function, particularly cochlear function and its protection from oxidative stress. The specific tissue distribution of adenosine receptors in the mammalian cochlea implicates adenosine signalling in sensory transduction and auditory neurotransmission although functional studies have demonstrated that adenosine stimulates cochlear blood flow, but does not alter the resting and sound-evoked auditory potentials. An interest in a potential otoprotective role for adenosine has recently evolved, fuelled by the capacity of A1 adenosine receptors to prevent cochlear injury caused by acoustic trauma and ototoxic drugs. The balance between A1 and A2A receptors is conceived as critical for cochlear response to oxidative stress, which is an underlying mechanism of the most common inner ear pathologies (e.g. noise-induced and age-related hearing loss, drug ototoxicity). Enzymes involved in adenosine metabolism, adenosine kinase and adenosine deaminase, are also emerging as attractive targets for controlling oxidative stress in the cochlea. Other possible targets include ectonucleotidases that generate adenosine from extracellular ATP, and nucleoside transporters, which regulate adenosine concentrations on both sides of the plasma membrane. Developments of selective adenosine receptor agonists and antagonists that can cross the blood-cochlea barrier are bolstering efforts to develop therapeutic interventions aimed at ameliorating cochlear injury. Manipulations of the adenosine signalling system thus hold significant promise in the therapeutic management of oxidative stress in the cochlea. PMID:20190966

Relationship between auditory thresholds, central spontaneous activity and hair cell loss after acoustic trauma

PubMed Central

Mulders, W.H.A.M.; Ding, D.; Salvi, R.; Robertson, D.

2011-01-01

Acoustic trauma caused by exposure to a very loud sound increases spontaneous activity in central auditory structures such as the inferior colliculus. This hyperactivity has been suggested as a neural substrate for tinnitus, a phantom hearing sensation. In previous studies we have described a tentative link between the frequency region of hearing impairment and the corresponding tonotopic regions in the inferior colliculus showing hyperactivity. In this study we further investigated the relationship between cochlear compound action potential threshold loss, cochlear outer and inner hair cell loss and central hyperactivity in inferior colliculus of guinea pigs. Two weeks after a 10 kHz pure tone acoustic trauma, a tight relationship was demonstrated between the frequency region of compound action potential threshold loss and frequency regions in the inferior colliculus showing hyperactivity. Extending the duration of the acoustic trauma from 1 to 2 h did not result in significant increases in final cochlear threshold loss, but did result in a further increase of spontaneous firing rates in the inferior colliculus. Interestingly, hair cell loss was not present in the frequency regions where elevated cochlear thresholds and central hyperactivity were measured, suggesting that subtle changes in hair cell or primary afferent neural function are sufficient for central hyperactivity to be triggered and maintained. PMID:21491427

Speaker normalization for chinese vowel recognition in cochlear implants.

PubMed

Luo, Xin; Fu, Qian-Jie

2005-07-01

Because of the limited spectra-temporal resolution associated with cochlear implants, implant patients often have greater difficulty with multitalker speech recognition. The present study investigated whether multitalker speech recognition can be improved by applying speaker normalization techniques to cochlear implant speech processing. Multitalker Chinese vowel recognition was tested with normal-hearing Chinese-speaking subjects listening to a 4-channel cochlear implant simulation, with and without speaker normalization. For each subject, speaker normalization was referenced to the speaker that produced the best recognition performance under conditions without speaker normalization. To match the remaining speakers to this "optimal" output pattern, the overall frequency range of the analysis filter bank was adjusted for each speaker according to the ratio of the mean third formant frequency values between the specific speaker and the reference speaker. Results showed that speaker normalization provided a small but significant improvement in subjects' overall recognition performance. After speaker normalization, subjects' patterns of recognition performance across speakers changed, demonstrating the potential for speaker-dependent effects with the proposed normalization technique.

Analysis of the cochlear microphonic to a low-frequency tone embedded in filtered noise

PubMed Central

Chertoff, Mark E.; Earl, Brian R.; Diaz, Francisco J.; Sorensen, Janna L.

2012-01-01

The cochlear microphonic was recorded in response to a 733 Hz tone embedded in noise that was high-pass filtered at 25 different frequencies. The amplitude of the cochlear microphonic increased as the high-pass cutoff frequency of the noise increased. The amplitude growth for a 60 dB SPL tone was steeper and saturated sooner than that of an 80 dB SPL tone. The growth for both signal levels, however, was not entirely cumulative with plateaus occurring at about 4 and 7 mm from the apex. A phenomenological model of the electrical potential in the cochlea that included a hair cell probability function and spiral geometry of the cochlea could account for both the slope of the growth functions and the plateau regions. This suggests that with high-pass-filtered noise, the cochlear microphonic recorded at the round window comes from the electric field generated at the source directed towards the electrode and not down the longitudinal axis of the cochlea. PMID:23145616

Robot-assisted three-dimensional registration for cochlear implant surgery using a common-path swept-source optical coherence tomography probe

NASA Astrophysics Data System (ADS)

Gurbani, Saumya S.; Wilkening, Paul; Zhao, Mingtao; Gonenc, Berk; Cheon, Gyeong Woo; Iordachita, Iulian I.; Chien, Wade; Taylor, Russell H.; Niparko, John K.; Kang, Jin U.

2014-05-01

Cochlear implantation offers the potential to restore sensitive hearing in patients with severe to profound deafness. However, surgical placement of the electrode array within the cochlea can produce trauma to sensorineural components, particularly if the initial turn of the cochlea is not successfully navigated as the array is advanced. In this work, we present a robot-mounted common-path swept-source optical coherence tomography endoscopic platform for three-dimensional (3-D) optical coherence tomography (OCT) registration and preoperative surgical planning for cochlear implant surgery. The platform is composed of a common-path 600-μm diameter fiber optic rotary probe attached to a five degrees of freedom robot capable of 1 μm precision movement. The system is tested on a dry fixed ex vivo human temporal bone, and we demonstrate the feasibility of a 3-D OCT registration of the cochlea to accurately describe the spatial and angular profiles of the canal formed by the scala tympani into the first cochlear turn.

Surgical evaluation of candidates for cochlear implants

NASA Technical Reports Server (NTRS)

Black, F. O.; Lilly, D. J.; Fowler, L. P.; Stypulkowski, P. H.

1987-01-01

The customary presentation of surgical procedures to patients in the United States consists of discussions on alternative treatment methods, risks of the procedure(s) under consideration, and potential benefits for the patient. Because the contents of the normal speech signal have not been defined in a way that permits a surgeon systematically to provide alternative auditory signals to a deaf patient, the burden is placed on the surgeon to make an arbitrary selection of candidates and available devices for cochlear prosthetic implantation. In an attempt to obtain some information regarding the ability of a deaf patient to use electrical signals to detect and understand speech, the Good Samaritan Hospital and Neurological Sciences Institute cochlear implant team has routinely performed tympanotomies using local anesthesia and has positioned temporary electrodes onto the round windows of implant candidates. The purpose of this paper is to review our experience with this procedure and to provide some observations that may be useful in a comprehensive preoperative evaluation for totally deaf patients who are being considered for cochlear implantation.

The Group Delay and Suppression Pattern of the Cochlear Microphonic Potential Recorded at the Round Window

PubMed Central

He, Wenxuan; Porsov, Edward; Kemp, David; Nuttall, Alfred L.; Ren, Tianying

2012-01-01

Background It is commonly assumed that the cochlear microphonic potential (CM) recorded from the round window (RW) is generated at the cochlear base. Based on this assumption, the low-frequency RW CM has been measured for evaluating the integrity of mechanoelectrical transduction of outer hair cells at the cochlear base and for studying sound propagation inside the cochlea. However, the group delay and the origin of the low-frequency RW CM have not been demonstrated experimentally. Methodology/Principal Findings This study quantified the intra-cochlear group delay of the RW CM by measuring RW CM and vibrations at the stapes and basilar membrane in gerbils. At low sound levels, the RW CM showed a significant group delay and a nonlinear growth at frequencies below 2 kHz. However, at high sound levels or at frequencies above 2 kHz, the RW CM magnitude increased proportionally with sound pressure, and the CM phase in respect to the stapes showed no significant group delay. After the local application of tetrodotoxin the RW CM below 2 kHz became linear and showed a negligible group delay. In contrast to RW CM phase, the BM vibration measured at location ∼2.5 mm from the base showed high sensitivity, sharp tuning, and nonlinearity with a frequency-dependent group delay. At low or intermediate sound levels, low-frequency RW CMs were suppressed by an additional tone near the probe-tone frequency while, at high sound levels, they were partially suppressed only at high frequencies. Conclusions/Significance We conclude that the group delay of the RW CM provides no temporal information on the wave propagation inside the cochlea, and that significant group delay of low-frequency CMs results from the auditory nerve neurophonic potential. Suppression data demonstrate that the generation site of the low-frequency RW CM shifts from apex to base as the probe-tone level increases. PMID:22470560

Development and evaluation of the Nurotron 26-electrode cochlear implant system.

PubMed

Zeng, Fan-Gang; Rebscher, Stephen J; Fu, Qian-Jie; Chen, Hongbin; Sun, Xiaoan; Yin, Li; Ping, Lichuan; Feng, Haihong; Yang, Shiming; Gong, Shusheng; Yang, Beibei; Kang, Hou-Yong; Gao, Na; Chi, Fanglu

2015-04-01

Although the cochlear implant has been widely acknowledged as the most successful neural prosthesis, only a fraction of hearing-impaired people who can potentially benefit from a cochlear implant have actually received one due to its limited awareness, accessibility, and affordability. To help overcome these limitations, a 26-electrode cochlear implant has been developed to receive China's Food and Drug Administration (CFDA) approval in 2011 and Conformité Européenne (CE) Marking in 2012. The present article describes design philosophy, system specification, and technical verification of the Nurotron device, which includes advanced digital signal processing and 4 current sources with multiple amplitude resolutions that not only are compatible with perceptual capability but also allow interleaved or simultaneous stimulation. The article also presents 3-year longitudinal evaluation data from 60 human subjects who have received the Nurotron device. The objective measures show that electrode impedance decreased within the first month of device use, but was stable until a slight increase at the end of two years. The subjective loudness measures show that electric stimulation threshold was stable while the maximal comfort level increased over the 3 years. Mandarin sentence recognition increased from the pre-surgical 0%-correct score to a plateau of about 80% correct with 6-month use of the device. Both indirect and direct comparisons indicate indistinguishable performance differences between the Nurotron system and other commercially available devices. The present 26-electrode cochlear implant has already helped to lower the price of cochlear implantation in China and will likely contribute to increased cochlear implant access and success in the rest of the world. This article is part of a Special Issue entitled . Copyright © 2014 The Authors. Published by Elsevier B.V. All rights reserved.

Value of intracochlear electrically evoked auditory brainstem response after cochlear implantation in patients with narrow internal auditory canal.

PubMed

Song, Mee Hyun; Bae, Mi Ran; Kim, Hee Nam; Lee, Won-Sang; Yang, Won Sun; Choi, Jae Young

2010-08-01

Cochlear implantation in patients with narrow internal auditory canal (IAC) can result in variable outcomes; however, preoperative evaluations have limitations in accurately predicting outcomes. In this study, we analyzed the outcomes of cochlear implantation in patients with narrow IAC and correlated the intracochlear electrically evoked auditory brainstem response (EABR) findings to postoperative performance to determine the prognostic significance of intracochlear EABR. Retrospective case series at a tertiary hospital. Thirteen profoundly deaf patients with narrow IAC who received cochlear implantation from 2002 to 2008 were included in this study. Postoperative performance was evaluated after at least 12 months of follow-up, and postoperative intracochlear EABR was measured to determine its correlation with outcome. The clinical significance of electrically evoked compound action potential (ECAP) was also analyzed. Patients with narrow IAC showed postoperative auditory performances ranging from CAP 0 to 4 after cochlear implantation. Intracochlear EABR measured postoperatively demonstrated prognostic value in the prediction of long-term outcomes, whereas ECAP measurements failed to show a significant correlation with outcome. Consistent with the advantages of intracochlear EABR over extracochlear EABR, this study demonstrates that intracochlear EABR has prognostic significance in predicting long-term outcomes in patients with narrow IAC. Intracochlear EABR measured either intraoperatively or in the early postoperative period may play an important role in deciding whether to continue with auditory rehabilitation using a cochlear implant or to switch to an auditory brainstem implant so as not to miss the optimal timing for language development.

Non-ototoxic local delivery of bisphosphonate to the mammalian cochlea

PubMed Central

Kang, Woo Seok; Sun, Shuting; Nguyen, Kim; Kashemirov, Boris; McKenna, Charles E.; Hacking, S. Adam; Quesnel, Alicia M.; Sewell, William F.; McKenna, Michael J.; Jung, David H.

2015-01-01

Hypothesis Local delivery of bisphosphonates results in superior localization of these compounds for the treatment of cochlear otosclerosis, without ototoxicity. Background Otosclerosis is a common disorder of abnormal bone remodeling within the human otic capsule. It is a frequent cause of conductive hearing loss from stapes fixation. Large lesions that penetrate the cochlear endosteum and injure the spiral ligament result in sensorineural hearing loss. Nitrogen-containing bisphosphonates (e.g., zoledronate) are potent inhibitors of bone remodeling with proven efficacy in the treatment of metabolic bone diseases, including otosclerosis. Local delivery to the cochlea may allow for improved drug targeting, higher local concentrations, and the avoidance of systemic complications. In this study, we utilize a fluorescently labeled bisphosphonate compound (6-FAM-ZOL) to determine drug localization and concentration within the otic capsule. Various methods for delivery are compared. Ototoxicity is evaluated by ABR and DPOAEs. Methods 6-FAM-ZOL was administered to guinea pigs via intraperitoneal injection, placement of alginate beads onto the round window membrane (RWM), or microfluidic pump infusion via a cochleostomy. Hearing was evaluated. Specimens were embedded into resin blocks, ground to a mid-modiolar section, and quantitatively imaged using fluorescence microscopy. Results There was a dose-dependent increase in fluorescent signal following systemic 6-FAM-ZOL treatment. Local delivery via the RWM or a cochleostomy increased delivery efficiency. No significant ototoxicity was observed following either systemic or local 6-FAM-ZOL delivery. Conclusions These findings establish important pre-clinical parameters for the treatment of cochlear otosclerosis in humans. PMID:25996080

Thin and open vessel windows for intra-vital fluorescence imaging of murine cochlear blood flow

PubMed Central

Shi, Xiaorui; Zhang, Fei; Urdang, Zachary; Dai, Min; Neng, Lingling; Zhang, Jinhui; Chen, Songlin; Ramamoorthy, Sripriya; Nuttall, Alfred L.

2014-01-01

Normal microvessel structure and function in the cochlea is essential for maintaining the ionic and metabolic homeostasis required for hearing function. Abnormal cochlear microcirculation has long been considered an etiologic factor in hearing disorders. A better understanding of cochlear blood flow (CoBF) will enable more effective amelioration of hearing disorders that result from aberrant blood flow. However, establishing the direct relationship between CoBF and other cellular events in the lateral wall and response to physio-pathological stress remains a challenge due to the lack of feasible interrogation methods and difficulty in accessing the inner ear. Here we report on new methods for studying the CoBF in a mouse model using a thin or open vessel-window in combination with fluorescence intra-vital microscopy (IVM). An open vessel-window enables investigation of vascular cell biology and blood flow permeability, including pericyte (PC) contractility, bone marrow cell migration, and endothelial barrier leakage, in wild type and fluorescent protein-labeled transgenic mouse models with high spatial and temporal resolution. Alternatively, the thin vessel-window method minimizes disruption of the homeostatic balance in the lateral wall and enables study CoBF under relatively intact physiological conditions. A thin vessel-window method can also be used for time-based studies of physiological and pathological processes. Although the small size of the mouse cochlea makes surgery difficult, the methods are sufficiently developed for studying the structural and functional changes in CoBF under normal and pathological conditions. PMID:24780131

Cochlear implantation (CI) for prelingual deafness: the relevance of studies of brain organization and the role of first language acquisition in considering outcome success.

PubMed

Campbell, Ruth; MacSweeney, Mairéad; Woll, Bencie

2014-01-01

Cochlear implantation (CI) for profound congenital hearing impairment, while often successful in restoring hearing to the deaf child, does not always result in effective speech processing. Exposure to non-auditory signals during the pre-implantation period is widely held to be responsible for such failures. Here, we question the inference that such exposure irreparably distorts the function of auditory cortex, negatively impacting the efficacy of CI. Animal studies suggest that in congenital early deafness there is a disconnection between (disordered) activation in primary auditory cortex (A1) and activation in secondary auditory cortex (A2). In humans, one factor contributing to this functional decoupling is assumed to be abnormal activation of A1 by visual projections-including exposure to sign language. In this paper we show that that this abnormal activation of A1 does not routinely occur, while A2 functions effectively supramodally and multimodally to deliver spoken language irrespective of hearing status. What, then, is responsible for poor outcomes for some individuals with CI and for apparent abnormalities in cortical organization in these people? Since infancy is a critical period for the acquisition of language, deaf children born to hearing parents are at risk of developing inefficient neural structures to support skilled language processing. A sign language, acquired by a deaf child as a first language in a signing environment, is cortically organized like a heard spoken language in terms of specialization of the dominant perisylvian system. However, very few deaf children are exposed to sign language in early infancy. Moreover, no studies to date have examined sign language proficiency in relation to cortical organization in individuals with CI. Given the paucity of such relevant findings, we suggest that the best guarantee of good language outcome after CI is the establishment of a secure first language pre-implant-however that may be achieved, and whatever the success of auditory restoration.

Cochlear implantation (CI) for prelingual deafness: the relevance of studies of brain organization and the role of first language acquisition in considering outcome success

PubMed Central

Campbell, Ruth; MacSweeney, Mairéad; Woll, Bencie

2014-01-01

Cochlear implantation (CI) for profound congenital hearing impairment, while often successful in restoring hearing to the deaf child, does not always result in effective speech processing. Exposure to non-auditory signals during the pre-implantation period is widely held to be responsible for such failures. Here, we question the inference that such exposure irreparably distorts the function of auditory cortex, negatively impacting the efficacy of CI. Animal studies suggest that in congenital early deafness there is a disconnection between (disordered) activation in primary auditory cortex (A1) and activation in secondary auditory cortex (A2). In humans, one factor contributing to this functional decoupling is assumed to be abnormal activation of A1 by visual projections—including exposure to sign language. In this paper we show that that this abnormal activation of A1 does not routinely occur, while A2 functions effectively supramodally and multimodally to deliver spoken language irrespective of hearing status. What, then, is responsible for poor outcomes for some individuals with CI and for apparent abnormalities in cortical organization in these people? Since infancy is a critical period for the acquisition of language, deaf children born to hearing parents are at risk of developing inefficient neural structures to support skilled language processing. A sign language, acquired by a deaf child as a first language in a signing environment, is cortically organized like a heard spoken language in terms of specialization of the dominant perisylvian system. However, very few deaf children are exposed to sign language in early infancy. Moreover, no studies to date have examined sign language proficiency in relation to cortical organization in individuals with CI. Given the paucity of such relevant findings, we suggest that the best guarantee of good language outcome after CI is the establishment of a secure first language pre-implant—however that may be achieved, and whatever the success of auditory restoration. PMID:25368567

Mechanoelectrical transduction of adult outer hair cells studied in a gerbil hemicochlea.

PubMed

He, David Z Z; Jia, Shuping; Dallos, Peter

2004-06-17

Sensory receptor cells of the mammalian cochlea are morphologically and functionally dichotomized. Inner hair cells transmit auditory information to the brain, whereas outer hair cells (OHC) amplify the mechanical signal, which is then transduced by inner hair cells. Amplification by OHCs is probably mediated by their somatic motility in a mechanical feedback process. OHC motility in vivo is thought to be driven by the cell's receptor potential. The first steps towards the generation of the receptor potential are the deflection of the stereociliary bundle, and the subsequent flow of transducer current through the mechanosensitive transducer channels located at their tips. Quantitative relations between transducer currents and basilar membrane displacements are lacking, as well as their variation along the cochlear length. To address this, we simultaneously recorded OHC transducer currents (or receptor potentials) and basilar membrane motion in an excised and bisected cochlea, the hemicochlea. This preparation permits recordings from adult OHCs at various cochlear locations while the basilar membrane is mechanically stimulated. Furthermore, the stereocilia are deflected by the same means of stimulation as in vivo. Here we show that asymmetrical transducer currents and receptor potentials are significantly larger than previously thought, they possess a highly restricted dynamic range and strongly depend on cochlear location.

A deafness mechanism of digenic Cx26 (GJB2) and Cx30 (GJB6) mutations: Reduction of endocochlear potential by impairment of heterogeneous gap junctional function in the cochlear lateral wall.

PubMed

Mei, Ling; Chen, Jin; Zong, Liang; Zhu, Yan; Liang, Chun; Jones, Raleigh O; Zhao, Hong-Bo

2017-12-01

Digenic Connexin26 (Cx26, GJB2) and Cx30 (GJB6) heterozygous mutations are the second most frequent cause of recessive deafness in humans. However, the underlying deafness mechanism remains unclear. In this study, we created different double Cx26 and Cx30 heterozygous (Cx26 +/- /Cx30 +/- ) mouse models to investigate the underlying pathological changes and deafness mechanism. We found that double Cx26 +/- /Cx30 +/- heterozygous mice had hearing loss. Endocochlear potential (EP), which is a driving force for hair cells producing auditory receptor current, was reduced. However, unlike Cx26 homozygous knockout (Cx26 -/- ) mice, the cochlea in Cx26 +/- /Cx30 +/- mice displayed normal development and had no apparent hair cell degeneration. Gap junctions (GJs) in the cochlea form two independent networks: the epithelial cell GJ network in the organ of Corti and the connective tissue GJ network in the cochlear lateral wall. We further found that double heterozygous deletion of Cx26 and Cx30 in the epithelial cells did not reduce EP and had normal hearing, suggesting that Cx26 +/- /Cx30 +/- may mainly impair gap junctional functions in the cochlear lateral wall and lead to EP reduction and hearing loss. Most of Cx26 and Cx30 in the cochlear lateral wall co-expressed in the same gap junctional plaques. Moreover, sole Cx26 +/- or Cx30 +/- heterozygous mice had no hearing loss. These data further suggest that digenic Cx26 and Cx30 mutations may impair heterozygous coupling of Cx26 and Cx30 in the cochlear lateral wall to reduce EP, thereby leading to hearing loss. Copyright © 2017 Elsevier Inc. All rights reserved.

Systematic review of compound action potentials as predictors for cochlear implant performance.

PubMed

van Eijl, Ruben H M; Buitenhuis, Patrick J; Stegeman, Inge; Klis, Sjaak F L; Grolman, Wilko

2017-02-01

The variability in speech perception between cochlear implant users is thought to result from the degeneration of the auditory nerve. Degeneration of the auditory nerve, histologically assessed, correlates with electrophysiologically acquired measures, such as electrically evoked compound action potentials (eCAPs) in experimental animals. To predict degeneration of the auditory nerve in humans, where histology is impossible, this paper reviews the correlation between speech perception and eCAP recordings in cochlear implant patients. PubMed and Embase. We performed a systematic search for articles containing the following major themes: cochlear implants, evoked potentials, and speech perception. Two investigators independently conducted title-abstract screening, full-text screening, and critical appraisal. Data were extracted from the remaining articles. Twenty-five of 1,429 identified articles described a correlation between speech perception and eCAP attributes. Due to study heterogeneity, a meta-analysis was not feasible, and studies were descriptively analyzed. Several studies investigating presence of the eCAP, recovery time constant, slope of the amplitude growth function, and spatial selectivity showed significant correlations with speech perception. In contrast, neural adaptation, eCAP threshold, and change with varying interphase gap did not significantly correlate with speech perception in any of the identified studies. Significant correlations between speech perception and parameters obtained through eCAP recordings have been documented in literature; however, reporting was ambiguous. There is insufficient evidence for eCAPs as a predictive factor for speech perception. More research is needed to further investigate this relation. Laryngoscope, 2016 127:476-487, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Refinement of Molecular Diagnostic Protocol of Auditory Neuropathy Spectrum Disorder

PubMed Central

Chang, Mun Young; Kim, Ah Reum; Kim, Nayoung K.D.; Lee, Chung; Park, Woong-Yang; Choi, Byung Yoon

2015-01-01

Abstract Auditory neuropathy spectrum disorder (ANSD) is a sensorineural hearing disorder caused by dysfunction of auditory neural conduction. ANSD has a heterogeneous etiology, including genetic factors; the response to cochlear implantation significantly varies depending on the etiology. The results of timely cochlear implantation for OTOF-related ANSD (DFNB9) have been reported to be good. Therefore, identifying the causative gene of ANSD, especially OTOF, is an important issue to rehabilitate these patients. Six sporadic ANSD subjects without anatomical abnormality of the cochlear nerve, including the 4 subjects that were previously reported to be without detectable OTOF mutation, were included. We performed targeted resequencing (TRS) of known deafness genes and multiphasic bioinformatics analyses of the data that ensured detection of capture failure and structural variations. Exclusion of SNP was also double checked. The TRS data previously obtained from 2 subjects were reanalyzed. Through this study, we detected 2 mutant alleles of OTOF from 5 (83.3%) of 6 ANSD subjects. All of the 5 subjects carried at least 1 mutant allele carrying p.R1939Q. This variant was categorized as a simple SNP (rs201326023) in the database and it resided in the exon with frequent capture failures, which previously led to exclusion of this variant from eligible candidacy mistakenly. In addition, we detected a structural variation within OTOF from a previously undiagnosed ANSD subject, which was the second structural variation reported in DFNB9 subjects to date. We identify a strong etiologic homogeneity of prelingual ANSD in case of the anatomically normal cochlear nerve in Koreans and now report DFNB9 as the single overwhelming cause. Multiphasic analysis of TRS data ensuring detection of capture failure and structural variations would be expected to reveal DFNB9 from a substantial portion of previously undiagnosed ANSD subjects in Koreans. Based on our results, we propose a novel strategy that incorporates imaging studies, prevalent mutation screening and multiphasic analysis of TRS data in a stepwise manner to correctly detect DFNB9 in Koreans. PMID:26632695

Cochlear implant users move in time to the beat of drum music.

PubMed

Phillips-Silver, Jessica; Toiviainen, Petri; Gosselin, Nathalie; Turgeon, Christine; Lepore, Franco; Peretz, Isabelle

2015-03-01

Cochlear implant users show a profile of residual, yet poorly understood, musical abilities. An ability that has received little to no attention in this population is entrainment to a musical beat. We show for the first time that a heterogeneous group of cochlear implant users is able to find the beat and move their bodies in time to Latin Merengue music, especially when the music is presented in unpitched drum tones. These findings not only reveal a hidden capacity for feeling musical rhythm through the body in the deaf and hearing impaired population, but illuminate promising avenues for designing early childhood musical training that can engage implanted children in social musical activities with benefits potentially extending to non-musical domains. Copyright © 2015 Elsevier B.V. All rights reserved.

Coding of sounds in the auditory system and its relevance to signal processing and coding in cochlear implants.

PubMed

Moore, Brian C J

2003-03-01

To review how the properties of sounds are "coded" in the normal auditory system and to discuss the extent to which cochlear implants can and do represent these codes. Data are taken from published studies of the response of the cochlea and auditory nerve to simple and complex stimuli, in both the normal and the electrically stimulated ear. REVIEW CONTENT: The review describes: 1) the coding in the normal auditory system of overall level (which partly determines perceived loudness), spectral shape (which partly determines perceived timbre and the identity of speech sounds), periodicity (which partly determines pitch), and sound location; 2) the role of the active mechanism in the cochlea, and particularly the fast-acting compression associated with that mechanism; 3) the neural response patterns evoked by cochlear implants; and 4) how the response patterns evoked by implants differ from those observed in the normal auditory system in response to sound. A series of specific issues is then discussed, including: 1) how to compensate for the loss of cochlear compression; 2) the effective number of independent channels in a normal ear and in cochlear implantees; 3) the importance of independence of responses across neurons; 4) the stochastic nature of normal neural responses; 5) the possible role of across-channel coincidence detection; and 6) potential benefits of binaural implantation. Current cochlear implants do not adequately reproduce several aspects of the neural coding of sound in the normal auditory system. Improved electrode arrays and coding systems may lead to improved coding and, it is hoped, to better performance.

Exploring views on current and future cochlear implant service delivery: the perspectives of users, parents and professionals at cochlear implant centres and in the community.

PubMed

Athalye, Sheetal; Archbold, Sue; Mulla, Imran; Lutman, Mark; Nikolopoulous, Thomas

2015-09-01

The objective of this survey was to explore the perceptions of implant users/carers and professionals across the UK about current and future cochlear implant service delivery and the challenges. Data were collected via an online questionnaire consisting of totally 22 questions. The questionnaire contained both open- and close-ended questions. Totally, seven hundred and forty-eight responses were received. In spite of the wide range of respondents, there was a broad consensus of opinion across groups. The majority of participants were satisfied with the service they currently receive, but wanted some changes. They reported their current experience of implant services to be mainly driven by decisions made by the implant team. For the future, they preferred the service to be mainly driven by decisions made jointly by the team and the user and/or parent/carer. The majority of participants wanted the cochlear implant services to be integrated into local audiology and other services such as education. Restrictions on number of candidates funded and political decisions and issues were seen as major challenges. Qualitative analysis of the open-ended responses supported the questionnaire responses. This research highlighted the benefits and limitations of the current cochlear implant service delivery as well as the potential implications for the long term. While respondents were generally happy with the current cochlear implant service provision, they expressed some concerns about the long-term sustainability and management, wanting integration into the local services, and more involvement of parents and users in decisions.

Auditory maturation in premature infants: a potential pitfall for early cochlear implantation.

PubMed

Hof, Janny R; Stokroos, Robert J; Wix, Eduard; Chenault, Mickey; Gelders, Els; Brokx, Jan

2013-08-01

To describe spontaneous hearing improvement in the first years of life of a number of preterm neonates relative to cochlear implant candidacy. Retrospective case study. Hearing levels of 14 preterm neonates (mean gestational age at birth = 29 weeks) referred after newborn hearing screening were evaluated. Initial hearing thresholds ranged from 40 to 105 dBHL (mean = 85 dBHL). Hearing level improved to normal levels for four neonates and to moderate levels for five, whereas for five neonates, no improvement in hearing thresholds was observed and cochlear implantation was recommended. Three of the four neonates in whom the hearing improved to normal levels were born prior to 28 weeks gestational age. Hearing improvement was mainly observed prior to a gestational age of 80 weeks. Delayed maturation of an immature auditory pathway might be an important reason for referral after newborn hearing screening in premature infants. Caution is advised regarding early cochlear implantation in preterm born infants. Audiological follow-ups until at least 80 weeks gestational age are therefore recommended. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

High resolution micro-CT scanning as an innovative tool for evaluation of the surgical positioning of cochlear implant electrodes.

PubMed

Postnov, A; Zarowski, A; De Clerck, N; Vanpoucke, F; Offeciers, F E; Van Dyck, D; Peeters, S

2006-05-01

X-ray microtomography (micro-CT) is a new technique allowing for visualization of the internal structure of opaque specimens with a quasi-histological quality. Among multiple potential applications, the use of this technique in otology is very promising. Micro-CT appears to be ideally suited for in vitro visualization of the inner ear tissues as well as for evaluation of the electrode damage and/or surgical insertion trauma during implantation of the cochlear implant electrodes. This technique can greatly aid in design and development of new cochlear implant electrodes and is applicable for temporal bone studies. The main advantage of micro-CT is the practically artefact-free preparation of the samples and the possibility of evaluation of the interesting parameters along the whole insertion depth of the electrode. This paper presents the results of the first application of micro-CT for visualization of the inner ear structures in human temporal bones and for evaluation of the surgical positioning of the cochlear implant electrodes relative to the intracochlear soft tissues.

Optical coherence tomography as a guide for cochlear implant surgery?

NASA Astrophysics Data System (ADS)

Just, T.; Lankenau, E.; Hüttmann, G.; Pau, H. W.

2008-02-01

To assess the potential use of optical coherence tomography (OCT) in cochlear implant surgery, OCT was applied in human temporal bones before cochleostomy. The question was whether OCT might provide information about the cochlear topography, especially about the site of the scala tympani. OCT was carried out on human temporal bone preparations, in which the cochleostomy was performed leaving the membranous labyrinth and the fluid-filled inner ear intact. A specially equipped operating microscope with integrated OCT prototype was used. Spectral-domain (SD)-OCT was used for all investigations. On all scans, OCT supplied information about inner ear structures, such as scala tympani, scala vestibuli while the membranous labyrinth was still intact. In the fresh temporal bone the scala media, basilar membrane and the Reissner's membrane were identified. This OCT study clearly documents the possibility to identify inner ear structures, especially the scala tympani without opening its enveloping membranes. These findings may have an impact on cochlear implant surgery, especially as an orientation guide to localize the scala tympani precisely before opening the fluid filled inner ear.

Cochlear Implantation in Older Adults

PubMed Central

Lin, Frank R.; Chien, Wade W.; Li, Lingsheng; Niparko, John K.; Francis, Howard W.

2012-01-01

Cochlear implants allow individuals with severe-to-profound hearing loss access to sound and spoken language. The number of older adults in the United States who are potential candidates for cochlear implantation is approximately 150,000 and will continue to increase with the aging of the population. Should cochlear implantation (CI) be routinely recommended for these older adults, and do these individuals benefit from CI? We reviewed our 12 year experience with cochlear implantation in adults ≥60 years (n = 445) at Johns Hopkins to investigate the impact of CI on speech understanding and to identify factors associated with speech performance. Complete data on speech outcomes at baseline and 1 year post-CI were available for 83 individuals. Our results demonstrate that cochlear implantation in adults ≥60 years consistently improved speech understanding scores with a mean increase of 60. 0% (S. D. 24. 1) on HINT sentences in quiet . The magnitude of the gain in speech scores was negatively associated with age at implantation such that for every increasing year of age at CI the gain in speech scores was 1. 3 percentage points less (95% CI: 0. 6 – 1. 9) after adjusting for age at hearing loss onset. Conversely, individuals with higher pre-CI speech scores (HINT scores between 40–60%) had significantly greater post-CI speech scores by a mean of 10. 0 percentage points (95% CI: 0. 4 – 19. 6) than those with lower pre-CI speech scores (HINT <40%) after adjusting for age at CI and age at hearing loss onset. These results suggest that older adult CI candidates who are younger at implantation and with higher preoperative speech scores obtain the highest speech understanding scores after cochlear implantation with possible implications for current Medicare policy. Finally, we provide an extended discussion of the epidemiology and impact of hearing loss in older adults. Future research of CI in older adults should expand beyond simple speech outcomes to take into account the broad cognitive, social, and physical functioning outcomes that are likely detrimentally impacted by hearing loss and may be mitigated by cochlear implantation. PMID:22932787

A speech processing study using an acoustic model of a multiple-channel cochlear implant

NASA Astrophysics Data System (ADS)

Xu, Ying

1998-10-01

A cochlear implant is an electronic device designed to provide sound information for adults and children who have bilateral profound hearing loss. The task of representing speech signals as electrical stimuli is central to the design and performance of cochlear implants. Studies have shown that the current speech- processing strategies provide significant benefits to cochlear implant users. However, the evaluation and development of speech-processing strategies have been complicated by hardware limitations and large variability in user performance. To alleviate these problems, an acoustic model of a cochlear implant with the SPEAK strategy is implemented in this study, in which a set of acoustic stimuli whose psychophysical characteristics are as close as possible to those produced by a cochlear implant are presented on normal-hearing subjects. To test the effectiveness and feasibility of this acoustic model, a psychophysical experiment was conducted to match the performance of a normal-hearing listener using model- processed signals to that of a cochlear implant user. Good agreement was found between an implanted patient and an age-matched normal-hearing subject in a dynamic signal discrimination experiment, indicating that this acoustic model is a reasonably good approximation of a cochlear implant with the SPEAK strategy. The acoustic model was then used to examine the potential of the SPEAK strategy in terms of its temporal and frequency encoding of speech. It was hypothesized that better temporal and frequency encoding of speech can be accomplished by higher stimulation rates and a larger number of activated channels. Vowel and consonant recognition tests were conducted on normal-hearing subjects using speech tokens processed by the acoustic model, with different combinations of stimulation rate and number of activated channels. The results showed that vowel recognition was best at 600 pps and 8 activated channels, but further increases in stimulation rate and channel numbers were not beneficial. Manipulations of stimulation rate and number of activated channels did not appreciably affect consonant recognition. These results suggest that overall speech performance may improve by appropriately increasing stimulation rate and number of activated channels. Future revision of this acoustic model is necessary to provide more accurate amplitude representation of speech.

Electroretinogram assessment of children with sensorineural hearing loss: implications for screening.

PubMed

West, Stephanie K; Hindocha, Maya; Hogg, Chris R; Holder, Graham E; Moore, Anthony T; Reddy, M Ashwin

2015-10-01

The guidelines of the National Deaf Children's Society recommend that children with sensorineural hearing loss (SNHL) be routinely screened for ophthalmological problems and suggest electroretinography (ERG) to exclude Usher syndrome. The present study reports the nature and prevalence of abnormal ERG findings in a cohort of children with SNHL undergoing ERG with the aim of identifying risk factors for the diagnosis of Usher syndrome. The medical records of children (<18 years of age) with SNHL referred for ERG at Moorfields Eye Hospital, London, between January 2009 and December 2011 were retrospectively reviewed. Patients were included if they had been referred with SNHL by an audiological medicine consultant and the primary indication for electrodiagnostic testing was possible Usher syndrome. A total of 84 cases met inclusion criteria of which 13 (15%) had ERG findings showing rod-cone dysfunction consistent with a diagnosis of Usher syndrome. Two patients with retinal pigmentary changes had normal ERGs and were diagnosed with rubella retinopathy based on the clinical findings. Risk factor analysis showed that age of ≥8 years at the time of ERG, sex, and bilateral hearing loss were not predictive of a diagnosis of Usher syndrome. However, the presence of or referral for cochlear implants, having relevant symptoms and/or clinical signs consistent with a retinal dystrophy, and profound hearing loss were all highly predictive. ERG is a useful diagnostic tool in children with SNHL and should be performed in children with SNHL who have cochlear implants and/or have signs or symptoms of retinal dystrophy. A focused approach could have potential cost-saving benefit. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

On the stability and compressive nonlinearity of a physiologically based model of the cochlea

DOE Office of Scientific and Technical Information (OSTI.GOV)

Nankali, Amir; Grosh, Karl; Department of Biomedical Engineering, University of Michigan, Ann Arbor, Michigan

Hearing relies on a series of coupled electrical, acoustical (fluidic) and mechanical interactions inside the cochlea that enable sound processing. A positive feedback mechanism within the cochlea, called the cochlear amplifier, provides amplitude and frequency selectivity in the mammalian auditory system. The cochlear amplifier and stability are studied using a nonlinear, micromechanical model of the Organ of Corti (OoC) coupled to the electrical potentials in the cochlear ducts. It is observed that the mechano-electrical transduction (MET) sensitivity and somatic motility of the outer hair cell (OHC), control the cochlear stability. Increasing MET sensitivity beyond a critical value, while electromechanical couplingmore » coefficient is within a specific range, causes instability. We show that instability in this model is generated through a supercritical Hopf bifurcation. A reduced order model of the system is approximated and it is shown that the tectorial membrane (TM) transverse mode effect on the dynamics is significant while the radial mode can be simplified from the equations. The cochlear amplifier in this model exhibits good agreement with the experimental data. A comprehensive 3-dimensional model based on the cross sectional model is simulated and the results are compared. It is indicated that the global model qualitatively inherits some characteristics of the local model, but the longitudinal coupling along the cochlea shifts the stability boundary (i.e., Hopf bifurcation point) and enhances stability.« less

Two-photon microscopy allows imaging and characterization of cochlear microvasculature in vivo.

PubMed

Ihler, Friedrich; Bertlich, Mattis; Weiss, Bernhard; Dietzel, Steffen; Canis, Martin

2015-01-01

Impairment of cochlear blood flow has been discussed as factor in the pathophysiology of various inner ear disorders. However, the microscopic study of cochlear microcirculation is limited due to small scale and anatomical constraints. Here, two-photon fluorescence microscopy is applied to visualize cochlear microvessels. Guinea pigs were injected with Fluorescein isothiocyanate- or Texas red-dextrane as plasma marker. Intravital microscopy was performed in four animals and explanted cochleae from four animals were studied. The vascular architecture of the cochlea was visualized up to a depth of 90.0±22.7 μm. Imaging yielded a mean contrast-to-noise ratio (CNR) of 3.3±1.7. Mean diameter in vivo was 16.5±6.0 μm for arterioles and 8.0±2.4 μm for capillaries. In explanted cochleae, the diameter of radiating arterioles and capillaries was measured with 12.2±1.6 μm and 6.6±1.0 μm, respectively. The difference between capillaries and arterioles was statistically significant in both experimental setups (P<0.001 and P=0.022, two-way ANOVA). Measured vessel diameters in vivo and ex vivo were in agreement with published data. We conclude that two-photon fluorescence microscopy allows the investigation of cochlear microvessels and is potentially a valuable tool for inner ear research.

Effects of sustained release dexamethasone hydrogels in hearing preservation cochlear implantation.

PubMed

Honeder, Clemens; Zhu, Chengjing; Schöpper, Hanna; Gausterer, Julia Clara; Walter, Manuel; Landegger, Lukas David; Saidov, Nodir; Riss, Dominik; Plasenzotti, Roberto; Gabor, Franz; Arnoldner, Christoph

2016-11-01

It has been shown that glucocorticoids reduce the hearing threshold shifts associated with cochlear implantation. Previous studies evaluated the administration of glucocorticoids immediately before surgery or the repeated pre- or perioperative systemic application of glucocorticoids. The aim of this study was to evaluate the effects of a sustained release dexamethasone hydrogel in hearing preservation cochlear implantation. To address this issue, a guinea pig model of cochlear implantation was used. 30 normal hearing pigmented guinea pigs were randomized into a group receiving a single dose of a dexamethasone/poloxamer407 hydrogel one day prior to surgery, a second group receiving the hydrogel seven days prior to surgery and a control group. A silicone cochlear implant electrode designed for the use in guinea pigs was inserted to a depth of 5 mm through a cochleostomy. Compound action potentials of the auditory nerve (frequency range 0.5-32 kHz) were measured preoperatively, directly postoperatively and on postoperative days 3, 7, 14, 21 and 28. Following the last audiometry, temporal bones were harvested and histologically evaluated. Dexamethasone hydrogel application one day prior to surgery resulted in significantly reduced hearing threshold shifts at low, middle and high frequencies measured at postoperative day 28 (p < 0.05). Application of the hydrogel seven days prior to surgery did not show such an effect. Dexamethasone application one day prior to surgery resulted in increased outer hair cell counts in the cochlear apex and in reduced spiral ganglion cell counts in the basal and middle turn of the cochlea, a finding that was associated with a higher rate of electrode translocation in this group. In this study, we were able to demonstrate functional benefits of a single preoperative intratympanic application of a sustained release dexamethasone hydrogel in a guinea pig model of cochlear implantation. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

Thin and open vessel windows for intra-vital fluorescence imaging of murine cochlear blood flow.

PubMed

Shi, Xiaorui; Zhang, Fei; Urdang, Zachary; Dai, Min; Neng, Lingling; Zhang, Jinhui; Chen, Songlin; Ramamoorthy, Sripriya; Nuttall, Alfred L

2014-07-01

Normal microvessel structure and function in the cochlea is essential for maintaining the ionic and metabolic homeostasis required for hearing function. Abnormal cochlear microcirculation has long been considered an etiologic factor in hearing disorders. A better understanding of cochlear blood flow (CoBF) will enable more effective amelioration of hearing disorders that result from aberrant blood flow. However, establishing the direct relationship between CoBF and other cellular events in the lateral wall and response to physio-pathological stress remains a challenge due to the lack of feasible interrogation methods and difficulty in accessing the inner ear. Here we report on new methods for studying the CoBF in a mouse model using a thin or open vessel-window in combination with fluorescence intra-vital microscopy (IVM). An open vessel-window enables investigation of vascular cell biology and blood flow permeability, including pericyte (PC) contractility, bone marrow cell migration, and endothelial barrier leakage, in wild type and fluorescent protein-labeled transgenic mouse models with high spatial and temporal resolution. Alternatively, the thin vessel-window method minimizes disruption of the homeostatic balance in the lateral wall and enables study CoBF under relatively intact physiological conditions. A thin vessel-window method can also be used for time-based studies of physiological and pathological processes. Although the small size of the mouse cochlea makes surgery difficult, the methods are sufficiently developed for studying the structural and functional changes in CoBF under normal and pathological conditions. Copyright © 2014 Elsevier B.V. All rights reserved.

Synaptic Plasticity after Chemical Deafening and Electrical Stimulation of the Auditory Nerve in Cats

PubMed Central

Ryugo, D.K.; Baker, C.A.; Montey, K.L.; Chang, L.Y.; Coco, A.; Fallon, J.B.; Shepherd, R.K.

2010-01-01

The effects of deafness on brain structure and function have been studied using animal models of congenital deafness that include surgical ablation of the organ of Corti, acoustic trauma, ototoxic drugs, and hereditary deafness. This report describes the morphologic plasticity of auditory nerve synapses in response to ototoxic deafening and chronic electrical stimulation of the auditory nerve. Normal kittens were deafened by neonatal administration of neomycin that eliminated auditory receptor cells. Some of these cats were raised deaf, whereas others were chronically implanted with cochlear electrodes at two months of age and electrically stimulated for up to 12 months. The large endings of the auditory nerve, endbulbs of Held, were studied because they hold a key position in the timing pathway for sound localization, are readily identifiable, and exhibit deafness-associated abnormalities. Compared to normal hearing cats, synapses of ototoxically deafened cats displayed expanded postsynaptic densities, a decrease in synaptic vesicle (SV) density, and a reduction in the somatic size of spherical bushy cells (SBCs). When compared to normal hearing cats, endbulbs of ototoxically deafened cats that received cochlear stimulation expressed postsynaptic densities (PSDs) that were statistically identical in size, showed a 32.8% reduction in SV density, and whose target SBCs had a 25.5% reduction in soma area. These results demonstrate that electrical stimulation via a cochlear implant in chemically-deafened cats preserves PSD size but not other aspects of synapse morphology. The results further suggest that the effects of ototoxic deafness are not identical to those of hereditary deafness. PMID:20127807

New Criteria of Indication and Selection of Patients to Cochlear Implant

PubMed Central

Sampaio, André L. L.; Araújo, Mercêdes F. S.; Oliveira, Carlos A. C. P.

2011-01-01

Numerous changes continue to occur in cochlear implant candidacy. In general, these have been accompanied by concomitant and satisfactory changes in surgical techniques. Together, this has advanced the utility and safety of cochlear implantation. Most devices are now approved for use in patients with severe to profound unilateral hearing loss rather then the prior requirement of a bilateral profound loss. Furthermore, studies have begun utilizing short electrode arrays for shallow insertion in patients with considerable low-frequency residual hearing. This technique will allow the recipient to continue to use acoustically amplified hearing for the low frequencies simultaneously with a cochlear implant for the high frequencies. The advances in design of, and indications for, cochlear implants have been matched by improvements in surgical techniques and decrease in complications. The resulting improvements in safety and efficacy have further encouraged the use of these devices. This paper will review the new concepts in the candidacy of cochlear implant. Medline data base was used to search articles dealing with the following topics: cochlear implant in younger children, cochlear implant and hearing preservation, cochlear implant for unilateral deafness and tinnitus, genetic hearing loss and cochlear implant, bilateral cochlear implant, neuropathy and cochlear implant and neural plasticity, and the selection of patients for cochlear implant. PMID:22013448

Perilymph Kinetics of FITC-Dextran Reveals Homeostasis Dominated by the Cochlear Aqueduct and Cerebrospinal Fluid.

PubMed

Salt, A N; Gill, R M; Hartsock, J J

2015-06-01

Understanding how drugs are distributed in perilymph following local applications is important as local drug therapies are increasingly used to treat disorders of the inner ear. The potential contribution of cerebrospinal fluid (CSF) entry to perilymph homeostasis has been controversial for over half a century, largely due to artifactual contamination of collected perilymph samples with CSF. Measures of perilymph flow and of drug distribution following round window niche applications have both suggested a slow, apically directed flow occurs along scala tympani (ST) in the normal, sealed cochlea. In the present study, we have used fluorescein isothiocyanate-dextran as a marker to study perilymph kinetics in guinea pigs. Dextran is lost from perilymph more slowly than other substances so far quantified. Dextran solutions were injected from pipettes sealed into the lateral semicircular canal (SCC), the cochlear apex, or the basal turn of ST. After varying delays, sequential perilymph samples were taken from the cochlear apex or lateral SCC, allowing dextran distribution along the perilymphatic spaces to be quantified. Variability was low and findings were consistent with the injection procedure driving volume flow towards the cochlear aqueduct, and with volume flow during perilymph sampling driven by CSF entry at the aqueduct. The decline of dextran with time in the period between injection and sampling was consistent with both a slow volume influx of CSF (~30 nL/min) entering the basal turn of ST at the cochlear aqueduct and a CSF-perilymph exchange driven by pressure-driven fluid oscillation across the cochlear aqueduct. Sample data also allowed contributions of other processes, such as communications with adjacent compartments, to be quantified. The study demonstrates that drug kinetics in the basal turn of ST is complex and is influenced by a considerable number of interacting processes.

Intracochlear Scala Media Pressure Measurement: Implications for Models of Cochlear Mechanics.

PubMed

Kale, Sushrut S; Olson, Elizabeth S

2015-12-15

Models of the active cochlea build upon the underlying passive mechanics. Passive cochlear mechanics is based on physical and geometrical properties of the cochlea and the fluid-tissue interaction between the cochlear partition and the surrounding fluid. Although the fluid-tissue interaction between the basilar membrane and the fluid in scala tympani (ST) has been explored in both active and passive cochleae, there was no experimental data on the fluid-tissue interaction on the scala media (SM) side of the partition. To this aim, we measured sound-evoked intracochlear pressure in SM close to the partition using micropressure sensors. All the SM pressure data are from passive cochleae, likely because the SM cochleostomy led to loss of endocochlear potential. Thus, these experiments are studies of passive cochlear mechanics. SM pressure close to the tissue showed a pattern of peaks and notches, which could be explained as an interaction between fast and slow (i.e., traveling wave) pressure modes. In several animals SM and ST pressure were measured in the same cochlea. Similar to previous studies, ST-pressure was dominated by a slow, traveling wave mode at stimulus frequencies in the vicinity of the best frequency of the measurement location, and by a fast mode above best frequency. Antisymmetric pressure between SM and ST supported the classic single-partition cochlear models, or a dual-partition model with tight coupling between partitions. From the SM and ST pressure we calculated slow and fast modes, and from active ST pressure we extrapolated the passive findings to the active case. The passive slow mode estimated from SM and ST data was low-pass in nature, as predicted by cochlear models. Copyright © 2015 Biophysical Society. Published by Elsevier Inc. All rights reserved.

Intracochlear Scala Media Pressure Measurement: Implications for Models of Cochlear Mechanics

PubMed Central

Kale, Sushrut S.; Olson, Elizabeth S.

2015-01-01

Models of the active cochlea build upon the underlying passive mechanics. Passive cochlear mechanics is based on physical and geometrical properties of the cochlea and the fluid-tissue interaction between the cochlear partition and the surrounding fluid. Although the fluid-tissue interaction between the basilar membrane and the fluid in scala tympani (ST) has been explored in both active and passive cochleae, there was no experimental data on the fluid-tissue interaction on the scala media (SM) side of the partition. To this aim, we measured sound-evoked intracochlear pressure in SM close to the partition using micropressure sensors. All the SM pressure data are from passive cochleae, likely because the SM cochleostomy led to loss of endocochlear potential. Thus, these experiments are studies of passive cochlear mechanics. SM pressure close to the tissue showed a pattern of peaks and notches, which could be explained as an interaction between fast and slow (i.e., traveling wave) pressure modes. In several animals SM and ST pressure were measured in the same cochlea. Similar to previous studies, ST-pressure was dominated by a slow, traveling wave mode at stimulus frequencies in the vicinity of the best frequency of the measurement location, and by a fast mode above best frequency. Antisymmetric pressure between SM and ST supported the classic single-partition cochlear models, or a dual-partition model with tight coupling between partitions. From the SM and ST pressure we calculated slow and fast modes, and from active ST pressure we extrapolated the passive findings to the active case. The passive slow mode estimated from SM and ST data was low-pass in nature, as predicted by cochlear models. PMID:26682824

Vestibular sensory functional status of cochlear implanted ears versus non-implanted ears in bilateral profound deaf adults.

PubMed

Cozma, Romică Sebastian; Dima-Cozma, Lucia Corina; Rădulescu, Luminiţa Mihaela; Hera, Maria Cristina; Mârţu, Cristian; Olariu, Raluca; Cobzeanu, Bogdan Mihail; Bitere, Oana Roxana; Cobzeanu, Mihail Dan

2018-01-01

Patients with hearing loss who underwent cochlear implantation can present symptomatic or asymptomatic vestibular damages earlier or later after the surgery. The vestibular permanent lesions could be acute, produced by surgical trauma or could be progressive due to local morphological changes made by the presence of the portelectrode in the inner ear (fibrosis related, ossification, basilar membrane distortion, endolymphatic hydrops). Besides histopathological findings in inner ear of cochlear implanted patients, the vestibular permanent damages could be found by assessment of clinical vestibular status. This study reports the sensorial vestibular functional findings for adults in cochlear implanted ears related to the electrode insertion type (cochleostomy or round window approach) and comparing to non-implanted deaf ears. A total of 20 adult patients with 32 cochlear implanted ears (12 patients with binaural cochlear implant and eight with monoaural) were selected for postoperatory vestibular examination by cervical and ocular vestibular myogenic potentials and vestibular caloric tests. The same tests were made for a control group of 22 non-implanted deaf ears. Functional testing results were reported related to the electrode insertion approach. For the cochleostomy group, we found different deficits: in 40% for saccular function, 44% for utricular function, and 12% horizontal canal dysfunction. In round window group, the deficit was present in 14.29% for saccular function, 28.57% for utricular function, and 28.58% for horizontal canal. In 46.88% of implanted ears, the vestibular function was completely preserved on all tested sensors. In conclusion, the vestibular functional status after inner ear surgery presents sensorial damages in 53.12% ears compare with the vestibular dysfunction existing in 50% of deaf non-operated ears. Round window insertion allows for better conservation of the vestibular function.

Pneumococcal meningitis: development of a new animal model

PubMed Central

Wei, Benjamin P.C.; Shepherd, Robert K.; Robins-Browne, Roy M.; Clark, Graeme M.; O’Leary, Stephen J.

2007-01-01

Hypothesis The rat is a suitable animal to establish a model for the study of pneumococcal meningitis post cochlear implantation Background There has been an increase in the number of cases of cochlear implant-related meningitis. The most common organism identified was Streptococcus pneumoniae. Whether cochlear implantation increases the risk of pneumococcal meningitis in healthy subjects without other risk factors remains to be determined. Previous animal studies do not focus on the pathogenesis and risk of pneumococcal meningitis post implantation and are based on relatively small animal numbers, making it difficult to assess the cause and effect relationship. There is, therefore, a need to develop a new animal model allowing direct examination of the pathogenesis of meningitis in the presence of a cochlear implant. Methods Eighteen non-implanted rats were infected with 1× 106 and 1 × 108 colony forming units (CFU) of a clinical isolate of S. pneumoniae via three different inoculation routes (middle ear, inner ear and intraperitoneal) to examine for evidence of meningitis over 24 hours. Six implanted rats were infected with the highest amount of bacteria possible for each route of inoculation (4 × 1010 CFU intraperitoneal, 3 × 108CFU middle ear, 1 × 106 CFU inner ear) to examine for evidence of meningitis with the presence of an implant. Histological pattern of cochlear infections for each of the three different inoculating routes were examined. Results Pneumococcal meningitis was evident in all 6 implanted animals for each of the three different routes of inoculation. Once in the inner ear, bacteria were found to enter the central nervous system either via the cochlear aqueduct or canaliculi perforantes of osseous spiral lamina, reaching the perineural and perivascular space then the internal acoustic meatus. The rate, extent and pattern of infection within the cochleae depended on the route of inoculation. Finally, there was no evidence of pneumococcal meningitis observed in 18 non-implanted rats inoculated at a lower concentration of S. pneumoniae when observed for 24 hours post-inoculation. Conclusion Meningitis in implanted rats following inoculation with a clinical isolate of S. pneumoniae is possible via all three potential routes of infection via the upper respiratory tract. The lack of meningitis observed in the 18 non-implanted rats suggests longer post-inoculation monitoring periods are required to ensure whether or not meningitis will develop. Based on this work we have developed a new animal model that will allow quantitative risk assessment of meningitis post cochlear implantation, and the assessment of the efficacy of potential interventional strategies in future studies. PMID:16936571

4-aminopyridine in scala media reversibly alters the cochlear potentials and suppresses electrically evoked oto-acoustic emissions.

PubMed

Kirk, D L; Yates, G K

1998-01-01

Iontophoresis of 4-aminopyridine into scala media of the guinea pig cochlea caused elevation of the thresholds of the compound action potential of the auditory nerve, loss of amplitude of the extracellular cochlear microphonic response (CM), increase in the endocochlear potential (EP) and reduction in the amplitude of electrically evoked oto-acoustic emissions (EEOAEs). These changes were reversible over 10-20 min. The reciprocity of the changes in the CM and the EP was consistent with an interruption of both DC and AC currents through outer hair cells (OHCs), probably by blockade of mechano-electrical transduction (MET) channels in OHCs. Reductions in EEOAEs were consistent with the extrinsically applied generating current entering the OHC via the MET channels. Implications for the activation of OHC electromotility in vivo are discussed.

Swept-sine noise-induced damage as a hearing loss model for preclinical assays

PubMed Central

Sanz, Lorena; Murillo-Cuesta, Silvia; Cobo, Pedro; Cediel-Algovia, Rafael; Contreras, Julio; Rivera, Teresa; Varela-Nieto, Isabel; Avendaño, Carlos

2015-01-01

Mouse models are key tools for studying cochlear alterations in noise-induced hearing loss (NIHL) and for evaluating new therapies. Stimuli used to induce deafness in mice are usually white and octave band noises that include very low frequencies, considering the large mouse auditory range. We designed different sound stimuli, enriched in frequencies up to 20 kHz (“violet” noises) to examine their impact on hearing thresholds and cochlear cytoarchitecture after short exposure. In addition, we developed a cytocochleogram to quantitatively assess the ensuing structural degeneration and its functional correlation. Finally, we used this mouse model and cochleogram procedure to evaluate the potential therapeutic effect of transforming growth factor β1 (TGF-β1) inhibitors P17 and P144 on NIHL. CBA mice were exposed to violet swept-sine noise (VS) with different frequency ranges (2–20 or 9–13 kHz) and levels (105 or 120 dB SPL) for 30 min. Mice were evaluated by auditory brainstem response (ABR) and otoacoustic emission tests prior to and 2, 14 and 28 days after noise exposure. Cochlear pathology was assessed with gross histology; hair cell number was estimated by a stereological counting method. Our results indicate that functional and morphological changes induced by VS depend on the sound level and frequency composition. Partial hearing recovery followed the exposure to 105 dB SPL, whereas permanent cochlear damage resulted from the exposure to 120 dB SPL. Exposure to 9–13 kHz noise caused an auditory threshold shift (TS) in those frequencies that correlated with hair cell loss in the corresponding areas of the cochlea that were spotted on the cytocochleogram. In summary, we present mouse models of NIHL, which depending on the sound properties of the noise, cause different degrees of cochlear damage, and could therefore be used to study molecules which are potential players in hearing loss protection and repair. PMID:25762930

High-frequency hearing impairment assessed with cochlear microphonics.

PubMed

Zhang, Ming

2012-09-01

Cochlear microphonic (CM) measurements may potentially become a supplementary approach to otoacoustic emission (OAE) measurements for assessing low-frequency cochlear functions in the clinic. The objective of this study was to investigate the measurement of CMs in subjects with high-frequency hearing loss. Currently, CMs can be measured using electrocochleography (ECochG or ECoG) techniques. Both CMs and OAEs are cochlear responses, while auditory brainstem responses (ABRs) are not. However, there are inherent limitations associated with OAE measurements such as acoustic noise, which can conceal low-frequency OAEs measured in the clinic. However, CM measurements may not have these limitations. CMs were measured in human subjects using an ear canal electrode. The CMs were compared between the high-frequency hearing loss group and the normal-hearing control group. Distortion product OAEs (DPOAEs) and audiogram were also measured. The DPOAE and audiogram measurements indicate that the subjects were correctly selected for the two groups. Low-frequency CM waveforms (CMWs) can be measured using ear canal electrodes in high-frequency hearing loss subjects. The difference in amplitudes of CMWs between the high-frequency hearing loss group and the normal-hearing group is insignificant at low frequencies but significant at high frequencies.

Music Engineering as a Novel Strategy for Enhancing Music Enjoyment in the Cochlear Implant Recipient.

PubMed

Kohlberg, Gavriel D; Mancuso, Dean M; Chari, Divya A; Lalwani, Anil K

2015-01-01

Enjoyment of music remains an elusive goal following cochlear implantation. We test the hypothesis that reengineering music to reduce its complexity can enhance the listening experience for the cochlear implant (CI) listener. Normal hearing (NH) adults (N = 16) and CI listeners (N = 9) evaluated a piece of country music on three enjoyment modalities: pleasantness, musicality, and naturalness. Participants listened to the original version along with 20 modified, less complex, versions created by including subsets of the musical instruments from the original song. NH participants listened to the segments both with and without CI simulation processing. Compared to the original song, modified versions containing only 1-3 instruments were less enjoyable to the NH listeners but more enjoyable to the CI listeners and the NH listeners with CI simulation. Excluding vocals and including rhythmic instruments improved enjoyment for NH listeners with CI simulation but made no difference for CI listeners. Reengineering a piece of music to reduce its complexity has the potential to enhance music enjoyment for the cochlear implantee. Thus, in addition to improvements in software and hardware, engineering music specifically for the CI listener may be an alternative means to enhance their listening experience.

Target structures in the cochlea for infrared neural stimulation (INS)

NASA Astrophysics Data System (ADS)

Young, Hunter; Tan, Xiaodong; Richter, Claus-Peter

2014-03-01

Spatial selective infrared neural stimulation has potential to improve neural prostheses, including cochlear implants. The heating of a confined target volume depolarizes the cell membrane and results in an action potential. Tissue heating may also result in the generation of a stress relaxation wave causing mechanical stimulation of hair cells in the cochlea, creating an optoacoustic response. Data are presented that quantify the effect of an acoustical stimulus (noise masker) on the response obtained with INS in normal hearing, and chronic deaf animals. While in normal hearing animals an acoustic masker can reduce the response to INS, in chronic deaf animals this effect has not been detected. The responses to INS remain stable following the different degrees of cochlear damage.

Cochlear Implants.

ERIC Educational Resources Information Center

Clark, Catherine; Scott, Larry

This brochure explains what a cochlear implant is, lists the types of individuals with deafness who may be helped by a cochlear implant, describes the process of evaluating people for cochlear implants, discusses the surgical process for implanting the aid, traces the path of sound through the cochlear implant to the brain, notes the costs of…

Position-dependent patterning of spontaneous action potentials in immature cochlear inner hair cells

PubMed Central

Johnson, Stuart L.; Eckrich, Tobias; Kuhn, Stephanie; Zampini, Valeria; Franz, Christoph; Ranatunga, Kishani M.; Roberts, Terri P.; Masetto, Sergio; Knipper, Marlies; Kros, Corné J.; Marcotti, Walter

2011-01-01

Spontaneous action potential activity is crucial for mammalian sensory system development. In the auditory system, patterned firing activity has been observed in immature spiral ganglion cells and brain-stem neurons and is likely to depend on cochlear inner hair cell (IHC) action potentials. It remains uncertain whether spiking activity is intrinsic to developing IHCs and whether it shows patterning. We found that action potentials are intrinsically generated by immature IHCs of altricial rodents and that apical IHCs exhibit bursting activity as opposed to more sustained firing in basal cells. We show that the efferent neurotransmitter ACh, by fine-tuning the IHC’s resting membrane potential (Vm), is crucial for the bursting pattern in apical cells. Endogenous extracellular ATP also contributes to the Vm of apical and basal IHCs by activating SK2 channels. We hypothesize that the difference in firing pattern along the cochlea instructs the tonotopic differentiation of IHCs and auditory pathway. PMID:21572434

Position-dependent patterning of spontaneous action potentials in immature cochlear inner hair cells.

PubMed

Johnson, Stuart L; Eckrich, Tobias; Kuhn, Stephanie; Zampini, Valeria; Franz, Christoph; Ranatunga, Kishani M; Roberts, Terri P; Masetto, Sergio; Knipper, Marlies; Kros, Corné J; Marcotti, Walter

2011-06-01

Spontaneous action potential activity is crucial for mammalian sensory system development. In the auditory system, patterned firing activity has been observed in immature spiral ganglion and brain-stem neurons and is likely to depend on cochlear inner hair cell (IHC) action potentials. It remains uncertain whether spiking activity is intrinsic to developing IHCs and whether it shows patterning. We found that action potentials were intrinsically generated by immature IHCs of altricial rodents and that apical IHCs showed bursting activity as opposed to more sustained firing in basal cells. We show that the efferent neurotransmitter acetylcholine fine-tunes the IHC's resting membrane potential (V(m)), and as such is crucial for the bursting pattern in apical cells. Endogenous extracellular ATP also contributes to the V(m) of apical and basal IHCs by triggering small-conductance Ca(2+)-activated K(+) (SK2) channels. We propose that the difference in firing pattern along the cochlea instructs the tonotopic differentiation of IHCs and auditory pathway.

Spectral-Temporal Modulated Ripple Discrimination by Children With Cochlear Implants.

PubMed

Landsberger, David M; Padilla, Monica; Martinez, Amy S; Eisenberg, Laurie S

A postlingually implanted adult typically develops hearing with an intact auditory system, followed by periods of deafness (or near deafness) and adaptation to the implant. For an early implanted child whose brain is highly plastic, the auditory system matures with consistent input from a cochlear implant. It is likely that the auditory system of early implanted cochlear implant users is fundamentally different than postlingually implanted adults. The purpose of this study is to compare the basic psychophysical capabilities and limitations of these two populations on a spectral resolution task to determine potential effects of early deprivation and plasticity. Performance on a spectral resolution task (Spectral-temporally Modulated Ripple Test [SMRT]) was measured for 20 bilaterally implanted, prelingually deafened children (between 5 and 13 years of age) and 20 hearing children within the same age range. Additionally, 15 bilaterally implanted, postlingually deafened adults, and 10 hearing adults were tested on the same task. Cochlear implant users (adults and children) were tested bilaterally, and with each ear alone. Hearing listeners (adults and children) were tested with the unprocessed SMRT and with a vocoded version that simulates an 8-channel cochlear implant. For children with normal hearing, a positive correlation was found between age and SMRT score for both the unprocessed and vocoded versions. Older hearing children performed similarly to hearing adults in both the unprocessed and vocoded test conditions. However, for children with cochlear implants, no significant relationship was found between SMRT score and chronological age, age at implantation, or years of implant experience. Performance by children with cochlear implants was poorer than performance by cochlear implanted adults. It was also found that children implanted sequentially tended to have better scores with the first implant compared with the second implant. This difference was not observed for adults. An additional finding was that SMRT score was negatively correlated with age for adults with implants. Results from this study suggest that basic psychophysical capabilities of early implanted children and postlingually implanted adults differ when assessed in the sound field using their personal implant processors. Because spectral resolution does not improve with age for early implanted children, it seems likely that the sparse representation of the signal provided by a cochlear implant limits spectral resolution development. These results are supported by the finding that postlingually implanted adults, whose auditory systems matured before the onset of hearing loss, perform significantly better than early implanted children on the spectral resolution test.

Cytomegalovirus (CMV) Infection Causes Degeneration of Cochlear Vasculature and Hearing Loss in a Mouse Model.

PubMed

Carraro, Mattia; Almishaal, Ali; Hillas, Elaine; Firpo, Matthew; Park, Albert; Harrison, Robert V

2017-04-01

Cytomegalovirus (CMV) infection is one of the most common causes of congenital hearing loss in children. We have used a murine model of CMV infection to reveal functional and structural cochlear pathogenesis. The cerebral cortex of Balb/c mice (Mus musculus) was inoculated with 2000 pfu (plaque forming units) of murine CMV on postnatal day 3. At 6 weeks of age, cochlear function was monitored using auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) measures. Histological assessment of cochlear vasculature using a corrosion cast technique was made at 8 weeks. Vascular casts of mCMV-damaged cochleas, and those of untreated control animals, were examined using scanning electron microscopy. We find very large variations in the degree of vascular damage in animals given identical viral injections (2000 pfu). The primary lesion caused by CMV infection is to the stria vascularis and to the adjacent spiral limbus capillary network. Capillary beds of the spiral ligament are generally less affected. The initial vascular damage is found in the mid-apical turn and appears to progress to more basal cochlear regions. After viral migration to the inner ear, the stria vascularis is the primary affected structure. We suggest that initial auditory threshold losses may relate to the poor development or maintenance of the endocochlear potential caused by strial dysfunction. Our increased understanding of the pathogenesis of CMV-related hearing loss is important for defining methods for early detection and treatment.

Speech Perception in Tones and Noise via Cochlear Implants Reveals Influence of Spectral Resolution on Temporal Processing

PubMed Central

Kreft, Heather A.

2014-01-01

Under normal conditions, human speech is remarkably robust to degradation by noise and other distortions. However, people with hearing loss, including those with cochlear implants, often experience great difficulty in understanding speech in noisy environments. Recent work with normal-hearing listeners has shown that the amplitude fluctuations inherent in noise contribute strongly to the masking of speech. In contrast, this study shows that speech perception via a cochlear implant is unaffected by the inherent temporal fluctuations of noise. This qualitative difference between acoustic and electric auditory perception does not seem to be due to differences in underlying temporal acuity but can instead be explained by the poorer spectral resolution of cochlear implants, relative to the normally functioning ear, which leads to an effective smoothing of the inherent temporal-envelope fluctuations of noise. The outcome suggests an unexpected trade-off between the detrimental effects of poorer spectral resolution and the beneficial effects of a smoother noise temporal envelope. This trade-off provides an explanation for the long-standing puzzle of why strong correlations between speech understanding and spectral resolution have remained elusive. The results also provide a potential explanation for why cochlear-implant users and hearing-impaired listeners exhibit reduced or absent masking release when large and relatively slow temporal fluctuations are introduced in noise maskers. The multitone maskers used here may provide an effective new diagnostic tool for assessing functional hearing loss and reduced spectral resolution. PMID:25315376

Speech Recognition of Bimodal Cochlear Implant Recipients Using a Wireless Audio Streaming Accessory for the Telephone.

PubMed

Wolfe, Jace; Morais, Mila; Schafer, Erin

2016-02-01

The goals of the present investigation were (1) to evaluate recognition of recorded speech presented over a mobile telephone for a group of adult bimodal cochlear implant users, and (2) to measure the potential benefits of wireless hearing assistance technology (HAT) for mobile telephone speech recognition using bimodal stimulation (i.e., a cochlear implant in one ear and a hearing aid on the other ear). A three-by-two-way repeated measures design was used to evaluate mobile telephone sentence-recognition performance differences obtained in quiet and in noise with and without the wireless HAT accessory coupled to the hearing aid alone, CI sound processor alone, and in the bimodal condition. Outpatient cochlear implant clinic. Sixteen bimodal users with Nucleus 24, Freedom, CI512, or CI422 cochlear implants participated in this study. Performance was measured with and without the use of a wireless HAT for the telephone used with the hearing aid alone, CI alone, and bimodal condition. CNC word recognition in quiet and in noise with and without the use of a wireless HAT telephone accessory in the hearing aid alone, CI alone, and bimodal conditions. Results suggested that the bimodal condition gave significantly better speech recognition on the mobile telephone with the wireless HAT. A wireless HAT for the mobile telephone provides bimodal users with significant improvement in word recognition in quiet and in noise over the mobile telephone.

Inner ear injury caused by air intrusion to the scala vestibuli of the cochlea.

PubMed

Kobayashi, T; Sakurada, T; Ohyama, K; Takasaka, M

1993-11-01

In a previous communication, we demonstrated that the introduction of air into the scala tympani of the cochlea causes a decrease of cochlear potentials; however, the change in endocochlear dc potential (EP) was mild and the decreased cochlear microphonics (CM) and compound action potentials (CAP) were, at least partially, reversible. In contrast, we have now found that air perfusion (3-60 microliters/min) in the scala vestibuli decreased cochlear potentials more drastically than that in the scala tympani. The change in the EP after air perfusion in the scala vestibuli was characterized by a decrease of the negative EP in response to anoxia. The CM drastically decreased upon the initiation of air perfusion and no recovery was observed after refilling of the perilymph. Histological examination showed collapse of Reissner's membrane in 12 out of 17 cochleas examined. The extent and frequency of the collapse increased with an increase in the amount of air perfused in the scala vestibuli. As the minimal amount of air needed to cause inner ear damage by air perfusion in the scala vestibuli is as small as 3 microliters, it is possible that the prognosis is worse in cases with fistula of the oval window compared to that of the round window area, if the pneumolabyrinth is involved in the pathophysiology of perilymphatic fistula. It is also indicated that air inflation of the middle ear is dangerous in cases with fistula in the oval window.

What Does Music Sound Like for a Cochlear Implant User?

PubMed

Jiam, Nicole T; Caldwell, Meredith T; Limb, Charles J

2017-09-01

Cochlear implant research and product development over the past 40 years have been heavily focused on speech comprehension with little emphasis on music listening and enjoyment. The relatively little understanding of how music sounds in a cochlear implant user stands in stark contrast to the overall degree of importance the public places on music and quality of life. The purpose of this article is to describe what music sounds like to cochlear implant users, using a combination of existing research studies and listener descriptions. We examined the published literature on music perception in cochlear implant users, particularly postlingual cochlear implant users, with an emphasis on the primary elements of music and recorded music. Additionally, we administered an informal survey to cochlear implant users to gather first-hand descriptions of music listening experience and satisfaction from the cochlear implant population. Limitations in cochlear implant technology lead to a music listening experience that is significantly distorted compared with that of normal hearing listeners. On the basis of many studies and sources, we describe how music is frequently perceived as out-of-tune, dissonant, indistinct, emotionless, and weak in bass frequencies, especially for postlingual cochlear implant users-which may in part explain why music enjoyment and participation levels are lower after implantation. Additionally, cochlear implant users report difficulty in specific musical contexts based on factors including but not limited to genre, presence of lyrics, timbres (woodwinds, brass, instrument families), and complexity of the perceived music. Future research and cochlear implant development should target these areas as parameters for improvement in cochlear implant-mediated music perception.

Phantom auditory perception (tinnitus): mechanisms of generation and perception.

PubMed

Jastreboff, P J

1990-08-01

Phantom auditory perception--tinnitus--is a symptom of many pathologies. Although there are a number of theories postulating certain mechanisms of its generation, none have been proven yet. This paper analyses the phenomenon of tinnitus from the point of view of general neurophysiology. Existing theories and their extrapolation are presented, together with some new potential mechanisms of tinnitus generation, encompassing the involvement of calcium and calcium channels in cochlear function, with implications for malfunction and aging of the auditory and vestibular systems. It is hypothesized that most tinnitus results from the perception of abnormal activity, defined as activity which cannot be induced by any combination of external sounds. Moreover, it is hypothesized that signal recognition and classification circuits, working on holographic or neuronal network-like representation, are involved in the perception of tinnitus and are subject to plastic modification. Furthermore, it is proposed that all levels of the nervous system, to varying degrees, are involved in tinnitus manifestation. These concepts are used to unravel the inexplicable, unique features of tinnitus and its masking. Some clinical implications of these theories are suggested.

Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities.

PubMed

Hennersdorf, Florian; Friese, Natascha; Löwenheim, Hubert; Tropitzsch, Anke; Ernemann, Ulrike; Bisdas, Sotirios

2014-06-01

Goldenhar syndrome is a developmental disorder presenting with orofacial and vertebral anomalies, which are also accompanied by abnormalities in other organs. We examined temporal bone changes with special emphasis on inner ear abnormalities in these patients. A retrospective review of 7 new cases in addition to a previously published series of 14 cases with clinically diagnosed Goldenhar syndrome was carried out to search for inner ear anomalies. In addition, temporal bone imaging studies from the literature were summarized and compared with our results. Departments of Neuroradiology and Otorhinolaryngology at a university hospital. In addition to the previous series of 14 patients, 7 new patients with Goldenhar syndrome were identified. Patients underwent otologic examination, audiometric studies, and high-resolution computed tomography (CT) or magnetic resonance imaging (MRI) of the temporal bone. Temporal bone changes and specifically inner ear malformations. Nineteen of 21 patients showed changes of the external and middle ear correlating with the literature. Seven of 21 patients showed inner ear abnormalities constituting one-third of all patients. These ranged from mild such as vestibular enlargement to severe defects such as cochlear hypoplasia and common cavity. Inner ear abnormalities were present in one-third of patients. Although in some cases, these might not be of clinical significance, some patients show severe defects of the inner ear requiring more complex hearing loss therapy. Therefore, imaging of the temporal bone structures is important in the care of these patients.

[SOX10 mutation is relevant to inner ear malformation in patients with Waardenburg syndrome].

PubMed

Xu, G Y; Hao, Q Q; Zhong, L L; Ren, W; Yan, Y; Liu, R Y; Li, J N; Guo, W W; Zhao, H; Yang, S M

2016-11-07

Objective: To determine the relevance between the SOX 10 mutation and Waardenburg syndrome (WS) accompanied with inner ear abnormality by analyzing the inner ear imaging results and molecular and genetic results of the WS patients with the SOX 10 mutation. Methods: This study included 36 WS in patients during 2001 and 2015 in the department of otorhinolaryngology head and neck surgery, Chinese Peoples's Liberation Army General Hospital. The condition of the inner ear of each patient was assessed by analyzing HRCT scans of the temporal bone and MRI scans of the brain and internal auditory canal. Meanwhile, the possible pathogenic genes of WS, including SOX10, MITF , and PAX 3, were also screened. Patients were divided into two groups according to SOX 10 mutation.The Fisher accuracy test was used to determine statistical difference of inner ear deformation incidence between the two groups. Results: Among all 36 patients, 12 were found to have inner ear abnormality. Most abnormalities were posterior semicircular canal deformations, some accompanied with cochlear deformation and an enlarged vestibule. Among all patients, 9 patients were SOX 10 heterozygous mutation carriers, among which six showed bilateral inner ear abnormality. Fisher accuracy test results suggested a significant correlation between the SOX 10 mutation and inner ear abnormality in WS patients ( P =0.036). Conclusion: This study found that WS patients with the SOX 10 mutation are more likely to have deformed inner ears when compared to WS patients without the SOX 10 mutation.

Response to a pure tone in a nonlinear mechanical-electrical-acoustical model of the cochlea.

PubMed

Meaud, Julien; Grosh, Karl

2012-03-21

In this article, a nonlinear mathematical model is developed based on the physiology of the cochlea of the guinea pig. The three-dimensional intracochlear fluid dynamics are coupled to a micromechanical model of the organ of Corti and to electrical potentials in the cochlear ducts and outer hair cells (OHC). OHC somatic electromotility is modeled by linearized piezoelectric relations whereas the OHC hair-bundle mechanoelectrical transduction current is modeled as a nonlinear function of the hair-bundle deflection. The steady-state response of the cochlea to a single tone is simulated in the frequency domain using an alternating frequency time scheme. Compressive nonlinearity, harmonic distortion, and DC shift on the basilar membrane (BM), tectorial membrane (TM), and OHC potentials are predicted using a single set of parameters. The predictions of the model are verified by comparing simulations to available in vivo experimental data for basal cochlear mechanics. In particular, the model predicts more amplification on the reticular lamina (RL) side of the cochlear partition than on the BM, which replicates recent measurements. Moreover, small harmonic distortion and DC shifts are predicted on the BM, whereas more significant harmonic distortion and DC shifts are predicted in the RL and TM displacements and in the OHC potentials. Copyright © 2012 Biophysical Society. Published by Elsevier Inc. All rights reserved.

Effect of cochlear nerve electrocautery on the adult cochlear nucleus.

PubMed

Iseli, Claire E; Merwin, William H; Klatt-Cromwell, Cristine; Hutson, Kendall A; Ewend, Matthew G; Adunka, Oliver F; Fitzpatrick, Douglas C; Buchman, Craig A

2015-04-01

Electrocauterization and subsequent transection of the cochlear nerve induce greater injury to the cochlear nucleus than sharp transection alone. Some studies show that neurofibromatosis Type 2 (NF2) patients fit with auditory brainstem implants (ABIs) fail to achieve speech perception abilities similar to ABI recipients without NF2. Reasons for these differences remain speculative. One hypothesis posits poorer performance to surgically induced trauma to the cochlear nucleus from electrocautery. Sustained electrosurgical depolarization of the cochlear nerve may cause excitotoxic-induced postsynaptic nuclear injury. Equally plausible is that cautery in the vicinity of the cochlear nucleus induces necrosis. The cochlear nerve was transected in anesthetized adult gerbils sharply with or without bipolar electrocautery at varying intensities. Gerbils were perfused at 1, 3, 5, and 7 days postoperatively; their brainstem and cochleas were embedded in paraffin and sectioned at 10 μm. Alternate sections were stained with flourescent markers for neuronal injury or Nissl substance. In additional experiments, anterograde tracers were applied directly to a sectioned eighth nerve to verify that fluorescent-labeled profiles seen were terminating auditory nerve fibers. Cochlear nerve injury was observed from 72 hours postoperatively and was identical across cases regardless of surgical technique. Postsynaptic cochlear nucleus injury was not seen after distal transection of the nerve. By contrast, proximal transection was associated with trauma to the cochlear nucleus. Distal application of bipolar electrocautery seems safe for the cochlear nucleus. Application near the root entry zone must be used cautiously because this may compromise nuclear viability needed to support ABI stimulation.

Lead exposure results in hearing loss and disruption of the cochlear blood-labyrinth barrier and the protective role of iron supplement.

PubMed

Liu, Xinqin; Zheng, Gang; Wu, Yongxiang; Shen, Xuefeng; Jing, Jinfei; Yu, Tao; Song, Han; Chen, Jingyuan; Luo, Wenjing

2013-12-01

This study was designed to investigate the impact of lead (Pb(2+)) on the auditory system and its molecular mechanisms. Pb(AC)2 was administrated to male SD rats aged 21-22 d for 8 weeks at a dose of 300ppm. Male guinea pigs were also administrated with 50mg/kg Pb(AC)2 two times a week for 8 weeks. The auditory nerve-brainstem evoked responses (ABR) was recorded and the morphological changes of the outer hair cells (OHCs) were observed with Phallodin-FITC staining. In addition, the integrity of the blood-labyrinth barrier was observed by TEM and the expression of tight junction proteins (TJPs) in the cochlear stria vascularis was determined by immunofluorescence. Our results showed that Pb(2+) exposure resulted in increased ABR threshold in both rats and guinea pigs. Abnormal shapes and loss of OHCs were found in the cochlear basilar membrane following the Pb(2+) exposure. TEM study showed that the tight junctions between the endothelial cells and the border cells were lost and disrupted. Down-regulation of the occludin, ZO-1 and claudin-5 in the stria vascularis suggested that the increased permeability of the blood-labyrinth barrier may attribute to the Pb(2+)-induced decrease of TJPs' expression. Additionally, Fe(2+) supplement partly reversed the Pb(2+)-induced hearing loss and down-regulation of TJPs. Taken together, these data indicate that the disruption of blood-labyrinth barrier by down-regulating the expression of TJPs plays a role in the Pb(2+)-induced hearing loss, and Fe(2+) supplement protects the auditory system against Pb(2+)-induced toxicity and may have significant clinical implications. Copyright © 2013 Elsevier Inc. All rights reserved.

Music Perception of Cochlear Implant Recipients with Implications for Music Instruction: A Review of Literature

PubMed Central

Hsiao, Feilin; Gfeller, Kate

2013-01-01

This review of literature presents a systematic analysis of the capabilities and limitations of cochlear implant recipients regarding music perception. Specifically, it a) analyzes individual components of music (e.g., rhythm, timbre, and pitch) as they interface with the technical characteristics of cochlear implants and the perceptual abilities of cochlear implant recipients; and b) describes accommodations for music instruction that support successful participation of children with cochlear implants. This article consolidates research studies from various disciplines (audiology, hearing science, speech-language pathology, cochlear implants, and music therapy) to provide practical recommendations for educators in fostering the musical growth of children with cochlear implants. PMID:23469365

Outcomes of cochlear implantation in deaf children of deaf parents: comparative study.

PubMed

Hassanzadeh, S

2012-10-01

This retrospective study compared the cochlear implantation outcomes of first- and second-generation deaf children. The study group consisted of seven deaf, cochlear-implanted children with deaf parents. An equal number of deaf children with normal-hearing parents were selected by matched sampling as a reference group. Participants were matched based on onset and severity of deafness, duration of deafness, age at cochlear implantation, duration of cochlear implantation, gender, and cochlear implant model. We used the Persian Auditory Perception Test for the Hearing Impaired, the Speech Intelligibility Rating scale, and the Sentence Imitation Test, in order to measure participants' speech perception, speech production and language development, respectively. Both groups of children showed auditory and speech development. However, the second-generation deaf children (i.e. deaf children of deaf parents) exceeded the cochlear implantation performance of the deaf children with hearing parents. This study confirms that second-generation deaf children exceed deaf children of hearing parents in terms of cochlear implantation performance. Encouraging deaf children to communicate in sign language from a very early age, before cochlear implantation, appears to improve their ability to learn spoken language after cochlear implantation.

Cochlear implant revision surgeries in children.

PubMed

Amaral, Maria Stella Arantes do; Reis, Ana Cláudia Mirândola B; Massuda, Eduardo T; Hyppolito, Miguel Angelo

2018-02-16

The surgery during which the cochlear implant internal device is implanted is not entirely free of risks and may produce problems that will require revision surgeries. To verify the indications for cochlear implantation revision surgery for the cochlear implant internal device, its effectiveness and its correlation with certain variables related to language and hearing. A retrospective study of patients under 18 years submitted to cochlear implant Surgery from 2004 to 2015 in a public hospital in Brazil. Data collected were: age at the time of implantation, gender, etiology of the hearing loss, audiological and oral language characteristics of each patient before and after Cochlear Implant surgery and any need for surgical revision and the reason for it. Two hundred and sixty-five surgeries were performed in 236 patients. Eight patients received a bilateral cochlear implant and 10 patients required revision surgery. Thirty-two surgeries were necessary for these 10 children (1 bilateral cochlear implant), of which 21 were revision surgeries. In 2 children, cochlear implant removal was necessary, without reimplantation, one with cochlear malformation due to incomplete partition type I and another due to trauma. With respect to the cause for revision surgery, of the 8 children who were successfully reimplanted, four had cochlear calcification following meningitis, one followed trauma, one exhibited a facial nerve malformation, one experienced a failure of the cochlear implant internal device and one revision surgery was necessary because the electrode was twisted. The incidence of the cochlear implant revision surgery was 4.23%. The period following the revision surgeries revealed an improvement in the subject's hearing and language performance, indicating that these surgeries are valid in most cases. Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Parental expectations and outcomes of pediatric cochlear implantation.

PubMed

Piazza, Elizabeth; Kandathil, Cherian; Carron, Jeffrey D

2009-10-01

Cochlear implants have been used with increasing frequency over the past twenty years, including very young patients. To determine if parents are satisfied with their children's performance after cochlear implantation. Survey mailed to parents of children receiving cochlear implants. 31 questionnaires were returned out of 69 mailed (45 %). The vast majority of responding parents felt that their children benefited substantially from cochlear implant surgery. Cochlear implantation is effective in helping children develop auditory-oral communication skills. Access to auditory/oral communication programs in this state remains an obstacle in postoperative habilitation.

Spatial channel interactions in cochlear implants

NASA Astrophysics Data System (ADS)

Tang, Qing; Benítez, Raul; Zeng, Fan-Gang

2011-08-01

The modern multi-channel cochlear implant is widely considered to be the most successful neural prosthesis owing to its ability to restore partial hearing to post-lingually deafened adults and to allow essentially normal language development in pre-lingually deafened children. However, the implant performance varies greatly in individuals and is still limited in background noise, tonal language understanding, and music perception. One main cause for the individual variability and the limited performance in cochlear implants is spatial channel interaction from the stimulating electrodes to the auditory nerve and brain. Here we systematically examined spatial channel interactions at the physical, physiological, and perceptual levels in the same five modern cochlear implant subjects. The physical interaction was examined using an electric field imaging technique, which measured the voltage distribution as a function of the electrode position in the cochlea in response to the stimulation of a single electrode. The physiological interaction was examined by recording electrically evoked compound action potentials as a function of the electrode position in response to the stimulation of the same single electrode position. The perceptual interactions were characterized by changes in detection threshold as well as loudness summation in response to in-phase or out-of-phase dual-electrode stimulation. To minimize potentially confounding effects of temporal factors on spatial channel interactions, stimulus rates were limited to 100 Hz or less in all measurements. Several quantitative channel interaction indexes were developed to define and compare the width, slope and symmetry of the spatial excitation patterns derived from these physical, physiological and perceptual measures. The electric field imaging data revealed a broad but uniformly asymmetrical intracochlear electric field pattern, with the apical side producing a wider half-width and shallower slope than the basal side. In contrast, the evoked compound action potential and perceptual channel interaction data showed much greater individual variability. It is likely that actual reduction in neural and higher level interactions, instead of simple sharpening of the electric current field, would be the key to predicting and hopefully improving the variable cochlear implant performance. The present results are obtained with auditory prostheses but can be applied to other neural prostheses, in which independent spatial channels, rather than a high stimulation rate, are critical to their performance.

Clinical application of neurotrophic factors: the potential for primary auditory neuron protection

PubMed Central

Gillespie, Lisa N.; Shepherd, Robert K.

2007-01-01

Sensorineural hearing loss, as a result of damage to or destruction of the sensory epithelia within the cochlea, is a common cause of deafness. The subsequent degeneration of the neural elements within the inner ear may impinge upon the efficacy of the cochlear implant. Experimental studies have demonstrated that neurotrophic factors can prevent this degeneration in animal models of deafness, and can even provide functional benefits. Neurotrophic factor therapy may, therefore, provide similar protective effects in humans, resulting in improved speech perception outcomes among cochlear implant patients. There are, however, numerous issues pertaining to delivery techniques and treatment regimes which need to be addressed prior to any clinical application. This review considers these issues in view of the potential therapeutic application of neurotrophic factors within the auditory system. PMID:16262651

Bidirectional synaptic plasticity in the cerebellum-like mammalian dorsal cochlear nucleus

NASA Astrophysics Data System (ADS)

Fujino, Kiyohiro; Oertel, Donata

2003-01-01

The dorsal cochlear nucleus integrates acoustic with multimodal sensory inputs from widespread areas of the brain. Multimodal inputs are brought to spiny dendrites of fusiform and cartwheel cells in the molecular layer by parallel fibers through synapses that are subject to long-term potentiation and long-term depression. Acoustic cues are brought to smooth dendrites of fusiform cells in the deep layer by auditory nerve fibers through synapses that do not show plasticity. Plasticity requires Ca2+-induced Ca2+ release; its sensitivity to antagonists of N-methyl-D-aspartate and metabotropic glutamate receptors differs in fusiform and cartwheel cells.

Drug delivery into the cochlear apex: Improved control to sequentially affect finely spaced regions along the entire length of the cochlear spiral.

PubMed

Lichtenhan, J T; Hartsock, J; Dornhoffer, J R; Donovan, K M; Salt, A N

2016-11-01

Administering pharmaceuticals to the scala tympani of the inner ear is a common approach to study cochlear physiology and mechanics. We present here a novel method for in vivo drug delivery in a controlled manner to sealed ears. Injections of ototoxic solutions were applied from a pipette sealed into a fenestra in the cochlear apex, progressively driving solutions along the length of scala tympani toward the cochlear aqueduct at the base. Drugs can be delivered rapidly or slowly. In this report we focus on slow delivery in which the injection rate is automatically adjusted to account for varying cross sectional area of the scala tympani, therefore driving a solution front at uniform rate. Objective measurements originating from finely spaced, low- to high-characteristic cochlear frequency places were sequentially affected. Comparison with existing methods(s): Controlled administration of pharmaceuticals into the cochlear apex overcomes a number of serious limitations of previously established methods such as cochlear perfusions with an injection pipette in the cochlear base: The drug concentration achieved is more precisely controlled, drug concentrations remain in scala tympani and are not rapidly washed out by cerebrospinal fluid flow, and the entire length of the cochlear spiral can be treated quickly or slowly with time. Controlled administration of solutions into the cochlear apex can be a powerful approach to sequentially effect objective measurements originating from finely spaced cochlear regions and allows, for the first time, the spatial origin of CAPs to be objectively defined. Copyright © 2016 Elsevier B.V. All rights reserved.

Drug delivery into the cochlear apex: Improved control to sequentially affect finely spaced regions along the entire length of the cochlear spiral

PubMed Central

Lichtenhan, JT; Hartsock, J; Dornhoffer, JR; Donovan, KM; Salt, AN

2016-01-01

Background Administering pharmaceuticals to the scala tympani of the inner ear is a common approach to study cochlear physiology and mechanics. We present here a novel method for in vivo drug delivery in a controlled manner to sealed ears. New method Injections of ototoxic solutions were applied from a pipette sealed into a fenestra in the cochlear apex, progressively driving solutions along the length of scala tympani toward the cochlear aqueduct at the base. Drugs can be delivered rapidly or slowly. In this report we focus on slow delivery in which the injection rate is automatically adjusted to account for varying cross sectional area of the scala tympani, therefore driving a solution front at uniform rate. Results Objective measurements originating from finely spaced, low- to high-characteristic cochlear frequency places were sequentially affected. Comparison with existing methods(s): Controlled administration of pharmaceuticals into the cochlear apex overcomes a number of serious limitations of previously established methods such as cochlear perfusions with an injection pipette in the cochlear base: The drug concentration achieved is more precisely controlled, drug concentrations remain in scala tympani and are not rapidly washed out by cerebrospinal fluid flow, and the entire length of the cochlear spiral can be treated quickly or slowly with time. Conclusions Controlled administration of solutions into the cochlear apex can be a powerful approach to sequentially effect objective measurements originating from finely spaced cochlear regions and allows, for the first time, the spatial origin of CAPs to be objectively defined. PMID:27506463

Conditional Deletion of N-Myc Disrupts Neurosensory and Non-sensory Development of the Ear

PubMed Central

Kopecky, Benjamin; Santi, Peter; Johnson, Shane; Schmitz, Heather; Fritzsch, Bernd

2011-01-01

Ear development requires interactions of transcription factors for proliferation and differentiation. The proto-oncogene N-Myc is a member of the Myc family that regulate proliferation. To investigate the function of N-Myc, we conditionally knocked out N-Myc in the ear using Tg(Pax2-Cre) and Foxg 1KiCre. N-Myc CKOs had reduced growth of the ear, abnormal morphology including fused sensory epithelia, disrupted histology, and disorganized neuronal innervation. Using Thin-Sheet Laser Imaging Microscopy (TSLIM), 3D reconstruction and quantification of the cochlea revealed a greater than fifty percent size reduction. Immunochemistry and in situ hybridization showed a gravistatic organ-cochlear fusion and a “circularized” apex with no clear inner and outer hair cells. Furthermore, the abnormally developed cochlea had cross innervation from the vestibular ganglion near the basal tip. These findings are put in the context of the possible functional relationship of N-Myc with a number of other cell proliferative and fate determining genes during ear development. PMID:21448975

Serotonin projection patterns to the cochlear nucleus.

PubMed

Thompson, A M; Thompson, G C

2001-07-13

The cochlear nucleus is well known as an obligatory relay center for primary auditory nerve fibers. Perhaps not so well known is the neural input to the cochlear nucleus from cells containing serotonin that reside near the midline in the midbrain raphe region. Although the specific locations of the main, if not sole, sources of serotonin within the dorsal cochlear nucleus subdivision are known to be the dorsal and median raphe nuclei, sources of serotonin located within other cochlear nucleus subdivisions are not currently known. Anterograde tract tracing was used to label fibers originating from the dorsal and median raphe nuclei while fluorescence immunohistochemistry was used to simultaneously label specific serotonin fibers in cat. Biotinylated dextran amine was injected into the dorsal and median raphe nuclei and was visualized with Texas Red, while serotonin was visualized with fluorescein. Thus, double-labeled fibers were unequivocally identified as serotoninergic and originating from one of the labeled neurons within the dorsal and median raphe nuclei. Double-labeled fiber segments, typically of fine caliber with oval varicosities, were observed in many areas of the cochlear nucleus. They were found in the molecular layer of the dorsal cochlear nucleus, in the small cell cap region, and in the granule cell and external regions of the cochlear nuclei, bilaterally, of all cats. However, the density of these double-labeled fiber segments varied considerably depending upon the exact region in which they were found. Fiber segments were most dense in the dorsal cochlear nucleus (especially in the molecular layer) and the large spherical cell area of the anteroventral cochlear nucleus; they were moderately dense in the small cell cap region; and fiber segments were least dense in the octopus and multipolar cell regions of the posteroventral cochlear nucleus. Because of the presence of labeled fiber segments in subdivisions of the cochlear nucleus other than the dorsal cochlear nucleus, we concluded that the serotoninergic projection pattern to the cochlear nucleus is divergent and non-specific. Double-labeled fiber segments were also present, but sparse, in the superior olive, localized mainly in periolivary regions; this indicated that the divergence of dorsal and median raphe neurons that extends throughout regions of the cochlear nucleus also extended well beyond the cochlear nucleus to include at least the superior olivary complex as well.

Experience in cochlear reimplantation. Descriptive study of a 20-year period.

PubMed

Gutiérrez-Salazar, Andrés; Cop, Constanze; Osorio-Acosta, Ángel; Borkoski-Barreiro, Silvia; Falcón-González, Juan C; Ramos-Macías, Ángel

2015-01-01

The cochlear implant is a surgical procedure that has increased substantially, because the paediatric population is diagnosed and implanted early and because there are increased potential indications. This device has the inherent risk of failure in performance, as dies any active medical device, which is the most common cause of implant removal. Our goal was to understand what the causes that produced removal in our series were, and confirm if these conformed to reality as reviewed in the literature. This was a retrospective, descriptive, observational study of 859 cochlear implant surgeries carried out between October 1991 and May 2011. The causes of implant removal were classified according to the European Consensus Statement on Cochlear Implant Failures and Explantations. The reimplantation rate was 6.16% (n=51). The most common reason for removal was technical device failure (45.5%), followed by infection/rejection (23.6%) and upgrade (12.7%). Less common causes: there were 3 cases (5.6%) of electrode misplacement, 2 cases (3.6%) of labyrinthine ossification, 2 (3.6%) as a result of head trauma, 2 (3.6%) from need for nuclear magnetic resonance imaging and 1 case (1.8%) from psychiatric illness. Cochlear reimplantation is a safe procedure, with a low complication rate. In our centre, it reaches an overall rate of 6.16%. Technical device failure remains the most common cause of this procedure, although there is a significant percentage of reimplantation for device update. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

CO-LOCALIZATION OF THE VANILLOID CAPSAICIN RECEPTOR AND SUBSTANCE P IN SENSORY NERVE FIBERS INNERVATING COCHLEAR AND VERTEBRO-BASILAR ARTERIES

PubMed Central

VASS, Z.; DAI, C. F.; STEYGER, P. S.; JANCSÓ, G.; TRUNE, D. R.; NUTTALL, A. L.

2014-01-01

Evidence suggests that capsaicin-sensitive substance P (SP)-containing trigeminal ganglion neurons innervate the spiral modiolar artery (SMA), radiating arterioles, and the stria vascularis of the cochlea. Antidromic electrical or chemical stimulation of trigeminal sensory nerves results in neurogenic plasma extravasation in inner ear tissues. The primary aim of this study was to reveal the possible morphological basis of cochlear vascular changes mediated by capsaicin-sensitive sensory nerves. Therefore, the distribution of SP and capsaicin receptor (transient receptor potential vanilloid type 1—TRPV1) was investigated by double immunolabeling to demonstrate the anatomical relationships between the cochlear and vertebro-basilar blood vessels and the trigeminal sensory fiber system. Extensive TRPV1 and SP expression and co-localization were observed in axons within the adventitial layer of the basilar artery, the anterior inferior cerebellar artery, the SMA, and the radiating arterioles of the cochlea. There appears to be a functional relationship between the trigeminal ganglion and the cochlear blood vessels since electrical stimulation of the trigeminal ganglion induced significant plasma extravasation from the SMA and the radiating arterioles. The findings suggest that stimulation of paravascular afferent nerves may result in permeability changes in the basilar and cochlear vascular bed and may contribute to the mechanisms of vertebro-basilar type of headache through the release of SP and stimulation of TPVR1, respectively. We propose that vertigo, tinnitus, and hearing deficits associated with migraine may arise from perturbations of capsaicin-sensitive trigeminal sensory ganglion neurons projecting to the cochlea. PMID:15026132

Auditory Cortical Maturation in a Child with Cochlear Implant: Analysis of Electrophysiological and Behavioral Measures

PubMed Central

Silva, Liliane Aparecida Fagundes; Couto, Maria Inês Vieira; Tsuji, Robinson Koji; Bento, Ricardo Ferreira; de Carvalho, Ana Claudia Martinho; Matas, Carla Gentile

2015-01-01

The purpose of this study was to longitudinally assess the behavioral and electrophysiological hearing changes of a girl inserted in a CI program, who had bilateral profound sensorineural hearing loss and underwent surgery of cochlear implantation with electrode activation at 21 months of age. She was evaluated using the P1 component of Long Latency Auditory Evoked Potential (LLAEP); speech perception tests of the Glendonald Auditory Screening Procedure (GASP); Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS); and Meaningful Use of Speech Scales (MUSS). The study was conducted prior to activation and after three, nine, and 18 months of cochlear implant activation. The results of the LLAEP were compared with data from a hearing child matched by gender and chronological age. The results of the LLAEP of the child with cochlear implant showed gradual decrease in latency of the P1 component after auditory stimulation (172 ms–134 ms). In the GASP, IT-MAIS, and MUSS, gradual development of listening skills and oral language was observed. The values of the LLAEP of the hearing child were expected for chronological age (132 ms–128 ms). The use of different clinical instruments allow a better understanding of the auditory habilitation and rehabilitation process via CI. PMID:26881163

The effect of BAPTA and 4AP in scala media on transduction and cochlear gain.

PubMed

Sellick, P M; Robertson, D; Patuzzi, R

2006-01-01

We have injected by iontophoresis 4-amino-pyridine, a K+ channel blocker and BAPTA, (a Ca++ chelator), into scala media of the first three turns of the guinea pig cochlea. We measured the reduction in outer hair cell (OHC) receptor current, as indicated by cochlear microphonic measured in scala media evoked by a 207 Hz tone, and compared this with the elevation of the cochlear action potential (CAP) threshold. We found that in the basal turn, for frequencies between 12 and 21 kHz, CAP threshold was elevated by about 30 dB, while in the second turn, at the 3 kHz place, the maximum elevation was 15 dB. In the third turn, iontophoresis of 4AP and BAPTA reduced CM by similar amounts to that in the basal and second turn, but caused negligible elevation of CAP threshold. We conclude that the gain of the cochlear amplifier is maximal for basal turn frequencies, is halved at 3 kHz, and is reduced to close to one for frequencies below 1 kHz (no active gain). The effect of 4AP and BAPTA on neural threshold and the receptor current represented by CM may be explained by their action on OHC transduction without the involvement of IHCs.

A surgical approach appropriate for targeted cochlear gene therapy in the mouse.

PubMed

Jero, J; Tseng, C J; Mhatre, A N; Lalwani, A K

2001-01-01

Therapeutic manipulations of the mammalian cochlea, including cochlear gene transfer, have been predominantly studied using the guinea pig as the experimental model. With the significant developments in mouse genomics and the availability of mutant strains of mice with well-characterized hearing loss, the mouse justifiably will be the preferred animal model for therapeutic manipulations. However, the potential advantages of the mouse model have not been fully realized due to the surgical difficulty of accessing its small cochlea. This study describes a ventral approach, instead of the routinely used postauricular approach in other rodents, for accessing the mouse middle and inner ear, and its application in cochlear gene transfer. This ventral approach enabled rapid and direct delivery of liposome-transgene complex to the mouse inner ear while avoiding blood loss, facial nerve morbidity, and mortality. Transgene expression at 3 days was detected in Reissner's membrane, spiral limbus, spiral ligament, and spiral ganglion cells, in a pattern similar to that previously described in the guinea pig. The successful access and delivery of material to the mouse cochlea and the replication of gene expression seen in the guinea pig demonstrated in this study should promote the use of the mouse in future studies investigating targeted cochlear therapy.

Development of micro-electromechanical system (MEMS) cochlear biomodel

NASA Astrophysics Data System (ADS)

Ngelayang, Thailis Bounya Anak; Latif, Rhonira

2015-05-01

Human cochlear is undeniably one of the most amazing organs in human body. The functional mechanism is very unique in terms of its ability to convert the sound waves in the form of mechanical vibrations into the electrical nerve impulses. It is known that the normal human auditory system can perceive the audible frequency range between 20 Hz to 20 kHz. Scientists have conducted several researches trying to build the artificial basilar membrane in the human cochlea (cochlear biomodel). Micro-electromechanical system (MEMS) is one of the potential inventions that have the ability to mimic the active behavior of the basilar membrane. In this paper, an array of MEMS bridge beams that are mechanically sensitive to the perceived audible frequency has been proposed. An array of bridge bridge beams with 0.5 µm thickness and length varying from 200 µm to 2000 µm have been designed operate within the audible frequency range. In the bridge beams design, aluminium (Al), copper (Cu), tantalum (Ta) and platinum (Pt) have considered as the material for the bridge beam structure. From the finite element (FE) and lumped element (LE) models of the MEMS bridge beams, platinum has been found to be the best material for the cochlear biomodel design, closely mimicking the basilar membrane.

VEMP recording by binaural simultaneous stimulation in subjects with vestibulo-cochlear disorders.

PubMed

Murofushi, Toshihisa; Takai, Yoshinari; Iwasaki, Shinichi; Matsuzaki, Masaki

2005-10-01

To reduce the testing time of vestibular evoked myogenic potentials (VEMP) and the physical efforts of subjects, we studied if VEMP recording by binaural simultaneous stimulation in patients with vestibulo-cochlear disorders can be applicable as a screening test. Twenty-eight patients with vestibulo-cochlear disorders (12 men and 16 women, 22 to 77 years of age) were enrolled in this study. Patients were presented with binaural or monaural click (95 dBnHL) stimulation to record VEMPs. Their VEMP responses to binaural simultaneous stimulation were compared with those of monaural individual stimulation. Twenty-six of the 28 patients (93%) showed the same results in binaural simultaneous stimulation as in monaural individual stimulation. Amplitudes of p13-n23 to binaural simultaneous stimulation showed significant correlation to those of monaural individual stimulation ( r =0.774, P <0.0001 t -test). Peak latencies of p13 and n23 of binaural simultaneous stimulation also showed significant correlation to those of monaural individual stimulation (p13: r =0.684, P <0.0001 t -test, n23: r =0.657, P <0.0001 t -test). The binaural simultaneous stimulation method for VEMP recording is applicable for patients with vestibulo-cochlear disorders as a screening test in the clinic.

Music Engineering as a Novel Strategy for Enhancing Music Enjoyment in the Cochlear Implant Recipient

PubMed Central

Kohlberg, Gavriel D.; Mancuso, Dean M.; Chari, Divya A.; Lalwani, Anil K.

2015-01-01

Objective. Enjoyment of music remains an elusive goal following cochlear implantation. We test the hypothesis that reengineering music to reduce its complexity can enhance the listening experience for the cochlear implant (CI) listener. Methods. Normal hearing (NH) adults (N = 16) and CI listeners (N = 9) evaluated a piece of country music on three enjoyment modalities: pleasantness, musicality, and naturalness. Participants listened to the original version along with 20 modified, less complex, versions created by including subsets of the musical instruments from the original song. NH participants listened to the segments both with and without CI simulation processing. Results. Compared to the original song, modified versions containing only 1–3 instruments were less enjoyable to the NH listeners but more enjoyable to the CI listeners and the NH listeners with CI simulation. Excluding vocals and including rhythmic instruments improved enjoyment for NH listeners with CI simulation but made no difference for CI listeners. Conclusions. Reengineering a piece of music to reduce its complexity has the potential to enhance music enjoyment for the cochlear implantee. Thus, in addition to improvements in software and hardware, engineering music specifically for the CI listener may be an alternative means to enhance their listening experience. PMID:26543322

Development of micro-electromechanical system (MEMS) cochlear biomodel

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ngelayang, Thailis Bounya Anak; Latif, Rhonira

Human cochlear is undeniably one of the most amazing organs in human body. The functional mechanism is very unique in terms of its ability to convert the sound waves in the form of mechanical vibrations into the electrical nerve impulses. It is known that the normal human auditory system can perceive the audible frequency range between 20 Hz to 20 kHz. Scientists have conducted several researches trying to build the artificial basilar membrane in the human cochlea (cochlear biomodel). Micro-electromechanical system (MEMS) is one of the potential inventions that have the ability to mimic the active behavior of the basilar membrane. Inmore » this paper, an array of MEMS bridge beams that are mechanically sensitive to the perceived audible frequency has been proposed. An array of bridge bridge beams with 0.5 µm thickness and length varying from 200 µm to 2000 µm have been designed operate within the audible frequency range. In the bridge beams design, aluminium (Al), copper (Cu), tantalum (Ta) and platinum (Pt) have considered as the material for the bridge beam structure. From the finite element (FE) and lumped element (LE) models of the MEMS bridge beams, platinum has been found to be the best material for the cochlear biomodel design, closely mimicking the basilar membrane.« less

Limits on normal cochlear 'third' windows provided by previous investigations of additional sound paths into and out of the cat inner ear.

PubMed

Rosowski, John J; Bowers, Peter; Nakajima, Hideko H

2018-03-01

While most models of cochlear function assume the presence of only two windows into the mammalian cochlea (the oval and round windows), a position that is generally supported by several lines of data, there is evidence for additional sound paths into and out of the inner ear in normal mammals. In this report we review the existing evidence for and against the 'two-window' hypothesis. We then determine how existing data and inner-ear anatomy restrict transmission of sound through these additional sound pathways in cat by utilizing a well-tested model of the cat inner ear, together with anatomical descriptions of the cat cochlear and vestibular aqueducts (potential additional windows to the cochlea). We conclude: (1) The existing data place limits on the size of the cochlear and vestibular aqueducts in cat and are consistent with small volume-velocities through these ducts during ossicular stimulation of the cochlea, (2) the predicted volume velocities produced by aqueducts with diameters half the size of the bony diameters match the functional data within ±10 dB, and (3) these additional volume velocity paths contribute to the inner ear's response to non-acoustic stimulation and conductive pathology. Copyright © 2017 Elsevier B.V. All rights reserved.

Using Zebra-speech to study sequential and simultaneous speech segregation in a cochlear-implant simulation.

PubMed

Gaudrain, Etienne; Carlyon, Robert P

2013-01-01

Previous studies have suggested that cochlear implant users may have particular difficulties exploiting opportunities to glimpse clear segments of a target speech signal in the presence of a fluctuating masker. Although it has been proposed that this difficulty is associated with a deficit in linking the glimpsed segments across time, the details of this mechanism are yet to be explained. The present study introduces a method called Zebra-speech developed to investigate the relative contribution of simultaneous and sequential segregation mechanisms in concurrent speech perception, using a noise-band vocoder to simulate cochlear implants. One experiment showed that the saliency of the difference between the target and the masker is a key factor for Zebra-speech perception, as it is for sequential segregation. Furthermore, forward masking played little or no role, confirming that intelligibility was not limited by energetic masking but by across-time linkage abilities. In another experiment, a binaural cue was used to distinguish the target and the masker. It showed that the relative contribution of simultaneous and sequential segregation depended on the spectral resolution, with listeners relying more on sequential segregation when the spectral resolution was reduced. The potential of Zebra-speech as a segregation enhancement strategy for cochlear implants is discussed.

Using Zebra-speech to study sequential and simultaneous speech segregation in a cochlear-implant simulation

PubMed Central

Gaudrain, Etienne; Carlyon, Robert P.

2013-01-01

Previous studies have suggested that cochlear implant users may have particular difficulties exploiting opportunities to glimpse clear segments of a target speech signal in the presence of a fluctuating masker. Although it has been proposed that this difficulty is associated with a deficit in linking the glimpsed segments across time, the details of this mechanism are yet to be explained. The present study introduces a method called Zebra-speech developed to investigate the relative contribution of simultaneous and sequential segregation mechanisms in concurrent speech perception, using a noise-band vocoder to simulate cochlear implants. One experiment showed that the saliency of the difference between the target and the masker is a key factor for Zebra-speech perception, as it is for sequential segregation. Furthermore, forward masking played little or no role, confirming that intelligibility was not limited by energetic masking but by across-time linkage abilities. In another experiment, a binaural cue was used to distinguish target and masker. It showed that the relative contribution of simultaneous and sequential segregation depended on the spectral resolution, with listeners relying more on sequential segregation when the spectral resolution was reduced. The potential of Zebra-speech as a segregation enhancement strategy for cochlear implants is discussed. PMID:23297922

Cochlear implantation in patients with bilateral cochlear trauma.

PubMed

Serin, Gediz Murat; Derinsu, Ufuk; Sari, Murat; Gergin, Ozgül; Ciprut, Ayça; Akdaş, Ferda; Batman, Cağlar

2010-01-01

Temporal bone fracture, which involves the otic capsule, can lead to complete loss of auditory and vestibular functions, whereas the patients without fractures may experience profound sensorineural hearing loss due to cochlear concussion. Cochlear implant is indicated in profound sensorineural hearing loss due to cochlear trauma but who still have an intact auditory nerve. This is a retrospective review study. We report 5 cases of postlingually deafened patients caused by cochlear trauma, who underwent cochlear implantation. Preoperative and postoperative hearing performance will be presented. These patients are cochlear implanted after the cochlear trauma in our department between 2001 and 2006. All patients performed very well with their implants, obtained open-set speech understanding. They all became good telephone users after implantation. Their performance in speech understanding was comparable to standard postlingual adult patients implanted. Cochlear implantation is an effective aural rehabilitation in profound sensorineural hearing loss caused by temporal bone trauma. Preoperative temporal bone computed tomography, magnetic resonance imaging, and promontorium stimulation testing are necessary to make decision for the surgery and to determine the side to be implanted. Surgery could be challenging and complicated because of anatomical irregularity. Moreover, fibrosis and partial or total ossification within the cochlea must be expected. Copyright 2010. Published by Elsevier Inc.

Long-Term Asymmetric Hearing Affects Cochlear Implantation Outcomes Differently in Adults with Pre- and Postlingual Hearing Loss

PubMed Central

Boisvert, Isabelle; McMahon, Catherine M.; Dowell, Richard C.; Lyxell, Björn

2015-01-01

In many countries, a single cochlear implant is offered as a treatment for a bilateral hearing loss. In cases where there is asymmetry in the amount of sound deprivation between the ears, there is a dilemma in choosing which ear should be implanted. In many clinics, the choice of ear has been guided by an assumption that the reorganisation of the auditory pathways caused by longer duration of deafness in one ear is associated with poorer implantation outcomes for that ear. This assumption, however, is mainly derived from studies of early childhood deafness. This study compared outcomes following implantation of the better or poorer ear in cases of long-term hearing asymmetries. Audiological records of 146 adults with bilateral hearing loss using a single hearing aid were reviewed. The unaided ear had 15 to 72 years of unaided severe to profound hearing loss before unilateral cochlear implantation. 98 received the implant in their long-term sound-deprived ear. A multiple regression analysis was conducted to assess the relative contribution of potential predictors to speech recognition performance after implantation. Duration of bilateral significant hearing loss and the presence of a prelingual hearing loss explained the majority of variance in speech recognition performance following cochlear implantation. For participants with postlingual hearing loss, similar outcomes were obtained by implanting either ear. With prelingual hearing loss, poorer outcomes were obtained when implanting the long-term sound-deprived ear, but the duration of the sound deprivation in the implanted ear did not reliably predict outcomes. Contrary to an apparent clinical consensus, duration of sound deprivation in one ear has limited value in predicting speech recognition outcomes of cochlear implantation in that ear. Outcomes of cochlear implantation are more closely related to the period of time for which the brain is deprived of auditory stimulation from both ears. PMID:26043227

Improvement in cochlear flow with Pycnogenol® in patients with tinnitus: a pilot evaluation.

PubMed

Grossi, M G; Belcaro, G; Cesarone, M R; Dugall, M; Hosoi, M; Cacchio, M; Ippolito, E; Bavera, P

2010-06-01

The aim of this preliminary evaluation was to study the efficacy of Pycnogenol in improving cochlear flow in patients with mild-to-moderate tinnitus present for at least two weeks (without vertigo or important hearing loss), possibly associated with cochlear hypo-perfusion. Patients with mild-to-moderate, idiopatic, monolateral tinnitus present for at least 2 weeks were included; no vertigo or important hearing loss had been found in a specific examination. The origin of tinnitus had been sudden (hours or days). Fifty-eight patients used Pycnogenol: 24 used 150 mg/day (group A; mean age 43.2+/-4.3) and 34 patients 100 mg/day (group B: mean age 42.4+/-3.8). Controls included 24 patients (mean age 42.3+/-4.5). The groups were comparable for their clinical problem and age and sex. The average duration of treatment was 34.3+/-3.1 days. No side effects were observed and no drop-outs occurred. The variations in cochlear flow velocity (in cm/s at the cochlear artery), at inclusion and after four weeks of treatment indicated that flow velocity at the level of the affected ear was significantly lower (both the diastolic and systolic components; P<0.05) in comparison with the other ear. The treatment favored an improvement in systolic (P<0.05) and diastolic flow velocity (P<0.05) in the two treatment groups A+B. The increase in flow velocity was very limited and not significant in controls. These results suggest that in selected patients with tinnitus and altered perfusion, Pycnogenol is effective in a short period of time in relieving tinnitus symptoms by improving cochlear blood flow. The effect is more pronounced with higher Pycnogenol dosage. More studies should be planned to better evaluate the pathology and potential applications of Pycnogenol in a larger number of patients who are currently without a real therapeutic solution.

Speech perception comparisons using an implanted and an external microphone in existing cochlear implant users.

PubMed

Jenkins, Herman A; Uhler, Kristin

2012-01-01

To compare the speech understanding abilities of cochlear implant listeners using 2 microphone technologies, the Otologics fully implantable Carina and the Cochlear Freedom microphones. Feasibility study using direct comparison of the 2 microphones, nonrandomized and nonblinded within case studies. Tertiary referral center hospital outpatient clinic. Four subjects with greater than 1 year of unilateral listening experience with the Freedom Cochlear Implant and a CNC word score higher than 40%. A Carina microphone coupled to a percutaneous plug was implanted on the ipsilateral side of the cochlear implant. Two months were allowed for healing before connecting to the Carina microphone. The percutaneous plug was connected to a body worn external processor with output leads inserted into the auxiliary port of the Freedom processor. Subjects were instructed to use each of the 2 microphones for half of their daily implant use. Aided pure tone thresholds, consonant-nucleus-consonant (CNC), Bamford-Kowel-Bench Speech in Noise test (BKN-SIN), and Abbreviated Profile of Hearing Aid Benefit. All subjects had sound perceptions using both microphones. The loudness and quality of the sound was judged to be poorer with the Carina in the first 2 subjects. The latter 2 demonstrated essential equivalence in the second two listeners, with the exception of the Abbreviated Profile of Hearing Aid Benefit reporting greater percentage of problems for the Carina in the background noise situation for subject 0011-003PP. CNC word scores were better with the Freedom than the Carina in all 4 subjects. The latter 2 showed improved speech perception abilities with the Carina, compared with the first 2. The BKB-SIN showed consistently better results with the Freedom in noise. Early observations indicate that it is potentially feasible to use the fully implanted Carina microphone with the Freedom Cochlear Implant. The authors would anticipate that outcomes would improve as more knowledge is gained in signal processing and with the fabrication of an integrated device.

The effect of different cochlear implant microphones on acoustic hearing individuals’ binaural benefits for speech perception in noise

PubMed Central

Aronoff, Justin M.; Freed, Daniel J.; Fisher, Laurel M.; Pal, Ivan; Soli, Sigfrid D.

2011-01-01

Objectives Cochlear implant microphones differ in placement, frequency response, and other characteristics such as whether they are directional. Although normal hearing individuals are often used as controls in studies examining cochlear implant users’ binaural benefits, the considerable differences across cochlear implant microphones make such comparisons potentially misleading. The goal of this study was to examine binaural benefits for speech perception in noise for normal hearing individuals using stimuli processed by head-related transfer functions (HRTFs) based on the different cochlear implant microphones. Design HRTFs were created for different cochlear implant microphones and used to test participants on the Hearing in Noise Test. Experiment 1 tested cochlear implant users and normal hearing individuals with HRTF-processed stimuli and with sound field testing to determine whether the HRTFs adequately simulated sound field testing. Experiment 2 determined the measurement error and performance-intensity function for the Hearing in Noise Test with normal hearing individuals listening to stimuli processed with the various HRTFs. Experiment 3 compared normal hearing listeners’ performance across HRTFs to determine how the HRTFs affected performance. Experiment 4 evaluated binaural benefits for normal hearing listeners using the various HRTFs, including ones that were modified to investigate the contributions of interaural time and level cues. Results The results indicated that the HRTFs adequately simulated sound field testing for the Hearing in Noise Test. They also demonstrated that the test-retest reliability and performance-intensity function were consistent across HRTFs, and that the measurement error for the test was 1.3 dB, with a change in signal-to-noise ratio of 1 dB reflecting a 10% change in intelligibility. There were significant differences in performance when using the various HRTFs, with particularly good thresholds for the HRTF based on the directional microphone when the speech and masker were spatially separated, emphasizing the importance of measuring binaural benefits separately for each HRTF. Evaluation of binaural benefits indicated that binaural squelch and spatial release from masking were found for all HRTFs and binaural summation was found for all but one HRTF, although binaural summation was less robust than the other types of binaural benefits. Additionally, the results indicated that neither interaural time nor level cues dominated binaural benefits for the normal hearing participants. Conclusions This study provides a means to measure the degree to which cochlear implant microphones affect acoustic hearing with respect to speech perception in noise. It also provides measures that can be used to evaluate the independent contributions of interaural time and level cues. These measures provide tools that can aid researchers in understanding and improving binaural benefits in acoustic hearing individuals listening via cochlear implant microphones. PMID:21412155

[Site of prelingual cochlear stimulation and its effect on electrically evoked compound action potentials and refractory using the Nucleus 24 standard].

PubMed

Ma, R Y; Li, W; Jiang, X J

2016-12-01

Objective: To investigate the correlation between the site of prelingual cochlear stimulation and its effect on electrically evoked compound action potentials. Method: Recordings of auditory nerve responses were conducted in 32 prelingual subjects to demonstrate the feasibility of ECAP recordings using the nerve response telemetry(NRT) feature of the Nucleus CI24R(CA) system software. These recordings were then analyzed based on the site of cochlear stimulation defined as basal, middle and apical to determine if the amplitude, threshold and slope of the amplitude growth function and the refractory time differs depending on the region of stimulation. Result: Findings of our prelingual children showed significant differences in the ECAP recordings depending on the stimulation site. Comparing the apical with the basal region, on average higher amplitudes, lower thresholds and steeper slopes of the amplitude growth function hadbeen observed. The refractory time showed an overall dependence on cochlear region; however post-hoc tests showed no significant effect between individual regions. Conclusion: Obtaining ECAP recordings is also possible in the most apical region of the cochlea. However, differences can be observed depending on the region of the cochlea stimulated. Specifically, significant higher ECAP amplitude, lower thresholds and steeper amplitude growth function slopes have been observed in the apical region. These differences between prelingual children and adults could be explained by the location of the stimulating electrode with respect to the neural tissue in the cochlea, a higher density, or an increased neural survival rate of neural tissue in the apex. Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.

Assessing the Electrode-Neuron Interface with the Electrically Evoked Compound Action Potential, Electrode Position, and Behavioral Thresholds.

PubMed

DeVries, Lindsay; Scheperle, Rachel; Bierer, Julie Arenberg

2016-06-01

Variability in speech perception scores among cochlear implant listeners may largely reflect the variable efficacy of implant electrodes to convey stimulus information to the auditory nerve. In the present study, three metrics were applied to assess the quality of the electrode-neuron interface of individual cochlear implant channels: the electrically evoked compound action potential (ECAP), the estimation of electrode position using computerized tomography (CT), and behavioral thresholds using focused stimulation. The primary motivation of this approach is to evaluate the ECAP as a site-specific measure of the electrode-neuron interface in the context of two peripheral factors that likely contribute to degraded perception: large electrode-to-modiolus distance and reduced neural density. Ten unilaterally implanted adults with Advanced Bionics HiRes90k devices participated. ECAPs were elicited with monopolar stimulation within a forward-masking paradigm to construct channel interaction functions (CIF), behavioral thresholds were obtained with quadrupolar (sQP) stimulation, and data from imaging provided estimates of electrode-to-modiolus distance and scalar location (scala tympani (ST), intermediate, or scala vestibuli (SV)) for each electrode. The width of the ECAP CIF was positively correlated with electrode-to-modiolus distance; both of these measures were also influenced by scalar position. The ECAP peak amplitude was negatively correlated with behavioral thresholds. Moreover, subjects with low behavioral thresholds and large ECAP amplitudes, averaged across electrodes, tended to have higher speech perception scores. These results suggest a potential clinical role for the ECAP in the objective assessment of individual cochlear implant channels, with the potential to improve speech perception outcomes.

Determining electrically evoked compound action potential thresholds: a comparison of computer versus human analysis methods.

PubMed

Glassman, E Katelyn; Hughes, Michelle L

2013-01-01

Current cochlear implants (CIs) have telemetry capabilities for measuring the electrically evoked compound action potential (ECAP). Neural Response Telemetry (Cochlear) and Neural Response Imaging (Advanced Bionics [AB]) can measure ECAP responses across a range of stimulus levels to obtain an amplitude growth function. Software-specific algorithms automatically mark the leading negative peak, N1, and the following positive peak/plateau, P2, and apply linear regression to estimate ECAP threshold. Alternatively, clinicians may apply expert judgments to modify the peak markers placed by the software algorithms, or use visual detection to identify the lowest level yielding a measurable ECAP response. The goals of this study were to: (1) assess the variability between human and computer decisions for (a) marking N1 and P2 and (b) determining linear-regression threshold (LRT) and visual-detection threshold (VDT); and (2) compare LRT and VDT methods within and across human- and computer-decision methods. ECAP amplitude-growth functions were measured for three electrodes in each of 20 ears (10 Cochlear Nucleus® 24RE/CI512, and 10 AB CII/90K). LRT, defined as the current level yielding an ECAP with zero amplitude, was calculated for both computer- (C-LRT) and human-picked peaks (H-LRT). VDT, defined as the lowest level resulting in a measurable ECAP response, was also calculated for both computer- (C-VDT) and human-picked peaks (H-VDT). Because Neural Response Imaging assigns peak markers to all waveforms but does not include waveforms with amplitudes less than 20 μV in its regression calculation, C-VDT for AB subjects was defined as the lowest current level yielding an amplitude of 20 μV or more. Overall, there were significant correlations between human and computer decisions for peak-marker placement, LRT, and VDT for both manufacturers (r = 0.78-1.00, p < 0.001). For Cochlear devices, LRT and VDT correlated equally well for both computer- and human-picked peaks (r = 0.98-0.99, p < 0.001), which likely reflects the well-defined Neural Response Telemetry algorithm and the lower noise floor in the 24RE and CI512 devices. For AB devices, correlations between LRT and VDT for both peak-picker methods were weaker than for Cochlear devices (r = 0.69-0.85, p < 0.001), which likely reflect the higher noise floor of the system. Disagreement between computer and human decisions regarding the presence of an ECAP response occurred for 5 % of traces for Cochlear devices and 2.1 % of traces for AB devices. Results indicate that human and computer peak-picking methods can be used with similar accuracy for both Cochlear and AB devices. Either C-VDT or C-LRT can be used with equal confidence for Cochlear 24RE and CI512 recipients because both methods are strongly correlated with human decisions. However, for AB devices, greater variability exists between different threshold-determination methods. This finding should be considered in the context of using ECAP measures to assist with programming CIs.

A nonlinear cochlear model with the outer hair cell piezoelectric activity

NASA Astrophysics Data System (ADS)

Jiang, Xiaoai; Grosh, Karl

2003-10-01

In this paper we present a simple cochlear model which captures the most important aspect of nonlinearity in the cochlea-the nonlinearity caused by the piezoelectric-like activity of outer hair cells and the variable conductance of the outer hair cell stereocilia. A one-dimensional long-wave model is built to simulate the dynamic response of the fluid-loaded basilar membrane. The basilar membrane is simulated as isolated linear oscillators along the cochlear length, and its motion is coupled with the fluid pressure and the nonlinear force produced by the outer hair cells. As the basilar membrane moves, the fluid shears stereocilia, and the resulting ion flow changes the transmembrane potential of the outer hair cells and subsequently their length, leading to further movement of the basilar membrane. The piezoelectric-like activity of the outer hair cell is simulated by a current source, and stereocilia motion is modeled as a varying conductance that changes as the basilar membrane moves. A solution in the time domain will be presented. [Work supported by NIH.

TRPC1 is required for survival and proliferation of cochlear spiral ganglion stem/progenitor cells.

PubMed

Chen, Hsin-Chien; Wang, Chih-Hung; Shih, Cheng-Ping; Chueh, Sheau-Huei; Liu, Shu-Fan; Chen, Hang-Kang; Lin, Yi-Chun

2015-12-01

The present studies were designed to test the hypothesis that canonical transient receptor potential channel 1 (TRPC1) is required for the proliferation of cochlear spiral ganglion stem/progenitor cells (SPCs). TRPC1 were detected and evaluated in postnatal day 1 CBA/CaJ mice pups derived-cochlear spiral ganglion SPCs by reverse transcription-polymerase chain reaction, Western blot, immunocytochemistry, and calcium imaging. The cell viability and proliferation of the spiral ganglion SPCs following si-RNA mediated knockdown of TRPC1 or addition of TRPC channel blocker SKF9635 were compared to controls. In spiral ganglion SPCs, TRPC1 was found to be the most abundantly expressed TRPC subunit and shown to contribute to store-operated calcium entry. Silencing of TRPC1 or addition of TRPC channel blockers significantly decreased the rate of cell proliferation. The results suggest that TRPC1 might serve as an essential molecule in regulating the proliferation of spiral ganglion SPCs. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Cannabinoids, cannabinoid receptors and tinnitus.

PubMed

Smith, Paul F; Zheng, Yiwen

2016-02-01

One hypothesis suggests that tinnitus is a form of sensory epilepsy, arising partly from neuronal hyperactivity in auditory regions of the brain such as the cochlear nucleus and inferior colliculus. Although there is currently no effective drug treatment for tinnitus, anti-epileptic drugs are used in some cases as a potential treatment option. There is increasing evidence to suggest that cannabinoid drugs, i.e. cannabinoid receptor agonists, can also have anti-epileptic effects, at least in some cases and in some parts of the brain. It has been reported that cannabinoid CB1 receptors and the endogenous cannabinoid, 2-arachidonylglycerol (2-AG), are expressed in the cochlear nucleus and that they are involved in the regulation of plasticity. This review explores the question of whether cannabinoid receptor agonists are likely to be pro- or anti-epileptic in the cochlear nucleus and therefore whether cannabinoids and Cannabis itself are likely to make tinnitus better or worse. Copyright © 2015 Elsevier B.V. All rights reserved.

Long term effects of BAPTA in scala media on cochlear function.

PubMed

Sellick, Peter M

2007-09-01

BAPTA was iontophoresed or allowed to diffuse into the scala media of the first turn of the guinea pig cochlea via pipettes inserted through the round window and basilar membrane. Cochlear action potential (CAP) thresholds for basal turn frequencies were elevated, scala media cochlear microphonic in response to a 207Hz tone were drastically reduced and the distortion products 2f1-f2 and f2-f2 in response to primaries set at 18 and 21.6kHz were eliminated or severely reduced. The animals were recovered and the above measurements repeated between 24 and 240h after the application of BAPTA. In all animals thresholds for basal turn frequencies remained elevated, and the distortion components were severely reduced. The endolymphatic potential (EP), measured through the basilar membrane on recovery, was not significantly different from the values measured before BAPTA was applied. If the effect of BAPTA, in lowering endolymphatic Ca(2+) concentration, is restricted to the destruction of tip links, as has been shown in many other preparations, then these results suggest that this effect has permanent consequences, either because the tip links failed to regenerate or because their destruction precipitated the degeneration of OHCs. These results may have a bearing on the mechanisms behind permanent threshold shift.

Displacements of the organ of Corti by gel injections into the cochlear apex

PubMed Central

Salt, Alec N.; Brown, Daniel J.; Hartsock, Jared J.; Plontke, Stefan K.

2009-01-01

In order to transduce sounds efficiently, the stereocilia of hair cells in the organ of Corti must be positioned optimally. Mechanical displacements, such as pressure differentials across the organ caused by endolymphatic hydrops, may impair sensitivity. Studying this phenomenon has been limited by the technical difficulty of inducing sustained displacements of stereocilia in vivo. We have found that small injections (0.5 to 2 μL) of Healon gel into the cochlear apex of guinea pigs produced sustained changes of endocochlear potential (EP), summating potential (SP) and transducer operating point (OP) in a manner consistent with a mechanically-induced position change of the organ of Corti in the basal turn. Induced changes immediately recovered when injection ceased. In addition, effects of low-frequency bias tones on EP, SP and OP were enhanced during the injection of gel and remained hypersensitive after injection ceased. This is thought to result from the viscous gel mechanically limiting pressure shunting through the helicotrema. Cochlear microphonics measured as frequency was varied showed enhancement below 100 Hz but most notably in the sub-auditory range. Sensitivity to low-frequency biasing was also enhanced in animals with surgically-induced endolymphatic hydrops, suggesting that obstruction of the perilymphatic space by hydrops could contribute to the pathophysiology of this condition. PMID:19217935

Three-dimensional current flow in a large-scale model of the cochlea and the mechanism of amplification of sound.

PubMed

Mistrík, Pavel; Mullaley, Chris; Mammano, Fabio; Ashmore, Jonathan

2009-03-06

The mammalian inner ear uses its sensory hair cells to detect and amplify incoming sound. It is unclear whether cochlear amplification arises uniquely from a voltage-dependent mechanism (electromotility) associated with outer hair cells (OHCs) or whether other mechanisms are necessary, for the voltage response of OHCs is apparently attenuated excessively by the membrane electrical filter. The cochlea contains many thousands of hair cells organized in extensive arrays, embedded in an electrically coupled system of supporting cells. We have therefore constructed a multi-element, large-scale computational model of cochlear sound transduction to study the underlying potassium (K+) recirculation. We have included experimentally determined parameters of cochlear macromechanics, which govern sound transduction, and data on hair cells' electrical parameters including tonotopical variation in the membrane conductance of OHCs. In agreement with the experiment, the model predicts an exponential decay of extracellular longitudinal K+ current spread. In contrast to the predictions from isolated cells, it also predicts low attenuation of the OHC transmembrane receptor potential (-5 dB per decade) in the 0.2-30 kHz range. This suggests that OHC electromotility could be driven by the transmembrane potential. Furthermore, the OHC electromotility could serve as a single amplification mechanism over the entire hearing range.

Cochlear implant in Hong Kong Cantonese.

PubMed

Tang, S O; Luk, W S; Lau, C C; So, K W; Wong, C M; Yiu, M L; Kwok, C L

1990-11-01

Cochlear implant surgery was performed in four Cantonese-speaking postlingually deaf Chinese adults, using the House/3M single channel device. This article outlines the methodology, including preoperative assessment and postoperative rehabilitation; and explains the necessary modifications in speech and audiologic work-up in Cantonese-speaking patients. Salient features of Cantonese phonetics, especially its tonal characteristics, are described. The findings of the study are presented. The results of the cochlear implant would suggest a performance superior to that of the hearing aid. Furthermore, the cochlear implant is able to detect tonal cues. This quality of the cochlear implant may prove to be a valuable asset to a tonal language-speaking cochlear implantee.

Visualization and contractile activity of cochlear pericytes in the capillaries of the spiral ligament.

PubMed

Dai, Min; Nuttall, Alfred; Yang, Yue; Shi, Xiaorui

2009-08-01

Pericytes, mural cells located on microvessels, are considered to play an important role in the formation of the vasculature and the regulation of local blood flow in some organs. Little is known about the physiology of cochlear pericytes. In order to investigate the function of cochlear pericytes, we developed a method to visualize cochlear pericytes using diaminofluorescein-2 diacetate (DAF-2DA) and intravital fluorescence microscopy. This method can permit the study of the effect of vasoactive agents on pericytes under the in vivo and normal physiological condition. The specificity of the labeling method was verified by the immunofluorescence labeling of pericyte maker proteins such as desmin, neural proteoglycan (NG2), and thymocyte differentiation antigen 1 (Thy-1). Superfused K(+) and Ca(2+) to the cochlear lateral wall resulted in localized constriction of capillaries at pericyte locations both in vivo and in vitro, while there was no obvious change in cochlear capillary diameters with application of the adrenergic neurotransmitter noradrenaline. The method could be an effective way to visualize cochlear pericytes and microvessels and study lateral wall vascular physiology. Moreover, we demonstrate for the first time that cochlear pericytes have contractility, which may be important for regulation of cochlear blood flow.

Perceptually aligning apical frequency regions leads to more binaural fusion of speech in a cochlear implant simulation.

PubMed

Staisloff, Hannah E; Lee, Daniel H; Aronoff, Justin M

2016-07-01

For bilateral cochlear implant users, the left and right arrays are typically not physically aligned, resulting in a degradation of binaural fusion, which can be detrimental to binaural abilities. Perceptually aligning the two arrays can be accomplished by disabling electrodes in one ear that do not have a perceptually corresponding electrode in the other side. However, disabling electrodes at the edges of the array will cause compression of the input frequency range into a smaller cochlear extent, which may result in reduced spectral resolution. An alternative approach to overcome this mismatch would be to only align one edge of the array. By aligning either only the apical or basal end of the arrays, fewer electrodes would be disabled, potentially causing less reduction in spectral resolution. The goal of this study was to determine the relative effect of aligning either the basal or apical end of the electrode with regards to binaural fusion. A vocoder was used to simulate cochlear implant listening conditions in normal hearing listeners. Speech signals were vocoded such that the two ears were either predominantly aligned at only the basal or apical end of the simulated arrays. The experiment was then repeated with a spectrally inverted vocoder to determine whether the detrimental effects on fusion were related to the spectral-temporal characteristics of the stimuli or the location in the cochlea where the misalignment occurred. In Experiment 1, aligning the basal portion of the simulated arrays led to significantly less binaural fusion than aligning the apical portions of the simulated array. However, when the input was spectrally inverted, aligning the apical portion of the simulated array led to significantly less binaural fusion than aligning the basal portions of the simulated arrays. These results suggest that, for speech, with its predominantly low frequency spectral-temporal modulations, it is more important to perceptually align the apical portion of the array to better preserve binaural fusion. By partially aligning these arrays, cochlear implant users could potentially increase their ability to fuse speech sounds presented to the two ears while maximizing spectral resolution. Copyright © 2016 Elsevier B.V. All rights reserved.

Cochlear implants: system design, integration, and evaluation.

PubMed

Zeng, Fan-Gang; Rebscher, Stephen; Harrison, William; Sun, Xiaoan; Feng, Haihong

2008-01-01

As the most successful neural prosthesis, cochlear implants have provided partial hearing to more than 120000 persons worldwide; half of which being pediatric users who are able to develop nearly normal language. Biomedical engineers have played a central role in the design, integration and evaluation of the cochlear implant system, but the overall success is a result of collaborative work with physiologists, psychologists, physicians, educators, and entrepreneurs. This review presents broad yet in-depth academic and industrial perspectives on the underlying research and ongoing development of cochlear implants. The introduction accounts for major events and advances in cochlear implants, including dynamic interplays among engineers, scientists, physicians, and policy makers. The review takes a system approach to address critical issues in cochlear implant research and development. First, the cochlear implant system design and specifications are laid out. Second, the design goals, principles, and methods of the subsystem components are identified from the external speech processor and radio frequency transmission link to the internal receiver, stimulator and electrode arrays. Third, system integration and functional evaluation are presented with respect to safety, reliability, and challenges facing the present and future cochlear implant designers and users. Finally, issues beyond cochlear implants are discussed to address treatment options for the entire spectrum of hearing impairment as well as to use the cochlear implant as a model to design and evaluate other similar neural prostheses such as vestibular and retinal implants.

Sound-direction identification with bilateral cochlear implants.

PubMed

Neuman, Arlene C; Haravon, Anita; Sislian, Nicole; Waltzman, Susan B

2007-02-01

The purpose of this study was to compare the accuracy of sound-direction identification in the horizontal plane by bilateral cochlear implant users when localization was measured with pink noise and with speech stimuli. Eight adults who were bilateral users of Nucleus 24 Contour devices participated in the study. All had received implants in both ears in a single surgery. Sound-direction identification was measured in a large classroom by using a nine-loudspeaker array. Localization was tested in three listening conditions (bilateral cochlear implants, left cochlear implant, and right cochlear implant), using two different stimuli (a speech stimulus and pink noise bursts) in a repeated-measures design. Sound-direction identification accuracy was significantly better when using two implants than when using a single implant. The mean root-mean-square error was 29 degrees for the bilateral condition, 54 degrees for the left cochlear implant, and 46.5 degrees for the right cochlear implant condition. Unilateral accuracy was similar for right cochlear implant and left cochlear implant performance. Sound-direction identification performance was similar for speech and pink noise stimuli. The data obtained in this study add to the growing body of evidence that sound-direction identification with bilateral cochlear implants is better than with a single implant. The similarity in localization performance obtained with the speech and pink noise supports the use of either stimulus for measuring sound-direction identification.

Ozone Prevents Cochlear Damage From Ischemia-Reperfusion Injury in Guinea Pigs.

PubMed

Onal, Merih; Elsurer, Cagdas; Selimoglu, Nebil; Yilmaz, Mustafa; Erdogan, Ender; Bengi Celik, Jale; Kal, Oznur; Onal, Ozkan

2017-08-01

The cochlea is an end organ, which is metabolically dependent on a nutrient and oxygen supply to maintain its normal physiological function. Cochlear ischemia and reperfusion (IR) injury is considered one of the most important causes of human idiopathic sudden sensorineural hearing loss. The aim of the present study was to study the efficacy of ozone therapy against cochlear damage caused by IR injury and to investigate the potential clinical use of this treatment for sudden deafness. Twenty-eight guinea pigs were randomized into four groups. The sham group (S) (n = 7) was administered physiological saline intraperitoneally (i.p.) for 7 days. The ozone group (O) (n = 7) was administered 1 mg/kg of ozone i.p. for 7 days. In the IR + O group (n = 7), 1 mg/kg of ozone was administered i.p. for 7 days before IR injury. On the eighth day, the IR + O group was subjected to cochlear ischemia for 15 min by occluding the bilateral vertebral artery and vein with a nontraumatic clamp and then reperfusion for 2 h. The IR group was subjected to cochlear IR injury. After the IR procedure, the guinea pigs were sacrificed on the same day. In a general histological evaluation, cochlear and spiral ganglionic tissues were examined with a light microscope, and apoptotic cells were counted by terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) staining. The apoptotic index (AI) was then calculated. Blood samples were sent for analyses of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), catalase, malondialdehyde (MDA), the total oxidant score (TOS), and total antioxidant capacity (TAC). Data were evaluated statistically using the Kruskal-Wallis test. The AI was highest in the IR group. The AI of the IR + O group was lower than that of the IR group. The biochemical antioxidant parameters SOD and GSH-Px and the TAC values were highest in the O group and lowest in the IR group. The MDA level and TOS were highest in the IR group and lowest in the O group. Controlled ozone administration stimulated endogenous antioxidant defense systems, thereby helping the body to combat IR injury. Although this study revealed a statistically significant decrease in cochlear IR damage following ozone therapy, further studies will be necessary to explain the protective mechanisms of ozone therapy in cochlear IR injury. © 2017 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

Cochlear Implants Special Issue Article: Vocal Emotion Recognition by Normal-Hearing Listeners and Cochlear Implant Users

PubMed Central

Luo, Xin; Fu, Qian-Jie; Galvin, John J.

2007-01-01

The present study investigated the ability of normal-hearing listeners and cochlear implant users to recognize vocal emotions. Sentences were produced by 1 male and 1 female talker according to 5 target emotions: angry, anxious, happy, sad, and neutral. Overall amplitude differences between the stimuli were either preserved or normalized. In experiment 1, vocal emotion recognition was measured in normal-hearing and cochlear implant listeners; cochlear implant subjects were tested using their clinically assigned processors. When overall amplitude cues were preserved, normal-hearing listeners achieved near-perfect performance, whereas listeners with cochlear implant recognized less than half of the target emotions. Removing the overall amplitude cues significantly worsened mean normal-hearing and cochlear implant performance. In experiment 2, vocal emotion recognition was measured in listeners with cochlear implant as a function of the number of channels (from 1 to 8) and envelope filter cutoff frequency (50 vs 400 Hz) in experimental speech processors. In experiment 3, vocal emotion recognition was measured in normal-hearing listeners as a function of the number of channels (from 1 to 16) and envelope filter cutoff frequency (50 vs 500 Hz) in acoustic cochlear implant simulations. Results from experiments 2 and 3 showed that both cochlear implant and normal-hearing performance significantly improved as the number of channels or the envelope filter cutoff frequency was increased. The results suggest that spectral, temporal, and overall amplitude cues each contribute to vocal emotion recognition. The poorer cochlear implant performance is most likely attributable to the lack of salient pitch cues and the limited functional spectral resolution. PMID:18003871

[Emotional response to music by postlingually-deafened adult cochlear implant users].

PubMed

Wang, Shuo; Dong, Ruijuan; Zhou, Yun; Li, Jing; Qi, Beier; Liu, Bo

2012-10-01

To assess the emotional response to music by postlingually-deafened adult cochlear implant users. Munich music questionnaire (MUMU) was used to match the music experience and the motivation of use of music between 12 normal-hearing and 12 cochlear implant subjects. Emotion rating test in Musical Sounds in Cochlear Implants (MuSIC) test battery was used to assess the emotion perception ability for both normal-hearing and cochlear implant subjects. A total of 15 pieces of music phases were used. Responses were given by selecting the rating scales from 1 to 10. "1" represents "very sad" feeling, and "10" represents "very happy feeling. In comparison with normal-hearing subjects, 12 cochlear implant subjects made less active use of music for emotional purpose. The emotion ratings for cochlear implant subjects were similar to normal-hearing subjects, but with large variability. Post-lingually deafened cochlear implant subjects on average performed similarly in emotion rating tasks relative to normal-hearing subjects, but their active use of music for emotional purpose was obviously less than normal-hearing subjects.

The cochlear size of bats and rodents derived from MRI images and histology.

PubMed

Hsiao, Chun Jen; Jen, Philip Hung-Sun; Wu, Chung Hsin

2015-05-27

From the evolutionary perspective, the ear of each animal species is built for effective processing of the biologically relevant signals used for communication and acoustically guided orientation. Because the sound pulses used by echolocating bats for orientation and rodents for communication are quite different, the basic design of the mammalian auditory system commonly shared by echolocating bats must be specialized in some manner to effectively process their species-specific sounds. The present study examines the difference in the cochlea of these animal species using MRI images and histological techniques. We report here that, although all these animal species share a similar cochlear structure, they vary in their cochlear size and turns. Bats using constant frequency-frequency-modulated pulses (CF-FM bats) and frequency-modulated pulses (FM bats) for echolocation have a larger cochlear size and more cochlear turns than rodents (mice and rats). However, CF-FM bats have the largest cochlear size and most cochlear turns. This difference in cochlear size and turns of these animal species is discussed in relation to their biologically relevant sounds and acoustic behavior.

Guideline on cochlear implants.

PubMed

Manrique, Manuel; Ramos, Ángel; de Paula Vernetta, Carlos; Gil-Carcedo, Elisa; Lassaleta, Luis; Sanchez-Cuadrado, Isabel; Espinosa, Juan Manuel; Batuecas, Ángel; Cenjor, Carlos; Lavilla, María José; Núñez, Faustino; Cavalle, Laura; Huarte, Alicia

2018-03-26

In the last decade numerous hospitals have started to work with patients who are candidates for a cochlear implant (CI) and there have been numerous and relevant advances in the treatment of sensorineural hearing loss that extended the indications for cochlear implants. To provide a guideline on cochlear implants to specialists in otorhinolaryngology, other medical specialities, health authorities and society in general. The Scientific Committees of Otology, Otoneurology and Audiology from the Spanish Society of Otolaryngology and Head and Neck Surgery (SEORL-CCC), in a coordinated and agreed way, performed a review of the current state of CI based on the existing regulations and in the scientific publications referenced in the bibliography of the document drafted. The clinical guideline on cochlear implants provides information on: a) Definition and description of Cochlear Implant; b) Indications for cochlear implants; c) Organizational requirements for a cochlear implant programme. A clinical guideline on cochlear implants has been developed by a Committee of Experts of the SEORL-CCC, to help and guide all the health professionals involved in this field of CI in decision-making to treathearing impairment. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Effect of shorter pulse duration in cochlear neural activation with an 810-nm near-infrared laser.

PubMed

Wang, Jingxuan; Tian, Lan; Lu, Jianren; Xia, Ming; Wei, Ying

2017-02-01

Optical neural stimulation in the cochlea has been presented as an alternative technique to the electrical stimulation due to its potential in spatially selectivity enhancement. So far, few studies have selected the near-infrared (NIR) laser in cochlear neural stimulation and limited optical parameter space has been examined. This paper focused on investigating the optical parameter effect on NIR stimulation of auditory neurons, especially under shorter pulse durations. The spiral ganglion neurons in the cochlea of deafened guinea pigs were stimulated with a pulsed 810-nm NIR laser in vivo. The laser radiation was delivered by an optical fiber and irradiated towards the modiolus. Optically evoked auditory brainstem responses (OABRs) with various optical parameters were recorded and investigated. The OABRs could be elicited with the cochlear deafened animals by using the 810-nm laser in a wide pulse duration ranged from 20 to 1000 μs. Results showed that the OABR intensity increased along with the increasing laser radiant exposure of limited range at each specific pulse duration. In addition, for the pulse durations from 20 to 300 μs, the OABR intensity increased monotonically along with the pulse duration broadening. While for pulse durations above 300 μs, the OABR intensity basically kept stable with the increasing pulse duration. The 810-nm NIR laser could be an effective stimulus in evoking the cochlear neuron response. Our experimental data provided evidence to optimize the pulse duration range, and the results suggested that the pulse durations from 20 to 300 μs could be the optimized range in cochlear neural activation with the 810-nm-wavelength laser.

Survival, migration, and differentiation of Sox1-GFP embryonic stem cells in coculture with an auditory brainstem slice preparation.

PubMed

Glavaski-Joksimovic, Aleksandra; Thonabulsombat, Charoensri; Wendt, Malin; Eriksson, Mikael; Palmgren, Björn; Jonsson, Anna; Olivius, Petri

2008-03-01

The poor regeneration capability of the mammalian hearing organ has initiated different approaches to enhance its functionality after injury. To evaluate a potential neuronal repair paradigm in the inner ear and cochlear nerve we have previously used embryonic neuronal tissue and stem cells for implantation in vivo and in vitro. At present, we have used in vitro techniques to study the survival and differentiation of Sox1-green fluorescent protein (GFP) mouse embryonic stem (ES) cells as a monoculture or as a coculture with rat auditory brainstem slices. For the coculture, 300 microm-thick brainstem slices encompassing the cochlear nucleus and cochlear nerve were prepared from postnatal SD rats. The slices were propagated using the membrane interface method and the cochlear nuclei were prelabeled with DiI. After some days in culture a suspension of Sox1 cells was deposited next to the brainstem slice. Following deposition Sox1 cells migrated toward the brainstem and onto the cochlear nucleus. GFP was not detectable in undifferentiated ES cells but became evident during neural differentiation. Up to 2 weeks after transplantation the cocultures were fixed. The undifferentiated cells were evaluated with antibodies against progenitor cells whereas the differentiated cells were determined with neuronal and glial markers. The morphological and immunohistochemical data indicated that Sox1 cells in monoculture differentiated into a higher percentage of glial cells than neurons. However, when a coculture was used a significantly lower percentage of Sox1 cells differentiated into glial cells. The results demonstrate that a coculture of Sox1 cells and auditory brainstem present a useful model to study stem cell differentiation.

A novel perfusion-based method for cochlear implant electrode insertion.

PubMed

Kale, Sushrut; Cervantes, Vanessa M; Wu, Mailing R; Pisano, Dominic V; Sheth, Nakul; Olson, Elizabeth S

2014-08-01

A cochlear implant (CI) restores partial hearing to profoundly deaf individuals. CI electrodes are inserted manually in the cochlea and surgeons rely on tactile feedback from the implant to determine when to stop the insertion. This manual insertion method results in a large degree of variability in surgical outcomes and intra-cochlear trauma. Additionally, implants often span only the basal turn. In the present study we report on the development of a new method to assist CI electrode insertion. The design objectives are (1) an automated and standardized insertion technique across patients with (2) more apical insertion than is possible by the contemporary methods, while (3) minimizing insertion trauma. The method relies on a viscous fluid flow through the cochlea to carry the electrode array with it. A small cochleostomy (∼100-150 um in diameter) is made in scala vestibuli (SV) and the round window (RW) membrane is opened. A flow of diluted Sodium Hyaluronate (also known as Hyaluronic Acid, (HA)) is set up from the RW to the SV opening using a perfusion pump that sets up a unidirectional flow. Once the flow is established an implant is dropped into the ongoing flow. Here we present a proof-of-concept study where we used this technique to insert silicone implants all the way to the cochlear apex in rats and gerbils. In light-microscopic histology, the implantation occurred without cochlear trauma. To further assess the ototoxicity of the HA perfusion, we measured compound action potential (CAP) thresholds following the perfusion of HA, and found that the CAP thresholds were substantially elevated. Thus, at this point the method is promising, and requires further development to become clinically viable. Copyright © 2014 Elsevier B.V. All rights reserved.

Effect of embedded dexamethasone in cochlear implant array on insertion forces in an artificial model of scala tympani.

PubMed

Nguyen, Yann; Bernardeschi, Daniele; Kazmitcheff, Guillaume; Miroir, Mathieu; Vauchel, Thomas; Ferrary, Evelyne; Sterkers, Olivier

2015-02-01

Loading otoprotective drug into cochlear implant might change its mechanical properties, thus compromising atraumatic insertion. This study evaluated the effect of incorporation of dexamethasone (DXM) in the silicone of cochlear implant arrays on insertion forces. Local administration of DXM with embedded array can potentially reduce inflammation and fibrosis after cochlear implantation procedure to improve hearing preservation and reduce long-term impedances. Four models of arrays have been tested: 0.5-mm distal diameter array (n = 5) used as a control, drug-free 0.4-mm distal diameter array (n = 5), 0.4-mm distal diameter array with 1% eluded DXM silicone (n = 5), and 0.4-mm distal diameter array with 10% eluded DXM silicone (n = 5). Via a motorized insertion bench, each array has been inserted into an artificial scala tympani model. The forces were recorded by a 6-axis force sensor. Each array was tested seven times for a total number of 140 insertions. During the first 10-mm insertion, no difference between the four models was observed. From 10- to 24-mm insertion, the 0.5-mm distal diameter array presented higher insertion forces than the drug-free 0.4-mm distal diameter arrays, with or without DXM. Friction forces for drug-free 0.4-mm distal diameter array and 0.4-mm distal diameter DXM eluded arrays were similar on all insertion lengths. Incorporation of DXM in silicone for cochlear implant design does not change electrode array insertion forces. It does not raise the risk of trauma during array insertion, making it suitable for long-term in situ administration to the cochlea.

Cochlear implants in children implanted in Jordan: A parental overview.

PubMed

Alkhamra, Rana A

2015-07-01

Exploring the perspective of parents on the cochlear implant process in Jordan. Sixty parents of deaf children were surveyed on the information gathering process prior to cochlear implant surgery, and their implant outcome expectations post-surgery. Whether child or parent characteristics may impact parents' post-surgical expectations was explored. Although parents used a variety of information sources when considering a cochlear implant, the ear, nose and throat doctor comprised their major source of information (60%). Parents received a range of information prior to cochlear implant but agreed (93.3%) on the need for a multidisciplinary team approach. Post-surgically, parents' expected major developments in the areas of spoken language (97%), and auditory skills (100%). Receiving education in mainstream schools (92%) was expected too. Parents perceived the cochlear implant decision as the best decision they can make for their child (98.3%). A significant correlation was found between parents contentment with the cochlear implant decision and expecting developments in the area of reading and writing (r=0.7). Child's age at implantation and age at hearing loss diagnosis significantly affected parents' post-implant outcome expectations (p<0.05). Despite the general satisfaction from the information quantity and quality prior to cochlear implant, parents agree on the need for a comprehensive multidisciplinary team approach during the different stages of the cochlear implant process. Parents' education about cochlear implants prior to the surgery can affect their post-surgical outcome expectations. The parental perspective presented in this study can help professionals develop better understanding of parents' needs and expectations and henceforth improve their services and support during the different stages of the cochlear implant process. Copyright © 2015. Published by Elsevier Ireland Ltd.

In vivo imaging of mammalian cochlear blood flow using fluorescence microendoscopy.

PubMed

Monfared, Ashkan; Blevins, Nikolas H; Cheung, Eunice L M; Jung, Juergen C; Popelka, Gerald; Schnitzer, Mark J

2006-02-01

We sought to develop techniques for visualizing cochlear blood flow in live mammalian subjects using fluorescence microendoscopy. Inner ear microcirculation appears to be intimately involved in cochlear function. Blood velocity measurements suggest that intense sounds can alter cochlear blood flow. Disruption of cochlear blood flow may be a significant cause of hearing impairment, including sudden sensorineural hearing loss. However, inability to image cochlear blood flow in a nondestructive manner has limited investigation of the role of inner ear microcirculation in hearing function. Present techniques for imaging cochlear microcirculation using intravital light microscopy involve extensive perturbations to cochlear structure, precluding application in human patients. The few previous endoscopy studies of the cochlea have suffered from optical resolution insufficient for visualizing cochlear microvasculature. Fluorescence microendoscopy is an emerging minimally invasive imaging modality that provides micron-scale resolution in tissues inaccessible to light microscopy. In this article, we describe the use of fluorescence microendoscopy in live guinea pigs to image capillary blood flow and movements of individual red blood cells within the basal turn of the cochlea. We anesthetized eight adult guinea pigs and accessed the inner ear through the mastoid bulla. After intravenous injection of fluorescein dye, we made a limited cochleostomy and introduced a compound doublet gradient refractive index endoscope probe 1 mm in diameter into the inner ear. We then imaged cochlear blood flow within individual vessels in an epifluorescence configuration using one-photon fluorescence microendoscopy. We observed single red blood cells passing through individual capillaries in several cochlear structures, including the round window membrane, spiral ligament, osseous spiral lamina, and basilar membrane. Blood flow velocities within inner ear capillaries varied widely, with observed speeds reaching up to approximately 500 microm/s. Fluorescence microendoscopy permits visualization of cochlear microcirculation with micron-scale optical resolution and determination of blood flow velocities through analysis of video sequences.

Electrophysiologic identification of the cochlear nerve fibers during cerebello-pontine angle surgery.

PubMed

Colletti, V; Fiorino, F G

1993-11-01

To facilitate identification and preservation of the auditory nerve during cerebello-pontine angle surgery, bipolar recording of cochlear nerve compound action potentials (CNAPs) was performed. Two silver wires insulated with teflon up to the exposed ends were utilized as electrodes. They were twisted together, the distance between the two tips being 1 mm or less. Rarefaction polarity clicks (31/s) ranging from the psychoacoustical threshold to 120 dB pe SPL were used as stimuli. The investigation was performed in three groups of patients. The first group consisted of 9 patients submitted to vestibular neurectomy and 4 patients operated on by microvascular decompression of the eighth nerve. The second group comprised 8 patients with acoustic tumors smaller than 2 mm and serviceable hearing. Postoperative audiometric results in the subjects in the second group were compared with those obtained in well-matched homogeneous controls consisting of patients with acoustic neuroma operated on without the aid of CNAP recording. Bipolar recording from the eighth nerve was extremely selective, a good response being obtained only when positioning the electrode on the cochlear portion of the eighth nerve. During removal of the acoustic neuroma, repeated bipolar probing of the tumor and eighth nerve facilitated the task of distinguishing the cochlear nerve from other nervous structures and from the tumor, and contributed to preserving hearing in most patients.

Contribution of nonimplanted ear to pitch perception for prelingually deafened cochlear implant recipients.

PubMed

Chen, Joshua Kuang-Chao; Chuang, Ann Yi-Chiun; McMahon, Catherine; Tung, Tao-Hsin; Li, Lieber Po-Hung

2014-09-01

Bimodal stimulation (BMS) has been shown to be beneficial for the performance of pitch ranking in postlingually deafened adults. However, the contribution of nonimplanted ears to pitch perception with respect to duration of hearing aid (HAs) use for prelingually cochlear implantees remained unclear. This study aimed to investigate whether experiences/duration of HAs use in the nonimplanted ear improved pitch perception ability in this population of subjects. Twenty-nine children with congenital/prelingual deafness of profound degree were studied. Test stimuli consisted of 2 sequential piano tones, ranging from C (256 Hz) to B (495 Hz). Children were asked to identify the pitch relationship between the 2 tones (i.e., same, higher, or lower). Duration of HAs use was the major factor related to the correct rate for pitch perception. Overall correct rate for pitch perception (O) could be best predicted by duration of HAs use (DuA) (O = 0.561XDuA, r = 0. 315, p = 0.002). Experiences of HAs use appear to improve pitch perception ability in prelingually cochlear implantees. This suggests that incorporation of HAs use early in life and through the postoperative rehabilitation program for prelingually deafened children with cochlear implants would be beneficial, although an association does not guarantee causality. A longitudinal study is needed to show whether improvement of music performance with duration of HAs use in these children is measurable using auditory evoked potentials.

Bilateral cochlear implantation in a patient with bilateral temporal bone fractures.

PubMed

Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan

2011-01-01

With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation. Copyright © 2011 Elsevier Inc. All rights reserved.

Effect of Pulse Polarity on Thresholds and on Non-monotonic Loudness Growth in Cochlear Implant Users.

PubMed

Macherey, Olivier; Carlyon, Robert P; Chatron, Jacques; Roman, Stéphane

2017-06-01

Most cochlear implants (CIs) activate their electrodes non-simultaneously in order to eliminate electrical field interactions. However, the membrane of auditory nerve fibers needs time to return to its resting state, causing the probability of firing to a pulse to be affected by previous pulses. Here, we provide new evidence on the effect of pulse polarity and current level on these interactions. In experiment 1, detection thresholds and most comfortable levels (MCLs) were measured in CI users for 100-Hz pulse trains consisting of two consecutive biphasic pulses of the same or of opposite polarity. All combinations of polarities were studied: anodic-cathodic-anodic-cathodic (ACAC), CACA, ACCA, and CAAC. Thresholds were lower when the adjacent phases of the two pulses had the same polarity (ACCA and CAAC) than when they were different (ACAC and CACA). Some subjects showed a lower threshold for ACCA than for CAAC while others showed the opposite trend demonstrating that polarity sensitivity at threshold is genuine and subject- or electrode-dependent. In contrast, anodic (CAAC) pulses always showed a lower MCL than cathodic (ACCA) pulses, confirming previous reports. In experiments 2 and 3, the subjects compared the loudness of several pulse trains differing in current level separately for ACCA and CAAC. For 40 % of the electrodes tested, loudness grew non-monotonically as a function of current level for ACCA but never for CAAC. This finding may relate to a conduction block of the action potentials along the fibers induced by a strong hyperpolarization of their central processes. Further analysis showed that the electrodes showing a lower threshold for ACCA than for CAAC were more likely to yield a non-monotonic loudness growth. It is proposed that polarity sensitivity at threshold reflects the local neural health and that anodic asymmetric pulses should preferably be used to convey sound information while avoiding abnormal loudness percepts.

Close-field electroporation gene delivery using the cochlear implant electrode array enhances the bionic ear.

PubMed

Pinyon, Jeremy L; Tadros, Sherif F; Froud, Kristina E; Y Wong, Ann C; Tompson, Isabella T; Crawford, Edward N; Ko, Myungseo; Morris, Renée; Klugmann, Matthias; Housley, Gary D

2014-04-23

The cochlear implant is the most successful bionic prosthesis and has transformed the lives of people with profound hearing loss. However, the performance of the "bionic ear" is still largely constrained by the neural interface itself. Current spread inherent to broad monopolar stimulation of the spiral ganglion neuron somata obviates the intrinsic tonotopic mapping of the cochlear nerve. We show in the guinea pig that neurotrophin gene therapy integrated into the cochlear implant improves its performance by stimulating spiral ganglion neurite regeneration. We used the cochlear implant electrode array for novel "close-field" electroporation to transduce mesenchymal cells lining the cochlear perilymphatic canals with a naked complementary DNA gene construct driving expression of brain-derived neurotrophic factor (BDNF) and a green fluorescent protein (GFP) reporter. The focusing of electric fields by particular cochlear implant electrode configurations led to surprisingly efficient gene delivery to adjacent mesenchymal cells. The resulting BDNF expression stimulated regeneration of spiral ganglion neurites, which had atrophied 2 weeks after ototoxic treatment, in a bilateral sensorineural deafness model. In this model, delivery of a control GFP-only vector failed to restore neuron structure, with atrophied neurons indistinguishable from unimplanted cochleae. With BDNF therapy, the regenerated spiral ganglion neurites extended close to the cochlear implant electrodes, with localized ectopic branching. This neural remodeling enabled bipolar stimulation via the cochlear implant array, with low stimulus thresholds and expanded dynamic range of the cochlear nerve, determined via electrically evoked auditory brainstem responses. This development may broadly improve neural interfaces and extend molecular medicine applications.

Predicting social functioning in children with a cochlear implant and in normal-hearing children: the role of emotion regulation.

PubMed

Wiefferink, Carin H; Rieffe, Carolien; Ketelaar, Lizet; Frijns, Johan H M

2012-06-01

The purpose of the present study was to compare children with a cochlear implant and normal hearing children on aspects of emotion regulation (emotion expression and coping strategies) and social functioning (social competence and externalizing behaviors) and the relation between emotion regulation and social functioning. Participants were 69 children with cochlear implants (CI children) and 67 normal hearing children (NH children) aged 1.5-5 years. Parents answered questionnaires about their children's language skills, social functioning, and emotion regulation. Children also completed simple tasks to measure their emotion regulation abilities. Cochlear implant children had fewer adequate emotion regulation strategies and were less socially competent than normal hearing children. The parents of cochlear implant children did not report fewer externalizing behaviors than those of normal hearing children. While social competence in normal hearing children was strongly related to emotion regulation, cochlear implant children regulated their emotions in ways that were unrelated with social competence. On the other hand, emotion regulation explained externalizing behaviors better in cochlear implant children than in normal hearing children. While better language skills were related to higher social competence in both groups, they were related to fewer externalizing behaviors only in cochlear implant children. Our results indicate that cochlear implant children have less adequate emotion-regulation strategies and less social competence than normal hearing children. Since they received their implants relatively recently, they might eventually catch up with their hearing peers. Longitudinal studies should further explore the development of emotion regulation and social functioning in cochlear implant children. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

[The development of musicality in children after cochlear implantation].

PubMed

Zheng, Yan; Liu, Bo; Dong, Ruijuan; Xu, Tianqiu; Chen, Jing; Chen, Xuejing; Zhong, Yan; Meng, Chao; Wang, Hong; Chen, Xueqing

2014-08-01

The purpose of this study is to analyze the development of musicality in children after cochlear implantation, and provide a clinical database for the evaluation of their musicality. Twenty-six children with cochlear implants (CI group) participated in this research. They received cochlear implants at the age of 11 to 68 months with a mean of 35.6 months. Seventy-six infants as a control group aged from 1 to 24 months with a mean of 6.1 months participated in this study, whose hearing were considered normal by passing the case history collection, high-risk registers for hearing loss and hearing screening using DPOAE. The music and young children with CIs: Musicality Rating Scale was used to evaluate their musicality. The evaluation was performed before cochlear implantation and 1, 3, 6, 9, 12, 24 months after cochlear implantation for children with cochlear implants. The evaluation was also performed at 1, 3, 6, 9, 12, 24 months for children with normal hearing. The mean scores of musicality showed significant improvements with time of CI use for CI group (P<0.05). The mean scores of musicality also showed significant improvements with time for control group (P<0.05). There were no significant differences in mean scores between CI group and control group at 1, 3, 6, 9, 12 months of hearing age by rank sum test (P>0.05). Significant difference was noted between the two groups at 24 months (P<0.05). The musicality of children with cochlear implants improved significantly with time after cochlear implantation. The most rapid growth was found in the first year after cochlear implantation.

Cochlear Implants:System Design, Integration and Evaluation

PubMed Central

Rebscher, Stephen; Harrison, William V.; Sun, Xiaoan; Feng, Haihong

2009-01-01

As the most successful neural prosthesis, cochlear implants have provided partial hearing to more than 120,000 persons worldwide; half of which being pediatric users who are able to develop nearly normal language. Biomedical engineers have played a central role in the design, integration and evaluation of the cochlear implant system, but the overall success is a result of collaborative work with physiologists, psychologists, physicians, educators, and entrepreneurs. This review presents broad yet in-depth academic and industrial perspectives on the underlying research and ongoing development of cochlear implants. The introduction accounts for major events and advances in cochlear implants, including dynamic interplays among engineers, scientists, physicians, and policy makers. The review takes a system approach to address critical issues from design and specifications to integration and evaluation. First, the cochlear implant system design and specifications are laid out. Second, the design goals, principles, and methods of the subsystem components are identified from the external speech processor and radio frequency transmission link to the internal receiver, stimulator and electrode arrays. Third, system integration and functional evaluation are presented with respect to safety, reliability, and challenges facing the present and future cochlear implant designers and users. Finally, issues beyond cochlear implants are discussed to address treatment options for the entire spectrum of hearing impairment as well as to use the cochlear implant as a model to design and evaluate other similar neural prostheses such as vestibular and retinal implants. PMID:19946565

Protective role of hydrogen sulfide against noise-induced cochlear damage: a chronic intracochlear infusion model.

PubMed

Li, Xu; Mao, Xiao-Bo; Hei, Ren-Yi; Zhang, Zhi-Bin; Wen, Li-Ting; Zhang, Peng-Zhi; Qiu, Jian-Hua; Qiao, Li

2011-01-01

A reduction in cochlear blood flow plays an essential role in noise-induced hearing loss (NIHL). The timely regulation of cochlear perfusion determines the progression and prognosis of NIHL. Hydrogen sulfide (H(2)S) has attracted increasing interest as a vasodilator in cardiovascular systems. This study identified the role of H(2)S in cochlear blood flow regulation and noise protection. The gene and protein expression of the H(2)S synthetase cystathionine-γ-lyase (CSE) in the rat cochlea was examined using immunofluorescence and real-time PCR. Cochlear CSE mRNA levels varied according to the duration of noise exposure. A chronic intracochlear infusion model was built and artificial perilymph (AP), NaHS or DL-propargylglycine (PPG) were locally administered. Local sodium hydrosulfide (NaHS) significantly increased cochlear perfusion post-noise exposure. Cochlear morphological damage and hearing loss were alleviated in the NaHS group as measured by conventional auditory brainstem response (ABR), cochlear scanning electron microscope (SEM) and outer hair cell (OHC) count. The highest percentage of OHC loss occurred in the PPG group. Our results suggest that H(2)S plays an important role in the regulation of cochlear blood flow and the protection against noise. Further studies may identify a new preventive and therapeutic perspective on NIHL and other blood supply-related inner ear diseases.

Cochlear Patency After Transmastoid Labyrinthectomy for Ménière's Syndrome.

PubMed

Sargent, Eric W; Liao, Eric; Gonda, Roger L

2016-08-01

Labyrinthectomy is considered the "gold standard" in the treatment of intractable vertigo attacks because of Ménière's Disease (MD) but sacrifices all residual hearing. Interest in auditory rehabilitation has lead to cochlear implantation in some patients. Concern remains that the cochlear lumen may fill with tissue or bone after surgery. This study sought to determine the incidence of obliteration of the cochlea after transmastoid labyrinthectomy. Retrospective observational study. Tertiary referral center. Eighteen patients with intractable vertigo from MD who underwent surgery. Transmastoid labyrinthectomy between 2008 and 2013. Cochleas were imaged with unenhanced, heavily T2-weighted magnetic resonance imaging (MRI). Presence of symmetrical cochlear fluid signals on MRI. There was no loss of fluid signal in the cochleas of operated ear compared with the contralateral, unoperated ear in any subject an average of 3 years (standard deviation [SD]: 1.2) after surgery. Five of 18 patients had the vestibule blocked with bone wax at the time of surgery. Blocking the vestibule with bone wax did not change the cochlear fluid signal. The risk of cochlear obstruction after labyrinthectomy for MD is very low. The significance of this finding is that patients with MD who undergo labyrinthectomy will likely remain candidates for cochlear implantation in the labyrinthectomized ear long after surgery if this becomes needed. Immediate cochlear implantation or placement of a cochlear lumen keeper during labyrinthectomy for MD is probably not necessary.

A central factor in pure tone auditory fatigue.

DOT National Transportation Integrated Search

1963-09-01

A long accumulation of psychophysical and physiological evidence indicates that auditory fatigue has its locus of effect in the cochlea; transfer studies with negative or questionable results, and studies of cochlear chemistry and potentials with pos...

Masking of infrared neural stimulation (INS) in hearing and deaf guinea pigs

NASA Astrophysics Data System (ADS)

Kadakia, Sama; Young, Hunter; Richter, Claus-Peter

2013-03-01

Spatial selective infrared neural stimulation has potential to improve neural prostheses, including cochlear implants. The heating of a confined target volume depolarizes the cell membrane and results in an action potential. Tissue heating may also results in thermal damage or the generation of a stress relaxation wave. Stress relaxation waves may result in a direct mechanical stimulation of remaining hair cells in the cochlea, so called optophony. Data are presented that quantify the effect of an acoustical stimulus (noise masker) on the response obtained with INS in normal hearing, acutely deafened, and chronic deaf animals. While in normal hearing animals an acoustic masker can reduce the response to INS, in acutely deafened animals the masking effect is reduced, and in chronic deaf animals this effect has not been detected. The responses to INS remain stable following the different degrees of cochlear damage.

A connection between the Efferent Auditory System and Noise-Induced Tinnitus Generation. Reduced contralateral suppression of TEOAEs in patients with noise-induced tinnitus.

PubMed

Lalaki, Panagiota; Hatzopoulos, Stavros; Lorito, Guiscardo; Kochanek, Krzysztof; Sliwa, Lech; Skarzynski, Henryk

2011-07-01

Subjective tinnitus is an auditory perception that is not caused by external stimulation, its source being anywhere in the auditory system. Furthermore, evidence exists that exposure to noise alters cochlear micromechanics, either directly or through complex feed-back mechanisms, involving the medial olivocochlear efferent system. The aim of this study was to assess the role of the efferent auditory system in noise-induced tinnitus generation. Contralateral sound-activated suppression of TEOAEs was performed in a group of 28 subjects with noise-induced tinnitus (NIT) versus a group of 35 subjects with normal hearing and tinnitus, without any history of exposure to intense occupational or recreational noise (idiopathic tinnitus-IT). Thirty healthy, normally hearing volunteers were used as controls for the efferent suppression test. Suppression of the TEOAE amplitude less than 1 dB SPL was considered abnormal, giving a false positive rate of 6.7%. Eighteen out of 28 (64.3%) patients of the NIT group and 9 out of 35 (25.7%) patients of the IT group showed abnormal suppression values, which were significantly different from the controls' (p<0.0001 and p<0.045, respectively). The abnormal activity of the efferent auditory system in NIT cases might indicate that either the activity of the efferent fibers innervating the outer hair cells (OHCs) is impaired or that the damaged OHCs themselves respond abnormally to the efferent stimulation.

Betahistine metabolites, aminoethylpyridine, and hydroxyethylpyridine increase cochlear blood flow in guinea pigs in vivo.

PubMed

Bertlich, Mattis; Ihler, Fritz; Sharaf, Kariem; Weiss, Bernhard G; Strupp, Michael; Canis, Martin

2014-10-01

Betahistine is a histamine-like drug that is used in the treatment of Ménière's disease. It is commonly believed that betahistine increases cochlear blood flow and thus decreases the endolymphatic hydrops that is the cause of Ménière's. Despite common clinical use, there is little understanding of the kinetics or effects of its metabolites. This study investigated the effect of the betahistine metabolites aminoethylpyridine, hydroxyethylpyridine, and pyridylacetic acid on cochlear microcirculation. Guinea pigs were randomly assigned to one of the groups: placebo, betahistine, or equimolar amounts of aminoethylpyridine, hydroxyethylpyridine, or pyridylacetic acid. Cochlear blood flow and mean arterial pressure were recorded for three minutes before and 15 minutes after treatment. Thirty Dunkin-Hartley guinea pigs assigned to one of five groups with six guinea pigs per group. Betahistine, aminoethylpyridine, and hydroxyethylpyridine caused a significant increase in cochlear blood flow in comparison to placebo. The effect seen under aminoethylpyridin was greatest. The group treated with pyridylacetic acid showed no significant effect on cochlear blood flow. Aminoethylpyridine and hydroxyethylpyridine are, like betahistine, able to increase cochlear blood flow significantly. The effect of aminoethylpyridine was greatest. Pyridylacetic acid had no effect on cochlear microcirculation.

[Cochlear implantation in patients with Waardenburg syndrome type II].

PubMed

Wan, Liangcai; Guo, Menghe; Chen, Shuaijun; Liu, Shuangriu; Chen, Hao; Gong, Jian

2010-05-01

To describe the multi-channel cochlear implantation in patients with Waardenburg syndrome including surgeries, pre and postoperative hearing assessments as well as outcomes of speech recognition. Multi-channel cochlear implantation surgeries have been performed in 12 cases with Waardenburg syndrome type II in our department from 2000 to 2008. All the patients received multi-channel cochlear implantation through transmastoid facial recess approach. The postoperative outcomes of 12 cases were compared with 12 cases with no inner ear malformation as a control group. The electrodes were totally inserted into the cochlear successfully, there was no facial paralysis and cerebrospinal fluid leakage occurred after operation. The hearing threshold in this series were similar to that of the normal cochlear implantation. After more than half a year of speech rehabilitation, the abilities of speech discrimination and spoken language of all the patients were improved compared with that of preoperation. Multi-channel cochlear implantation could be performed in the cases with Waardenburg syndrome, preoperative hearing and images assessments should be done.

[Cochlear implantation through the middle fossa approach].

PubMed

Szyfter, W; Colletti, V; Pruszewicz, A; Kopeć, T; Szymiec, E; Kawczyński, M; Karlik, M

2001-01-01

The inner part of cochlear implant is inserted into inner ear during surgery through mastoid and middle ear. It is a classical method, used in the majority cochlear centers in the world. This is not a suitable method in case of chronic otitis media and middle ear malformation. In these cases Colletti proposed the middle fossa approach and cochlear implant insertion omitting middle ear structures. In patient with bilateral chronic otitis media underwent a few ears operations without obtaining dry postoperative cavity. Cochlear implantation through the middle fossa approach was performed in this patient. The bone fenster was cut, temporal lobe was bent and petrosus pyramid upper surface was exposed. When the superficial petrosal greater nerve, facial nerve and arcuate eminence were localised, the cochlear was open in the basal turn and electrode were inserted. The patient achieves good results in the postoperative speech rehabilitation. It confirmed Colletti tesis that deeper electrode insertion in the cochlear implantation through the middle fossa approach enable use of low and middle frequencies, which are very important in speech understanding.

United Kingdom national paediatric bilateral cochlear implant audit: preliminary results.

PubMed

Cullington, Helen; Bele, Devyanee; Brinton, Julie; Lutman, Mark

2013-11-01

Prior to 2009, United Kingdom (UK) public funding was mainly only available for children to receive unilateral cochlear implants. In 2009, the National Institute for Health and Care Excellence published guidance for cochlear implantation following their review. According to these guidelines, all suitable children are eligible to have simultaneous bilateral cochlear implants or a sequential bilateral cochlear implant if they had received the first before the guidelines were published. Fifteen UK cochlear implant centres formed a consortium to carry out a multi-centre audit. The audit involves collecting data from simultaneously and sequentially implanted children at four intervals: before bilateral cochlear implants or before the sequential implant, 1, 2, and 3 years after bilateral implants. The measures include localization, speech recognition in quiet and background noise, speech production, listening, vocabulary, parental perception, quality of life, and surgical data including complications. The audit has now passed the 2-year point, and data have been received on 850 children. This article provides a first view of some data received up until March 2012.

Musical ear syndrome in adult cochlear implant patients.

PubMed

Low, W-K; Tham, C A; D'Souza, V-D; Teng, S-W

2013-09-01

Except for a single case report, musical ear syndrome in cochlear implantees has not been studied. We aimed to study the prevalence and nature of musical ear syndrome among adult cochlear implant patients, as well as the effect on their emotional well-being. STUDY DESIGN, PATIENTS AND INTERVENTION: A cross-sectional survey of patients aged 18 years and above who had received cochlear implants for profound hearing loss between 1997 and 2010. Of the 82 patients studied, 18 (22 per cent) were found to have experienced musical ear syndrome. Seven and 11 patients had musical ear syndrome prior to and after cochlear implantation, respectively. The character of musical ear syndrome symptoms was described as instrumental music (n = 2), singing (6) or both (10). Fourteen patients reported an adverse emotional effect, with three expressing ‘intolerance’. In this study, 22 per cent of cochlear implantees experienced musical ear syndrome. These symptoms affected patients’ emotional state, but most coped well. Musical ear syndrome can occur prior to and after cochlear implantation.

Large endolymphatic potentials from low-frequency and infrasonic tones in the guinea pig.

PubMed

Salt, Alec N; Lichtenhan, Jeffery T; Gill, Ruth M; Hartsock, Jared J

2013-03-01

Responses of the ear to low-frequency and infrasonic sounds have not been extensively studied. Understanding how the ear responds to low frequencies is increasingly important as environmental infrasounds are becoming more pervasive from sources such as wind turbines. This study shows endolymphatic potentials in the third cochlear turn from acoustic infrasound (5 Hz) are larger than from tones in the audible range (e.g., 50 and 500 Hz), in some cases with peak-to-peak amplitude greater than 20 mV. These large potentials were suppressed by higher-frequency tones and were rapidly abolished by perilymphatic injection of KCl at the cochlear apex, demonstrating their third-turn origins. Endolymphatic iso-potentials from 5 to 500 Hz were enhanced relative to perilymphatic potentials as frequency was lowered. Probe and infrasonic bias tones were used to study the origin of the enhanced potentials. Potentials were best explained as a saturating response summed with a sinusoidal voltage (Vo), that was phase delayed by an average of 60° relative to the biasing effects of the infrasound. Vo is thought to arise indirectly from hair cell activity, such as from strial potential changes caused by sustained current changes through the hair cells in each half cycle of the infrasound.

Audiological follow-up of 24 patients affected by Williams syndrome.

PubMed

Barozzi, Stefania; Soi, Daniela; Spreafico, Emanuela; Borghi, Anna; Comiotto, Elisabetta; Gagliardi, Chiara; Selicorni, Angelo; Forti, Stella; Cesarani, Antonio; Brambilla, Daniele

2013-09-01

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in most patients. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Evaluation of the Normal Cochlear Second Interscalar Ridge Angle and Depth on 3D T2-Weighted Images: A Tool for the Diagnosis of Scala Communis and Incomplete Partition Type II.

PubMed

Booth, T N; Wick, C; Clarke, R; Kutz, J W; Medina, M; Gorsage, D; Xi, Y; Isaacson, B

2018-05-01

Cochlear malformations may be be subtle on imaging studies. The purpose of this study was to evaluate the angle and depth of the lateral second interscalar ridge or notch in ears without sensorineural hearing loss (normal ears) and compare them with ears that have a documented incomplete type II partition malformation. The second interscalar ridge notch angle and depth were measured on MR imaging in normal ears by a single experienced neuroradiologist. The images of normal and incomplete partition II malformation ears were then randomly mixed for 2 novice evaluators to measure both the second interscalar ridge notch angle and depth in a blinded manner. For the mixed group, interobserver agreement was calculated, normal and abnormal ear measurements were compared, and receiver operating characteristic curves were generated. The 94 normal ears had a mean second interscalar ridge angle of 80.86° ± 11.4° and depth of 0.54 ± 0.14 mm with the 98th percentile for an angle of 101° and a depth of 0.3 mm. In the mixed group, agreement between the 2 readers was excellent, with significant differences for angle and depth found between normal and incomplete partition type II ears for angle and depth on average ( P < .001). Receiver operating characteristic cutoffs for delineating normal from abnormal ears were similar for both readers (depth, 0.31/0.34 mm; angle, 114°/104°). A measured angle of >114° and a depth of the second interscalar ridge notch of ≤0.31 mm suggest the diagnosis of incomplete partition type II malformation and scala communis. These measurements can be accurately made by novice readers. © 2018 by American Journal of Neuroradiology.

Importance of cochlear health for implant function.

PubMed

Pfingst, Bryan E; Zhou, Ning; Colesa, Deborah J; Watts, Melissa M; Strahl, Stefan B; Garadat, Soha N; Schvartz-Leyzac, Kara C; Budenz, Cameron L; Raphael, Yehoash; Zwolan, Teresa A

2015-04-01

Amazing progress has been made in providing useful hearing to hearing-impaired individuals using cochlear implants, but challenges remain. One such challenge is understanding the effects of partial degeneration of the auditory nerve, the target of cochlear implant stimulation. Here we review studies from our human and animal laboratories aimed at characterizing the health of the implanted cochlea and the auditory nerve. We use the data on cochlear and neural health to guide rehabilitation strategies. The data also motivate the development of tissue-engineering procedures to preserve or build a healthy cochlea and improve performance obtained by cochlear implant recipients or eventually replace the need for a cochlear implant. This article is part of a Special Issue entitled . Copyright © 2014 Elsevier B.V. All rights reserved.

The influence of cochlear shape on low-frequency hearing.

PubMed

Manoussaki, Daphne; Chadwick, Richard S; Ketten, Darlene R; Arruda, Julie; Dimitriadis, Emilios K; O'Malley, Jen T

2008-04-22

The conventional theory about the snail shell shape of the mammalian cochlea is that it evolved essentially and perhaps solely to conserve space inside the skull. Recently, a theory proposed that the spiral's graded curvature enhances the cochlea's mechanical response to low frequencies. This article provides a multispecies analysis of cochlear shape to test this theory and demonstrates that the ratio of the radii of curvature from the outermost and innermost turns of the cochlear spiral is a significant cochlear feature that correlates strongly with low-frequency hearing limits. The ratio, which is a measure of curvature gradient, is a reflection of the ability of cochlear curvature to focus acoustic energy at the outer wall of the cochlear canal as the wave propagates toward the apex of the cochlea.

Bioinformatic Integration of Molecular Networks and Major Pathways Involved in Mice Cochlear and Vestibular Supporting Cells.

PubMed

Requena, Teresa; Gallego-Martinez, Alvaro; Lopez-Escamez, Jose A

2018-01-01

Background : Cochlear and vestibular epithelial non-hair cells (ENHCs) are the supporting elements of the cellular architecture in the organ of Corti and the vestibular neuroepithelium in the inner ear. Intercellular and cell-extracellular matrix interactions are essential to prevent an abnormal ion redistribution leading to hearing and vestibular loss. The aim of this study is to define the main pathways and molecular networks in the mouse ENHCs. Methods : We retrieved microarray and RNA-seq datasets from mouse epithelial sensory and non-sensory cells from gEAR portal (http://umgear.org/index.html) and obtained gene expression fold-change between ENHCs and non-epithelial cells (NECs) against HCs for each gene. Differentially expressed genes (DEG) with a log2 fold change between 1 and -1 were discarded. The remaining genes were selected to search for interactions using Ingenuity Pathway Analysis and STRING platform. Specific molecular networks for ENHCs in the cochlea and the vestibular organs were generated and significant pathways were identified. Results : Between 1723 and 1559 DEG were found in the mouse cochlear and vestibular tissues, respectively. Six main pathways showed enrichment in the supporting cells in both tissues: (1) "Inhibition of Matrix Metalloproteases"; (2) "Calcium Transport I"; (3) "Calcium Signaling"; (4) "Leukocyte Extravasation Signaling"; (5) "Signaling by Rho Family GTPases"; and (6) "Axonal Guidance Si". In the mouse cochlea, ENHCs showed a significant enrichment in 18 pathways highlighting "axonal guidance signaling (AGS)" ( p = 4.37 × 10 -8 ) and "RhoGDI Signaling" ( p = 3.31 × 10 -8 ). In the vestibular dataset, there were 20 enriched pathways in ENHCs, the most significant being "Leukocyte Extravasation Signaling" ( p = 8.71 × 10 -6 ), "Signaling by Rho Family GTPases" ( p = 1.20 × 10 -5 ) and "Calcium Signaling" ( p = 1.20 × 10 -5 ). Among the top ranked networks, the most biologically significant network contained the "auditory and vestibular system development and function" terms. We also found 108 genes showing tonotopic gene expression in the cochlear ENHCs. Conclusions : We have predicted the main pathways and molecular networks for ENHCs in the organ of Corti and vestibular neuroepithelium. These pathways will facilitate the design of molecular maps to select novel candidate genes for hearing or vestibular loss to conduct functional studies.

Recognition of Speech from the Television with Use of a Wireless Technology Designed for Cochlear Implants.

PubMed

Duke, Mila Morais; Wolfe, Jace; Schafer, Erin

2016-05-01

Cochlear implant (CI) recipients often experience difficulty understanding speech in noise and speech that originates from a distance. Many CI recipients also experience difficulty understanding speech originating from a television. Use of hearing assistance technology (HAT) may improve speech recognition in noise and for signals that originate from more than a few feet from the listener; however, there are no published studies evaluating the potential benefits of a wireless HAT designed to deliver audio signals from a television directly to a CI sound processor. The objective of this study was to compare speech recognition in quiet and in noise of CI recipients with the use of their CI alone and with the use of their CI and a wireless HAT (Cochlear Wireless TV Streamer). A two-way repeated measures design was used to evaluate performance differences obtained in quiet and in competing noise (65 dBA) with the CI sound processor alone and with the sound processor coupled to the Cochlear Wireless TV Streamer. Sixteen users of Cochlear Nucleus 24 Freedom, CI512, and CI422 implants were included in the study. Participants were evaluated in four conditions including use of the sound processor alone and use of the sound processor with the wireless streamer in quiet and in the presence of competing noise at 65 dBA. Speech recognition was evaluated in each condition with two full lists of Computer-Assisted Speech Perception Testing and Training Sentence-Level Test sentences presented from a light-emitting diode television. Speech recognition in noise was significantly better with use of the wireless streamer compared to participants' performance with their CI sound processor alone. There was also a nonsignificant trend toward better performance in quiet with use of the TV Streamer. Performance was significantly poorer when evaluated in noise compared to performance in quiet when the TV Streamer was not used. Use of the Cochlear Wireless TV Streamer designed to stream audio from a television directly to a CI sound processor provides better speech recognition in quiet and in noise when compared to performance obtained with use of the CI sound processor alone. American Academy of Audiology.

Working memory in Farsi-speaking children with normal development and cochlear implant.

PubMed

Soleymani, Zahra; Amidfar, Meysam; Dadgar, Hooshang; Jalaie, Shohre

2014-04-01

Working memory has an important role in language acquisition and development of cognition skills. The ability of encoding, storage and retrieval of phonological codes, as activities of working memory, acquired by audition sense. Children with cochlear implant experience a period that they are not able to perceive sounds. In order to assess the effect of hearing on working memory, we investigated working memory as a cognition skill in children with normal development and cochlear implant. Fifty students with normal hearing and 50 students with cochlear implant aged 5-7 years participated in this study. Children educated in the preschool, the first and second grades. Children with normal development were matched based on age, gender, and grade of education with cochlear implant. Two components of working memory including phonological loop and central executive were compared between two groups. Phonological loop assessed by nonword repetition task and forward digit span. To assess central executive component backward digit span was used. The developmental trend was studied in children with normal development and cochlear implant as well. The effect of age at implantation in children with cochlear implants on components of working memory was investigated. There are significant differences between children with normal development and cochlear implant in all tasks that assess working memory (p < 0.001). The children's age at implantation was negatively correlated with all tasks (p < 0.001). In contrast, duration of usage of cochlear implant set was positively correlated with all tasks (p < 0.001). The comparison of working memory between different grades showed significant differences both in children with normal development and in children with cochlear implant (p < 0.05). These results implied that children with cochlear implant may experience difficulties in working memory. Therefore, these children have problems in encoding, practicing, and repeating phonological units. The results also suggested working memory develops when the child grows up. In cochlear implant children, with decreasing age at implantation and increasing their experience in perceiving sound, working memory skills improved. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Pilot study on the use of data mining to identify cochlear implant candidates.

PubMed

Grisel, Jedidiah J; Schafer, Erin; Lam, Anne; Griffin, Terry

2018-05-01

The goal of this pilot study was to determine the clinical utility of data-mining software that screens for cochlear implant (CI) candidacy. The Auditory Implant Initiative developed a software module that screens for CI candidates via integration with a software system (Noah 4) that serves as a depository for hearing test data. To identify candidates, patient audiograms from one practice were exported into the screening module. Candidates were tracked to determine if any eventually underwent implantation. After loading 4836 audiograms from the Noah 4 system, the screening module identified 558 potential CI candidates. After reviewing the data for the potential candidates, 117 were targeted and invited to an educational event. Following the event, a total of six candidates were evaluated, and two were implanted. This objective approach to identifying candidates has the potential to address the gross underutilization of CIs by removing any bias or lack of knowledge regarding the management of severe to profound sensorineural hearing loss with CIs. The screening module was an effective tool for identifying potential CI candidates at one ENT practice. On a larger scale, the screening module has the potential to impact thousands of CI candidates worldwide.

Lack of financial barriers to pediatric cochlear implantation: impact of socioeconomic status on access and outcomes.

PubMed

Chang, David T; Ko, Alvin B; Murray, Gail S; Arnold, James E; Megerian, Cliff A

2010-07-01

(1) To analyze if socioeconomic status influences access to cochlear implantation in an environment with adequate Medicaid reimbursement. (2) To determine the impact of socioeconomic status on outcomes after unilateral cochlear implantation. Retrospective cohort study. University Hospitals Case Medical Center and Rainbow Babies and Children's Hospital (tertiary referral center), Cleveland, Ohio. Pediatric patients (age range, newborn to 18 years) who received unilateral cochlear implantation during the period 1996 to 2008. Access to cochlear implantation after referral to a cochlear implant center, postoperative complications, compliance with follow-up appointments, and access to sequential bilateral cochlear implantation. A total of 133 pediatric patients were included in this study; 64 were Medicaid-insured patients and 69 were privately insured patients. There was no statistical difference in the odds of initial cochlear implantation, age at referral, or age at implantation between the 2 groups. The odds of prelingual Medicaid-insured patients receiving sequential bilateral cochlear implantation was less than half that of the privately insured group (odds ratio [OR], 0.43; P = .03). The odds of complications in Medicaid-insured children were almost 5-fold greater than the odds for privately insured children (OR, 4.6; P = .03). There were 10 complications in 51 Medicaid-insured patients (19.6%) as opposed to 3 in 61 privately insured patients (4.9%). Medicaid-insured patients missed substantially more follow-up appointments overall (35% vs 23%) and more consecutive visits (1.9 vs 1.1) compared with privately insured patients. In an environment with adequate Medicaid reimbursement, eligible children have equal access to cochlear implantation, regardless of socioeconomic background. However, lower socioeconomic background is associated with higher rates of postoperative complications, worse follow-up compliance, and lower rates of sequential bilateral implantation, observed herein in Medicaid-insured patients. These findings present opportunities for cochlear implant centers to create programs to address such downstream disparities.

Benefit and predictive factors for speech perception outcomes in pediatric bilateral cochlear implant recipients.

PubMed

Chang, Young-Soo; Hong, Sung Hwa; Kim, Eun Yeon; Choi, Ji Eun; Chung, Won-Ho; Cho, Yang-Sun; Moon, Il Joon

2018-05-18

Despite recent advancement in the prediction of cochlear implant outcome, the benefit of bilateral procedures compared to bimodal stimulation and how we predict speech perception outcomes of sequential bilateral cochlear implant based on bimodal auditory performance in children remain unclear. This investigation was performed: (1) to determine the benefit of sequential bilateral cochlear implant and (2) to identify the associated factors for the outcome of sequential bilateral cochlear implant. Observational and retrospective study. We retrospectively analyzed 29 patients with sequential cochlear implant following bimodal-fitting condition. Audiological evaluations were performed; the categories of auditory performance scores, speech perception with monosyllable and disyllables words, and the Korean version of Ling. Audiological evaluations were performed before sequential cochlear implant with the bimodal fitting condition (CI1+HA) and one year after the sequential cochlear implant with bilateral cochlear implant condition (CI1+CI2). The good Performance Group (GP) was defined as follows; 90% or higher in monosyllable and bisyllable tests with auditory-only condition or 20% or higher improvement of the scores with CI1+CI2. Age at first implantation, inter-implant interval, categories of auditory performance score, and various comorbidities were analyzed by logistic regression analysis. Compared to the CI1+HA, CI1+CI2 provided significant benefit in categories of auditory performance, speech perception, and Korean version of Ling results. Preoperative categories of auditory performance scores were the only associated factor for being GP (odds ratio=4.38, 95% confidence interval - 95%=1.07-17.93, p=0.04). The children with limited language development in bimodal condition should be considered as the sequential bilateral cochlear implant and preoperative categories of auditory performance score could be used as the predictor in speech perception after sequential cochlear implant. Copyright © 2018 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Local cochlear damage reduces local nonlinearity and decreases generator-type cochlear emissions while increasing reflector-type emissions.

PubMed

Dong, Wei; Olson, Elizabeth S

2010-03-01

Distortion product otoacoustic emissions (DPOAEs) originate in cochlear nonlinearity and emerge into the ear canal as an apparent sum of emission types, one of which (generator) travels directly out and the other (reflector) travels out following linear reflection. The present study explores intracochlear sources of DPOAEs via simultaneous ear canal and intracochlear pressure measurements in gerbils. A locally damaged cochlea was produced with reduced local intracochlear nonlinearity and significant elevation of the compound action potential thresholds at frequencies represented within the damaged region. In the DPOAE the comparison of healthy to locally damaged cochleae showed the following: (1) In the broad frequency region corresponding to the locally damaged best frequency, DPOAEs evoked by wider f(2)/f(1) stimuli decreased, consistent with the reduction in local nonlinearity. (2) DPOAEs evoked by narrow f(2)/f(1) stimuli often had a bimodal change, decreasing in a lower frequency band and increasing in a band just adjacent and higher, and the DPOAE phase-vs-frequency slope steepened. These changes confirm the complex nature of the DPOAE.

Does the surgical approach in cochlear implantation influence the occurrence of postoperative vertigo?

PubMed

Todt, Ingo; Basta, Dietmar; Ernst, Arne

2008-01-01

To investigate the impact of different cochleostomy techniques on vestibular receptor integrity and vertigo after cochlear implantation. Retrospective cohort study. A total of 62 patients (17 to 84 years of age) underwent implantation via an anterior or round window insertion approach. Two groups of cochlear implant patients were compared with respect to their pre- and postoperative vestibular function and the occurrence of postoperative vertigo. The data were related to the different cochleostomy techniques. The patients were tested by a questionnaire (dizziness handicap inventory, DIH), caloric irrigation (vestibulo-ocular reflex, VOR) for the function of the lateral SCC and by vestibular evoked myogenic potential (VEMP) recordings for saccular function. Significant differences of postoperative VEMP responses (50% vs 13%) and electromystagmography (ENG) results (42.9% vs 9.4%) were found with respect to the 2 different insertion techniques. The number of patients with vertigo after the surgery as evidenced by DHI (23% vs 12.5%) was significantly different. The used round window approach for electrode insertion should be preferred to decrease the risk of loss of vestibular function and the occurrence of vertigo.

LOSS OF SESTRIN 2 POTENTIATES THE EARLY ONSET OF AGE-RELATED SENSORY CELL DEGENERATION IN THE COCHLEA

PubMed Central

ZHANG, CELIA; SUN, WEI; LI, JI; XIONG, BINBIN; FRYE, MITCHELL D.; DING, DALIAN; SALVI, RICHARD; KIM, MI-JUNG; SOMEYA, SHINICHI; HU, BO HUA

2017-01-01

Sestrin 2 (SESN2) is a stress-inducible protein that protects tissues from oxidative stress and delays the aging process. However, its role in maintaining the functional and structural integrity of the cochlea is largely unknown. Here, we report the expression of SESN2 protein in the sensory epithelium, particularly in hair cells. Using C57BL/6J mice, a mouse model of age-related cochlear degeneration, we observed a significant age-related reduction in SESN2 expression in cochlear tissues that was associated with early onset hearing loss and accelerated age-related sensory cell degeneration that progressed from the base toward the apex of the cochlea. Hair cell death occurred by caspase-8 mediated apoptosis. Compared to C57BL/6J control mice, Sesn2 KO mice displayed enhanced expression of proinflammatory genes and activation of basilar membrane macrophages, suggesting that loss of SESN2 function provokes the immune response. Together, these results suggest that Sesn2 plays an important role in cochlear homeostasis and immune responses to stress. PMID:28818524

Focused tight dressing does not prevent cochlear implant magnet migration under 1.5 Tesla MRI.

PubMed

Cuda, D; Murri, A; Succo, G

2013-04-01

We report a retrospective case of inner magnet migration, which occurred after 1.5 Tesla MRI scanning in an adult recipient of a bilateral cochlear implant (CI) despite a focused head dressing. The patient, bilaterally implanted with Nucleus 5 CIs (Cochlear LTD, Sydney, Australia), underwent a 1.5 Tesla cholangio-MRI scan for biliary duct pathology. In subsequent days, a focal skin alteration appeared over the left inner coil. Plain skull radiographs showed partial magnet migration on the left side. Surgical exploration confirmed magnet twisting; the magnet was effectively repositioned. Left CI performance was restored to pre-migration level. The wound healed without complications. Thus, focused dressing does not prevent magnet migration in CI recipients undergoing 1.5 Tesla MRI. All patients should be counselled on this potential complication. A minor surgical procedure is required to reposition the magnet. Nevertheless, timely diagnosis is necessary to prevent skin breakdown and subsequent device contamination. Plain skull radiograph is very effective in identifying magnet twisting; it should be performed systematically after MRI or minimally on all suspected cases.

A comparison of speech intonation production and perception abilities of Farsi speaking cochlear implanted and normal hearing children.

PubMed

Moein, Narges; Khoddami, Seyyedeh Maryam; Shahbodaghi, Mohammad Rahim

2017-10-01

Cochlear implant prosthesis facilitates spoken language development and speech comprehension in children with severe-profound hearing loss. However, this prosthesis is limited in encoding information about fundamental frequency and pitch that are essentially for recognition of speech prosody. The purpose of the present study is to investigate the perception and production of intonation in cochlear implant children and comparison with normal hearing children. This study carried out on 25 cochlear implanted children and 50 children with normal hearing. First, using 10 action pictures statements and questions sentences were extracted. Fundamental frequency and pitch changes were identified using Praat software. Then, these sentences were judged by 7 adult listeners. In second stage 20 sentences were played for child and he/she determined whether it was in a question form or statement one. Performance of cochlear implanted children in perception and production of intonation was significantly lower than children with normal hearing. The difference between fundamental frequency and pitch changes in cochlear implanted children and children with normal hearing was significant (P < 0/05). Cochlear implanted children performance in perception and production of intonation has significant correlation with child's age surgery and duration of prosthesis use (P < 0/05). The findings of the current study show that cochlear prostheses have limited application in facilitating the perception and production of intonation in cochlear implanted children. It should be noted that the child's age at the surgery and duration of prosthesis's use is important in reduction of this limitation. According to these findings, speech and language pathologists should consider intervention of intonation in treatment program of cochlear implanted children. Copyright © 2017 Elsevier B.V. All rights reserved.

The Role of MRI in Diagnosing Neurovascular Compression of the Cochlear Nerve Resulting in Typewriter Tinnitus.

PubMed

Bae, Y J; Jeon, Y J; Choi, B S; Koo, J-W; Song, J-J

2017-06-01

Typewriter tinnitus, a symptom characterized by paroxysmal attacks of staccato sounds, has been thought to be caused by neurovascular compression of the cochlear nerve, but the correlation between radiologic evidence of neurovascular compression of the cochlear nerve and symptom presentation has not been thoroughly investigated. The purpose of this study was to examine whether radiologic evidence of neurovascular compression of the cochlear nerve is pathognomonic in typewriter tinnitus. Fifteen carbamazepine-responding patients with typewriter tinnitus and 8 control subjects were evaluated with a 3D T2-weighted volume isotropic turbo spin-echo acquisition sequence. Groups 1 (16 symptomatic sides), 2 (14 asymptomatic sides), and 3 (16 control sides) were compared with regard to the anatomic relation between the vascular loop and the internal auditory canal and the presence of neurovascular compression of the cochlear nerve with/without angulation/indentation. The anatomic location of the vascular loop was not significantly different among the 3 groups (all, P > .05). Meanwhile, neurovascular compression of the cochlear nerve on MR imaging was significantly higher in group 1 than in group 3 ( P = .032). However, considerable false-positive (no symptoms with neurovascular compression of the cochlear nerve on MR imaging) and false-negative (typewriter tinnitus without demonstrable neurovascular compression of the cochlear nerve) findings were also observed. Neurovascular compression of the cochlear nerve was more frequently detected on the symptomatic side of patients with typewriter tinnitus compared with the asymptomatic side of these patients or on both sides of control subjects on MR imaging. However, considering false-positive and false-negative findings, meticulous history-taking and the response to the initial carbamazepine trial should be regarded as more reliable diagnostic clues than radiologic evidence of neurovascular compression of the cochlear nerve. © 2017 by American Journal of Neuroradiology.

Trends in cochlear implants.

PubMed

Zeng, Fan-Gang

2004-01-01

More than 60,000 people worldwide use cochlear implants as a means to restore functional hearing. Although individual performance variability is still high, an average implant user can talk on the phone in a quiet environment. Cochlear-implant research has also matured as a field, as evidenced by the exponential growth in both the patient population and scientific publication. The present report examines current issues related to audiologic, clinical, engineering, anatomic, and physiologic aspects of cochlear implants, focusing on their psychophysical, speech, music, and cognitive performance. This report also forecasts clinical and research trends related to presurgical evaluation, fitting protocols, signal processing, and postsurgical rehabilitation in cochlear implants. Finally, a future landscape in amplification is presented that requires a unique, yet complementary, contribution from hearing aids, middle ear implants, and cochlear implants to achieve a total solution to the entire spectrum of hearing loss treatment and management.

Response Properties of Cochlear Nucleus Neurons in Monkeys

PubMed Central

Roth, G. Linn; Recio, A.

2009-01-01

Much of what is known about how the cochlear nuclei participate in mammalian hearing comes from studies of non-primate mammalian species. To determine to what extent the cochlear nuclei of primates resemble those of other mammalian orders, we have recorded responses to sound in three primate species: marmosets, Cynomolgus macaques, and squirrel monkeys. These recordings show that the same types of temporal firing patterns are found in primates that have been described in other mammals. Responses to tones of neurons in the ventral cochlear nucleus have similar tuning, latencies, post-stimulus time and interspike interval histograms as those recorded in non-primate cochlear nucleus neurons. In the dorsal cochlear nucleus, too, responses were similar. From these results it is evident that insights gained from non-primate studies can be applied to the peripheral auditory system of primates. PMID:19531377

Comparison of Social Interaction between Cochlear-Implanted Children with Normal Intelligence Undergoing Auditory Verbal Therapy and Normal-Hearing Children: A Pilot Study.

PubMed

Monshizadeh, Leila; Vameghi, Roshanak; Sajedi, Firoozeh; Yadegari, Fariba; Hashemi, Seyed Basir; Kirchem, Petra; Kasbi, Fatemeh

2018-04-01

A cochlear implant is a device that helps hearing-impaired children by transmitting sound signals to the brain and helping them improve their speech, language, and social interaction. Although various studies have investigated the different aspects of speech perception and language acquisition in cochlear-implanted children, little is known about their social skills, particularly Persian-speaking cochlear-implanted children. Considering the growing number of cochlear implants being performed in Iran and the increasing importance of developing near-normal social skills as one of the ultimate goals of cochlear implantation, this study was performed to compare the social interaction between Iranian cochlear-implanted children who have undergone rehabilitation (auditory verbal therapy) after surgery and normal-hearing children. This descriptive-analytical study compared the social interaction level of 30 children with normal hearing and 30 with cochlear implants who were conveniently selected. The Raven test was administered to the both groups to ensure normal intelligence quotient. The social interaction status of both groups was evaluated using the Vineland Adaptive Behavior Scale, and statistical analysis was performed using Statistical Package for Social Sciences (SPSS) version 21. After controlling age as a covariate variable, no significant difference was observed between the social interaction scores of both the groups (p > 0.05). In addition, social interaction had no correlation with sex in either group. Cochlear implantation followed by auditory verbal rehabilitation helps children with sensorineural hearing loss to have normal social interactions, regardless of their sex.

Effects of residual hearing on cochlear implant outcomes in children: A systematic-review.

PubMed

Chiossi, Julia Santos Costa; Hyppolito, Miguel Angelo

2017-09-01

to investigate if preoperative residual hearing in prelingually deafened children can interfere on cochlear implant indication and outcomes. a systematic-review was conducted in five international databases up to November-2016, to locate articles that evaluated cochlear implantation in children with some degree of preoperative residual hearing. Outcomes were auditory, language and cognition performances after cochlear implant. The quality of the studies was assessed and classified according to the Oxford Levels of Evidence table - 2011. Risk of biases were also described. From the 30 articles reviewed, two types of questions were identified: (a) what are the benefits of cochlear implantation in children with residual hearing? (b) is the preoperative residual hearing a predictor of cochlear implant outcome? Studies ranged from 04 to 188 subjects, evaluating populations between 1.8 and 10.3 years old. The definition of residual hearing varied between studies. The majority of articles (n = 22) evaluated speech perception as the outcome and 14 also assessed language and speech production. There is evidence that cochlear implant is beneficial to children with residual hearing. Preoperative residual hearing seems to be valuable to predict speech perception outcomes after cochlear implantation, even though the mechanism of how it happens is not clear. More extensive researches must be conducted in order to make recommendations and to set prognosis for cochlear implants based on children preoperative residual hearing. Copyright © 2017 Elsevier B.V. All rights reserved.

Use of suprathreshold stochastic resonance in cochlear implant coding

NASA Astrophysics Data System (ADS)

Allingham, David; Stocks, Nigel G.; Morse, Robert P.

2003-05-01

In this article we discuss the possible use of a novel form of stochastic resonance, termed suprathreshold stochastic resonance (SSR), to improve signal encoding/transmission in cochlear implants. A model, based on the leaky-integrate-and-fire (LIF) neuron, has been developed from physiological data and use to model information flow in a population of cochlear nerve fibers. It is demonstrated that information flow can, in principle, be enhanced by the SSR effect. Furthermore, SSR was found to enhance information transmission for signal parameters that are commonly encountered in cochlear implants. This, therefore, gives hope that SSR may be implemented in cochlear implants to improve speech comprehension.

Headbobber: A Combined Morphogenetic and Cochleosaccular Mouse Model to Study 10qter Deletions in Human Deafness

PubMed Central

Buniello, Annalisa; Hardisty-Hughes, Rachel E.; Pass, Johanna C.; Bober, Eva; Smith, Richard J.; Steel, Karen P.

2013-01-01

The recessive mouse mutant headbobber (hb) displays the characteristic behavioural traits associated with vestibular defects including headbobbing, circling and deafness. This mutation was caused by the insertion of a transgene into distal chromosome 7 affecting expression of native genes. We show that the inner ear of hb/hb mutants lacks semicircular canals and cristae, and the saccule and utricle are fused together in a single utriculosaccular sac. Moreover, we detect severe abnormalities of the cochlear sensory hair cells, the stria vascularis looks severely disorganised, Reissner's membrane is collapsed and no endocochlear potential is detected. Myo7a and Kcnj10 expression analysis show a lack of the melanocyte-like intermediate cells in hb/hb stria vascularis, which can explain the absence of endocochlear potential. We use Trp2 as a marker of melanoblasts migrating from the neural crest at E12.5 and show that they do not interdigitate into the developing strial epithelium, associated with abnormal persistence of the basal lamina in the hb/hb cochlea. We perform array CGH, deep sequencing as well as an extensive expression analysis of candidate genes in the headbobber region of hb/hb and littermate controls, and conclude that the headbobber phenotype is caused by: 1) effect of a 648 kb deletion on distal Chr7, resulting in the loss of three protein coding genes (Gpr26, Cpmx2 and Chst15) with expression in the inner ear but unknown function; and 2) indirect, long range effect of the deletion on the expression of neighboring genes on Chr7, associated with downregulation of Hmx3, Hmx2 and Nkx1.2 homeobox transcription factors. Interestingly, deletions of the orthologous region in humans, affecting the same genes, have been reported in nineteen patients with common features including sensorineural hearing loss and vestibular problems. Therefore, we propose that headbobber is a useful model to gain insight into the mechanisms underlying deafness in human 10qter deletion syndrome. PMID:23457544

Round-window delivery of neurotrophin 3 regenerates cochlear synapses after acoustic overexposure.

PubMed

Suzuki, Jun; Corfas, Gabriel; Liberman, M Charles

2016-04-25

In acquired sensorineural hearing loss, such as that produced by noise or aging, there can be massive loss of the synaptic connections between cochlear sensory cells and primary sensory neurons, without loss of the sensory cells themselves. Because the cell bodies and central projections of these cochlear neurons survive for months to years, there is a long therapeutic window in which to re-establish functional connections and improve hearing ability. Here we show in noise-exposed mice that local delivery of neurotrophin-3 (NT-3) to the round window niche, 24 hours after an exposure that causes an immediate loss of up to 50% loss of synapses in the cochlear basal region, can regenerate pre- and post-synaptic elements at the hair cell / cochlear nerve interface. This synaptic regeneration, as documented by confocal microscopy of immunostained cochlear sensory epithelia, was coupled with a corresponding functional recovery, as seen in the suprathreshold amplitude of auditory brainstem response Wave 1. Cochlear delivery of neurotrophins in humans is likely achievable as an office procedure via transtympanic injection, making our results highly significant in a translational context.

Stronger efferent suppression of cochlear neural potentials by contralateral acoustic stimulation in awake than in anesthetized chinchilla.

PubMed

Aedo, Cristian; Tapia, Eduardo; Pavez, Elizabeth; Elgueda, Diego; Delano, Paul H; Robles, Luis

2015-01-01

There are two types of sensory cells in the mammalian cochlea, inner hair cells, which make synaptic contact with auditory-nerve afferent fibers, and outer hair cells that are innervated by crossed and uncrossed medial olivocochlear (MOC) efferent fibers. Contralateral acoustic stimulation activates the uncrossed efferent MOC fibers reducing cochlear neural responses, thus modifying the input to the central auditory system. The chinchilla, among all studied mammals, displays the lowest percentage of uncrossed MOC fibers raising questions about the strength and frequency distribution of the contralateral-sound effect in this species. On the other hand, MOC effects on cochlear sensitivity have been mainly studied in anesthetized animals and since the MOC-neuron activity depends on the level of anesthesia, it is important to assess the influence of anesthesia in the strength of efferent effects. Seven adult chinchillas (Chinchilla laniger) were chronically implanted with round-window electrodes in both cochleae. We compared the effect of contralateral sound in awake and anesthetized condition. Compound action potentials (CAP) and cochlear microphonics (CM) were measured in the ipsilateral cochlea in response to tones in absence and presence of contralateral sound. Control measurements performed after middle-ear muscles section in one animal discarded any possible middle-ear reflex activation. Contralateral sound produced CAP amplitude reductions in all chinchillas, with suppression effects greater by about 1-3 dB in awake than in anesthetized animals. In contrast, CM amplitude increases of up to 1.9 dB were found in only three awake chinchillas. In both conditions the strongest efferent effects were produced by contralateral tones at frequencies equal or close to those of ipsilateral tones. Contralateral CAP suppressions for 1-6 kHz ipsilateral tones corresponded to a span of uncrossed MOC fiber innervation reaching at least the central third of the chinchilla cochlea.

Stochastic information transfer from cochlear implant electrodes to auditory nerve fibers

NASA Astrophysics Data System (ADS)

Gao, Xiao; Grayden, David B.; McDonnell, Mark D.

2014-08-01

Cochlear implants, also called bionic ears, are implanted neural prostheses that can restore lost human hearing function by direct electrical stimulation of auditory nerve fibers. Previously, an information-theoretic framework for numerically estimating the optimal number of electrodes in cochlear implants has been devised. This approach relies on a model of stochastic action potential generation and a discrete memoryless channel model of the interface between the array of electrodes and the auditory nerve fibers. Using these models, the stochastic information transfer from cochlear implant electrodes to auditory nerve fibers is estimated from the mutual information between channel inputs (the locations of electrodes) and channel outputs (the set of electrode-activated nerve fibers). Here we describe a revised model of the channel output in the framework that avoids the side effects caused by an "ambiguity state" in the original model and also makes fewer assumptions about perceptual processing in the brain. A detailed comparison of how different assumptions on fibers and current spread modes impact on the information transfer in the original model and in the revised model is presented. We also mathematically derive an upper bound on the mutual information in the revised model, which becomes tighter as the number of electrodes increases. We found that the revised model leads to a significantly larger maximum mutual information and corresponding number of electrodes compared with the original model and conclude that the assumptions made in this part of the modeling framework are crucial to the model's overall utility.

A comparison between neural response telemetry via cochleostomy or the round window approach in cochlear implantation.

PubMed

Hamerschmidt, Rogério; Schuch, Luiz Henrique; Rezende, Rodrigo Kopp; Wiemes, Gislaine Richter Minhoto; Oliveira, Adriana Kosma Pires de; Mocellin, Marcos

2012-01-01

There are two techniques for cochlear implant (CI) electrode placement: cochleostomy and the round window (RW) approach. This study aims to compare neural response telemetry (NRT) results immediately after surgery to check for possible differences on auditory nerve stimulation between these two techniques. This is a prospective cross-sectional study. Twenty-three patients were enrolled. Six patients underwent surgery by cochleostomy and 17 had it through the RW approach. Mean charge units (MCU) for high frequency sounds: patients submitted to the RW approach had a mean value of 190.4 (± 29.2) while cochleostomy patients averaged 187.8 (± 32.7); p = 0.71. MCU for mid frequency sounds: patients submitted to the RW approach had a mean value of 192.5 (± 22) while cochleostomy patients averaged 178.5 (± 18.5); p = 0.23. MCU for low frequency sounds: patients submitted to the RW approach had a mean value of 183.3 (± 25) while cochleostomy patients averaged 163.8 (± 19.3); p = 0.19. This study showed no differences in the action potential of the distal portion of the auditory nerve in patients with multichannel cochlear implants submitted to surgery by cochleostomy or through the RW approach, using the implant itself to generate stimuli and record responses. Both techniques equally stimulate the cochlear nerve. Therefore, the choice of approach can be made based on the surgeon's own preference and experience.

Differential distribution of adenosine receptors in rat cochlea.

PubMed

Vlajkovic, Srdjan M; Abi, Shukri; Wang, Carol J H; Housley, Gary D; Thorne, Peter R

2007-06-01

Adenosine is a constitutive cell metabolite that can be released from cells via specific bi-directional transporters and is an end-point for nucleotide hydrolysis. In the extracellular space, adenosine becomes a signalling molecule for P1 (adenosine) receptors that modulate physiological responses in a wide range of mammalian tissues. Whereas adenosine signalling has been implicated in the regulation of cochlear blood flow and in cochlear protection from oxidative damage, the potential roles for adenosine signalling in the modulation of sound transduction and auditory neurotransmission have not been established. We have characterised the expression and distribution of adenosine receptors in the rat cochlea. mRNA transcripts for all four subtypes of adenosine receptors (A(1), A(2A), A(2B) and A(3)) were detected in dissected cochlear tissue by using reverse transcription/polymerase chain reaction analysis. The protein distribution for the A(1), A(2A) and A(3) receptor subtypes was identified by immunoperoxidase histochemistry and confocal immunofluorescence labelling. These receptors were differentially expressed in the organ of Corti, spiral ganglion neurones, lateral wall tissues and cochlear blood vessels. The distribution of adenosine receptors in sensory and neural tissues and in the vasculature coincided with other elements of purinergic signalling (P2X and P2Y receptors, ectonucleotidases), consistent with the integrative regulation of many physiological processes in the cochlea by extracellular nucleotides and nucleosides. Our study provides a framework for further investigation of adenosine signalling in the inner ear, including putative roles in oxidative stress responses.

Probabilistic Tractography of the Cranial Nerves in Vestibular Schwannoma.

PubMed

Zolal, Amir; Juratli, Tareq A; Podlesek, Dino; Rieger, Bernhard; Kitzler, Hagen H; Linn, Jennifer; Schackert, Gabriele; Sobottka, Stephan B

2017-11-01

Multiple recent studies have reported on diffusion tensor-based fiber tracking of cranial nerves in vestibular schwannoma, with conflicting results as to the accuracy of the method and the occurrence of cochlear nerve depiction. Probabilistic nontensor-based tractography might offer advantages in terms of better extraction of directional information from the underlying data in cranial nerves, which are of subvoxel size. Twenty-one patients with large vestibular schwannomas were recruited. The probabilistic tracking was run preoperatively and the position of the potential depictions of the facial and cochlear nerves was estimated postoperatively by 3 independent observers in a blinded fashion. The true position of the nerve was determined intraoperatively by the surgeon. Thereafter, the imaging-based estimated position was compared with the intraoperatively determined position. Tumor size, cystic appearance, and postoperative House-Brackmann score were analyzed with regard to the accuracy of the depiction of the nerves. The probabilistic tracking showed a connection that correlated to the position of the facial nerve in 81% of the cases and to the position of the cochlear nerve in 33% of the cases. Altogether, the resulting depiction did not correspond to the intraoperative position of any of the nerves in 3 cases. In a majority of cases, the position of the facial nerve, but not of the cochlear nerve, could be estimated by evaluation of the probabilistic tracking results. However, false depictions not corresponding to any nerve do occur and cannot be discerned as such from the image only. Copyright © 2017 Elsevier Inc. All rights reserved.

Auditory Midbrain Implant: Research and Development Towards a Second Clinical Trial

PubMed Central

Lim, Hubert H.; Lenarz, Thomas

2015-01-01

The cochlear implant is considered one of the most successful neural prostheses to date, which was made possible by visionaries who continued to develop the cochlear implant through multiple technological and clinical challenges. However, patients without a functional auditory nerve or implantable cochlea cannot benefit from a cochlear implant. The focus of the paper is to review the development and translation of a new type of central auditory prosthesis for this group of patients, which is known as the auditory midbrain implant (AMI) and is designed for electrical stimulation within the inferior colliculus. The rationale and results for the first AMI clinical study using a multi-site single-shank array will be presented initially. Although the AMI has achieved encouraging results in terms of safety and improvements in lip-reading capabilities and environmental awareness, it has not yet provided sufficient speech perception. Animal and human data will then be presented to show that a two-shank AMI array can potentially improve hearing performance by targeting specific neurons of the inferior colliculus. Modifications to the AMI array design, stimulation strategy, and surgical approach have been made that are expected to improve hearing performance in the patients implanted with a two-shank array in an upcoming clinical trial funded by the National Institutes of Health. Positive outcomes from this clinical trial will motivate new efforts and developments toward improving central auditory prostheses for those who cannot sufficiently benefit from cochlear implants. PMID:25613994

Prevention of Noise Damage to Cochlear Synapses

DTIC Science & Technology

2015-10-01

AWARD NUMBER: W81XWH-14-1-0494 TITLE: Prevention of Noise Damage to Cochlear Synapses PRINCIPAL INVESTIGATOR: Steven Green CONTRACTING...to Cochlear Synapses 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-14-1-0494 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Steven Green 5d. PROJECT...ABSTRACT Noise-induced synaptopathy is the result of excitotoxic trauma to cochlear synapses due to glutamate released from the hair cells. Excitotoxic

Scala vestibuli cochlear implantation in patients with partially ossified cochleas.

PubMed

Berrettini, Stefano; Forli, Francesca; Neri, Emanuele; Segnini, Giovanni; Franceschini, Stefano Sellari

2002-11-01

Partial cochlear obstruction is a relatively common finding in candidates for cochlear implants and frequently involves the inferior segment of the scala tympani in the basal turn of the cochlea. In such patients, the scala vestibuli is often patent and offers an alternative site for implantation. The current report describes two patients with such partial obstruction of the inferior segment of the basal cochlear turn, caused in one case by systemic vasculitis (Takayasu's disease) and in the other by obliterative otosclerosis. A scala vestibuli implantation allowed for complete insertion of the electrode array. No problems were encountered during the surgical procedures and the good post-operative hearing and communicative outcomes achieved were similar to those reported in patients without cochlear ossification. The importance of accurate pre-operative radiological study of the inner ear is underscored, to disclose the presence and define the features of the cochlear ossification and ultimately to properly plan the surgical approach.

Hearing rehabilitation with a binaural cochlear implant in a patient with Erdheim-Chester disease.

PubMed

Querat, Charlotte; Thai-Van, Hung; Durand, Denis Vital; Cotton, François; Gallego, Stéphane; Truy, Eric

2015-09-01

Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis. This paper reports an exceptional case of bilateral neural involvement, responsible for profound hearing loss. Bilateral cochlear implantation was performed. We present a 57-year-old man affected by ECD with profound bilateral hearing loss. The patient underwent cochlear implantation with a binaural Digisonic(®) cochlear implant, 7 years after the initial diagnosis. Speech intelligibility rose to a plateau after about 6 months of cochlear implant use. The average outcome of speech intelligibility over time was 55% for dissyllabic words without lip reading, and 70% for sentences. Perception score decreased before the patient died from ECD. A description of the ECD and its otological manifestations is presented. This paper reports the effective hearing rehabilitation of profound bilateral hearing loss by the means of a binaural Digisonic(®) cochlear implant.

Spiral Form of the Human Cochlea Results from Spatial Constraints.

PubMed

Pietsch, M; Aguirre Dávila, L; Erfurt, P; Avci, E; Lenarz, T; Kral, A

2017-08-08

The human inner ear has an intricate spiral shape often compared to shells of mollusks, particularly to the nautilus shell. It has inspired many functional hearing theories. The reasons for this complex geometry remain unresolved. We digitized 138 human cochleae at microscopic resolution and observed an astonishing interindividual variability in the shape. A 3D analytical cochlear model was developed that fits the analyzed data with high precision. The cochlear geometry neither matched a proposed function, namely sound focusing similar to a whispering gallery, nor did it have the form of a nautilus. Instead, the innate cochlear blueprint and its actual ontogenetic variants were determined by spatial constraints and resulted from an efficient packing of the cochlear duct within the petrous bone. The analytical model predicts well the individual 3D cochlear geometry from few clinical measures and represents a clinical tool for an individualized approach to neurosensory restoration with cochlear implants.

Trends in Cochlear Implants

PubMed Central

Zeng, Fan-Gang

2004-01-01

More than 60,000 people worldwide use cochlear implants as a means to restore functional hearing. Although individual performance variability is still high, an average implant user can talk on the phone in a quiet environment. Cochlear-implant research has also matured as a field, as evidenced by the exponential growth in both the patient population and scientific publication. The present report examines current issues related to audiologic, clinical, engineering, anatomic, and physiologic aspects of cochlear implants, focusing on their psychophysical, speech, music, and cognitive performance. This report also forecasts clinical and research trends related to presurgical evaluation, fitting protocols, signal processing, and postsurgical rehabilitation in cochlear implants. Finally, a future landscape in amplification is presented that requires a unique, yet complementary, contribution from hearing aids, middle ear implants, and cochlear implants to achieve a total solution to the entire spectrum of hearing loss treatment and management. PMID:15247993

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.

PubMed

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

PubMed Central

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome. PMID:27376080

A Preliminary Investigation of the Air-Bone Gap: Changes in Intracochlear Sound Pressure With Air- and Bone-conducted Stimuli After Cochlear Implantation.

PubMed

Banakis Hartl, Renee M; Mattingly, Jameson K; Greene, Nathaniel T; Jenkins, Herman A; Cass, Stephen P; Tollin, Daniel J

2016-10-01

A cochlear implant electrode within the cochlea contributes to the air-bone gap (ABG) component of postoperative changes in residual hearing after electrode insertion. Preservation of residual hearing after cochlear implantation has gained importance as simultaneous electric-acoustic stimulation allows for improved speech outcomes. Postoperative loss of residual hearing has previously been attributed to sensorineural changes; however, presence of increased postoperative ABG remains unexplained and could result in part from altered cochlear mechanics. Here, we sought to investigate changes to these mechanics via intracochlear pressure measurements before and after electrode implantation to quantify the contribution to postoperative ABG. Human cadaveric heads were implanted with titanium fixtures for bone conduction transducers. Velocities of stapes capitulum and cochlear promontory between the two windows were measured using single-axis laser Doppler vibrometry and fiber-optic sensors measured intracochlear pressures in scala vestibuli and tympani for air- and bone-conducted stimuli before and after cochlear implant electrode insertion through the round window. Intracochlear pressures revealed only slightly reduced responses to air-conducted stimuli consistent with previous literature. No significant changes were noted to bone-conducted stimuli after implantation. Velocities of the stapes capitulum and the cochlear promontory to both stimuli were stable after electrode placement. Presence of a cochlear implant electrode causes alterations in intracochlear sound pressure levels to air, but not bone, conducted stimuli and helps to explain changes in residual hearing noted clinically. These results suggest the possibility of a cochlear conductive component to postoperative changes in hearing sensitivity.

SOBP Is Mutated in Syndromic and Nonsyndromic Intellectual Disability and Is Highly Expressed in the Brain Limbic System

PubMed Central

Birk, Efrat; Har-Zahav, Adi; Manzini, Chiara M.; Pasmanik-Chor, Metsada; Kornreich, Liora; Walsh, Christopher A.; Noben-Trauth, Konrad; Albin, Adi; Simon, Amos J.; Colleaux, Laurence; Morad, Yair; Rainshtein, Limor; Tischfield, David J.; Wang, Peter; Magal, Nurit; Maya, Idit; Shoshani, Noa; Rechavi, Gideon; Gothelf, Doron; Maydan, Gal; Shohat, Mordechai; Basel-Vanagaite, Lina

2010-01-01

Intellectual disability (ID) affects 1%–3% of the general population. We recently reported on a family with autosomal-recessive mental retardation with anterior maxillary protrusion and strabismus (MRAMS) syndrome. One of the reported patients with ID did not have dysmorphic features but did have temporal lobe epilepsy and psychosis. We report on the identification of a truncating mutation in the SOBP that is responsible for causing both syndromic and nonsyndromic ID in the same family. The protein encoded by the SOBP, sine oculis binding protein ortholog, is a nuclear zinc finger protein. In mice, Sobp (also known as Jxc1) is critical for patterning of the organ of Corti; one of our patients has a subclinical cochlear hearing loss but no gross cochlear abnormalities. In situ RNA expression studies in postnatal mouse brain showed strong expression in the limbic system at the time interval of active synaptogenesis. The limbic system regulates learning, memory, and affective behavior, but limbic circuitry expression of other genes mutated in ID is unusual. By comparing the protein content of the +/jc to jc/jc mice brains with the use of proteomics, we detected 24 proteins with greater than 1.5-fold differences in expression, including two interacting proteins, dynamin and pacsin1. This study shows mutated SOBP involvement in syndromic and nonsyndromic ID with psychosis in humans. PMID:21035105

Cochlear implants in Waardenburg syndrome.

PubMed

Cullen, Robert D; Zdanski, Carlton; Roush, Patricia; Brown, Carolyn; Teagle, Holly; Pillsbury, Harold C; Buchman, Craig

2006-07-01

Waardenburg syndrome is an autosomal-dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation. Pediatric cochlear implant recipients with documented evidence of Waardenburg syndrome underwent retrospective case review. All patients received their cochlear implants at the study institution followed by outpatient auditory habilitation. Charts were reviewed for etiology and duration of deafness, age at time of cochlear implantation, perioperative complications, duration of use, and performance outcomes. Results of standard tests batteries for speech perception and production administered as a part of the patients' auditory habilitation were reviewed. Seven patients with Waardenburg syndrome and cochlear implants were identified. The average age at implantation was 37 months (range, 18-64 months) and the average duration of use was 69 months (range, 12-143 months). All of these patients are active users of their devices and perform very well after implantation. There were no major complications in this small group of patients. Children with congenital sensorineural hearing loss without other comorbidities (e.g., developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. Patients with Waardenburg syndrome can be expected to have above-average performance after cochlear implantation.

Human neural tuning estimated from compound action potentials in normal hearing human volunteers

NASA Astrophysics Data System (ADS)

Verschooten, Eric; Desloovere, Christian; Joris, Philip X.

2015-12-01

The sharpness of cochlear frequency tuning in humans is debated. Evoked otoacoustic emissions and psychophysical measurements suggest sharper tuning in humans than in laboratory animals [15], but this is disputed based on comparisons of behavioral and electrophysiological measurements across species [14]. Here we used evoked mass potentials to electrophysiologically quantify tuning (Q10) in humans. We combined a notched noise forward masking paradigm [9] with the recording of trans tympanic compound action potentials (CAP) from masked probe tones in awake human and anesthetized monkey (Macaca mulatta). We compare our results to data obtained with the same paradigm in cat and chinchilla [16], and find that CAP-Q10values in human are ˜1.6x higher than in cat and chinchilla and ˜1.3x higher than in monkey. To estimate frequency tuning of single auditory nerve fibers (ANFs) in humans, we derive conversion functions from ANFs in cat, chinchilla, and monkey and apply these to the human CAP measurements. The data suggest that sharp cochlear tuning is a feature of old-world primates.

Surgical Management of the Pediatric Cochlear Implant Patient.

ERIC Educational Resources Information Center

Cohen, Seth M.; Haynes, David S.

2003-01-01

This article discusses the surgical management of children receiving cochlear implants. It identifies preoperative considerations to select patients likely to benefit, contraindications, some new surgical techniques, complications, special considerations (otitis media, meningitis, head growth, inner ear malformations, and cochlear obstruction).…

Benefits of Localization and Speech Perception with Multiple Noise Sources in Listeners with a Short-electrode Cochlear Implant

PubMed Central

Dunn, Camille C.; Perreau, Ann; Gantz, Bruce; Tyler, Richard

2009-01-01

Background Research suggests that for individuals with significant low-frequency hearing, implantation of a short-electrode cochlear implant may provide benefits of improved speech perception abilities. Because this strategy combines acoustic and electrical hearing within the same ear while at the same time preserving low-frequency residual acoustic hearing in both ears, localization abilities may also be improved. However, very little research has focused on the localization and spatial hearing abilities of users with a short-electrode cochlear implant. Purpose The purpose of this study was to evaluate localization abilities for listeners with a short-electrode cochlear implant who continue to wear hearing aids in both ears. A secondary purpose was to document speech perception abilities using a speech in noise test with spatially-separate noise sources. Research Design Eleven subjects that utilized a short-electrode cochlear implant and bilateral hearing aids were tested on localization and speech perception with multiple noise locations using an eight-loudspeaker array. Performance was assessed across four listening conditions using various combinations of cochlear implant and/or hearing aid use. Results Results for localization showed no significant difference between using bilateral hearing aids and bilateral hearing aids plus the cochlear implant. However, there was a significant difference between the bilateral hearing aid condition and the implant plus use of a contralateral hearing aid for all eleven subjects. Results for speech perception showed a significant benefit when using bilateral hearing aids plus the cochlear implant over use of the implant plus only one hearing aid. Conclusion Combined use of both hearing aids and the cochlear implant show significant benefits for both localization and speech perception in noise for users with a short-electrode cochlear implant. These results emphasize the importance of low-frequency information in two ears for the purpose of localization and speech perception in noise. PMID:20085199

Benefits of localization and speech perception with multiple noise sources in listeners with a short-electrode cochlear implant.

PubMed

Dunn, Camille C; Perreau, Ann; Gantz, Bruce; Tyler, Richard S

2010-01-01

Research suggests that for individuals with significant low-frequency hearing, implantation of a short-electrode cochlear implant may provide benefits of improved speech perception abilities. Because this strategy combines acoustic and electrical hearing within the same ear while at the same time preserving low-frequency residual acoustic hearing in both ears, localization abilities may also be improved. However, very little research has focused on the localization and spatial hearing abilities of users with a short-electrode cochlear implant. The purpose of this study was to evaluate localization abilities for listeners with a short-electrode cochlear implant who continue to wear hearing aids in both ears. A secondary purpose was to document speech perception abilities using a speech-in-noise test with spatially separate noise sources. Eleven subjects that utilized a short-electrode cochlear implant and bilateral hearing aids were tested on localization and speech perception with multiple noise locations using an eight-loudspeaker array. Performance was assessed across four listening conditions using various combinations of cochlear implant and/or hearing aid use. Results for localization showed no significant difference between using bilateral hearing aids and bilateral hearing aids plus the cochlear implant. However, there was a significant difference between the bilateral hearing aid condition and the implant plus use of a contralateral hearing aid for all 11 subjects. Results for speech perception showed a significant benefit when using bilateral hearing aids plus the cochlear implant over use of the implant plus only one hearing aid. Combined use of both hearing aids and the cochlear implant show significant benefits for both localization and speech perception in noise for users with a short-electrode cochlear implant. These results emphasize the importance of low-frequency information in two ears for the purpose of localization and speech perception in noise.

Artificial organs: recent progress in artificial hearing and vision.

PubMed

Ifukube, Tohru

2009-01-01

Artificial sensory organs are a prosthetic means of sending visual or auditory information to the brain by electrical stimulation of the optic or auditory nerves to assist visually impaired or hearing-impaired people. However, clinical application of artificial sensory organs, except for cochlear implants, is still a trial-and-error process. This is because how and where the information transmitted to the brain is processed is still unknown, and also because changes in brain function (plasticity) remain unknown, even though brain plasticity plays an important role in meaningful interpretation of new sensory stimuli. This article discusses some basic unresolved issues and potential solutions in the development of artificial sensory organs such as cochlear implants, brainstem implants, artificial vision, and artificial retinas.

Evaluation of the tripolar electrode stimulation method by numerical analysis and animal experiments for cochlear implants.

PubMed

Miyoshi, S; Sakajiri, M; Ifukube, T; Matsushima, J

1997-01-01

We have proposed the Tripolar Electrode Stimulation Method (TESM) which may enable us to narrow the stimulation region and to move continuously the stimulation site for the cochlear implants. We evaluated whether or not TESM works according to a theory based on numerical analysis using the auditory nerve fiber model. In this simulation, the sum of the excited model fibers were compared with the compound actions potentials obtained from animal experiments. As a result, this experiment showed that TESM could narrow a stimulation region by controlling the sum of the currents emitted from the electrodes on both sides, and continuously move a stimulation site by changing the ratio of the currents emitted from the electrodes on both sides.

Propagation-based phase-contrast x-ray tomography of cochlea using a compact synchrotron source.

PubMed

Töpperwien, Mareike; Gradl, Regine; Keppeler, Daniel; Vassholz, Malte; Meyer, Alexander; Hessler, Roland; Achterhold, Klaus; Gleich, Bernhard; Dierolf, Martin; Pfeiffer, Franz; Moser, Tobias; Salditt, Tim

2018-03-21

We demonstrate that phase retrieval and tomographic imaging at the organ level of small animals can be advantageously carried out using the monochromatic radiation emitted by a compact x-ray light source, without further optical elements apart from source and detector. This approach allows to carry out microtomography experiments which - due to the large performance gap with respect to conventional laboratory instruments - so far were usually limited to synchrotron sources. We demonstrate the potential by mapping the functional soft tissue within the guinea pig and marmoset cochlea, including in the latter case an electrical cochlear implant. We show how 3d microanatomical studies without dissection or microscopic imaging can enhance future research on cochlear implants.

Quantitative polarized light microscopy of unstained mammalian cochlear sections

NASA Astrophysics Data System (ADS)

Kalwani, Neil M.; Ong, Cheng Ai; Lysaght, Andrew C.; Haward, Simon J.; McKinley, Gareth H.; Stankovic, Konstantina M.

2013-02-01

Hearing loss is the most common sensory deficit in the world, and most frequently it originates in the inner ear. Yet, the inner ear has been difficult to access for diagnosis because of its small size, delicate nature, complex three-dimensional anatomy, and encasement in the densest bone in the body. Evolving optical methods are promising to afford cellular diagnosis of pathologic changes in the inner ear. To appropriately interpret results from these emerging technologies, it is important to characterize optical properties of cochlear tissues. Here, we focus on that characterization using quantitative polarized light microscopy (qPLM) applied to unstained cochlear sections of the mouse, a common animal model of human hearing loss. We find that the most birefringent cochlear materials are collagen fibrils and myelin. Retardance of the otic capsule, the spiral ligament, and the basilar membrane are substantially higher than that of other cochlear structures. Retardance of the spiral ligament and the basilar membrane decrease from the cochlear base to the apex, compared with the more uniform retardance of other structures. The intricate structural details revealed by qPLM of unstained cochlear sections ex vivo strongly motivate future application of polarization-sensitive optical coherence tomography to human cochlea in vivo.

Quantitative polarized light microscopy of unstained mammalian cochlear sections

PubMed Central

Kalwani, Neil M.; Ong, Cheng Ai; Lysaght, Andrew C.; Haward, Simon J.; McKinley, Gareth H.

2013-01-01

Abstract. Hearing loss is the most common sensory deficit in the world, and most frequently it originates in the inner ear. Yet, the inner ear has been difficult to access for diagnosis because of its small size, delicate nature, complex three-dimensional anatomy, and encasement in the densest bone in the body. Evolving optical methods are promising to afford cellular diagnosis of pathologic changes in the inner ear. To appropriately interpret results from these emerging technologies, it is important to characterize optical properties of cochlear tissues. Here, we focus on that characterization using quantitative polarized light microscopy (qPLM) applied to unstained cochlear sections of the mouse, a common animal model of human hearing loss. We find that the most birefringent cochlear materials are collagen fibrils and myelin. Retardance of the otic capsule, the spiral ligament, and the basilar membrane are substantially higher than that of other cochlear structures. Retardance of the spiral ligament and the basilar membrane decrease from the cochlear base to the apex, compared with the more uniform retardance of other structures. The intricate structural details revealed by qPLM of unstained cochlear sections ex vivo strongly motivate future application of polarization-sensitive optical coherence tomography to human cochlea in vivo. PMID:23407909

Glutamate co-transmission from developing medial nucleus of the trapezoid body - Lateral superior olive synapses is cochlear dependent in kanamycin-treated rats

DOE Office of Scientific and Technical Information (OSTI.GOV)

Lee, Jae Ho; Pradhan, Jonu; Maskey, Dhiraj

Research highlights: {yields} Glutamate co-transmission is enhanced in kanamycin-treated rats. {yields} VGLUT3 expression is increased in kanamycin-treated rats. {yields} GlyR expression is decreased in kanamycin-treated rats. {yields} GlyR, VGLUT3 expression patterns are asymmetric in unilaterally cochlear ablated rat. -- Abstract: Cochlear dependency of glutamate co-transmission at the medial nucleus of the trapezoid body (MNTB) - the lateral superior olive (LSO) synapses was investigated using developing rats treated with high dose kanamycin. Rats were treated with kanamycin from postnatal day (P) 3 to P8. A scanning electron microscopic study on P9 demonstrated partial cochlear hair cell damage. A whole cell voltagemore » clamp experiment demonstrated the increased glutamatergic portion of postsynaptic currents (PSCs) elicited by MNTB stimulation in P9-P11 kanamycin-treated rats. The enhanced VGLUT3 immunoreactivities (IRs) in kanamycin-treated rats and asymmetric VGLUT3 IRs in the LSO of unilaterally cochlear ablated rats supported the electrophysiologic data. Taken together, it is concluded that glutamate co-transmission is cochlear-dependent and enhanced glutamate co-transmission in kanamycin-treated rats is induced by partial cochlear damage.« less

Meningitis following cochlear implantation: pathomechanisms, clinical symptoms, conservative and surgical treatments.

PubMed

Arnold, W; Bredberg, G; Gstöttner, W; Helms, J; Hildmann, H; Kiratzidis, T; Müller, J; Ramsden, R T; Roland, P; Walterspiel, J N

2002-01-01

Pneumococcal otogenic meningitis is a rare postsurgical complication that can develop following stapedectomy or after cochlear implantation. The bacterial infection can be fatal in some instances. A recent increase in the incidence of otogenic meningitis among cochlear implant wearers is of concern. The majority of meningitis cases are associated with a 2-component electrode manufactured by one cochlear implant company. The device with the added 'positioner' component has been withdrawn from the market (FDA Public Health Web Notification: Cochlear Implant Recipients may be at Greater Risk for Meningitis, Updated: August 29, 2002, www.fda.gov/cdrh/safety/cochlear.html). Not all cases have been subsequent to otitis media and symptoms have developed from less than 24 h up to a few years after implantation. The purpose of this paper is to review and discuss the pathogenesis, pathology/bacteriology and to elaborate on some clinical features of otogenic meningitis in implanted children and adults. Essential aspects of surgery, electrode design, and cochleostomy seal are discussed. Conclusions are drawn from the available data and recommendations are made for good practice in cochlear implantation and follow-up. Copyright 2002 S. Karger AG, Basel

The development of the Nucleus Freedom Cochlear implant system.

PubMed

Patrick, James F; Busby, Peter A; Gibson, Peter J

2006-12-01

Cochlear Limited (Cochlear) released the fourth-generation cochlear implant system, Nucleus Freedom, in 2005. Freedom is based on 25 years of experience in cochlear implant research and development and incorporates advances in medicine, implantable materials, electronic technology, and sound coding. This article presents the development of Cochlear's implant systems, with an overview of the first 3 generations, and details of the Freedom system: the CI24RE receiver-stimulator, the Contour Advance electrode, the modular Freedom processor, the available speech coding strategies, the input processing options of Smart Sound to improve the signal before coding as electrical signals, and the programming software. Preliminary results from multicenter studies with the Freedom system are reported, demonstrating better levels of performance compared with the previous systems. The final section presents the most recent implant reliability data, with the early findings at 18 months showing improved reliability of the Freedom implant compared with the earlier Nucleus 3 System. Also reported are some of the findings of Cochlear's collaborative research programs to improve recipient outcomes. Included are studies showing the benefits from bilateral implants, electroacoustic stimulation using an ipsilateral and/or contralateral hearing aid, advanced speech coding, and streamlined speech processor programming.

Use of a gentamicin-impregnated collagen sheet (Collatamp(®)) in the management of major soft tissue complications in pediatric cochlear implants.

PubMed

Benito-González, Fernando; Benito, Jose; Sánchez, Luis Alberto Guardado; Estevez Alonso, Santiago; Muñoz Herrera, Angel; Batuecas-Caletrio, Angel

2014-09-01

The objective was to report the effectiveness of salvage treatment in soft tissue infection around cochlear implants with an absorbable gentamicin collagen sheet and a periosteum and skin rotation flaps. Three patients with cochlear implant and persistent surrounding soft tissue infection are included. All of them underwent antibiotic treatment prior to surgery without any response. In this study preoperative and postoperative audiograms were practiced. Surgical excision of infectious skin and a periosteum and skin rotation flaps were performed. The cochlear implant was refixed in the temporal bone and a gentamicin-impregnated collagen sheet was located covering the cochlear implant. headings In all patients with soft tissue infection around the cochlear implant, infection was completely resolved. It was not necessary to remove the device in any case. The use of an absorbable gentamicin-impregnated collagen sheet is not described for the management of soft tissue complications in pediatric cochlear implant patients. The local application of high concentrations of antibiotic administered by this sheet may be effective against resistant bacteria and, in conjunction with surgery, may resolve this type of complications.

A cool approach to reducing electrode-induced trauma: Localized therapeutic hypothermia conserves residual hearing in cochlear implantation.

PubMed

Tamames, Ilmar; King, Curtis; Bas, Esperanza; Dietrich, W Dalton; Telischi, Fred; Rajguru, Suhrud M

2016-09-01

The trauma caused during cochlear implant insertion can lead to cell death and a loss of residual hair cells in the cochlea. Various therapeutic approaches have been studied to prevent cochlear implant-induced residual hearing loss with limited success. In the present study, we show the efficacy of mild to moderate therapeutic hypothermia of 4 to 6 °C applied to the cochlea in reducing residual hearing loss associated with the electrode insertion trauma. Rats were randomly distributed in three groups: control contralateral cochleae, normothermic implanted cochleae and hypothermic implanted cochleae. Localized hypothermia was delivered to the middle turn of the cochlea for 20 min before and after implantation using a custom-designed probe perfused with cooled fluorocarbon. Auditory brainstem responses (ABRs) were recorded to assess the hearing function prior to and post-cochlear implantation at various time points up to 30 days. At the conclusion of the trials, inner ears were harvested for histology and cell count. The approach was extended to cadaver temporal bones to study the potential surgical approach and efficacy of our device. In this case, the hypothermia probe was placed next to the round window niche via the facial recess or a myringotomy. A significant loss of residual hearing was observed in the normothermic implant group. Comparatively, the residual hearing in the cochleae receiving therapeutic hypothermia was significantly conserved. Histology confirmed a significant loss of outer hair cells in normothermic cochleae receiving the surgical trauma when compared to the hypothermia treated group. In human temporal bones, a controlled and effective cooling of the cochlea was achieved using our approach. Collectively, these results suggest that therapeutic hypothermia during cochlear implantation may reduce traumatic effects of electrode insertion and improve conservation of residual hearing. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

Effects of perilymphatic pressure, sodium nitroprusside, and bupivacaine on cochlear fluid pH of guinea pigs.

PubMed

Suzuki, Masaaki; Kotani, Ryosuke

2015-01-01

Hydrostatic positive pressure and vasoconstrictor acidified the cochlear fluids, whereas the vasodilator made the fluids alkaline. CBF might play a role in regulating cochlea fluid pH. Cochlea fluid pH is highly dependent on the HCO3(-)/CO2 buffer system. Cochlear blood flow (CBF) supplies O2 and removes CO2. It is speculated that cochlear blood flow changes might affect the balance of the HCO3(-)/CO2 buffer system in the cochlea. It is known that the elevation of inner ear pressure decreases the CBF, and local application of vasodilating or vasoconstricting agents directly to the cochlea changes the CBF. The purpose of this study was to elucidate the effect of positive hydrostatic inner ear pressure and application of a vasodilator and vasoconstrictor of cochlear vessels on the pH of the endolymph and perilymph. The authors performed animal physiological experiments on 30 guinea pigs. Hydrostatic positive pressure was infused through a glass capillary tube inserted into the scala tympani of the basal turn. The vasodilator, nitric oxide donor (sodium nitroprusside; SNP), and the vasoconstrictor, bupivacaine, were placed topically onto the round window of the guinea pig cochlea. Endolymph pH (pHe) and endocochlear potential (EP) were monitored by double-barreled ion-selective microelectrodes in the second turn of the guinea pig cochlea. During the topical application study, scala vestibuli perilymph pH (pHv) was also measured simultaneously in the second turn. The application of hydrostatic positive pressure caused a decrease in pHe and EP. Positive perilymphatic pressure caused the endolymph to become acidic pressure-dependently. Application of 3.0% SNP evoked an increase in both the pHe and pHv, following by a gradual recovery to baseline levels. On the other hand, 0.5% bupivacaine caused a decrease in both the pHe and pHv. The EP during topical application showed slight, non-significant changes.

Biohybrid cochlear implants in human neurosensory restoration.

PubMed

Roemer, Ariane; Köhl, Ulrike; Majdani, Omid; Klöß, Stephan; Falk, Christine; Haumann, Sabine; Lenarz, Thomas; Kral, Andrej; Warnecke, Athanasia

2016-10-07

The success of cochlear implantation may be further improved by minimizing implantation trauma. The physical trauma of implantation and subsequent immunological sequelae can affect residual hearing and the viability of the spiral ganglion. An ideal electrode should therefore decrease post-implantation trauma and provide support to the residual spiral ganglion population. Combining a flexible electrode with cells producing and releasing protective factors could present a potential means to achieve this. Mononuclear cells obtained from bone marrow (BM-MNC) consist of mesenchymal and hematopoietic progenitor cells. They possess the innate capacity to induce repair of traumatized tissue and to modulate immunological reactions. Human bone marrow was obtained from the patients that received treatment with biohybrid electrodes. Autologous mononuclear cells were isolated from bone marrow (BM-MNC) by centrifugation using the Regenlab™ THT-centrifugation tubes. Isolated BM-MNC were characterised using flow cytometry. In addition, the release of cytokines was analysed and their biological effect tested on spiral ganglion neurons isolated from neonatal rats. Fibrin adhesive (Tisseal™) was used for the coating of silicone-based cochlear implant electrode arrays for human use in order to generate biohybrid electrodes. Toxicity of the fibrin adhesive and influence on insertion, as well on the cell coating, was investigated. Furthermore, biohybrid electrodes were implanted in three patients. Human BM-MNC release cytokines, chemokines, and growth factors that exert anti-inflammatory and neuroprotective effects. Using fibrin adhesive as a carrier for BM-MNC, a simple and effective cell coating procedure for cochlear implant electrodes was developed that can be utilised on-site in the operating room for the generation of biohybrid electrodes for intracochlear cell-based drug delivery. A safety study demonstrated the feasibility of autologous progenitor cell transplantation in humans as an adjuvant to cochlear implantation for neurosensory restoration. This is the first report of the use of autologous cell transplantation to the human inner ear. Due to the simplicity of this procedure, we hope to initiate its widespread utilization in various fields.

Challenging aspects of contemporary cochlear implant electrode array design.

PubMed

Mistrík, Pavel; Jolly, Claude; Sieber, Daniel; Hochmair, Ingeborg

2017-12-01

A design comparison of current perimodiolar and lateral wall electrode arrays of the cochlear implant (CI) is provided. The focus is on functional features such as acoustic frequency coverage and tonotopic mapping, battery consumption and dynamic range. A traumacity of their insertion is also evaluated. Review of up-to-date literature. Perimodiolar electrode arrays are positioned in the basal turn of the cochlea near the modiolus. They are designed to initiate the action potential in the proximity to the neural soma located in spiral ganglion. On the other hand, lateral wall electrode arrays can be inserted deeper inside the cochlea, as they are located along the lateral wall and such insertion trajectory is less traumatic. This class of arrays targets primarily surviving neural peripheral processes. Due to their larger insertion depth, lateral wall arrays can deliver lower acoustic frequencies in manner better corresponding to cochlear tonotopicity. In fact, spiral ganglion sections containing auditory nerve fibres tuned to low acoustic frequencies are located deeper than 1 and half turn inside the cochlea. For this reason, a significant frequency mismatch might be occurring for apical electrodes in perimodiolar arrays, detrimental to speech perception. Tonal languages such as Mandarin might be therefore better treated with lateral wall arrays. On the other hand, closer proximity to target tissue results in lower psychophysical threshold levels for perimodiolar arrays. However, the maximal comfort level is also lower, paradoxically resulting in narrower dynamic range than that of lateral wall arrays. Battery consumption is comparable for both types of arrays. Lateral wall arrays are less likely to cause trauma to cochlear structures. As the current trend in cochlear implantation is the maximal protection of residual acoustic hearing, the lateral wall arrays seem more suitable for hearing preservation CI surgeries. Future development could focus on combining the advantages of both types: perimodiolar location in the basal turn extended to lateral wall location for higher turn locations.

Comparison of speech perception performance between Sprint/Esprit 3G and Freedom processors in children implanted with nucleus cochlear implants.

PubMed

Santarelli, Rosamaria; Magnavita, Vincenzo; De Filippi, Roberta; Ventura, Laura; Genovese, Elisabetta; Arslan, Edoardo

2009-04-01

To compare speech perception performance in children fitted with previous generation Nucleus sound processor, Sprint or Esprit 3G, and the Freedom, the most recently released system from the Cochlear Corporation that features a larger input dynamic range. Prospective intrasubject comparative study. University Medical Center. Seventeen prelingually deafened children who had received the Nucleus 24 cochlear implant and used the Sprint or Esprit 3G sound processor. Cochlear implantation with Cochlear device. Speech perception was evaluated at baseline (Sprint, n = 11; Esprit 3G, n = 6) and after 1 month's experience with the Freedom sound processor. Identification and recognition of disyllabic words and identification of vowels were performed via recorded voice in quiet (70 dB [A]), in the presence of background noise at various levels of signal-to-noise ratio (+10, +5, 0, -5) and at a soft presentation level (60 dB [A]). Consonant identification and recognition of disyllabic words, trisyllabic words, and sentences were evaluated in live voice. Frequency discrimination was measured in a subset of subjects (n = 5) by using an adaptive, 3-interval, 3-alternative, forced-choice procedure. Identification of disyllabic words administered at a soft presentation level showed a significant increase when switching to the Freedom compared with the previously worn processor in children using the Sprint or Esprit 3G. Identification and recognition of disyllabic words in the presence of background noise as well as consonant identification and sentence recognition increased significantly for the Freedom compared with the previously worn device only in children fitted with the Sprint. Frequency discrimination was significantly better when switching to the Freedom compared with the previously worn processor. Serial comparisons revealed that that speech perception performance evaluated in children aged 5 to 15 years was superior with the Freedom than previous generations of Nucleus sound processors. These differences are deemed to ensue from an increased input dynamic range, a feature that offers potentially enhanced phonemic discrimination.

Feasibility of personalised remote long-term follow-up of people with cochlear implants: a randomised controlled trial.

PubMed

Cullington, Helen; Kitterick, Padraig; Weal, Mark; Margol-Gromada, Magdalena

2018-04-20

Substantial resources are required to provide lifelong postoperative care to people with cochlear implants. Most patients visit the clinic annually. We introduced a person-centred remote follow-up pathway, giving patients telemedicine tools to use at home so they would only visit the centre when intervention was required. To assess the feasibility of comparing a remote care pathway with the standard pathway in adults using cochlear implants. Two-arm randomised controlled trial. Randomisation used a minimisation approach, controlling for potential confounding factors. Participant blinding was not possible, but baseline measures occurred before allocation. University of Southampton Auditory Implant Service: provider of National Health Service care. 60 adults who had used cochlear implants for at least 6 months. Control group (n=30) followed usual care pathway.Remote care group (n=30) received care remotely for 6 months incorporating: home hearing in noise test, online support tool and self-adjustment of device (only 10 had compatible equipment). Primary: change in patient activation; measured using the Patient Activation Measure.Secondary: change in hearing and quality of life; qualitative feedback from patients and clinicians. One participant in the remote care group dropped out. The remote care group showed a greater increase in patient activation than the control group. Changes in hearing differed between the groups. The remote care group improved on the Triple Digit Test hearing test; the control group perceived their hearing was worse on the Speech, Spatial and Qualities of Hearing Scale questionnaire. Quality of life remained unchanged in both groups. Patients and clinicians were generally positive about remote care tools and wanted to continue. Adults with cochlear implants were willing to be randomised and complied with the protocol. Personalised remote care for long-term follow-up is feasible and acceptable, leading to more empowered patients. ISRCTN14644286. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Spiral ligament and stria vascularis changes in cochlear otosclerosis: effect on hearing level.

PubMed

Doherty, Joni K; Linthicum, Fred H

2004-07-01

To investigate the effect of changes within the spiral ligament and stria vascularis on hearing in cochlear otosclerosis, we examined spiral ligament hyalinization, stria vascularis atrophy, and sensory hearing loss in cochlear otosclerosis and described changes in ion transport molecule expression. Retrospective. Tertiary referral center. Thirty-two cochleae from 24 temporal bone donors with histologic evidence of cochlear otosclerosis, including spiral ligament hyalinization. Audiography. Measurements of spiral ligament width, stria vascularis, and bone-conduction thresholds were compared by the amount of hyalinization. Expression of the ion transport molecules Na,K-ATPase, connexin 26, and carbonic anhydrase II were assessed by immunohistochemical techniques. Hyalinization most often involved the posterior basal turn (88%) and the posterior middle turn (27%). Spiral ligament hyalinization correlated significantly with stria vascularis atrophy in the posterior middle turn of the cochlea (rho = -0.63, p < 0.01). There was a trend toward a significant association in the posterior basal turn (rho = -0.31, p < 0.08). Bone-conduction thresholds at 2,000 and 4,000 Hz were significantly associated with the amount of stria vascularis atrophy (rho = -0.44, -0.40, p < 0.05). In addition, we observed decreased immunostaining for both carbonic anhydrase II with Type I fibrocytes and Na,K-ATPase with stria vascularis and Type II and Type IV fibrocytes of the spiral ligament in cochlear otosclerosis sections compared with normal cochlea. Na,K-ATPase staining within the stria vascularis was further decreased in the presence of spiral ligament hyalinization. No significant differences were seen with connexin 26 immunostaining. However, immunostaining results were somewhat inconsistent. These data suggest that spiral ligament structure and function are essential for stria vascularis survival. In addition, dampened expression of ion transport molecules within the spiral ligament and stria vascularis may disrupt potassium ion recycling, resulting in loss of endocochlear potential and sensory hearing loss.

Feasibility of personalised remote long-term follow-up of people with cochlear implants: a randomised controlled trial

PubMed Central

Kitterick, Padraig; Weal, Mark; Margol-Gromada, Magdalena

2018-01-01

Introduction Substantial resources are required to provide lifelong postoperative care to people with cochlear implants. Most patients visit the clinic annually. We introduced a person-centred remote follow-up pathway, giving patients telemedicine tools to use at home so they would only visit the centre when intervention was required. Objectives To assess the feasibility of comparing a remote care pathway with the standard pathway in adults using cochlear implants. Design Two-arm randomised controlled trial. Randomisation used a minimisation approach, controlling for potential confounding factors. Participant blinding was not possible, but baseline measures occurred before allocation. Setting University of Southampton Auditory Implant Service: provider of National Health Service care. Participants 60 adults who had used cochlear implants for at least 6 months. Interventions Control group (n=30) followed usual care pathway. Remote care group (n=30) received care remotely for 6 months incorporating: home hearing in noise test, online support tool and self-adjustment of device (only 10 had compatible equipment). Main outcome measures Primary: change in patient activation; measured using the Patient Activation Measure. Secondary: change in hearing and quality of life; qualitative feedback from patients and clinicians. Results One participant in the remote care group dropped out. The remote care group showed a greater increase in patient activation than the control group. Changes in hearing differed between the groups. The remote care group improved on the Triple Digit Test hearing test; the control group perceived their hearing was worse on the Speech, Spatial and Qualities of Hearing Scale questionnaire. Quality of life remained unchanged in both groups. Patients and clinicians were generally positive about remote care tools and wanted to continue. Conclusions Adults with cochlear implants were willing to be randomised and complied with the protocol. Personalised remote care for long-term follow-up is feasible and acceptable, leading to more empowered patients. Trial registration number ISRCTN14644286. PMID:29678970

Cochlear Implantation after Bilateral Transverse Temporal Bone Fractures

PubMed Central

Shin, Jong-Heon; Park, SooChan; Baek, Sam-Hyun

2008-01-01

Patients deafened by a severe head injury are rarely encountered. We report a case of a 65-yr-old man with bilateral transverse temporal bone fractures due to head injury. He underwent cochlear implant and achieved a satisfactory auditory rehabilitation. Imaging studies of temporal bone before performing a cochlear implantation provide important information on a patient with bilateral temporal bone fractures. Cochlear implantations with careful planning in such a patient may be a very effective method for aural rehabilitation. PMID:19434252

Reduced electromotility of outer hair cells associated with connexin-related forms of deafness: an in silico study of a cochlear network mechanism.

PubMed

Mistrík, Pavel; Ashmore, Jonathan F

2010-12-01

Mutations in the GJB2 gene encoding for the connexin 26 (Cx26) protein are the most common source of nonsyndromic forms of deafness. Cx26 is a building block of gap junctions (GJs) which establish electrical connectivity in distinct cochlear compartments by allowing intercellular ionic (and metabolic) exchange. Animal models of the Cx26 deficiency in the organ of Corti seem to suggest that the hearing loss and the degeneration of outer hair cells (OHCs) and inner hair cells is due to failed K(+) and metabolite homeostasis. However, OHCs can develop normally in some mutants, suggesting that the hair cells death is not the universal mechanism. In search for alternatives, we have developed an in silico large scale three-dimensional model of electrical current flow in the cochlea in the small signal, linearised, regime. The effect of mutations was analysed by varying the magnitude of resistive components representing the GJ network in the organ of Corti. The simulations indeed show that reduced GJ conductivity increases the attenuation of the OHC transmembrane potential at frequencies above 5 kHz from 6.1 dB/decade in the wild-type to 14.2 dB/decade. As a consequence of increased GJ electrical filtering, the OHC transmembrane potential is reduced by up to 35 dB at frequencies >10 kHz. OHC electromotility, driven by this potential, is crucial for sound amplification, cochlear sensitivity and frequency selectivity. Therefore, we conclude that reduced OHC electromotility may represent an additional mechanism underlying deafness in the presence of Cx26 mutations and may explain lowered OHC functionality in particular reported Cx26 mutants.

Clinical assessment of pitch perception.

PubMed

Vaerenberg, Bart; Pascu, Alexandru; Del Bo, Luca; Schauwers, Karen; De Ceulaer, Geert; Daemers, Kristin; Coene, Martine; Govaerts, Paul J

2011-07-01

The perception of pitch has recently gained attention. At present, clinical audiologic tests to assess this are hardly available. This article reports on the development of a clinical test using harmonic intonation (HI) and disharmonic intonation (DI). Prospective collection of normative data and pilot study in hearing-impaired subjects. Tertiary referral center. Normative data were collected from 90 normal-hearing subjects recruited from 3 different language backgrounds. The pilot study was conducted on 18 hearing-impaired individuals who were selected into 3 pathologic groups: high-frequency hearing loss (HF), low-frequency hearing loss (LF), and cochlear implant users (CI). Normative data collection and exploratory diagnostics by means of the newly constructed HI/DI tests using intonation patterns to find the just noticeable difference (JND) for pitch discrimination in low-frequency harmonic complex sounds presented in a same-different task. JND for pitch discrimination using HI/DI tests in the hearing population and pathologic groups. Normative data are presented in 5 parameter statistics and box-and-whisker plots showing median JNDs of 2 (HI) and 3 Hz (DI). The results on both tests are statistically abnormal in LF and CI subjects, whereas they are not significantly abnormal in the HF group. The HI and DI tests allow the clinical assessment of low-frequency pitch perception. The data obtained in this study define the normal zone for both tests. Preliminary results indicate possible abnormal TFS perception in some hearing-impaired subjects.

The contribution of short-term memory capacity to reading ability in adolescents with cochlear implants.

PubMed

Edwards, Lindsey; Aitkenhead, Lynne; Langdon, Dawn

2016-11-01

This study aimed to establish the relationship between short-term memory capacity and reading skills in adolescents with cochlear implants. A between-groups design compared a group of young people with cochlear implants with a group of hearing peers on measures of reading, and auditory and visual short-term memory capacity. The groups were matched for non-verbal IQ and age. The adolescents with cochlear implants were recruited from the Cochlear Implant Programme at a specialist children's hospital. The hearing participants were recruited from the same schools as those attended by the implanted adolescents. Participants were 18 cochlear implant users and 14 hearing controls, aged between 12 and 18 years. All used English as their main language and had no significant learning disability or neuro-developmental disorder. Short-term memory capacity was assessed in the auditory modality using Forward and Reverse Digit Span from the WISC IV UK, and visually using Forward and Reverse Memory from the Leiter-R. Individual word reading, reading comprehension and pseudoword decoding were assessed using the WIAT II UK. A series of ANOVAs revealed that the adolescents with cochlear implants had significantly poorer auditory short-term memory capacity and reading skills (on all measures) compared with their hearing peers. However, when Forward Digit Span was entered into the analyses as a covariate, none of the differences remained statistically significant. Deficits in immediate auditory memory persist into adolescence in deaf children with cochlear implants. Short-term auditory memory capacity is an important neurocognitive process in the development of reading skills after cochlear implantation in childhood that remains evident in later adolescence. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

A preliminary investigation of the air-bone gap: Changes in intracochlear sound pressure with air- and bone-conducted stimuli after cochlear implantation

PubMed Central

Banakis Hartl, Renee M.; Mattingly, Jameson K.; Greene, Nathaniel T.; Jenkins, Herman A.; Cass, Stephen P.; Tollin, Daniel J.

2016-01-01

Hypothesis A cochlear implant electrode within the cochlea contributes to the air-bone gap (ABG) component of postoperative changes in residual hearing after electrode insertion. Background Preservation of residual hearing after cochlear implantation has gained importance as simultaneous electric-acoustic stimulation allows for improved speech outcomes. Postoperative loss of residual hearing has previously been attributed to sensorineural changes; however, presence of increased postoperative air-bone gap remains unexplained and could result in part from altered cochlear mechanics. Here, we sought to investigate changes to these mechanics via intracochlear pressure measurements before and after electrode implantation to quantify the contribution to postoperative air-bone gap. Methods Human cadaveric heads were implanted with titanium fixtures for bone conduction transducers. Velocities of stapes capitulum and cochlear promontory between the two windows were measured using single-axis laser Doppler vibrometry and fiber-optic sensors measured intracochlear pressures in scala vestibuli and tympani for air- and bone-conducted stimuli before and after cochlear implant electrode insertion through the round window. Results Intracochlear pressures revealed only slightly reduced responses to air-conducted stimuli consistent with prior literature. No significant changes were noted to bone-conducted stimuli after implantation. Velocities of the stapes capitulum and the cochlear promontory to both stimuli were stable following electrode placement. Conclusion Presence of a cochlear implant electrode causes alterations in intracochlear sound pressure levels to air, but not bone, conducted stimuli and helps to explain changes in residual hearing noted clinically. These results suggest the possibility of a cochlear conductive component to postoperative changes in hearing sensitivity. PMID:27579835

Pediatric tinnitus: Incidence of imaging anomalies and the impact of hearing loss.

PubMed

Kerr, Rhorie; Kang, Elise; Hopkins, Brandon; Anne, Samantha

2017-12-01

Guidelines exist for evaluation and management of tinnitus in adults; however lack of evidence in children limits applicability of these guidelines to pediatric patients. Objective of this study is to determine the incidence of inner ear anomalies detected on imaging studies within the pediatric population with tinnitus and evaluate if presence of hearing loss increases the rate of detection of anomalies in comparison to normal hearing patients. Retrospective review of all children with diagnosis of tinnitus from 2010 to 2015 ;at a tertiary care academic center. 102 pediatric patients with tinnitus were identified. Overall, 53 patients had imaging studies with 6 abnormal findings (11.3%). 51/102 patients had hearing loss of which 33 had imaging studies demonstrating 6 inner ear anomalies detected. This is an incidence of 18.2% for inner ear anomalies identified in patients with hearing loss (95% confidence interval (CI) of 7.0-35.5%). 4 of these 6 inner ear anomalies detected were vestibular aqueduct abnormalities. The other two anomalies were cochlear hypoplasia and bilateral semicircular canal dysmorphism. 51 patients had no hearing loss and of these patients, 20 had imaging studies with no inner ear abnormalities detected. There was no statistical difference in incidence of abnormal imaging findings in patients with and without hearing loss (Fisher's exact test, p ;= ;0.072.) CONCLUSION: There is a high incidence of anomalies detected in imaging studies done in pediatric patients with tinnitus, especially in the presence of hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

Recognition and localization of speech by adult cochlear implant recipients wearing a digital hearing aid in the nonimplanted ear (bimodal hearing).

PubMed

Potts, Lisa G; Skinner, Margaret W; Litovsky, Ruth A; Strube, Michael J; Kuk, Francis

2009-06-01

The use of bilateral amplification is now common clinical practice for hearing aid users but not for cochlear implant recipients. In the past, most cochlear implant recipients were implanted in one ear and wore only a monaural cochlear implant processor. There has been recent interest in benefits arising from bilateral stimulation that may be present for cochlear implant recipients. One option for bilateral stimulation is the use of a cochlear implant in one ear and a hearing aid in the opposite nonimplanted ear (bimodal hearing). This study evaluated the effect of wearing a cochlear implant in one ear and a digital hearing aid in the opposite ear on speech recognition and localization. A repeated-measures correlational study was completed. Nineteen adult Cochlear Nucleus 24 implant recipients participated in the study. The participants were fit with a Widex Senso Vita 38 hearing aid to achieve maximum audibility and comfort within their dynamic range. Soundfield thresholds, loudness growth, speech recognition, localization, and subjective questionnaires were obtained six-eight weeks after the hearing aid fitting. Testing was completed in three conditions: hearing aid only, cochlear implant only, and cochlear implant and hearing aid (bimodal). All tests were repeated four weeks after the first test session. Repeated-measures analysis of variance was used to analyze the data. Significant effects were further examined using pairwise comparison of means or in the case of continuous moderators, regression analyses. The speech-recognition and localization tasks were unique, in that a speech stimulus presented from a variety of roaming azimuths (140 degree loudspeaker array) was used. Performance in the bimodal condition was significantly better for speech recognition and localization compared to the cochlear implant-only and hearing aid-only conditions. Performance was also different between these conditions when the location (i.e., side of the loudspeaker array that presented the word) was analyzed. In the bimodal condition, the speech-recognition and localization tasks were equal regardless of which side of the loudspeaker array presented the word, while performance was significantly poorer for the monaural conditions (hearing aid only and cochlear implant only) when the words were presented on the side with no stimulation. Binaural loudness summation of 1-3 dB was seen in soundfield thresholds and loudness growth in the bimodal condition. Measures of the audibility of sound with the hearing aid, including unaided thresholds, soundfield thresholds, and the Speech Intelligibility Index, were significant moderators of speech recognition and localization. Based on the questionnaire responses, participants showed a strong preference for bimodal stimulation. These findings suggest that a well-fit digital hearing aid worn in conjunction with a cochlear implant is beneficial to speech recognition and localization. The dynamic test procedures used in this study illustrate the importance of bilateral hearing for locating, identifying, and switching attention between multiple speakers. It is recommended that unilateral cochlear implant recipients, with measurable unaided hearing thresholds, be fit with a hearing aid.

Relationships among Professionals' Knowledge, Experience, and Expectations Regarding Cochlear Implants

ERIC Educational Resources Information Center

Ben-Itzhak, D.; Most, T.; Weisel, A.

2005-01-01

The present study examined the relationships between teachers' and communication clinicians' self-reported knowledge on cochlear implants and their expectations of CIs. The authors also explored these professionals' views regarding the child's communication mode, educational setting, and social options following cochlear implantation. The…

The Relationship between Cochlear Implants and Deaf Identity

ERIC Educational Resources Information Center

Chapman, Madeline; Dammeyer, Jesper

2017-01-01

The degree to which individuals with cochlear implants (CIs) experience communication difficulties has implications for social participation and identity development. However, few studies have examined the relationship between cochlear implantation, identity, and social participation. Using data from a Danish national survey of deaf adults, the…

Changing patterns of peanut agglutinin labelling in the dorsal cochlear nucleus correspond to axonal ingrowth.

PubMed Central

Riggs, G H; Schweitzer, L

1994-01-01

Various studies have suggested that glycoconjugates may influence connectivity and lamination in the developing central nervous system and may function as barriers to neuritic extension. It has been proposed that the peanut agglutinin lectin labels a glycoconjugate subserving a barrier function. We chose to investigate the distribution of this peanut-agglutinin-labelled glycoconjugate in the dorsal cochlear nucleus of the developing hamster since the development of the dorsal cochlear nucleus is well characterised and its axons obey laminar boundaries. The distribution of peanut agglutinin label throughout the cochlear nucleus delineated zones that cochlear axons fail to invade. In the dorsal cochlear nucleus, laminar differences were reduced on postnatal d 13 and virtually disappearing by postnatal d 23. Label in the molecular layer dissipated as axons and dendrites grew into this layer. These patterns of peanut agglutinin binding correspond to axonal ingrowth and are consistent with a barrier function for glycoconjugates in the molecular layer. Images Fig. 1 Fig. 2 Fig. 4 PMID:7961144

Modulation of Mcl-1 expression reduces age-related cochlear degeneration

PubMed Central

Yang, Wei Ping; Xu, Yang; Guo, Wei Wei; Liu, Hui Zhan; Hu, Bo Hua

2013-01-01

Mcl-1 is an anti-apoptotic member of the Bcl-2 family that modulates apoptosis-related signaling pathways and promotes cell survival. We have previously demonstrated a reduction of Mcl-1 expression in aging cochleae. To investigate whether restoring Mcl-1 expression would reduce aging-related cochlear degeneration, we developed a rat model of Mcl-1 overexpression. A plasmid encoding human Mcl-1/enhanced green fluorescent protein was applied to the round window of the cochlea. This in vivo treatment transfected both the sensory and supporting cells of the cochlear sensory epithelium and enhanced Mcl-1 expression at both the mRNA and the protein level. The upregulation of Mcl-1 expression reduced the progression of age-related cochlear dysfunction and sensory cell death. Furthermore, the transfection of Mcl-1 exerted its protective effect by suppressing cochlear apoptosis at the mitochondrial level. This study demonstrates that the genetic modulation of Mcl-1 expression reduces the progression of age-related cochlear degeneration. PMID:23790646

Gain and frequency tuning within the mouse cochlear apex

DOE Office of Scientific and Technical Information (OSTI.GOV)

Oghalai, John S.; Raphael, Patrick D.; Gao, Simon

Normal mammalian hearing requires cochlear outer hair cell active processes that amplify the traveling wave with high gain and sharp tuning, termed cochlear amplification. We have used optical coherence tomography to study cochlear amplification within the apical turn of the mouse cochlea. We measured not only classical basilar membrane vibratory tuning curves but also vibratory responses from the rest of the tissues that compose the organ of Corti. Basilar membrane tuning was sharp in live mice and broad in dead mice, whereas other regions of the organ of Corti demonstrated phase shifts consistent with additional filtering beyond that provided bymore » basilar membrane mechanics. We use these experimental data to support a conceptual framework of how cochlear amplification is tuned within the mouse cochlear apex. We will also study transgenic mice with targeted mutations that affect different biomechanical aspects of the organ of Corti in an effort to localize the underlying processes that produce this additional filtering.« less

Evidence of across-channel processing for spectral-ripple discrimination in cochlear implant listeners.

PubMed

Won, Jong Ho; Jones, Gary L; Drennan, Ward R; Jameyson, Elyse M; Rubinstein, Jay T

2011-10-01

Spectral-ripple discrimination has been used widely for psychoacoustical studies in normal-hearing, hearing-impaired, and cochlear implant listeners. The present study investigated the perceptual mechanism for spectral-ripple discrimination in cochlear implant listeners. The main goal of this study was to determine whether cochlear implant listeners use a local intensity cue or global spectral shape for spectral-ripple discrimination. The effect of electrode separation on spectral-ripple discrimination was also evaluated. Results showed that it is highly unlikely that cochlear implant listeners depend on a local intensity cue for spectral-ripple discrimination. A phenomenological model of spectral-ripple discrimination, as an "ideal observer," showed that a perceptual mechanism based on discrimination of a single intensity difference cannot account for performance of cochlear implant listeners. Spectral modulation depth and electrode separation were found to significantly affect spectral-ripple discrimination. The evidence supports the hypothesis that spectral-ripple discrimination involves integrating information from multiple channels. © 2011 Acoustical Society of America

Evidence of across-channel processing for spectral-ripple discrimination in cochlear implant listeners a

PubMed Central

Ho Won, Jong; Jones, Gary L.; Drennan, Ward R.; Jameyson, Elyse M.; Rubinstein, Jay T.

2011-01-01

Spectral-ripple discrimination has been used widely for psychoacoustical studies in normal-hearing, hearing-impaired, and cochlear implant listeners. The present study investigated the perceptual mechanism for spectral-ripple discrimination in cochlear implant listeners. The main goal of this study was to determine whether cochlear implant listeners use a local intensity cue or global spectral shape for spectral-ripple discrimination. The effect of electrode separation on spectral-ripple discrimination was also evaluated. Results showed that it is highly unlikely that cochlear implant listeners depend on a local intensity cue for spectral-ripple discrimination. A phenomenological model of spectral-ripple discrimination, as an “ideal observer,” showed that a perceptual mechanism based on discrimination of a single intensity difference cannot account for performance of cochlear implant listeners. Spectral modulation depth and electrode separation were found to significantly affect spectral-ripple discrimination. The evidence supports the hypothesis that spectral-ripple discrimination involves integrating information from multiple channels. PMID:21973363

International classification of reliability for implanted cochlear implant receiver stimulators.

PubMed

Battmer, Rolf-Dieter; Backous, Douglas D; Balkany, Thomas J; Briggs, Robert J S; Gantz, Bruce J; van Hasselt, Andrew; Kim, Chong Sun; Kubo, Takeshi; Lenarz, Thomas; Pillsbury, Harold C; O'Donoghue, Gerard M

2010-10-01

To design an international standard to be used when reporting reliability of the implanted components of cochlear implant systems to appropriate governmental authorities, cochlear implant (CI) centers, and for journal editors in evaluating manuscripts involving cochlear implant reliability. The International Consensus Group for Cochlear Implant Reliability Reporting was assembled to unify ongoing efforts in the United States, Europe, Asia, and Australia to create a consistent and comprehensive classification system for the implanted components of CI systems across manufacturers. All members of the consensus group are from tertiary referral cochlear implant centers. None. A clinically relevant classification scheme adapted from principles of ISO standard 5841-2:2000 originally designed for reporting reliability of cardiac pacemakers, pulse generators, or leads. Standard definitions for device failure, survival time, clinical benefit, reduced clinical benefit, and specification were generated. Time intervals for reporting back to implant centers for devices tested to be "out of specification," categorization of explanted devices, the method of cumulative survival reporting, and content of reliability reports to be issued by manufacturers was agreed upon by all members. The methodology for calculating Cumulative survival was adapted from ISO standard 5841-2:2000. The International Consensus Group on Cochlear Implant Device Reliability Reporting recommends compliance to this new standard in reporting reliability of implanted CI components by all manufacturers of CIs and the adoption of this standard as a minimal reporting guideline for editors of journals publishing cochlear implant research results.

Evaluation of Mandarin Chinese Speech Recognition in Adults with Cochlear Implants Using the Spectral Ripple Discrimination Test

PubMed Central

Dai, Chuanfu; Zhao, Zeqi; Zhang, Duo; Lei, Guanxiong

2018-01-01

Background The aim of this study was to explore the value of the spectral ripple discrimination test in speech recognition evaluation among a deaf (post-lingual) Mandarin-speaking population in China following cochlear implantation. Material/Methods The study included 23 Mandarin-speaking adult subjects with normal hearing (normal-hearing group) and 17 deaf adults who were former Mandarin-speakers, with cochlear implants (cochlear implantation group). The normal-hearing subjects were divided into men (n=10) and women (n=13). The spectral ripple discrimination thresholds between the groups were compared. The correlation between spectral ripple discrimination thresholds and Mandarin speech recognition rates in the cochlear implantation group were studied. Results Spectral ripple discrimination thresholds did not correlate with age (r=−0.19; p=0.22), and there was no significant difference in spectral ripple discrimination thresholds between the male and female groups (p=0.654). Spectral ripple discrimination thresholds of deaf adults with cochlear implants were significantly correlated with monosyllabic recognition rates (r=0.84; p=0.000). Conclusions In a Mandarin Chinese speaking population, spectral ripple discrimination thresholds of normal-hearing individuals were unaffected by both gender and age. Spectral ripple discrimination thresholds were correlated with Mandarin monosyllabic recognition rates of Mandarin-speaking in post-lingual deaf adults with cochlear implants. The spectral ripple discrimination test is a promising method for speech recognition evaluation in adults following cochlear implantation in China. PMID:29806954

Evaluation of Mandarin Chinese Speech Recognition in Adults with Cochlear Implants Using the Spectral Ripple Discrimination Test.

PubMed

Dai, Chuanfu; Zhao, Zeqi; Shen, Weidong; Zhang, Duo; Lei, Guanxiong; Qiao, Yuehua; Yang, Shiming

2018-05-28

BACKGROUND The aim of this study was to explore the value of the spectral ripple discrimination test in speech recognition evaluation among a deaf (post-lingual) Mandarin-speaking population in China following cochlear implantation. MATERIAL AND METHODS The study included 23 Mandarin-speaking adult subjects with normal hearing (normal-hearing group) and 17 deaf adults who were former Mandarin-speakers, with cochlear implants (cochlear implantation group). The normal-hearing subjects were divided into men (n=10) and women (n=13). The spectral ripple discrimination thresholds between the groups were compared. The correlation between spectral ripple discrimination thresholds and Mandarin speech recognition rates in the cochlear implantation group were studied. RESULTS Spectral ripple discrimination thresholds did not correlate with age (r=-0.19; p=0.22), and there was no significant difference in spectral ripple discrimination thresholds between the male and female groups (p=0.654). Spectral ripple discrimination thresholds of deaf adults with cochlear implants were significantly correlated with monosyllabic recognition rates (r=0.84; p=0.000). CONCLUSIONS In a Mandarin Chinese speaking population, spectral ripple discrimination thresholds of normal-hearing individuals were unaffected by both gender and age. Spectral ripple discrimination thresholds were correlated with Mandarin monosyllabic recognition rates of Mandarin-speaking in post-lingual deaf adults with cochlear implants. The spectral ripple discrimination test is a promising method for speech recognition evaluation in adults following cochlear implantation in China.

Modulation of cochlear responses in the guinea pig by low-frequency, phase-shifted maskers following noise trauma.

PubMed

Hoehmann, D; Müller, S; Dornhoffer, J L

1995-01-01

Low-frequency acoustic biasing using an intensive phase-shifted, low-frequency masker was studied according to its ability to determine disorders of cochlear micromechanics following noise trauma in the guinea pig as animal model. Statistical analyses proved that this technique allowed electrophysiological differentiation of controls versus groups with different degrees of experimentally induced threshold shifts. To substantiate group differences an intensity of at least 70 dB SPL was required for the 52 Hz masker and the difference in relation to the test-tone intensity had to be +/- 10 or +/- 20 dB SPL. The noise-traumatized cochlea could be identified by means of a threshold shift for the 5 microV pseudothreshold, a low modulation span of the compound action potential amplitude (< 25-50 microV frequency dependent), and reduced positive summating potential amplitude with negative non-modulating values within the different measurement phases for 1 and 2 kHz stimulation.

Early Vocabulary Development in Children with Bilateral Cochlear Implants

ERIC Educational Resources Information Center

Välimaa, Taina; Kunnari, Sari; Laukkanen-Nevala, Päivi; Lonka, Eila

2018-01-01

Background: Children with unilateral cochlear implants (CIs) may have delayed vocabulary development for an extended period after implantation. Bilateral cochlear implantation is reported to be associated with improved sound localization and enhanced speech perception in noise. This study proposed that bilateral implantation might also promote…

Deafblind People's Experiences of Cochlear Implantation

ERIC Educational Resources Information Center

Soper, Janet

2006-01-01

Cochlear implants are electronic devices that create the sensation of hearing in those who cannot obtain any benefit from conventional hearing aids. This article examines the experience of cochlear implantation in a select group of individuals with acquired deafblindness, focusing on three key themes: access to communication, information and…

Melodic contour identification by cochlear implant listeners.

PubMed

Galvin, John J; Fu, Qian-Jie; Nogaki, Geraldine

2007-06-01

While the cochlear implant provides many deaf patients with good speech understanding in quiet, music perception and appreciation with the cochlear implant remains a major challenge for most cochlear implant users. The present study investigated whether a closed-set melodic contour identification (MCI) task could be used to quantify cochlear implant users' ability to recognize musical melodies and whether MCI performance could be improved with moderate auditory training. The present study also compared MCI performance with familiar melody identification (FMI) performance, with and without MCI training. For the MCI task, test stimuli were melodic contours composed of 5 notes of equal duration whose frequencies corresponded to musical intervals. The interval between successive notes in each contour was varied between 1 and 5 semitones; the "root note" of the contours was also varied (A3, A4, and A5). Nine distinct musical patterns were generated for each interval and root note condition, resulting in a total of 135 musical contours. The identification of these melodic contours was measured in 11 cochlear implant users. FMI was also evaluated in the same subjects; recognition of 12 familiar melodies was tested with and without rhythm cues. MCI was also trained in 6 subjects, using custom software and melodic contours presented in a different frequency range from that used for testing. Results showed that MCI recognition performance was highly variable among cochlear implant users, ranging from 14% to 91% correct. For most subjects, MCI performance improved as the number of semitones between successive notes was increased; performance was slightly lower for the A3 root note condition. Mean FMI performance was 58% correct when rhythm cues were preserved and 29% correct when rhythm cues were removed. Statistical analyses revealed no significant correlation between MCI performance and FMI performance (with or without rhythmic cues). However, MCI performance was significantly correlated with vowel recognition performance; FMI performance was not correlated with cochlear implant subjects' phoneme recognition performance. Preliminary results also showed that the MCI training improved all subjects' MCI performance; the improved MCI performance also generalized to improved FMI performance. Preliminary data indicate that the closed-set MCI task is a viable approach toward quantifying an important component of cochlear implant users' music perception. The improvement in MCI performance and generalization to FMI performance with training suggests that MCI training may be useful for improving cochlear implant users' music perception and appreciation; such training may be necessary to properly evaluate patient performance, as acute measures may underestimate the amount of musical information transmitted by the cochlear implant device and received by cochlear implant listeners.

Evaluation of a wireless audio streaming accessory to improve mobile telephone performance of cochlear implant users.

PubMed

Wolfe, Jace; Morais Duke, Mila; Schafer, Erin; Cire, George; Menapace, Christine; O'Neill, Lori

2016-01-01

The objective of this study was to evaluate the potential improvement in word recognition in quiet and in noise obtained with use of a Bluetooth-compatible wireless hearing assistance technology (HAT) relative to the acoustic mobile telephone condition (e.g. the mobile telephone receiver held to the microphone of the sound processor). A two-way repeated measures design was used to evaluate differences in telephone word recognition obtained in quiet and in competing noise in the acoustic mobile telephone condition compared to performance obtained with use of the CI sound processor and a telephone HAT. Sixteen adult users of Nucleus cochlear implants and the Nucleus 6 sound processor were included in this study. Word recognition over the mobile telephone in quiet and in noise was significantly better with use of the wireless HAT compared to performance in the acoustic mobile telephone condition. Word recognition over the mobile telephone was better in quiet when compared to performance in noise. The results of this study indicate that use of a wireless HAT improves word recognition over the mobile telephone in quiet and in noise relative to performance in the acoustic mobile telephone condition for a group of adult cochlear implant recipients.

Hearing and hearing loss: Causes, effects, and treatments

NASA Astrophysics Data System (ADS)

Schmiedt, Richard A.

2003-04-01

Hearing loss can have multiple causes. The outer and middle ears are conductive pathways for acoustic energy to the inner ear (cochlea) and help shape our spectral sensitivity. Conductive hearing loss is mechanical in nature such that the energy transfer to the cochlea is impeded, often from eardrum perforations or middle ear fluid buildup. Beyond the middle ear, the cochlea comprises three interdependent systems necessary for normal hearing. The first is that of basilar-membrane micromechanics including the outer hair cells. This system forms the basis of the cochlear amplifier and is the most vulnerable to noise and drug exposure. The second system comprises the ion pumps in the lateral wall tissues of the cochlea. These highly metabolic cells provide energy to the cochlear amplifier in the form of electrochemical potentials. This second system is particularly vulnerable to the effects of aging. The third system comprises the inner hair cells and their associated sensory nerve fibers. This system is the transduction stage, changing mechanical vibrations to nerve impulses. New treatments for hearing loss are on the horizon; however, at present the best strategy is avoidance of cochlear trauma and the proper use of hearing aids. [Work supported by NIA and MUSC.

Cochlear perfusion with a viscous fluid.

PubMed

Wang, Yi; Olson, Elizabeth S

2016-07-01

The flow of viscous fluid in the cochlea induces shear forces, which could provide benefit in clinical practice, for example to guide cochlear implant insertion or produce static pressure to the cochlear partition or wall. From a research standpoint, studying the effects of a viscous fluid in the cochlea provides data for better understanding cochlear fluid mechanics. However, cochlear perfusion with a viscous fluid may damage the cochlea. In this work we studied the physiological and anatomical effects of perfusing the cochlea with a viscous fluid. Gerbil cochleae were perfused at a rate of 2.4 μL/min with artificial perilymph (AP) and sodium hyaluronate (Healon, HA) in four different concentrations (0.0625%, 0.125%, 0.25%, 0.5%). The different HA concentrations were applied either sequentially in the same cochlea or individually in different cochleae. The perfusion fluid entered from the round window and was withdrawn from basal scala vestibuli, in order to perfuse the entire perilymphatic space. Compound action potentials (CAP) were measured after each perfusion. After perfusion with increasing concentrations of HA in the order of increasing viscosity, the CAP thresholds generally increased. The threshold elevation after AP and 0.0625% HA perfusion was small or almost zero, and the 0.125% HA was a borderline case, while the higher concentrations significantly elevated CAP thresholds. Histology of the cochleae perfused with the 0.0625% HA showed an intact Reissner's membrane (RM), while in cochleae perfused with 0.125% and 0.25% HA RM was torn. Thus, the CAP threshold elevation was likely due to the broken RM, likely caused by the shear stress produced by the flow of the viscous fluid. Our results and analysis indicate that the cochlea can sustain, without a significant CAP threshold shift, up to a 1.5 Pa shear stress. Beside these finding, in the 0.125% and 0.25% HA perfusion cases, a temporary CAP threshold shift was observed, perhaps due to the presence and then clearance of viscous fluid within the cochlea, or to a temporary position shift of the Organ of Corti. After 0.5% HA perfusion, a short latency positive peak (P0) appeared in the CAP waveform. This P0 might be due to a change in the cochlea's traveling-wave pattern, or distortion in the cochlear microphonic. Copyright © 2016 Elsevier B.V. All rights reserved.

Cochlear perfusion with a viscous fluid

PubMed Central

Wang, Yi; Olson, Elizabeth S.

2016-01-01

The flow of viscous fluid in the cochlea induces shear forces, which could provide benefit in clinical practice, for example to guide cochlear implant insertion or produce static pressure to the cochlear partition or wall. From a research standpoint, studying the effects of a viscous fluid in the cochlea provides data for better understanding cochlear fluid mechanics. However, cochlear perfusion with a viscous fluid may damage the cochlea. In this work we studied the physiological and anatomical effects of perfusing the cochlea with a viscous fluid. Gerbil cochleae were perfused at a rate of 2.4 μL/min with artificial perilymph (AP) and sodium hyaluronate (Healon, HA) in four different concentrations (0.0625%, 0.125%, 0.25%, 0.5%). The different HA concentrations were applied either sequentially in the same cochlea or individually in different cochleae. The perfusion fluid entered from the round window and was withdrawnfrom basal scala vestibuli, in order to perfuse the entire perilymphatic space. Compound action potentials (CAP) were measured after each perfusion. After perfusion with increasing concentrations of HA in the order of increasing viscosity, the CAP thresholds generally increased. The threshold elevation after AP and 0.0625% HA perfusion was small or almost zero, and the 0.125% HA was a borderline case, while the higher concentrations significantly elevated CAP thresholds. Histology of the cochleae perfused with the 0.0625% HA showed an intact Reissner’s membrane, while in cochleae perfused with 0.125% and 0.25% HA Reissner’s membrane (RM) was torn. Thus, the CAP threshold elevation was likely due to the broken of RM, which likely caused by the shear stress produced by the flow of the viscous fluid. Our results and analysis indicate that the cochlea can sustain, without a significant CAP threshold shift, up to a 1.5 Pa shear stress. Beside these finding, in the 0.125% and 0.25% HA perfusion cases, a temporary CAP threshold shift was observed, perhaps due to the presence and then clearance of viscous fluid within the cochlea, or to a temporary position shift of the Organ of Corti. After 0.5% HA perfusion, a short latency positive peak (P0) appeared in the CAP wavefrom. This P0 might be due to a change in the cochlea’s traveling-wave pattern, or distortion in the cochlear microphonic. PMID:27220484

Exposures to fine particulate matter (PM2.5) and ozone above USA standards are associated with auditory brainstem dysmorphology and abnormal auditory brainstem evoked potentials in healthy young dogs.

PubMed

Calderón-Garcidueñas, Lilian; González-González, Luis O; Kulesza, Randy J; Fech, Tatiana M; Pérez-Guillé, Gabriela; Luna, Miguel Angel Jiménez-Bravo; Soriano-Rosales, Rosa Eugenia; Solorio, Edelmira; Miramontes-Higuera, José de Jesús; Gómez-Maqueo Chew, Aline; Bernal-Morúa, Alexia F; Mukherjee, Partha S; Torres-Jardón, Ricardo; Mills, Paul C; Wilson, Wayne J; Pérez-Guillé, Beatriz; D'Angiulli, Amedeo

2017-10-01

Delayed central conduction times in the auditory brainstem have been observed in Mexico City (MC) healthy children exposed to fine particulate matter (PM 2.5 ) and ozone (O 3 ) above the current United States Environmental Protection Agency (US-EPA) standards. MC children have α synuclein brainstem accumulation and medial superior olivary complex (MSO) dysmorphology. The present study used a dog model to investigate the potential effects of air pollution on the function and morphology of the auditory brainstem. Twenty-four dogs living in clean air v MC, average age 37.1 ± 26.3 months, underwent brainstem auditory evoked potential (BAEP) measurements. Eight dogs (4 MC, 4 Controls) were analysed for auditory brainstem morphology and histopathology. MC dogs showed ventral cochlear nuclei hypotrophy and MSO dysmorphology with a significant decrease in cell body size, decreased neuronal packing density with regions in the nucleus devoid of neurons and marked gliosis. MC dogs showed significant delayed BAEP absolute wave I, III and V latencies compared to controls. MC dogs show auditory nuclei dysmorphology and BAEPs consistent with an alteration of the generator sites of the auditory brainstem response waveform. This study puts forward the usefulness of BAEPs to study auditory brainstem neurodegenerative changes associated with air pollution in dogs. Recognition of the role of non-invasive BAEPs in urban dogs is warranted to elucidate novel neurodegenerative pathways link to air pollution and a promising early diagnostic strategy for Alzheimer's Disease. Copyright © 2017 Elsevier Inc. All rights reserved.

Cone-beam computed tomography in children with cochlear implants: The effect of electrode array position on ECAP.

PubMed

Lathuillière, Marine; Merklen, Fanny; Piron, Jean-Pierre; Sicard, Marielle; Villemus, Françoise; Menjot de Champfleur, Nicolas; Venail, Frédéric; Uziel, Alain; Mondain, Michel

2017-01-01

To assess the feasibility of using cone-beam computed tomography (CBCT) in young children with cochlear implants (CIs) and study the effect of intracochlear position on electrophysiological and behavioral measurements. A total of 40 children with either unilateral or bilateral cochlear implants were prospectively included in the study. Electrode placement and insertion angles were studied in 55 Cochlear ® implants (16 straight arrays and 39 perimodiolar arrays), using either CBCT or X-ray imaging. CBCT or X-ray imaging were scheduled when the children were leaving the recovery room. We recorded intraoperative and postoperative neural response telemetry threshold (T-NRT) values, intraoperative and postoperative electrode impedance values, as well as behavioral T (threshold) and C (comfort) levels on electrodes 1, 5, 10, 15 and 20. CBCT imaging was feasible without any sedation in 24 children (60%). Accidental scala vestibuli insertion was observed in 3 out of 24 implants as assessed by CBCT. The mean insertion angle was 339.7°±35.8°. The use of a perimodiolar array led to higher angles of insertion, lower postoperative T-NRT, as well as decreased behavioral T and C levels. We found no significant effect of either electrode array position or angle of insertion on electrophysiological data. CBCT appears to be a reliable tool for anatomical assessment of young children with CIs. Intracochlear position had no significant effect on the electrically evoked compound action potential (ECAP) threshold. Our CBCT protocol must be improved to increase the rate of successful investigations. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Temporal Sensitivity Measured Shortly After Cochlear Implantation Predicts 6-Month Speech Recognition Outcome.

PubMed

Erb, Julia; Ludwig, Alexandra Annemarie; Kunke, Dunja; Fuchs, Michael; Obleser, Jonas

2018-04-24

Psychoacoustic tests assessed shortly after cochlear implantation are useful predictors of the rehabilitative speech outcome. While largely independent, both spectral and temporal resolution tests are important to provide an accurate prediction of speech recognition. However, rapid tests of temporal sensitivity are currently lacking. Here, we propose a simple amplitude modulation rate discrimination (AMRD) paradigm that is validated by predicting future speech recognition in adult cochlear implant (CI) patients. In 34 newly implanted patients, we used an adaptive AMRD paradigm, where broadband noise was modulated at the speech-relevant rate of ~4 Hz. In a longitudinal study, speech recognition in quiet was assessed using the closed-set Freiburger number test shortly after cochlear implantation (t0) as well as the open-set Freiburger monosyllabic word test 6 months later (t6). Both AMRD thresholds at t0 (r = -0.51) and speech recognition scores at t0 (r = 0.56) predicted speech recognition scores at t6. However, AMRD and speech recognition at t0 were uncorrelated, suggesting that those measures capture partially distinct perceptual abilities. A multiple regression model predicting 6-month speech recognition outcome with deafness duration and speech recognition at t0 improved from adjusted R = 0.30 to adjusted R = 0.44 when AMRD threshold was added as a predictor. These findings identify AMRD thresholds as a reliable, nonredundant predictor above and beyond established speech tests for CI outcome. This AMRD test could potentially be developed into a rapid clinical temporal-resolution test to be integrated into the postoperative test battery to improve the reliability of speech outcome prognosis.

Measures of Working Memory Span and Verbal Rehearsal Speed in Deaf Children after Cochlear Implantation

PubMed Central

Pisoni, David B.; Cleary, Miranda

2012-01-01

Large individual differences in spoken word recognition performance have been found in deaf children after cochlear implantation. Recently, Pisoni and Geers (2000) reported that simple forward digit span measures of verbal working memory were significantly correlated with spoken word recognition scores even after potentially confounding variables were statistically controlled for. The present study replicates and extends these initial findings to the full set of 176 participants in the CID cochlear implant study. The pooled data indicate that despite statistical “partialling-out” of differences in chronological age, communication mode, duration of deafness, duration of device use, age at onset of deafness, number of active electrodes, and speech feature discrimination, significant correlations still remain between digit span and several measures of spoken word recognition. Strong correlations were also observed between speaking rate and both forward and backward digit span, a result that is similar to previously reported findings in normalhearing adults and children. The results suggest that perhaps as much as 20% of the currently unexplained variance in spoken word recognition scores may be independently accounted for by individual differences in cognitive factors related to the speed and efficiency with which phonological and lexical representations of spoken words are maintained in and retrieved from working memory. A smaller percentage, perhaps about 7% of the currently unexplained variance in spoken word recognition scores, may be accounted for in terms of working memory capacity. We discuss how these relationships may arise and their contribution to subsequent speech and language development in prelingually deaf children who use cochlear implants. PMID:12612485

Does quality of life depend on speech recognition performance for adult cochlear implant users?

PubMed

Capretta, Natalie R; Moberly, Aaron C

2016-03-01

Current postoperative clinical outcome measures for adults receiving cochlear implants (CIs) consist of testing speech recognition, primarily under quiet conditions. However, it is strongly suspected that results on these measures may not adequately reflect patients' quality of life (QOL) using their implants. This study aimed to evaluate whether QOL for CI users depends on speech recognition performance. Twenty-three postlingually deafened adults with CIs were assessed. Participants were tested for speech recognition (Central Institute for the Deaf word and AzBio sentence recognition in quiet) and completed three QOL measures-the Nijmegen Cochlear Implant Questionnaire; either the Hearing Handicap Inventory for Adults or the Hearing Handicap Inventory for the Elderly; and the Speech, Spatial and Qualities of Hearing Scale questionnaires-to assess a variety of QOL factors. Correlations were sought between speech recognition and QOL scores. Demographics, audiologic history, language, and cognitive skills were also examined as potential predictors of QOL. Only a few QOL scores significantly correlated with postoperative sentence or word recognition in quiet, and correlations were primarily isolated to speech-related subscales on QOL measures. Poorer pre- and postoperative unaided hearing predicted better QOL. Socioeconomic status, duration of deafness, age at implantation, duration of CI use, reading ability, vocabulary size, and cognitive status did not consistently predict QOL scores. For adult, postlingually deafened CI users, clinical speech recognition measures in quiet do not correlate broadly with QOL. Results suggest the need for additional outcome measures of the benefits and limitations of cochlear implantation. 4. Laryngoscope, 126:699-706, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Negotiating Reassurance: Parents' Narratives on Follow-Up after Cochlear Implantation

ERIC Educational Resources Information Center

Bruin, Marieke; Ohna, Stein Erik

2015-01-01

This study presents an analysis of parental experiences on follow-up after cochlear implantation. Data were constructed in semi-structured, individual interviews with the parents of 14 children who use cochlear implants. Drawing on narrative analysis, the study explores parental responses to insecurity concerning children's learning and…

Concept Learning and Heuristic Classification in Weak-Theory Domains

DTIC Science & Technology

1990-03-01

age and noise-induced cochlear age..gt.60 noise-induced cochlear air(mild) age-induced cochlear history(noise) norma ]_ear speechpoor)acousticneuroma...Annual review of computer science. Machine Learning, 4, 1990. (to appear). [18] R.T. Duran . Concept learning with incomplete data sets. Master’s thesis

Challenges in Optimizing Oral Communication in Children with Cochlear Implants.

ERIC Educational Resources Information Center

Ertmer, David J.

2002-01-01

This article introduces a forum that provides information for assisting speech-language pathologists and audiologists in fulfilling their professional responsibilities in a variety of settings to children with hearing impairments who have cochlear implants. A preview of the following articles on services for children with cochlear implants is…

Agile Development of Advanced Prototypes

DTIC Science & Technology

2014-11-01

prostheses: retinal implants, cochlear implants, and neuroprosthetics (EEG controlled artificial limbs); an interactive, virtual experience...demonstrations allowing users to experience, from a patient’s perspective life with three different prostheses: retinal implants, cochlear implants...three experiences were researched and developed. The applications are interactive demonstrations of retinal implants, cochlear implants, and

Voice emotion perception and production in cochlear implant users.

PubMed

Jiam, N T; Caldwell, M; Deroche, M L; Chatterjee, M; Limb, C J

2017-09-01

Voice emotion is a fundamental component of human social interaction and social development. Unfortunately, cochlear implant users are often forced to interface with highly degraded prosodic cues as a result of device constraints in extraction, processing, and transmission. As such, individuals with cochlear implants frequently demonstrate significant difficulty in recognizing voice emotions in comparison to their normal hearing counterparts. Cochlear implant-mediated perception and production of voice emotion is an important but relatively understudied area of research. However, a rich understanding of the voice emotion auditory processing offers opportunities to improve upon CI biomedical design and to develop training programs benefiting CI performance. In this review, we will address the issues, current literature, and future directions for improved voice emotion processing in cochlear implant users. Copyright © 2017 Elsevier B.V. All rights reserved.

Evaluating cochlear implant trauma to the scala vestibuli.

PubMed

Adunka, O; Kiefer, J; Unkelbach, M H; Radeloff, A; Gstoettner, W

2005-04-01

Placement of cochlear implant electrodes into the scala vestibuli may be intentional, e.g. in case of blocked scala tympani or unintentional as a result of trauma to the basilar membrane or erroneous location of the cochieostomy. The aim of this study was to evaluate the morphological consequences and cochlear trauma after implantation of different cochlear implant electrode arrays in the scala vestibuli. Human temporal bone study with histological and radiological evaluation. Twelve human cadaver temporal bones were implanted with different cochlear implant electrodes. Implanted bones were processed using a special method to section undecalcified bone. Cochlear trauma and intracochlear positions. All implanted electrodes were implanted into the scala vestibuli using a special approach that allows direct scala vestibuli insertions. Fractures of the osseous spiral lamina were evaluated in some bones in the basal cochlear regions. In most electrodes, delicate structures of the organ of Corti were left intact, however, Reissner's membrane was destroyed in all specimens and the electrode lay upon the tectorial membrane. In some bones the organ of Corti was destroyed. Scala vestibuli insertions did not cause severe trauma to osseous or neural structures, thus preserving the basis for electrostimulation of the cochlea. However, destruction of Reissner's membrane and impact on the Organ of Corti can be assumed to destroy residual hearing.

Piribedil affects dopamine turnover in cochleas stimulated by white noise.

PubMed

Gil-Loyzaga, P; Vicente-Torres, M A; Fernández-Mateos, P; Arce, A; Esquifino, A

1994-09-01

The presence of dopamine (DA) within the cochlea has been previously reported, indicating that its turnover increases under noise stimulation. In the present report, piribedil, a dopaminergic D2 agonist, was used in order to provide evidence of the activity of D2 receptors in the turnover of DA under noise stimulation. Long-Evans rats were intraperitoneally injected with distilled water or with a solution of piribedil one hour previously to either noise or silence exposure. Noise stimulation was performed in an anechoic chamber at 70, 90 or 110 dB SPL for one hour. The animals were then sacrificed and the cochlear contents of DA and its metabolites dihydroxyphenylacetic acid (DOPAC) and homovanillic acid (HVA) were quantified by HPLC with electrochemical detection. The administration of piribedil to animals kept in silence did not modify the cochlear DA, DOPAC and HVA content. Noise stimulation resulted in a decrease of the cochlear DA content and an increase of the cochlear DOPAC and HVA contents in vehicle treated animals. The administration of piribedil resulted in a blockade of this noise induced cochlear DA turnover. These results suggest that piribedil stimulates cochlear D2 receptors controlling the cochlear DA release. Piribedil action on D2 receptors could explain the improvement observed in some cochleo-vestibular diseases signs after piribedil treatment.

Considerations for Pediatric Cochlear Implant Recipients With Unilateral or Asymmetric Hearing Loss: Assessment, Device Fitting, and Habilitation.

PubMed

Greaver, Laura; Eskridge, Hannah; Teagle, Holly F B

2017-06-13

The purpose of this clinical report is to present case studies of children who are nontraditional candidates for cochlear implantation because they have significant residual hearing in 1 ear and to describe outcomes and considerations for their audiological management and habilitation. Case information is presented for 5 children with profound hearing loss in 1 ear and normal or mild-to-moderate hearing loss in the opposite ear and who have undergone unilateral cochlear implantation. Pre- and postoperative assessments were performed per typical clinic routines with modifications described. Postimplant habilitation was customized for each recipient using a combination of traditional methods, newer technologies, and commercial materials. The 5 children included in this report are consistent users of their cochlear implants and demonstrate speech recognition in the implanted ear when isolated from the better hearing ear. Candidacy criteria for cochlear implantation are evolving. Children with single-sided deafness or asymmetric hearing loss who have traditionally not been considered candidates for cochlear implantation should be evaluated on a case-by-case basis. Audiological management of these recipients is not vastly different compared with children who are traditional cochlear implant recipients. Assessment and habilitation techniques must be modified to isolate the implanted ear to obtain accurate results and to provide meaningful therapeutic intervention.

Spatiotemporal expression of Ezh2 in the developing mouse cochlear sensory epithelium.

PubMed

Chen, Yan; Li, Wenyan; Li, Wen; Chai, Renjie; Li, Huawei

2016-09-01

The enhancer of zeste 2 polycomb repressive complex 2 subunit (Ezh2) is a histone-lysine Nmethyltransferase enzyme that participates in DNA methylation. Ezh2 has also been reported to play crucial roles in stem cell proliferation and differentiation. However, the detailed expression profile of Ezh2 during mouse cochlear development has not been investigated. Here, we examined the spatiotemporal expression of Ezh2 in the cochlea during embryonic and postnatal development. Ezh2 expression began to be observed in the whole otocyst nuclei at embryonic day 9.5 (E9.5). At E12.5, Ezh2 was expressed in the nuclei of the cochlear prosensory epithelium. At E13.5 and E15.5, Ezh2 was expressed from the apical to the basal turns in the nuclei of the differentiating cochlear epithelium. At postnatal day (P) 0 and 7, the Ezh2 expression was located in the nuclei of the cochlear epithelium in all three turns and could be clearly seen in outer and inner hair cells, supporting cells, the stria vascularis, and spiral ganglion cells. Ezh2 continued to be expressed in the cochlear epithelium of adult mice. Our results provide the basic Ezh2 expression pattern and might be useful for further investigating the detailed role of Ezh2 during cochlear development.

[Audiophonological evaluation of 16 children fitted with cochlear implants for sensorineural hearing loss induced by bacterial meningitis].

PubMed

Teissier, N; Doehring, I; Noel-Petroff, N; Elmaleh-Bergès, M; Viala, P; François, M; Faye, A; Van Den Abbeele, T; Lorrot, M

2013-06-01

Bacterial meningitis (BM) is the primary etiology of acquired sensorineural hearing loss (SNHL) in children and may compromise language development. Since the 1990 s, cochlear implants (CIs) have become part of the management of children with profound SNHL with encouraging results. The aim of this study was to analyze the audiophonological performance of children before and after cochlear implantation for SNHL following bacterial meningitis. Retrospective study of all children fitted with CIs for bilateral severe to profound SNHL after bacterial meningitis in the Robert-Debré pediatric ENT department between August 1990 and March 2009. Audiophonological performance was assessed using the APCEI profile. Of the 283 children receiving implants during that period, 16 children (6%; 6 boys, 10 girls) underwent CI implantation after bacterial meningitis (Streptococcus pneumoniae in 8 cases, Neisseria meningitidis in 2 cases, and Haemophilus influenzae in 4 cases). The mean time from meningitis to SNHL was 8.3 months (median, 1.5 months; range, 1 day to 13 years). The mean time from meningitis to cochlear implantation was 2 years and 3 months (median, 7 months; range, 1 month to 13 years 3 months). Twelve children (75%) presented partial cochlear and/or vestibular ossification on presurgical CT scan. Three children received bilateral implants. Thirteen children (81%) developed early SNHL in the first 3 months, whereas 3 children developed SNHL more than 10 months after meningitis. As for the benefits of cochlear implantation, 11 children presented near to normal intelligibility and optimal use of their cochlear implant; 5 children presented partial benefits due to neurological sequelae (1), a long delay before implantation (1), technical problems (2), or a social problem in relation to low socioeconomic status (1). After bacterial meningitis, audiological evaluation must be made carefully during the first 3 months to detect early SNHL, but SNHL may also develop several years later. In case of profound SNHL and a modified signal of the labyrinth on the MRI, cochlear implantation must be performed without delay before cochlear and/or vestibular ossification. Cochlear implantation is an effective technique with good long-term audiologic results. The coexistence of neurological lesions may compromise the results, but it should not contraindicate a cochlear implantation. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Modeling open-set spoken word recognition in postlingually deafened adults after cochlear implantation: some preliminary results with the neighborhood activation model.

PubMed

Meyer, Ted A; Frisch, Stefan A; Pisoni, David B; Miyamoto, Richard T; Svirsky, Mario A

2003-07-01

Do cochlear implants provide enough information to allow adult cochlear implant users to understand words in ways that are similar to listeners with acoustic hearing? Can we use a computational model to gain insight into the underlying mechanisms used by cochlear implant users to recognize spoken words? The Neighborhood Activation Model has been shown to be a reasonable model of word recognition for listeners with normal hearing. The Neighborhood Activation Model assumes that words are recognized in relation to other similar-sounding words in a listener's lexicon. The probability of correctly identifying a word is based on the phoneme perception probabilities from a listener's closed-set consonant and vowel confusion matrices modified by the relative frequency of occurrence of the target word compared with similar-sounding words (neighbors). Common words with few similar-sounding neighbors are more likely to be selected as responses than less common words with many similar-sounding neighbors. Recent studies have shown that several of the assumptions of the Neighborhood Activation Model also hold true for cochlear implant users. Closed-set consonant and vowel confusion matrices were obtained from 26 postlingually deafened adults who use cochlear implants. Confusion matrices were used to represent input errors to the Neighborhood Activation Model. Responses to the different stimuli were then generated by the Neighborhood Activation Model after incorporating the frequency of occurrence counts of the stimuli and their neighbors. Model outputs were compared with obtained performance measures on the Consonant-Vowel Nucleus-Consonant word test. Information transmission analysis was used to assess whether the Neighborhood Activation Model was able to successfully generate and predict word and individual phoneme recognition by cochlear implant users. The Neighborhood Activation Model predicted Consonant-Vowel Nucleus-Consonant test words at levels similar to those correctly identified by the cochlear implant users. The Neighborhood Activation Model also predicted phoneme feature information well. The results obtained suggest that the Neighborhood Activation Model provides a reasonable explanation of word recognition by postlingually deafened adults after cochlear implantation. It appears that multichannel cochlear implants give cochlear implant users access to their mental lexicons in a manner that is similar to listeners with acoustic hearing. The lexical properties of the test stimuli used to assess performance are important to spoken-word recognition and should be included in further models of the word recognition process.

Psychophysical Map Stability in Bilateral Sequential Cochlear Implantation: Comparing Current Audiology Methods to a New Statistical Definition.

PubMed

Domville-Lewis, Chloe; Santa Maria, Peter L; Upson, Gemma; Chester-Browne, Ronel; Atlas, Marcus D

2015-01-01

The purpose of this study was to establish a statistical definition for stability in cochlear implant maps. Once defined, this study aimed to compare the duration taken to achieve a stable map in first and second implants in patients who underwent sequential bilateral cochlear implantation. This article also sought to evaluate a number of factors that potentially affect map stability. A retrospective cohort study of 33 patients with sensorineural hearing loss who received sequential bilateral cochlear implantation (Cochlear, Sydney, Australia), performed by the senior author. Psychophysical parameters of hearing threshold scores, comfort scores, and the dynamic range were measured for the apical, medial, and basal portions of the cochlear implant electrode at a range of intervals postimplantation. Stability was defined statistically as a less than 10% difference in threshold, comfort, and dynamic range scores over three consecutive mapping sessions. A senior cochlear implant audiologist, blinded to implant order and the statistical results, separately analyzed these psychophysical map parameters using current assessment methods. First and second implants were compared for duration to achieve stability, age, gender, the duration of deafness, etiology of deafness, time between the insertion of the first and second implant, and the presence or absence of preoperative hearing aids were evaluated and its relationship to stability. Statistical analysis included performing a two-tailed Student's t tests and least squares regression analysis, with a statistical significance set at p ≤ 0.05. There was a significant positive correlation between the devised statistical definition and the current audiology methods for assessing stability, with a Pearson correlation coefficient r = 0.36 and a least squares regression slope (b) of 0.41, df(58), 95% confidence interval 0.07 to 0.55 (p = 0.004). The average duration from device switch on to stability in the first implant was 87 days using current audiology methods and 81 days using the statistical definition, with no statistically significant difference between assessment methods (p = 0.2). The duration to achieve stability in the second implant was 51 days using current audiology methods and 60 days using the statistical method, and again no difference between the two assessment methods (p = 0.13). There was a significant reduction in the time to achieve stability in second implants for both audiology and statistical methods (p < 0.001 and p = 0.02, respectively). There was a difference in duration to achieve stability based on electrode array region, with basal portions taking longer to stabilize than apical in the first implant (p = 0.02) and both apical and medial segments in second implants (p = 0.004 and p = 0.01, respectively). No factors that were evaluated in this study, including gender, age, etiology of deafness, duration of deafness, time between implant insertion, and the preoperative hearing aid status, were correlated with stability duration in either stability assessment method. Our statistical definition can accurately predict cochlear implant map stability when compared with current audiology practices. Cochlear implants that are implanted second tend to stabilize sooner than the first, which has a significant impact on counseling before a second implant. No factors evaluated affected the duration required to achieve stability in this study.

Electrically evoked compound action potentials are different depending on the site of cochlear stimulation.

PubMed

van de Heyning, Paul; Arauz, Santiago L; Atlas, Marcus; Baumgartner, Wolf-Dieter; Caversaccio, Marco; Chester-Browne, Ronel; Estienne, Patricia; Gavilan, Javier; Godey, Benoit; Gstöttner, Wolfgang; Han, Demin; Hagen, Rudolph; Kompis, Martin; Kuzovkov, Vlad; Lassaletta, Luis; Lefevre, Franc; Li, Yongxin; Müller, Joachim; Parnes, Lorne; Kleine Punte, Andrea; Raine, Christopher; Rajan, Gunesh; Rivas, Adriana; Rivas, José Antonio; Royle, Nicola; Sprinzl, Georg; Stephan, Kurt; Walkowiak, Adam; Yanov, Yuri; Zimmermann, Kim; Zorowka, Patrick; Skarzynski, Henryk

2016-11-01

One of the many parameters that can affect cochlear implant (CI) users' performance is the site of presentation of electrical stimulation, from the CI, to the auditory nerve. Evoked compound action potential (ECAP) measurements are commonly used to verify nerve function by stimulating one electrode contact in the cochlea and recording the resulting action potentials on the other contacts of the electrode array. The present study aimed to determine if the ECAP amplitude differs between the apical, middle, and basal region of the cochlea, if double peak potentials were more likely in the apex than the basal region of the cochlea, and if there were differences in the ECAP threshold and recovery function across the cochlea. ECAP measurements were performed in the apical, middle, and basal region of the cochlea at fixed sites of stimulation with varying recording electrodes. One hundred and forty one adult subjects with severe to profound sensorineural hearing loss fitted with a Standard or FLEX SOFT electrode were included in this study. ECAP responses were captured using MAESTRO System Software (MED-EL). The ECAP amplitude, threshold, and slope were determined using amplitude growth sequences. The 50% recovery rate was assessed using independent single sequences that have two stimulation pulses (a masker and a probe pulse) separated by a variable inter-pulse interval. For all recordings, ECAP peaks were annotated semi-automatically. ECAP amplitudes were greater upon stimulation of the apical region compared to the basal region of the cochlea. ECAP slopes were steeper in the apical region compared to the basal region of the cochlea and ECAP thresholds were lower in the middle region compared to the basal region of the cochlea. The incidence of double peaks was greater upon stimulation of the apical region compared to the basal region of the cochlea. This data indicates that the site and intensity of cochlear stimulation affect ECAP properties.

The molecular genetics of Usher syndrome.

PubMed

Ahmed, Z M; Riazuddin, S; Riazuddin, S; Wilcox, E R

2003-06-01

Association of sensorineural deafness and progressive retinitis pigmentosa with and without a vestibular abnormality is the hallmark of Usher syndrome and involves at least 12 loci among three different clinical subtypes. Genes identified for the more commonly inherited loci are USH2A (encoding usherin), MYO7A (encoding myosin VIIa), CDH23 (encoding cadherin 23), PCDH15 (encoding protocadherin 15), USH1C (encoding harmonin), USH3A (encoding clarin 1), and USH1G (encoding SANS). Transcripts from all these genes are found in many tissues/cell types other than the inner ear and retina, but all are uniquely critical for retinal and cochlear cell function. Many of these protein products have been demonstrated to have direct interactions with each other and perform an essential role in stereocilia homeostasis.

Direct testing of the biasing effect of manipulations of endolymphatic pressure on cochlear mechanical function

NASA Astrophysics Data System (ADS)

LePage, Eric; Avan, Paul

2015-12-01

The history of cochlear mechanical investigations has been carried out in two largely separate sets of endeavours; those interested in auditory processing in animal models and those interested in the origin of adverse vestibular symptoms in humans. In respect of the first, mechanical vibratory data is considered pathological and not representative of pristine behaviour if it departs from the reigning model of sharp tuning and high hearing sensitivity. Conversely, when the description of the pathological behaviour is the focus, fluid movements responsible for hearing loss and vestibular symptoms dominate. Yet both extensive sets of data possess a common factor now being reconsidered for its potential to shed light on the mechanisms in general. The common factor is a mechanical bias — the departure of cochlear epithelial membranes from their usual resting position. In both cases the bias modulates hearing sensitivity and distorts tuning characteristics. Indeed several early sets of guinea pig mechanical data were dismissed as "pathological" when in hindsight, the primary effect influencing the data was not loss of outer hair cell function per se, but a mechanical bias unknowingly introduced in process of making the measurement. Such biases in the displacement of the basilar membrane from its position are common, and may be caused by low-frequency sounds (topically including infrasound) or by variations in fluid volume in the chambers particularly applying the case of endolymphatic hydrops. Most biases are quantified in terms of visualisation of fluid volume change, electric potential changes and otoacoustic emissions. Notably many previous studies have also searched for raised pressures with negative results. Yet these repeated findings are contrary to the widespread notion that, at least when homeostasis is lost, it is a rise in endolymphatic pressure which is responsible for membrane rupture and Meniere's attacks. This current investigation in Mongolian gerbils is aimed at quantifying hydrostatic pressures in cochlear chambers by direct measurement using a null-flow micropipette pressure measurement system, while simultaneously quantifying electric potentials and distortion products to provide indirect measures of displacement bias and hair cell integrity. We now suspect that during any experiment obtaining of good pressure seals is critical. Secondary penetrations, such as occur in neural recordings, are contra-indicated. When we address the issue of seals we see raised pressures in response to manipulations known to disturb homeostasis, viz. diuretics and hypoxia.

Recognition and Localization of Speech by Adult Cochlear Implant Recipients Wearing a Digital Hearing Aid in the Nonimplanted Ear (Bimodal Hearing)

PubMed Central

Potts, Lisa G.; Skinner, Margaret W.; Litovsky, Ruth A.; Strube, Michael J; Kuk, Francis

2010-01-01

Background The use of bilateral amplification is now common clinical practice for hearing aid users but not for cochlear implant recipients. In the past, most cochlear implant recipients were implanted in one ear and wore only a monaural cochlear implant processor. There has been recent interest in benefits arising from bilateral stimulation that may be present for cochlear implant recipients. One option for bilateral stimulation is the use of a cochlear implant in one ear and a hearing aid in the opposite nonimplanted ear (bimodal hearing). Purpose This study evaluated the effect of wearing a cochlear implant in one ear and a digital hearing aid in the opposite ear on speech recognition and localization. Research Design A repeated-measures correlational study was completed. Study Sample Nineteen adult Cochlear Nucleus 24 implant recipients participated in the study. Intervention The participants were fit with a Widex Senso Vita 38 hearing aid to achieve maximum audibility and comfort within their dynamic range. Data Collection and Analysis Soundfield thresholds, loudness growth, speech recognition, localization, and subjective questionnaires were obtained six–eight weeks after the hearing aid fitting. Testing was completed in three conditions: hearing aid only, cochlear implant only, and cochlear implant and hearing aid (bimodal). All tests were repeated four weeks after the first test session. Repeated-measures analysis of variance was used to analyze the data. Significant effects were further examined using pairwise comparison of means or in the case of continuous moderators, regression analyses. The speech-recognition and localization tasks were unique, in that a speech stimulus presented from a variety of roaming azimuths (140 degree loudspeaker array) was used. Results Performance in the bimodal condition was significantly better for speech recognition and localization compared to the cochlear implant–only and hearing aid–only conditions. Performance was also different between these conditions when the location (i.e., side of the loudspeaker array that presented the word) was analyzed. In the bimodal condition, the speech-recognition and localization tasks were equal regardless of which side of the loudspeaker array presented the word, while performance was significantly poorer for the monaural conditions (hearing aid only and cochlear implant only) when the words were presented on the side with no stimulation. Binaural loudness summation of 1–3 dB was seen in soundfield thresholds and loudness growth in the bimodal condition. Measures of the audibility of sound with the hearing aid, including unaided thresholds, soundfield thresholds, and the Speech Intelligibility Index, were significant moderators of speech recognition and localization. Based on the questionnaire responses, participants showed a strong preference for bimodal stimulation. Conclusions These findings suggest that a well-fit digital hearing aid worn in conjunction with a cochlear implant is beneficial to speech recognition and localization. The dynamic test procedures used in this study illustrate the importance of bilateral hearing for locating, identifying, and switching attention between multiple speakers. It is recommended that unilateral cochlear implant recipients, with measurable unaided hearing thresholds, be fit with a hearing aid. PMID:19594084

Timing Discrepancies of Early Intervention Hearing Services in Urban and Rural Cochlear Implant Recipients

PubMed Central

Bush, Matthew L.; Burton, Mary; Loan, Ashley; Shinn, Jennifer B.

2013-01-01

Objective The purpose of this study was to examine the timing of early intervention diagnostic and therapeutic services in cochlear implant recipients from rural and urban areas. Study design Retrospective case series review Setting Tertiary referral center Patients Cochlear implant recipients from a single comprehensive hearing institute born with severe congenital sensorineural hearing loss were examined. Timing of diagnostic and therapeutic services was examined. Intervention(s) Diagnosis, amplification, and eventual cochlear implantation for all patients in the study Main outcome measure(s) Time points of definitive diagnosis, amplification, and cochlear implantation for children from urban and rural regions were examined. Correlation analysis of distance to testing center and timing of services was also assessed. Results 40 children born with congenital hearing loss were included in the study and were diagnosed at a median age of 13 weeks after birth. Children from rural regions obtained amplification at a median age of 47.7 weeks after birth, while urban children were amplified at 26 weeks after birth. Cochlear implantation was performed at a median age of 182 weeks after birth in those from rural areas and at 104 weeks after birth in urban-dwelling patients. A linear relationship was identified between distance to the implant center and timing of hearing aid amplification (r=0.5, p=0.033) and cochlear implantation (r=0.5, p=0.016). Conclusions Children residing outside of metro areas may be at higher risk of delayed rehabilitative services and cochlear implantation than those residing in urban areas that may be closer in proximity to tertiary care centers. PMID:24136305

Quality-of-life benefit from cochlear implantation in the elderly.

PubMed

Vermeire, Katrien; Brokx, Jan P L; Wuyts, Floris L; Cochet, Ellen; Hofkens, Anouk; Van de Heyning, Paul H

2005-03-01

To compare the audiologic results of geriatric patients receiving cochlear implants with younger age groups and to evaluate the quality of life after cochlear implantation in the geriatric population by means of validated quality-of-life questionnaires. Cross-sectional study involving 89 postlingually deafened cochlear implant subjects. Tertiary referral center. A total of 89 postlingually deafened patients were included in the study, among which were 25 patients who were aged 70 years or older. All patients received a cochlear implant. Subjects were implanted with either the Laura, Nucleus 24, or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, CIS, or CIS+ coding strategies. Speech recognition was determined by means of phonetically balanced monosyllabic word lists. The Hearing Handicap Inventory for Adults, the Glasgow Benefit Inventory, and the scale for the prediction of hearing disability in sensorineural hearing loss were used to quantify the quality of life. Mean audiologic performance for the three groups increased significantly after implantation (p < 0.001). Postoperative audiologic performance of the geriatric population led to useful hearing, but these scores were significantly lower than for the younger age groups (p = 0.002). However, the quality-of-life outcomes for the geriatric group were similar to those of the younger age groups (p = 0.411 for the Hearing Handicap Inventory for Adults; p = 0.886 for the Glasgow Benefit Inventory). The results of this study prove that cochlear implantation in the elderly provides improvements in quality of life and speech understanding, similar to those for younger adult cochlear implant recipients.

EEG frontal asymmetry related to pleasantness of music perception in healthy children and cochlear implanted users.

PubMed

Vecchiato, G; Maglione, A G; Scorpecci, A; Malerba, P; Marsella, P; Di Francesco, G; Vitiello, S; Colosimo, A; Babiloni, Fabio

2012-01-01

Interestingly, the international debate about the quality of music fruition for cochlear implanted users does not take into account the hypothesis that bilateral users could perceive music in a more pleasant way with respect to monolateral users. In this scenario, the aim of the present study was to investigate if cerebral signs of pleasantness during music perception in healthy child are similar to those observed in monolateral and in bilateral cochlear implanted users. In fact, previous observations in literature on healthy subjects have indicated that variations of the frontal EEG alpha activity are correlated with the perceived pleasantness of the sensory stimulation received (approach-withdrawal theory). In particular, here we described differences between cortical activities estimated in the alpha frequency band for a healthy child and in patients having a monolateral or a bilateral cochlear implant during the fruition of a musical cartoon. The results of the present analysis showed that the alpha EEG asymmetry patterns observed in a healthy child and that of a bilateral cochlear implanted patient are congruent with the approach-withdrawal theory. Conversely, the scalp topographic distribution of EEG power spectra in the alpha band resulting from the monolateral cochlear user presents a different EEG pattern from the normal and bilateral implanted patients. Such differences could be explained at the light of the approach-withdrawal theory. In fact, the present findings support the hypothesis that a monolateral cochlear implanted user could perceive the music in a less pleasant way when compared to a healthy subject or to a bilateral cochlear user.

Educational, employment, and social participation of young adult graduates from the paediatric Southern Cochlear Implant Programme, New Zealand.

PubMed

Goh, Terence; Bird, Philip; Pearson, John; Mustard, Jill

2016-01-01

The purpose of this study is to observe the education and vocational achievements and social participation of cochlear implant recipients as they graduate from a paediatric cochlear implant programme and identify any significant associations that might exist. This study identified 56 patients from the Southern Cochlear Implant Programme (SCIP) who received cochlear implants before the age of 19 (paediatric) and are now over the age of 19 (adult). A questionnaire investigated their education, employment, and identity with the hearing and deaf communities. Also included were the satisfaction with life scale and Hearing Participation Scale (HPS). Subjects ranged in age from 19 to 32. Twenty-six patients responded to the questionnaire, including one non-user. Twenty identified strongly or very strongly with the hearing community. There was weak evidence of a linear association between strong identity with the hearing community and a higher HPS score. No other statistically significant associations were detected. Interestingly, 12 out of 26 participants found employment through family. Positive outcome trends in education and employment were seen in this study although no statistical significance was achieved. There is a strong bias for those who use their cochlear implants regularly, and there are no data available for those who do not use their cochlear implants for comparison as only one non-user completed the survey, despite efforts to include this group. This study shows that there is perceived benefit in implantation for patients who use it regularly but further research is needed with a more diverse group of cochlear implant recipients.

[Digital signal processing of a novel neuron discharge model stimulation strategy for cochlear implants].

PubMed

Yang, Yiwei; Xu, Yuejin; Miu, Jichang; Zhou, Linghong; Xiao, Zhongju

2012-10-01

To apply the classic leakage integrate-and-fire models, based on the mechanism of the generation of physiological auditory stimulation, in the information processing coding of cochlear implants to improve the auditory result. The results of algorithm simulation in digital signal processor (DSP) were imported into Matlab for a comparative analysis. Compared with CIS coding, the algorithm of membrane potential integrate-and-fire (MPIF) allowed more natural pulse discharge in a pseudo-random manner to better fit the physiological structures. The MPIF algorithm can effectively solve the problem of the dynamic structure of the delivered auditory information sequence issued in the auditory center and allowed integration of the stimulating pulses and time coding to ensure the coherence and relevance of the stimulating pulse time.

Local gene transfection in the cochlea (Review).

PubMed

Xia, Li; Yin, Shankai

2013-07-01

There is much interest in the potential application of vector-induced gene therapeutic approaches to several forms of hearing disorders due to the poor efficacy of existing treatments. The cochlea is an ideal site for local gene transfection due to its anatomical encapsulation and fluid flow within its ducts. However, this requires the development of novel technologies in materials science and microbial supply vectors for target gene delivery. This review focuses on the introduction of various viral and non-viral vectors as well as injection approaches to transfecting cochlear cells in vivo. Finally, the perspective of local gene therapy was discussed. Therapeutic approaches using local gene transfection may provide a means of cochlear cell and tissue protection and treatment in cases of exogenous hearing loss and endogenous disorders.

Pediatric Cochlear Implants: An Overview of the Alternatives in Education and Rehabilitation.

ERIC Educational Resources Information Center

Barnes, Judith M., Ed.; And Others

This collection of 10 papers is intended as a resource book integrating information on pediatric cochlear implants for educators, parents, implant teams, and rehabilitation specialists. The papers focus on the importance of providing an oral educational environment to maximize cochlear implant success. An introduction notes controversies in the…

Realization of Complex Onsets by Pediatric Users of Cochlear Implants

ERIC Educational Resources Information Center

Chin, Steven B.

2006-01-01

This study examined variations in English complex onset realizations by children who use cochlear implants. Data consisted of 227 productions of two-segment onset clusters from 12 children. In general, onset cluster realizations of children with cochlear implants did not differ markedly from those reported for children with normal hearing: null…

Contribution of Family Environment to Pediatric Cochlear Implant Users' Speech and Language Outcomes: Some Preliminary Findings

ERIC Educational Resources Information Center

Holt, Rachael Frush; Beer, Jessica; Kronenberger, William G.; Pisoni, David B.; Lalonde, Kaylah

2012-01-01

Purpose: To evaluate the family environments of children with cochlear implants and to examine relationships between family environment and postimplant language development and executive function. Method: Forty-five families of children with cochlear implants completed a self-report family environment questionnaire (Family Environment Scale-Fourth…

Taxonomic Knowledge of Children with and without Cochlear Implants

ERIC Educational Resources Information Center

Lund, Emily; Dinsmoor, Jessica

2016-01-01

Purpose: The purpose of this study was to compare the taxonomic vocabulary knowledge and organization of children with cochlear implants to (a) children with normal hearing matched for age, and (b) children matched for vocabulary development. Method: Ten children with cochlear implants, 10 age-matched children with normal hearing, and 10…

Children with Cochlear Implants in Australia: Educational Settings, Supports, and Outcomes

ERIC Educational Resources Information Center

Punch, Renee; Hyde, Merv

2010-01-01

This Australian study examined the communication, academic, and social outcomes of pediatric cochlear implantation from the perspectives of teachers working with children with cochlear implants. The children were aged from 1 to 18 years and attended a range of educational settings in early intervention, primary, and secondary schooling. One…

Cochlear Implants in Children with Hearing Loss: Maternal Expectations and Impact on the Family

ERIC Educational Resources Information Center

Zaidman-Zait, Anat; Most, Tova

2005-01-01

To facilitate evaluations of cochlear implant candidates and to promote (re)habilitation efficacy and collaboration with families, this study examined the expectations of 35 mothers with typical hearing and their beliefs and difficulties related to their child's hearing loss and current or future cochlear implantation. Questionnaires measured…

Persistent Language Delay versus Late Language Emergence in Children with Early Cochlear Implantation

ERIC Educational Resources Information Center

Geers, Ann E.; Nicholas, Johanna; Tobey, Emily; Davidson, Lisa

2016-01-01

Purpose: The purpose of the present investigation is to differentiate children using cochlear implants (CIs) who did or did not achieve age-appropriate language scores by mid-elementary grades and to identify risk factors for persistent language delay following early cochlear implantation. Materials and Method: Children receiving unilateral CIs at…

Serving Deaf Students Who Have Cochlear Implants. PEPNet Tipsheet

ERIC Educational Resources Information Center

Searls, J. Matt, Comp.

2010-01-01

Cochlear implants (CIs) are complex electronic devices surgically implanted under the skin behind the ear. These devices utilize electrodes placed in the inner ear (the cochlea) to stimulate the auditory nerve of individuals with significant permanent hearing loss. Cochlear implants may not be suitable for everyone. They are designed to provide…

Verbal Working Memory in Children with Cochlear Implants

ERIC Educational Resources Information Center

Nittrouer, Susan; Caldwell-Tarr, Amanda; Low, Keri E.; Lowenstein, Joanna H.

2017-01-01

Purpose: Verbal working memory in children with cochlear implants and children with normal hearing was examined. Participants: Ninety-three fourth graders (47 with normal hearing, 46 with cochlear implants) participated, all of whom were in a longitudinal study and had working memory assessed 2 years earlier. Method: A dual-component model of…

Emotion Understanding in Deaf Children with a Cochlear Implant

ERIC Educational Resources Information Center

Wiefferink, Carin H.; Rieffe, Carolien; Ketelaar, Lizet; De Raeve, Leo; Frijns, Johan H. M.

2013-01-01

It is still largely unknown how receiving a cochlear implant affects the emotion understanding in deaf children. We examined indices for emotion understanding and their associations with communication skills in children aged 2.5-5 years, both hearing children (n = 52) and deaf children with a cochlear implant (n = 57). 2 aspects of emotion…

Evaluating the Feasibility of Using Remote Technology for Cochlear Implants

ERIC Educational Resources Information Center

Goehring, Jenny L.; Hughes, Michelle L.; Baudhuin, Jacquelyn L.

2012-01-01

The use of remote technology to provide cochlear implant services has gained popularity in recent years. This article contains a review of research evaluating the feasibility of remote service delivery for recipients of cochlear implants. To date, published studies have determined that speech-processor programming levels and other objective tests…

Identifying cochlear implant channels with poor electrode-neuron interfaces: electrically evoked auditory brain stem responses measured with the partial tripolar configuration.

PubMed

Bierer, Julie Arenberg; Faulkner, Kathleen F; Tremblay, Kelly L

2011-01-01

The goal of this study was to compare cochlear implant behavioral measures and electrically evoked auditory brain stem responses (EABRs) obtained with a spatially focused electrode configuration. It has been shown previously that channels with high thresholds, when measured with the tripolar configuration, exhibit relatively broad psychophysical tuning curves. The elevated threshold and degraded spatial/spectral selectivity of such channels are consistent with a poor electrode-neuron interface, defined as suboptimal electrode placement or reduced nerve survival. However, the psychophysical methods required to obtain these data are time intensive and may not be practical during a clinical mapping session, especially for young children. Here, we have extended the previous investigation to determine whether a physiological approach could provide a similar assessment of channel functionality. We hypothesized that, in accordance with the perceptual measures, higher EABR thresholds would correlate with steeper EABR amplitude growth functions, reflecting a degraded electrode-neuron interface. Data were collected from six cochlear implant listeners implanted with the HiRes 90k cochlear implant (Advanced Bionics). Single-channel thresholds and most comfortable listening levels were obtained for stimuli that varied in presumed electrical field size by using the partial tripolar configuration, for which a fraction of current (σ) from a center active electrode returns through two neighboring electrodes and the remainder through a distant indifferent electrode. EABRs were obtained in each subject for the two channels having the highest and lowest tripolar (σ = 1 or 0.9) behavioral threshold. Evoked potentials were measured with both the monopolar (σ = 0) and a more focused partial tripolar (σ ≥ 0.50) configuration. Consistent with previous studies, EABR thresholds were highly and positively correlated with behavioral thresholds obtained with both the monopolar and partial tripolar configurations. The Wave V amplitude growth functions with increasing stimulus level showed the predicted effect of shallower growth for the partial tripolar than for the monopolar configuration, but this was observed only for the low-threshold channels. In contrast, high-threshold channels showed the opposite effect; steeper growth functions were seen for the partial tripolar configuration. These results suggest that behavioral thresholds or EABRs measured with a restricted stimulus can be used to identify potentially impaired cochlear implant channels. Channels having high thresholds and steep growth functions would likely not activate the appropriate spatially restricted region of the cochlea, leading to suboptimal perception. As a clinical tool, quick identification of impaired channels could lead to patient-specific mapping strategies and result in improved speech and music perception.

Scala tympani cochleostomy I: results of a survey.

PubMed

Adunka, Oliver F; Buchman, Craig A

2007-12-01

To assess current surgical techniques for scala tympani cochlear implantation among North American surgeons. A survey was distributed to all cochlear implant surgeons participating in the 2006 William House Cochlear Implant Study Group in Toronto, Canada. Participants were asked to anonymously identify their routine surgical practices. Images of trans-facial recess approaches to the round window and cochlear promontory were used in a multiple-choice fashion to assess the surgeon's typical exposure and cochleostomy location. Returned questionnaires were electronically processed and evaluated. Fifty-five (75%) of 73 returned surveys had adequate data validity and availability. Landmark identification and preferred cochleostomy locations varied greatly. About 20% of surgeons selected cochleostomy locations superior to the round window membrane. Cochleostomy size and location appeared to be influenced by surgical experience and whether or not the round window overhang was drilled off. This survey clearly documents marked variations in surgical techniques for scala tympani cochlear implantation. Future studies should more clearly define the surgical anatomy of this region for appropriate placement of a scala tympani cochleostomy. These findings may ultimately have an impact on hearing and neural preservation cochlear implant surgeries.

Comparison of intelligence quotients of first- and second-generation deaf children with cochlear implants.

PubMed

Amraei, K; Amirsalari, S; Ajalloueyan, M

2017-01-01

Hearing impairment is a common type of sensory loss in children. Studies indicate that children with hearing impairment are deficient in social, cognitive and communication skills. This study compared the intelligence quotients of first- and second-generation deaf children with cochlear implants. This research is causal-comparative. All 15 deaf children investigated had deaf parents and were selected from Baqiyatallah Cochlear Implant Center. The 15 children with cochlear implants were paired with similar children with hearing parents using purposive sampling. The findings show that the Hotelling trace of multivariate analysis of variance (F = 6.78, p < 0.01, η P 2  = 0.73) was significant. The tests of between-subjects effects for second-generation children was significantly higher than for first-generation children for all intelligence scales except knowledge. It can be assumed that second-generation children joined their family in the use of sign language as the primary experience before a cochlear implant. The use of sign language before cochlear implants is recommended. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Cochlear Implants and Psychiatric Assessments: a Norrie Disease Case Report.

PubMed

Jacques, Denis; Dubois, Thomas; Zdanowicz, Nicolas; Gilain, Chantal; Garin, Pierre

2017-09-01

It is important to perform psychiatric assessments of adult patients who are candidates for cochlear implants both to screen them for psychiatric disorders and to assess their understanding and compliance with the procedure. Deafness is a factor of difficulty for conducting in-depth psychiatric interviews, but concomitant blindness may make it impossible. After a description of Norrie disease, a rare disease in which blindness and deafness may occur together, we propose a case report of a patient suffering from the disease and who consulted in view of a cochlear implant. Early information on cochlear implants appears to be necessary before total deafness occurs in patients suffering from Norrie disease. An inventory of digital communication tools that can be used by the patient is also highly valuable. Research should be supported for a more systematic use of psychiatric assessments prior to cochlear implants. In the special case of Norrie disease, we recommend early screening for mental retardation and related psychotic disorders and, depending on the patient's level of understanding, preventive information on the benefits and limits of cochlear implants before total deafness occurs.

Music activities and responses of young cochlear implant recipients.

PubMed

van Besouw, Rachel M; Grasmeder, Mary L; Hamilton, Mary E; Baumann, Sarah E

2011-05-01

The development of auditory receptive skills and spoken language is often delayed in children who use cochlear implants, which may affect their appreciation of and responses to music. This in turn may be interpreted as disinterest in music. A questionnaire was developed to determine whether differences in exposure and responses to music exist between young cochlear implant recipients and their normally hearing peers. The questionnaire was developed by a multidisciplinary team and distributed to parents of preschool children with normal hearing and to parents of preschool children who had been implanted at least one year prior. The cochlear implant group comprised 23 children and was gender and age matched (within ±2 months) to a group of children with normal hearing. Young cochlear implant recipients receive similar exposure to audiovisual music media, parental singing and musical instruments at home. However, the data suggest that they receive less exposure to children's music presented without visual stimuli. Parents also reported less sophisticated responses to music for this group. The findings of this study have important implications concerning the provision of age-appropriate music habilitation materials and activities for young cochlear implant recipients.

Evaluation of a cochlear-implant processing strategy incorporating phantom stimulation and asymmetric pulses

PubMed Central

Monstrey, Jolijn; Deeks, John M.; Macherey, Olivier

2014-01-01

Objective To evaluate a speech-processing strategy in which the lowest frequency channel is conveyed using an asymmetric pulse shape and “phantom stimulation”, where current is injected into one intra-cochlear electrode and where the return current is shared between an intra-cochlear and an extra-cochlear electrode. This strategy is expected to provide more selective excitation of the cochlear apex, compared to a standard strategy where the lowest-frequency channel is conveyed by symmetric pulses in monopolar mode. In both strategies all other channels were conveyed by monopolar stimulation. Design Within-subjects comparison between the two strategies. Four experiments: (1) discrimination between the strategies, controlling for loudness differences, (2) consonant identification, (3) recognition of lowpass-filtered sentences in quiet, (4) sentence recognition in the presence of a competing speaker. Study sample Eight users of the Advanced Bionics CII/Hi-Res 90k cochlear implant. Results Listeners could easily discriminate between the two strategies but no consistent differences in performance were observed. Conclusions The proposed method does not improve speech perception, at least in the short term. PMID:25358027

Evaluation of a cochlear-implant processing strategy incorporating phantom stimulation and asymmetric pulses.

PubMed

Carlyon, Robert P; Monstrey, Jolijn; Deeks, John M; Macherey, Olivier

2014-12-01

To evaluate a speech-processing strategy in which the lowest frequency channel is conveyed using an asymmetric pulse shape and "phantom stimulation", where current is injected into one intra-cochlear electrode and where the return current is shared between an intra-cochlear and an extra-cochlear electrode. This strategy is expected to provide more selective excitation of the cochlear apex, compared to a standard strategy where the lowest-frequency channel is conveyed by symmetric pulses in monopolar mode. In both strategies all other channels were conveyed by monopolar stimulation. Within-subjects comparison between the two strategies. Four experiments: (1) discrimination between the strategies, controlling for loudness differences, (2) consonant identification, (3) recognition of lowpass-filtered sentences in quiet, (4) sentence recognition in the presence of a competing speaker. Eight users of the Advanced Bionics CII/Hi-Res 90k cochlear implant. Listeners could easily discriminate between the two strategies but no consistent differences in performance were observed. The proposed method does not improve speech perception, at least in the short term.

Bringing Hearing to the Deaf

DOE Office of Scientific and Technical Information (OSTI.GOV)

Shipsey, Ian

In his talk, Shipsey will discuss the cochlear implant, the first device to successfully allow the profoundly deaf to regain some sense of hearing. A cochlear implant is a small electronic apparatus. Unlike a normal hearing aid, which amplifies sound, a cochlear implant is surgically implanted behind the ear where it converts sound waves into electrical impulses. These implants have instigated a popular but controversial revolution in the treatment of deafness, and they serve as a model for research in neuroscience and biomedical engineering. Shipsey will discuss the physiology of natural hearing from the perspective of a physicist. He willmore » also touch on the function of cochlear implants in the context of historical treatments, electrical engineering, psychophysics, clinical evaluation of efficacy and personal experience. Finally, Shipsey will address the social implications of cochlear implantation and the future outlook for auditory prostheses.« less

Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder.

PubMed

Liu, Yuying; Dong, Ruijuan; Li, Yuling; Xu, Tianqiu; Li, Yongxin; Chen, Xueqing; Gong, Shusheng

2014-12-01

To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation. Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12-84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS). All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500Hz, 1000Hz, 2000Hz and 4000Hz) of aided hearing thresholds ranged from 17.5 to 57.5dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition. Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Tinnitus after Simultaneous and Sequential Bilateral Cochlear Implantation.

PubMed

Ramakers, Geerte G J; Kraaijenga, Véronique J C; Smulders, Yvette E; van Zon, Alice; Stegeman, Inge; Stokroos, Robert J; Free, Rolien H; Frijns, Johan H M; Huinck, Wendy J; Van Zanten, Gijsbert A; Grolman, Wilko

2017-01-01

There is an ongoing global discussion on whether or not bilateral cochlear implantation should be standard care for bilateral deafness. Contrary to unilateral cochlear implantation, however, little is known about the effect of bilateral cochlear implantation on tinnitus. To investigate tinnitus outcomes 1 year after bilateral cochlear implantation. Secondarily, to compare tinnitus outcomes between simultaneous and sequential bilateral cochlear implantation and to investigate long-term follow-up (3 years). This study is a secondary analysis as part of a multicenter randomized controlled trial. Thirty-eight postlingually deafened adults were included in the original trial, in which the presence of tinnitus was not an inclusion criterion. All participants received cochlear implants (CIs) because of profound hearing loss. Nineteen participants received bilateral CIs simultaneously and 19 participants received bilateral CIs sequentially with an inter-implant interval of 2 years. The prevalence and severity of tinnitus before and after simultaneous and sequential bilateral cochlear implantation were measured preoperatively and each year after implantation with the Tinnitus Handicap Inventory (THI) and Tinnitus Questionnaire (TQ). The prevalence of preoperative tinnitus was 42% (16/38). One year after bilateral implantation, there was a median difference of -8 (inter-quartile range (IQR): -28 to 4) in THI score and -9 (IQR: -17 to -9) in TQ score in the participants with preoperative tinnitus. Induction of tinnitus occurred in five participants, all in the simultaneous group, in the year after bilateral implantation. Although the preoperative and also the postoperative median THI and TQ scores were higher in the simultaneous group, the median difference scores were equal in both groups. In the simultaneous group, tinnitus scores fluctuated in the 3 years after implantation. In the sequential group, four patients had an additional benefit of the second CI: a total suppression of tinnitus compared with their unilateral situation. While bilateral cochlear implantation can have a positive effect on preoperative tinnitus complaints, the induction of (temporary or permanent) tinnitus was also reported. Dutch Trial Register NTR1722.

[Effect of rehabilitation for prelingual deaf children who use cochlear implants in conjunction with hearing aids in the opposite ears].

PubMed

Tian, Yanjing; Zhou, Huifang; Zhang, Jing; Yang, Dong; Xu, Yi; Guo, Yuxi

2012-10-01

To compare the effect of rehabilitation of prelingual deaf children who used a cochlear implant (CI) in one ear and a hearing aids in the opposite ear while the hearing level of the opposite ears are different. Hearing ability, language ability and learning ability was included in the content. The aim of this research is to investigate better style of rehabilitation, and to offer the best help to the prelingual deaf children. Accord ing to the hearing level of the ear opposite to the one wearing a cochlear implant and whether the opposite ear wear a hearing aid or not, 30 prelingual deaf children were divided into three groups, including cochlear implant with opposite severe hearing loss and hearing aid ear (CI+SHA), cochlear implant with opposite profound hearing loss and hearing aid ear (CI+PHA), cochlear implant only (CI). The effect of rehabilitation was assessed in six different times (3,6,9,12,15 and 18 months after the cochlear implants and hearing aids began to work). The longer time the rehabilitation spends, the better the hearing ability,language ability and the learning ability were. The hearing ability of CI+SHA was better than those of CI+PHA (P<0.05) and CI (P<0.05). The language ability and learning ability of CI-SHA was nearly equal to those of the other two groups. The prelingual deaf children should take much more time on rehabilitation. The effect of rehabilitation for prelingual deaf children who used cochlear implant in one ear and hearing aid in the other depend on the residual hearing level of the other ear. If a prelingual deaf children still has any residual hearing level in the ear opposite to the cochlear implant ear, it is better for him/her to wear a hearing aid in the ear.

Bringing Hearing to the Deaf--Cochlear Implants: a Technical and Personal Account

NASA Astrophysics Data System (ADS)

Shipsey, Ian

2006-04-01

Cochlear implants are the first device to successfully restore neural function. They have instigated a popular but controversial revolution in the treatment of deafness, and they serve as a model for research in neuroscience and biomedical engineering. In this talk the physiology of natural hearing will be reviewed from the perspective of a physicist, and the function of cochlear implants will be described in the context of historical treatments, electrical engineering, psychophysics, clinical evaluation of efficacy and personal experience. The social implications of cochlear implantation and the future outlook for auditory prostheses will also be discussed.

Microphone directionality, pre-emphasis filter, and wind noise in cochlear implants.

PubMed

Chung, King; McKibben, Nicholas

2011-10-01

Wind noise can be a nuisance or a debilitating masker for cochlear implant users in outdoor environments. Previous studies indicated that wind noise at the microphone/hearing aid output had high levels of low-frequency energy and the amount of noise generated is related to the microphone directionality. Currently, cochlear implants only offer either directional microphones or omnidirectional microphones for users at-large. As all cochlear implants utilize pre-emphasis filters to reduce low-frequency energy before the signal is encoded, effective wind noise reduction algorithms for hearing aids might not be applicable for cochlear implants. The purposes of this study were to investigate the effect of microphone directionality on speech recognition and perceived sound quality of cochlear implant users in wind noise and to derive effective wind noise reduction strategies for cochlear implants. A repeated-measure design was used to examine the effects of spectral and temporal masking created by wind noise recorded through directional and omnidirectional microphones and the effects of pre-emphasis filters on cochlear implant performance. A digital hearing aid was programmed to have linear amplification and relatively flat in-situ frequency responses for the directional and omnidirectional modes. The hearing aid output was then recorded from 0 to 360° at flow velocities of 4.5 and 13.5 m/sec in a quiet wind tunnel. Sixteen postlingually deafened adult cochlear implant listeners who reported to be able to communicate on the phone with friends and family without text messages participated in the study. Cochlear implant users listened to speech in wind noise recorded at locations that the directional and omnidirectional microphones yielded the lowest noise levels. Cochlear implant listeners repeated the sentences and rated the sound quality of the testing materials. Spectral and temporal characteristics of flow noise, as well as speech and/or noise characteristics before and after the pre-emphasis filter, were analyzed. Correlation coefficients between speech recognition scores and crest factors of wind noise before and after pre-emphasis filtering were also calculated. Listeners obtained higher scores using the omnidirectional than the directional microphone mode at 13.5 m/sec, but they obtained similar speech recognition scores for the two microphone modes at 4.5 m/sec. Higher correlation coefficients were obtained between speech recognition scores and crest factors of wind noise after pre-emphasis filtering rather than before filtering. Cochlear implant users would benefit from both directional and omnidirectional microphones to reduce far-field background noise and near-field wind noise. Automatic microphone switching algorithms can be more effective if the incoming signal were analyzed after pre-emphasis filters for microphone switching decisions. American Academy of Audiology.

Exploring the Language and Literacy Outcomes of Pediatric Cochlear Implant Users

PubMed Central

Spencer, Linda J.; Barker, Brittan A.; Tomblin, J. Bruce

2011-01-01

Objective The principal goal of this study was to investigate the relationship between language and literacy (i.e., reading and writing) skills in pediatric cochlear implant users. A peripheral objective was to identify the children's skills that were in need of remediation and subsequently to provide suggestions for remedial programming. It was predicted that the robust language skills often associated with children who have cochlear implant experience would facilitate the development of literacy skills. It was further proposed that the language and literacy skills of pediatric cochlear implant users would approximate the language and literacy skills of children with normal hearing. Design Sixteen pediatric cochlear implant users' language and literacy skills were evaluated and then compared with a reference group of 16 age-matched, normal-hearing children. All 32 participants were educated in mainstream classes within the public school system in the Midwest. The “Sentence Formulation” and “Concepts and Directions” subtests of the Clinical Evaluation of Language Fundamentals-3 test were used to evaluate receptive and expressive language skills. Reading comprehension was evaluated with the “Paragraph Comprehension” subtest of the Woodcock Reading Mastery Test. Performance measures for the writing analyses included productivity, complexity and grammaticality measures. Results Children with cochlear implants performed within 1 SD of the normal-hearing, age-matched children on measures of language comprehension, reading comprehension and writing accuracy. However, the children with cochlear implants performed significantly poorer than the children with normal hearing on the expressive “Sentence Formulation” subtest. The cochlear implant users also produced fewer words on the written narrative task than did the normal-hearing children, although there was not a significant difference between groups with respect to total words per clause. Furthermore there was a strong correlation between language performance and reading performance, as well as language performance and total words produced on the written performance measure for the children using cochlear implants. Conclusions The results of this study suggest that the language skills of pediatric cochlear implant users are related to and correlated with the development of literacy skills within these children. Consequently, the performance of the cochlear implant users, on various language and literacy measures, compared favorably to an age-matched group of children with normal hearing. There were significant differences in the ability of the cochlear implant users to correctly utilize grammatical structures such as conjunctions and correct verb forms when they were required to formulate written and oral sentences. Given this information, it would be appropriate for their educational or remedial language programs to emphasize the use and development of these structures. PMID:12799546

Class III myosins shape the auditory hair bundles by limiting microvilli and stereocilia growth

PubMed Central

Lelli, Andrea; Michel, Vincent; Boutet de Monvel, Jacques; Cortese, Matteo; Bosch-Grau, Montserrat; Aghaie, Asadollah; Perfettini, Isabelle; Dupont, Typhaine; Avan, Paul

2016-01-01

The precise architecture of hair bundles, the arrays of mechanosensitive microvilli-like stereocilia crowning the auditory hair cells, is essential to hearing. Myosin IIIa, defective in the late-onset deafness form DFNB30, has been proposed to transport espin-1 to the tips of stereocilia, thereby promoting their elongation. We show that Myo3a−/−Myo3b−/− mice lacking myosin IIIa and myosin IIIb are profoundly deaf, whereas Myo3a-cKO Myo3b−/− mice lacking myosin IIIb and losing myosin IIIa postnatally have normal hearing. Myo3a−/−Myo3b−/− cochlear hair bundles display robust mechanoelectrical transduction currents with normal kinetics but show severe embryonic abnormalities whose features rapidly change. These include abnormally tall and numerous microvilli or stereocilia, ungraded stereocilia bundles, and bundle rounding and closure. Surprisingly, espin-1 is properly targeted to Myo3a−/−Myo3b−/− stereocilia tips. Our results uncover the critical role that class III myosins play redundantly in hair-bundle morphogenesis; they unexpectedly limit the elongation of stereocilia and of subsequently regressing microvilli, thus contributing to the early hair bundle shaping. PMID:26754646

Cochlear implants and ex vivo BDNF gene therapy protect spiral ganglion neurons.

PubMed

Rejali, Darius; Lee, Valerie A; Abrashkin, Karen A; Humayun, Nousheen; Swiderski, Donald L; Raphael, Yehoash

2007-06-01

Spiral ganglion neurons often degenerate in the deaf ear, compromising the function of cochlear implants. Cochlear implant function can be improved by good preservation of the spiral ganglion neurons, which are the target of electrical stimulation by the implant. Brain derived neurotrophic factor (BDNF) has previously been shown to enhance spiral ganglion survival in experimentally deafened ears. Providing enhanced levels of BDNF in human ears may be accomplished by one of several different methods. The goal of these experiments was to test a modified design of the cochlear implant electrode that includes a coating of fibroblast cells transduced by a viral vector with a BDNF gene insert. To accomplish this type of ex vivo gene transfer, we transduced guinea pig fibroblasts with an adenovirus with a BDNF gene cassette insert, and determined that these cells secreted BDNF. We then attached BDNF-secreting cells to the cochlear implant electrode via an agarose gel, and implanted the electrode in the scala tympani. We determined that the BDNF expressing electrodes were able to preserve significantly more spiral ganglion neurons in the basal turns of the cochlea after 48 days of implantation when compared to control electrodes. This protective effect decreased in the higher cochlear turns. The data demonstrate the feasibility of combining cochlear implant therapy with ex vivo gene transfer for enhancing spiral ganglion neuron survival.

Is there a best side for cochlear implants in post-lingual patients?

PubMed

Amaral, Maria Stella Arantes do; Damico, Thiago A; Gonçales, Alina S; Reis, Ana C M B; Isaac, Myriam de Lima; Massuda, Eduardo T; Hyppolito, Miguel Angelo

2017-07-29

Cochlear Implant is a sensory prosthesis capable of restoring hearing in patients with severe or profound bilateral sensorineural hearing loss. To evaluate if there is a better side to be implanted in post-lingual patients. Retrospective longitudinal study. Participants were 40 subjects, of both sex, mean age of 47 years, with post-lingual hearing loss, users of unilateral cochlear implant for more than 12 months and less than 24 months, with asymmetric auditor reserve between the ears (difference of 10dBNA, In at least one of the frequencies with a response, between the ears), divided into two groups. Group A was composed of individuals with cochlear implant in the ear with better auditory reserve and Group B with auditory reserve lower in relation to the contralateral side. There was no statistical difference for the tonal auditory threshold before and after cochlear implant. A better speech perception in pre-cochlear implant tests was present in B (20%), but the final results are similar in both groups. The cochlear implant in the ear with the worst auditory residue favors a bimodal hearing, which would allow the binaural summation, without compromising the improvement of the audiometric threshold and the speech perception. Copyright © 2017 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Cochlear implants and medical tourism.

PubMed

McKinnon, Brian J; Bhatt, Nishant

2010-09-01

To compare the costs of medical tourism in cochlear implant surgery performed in India as compared to the United States. In addition, the cost savings of obtaining cochlear implant surgery in India were compare d to those of other surgical interventions obtained as a medical tourist. Searches were conducted on Medline and Google using the search terms: 'medical tourism', 'medical offshoring', 'medical outsourcing', 'cochlear implants' and 'cochlear implantation'. The information regarding cost of medical treatment was obtained from personal communication with individuals familiar with India's cochlear implantation medical tourism industry. The range of cost depended on length of stay as well as the device chosen. Generally the cost, inclusive of travel, surgery and device, was in the range of $21,000-30,000, as compared to a cost range of $40,000-$60,000 in the US. With the escalating cost of healthcare in the United States, it is not surprising that some patients would seek to obtain surgical care overseas at a fraction of the cost. Participants in medical tourism often have financial resources, but lack health insurance coverage. While cardiovascular and orthopedic surgery performed outside the United States in India at centers that cater to medical tourists are often performed at one-quarter to one-third of the cost that would have been paid in the United States, the cost differential for cochlear implants is not nearly as favorable.

The Influence of Cochlear Mechanical Dysfunction, Temporal Processing Deficits, and Age on the Intelligibility of Audible Speech in Noise for Hearing-Impaired Listeners

PubMed Central

Johannesen, Peter T.; Pérez-González, Patricia; Kalluri, Sridhar; Blanco, José L.

2016-01-01

The aim of this study was to assess the relative importance of cochlear mechanical dysfunction, temporal processing deficits, and age on the ability of hearing-impaired listeners to understand speech in noisy backgrounds. Sixty-eight listeners took part in the study. They were provided with linear, frequency-specific amplification to compensate for their audiometric losses, and intelligibility was assessed for speech-shaped noise (SSN) and a time-reversed two-talker masker (R2TM). Behavioral estimates of cochlear gain loss and residual compression were available from a previous study and were used as indicators of cochlear mechanical dysfunction. Temporal processing abilities were assessed using frequency modulation detection thresholds. Age, audiometric thresholds, and the difference between audiometric threshold and cochlear gain loss were also included in the analyses. Stepwise multiple linear regression models were used to assess the relative importance of the various factors for intelligibility. Results showed that (a) cochlear gain loss was unrelated to intelligibility, (b) residual cochlear compression was related to intelligibility in SSN but not in a R2TM, (c) temporal processing was strongly related to intelligibility in a R2TM and much less so in SSN, and (d) age per se impaired intelligibility. In summary, all factors affected intelligibility, but their relative importance varied across maskers. PMID:27604779

Parents' Views on Changing Communication after Cochlear Implantation

ERIC Educational Resources Information Center

Watson, Linda M.; Hardie, Tim; Archbold, Sue M.; Wheeler, Alexandra

2008-01-01

We sent questionnaires to families of all 288 children who had received cochlear implants at one center in the United Kingdom at least 5 years previously. Thus, it was a large, unselected group. We received 142 replies and 119 indicated that the child and family had changed their communication approach following cochlear implantation. In 113 cases…

Changes in Speech Production in an Early Deafened Adult with a Cochlear Implant

ERIC Educational Resources Information Center

Wong, Patrick C. M.

2007-01-01

Background and Aims: The current study is a first investigation reporting the speech production characteristics of an early deafened adult cochlear implant user after a course of speech-language treatment. Methods and Procedures: The participant is culturally deaf and received the cochlear implant when she was 43 years old. A 24-week ABCABC…

The Self-Regulation of a Child with Cochlear Implants within a School Environment

ERIC Educational Resources Information Center

Patton, Kristin L.

2013-01-01

The purpose of this qualitative research, which utilized a narrative design strategy, was to describe the process of self-regulation of a child who has bilateral cochlear implants within the social environment of school. The study investigated the use of self-regulatory strategies by the cochlear implant recipient. It also examined how the child…

Cochlear Implantation for Children with Hearing Loss and Multiple Disabilities: An Evaluation from an Educator's Perspective

ERIC Educational Resources Information Center

Bertram, Bodo

2004-01-01

In recent years, parents of children with hearing loss and various additional disabilities have shown increasing interest in cochlear implants. This article describes how the program in Hanover, Germany is attempting to respond to this interest. It discusses the selection process for children who receive cochlear implants who have multiple…

Spelling in Written Stories by School-Age Children with Cochlear Implants

ERIC Educational Resources Information Center

Straley, Sara G.; Werfel, Krystal L.; Hendricks, Alison Eisel

2016-01-01

This study evaluated the spelling of 3rd to 6th grade children with cochlear implants in written stories. Spelling was analysed using traditional correct/incorrect scoring as well as the Spelling Sensitivity Score, which provides linguistic information about spelling attempts. Children with cochlear implants spelled 86 per cent of words in stories…

Electrical field imaging as a means to predict the loudness of monopolar and tripolar stimuli in cochlear implant patients.

PubMed

Berenstein, Carlo K; Vanpoucke, Filiep J; Mulder, Jef J S; Mens, Lucas H M

2010-12-01

Tripolar and other electrode configurations that use simultaneous stimulation inside the cochlea have been tested to reduce channel interactions compared to the monopolar stimulation conventionally used in cochlear implant systems. However, these "focused" configurations require increased current levels to achieve sufficient loudness. In this study, we investigate whether highly accurate recordings of the intracochlear electrical field set up by monopolar and tripolar configurations correlate to their effect on loudness. We related the intra-scalar potential distribution to behavioral loudness, by introducing a free parameter (α) which parameterizes the degree to which the potential field peak set up inside the scala tympani is still present at the location of the targeted neural tissue. Loudness balancing was performed on four levels between behavioral threshold and the most comfortable loudness level in a group of 10 experienced Advanced Bionics cochlear implant users. The effect of the amount of focusing on loudness was well explained by α per subject location along the basilar membrane. We found that α was unaffected by presentation level. Moreover, the ratios between the monopolar and tripolar currents, balanced for equal loudness, were approximately the same for all presentation levels. This suggests a linear loudness growth with increasing current level and that the equal peak hypothesis may predict the loudness of threshold as well as at supra-threshold levels. These results suggest that advanced electrical field imaging, complemented with limited psychophysical testing, more specifically at only one presentation level, enables estimation of the loudness growth of complex electrode configurations. Copyright © 2010 Elsevier B.V. All rights reserved.

Noise alters guinea pig's blood-labyrinth barrier ultrastructure and permeability along with a decrease of cochlear Claudin-5 and Occludin.

PubMed

Wu, Yong-Xiang; Zhu, Guo-Xia; Liu, Xin-Qin; Sun, Fei; Zhou, Ke; Wang, Shuang; Wang, Chun-Mei; Jia, Jin-Wen; Song, Jian-Tao; Lu, Lian-Jun

2014-12-24

Noise exposure (NE) is a severe modern health hazard that induces hearing impairment. However, the noise-induced ultrastructural changes of blood-labyrinth barrier (BLB) and the potential involvements of tight junction proteins (TJP) remain inconclusive. We investigated the effects of NE on not only the ultrastructure of cochlea and permeability of BLB but also the expression of TJP within the guinea pig cochlea. Male albino guinea pigs were exposed to white noise for 4 h or 2 consecutive days (115 dB sound pressure level, 6 hours per day) and the hearing impairments and light microscopic change of BLB were evaluated with auditory brainstem responses (ABR) and the cochlear sensory epithelia surface preparation, respectively. The cochlear ultrastructure and BLB permeability after NE 2d were revealed with transmission electron microscope (TEM) and lanthanum nitrate-tracing techniques, respectively. The potential alterations of TJPs Claudin-5 and Occludin were quantified with immunohistochemistry and western blot. NE induced significant hearing impairment and NE 2d contributed to significant outer hair cell (OHC) loss that is most severe in the first row of outer hair cells. Furthermore, the loosen TJ and an obvious leakage of lanthanum nitrate particles beneath the basal lamina were revealed with TEM. Moreover, a dose-dependent decrease of Claudin-5 and Occludin was observed in the cochlea after NE. All these findings suggest that both decrease of Claudin-5 and Occludin and increased BLB permeability are involved in the pathologic process of noise-induced hearing impairment; however, the causal relationship and underlying mechanisms should be further investigated.

Initial Operative Experience and Short-term Hearing Preservation Results With a Mid-scala Cochlear Implant Electrode Array.

PubMed

Svrakic, Maja; Roland, J Thomas; McMenomey, Sean O; Svirsky, Mario A

2016-12-01

To describe our initial operative experience and hearing preservation results with the Advanced Bionics (AB) Mid Scala Electrode (MSE). Retrospective review. Tertiary referral center. Sixty-three MSE implants in pediatric and adult patients were compared with age- and sex-matched 1j electrode implants from the same manufacturer. All patients were severe to profoundly deaf. Cochlear implantation with either the AB 1j electrode or the AB MSE. The MSE and 1j electrodes were compared in their angular depth of insertion and pre to postoperative change in hearing thresholds. Hearing preservation was analyzed as a function of angular depth of insertion. Secondary outcome measures included operative time, incidence of abnormal intraoperative impedance and telemetry values, and incidence of postsurgical complications. Depth of insertion was similar for both electrodes, but was more consistent for the MSE array and more variable for the 1j array. Patients with MSE electrodes had better hearing preservation. Thresholds shifts at four audiometric frequencies ranging from 250 to 2000 Hz were 10, 7, 2, and 6 dB smaller for the MSE electrode than for the 1j (p < 0.05). Hearing preservation at low frequencies was worse with deeper insertion, regardless of array. Secondary outcome measures were similar for both electrodes. The MSE electrode resulted in more consistent insertion depth and somewhat better hearing preservation than the 1j electrode. Differences in other surgical outcome measures were small or unlikely to have a meaningful effect.

[Etiological, clinical and neuroradiological investigation of deaf children with additional neuropsychiatric disabilities].

PubMed

Chilosi, A M; Scusa, M F; Comparini, A; Genovese, E; Forli, F; Berrettini, S; Cipriani, P

2012-04-01

Sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of cases, but the epidemiology of associated disorders, in terms of type, frequency and aetiology is still not clearly defined. Additional disabilities in a deaf child have important consequences in assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation (CI) or hearing aids (HA). The aim of this paper was to evaluate frequency, type and severity of additional neurodevelopmental disabilities in children with profound bilateral sensorineural hearing loss and to investigate the relationship between disability and the etiology of deafness. Eighty children with profound bilateral sensorineural hearing loss (mean age 5.4 years) were investigated by means of a diagnostic protocol including clinical, neurodevelopmental, and audiological procedures together with genetic and neurometabolic tests and neuroradiological investigation by brain MRI. Fifty-five percent of the sample exhibited one or more disabilities in addition to deafness, with cognitive, behavioural-emotional and motor disorders being the most frequent. The risk of additional disabilities varied according to aetiology, with a higher incidence in hereditary syndromic deafness, in cases due to pre-perinatal pathology (in comparison to unknown and hereditary non syndromic forms) and in the presence of major brain abnormalities at MRI. Our results suggest that the aetiology of deafness may be a significant risk indicator for the presence of neuropsychiatric disorders. A multidimensional evaluation, including aetiological, neurodevelopmental and MRI investigation is needed for formulating prognosis and for planning therapeutic intervention, especially in those children candidated to cochlear implant.

Auditory hindbrain atrophy and anomalous calcium binding protein expression after neonatal exposure to monosodium glutamate.

PubMed

Foran, Lindsey; Blackburn, Kaitlyn; Kulesza, Randy J

2017-03-06

Glutamate is the most abundant excitatory neurotransmitter in the central nervous system, and is stored and released by both neurons and astrocytes. Despite the important role of glutamate as a neurotransmitter, elevated extracellular glutamate can result in excitotoxicity and apoptosis. Monosodium glutamate (MSG) is a naturally occurring sodium salt of glutamic acid that is used as a flavor enhancer in many processed foods. Previous studies have shown that MSG administration during the early postnatal period results in neurodegenerative changes in several forebrain regions, characterized by neuronal loss and neuroendocrine abnormalities. Systemic delivery of MSG during the neonatal period and induction of glutamate neurotoxicity in the cochlea have both been shown to result in fewer neurons in the spiral ganglion. We hypothesized that an MSG-induced loss of neurons in the spiral ganglion would have a significant impact on the number of neurons in the cochlear nuclei and superior olivary complex (SOC). Indeed, we found that exposure to MSG from postnatal days 4 through 10 resulted in significantly fewer neurons in the cochlear nuclei and SOC and significant dysmorphology in surviving neurons. Moreover, we found that neonatal MSG exposure resulted in a significant decrease in the expression of both calretinin and calbindin. These results suggest that neonatal exposure to MSG interferes with early development of the auditory brainstem and impacts expression of calcium binding proteins, both of which may lead to diminished auditory function. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

Identification of Pure-Tone Audiologic Thresholds for Pediatric Cochlear Implant Candidacy: A Systematic Review.

PubMed

de Kleijn, Jasper L; van Kalmthout, Ludwike W M; van der Vossen, Martijn J B; Vonck, Bernard M D; Topsakal, Vedat; Bruijnzeel, Hanneke

2018-05-24

Although current guidelines recommend cochlear implantation only for children with profound hearing impairment (HI) (>90 decibel [dB] hearing level [HL]), studies show that children with severe hearing impairment (>70-90 dB HL) could also benefit from cochlear implantation. To perform a systematic review to identify audiologic thresholds (in dB HL) that could serve as an audiologic candidacy criterion for pediatric cochlear implantation using 4 domains of speech and language development as independent outcome measures (speech production, speech perception, receptive language, and auditory performance). PubMed and Embase databases were searched up to June 28, 2017, to identify studies comparing speech and language development between children who were profoundly deaf using cochlear implants and children with severe hearing loss using hearing aids, because no studies are available directly comparing children with severe HI in both groups. If cochlear implant users with profound HI score better on speech and language tests than those with severe HI who use hearing aids, this outcome could support adjusting cochlear implantation candidacy criteria to lower audiologic thresholds. Literature search, screening, and article selection were performed using a predefined strategy. Article screening was executed independently by 4 authors in 2 pairs; consensus on article inclusion was reached by discussion between these 4 authors. This study is reported according to the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) statement. Title and abstract screening of 2822 articles resulted in selection of 130 articles for full-text review. Twenty-one studies were selected for critical appraisal, resulting in selection of 10 articles for data extraction. Two studies formulated audiologic thresholds (in dB HLs) at which children could qualify for cochlear implantation: (1) at 4-frequency pure-tone average (PTA) thresholds of 80 dB HL or greater based on speech perception and auditory performance subtests and (2) at PTA thresholds of 88 and 96 dB HL based on a speech perception subtest. In 8 of the 18 outcome measures, children with profound HI using cochlear implants performed similarly to children with severe HI using hearing aids. Better performance of cochlear implant users was shown with a picture-naming test and a speech perception in noise test. Owing to large heterogeneity in study population and selected tests, it was not possible to conduct a meta-analysis. Studies indicate that lower audiologic thresholds (≥80 dB HL) than are advised in current national and manufacturer guidelines would be appropriate as audiologic candidacy criteria for pediatric cochlear implantation.

Modeling Open-Set Spoken Word Recognition in Postlingually Deafened Adults after Cochlear Implantation: Some Preliminary Results with the Neighborhood Activation Model

PubMed Central

Meyer, Ted A.; Frisch, Stefan A.; Pisoni, David B.; Miyamoto, Richard T.; Svirsky, Mario A.

2012-01-01

Hypotheses Do cochlear implants provide enough information to allow adult cochlear implant users to understand words in ways that are similar to listeners with acoustic hearing? Can we use a computational model to gain insight into the underlying mechanisms used by cochlear implant users to recognize spoken words? Background The Neighborhood Activation Model has been shown to be a reasonable model of word recognition for listeners with normal hearing. The Neighborhood Activation Model assumes that words are recognized in relation to other similar-sounding words in a listener’s lexicon. The probability of correctly identifying a word is based on the phoneme perception probabilities from a listener’s closed-set consonant and vowel confusion matrices modified by the relative frequency of occurrence of the target word compared with similar-sounding words (neighbors). Common words with few similar-sounding neighbors are more likely to be selected as responses than less common words with many similar-sounding neighbors. Recent studies have shown that several of the assumptions of the Neighborhood Activation Model also hold true for cochlear implant users. Methods Closed-set consonant and vowel confusion matrices were obtained from 26 postlingually deafened adults who use cochlear implants. Confusion matrices were used to represent input errors to the Neighborhood Activation Model. Responses to the different stimuli were then generated by the Neighborhood Activation Model after incorporating the frequency of occurrence counts of the stimuli and their neighbors. Model outputs were compared with obtained performance measures on the Consonant-Vowel Nucleus-Consonant word test. Information transmission analysis was used to assess whether the Neighborhood Activation Model was able to successfully generate and predict word and individual phoneme recognition by cochlear implant users. Results The Neighborhood Activation Model predicted Consonant-Vowel Nucleus-Consonant test words at levels similar to those correctly identified by the cochlear implant users. The Neighborhood Activation Model also predicted phoneme feature information well. Conclusion The results obtained suggest that the Neighborhood Activation Model provides a reasonable explanation of word recognition by postlingually deafened adults after cochlear implantation. It appears that multichannel cochlear implants give cochlear implant users access to their mental lexicons in a manner that is similar to listeners with acoustic hearing. The lexical properties of the test stimuli used to assess performance are important to spoken-word recognition and should be included in further models of the word recognition process. PMID:12851554

Evaluation on health-related quality of life in deaf children with cochlear implant in China.

PubMed

Liu, Hong; Liu, Hong-Xiang; Kang, Hou-Yong; Gu, Zheng; Hong, Su-Ling

2016-09-01

Previous studies have shown that deaf children benefit considerably from cochlear implants. These improvements are found in areas such as speech perception, speech production, and audiology-verbal performance. Despite the increasing prevalence of cochlear implants in China, few studies have reported on health-related quality of life in children with cochlear implants. The main objective of this study was to explore health-related quality of life on children with cochlear implants in South-west China. A retrospective observational study of 213 CI users in Southwest China between 2010 and 2013. Participants were 213 individuals with bilateral severe-to-profound hearing loss who wore unilateral cochlear implants. The Nijmegen Cochlear Implant Questionnaire and Health Utility Index Mark III were used pre-implantation and 1 year post-implantation. Additionally, 1-year postoperative scores for Mandarin speech perception were compared with preoperative scores. Health-related quality of life improved post-operation with scores on the Nijmegen Cochlear Implant Questionnaire improving significantly in all subdomains, and the Health Utility Index 3 showing a significant improvement in the utility score and the subdomains of ''hearing," ''speech," and "emotion". Additionally, a significant improvement in speech recognition scores was found. No significant correlation was found between increased in quality of life and speech perception scores. Health-related quality of life and speech recognition in prelingual deaf children significantly improved post-operation. The lack of correlation between quality of life and speech perception suggests that when evaluating performance post-implantation in prelingual deaf children and adolescents, measures of both speech perception and quality of life should be used. Copyright © 2016. Published by Elsevier Ireland Ltd.

Jervell and Lange-Nielsen syndrome in cochlear implanted patients: our experience and a review of literature.

PubMed

Eftekharian, Ali; Mahani, Mozhgan Hosseinerezai

2015-09-01

To share our experience in cochlear implanted patients with Jervell and Lange-Nielsen syndrome (JLNS), to review the literature results and to disclose precautions which have to be taken dealing with these patients. Electrocardiograms (ECG) of 503 children with congenital bilateral profound hearing loss which were cochlear implanted in cochlear implant center of a tertiary hospital were evaluated for long QT syndrome. Clinical reports of the patients with JLNS were evaluated and a review of literature performed. The prevalence of disease was 0.79% (four cases) in our center which is in the range of literature reports (0-2.6%). None of our patients had a history of syncopal attack. Two patients (50%) were born from parents with consanguineous marriage. Considering all precautions their cochlear implant surgeries were done uneventfully. A review of the literature has identified sixteen reports on cochlear implantation in a total of 38 children with JLNS. Similar to our cases none of the authors reported cardiac events during device switch-on. Nine available reports about the outcome of cochlear implantation in these patients indicated good auditory outcome. It is recommended that all congenitally deaf patients have an ECG taken as a part of the evaluation. As auditory stimuli is reported to be a specific trigger, it is prudent to activate the processor with continuous heart monitoring even though there is no reported cardiac event during device switch-on. Cochlear implantation can be performed relatively safely in these patients if necessary precautions have been taken appropriately and their auditory outcome is good. Triggers of the cardiac events should be avoided throughout their life. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Assessment of the cochlear nerve to facial nerve size ratio using MR multiplanar reconstruction of the internal auditory canal in patients presenting with acquired long-standing hearing loss

PubMed Central

Hey, Constanze; Shaaban, Mohamed S; Elabd, Amr M; Hassan, Hebatallah H M; Gruber-Rouh, Tatjana; Kaltenbach, Benjamin; Harth, Marc; Ackermann, Hanns; Stöver, Timo; Vogl, Thomas J; Nour-Eldin, Nour-Eldin A

2017-01-01

Objective: To test using the facial nerve as a reference for assessment of the cochlear nerve size in patients with acquired long-standing sensorineural hearing loss (SNHL) using MRI multiplanar reconstruction. Methods: The study was retrospectively performed on 86 patients. Group 1 (study group, n = 53) with bilateral long-standing SNHL. Group 2 (control group, n = 33) without hearing loss. The nerve size was measured by drawing a region of interest around the cross-sectional circumference of the nerve in multiplanar reconstruction images. Results: No significant correlation was noted between the cochlear nerve and facial nerve size, and the patient's age, gender and weight (p > 0.05). In Group 1, the mean ratio of the cochlear to facial nerve size was 0.99 ± 0.30 (range: 0.52–1.86) and 1.12 ± 0.35 (range: 0.34–2.3) for the right and left sides, respectively. In Group 2, it was 1.18 ± 0.23 (range: 0.78–1.71) and 1.25 ± 0.25 (range: 0.85–1.94) for the right and left sides, respectively. The cochlear nerve size was statistically (p = 0.0004) smaller in Group 1 than in Group 2. Conclusion: The cochlear nerve size and the cochlear to facial nerve size ratio are significantly smaller in patients with acquired long-standing SNHL. Advances in knowledge: The facial nerve can be used as a reference for assessment of the cochlear nerve in patients with acquired long-standing SNHL. PMID:28368665

Cochlear implantation in autistic children with profound sensorineural hearing loss.

PubMed

Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz

2016-11-19

Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Auditory Speech Perception Development in Relation to Patient's Age with Cochlear Implant

PubMed Central

Ciscare, Grace Kelly Seixas; Mantello, Erika Barioni; Fortunato-Queiroz, Carla Aparecida Urzedo; Hyppolito, Miguel Angelo; Reis, Ana Cláudia Mirândola Barbosa dos

2017-01-01

Introduction  A cochlear implant in adolescent patients with pre-lingual deafness is still a debatable issue. Objective  The objective of this study is to analyze and compare the development of auditory speech perception in children with pre-lingual auditory impairment submitted to cochlear implant, in different age groups in the first year after implantation. Method  This is a retrospective study, documentary research, in which we analyzed 78 reports of children with severe bilateral sensorineural hearing loss, unilateral cochlear implant users of both sexes. They were divided into three groups: G1, 22 infants aged less than 42 months; G2, 28 infants aged between 43 to 83 months; and G3, 28 older than 84 months. We collected medical record data to characterize the patients, auditory thresholds with cochlear implants, assessment of speech perception, and auditory skills. Results  There was no statistical difference in the association of the results among groups G1, G2, and G3 with sex, caregiver education level, city of residence, and speech perception level. There was a moderate correlation between age and hearing aid use time, age and cochlear implants use time. There was a strong correlation between age and the age cochlear implants was performed, hearing aid use time and age CI was performed. Conclusion  There was no statistical difference in the speech perception in relation to the patient's age when cochlear implant was performed. There were statistically significant differences for the variables of auditory deprivation time between G3 - G1 and G2 - G1 and hearing aid use time between G3 - G2 and G3 - G1. PMID:28680487

Auditory Mechanics of the Tectorial Membrane and the Cochlear Spiral

PubMed Central

Gavara, Núria; Manoussaki, Daphne; Chadwick, Richard S.

2012-01-01

Purpose of review This review is timely and relevant since new experimental and theoretical findings suggest that cochlear mechanics from the nanoscale to the macroscale are affected by mechanical properties of the tectorial membrane and the spiral shape. Recent findings Main tectorial membrane themes covered are i) composition and morphology, ii) nanoscale mechanical interactions with the outer hair cell bundle, iii) macroscale longitudinal coupling, iv) fluid interaction with inner hair cell bundles, v) macroscale dynamics and waves. Main cochlear spiral themes are macroscale low-frequency energy focusing and microscale organ of Corti shear gain. Implications Findings from new experimental and theoretical models reveal exquisite sensitivity of cochlear mechanical performance to tectorial membrane structural organization, mechanics, and its positioning with respect to hair bundles. The cochlear spiral geometry is a major determinant of low frequency hearing. Suggestions are made for future research directions. PMID:21785353

[Topographic anatomy of the hook region and its significance for the choice of the surgical technique for the cochlear implantation].

PubMed

Yanov, Yu K; Kuzovkov, V E; Lilenko, A S; Kostevich, I V; Sugarova, S B; Amonov, A Sh

The mode of the introduction of the active electrode of a cochlear implant into the cochlea remains a key issue as far as cochlear implantation is concerned. Especially much attention has recently been given to the relationship between the anatomical features of the basal region of the cochlea (the so-called 'fish hook') and the possibility to approach it. We have undertaken the attempt to optimize the approach to the tympanic canal (scala tympanica) of the cochlea with a view to reducing to a minimum the risk of an injury to the cochlear structures in the course of cochlear implantation. A total of 35 cadaveric temporal bones were examined to measure the fine structures of the hook region and evaluate the risk of their damages associated with various approaches to the tympanic canal.

Congenitally deafblind children and cochlear implants: effects on communication.

PubMed

Dammeyer, Jesper

2009-01-01

There has been much research conducted demonstrating the positive benefits of cochlear implantation (CI) in children who are deaf. Research on CI in children who are both deaf and blind, however, is lacking. The purpose of this article is to present a study of five congenitally deafblind children who received cochlear implants between 2.2 and 4.2 years of age. Ratings of video observations were used to measure the children's early communication development with and without the use of their cochlear implants. In addition, parental interviews were used to assess the benefits parents perceived regarding their children's cochlear implants. Two examples are included in this article to illustrate the parents' perspectives about CI in their deafblind children. Benefits of CI in this cohort of children included improved attention and emotional response as well as greater use of objects in interaction with adults. The best overall outcome of CI is not spoken language but better communication.

Deep brain stimulation with a pre-existing cochlear implant: Surgical technique and outcome.

PubMed

Eddelman, Daniel; Wewel, Joshua; Wiet, R Mark; Metman, Leo V; Sani, Sepehr

2017-01-01

Patients with previously implanted cranial devices pose a special challenge in deep brain stimulation (DBS) surgery. We report the implantation of bilateral DBS leads in a patient with a cochlear implant. Technical nuances and long-term interdevice functionality are presented. A 70-year-old patient with advancing Parkinson's disease and a previously placed cochlear implant for sensorineural hearing loss was referred for placement of bilateral DBS in the subthalamic nucleus (STN). Prior to DBS, the patient underwent surgical removal of the subgaleal cochlear magnet, followed by stereotactic MRI, frame placement, stereotactic computed tomography (CT), and merging of imaging studies. This technique allowed for successful computational merging, MRI-guided targeting, and lead implantation with acceptable accuracy. Formal testing and programming of both the devices were successful without electrical interference. Successful DBS implantation with high resolution MRI-guided targeting is technically feasible in patients with previously implanted cochlear implants by following proper precautions.

Disorders of cochlear blood flow.

PubMed

Nakashima, Tsutomu; Naganawa, Shinji; Sone, Michihiko; Tominaga, Mitsuo; Hayashi, Hideo; Yamamoto, Hiroshi; Liu, Xiuli; Nuttall, Alfred L

2003-09-01

The cochlea is principally supplied from the inner ear artery (labyrinthine artery), which is usually a branch of the anterior inferior cerebellar artery. Cochlear blood flow is a function of cochlear perfusion pressure, which is calculated as the difference between mean arterial blood pressure and inner ear fluid pressure. Many otologic disorders such as noise-induced hearing loss, endolymphatic hydrops and presbycusis are suspected of being related to alterations in cochlear blood flow. However, the human cochlea is not easily accessible for investigation because this delicate sensory organ is hidden deep in the temporal bone. In patients with sensorineural hearing loss, magnetic resonance imaging, laser-Doppler flowmetry and ultrasonography have been used to investigate the status of cochlear blood flow. There have been many reports of hearing loss that were considered to be caused by blood flow disturbance in the cochlea. However, direct evidence of blood flow disturbance in the cochlea is still lacking in most of the cases.

Parallel evolution of auditory genes for echolocation in bats and toothed whales.

PubMed

Shen, Yong-Yi; Liang, Lu; Li, Gui-Sheng; Murphy, Robert W; Zhang, Ya-Ping

2012-06-01

The ability of bats and toothed whales to echolocate is a remarkable case of convergent evolution. Previous genetic studies have documented parallel evolution of nucleotide sequences in Prestin and KCNQ4, both of which are associated with voltage motility during the cochlear amplification of signals. Echolocation involves complex mechanisms. The most important factors include cochlear amplification, nerve transmission, and signal re-coding. Herein, we screen three genes that play different roles in this auditory system. Cadherin 23 (Cdh23) and its ligand, protocadherin 15 (Pcdh15), are essential for bundling motility in the sensory hair. Otoferlin (Otof) responds to nerve signal transmission in the auditory inner hair cell. Signals of parallel evolution occur in all three genes in the three groups of echolocators--two groups of bats (Yangochiroptera and Rhinolophoidea) plus the dolphin. Significant signals of positive selection also occur in Cdh23 in the Rhinolophoidea and dolphin, and Pcdh15 in Yangochiroptera. In addition, adult echolocating bats have higher levels of Otof expression in the auditory cortex than do their embryos and non-echolocation bats. Cdh23 and Pcdh15 encode the upper and lower parts of tip-links, and both genes show signals of convergent evolution and positive selection in echolocators, implying that they may co-evolve to optimize cochlear amplification. Convergent evolution and expression patterns of Otof suggest the potential role of nerve and brain in echolocation. Our synthesis of gene sequence and gene expression analyses reveals that positive selection, parallel evolution, and perhaps co-evolution and gene expression affect multiple hearing genes that play different roles in audition, including voltage and bundle motility in cochlear amplification, nerve transmission, and brain function.

The Potential Protective Effects of 2-aminoethyl Diphenylborinate against Inner Ear Acoustic Trauma: Experimental Study Using Transmission and Scanning Electron Microscopy.

PubMed

Kaymakçı, Mustafa; Acar, Mustafa; Burukoglu, Dilek; Kutlu, Hatice Mehtap; Shojaolsadati, Paria; Cingi, Cemal; Bayar Muluk, Nuray

2015-04-01

In this prospective experimental study, we investigated the preventive effects of 2-aminoethyl diphenylborinate (2-APB) in rats exposed to acoustic trauma (AT). Light microscopic, transmission electron microscopic (TEM), and scanning electron microscopic (SEM) examinations were performed. Eighteen healthy Wistar albino rats were divided into the following three groups: groups 1 (control), 2 (AT), and 3 (AT+APB). The rats in groups 2 and 3 were exposed to AT; in group 3 rats, 2-APB at 2 mg/kg was also administered, initially transperitoneally, after 10 min. During the light microscopic, TEM, and SEM examinations, the structures of the cochlear hair cells, stereocilia, and Deiter's cells were normal in the control group. In the AT group, the organ of Corti and proximate structures were damaged according to the light microscopic examination. During the TEM examination, intense cellular damage and stereocilia loss were detected, while during the SEM examination, extensive damage and stereocilia loss were observed. Decreased damage with preserved cochlear structure was detected during the light microscopic examination in the AT+APB group than in the AT group. During the TEM and SEM examinations, although stereocilia loss occurred in the AT+APB group, near-normal cell, cilia, and tectorial membrane structures were also observed in the AT+APB group compared with the AT group. 2-APB may have protective effects against AT damage of the cochlea. The main mechanism underlying this effect is the inhibition of the vasoconstriction of the cochlear spiral modiolar artery, thereby improving cochlear blood flow. We conclude that 2-APB may also be effective if used immediately following AT.

Cost-Effectiveness of Pediatric Cochlear Implantation in Rural China.

PubMed

Qiu, Jianxin; Yu, Chongxian; Ariyaratne, Thathya V; Foteff, Chris; Ke, Zhangmin; Sun, Yi; Zhang, Li; Qin, Feifei; Sanderson, Georgina

2017-07-01

To evaluate the cost utility of cochlear implantation (CI) for severe to profound sensorineural hearing loss (SNHL) among children from rural settings in P.R. China (China). A cost-utility analysis (CUA) was undertaken using data generated from a single-center substudy of the Cochlear Pediatric Implanted Recipient Observational Study (Cochlear P-IROS). The data were projected over a 20-year time horizon using a decision tree model. The Chinese healthcare payer and patient perspectives were adopted. Unilateral CI of children with a severe-to-profound SNHL compared with their preimplantation state of no treatment or amplification with hearing aids ("no CI" status). Incremental costs per quality adjusted life year (QALY) gained. The mean total discounted cost of unilateral CI was CNY 252,506 (37,876 USD), compared with CNY 29,005 (4,351 USD) for the no CI status from the healthcare payer plus patient perspective. A total discounted benefit of 8.9 QALYs was estimated for CI recipients compared with 6.7 QALYs for the no CI status. From the healthcare payer plus patient perspective, incremental cost-effectiveness ratio (ICER) for unilateral CI compared with no CI was CNY 100,561 (15,084 USD) per QALY. The healthcare payer perspective yielded an ICER of CNY 40,929 (6,139 USD) per QALY. Both ICERs fell within one to three times China's gross domestic product per capita (GDP, 2011-2015), considered "cost-effective" by World Health Organization (WHO) standards. Treatment with unilateral CI is a cost-effective hearing solution for children with severe to profound SNHL in rural China. Increased access to mainstream education and greater opportunities for employment, are potential downstream benefits of CI that may yield further societal and economic benefits. CI may be considered favorably for broader inclusion in medical insurance schemes across China.

Proteomics, bioinformatics and targeted gene expression analysis reveals up-regulation of cochlin and identifies other potential biomarkers in the mouse model for deafness in usher syndrome type 1F

PubMed Central

Chance, Mark R.; Chang, Jinsook; Liu, Shuqing; Gokulrangan, Giridharan; Chen, Daniel H.-C.; Lindsay, Aaron; Geng, Ruishuang; Zheng, Qing Y.; Alagramam, Kumar

2010-01-01

Proteins and protein networks associated with cochlear pathogenesis in the Ames waltzer (av) mouse, a model for deafness in Usher syndrome 1F (USH1F), were identified. Cochlear protein from wild-type and av mice at postnatal day 30, a time point in which cochlear pathology is well established, was analyzed by quantitative 2D gel electrophoresis followed by mass spectrometry (MS). The analytic gel resolved 2270 spots; 69 spots showed significant changes in intensity in the av cochlea compared with the control. The cochlin protein was identified in 20 peptide spots, most of which were up-regulated, while a few were down-regulated. Analysis of MS sequence data showed that, in the av cochlea, a set of full-length isoforms of cochlin was up-regulated, while isoforms missing the N-terminal FCH/LCCL domain were down-regulated. Protein interaction network analysis of all differentially expressed proteins was performed with Metacore software. That analysis revealed a number of statistically significant candidate protein networks predicted to be altered in the affected cochlea. Quantitative PCR (qPCR) analysis of select candidates from the proteomic and bioinformatic investigations showed up-regulation of Coch mRNA and those of p53, Brn3a and Nrf2, transcription factors linked to stress response and survival. Increased mRNA of Brn3a and Nrf2 has previously been associated with increased expression of cochlin in human glaucomatous trabecular meshwork. Our report strongly suggests that increased level of cochlin is an important etiologic factor leading to the degeneration of cochlear neuroepithelia in the USH1F model. PMID:20097680

Assessment of the influence of whole body vibration on Cochlear function

PubMed Central

2012-01-01

Background Whole body vibration (WBV) is a potentially harmful consequence resulting from the dissipation of energy by industrial machineries. The result of WBV exposure on the auditory system remains unknown. The objective of the present research was to evaluate the influence of WBV on cochlear function, in particular outer hair cell function. It is hypothesized that WBV impairs cochlear function resulting in decreased Distortion Product Otoacoustic Emission (DPOAE) levels (Ldp) in rabbits subjected to WBV. Methods Twelve rabbits were equally divided into vibration and control groups. Animals in vibration group were exposed to 1.0 ms-2 r.m.s vertical WBV at 4–8 Hz for 8 h/day during 5 consecutive days. Outer hair cell function was assessed by comparing repeated-measurements of DPOAE levels (Ldp) across a range of f2 frequencies in rabbits both exposed and unexposed to WBV. DPOAE level shifts (LSdp) were compared across ears, frequencies, groups, and times. Results No differences were seen over time in DPOAE levels in the non-exposed rabbits (p = 0.082). Post-exposure Ldp in rabbits exposed to WBV were significantly increased at all test frequencies in both ears compared to baseline measures (p = 0.021). The greatest increase in Ldp following exposure was seen at 5888.5 Hz (mean shift = 13.25 dB). Post-exposure Ldp in rabbits exposed to WBV were not significantly different between the right and left ears (p = 0.083). Conclusion WBV impairs cochlear function resulting in increased DPOAE responses in rabbits exposed to WBV. DPOAE level shifts occurred over a wide range of frequencies following prolonged WBV in rabbits. PMID:22720724

Monaural or binaural sound deprivation in postlingual hearing loss: Cochlear implant in the worse ear.

PubMed

Canale, Andrea; Dalmasso, Giulia; Dagna, Federico; Lacilla, Michelangelo; Montuschi, Carla; Rosa, Rosalba Di; Albera, Roberto

2016-08-01

To determine whether speech recognition scores (SRS) differ between adults with long-term auditory deprivation in the implanted ear and adults who received cochlear implant (CI) in the nonsound-deprived ear, either for hearing aid-assisted or due to rapidly deteriorating hearing loss. Retrospective study. Speech recognition scores at evaluations (3 and 14 months postimplantation) conducted with CI alone at 60-dB sound pressure level intensity were compared in 15 patients (4 with bilateral severe hearing loss; 11 with asymmetric hearing loss, 7 of which had contralateral hearing aid), all with long-term auditory deprivation (mean duration 16.9 years) (group A), and in 15 other patients with postlingual hearing loss (10 symmetric, 5 asymmetric with bimodal stimulation) (controls, group B). Comparison of mean percentage of correctly recognized words on speech audiometry at 3 and 14 months showed improvement within each group (P < 0.05). Between-group comparison showed no significant difference at 3 (P = 0.17) or 14 months (P = 0.46). Comparison of SRSs in group A (bimodal stimulation [n = 7] and binaural sound deprivation [n = 4]) versus group B showed no significant differences at 3 (bimodal stimulation P = 0.16; binaural sound deprivation P = 0.19) or 14 months (bimodal stimulation P = 0.14; binaural sound deprivation P = 0.82). Speech recognition scores in monaural and binaural sound-deprived ears did not significantly differ from ears with unilateral cochlear implantation in nonsound-deprived ears when tested with CI alone. Improvement in the implanted worse ear indicates that it could be a potential candidate ear for cochlear implantation even when sound deprived. 4. Laryngoscope, 126:1905-1910, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Metalloproteinases and their associated genes contribute to the functional integrity and noise-induced damage in the cochlear sensory epithelium

PubMed Central

Hu, Bo Hua; Cai, Qunfeng; Hu, Zihua; Patel, Minal; Bard, Jonathan; Jamison, Jennifer; Coling, Donald

2012-01-01

Matrix metalloproteinases (MMPs) and their related gene products regulate essential cellular functions. An imbalance in MMPs has been implicated in various neurological disorders, including traumatic injuries. Here, we report a role for MMPs and their related gene products in the modulation of cochlear responses to acoustic trauma in rats. The normal cochlea was shown to be enriched in MMP enzymatic activity, and this activity was reduced in a time-dependent fashion after traumatic noise injury. The analysis of gene expression by RNA-seq and qRT-PCR revealed the differential expression of MMPs and their related genes between functionally specialized regions of the sensory epithelium. The expression of these genes was dynamically regulated between the acute and chronic phases of noise-induced hearing loss. Moreover, noise-induced expression changes in two endogenous MMP inhibitors, Timp1 and Timp2, in sensory cells were dependent upon the stage of nuclear condensation, suggesting a specific role for MMP activity in sensory cell apoptosis. A short-term application of doxycycline, a broad-spectrum inhibitor of MMPs, prior to noise exposure reduced noise-induced hearing loss and sensory cell death. By contrast, a 7-day treatment compromised hearing sensitivity and potentiated noise-induced hearing loss. This detrimental effect of the long-term inhibition of MMPs on noise-induced hearing loss was further confirmed using targeted Mmp7 knockout mice. Together, these observations suggest that MMPs and their related genes participate in the regulation of cochlear responses to acoustic overstimulation and that the modulation of MMP activity can serve as a novel therapeutic target for the reduction of noise-induced cochlear damage. PMID:23100416

Simultaneous versus sequential bilateral cochlear implants in adults: Cost analysis in a US setting.

PubMed

Trinidade, Aaron; Page, Joshua C; Kennett, Sarah W; Cox, Matthew D; Dornhoffer, John L

2017-11-01

From a purely surgical efficiency point of view, simultaneous cochlear implantation (SimCI) is more cost-effective than sequential cochlear implantation (SeqCI) when total direct costs are considered (implant and hospital costs). However, in a setting where only SeqCI is practiced and a proportion of initially unilaterally implanted patients do not progress to a second implant, this may not be the case, especially when audiological costs are factored in. We present a cost analysis of such a scenario as would occur in our institution. Retrospective review and cost analysis. Between 2005 and 2015, 370 patients fulfilled the audiological criteria for bilateral implantation. Of those, 267 (72.1%) underwent unilateral cochlear implantation only, 101 (27.3%) progressed to SeqCI, and two underwent SimCI. The total hospital, surgical, and implant costs, and initial implant stimulation series audiological costs between August 2015 and August 2016 (29 adult patients) were used in this analysis. The total hospital, surgical, and implant costs for this period was $2,731,360.42. Based on previous local trends, if a projected eight (27.3%) of these patients decide to progress to SeqCI, this will cost an additional $750,811.04, resulting in an overall total of $3,482,171.46 for these 29 patients. Had all 29 undergone SimCI, the total projected cost would have been $3,332,991.75, representing a total potential saving of $149,179.67 (4.3%). In institutions where only SeqCI is allowed in adults, overall patient management may cost marginally more than if SimCI were practiced. This will be of interest to CI programs and health insurance companies. 4. Laryngoscope, 127:2615-2618, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Language, Mental State Vocabulary, and False Belief Understanding in Children with Cochlear Implants

ERIC Educational Resources Information Center

Peters, Kimberly; Remmel, Ethan; Richards, Debra

2009-01-01

Purpose: This study examined false belief understanding and its predictors in school-age children who are deaf with cochlear implants and who use spoken language. Method: False belief understanding was measured through an explanation-of-action task in 30 children between the ages of 3 and 12 years who used cochlear implants. Children told a…

Cyborgization: Deaf Education for Young Children in the Cochlear Implantation Era

ERIC Educational Resources Information Center

Valente, Joseph Michael

2011-01-01

The author, who was raised oral deaf himself, recounts a visit to a school for young deaf children and discovers that young d/Deaf children and their rights are subverted by the cochlear implantation empire. The hypercapitalist, techno-manic times of cochlear implantation has wreaked havoc to the lives of not only young children with deafness but…

Cochlear Implants. National Institutes of Health Consensus Development Conference Statement (May 4, 1988). Volume 7, Number 2.

ERIC Educational Resources Information Center

National Institutes of Health (DHHS), Bethesda, MD.

This paper reports the results of a Consensus Development Conference on Cochlear Implants sponsored by the National Institutes of Health to improve the hearing of children and adults with hearing impairments. The following questions are addressed: (1) Who is a suitable candidate for a cochlear implant? (2) What are the advantages and disadvantages…

Auditory Environment across the Life Span of Cochlear Implant Users: Insights from Data Logging

ERIC Educational Resources Information Center

Busch, Tobias; Vanpoucke, Filiep; van Wieringen, Astrid

2017-01-01

Purpose: We describe the natural auditory environment of people with cochlear implants (CIs), how it changes across the life span, and how it varies between individuals. Method: We performed a retrospective cross-sectional analysis of Cochlear Nucleus 6 CI sound-processor data logs. The logs were obtained from 1,501 people with CIs (ages 0-96…

Congenitally Deafblind Children and Cochlear Implants: Effects on Communication

ERIC Educational Resources Information Center

Dammeyer, Jesper

2009-01-01

There has been much research conducted demonstrating the positive benefits of cochlear implantation (CI) in children who are deaf. Research on CI in children who are both deaf and blind, however, is lacking. The purpose of this article is to present a study of five congenitally deafblind children who received cochlear implants between 2.2 and 4.2…

Searching for Cochlear Implant Information on the Internet Maze: Implications for Parents and Professionals

ERIC Educational Resources Information Center

Zaidman-Zait, Anat; Jamieson, Janet R.

2004-01-01

The present study has three purposes: (a) to determine who disseminates information on cochlear implants on the Web; (b) to describe a representative sample of Web sites that disseminate information on cochlear implants, with a focus on the content topics and their relevance to parents of deaf children; and (c) to discuss the practical issues of…

Benefit of Wearing a Hearing Aid on the Unimplanted Ear in Adult Users of a Cochlear Implant

ERIC Educational Resources Information Center

Dunn, Camille C.; Tyler, Richard S.; Witt, Shelley A.

2005-01-01

The purpose of this investigation was to document performance of participants wearing a cochlear implant and hearing aid in opposite ears on speech-perception and localization tests. Twelve individuals who wore a cochlear implant and a hearing aid on contralateral ears were tested on their abilities to understand words in quiet and sentences in…

A Longitudinal Study in Children with Sequential Bilateral Cochlear Implants: Time Course for the Second Implanted Ear and Bilateral Performance

ERIC Educational Resources Information Center

Reeder, Ruth M.; Firszt, Jill B.; Cadieux, Jamie H.; Strube, Michael J.

2017-01-01

Purpose: Whether, and if so when, a second-ear cochlear implant should be provided to older, unilaterally implanted children is an ongoing clinical question. This study evaluated rate of speech recognition progress for the second implanted ear and with bilateral cochlear implants in older sequentially implanted children and evaluated localization…

Educational Interpreters: Meeting the Communication Needs of Children with Cochlear Implants

ERIC Educational Resources Information Center

Melton, Julie; Higbee, Renee

2013-01-01

Since the early 1990s, when the U.S. Food and Drug Administration approved cochlear implants for deaf and hard of hearing children, the number of children who have cochlear implants has increased in mainstream settings. Recent research suggests that these students, like their deaf and hard of hearing peers without implants who use sign language,…

Prevention and management of cochlear implant infections.

PubMed

Gluth, Michael B; Singh, Rajesh; Atlas, Marcus D

2011-11-01

Understanding the issues of infection related to an implantable medical device is crucial to all cochlear implant teams. Furthermore, given the risk of central nervous system complications and the relatively high quantity of underlying resource investment associated with cochlear implantation, the stakes of infection are high. The optimal strategies to prevent and manage such infections are still evolving as good-quality prospective data to guide such management decisions are not yet abundant within the medical literature and many recommendations are based on retrospective reviews or anecdotal evidence. We will outline a general strategy to deal with cochlear implant-related infection based on both the authors' experience and the published literature.

Development of a phase-sensitive Fourier domain optical coherence tomography system to measure mouse organ of Corti vibrations in two cochlear turns

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ramamoorthy, Sripriya; Zhang, Yuan; Jacques, Steven

In this study, we have developed a phase-sensitive Fourier-domain optical coherence tomography system to simultaneously measure the in vivo inner ear vibrations in the hook area and second turn of the mouse cochlea. This technical development will enable measurement of intra-cochlear distortion products at ideal locations such as the distortion product generation site and reflection site. This information is necessary to un-mix the complex mixture of intra-cochlear waves comprising the DPOAE and thus leads to the non-invasive identification of the local region of cochlear damage.

Use of Neural Response Telemetry for Pediatric Cochlear Implants: Current Practice.

PubMed

Almosnino, Galit; Anne, Samantha; Schwartz, Seth R

2018-06-01

Evaluate usage trends of neural response telemetry (NRT) in cochlear implant centers across the nation and assess reported benefits of intraoperative NRT for pediatric cochlear implant recipients. Survey. All US cochlear implant centers (n = 110). A 15-question multiple-choice survey was distributed electronically to all centers. The survey captured demographic information of all centers, practice patterns surrounding the use of NRT, and the extent to which intraoperative NRT is of benefit. Thirty-two invited participants (29%) completed the survey. A majority of participants reported practicing in an academic center (66%), followed by a hospital setting (19%) and private practice (16%). Seventy-two percent of survey participants reported using NRT for pediatric cochlear implant recipients. Sixty-three percent felt it improved the ability to program at initial activation, and 50% of participants felt that NRT improves satisfaction at initial activation. This study suggests that a majority of surgeons use intraoperative NRT for pediatric cochlear implantation as an additional measure to ensure appropriate electrode placement and improve device activation. Larger studies are needed to better establish the relationship between intraoperative NRT and postoperative outcomes and justify the additional costs associated with intraoperative NRT.

Topics in Modeling of Cochlear Dynamics: Computation, Response and Stability Analysis

NASA Astrophysics Data System (ADS)

Filo, Maurice G.

This thesis touches upon several topics in cochlear modeling. Throughout the literature, mathematical models of the cochlea vary according to the degree of biological realism to be incorporated. This thesis casts the cochlear model as a continuous space-time dynamical system using operator language. This framework encompasses a wider class of cochlear models and makes the dynamics more transparent and easier to analyze before applying any numerical method to discretize space. In fact, several numerical methods are investigated to study the computational efficiency of the finite dimensional realizations in space. Furthermore, we study the effects of the active gain perturbations on the stability of the linearized dynamics. The stability analysis is used to explain possible mechanisms underlying spontaneous otoacoustic emissions and tinnitus. Dynamic Mode Decomposition (DMD) is introduced as a useful tool to analyze the response of nonlinear cochlear models. Cochlear response features are illustrated using DMD which has the advantage of explicitly revealing the spatial modes of vibrations occurring in the Basilar Membrane (BM). Finally, we address the dynamic estimation problem of BM vibrations using Extended Kalman Filters (EKF). Due to the limitations of noninvasive sensing schemes, such algorithms are inevitable to estimate the dynamic behavior of a living cochlea.

Voice gender identification by cochlear implant users: The role of spectral and temporal resolution

NASA Astrophysics Data System (ADS)

Fu, Qian-Jie; Chinchilla, Sherol; Nogaki, Geraldine; Galvin, John J.

2005-09-01

The present study explored the relative contributions of spectral and temporal information to voice gender identification by cochlear implant users and normal-hearing subjects. Cochlear implant listeners were tested using their everyday speech processors, while normal-hearing subjects were tested under speech processing conditions that simulated various degrees of spectral resolution, temporal resolution, and spectral mismatch. Voice gender identification was tested for two talker sets. In Talker Set 1, the mean fundamental frequency values of the male and female talkers differed by 100 Hz while in Talker Set 2, the mean values differed by 10 Hz. Cochlear implant listeners achieved higher levels of performance with Talker Set 1, while performance was significantly reduced for Talker Set 2. For normal-hearing listeners, performance was significantly affected by the spectral resolution, for both Talker Sets. With matched speech, temporal cues contributed to voice gender identification only for Talker Set 1 while spectral mismatch significantly reduced performance for both Talker Sets. The performance of cochlear implant listeners was similar to that of normal-hearing subjects listening to 4-8 spectral channels. The results suggest that, because of the reduced spectral resolution, cochlear implant patients may attend strongly to periodicity cues to distinguish voice gender.

Social participation of children and adolescents with cochlear implants: a qualitative analysis of parent, teacher, and child interviews.

PubMed

Punch, Renée; Hyde, Merv

2011-01-01

Psychosocial factors, including socioemotional well-being, peer relationships, and social inclusion with hearing and deaf peers, are increasingly becoming a focus of research investigating children with cochlear implants. The study reported here extends the largely quantitative findings of previous research through a qualitative analysis of interviews with parents, teachers, and pediatric cochlear implant users themselves in three eastern states of Australia. We interviewed 24 parents, 15 teachers, and 11 children and adolescents. The findings displayed commonalities across the three groups of participants, indicating positive experiences around the children's psychosocial development with their cochlear implants, but also ongoing difficulties communicating in groups of people and problems related to social skills. Some children had little contact with other deaf children (with or without cochlear implants) despite parents and teachers perceiving such contact beneficial. Children attending schools where there were other deaf children valued friendships with both deaf and hearing peers. Adolescence was a particularly difficult time for some as they struggled with feelings of self-consciousness about their deafness and external cochlear implant equipment and worries around friendships, dating, and their future place in the world. Recommendations for practice and further research are made.

Auditory Speech Perception Tests in Relation to the Coding Strategy in Cochlear Implant.

PubMed

Bazon, Aline Cristine; Mantello, Erika Barioni; Gonçales, Alina Sanches; Isaac, Myriam de Lima; Hyppolito, Miguel Angelo; Reis, Ana Cláudia Mirândola Barbosa

2016-07-01

The objective of the evaluation of auditory perception of cochlear implant users is to determine how the acoustic signal is processed, leading to the recognition and understanding of sound. To investigate the differences in the process of auditory speech perception in individuals with postlingual hearing loss wearing a cochlear implant, using two different speech coding strategies, and to analyze speech perception and handicap perception in relation to the strategy used. This study is prospective cross-sectional cohort study of a descriptive character. We selected ten cochlear implant users that were characterized by hearing threshold by the application of speech perception tests and of the Hearing Handicap Inventory for Adults. There was no significant difference when comparing the variables subject age, age at acquisition of hearing loss, etiology, time of hearing deprivation, time of cochlear implant use and mean hearing threshold with the cochlear implant with the shift in speech coding strategy. There was no relationship between lack of handicap perception and improvement in speech perception in both speech coding strategies used. There was no significant difference between the strategies evaluated and no relation was observed between them and the variables studied.

Effects of a Word-Learning Training on Children With Cochlear Implants

PubMed Central

Lund, Emily

2014-01-01

Preschool children with hearing loss who use cochlear implants demonstrate vocabulary delays when compared to their peers without hearing loss. These delays may be a result of deficient word-learning abilities; children with cochlear implants perform more poorly on rapid word-learning tasks than children with normal hearing. This study explored the malleability of rapid word learning of preschoolers with cochlear implants by evaluating the effects of a word-learning training on rapid word learning. A single-subject, multiple probe design across participants measured the impact of the training on children’s rapid word-learning performance. Participants included 5 preschool children with cochlear implants who had an expressive lexicon of less than 150 words. An investigator guided children to identify, repeat, and learn about unknown sets of words in 2-weekly sessions across 10 weeks. The probe measure, a rapid word-learning task with a different set of words than those taught during training, was collected in the baseline, training, and maintenance conditions. All participants improved their receptive rapid word-learning performance in the training condition. The functional relation indicates that the receptive rapid word-learning performance of children with cochlear implants is malleable. PMID:23981321

The cochlear implant as a tinnitus treatment.

PubMed

Vallés-Varela, Héctor; Royo-López, Juan; Carmen-Sampériz, Luis; Sebastián-Cortés, José M; Alfonso-Collado, Ignacio

2013-01-01

Tinnitus is a symptom of high prevalence in patients with cochlear pathology. We studied the evolution of tinnitus in patients undergoing unilateral cochlear implantation for treatment of profound hearing loss. This was a longitudinal, retrospective study of patients that underwent unilateral cochlear implantation and who had bilateral tinnitus. Tinnitus was assessed quantitatively and qualitatively before surgery and at 6 and 12 months after surgery. We evaluated 20 patients that underwent unilateral cochlear implantation with a Nucleus(®) CI24RE Contour Advance™ electrode device. During the periods in which the device was in operation, improvement or disappearance of tinnitus was evidenced in the ipsilateral ear in 65% of patients, and in the contralateral ear, in 50%. In periods in which the device was disconnected, improvement or disappearance of tinnitus was found in the ipsilateral ear in 50% of patients, and in the ear contralateral to the implant in 45% of the patients. In 10% of the patients, a new tinnitus appeared in the ipsilateral ear. The patients with profound hearing loss and bilateral tinnitus treated with unilateral cochlear implantation improved in a high percentage of cases, in the ipsilateral ear and in the contralateral ear. Copyright © 2012 Elsevier España, S.L. All rights reserved.

Clinical outcomes of scala vestibuli cochlear implantation in children with partial labyrinthine ossification.

PubMed

Lin, Yung-Song

2009-03-01

Cochlear implantation via the scala vestibuli is a viable approach in those with ossification in the scala tympani. With extended cochlear implant experience, there is no significant difference in the mapping parameters and auditory performance between those implanted via scala vestibuli and via scala tympani. To assess the clinical outcomes of cochlear implantation via scala vestibuli. In a cohort follow-up study, 11 prelingually deafened children who received cochlear implantation between age 3 and 10 years through the scala vestibuli served as participants. The mapping parameters (i.e. comfortable level (C), threshold level (T), dynamic range) and auditory performance of each participant were evaluated following initial cochlear implant stimulation, then at 3 month intervals for 2 years, then semi-annually. The follow-up period lasted for 9 years 9 months on average, with a minimum of 8 years 3 months. The clinical results of the mapping parameters and auditory performance of children implanted via the scala vestibuli were comparative to those who were implanted via the scala tympani. No balance problem was reported by any of these patients. One child exhibited residual low frequency hearing after implantation.

Current Research with Cochlear Implants at Arizona State University

PubMed Central

Dorman, Michael F.; Spahr, Anthony; Gifford, Rene H.; Cook, Sarah; Zhang, Ting; Loiselle, Louise; Yost, William; Cardy, Lara; Whittingham, JoAnne; Schramm, David

2013-01-01

In this article we review, and discuss the clinical implications of, five projects currently underway in the Cochlear Implant Laboratory at Arizona State University. The projects are (1) norming the AzBio sentence test, (2) comparing the performance of bilateral and bimodal cochlear implant (CI) patients in realistic listening environments, (3) accounting for the benefit provided to bimodal patients by low-frequency acoustic stimulation, (4) assessing localization by bilateral hearing aid patients and the implications of that work for hearing preservation patients, and (5) studying heart rate variability as a possible measure for quantifying the stress of listening via an implant. The long-term goals of the laboratory are to improve the performance of patients fit with cochlear implants and to understand the mechanisms, physiological or electronic, that underlie changes in performance. We began our work with cochlear implant patients in the mid-1980s and received our first grant from the National Institutes of Health (NIH) for work with implanted patients in 1989. Since that date our work with cochlear implant patients has been funded continuously by the NIH. In this report we describe some of the research currently being conducted in our laboratory. PMID:22668760

3D optical coherence tomography image registration for guiding cochlear implant insertion

NASA Astrophysics Data System (ADS)

Cheon, Gyeong-Woo; Jeong, Hyun-Woo; Chalasani, Preetham; Chien, Wade W.; Iordachita, Iulian; Taylor, Russell; Niparko, John; Kang, Jin U.

2014-03-01

In cochlear implant surgery, an electrode array is inserted into the cochlear canal to restore hearing to a person who is profoundly deaf or significantly hearing impaired. One critical part of the procedure is the insertion of the electrode array, which looks like a thin wire, into the cochlear canal. Although X-ray or computed tomography (CT) could be used as a reference to evaluate the pathway of the whole electrode array, there is no way to depict the intra-cochlear canal and basal turn intra-operatively to help guide insertion of the electrode array. Optical coherent tomography (OCT) is a highly effective way of visualizing internal structures of cochlea. Swept source OCT (SSOCT) having center wavelength of 1.3 micron and 2D Galvonometer mirrors was used to achieve 7-mm depth 3-D imaging. Graphics processing unit (GPU), OpenGL, C++ and C# were integrated for real-time volumetric rendering simultaneously. The 3D volume images taken by the OCT system were assembled and registered which could be used to guide a cochlear implant. We performed a feasibility study using both dry and wet temporal bones and the result is presented.

The importance of electrically evoked stapedial reflex in cochlear implant.

PubMed

Andrade, Kelly Cristina Lira de; Leal, Mariana de Carvalho; Muniz, Lilian Ferreira; Menezes, Pedro de Lemos; Albuquerque, Katia Maria Gomes de; Carnaúba, Aline Tenório Lins

2014-01-01

The most important stage in fitting a cochlear implant is the identification of its dynamic range. The use of objective measures, in particular the electrically elicited stapedius reflex, may provide suitable assistence for initial fitting of cochlear implant, especially in children or adult with multiple disorders, because they provide specific values that serve as the basis of early cochlear implant programming. Verify through a review the use of the electrically elicited stapedius reflex threshold during the activation and mapping process of cochlear implant. Bibliographical search on the Pubmed and Bireme plataforms, and also on Medline, LILACS and SciELO databases, with standard searches until September 2012, using specific keywords. For the selection and evaluation of scientific studies found in the search, criterias have been established, considering the following aspects: author, year/location, grade of recommendation/level of evidence, purpose, sample, age, mean age in years, evaluative testing, results and conclusion. Among 7,304 articles found, 7,080 were excluded from the title, 152 from the abstract, 17 from the article reading, 43 were repeated and 12 were selected for the study. The electrically elicited stapedius reflex may support when programming the cochlear implant, especially in patients with inconsistent responses.

The relationship between loudness intensity functions and the click-ABR wave V latency.

PubMed

Serpanos, Y C; O'Malley, H; Gravel, J S

1997-10-01

To assess the relationship of loudness growth and the click-evoked auditory brain stem response (ABR) wave V latency-intensity function (LIF) in listeners with normal hearing or cochlear hearing loss. The effect of hearing loss configuration on the intensity functions was also examined. Behavioral and electrophysiological intensity functions were obtained using click stimuli of comparable intensities in listeners with normal hearing (Group I; n = 10), and cochlear hearing loss of flat (Group II; n = 10) or sloping (Group III; n = 10) configurations. Individual intensity functions were obtained from measures of loudness growth using the psychophysical methods of absolute magnitude estimation and production of loudness (geometrically averaged to provide the measured loudness function), and from the wave V latency measures of the ABR. Slope analyses for the behavioral and electrophysiological intensity functions were separately performed by group. The loudness growth functions for the groups with cochlear hearing loss approximated the normal function at high intensities, with overall slope values consistent with those reported from previous psychophysical research. The ABR wave V LIF for the group with a flat configuration of cochlear hearing loss approximated the normal function at high intensities, and was displaced parallel to the normal function for the group with sloping configuration. The relationship between the behavioral and electrophysiological intensity functions was examined at individual intensities across the range of the functions for each subject. A significant relationship was obtained between loudness and the ABR wave V LIFs for the groups with normal hearing and flat configuration of cochlear hearing loss; the association was not significant (p = 0.10) for the group with a sloping configuration of cochlear hearing loss. The results of this study established a relationship between loudness and the ABR wave V latency for listeners with normal hearing, and flat cochlear hearing loss. In listeners with a sloping configuration of cochlear hearing loss, the relationship was not significant. This suggests that the click-evoked ABR may be used to estimate loudness growth at least for individuals with normal hearing and those with a flat configuration of cochlear hearing loss. Predictive equations were derived to estimate loudness growth for these groups. The use of frequency-specific stimuli may provide more precise information on the nature of the relationship between loudness growth and the ABR wave V latency, particularly for listeners with sloping configurations of cochlear hearing loss.

Response Changes During Insertion of a Cochlear Implant Using Extracochlear Electrocochleography.

PubMed

Giardina, Christopher K; Khan, Tatyana E; Pulver, Stephen H; Adunka, Oliver F; Buchman, Craig A; Brown, Kevin D; Pillsbury, Harold C; Fitzpatrick, Douglas C

2018-03-16

Electrocochleography is increasingly being utilized as an intraoperative monitor of cochlear function during cochlear implantation (CI). Intracochlear recordings from the advancing electrode can be obtained through the device by on-board capabilities. However, such recordings may not be ideal as a monitor because the recording electrode moves in relation to the neural and hair cell generators producing the responses. The purposes of this study were to compare two extracochlear recording locations in terms of signal strength and feasibility as intraoperative monitoring sites and to characterize changes in cochlear physiology during CI insertion. In 83 human subjects, responses to 90 dB nHL tone bursts were recorded both at the round window (RW) and then at an extracochlear position-either adjacent to the stapes or on the promontory just superior to the RW. Recording from the fixed, extracochlear position continued during insertion of the CI in 63 cases. Before CI insertion, responses to low-frequency tones at the RW were roughly 6 dB larger than when recording at either extracochlear site, but the two extracochlear sites did not differ from one another. During CI insertion, response losses from the promontory or adjacent to the stapes stayed within 5 dB in ≈61% (38/63) of cases, presumably indicating atraumatic insertions. Among responses which dropped more than 5 dB at any time during CI insertion, 12 subjects showed no response recovery, while in 13, the drop was followed by partial or complete response recovery by the end of CI insertion. In cases with recovery, the drop in response occurred relatively early (<15 mm insertion) compared to those where there was no recovery. Changes in response phase during the insertion occurred in some cases; these may indicate a change in the distributions of generators contributing to the response. Monitoring the electrocochleography during CI insertion from an extracochlear site reveals insertions that are potentially atraumatic show interaction with cochlear structures followed by response recovery or show interactions such that response losses persist to the end of recording.

Computational Evaluation of Cochlear Implant Surgery Outcomes Accounting for Uncertainty and Parameter Variability.

PubMed

Mangado, Nerea; Pons-Prats, Jordi; Coma, Martí; Mistrík, Pavel; Piella, Gemma; Ceresa, Mario; González Ballester, Miguel Á

2018-01-01

Cochlear implantation (CI) is a complex surgical procedure that restores hearing in patients with severe deafness. The successful outcome of the implanted device relies on a group of factors, some of them unpredictable or difficult to control. Uncertainties on the electrode array position and the electrical properties of the bone make it difficult to accurately compute the current propagation delivered by the implant and the resulting neural activation. In this context, we use uncertainty quantification methods to explore how these uncertainties propagate through all the stages of CI computational simulations. To this end, we employ an automatic framework, encompassing from the finite element generation of CI models to the assessment of the neural response induced by the implant stimulation. To estimate the confidence intervals of the simulated neural response, we propose two approaches. First, we encode the variability of the cochlear morphology among the population through a statistical shape model. This allows us to generate a population of virtual patients using Monte Carlo sampling and to assign to each of them a set of parameter values according to a statistical distribution. The framework is implemented and parallelized in a High Throughput Computing environment that enables to maximize the available computing resources. Secondly, we perform a patient-specific study to evaluate the computed neural response to seek the optimal post-implantation stimulus levels. Considering a single cochlear morphology, the uncertainty in tissue electrical resistivity and surgical insertion parameters is propagated using the Probabilistic Collocation method, which reduces the number of samples to evaluate. Results show that bone resistivity has the highest influence on CI outcomes. In conjunction with the variability of the cochlear length, worst outcomes are obtained for small cochleae with high resistivity values. However, the effect of the surgical insertion length on the CI outcomes could not be clearly observed, since its impact may be concealed by the other considered parameters. Whereas the Monte Carlo approach implies a high computational cost, Probabilistic Collocation presents a suitable trade-off between precision and computational time. Results suggest that the proposed framework has a great potential to help in both surgical planning decisions and in the audiological setting process.

Tumor necrosis factor-α enhances microvascular tone and reduces blood flow in the cochlea via enhanced sphingosine-1-phosphate signaling.

PubMed

Scherer, Elias Q; Yang, Jingli; Canis, Martin; Reimann, Katrin; Ivanov, Karolina; Diehl, Christian D; Backx, Peter H; Wier, W Gil; Strieth, Sebastian; Wangemann, Philine; Voigtlaender-Bolz, Julia; Lidington, Darcy; Bolz, Steffen-Sebastian

2010-11-01

We sought to demonstrate that tumor necrosis factor (TNF)-α, via sphingosine-1-phosphate signaling, has the potential to alter cochlear blood flow and thus, cause ischemic hearing loss. We performed intravital fluorescence microscopy to measure blood flow and capillary diameter in anesthetized guinea pigs. To measure capillary diameter ex vivo, capillary beds from the gerbil spiral ligament were isolated from the cochlear lateral wall and maintained in an organ bath. Isolated gerbil spiral modiolar arteries, maintained and transfected in organ culture, were used to measure calcium sensitivity (calcium-tone relationship). In a clinical study, a total of 12 adult patients presenting with typical symptoms of sudden hearing loss who were not responsive or only partially responsive to prednisolone treatment were identified and selected for etanercept treatment. Etanercept (25 mg s.c.) was self-administered twice a week for 12 weeks. TNF-α induced a proconstrictive state throughout the cochlear microvasculature, which reduced capillary diameter and cochlear blood flow in vivo. In vitro isolated preparations of the spiral modiolar artery and spiral ligament capillaries confirmed these observations. Antagonizing sphingosine-1-phosphate receptor 2 subtype signaling (by 1 μmol/L JTE013) attenuated the effects of TNF-α in all models. TNF-α activated sphingosine kinase 1 (Sk1) and induced its translocation to the smooth muscle cell membrane. Expression of a dominant-negative Sk1 mutant (Sk1(G82D)) eliminated both baseline spiral modiolar artery calcium sensitivity and TNF-α effects, whereas a nonphosphorylatable Sk1 mutant (Sk1(S225A)) blocked the effects of TNF-α only. A small group of etanercept-treated, hearing loss patients recovered according to a 1-phase exponential decay (half-life=1.56 ± 0.20 weeks), which matched the kinetics predicted for a vascular origin. TNF-α indeed reduces cochlear blood flow via activation of vascular sphingosine-1-phosphate signaling. This integrates hearing loss into the family of ischemic microvascular pathologies, with implications for risk stratification, diagnosis, and treatment.

Speech Recognition in Adults With Cochlear Implants: The Effects of Working Memory, Phonological Sensitivity, and Aging.

PubMed

Moberly, Aaron C; Harris, Michael S; Boyce, Lauren; Nittrouer, Susan

2017-04-14

Models of speech recognition suggest that "top-down" linguistic and cognitive functions, such as use of phonotactic constraints and working memory, facilitate recognition under conditions of degradation, such as in noise. The question addressed in this study was what happens to these functions when a listener who has experienced years of hearing loss obtains a cochlear implant. Thirty adults with cochlear implants and 30 age-matched controls with age-normal hearing underwent testing of verbal working memory using digit span and serial recall of words. Phonological capacities were assessed using a lexical decision task and nonword repetition. Recognition of words in sentences in speech-shaped noise was measured. Implant users had only slightly poorer working memory accuracy than did controls and only on serial recall of words; however, phonological sensitivity was highly impaired. Working memory did not facilitate speech recognition in noise for either group. Phonological sensitivity predicted sentence recognition for implant users but not for listeners with normal hearing. Clinical speech recognition outcomes for adult implant users relate to the ability of these users to process phonological information. Results suggest that phonological capacities may serve as potential clinical targets through rehabilitative training. Such novel interventions may be particularly helpful for older adult implant users.

Tauroursodeoxycholic acid attenuates gentamicin-induced cochlear hair cell death in vitro.

PubMed

Jia, Zhanwei; He, Qiang; Shan, Chunguang; Li, Fengyi

2018-09-15

Gentamycin is one of the most clinically used aminoglycoside antibiotics which induce intrinsic apoptosis of hair cells. Tauroursodeoxycholic acid (TUDCA) is known as safe cell-protective agent in disorders associated with apoptosis. We aimed to investigate the protective effects of TUDCA against gentamicin-induced ototoxicity. House Ear Institute-Organ of Corti 1(HEI-OC1) cells and explanted cochlear tissue were treated with gentamicin and TUDCA, followed by serial analyses including cell viability assay, hair cell staining, qPCR, ELISA and western blotting to determine the cell damage by the parameters relevant to cell apoptosis and endoplasmic reticulum stress. TUDCA significantly attenuated gentamicin-induced cell damage in cultured HEI-OC1 cells and explanted cochlear hair cells. TUDCA alleviated gentamicin-induced cell apoptosis, supported by the decreased Bax/Bcl2 ratio compared with that of gentamicin treated alone. TUDCA decreased gentamicin-induced nitric oxide production and protein nitration in both models. In addition, TUDCA suppressed gentamicin-induced endoplasmic reticulum stress as reflected by inversing the expression levels of Binding immunoglobulin protein (Bip), CCAAT/-enhancer-binding protein homologous protein (CHOP) and Caspase 3. TUDCA attenuated gentamicin-induced hair cell death by inhibiting protein nitration activation and ER stress, providing new insights into the new potential therapies for sensorineural deafness. Copyright © 2018 Elsevier B.V. All rights reserved.

Speech Recognition in Adults With Cochlear Implants: The Effects of Working Memory, Phonological Sensitivity, and Aging

PubMed Central

Harris, Michael S.; Boyce, Lauren; Nittrouer, Susan

2017-01-01

Purpose Models of speech recognition suggest that “top-down” linguistic and cognitive functions, such as use of phonotactic constraints and working memory, facilitate recognition under conditions of degradation, such as in noise. The question addressed in this study was what happens to these functions when a listener who has experienced years of hearing loss obtains a cochlear implant. Method Thirty adults with cochlear implants and 30 age-matched controls with age-normal hearing underwent testing of verbal working memory using digit span and serial recall of words. Phonological capacities were assessed using a lexical decision task and nonword repetition. Recognition of words in sentences in speech-shaped noise was measured. Results Implant users had only slightly poorer working memory accuracy than did controls and only on serial recall of words; however, phonological sensitivity was highly impaired. Working memory did not facilitate speech recognition in noise for either group. Phonological sensitivity predicted sentence recognition for implant users but not for listeners with normal hearing. Conclusion Clinical speech recognition outcomes for adult implant users relate to the ability of these users to process phonological information. Results suggest that phonological capacities may serve as potential clinical targets through rehabilitative training. Such novel interventions may be particularly helpful for older adult implant users. PMID:28384805

The perception of complex pitch in cochlear implants: A comparison of monopolar and tripolar stimulation.

PubMed

Fielden, Claire A; Kluk, Karolina; Boyle, Patrick J; McKay, Colette M

2015-10-01

Cochlear implant listeners typically perform poorly in tasks of complex pitch perception (e.g., musical pitch and voice pitch). One explanation is that wide current spread during implant activation creates channel interactions that may interfere with perception of temporal fundamental frequency information contained in the amplitude modulations within channels. Current focusing using a tripolar mode of stimulation has been proposed as a way of reducing channel interactions, minimising spread of excitation and potentially improving place and temporal pitch cues. The present study evaluated the effect of mode in a group of cochlear implant listeners on a pitch ranking task using male and female singing voices separated by either a half or a quarter octave. Results were variable across participants, but on average, pitch ranking was at chance level when the pitches were a quarter octave apart and improved when the difference was a half octave. No advantage was observed for tripolar over monopolar mode at either pitch interval, suggesting that previously published psychophysical advantages for focused modes may not translate into improvements in complex pitch ranking. Evaluation of the spectral centroid of the stimulation pattern, plus a lack of significant difference between male and female voices, suggested that participants may have had difficulty in accessing temporal pitch cues in either mode.

Pitch ranking, electrode discrimination, and physiological spread-of-excitation using Cochlear's dual-electrode mode.

PubMed

Goehring, Jenny L; Neff, Donna L; Baudhuin, Jacquelyn L; Hughes, Michelle L

2014-08-01

This study compared pitch ranking, electrode discrimination, and electrically evoked compound action potential (ECAP) spatial excitation patterns for adjacent physical electrodes (PEs) and the corresponding dual electrodes (DEs) for newer-generation Cochlear devices (Cochlear Ltd., Macquarie, New South Wales, Australia). The first goal was to determine whether pitch ranking and electrode discrimination yield similar outcomes for PEs and DEs. The second goal was to determine if the amount of spatial separation among ECAP excitation patterns (separation index, Σ) between adjacent PEs and the PE-DE pairs can predict performance on the psychophysical tasks. Using non-adaptive procedures, 13 subjects completed pitch ranking and electrode discrimination for adjacent PEs and the corresponding PE-DE pairs (DE versus each flanking PE) from the basal, middle, and apical electrode regions. Analysis of d' scores indicated that pitch-ranking and electrode-discrimination scores were not significantly different, but rather produced similar levels of performance. As expected, accuracy was significantly better for the PE-PE comparison than either PE-DE comparison. Correlations of the psychophysical versus ECAP Σ measures were positive; however, not all test/region correlations were significant across the array. Thus, the ECAP separation index is not sensitive enough to predict performance on behavioral tasks of pitch ranking or electrode discrimination for adjacent PEs or corresponding DEs.

Phonological Awareness and Print Knowledge of Preschool Children with Cochlear Implants

PubMed Central

Ambrose, Sophie E.; Fey, Marc E.; Eisenberg, Laurie S.

2012-01-01

Purpose To determine whether preschool-age children with cochlear implants have age-appropriate phonological awareness and print knowledge and to examine the relationships of these skills with related speech and language abilities. Method 24 children with cochlear implants (CIs) and 23 peers with normal hearing (NH), ages 36 to 60 months, participated. Children’s print knowledge, phonological awareness, language, speech production, and speech perception abilities were assessed. Results For phonological awareness, the CI group’s mean score fell within 1 standard deviation of the TOPEL’s normative sample mean but was more than 1 standard deviation below our NH group mean. The CI group’s performance did not differ significantly from that of the NH group for print knowledge. For the CI group, phonological awareness and print knowledge were significantly correlated with language, speech production, and speech perception. Together, these predictor variables accounted for 34% of variance in the CI group’s phonological awareness but no significant variance in their print knowledge. Conclusions Children with CIs have the potential to develop age-appropriate early literacy skills by preschool-age but are likely to lag behind their NH peers in phonological awareness. Intervention programs serving these children should target these skills with instruction and by facilitating speech and language development. PMID:22223887

Susceptibility of linear and nonlinear otoacoustic emission components to low-dose styrene exposure.

PubMed

Tognola, G; Chiaramello, E; Sisto, R; Moleti, A

2015-03-01

To investigate potential susceptibility of active cochlear mechanisms to low-level styrene exposure by comparing TEOAEs in workers and controls. Two advanced analysis techniques were applied to detect sub-clinical changes in linear and nonlinear cochlear mechanisms of OAE generation: the wavelet transform to decompose TEOAEs into time-frequency components and extract signal-to-noise ratio and latency of each component, and the bispectrum to detect and extract nonlinear TEOAE contributions as quadratic frequency couplings (QFCs). Two cohorts of workers were examined: subjects exposed exclusively to styrene (N = 9), and subjects exposed to styrene and noise (N = 6). The control group was perfectly matched by age and sex to the exposed group. Exposed subjects showed significantly lowered SNR in TEOAE components at mid-to-high frequencies (above 1.6 kHz) and a shift of QFC distribution towards lower frequencies than controls. No systematic differences were observed in latency. Low-level styrene exposure may have induced a modification of cochlear functionality as concerns linear and nonlinear OAE generation mechanisms. The lack of change in latency seems to suggest that the OAE components, where generation region and latency are tightly coupled, may not have been affected by styrene and noise exposure levels considered here.

An Objective Estimation of Air-Bone-Gap in Cochlear Implant Recipients with Residual Hearing Using Electrocochleography.

PubMed

Koka, Kanthaiah; Saoji, Aniket A; Attias, Joseph; Litvak, Leonid M

2017-01-01

Although, cochlear implants (CI) traditionally have been used to treat individuals with bilateral profound sensorineural hearing loss, a recent trend is to implant individuals with residual low-frequency hearing. Notably, many of these individuals demonstrate an air-bone gap (ABG) in low-frequency, pure-tone thresholds following implantation. An ABG is the difference between audiometric thresholds measured using air conduction (AC) and bone conduction (BC) stimulation. Although, behavioral AC thresholds are straightforward to assess, BC thresholds can be difficult to measure in individuals with severe-to-profound hearing loss because of vibrotactile responses to high-level, low-frequency stimulation and the potential contribution of hearing in the contralateral ear. Because of these technical barriers to measuring behavioral BC thresholds in implanted patients with residual hearing, it would be helpful to have an objective method for determining ABG. This study evaluated an innovative technique for measuring electrocochleographic (ECochG) responses using the cochlear microphonic (CM) response to assess AC and BC thresholds in implanted patients with residual hearing. Results showed high correlations between CM thresholds and behavioral audiograms for AC and BC conditions, thereby demonstrating the feasibility of using ECochG as an objective tool for quantifying ABG in CI recipients.

Boundary-integral modeling of cochlear hydrodynamics

NASA Astrophysics Data System (ADS)

Pozrikidis, C.

2008-04-01

A two-dimensional model that captures the essential features of the vibration of the basilar membrane of the cochlea is proposed. The flow due to the vibration of the stapes footplate and round window is modeled by a point source and a point sink, and the cochlear pressure is computed simultaneously with the oscillations of the basilar membrane. The mathematical formulation relies on the boundary-integral representation of the potential flow established far from the basilar membrane and cochlea side walls, neglecting the thin Stokes boundary layer lining these surfaces. The boundary-integral approach furnishes integral equations for the membrane vibration amplitude and pressure distribution on the upper or lower side of the membrane. Several approaches are discussed, and numerical solutions in the frequency domain are presented for a rectangular cochlea model using different membrane response functions. The numerical results reproduce and extend the theoretical predictions of previous authors and delineate the effect of physical and geometrical parameters. It is found that the membrane vibration depends weakly on the position of the membrane between the upper and lower wall of the cochlear channel and on the precise location of the oval and round windows. Solutions of the initial-value problem with a single-period sinusoidal impulse reveal the formation of a traveling wave packet that eventually disappears at the helicotrema.

Pitch ranking, electrode discrimination, and physiological spread-of-excitation using Cochlear's dual-electrode mode

PubMed Central

Goehring, Jenny L.; Neff, Donna L.; Baudhuin, Jacquelyn L.; Hughes, Michelle L.

2014-01-01

This study compared pitch ranking, electrode discrimination, and electrically evoked compound action potential (ECAP) spatial excitation patterns for adjacent physical electrodes (PEs) and the corresponding dual electrodes (DEs) for newer-generation Cochlear devices (Cochlear Ltd., Macquarie, New South Wales, Australia). The first goal was to determine whether pitch ranking and electrode discrimination yield similar outcomes for PEs and DEs. The second goal was to determine if the amount of spatial separation among ECAP excitation patterns (separation index, Σ) between adjacent PEs and the PE-DE pairs can predict performance on the psychophysical tasks. Using non-adaptive procedures, 13 subjects completed pitch ranking and electrode discrimination for adjacent PEs and the corresponding PE-DE pairs (DE versus each flanking PE) from the basal, middle, and apical electrode regions. Analysis of d′ scores indicated that pitch-ranking and electrode-discrimination scores were not significantly different, but rather produced similar levels of performance. As expected, accuracy was significantly better for the PE-PE comparison than either PE-DE comparison. Correlations of the psychophysical versus ECAP Σ measures were positive; however, not all test/region correlations were significant across the array. Thus, the ECAP separation index is not sensitive enough to predict performance on behavioral tasks of pitch ranking or electrode discrimination for adjacent PEs or corresponding DEs. PMID:25096106

Central Auditory Maturation and Behavioral Outcome in Children with Auditory Neuropathy Spectrum Disorder who Use Cochlear Implants

PubMed Central

Cardon, Garrett; Sharma, Anu

2013-01-01

Objective We examined cortical auditory development and behavioral outcomes in children with ANSD fitted with cochlear implants (CI). Design Cortical maturation, measured by P1 cortical auditory evoked potential (CAEP) latency, was regressed against scores on the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS). Implantation age was also considered in relation to CAEP findings. Study Sample Cross-sectional and longitudinal samples of 24 and 11 children, respectively, with ANSD fitted with CIs. Result P1 CAEP responses were present in all children after implantation, though previous findings suggest that only 50-75% of ANSD children with hearing aids show CAEP responses. P1 CAEP latency was significantly correlated with participants' IT-MAIS scores. Furthermore, more children implanted before age two years showed normal P1 latencies, while those implanted later mainly showed delayed latencies. Longitudinal analysis revealed that most children showed normal or improved cortical maturation after implantation. Conclusion Cochlear implantation resulted in measureable cortical auditory development for all children with ANSD. Children fitted with CIs under age two years were more likely to show age-appropriate CAEP responses within 6 months after implantation, suggesting a possible sensitive period for cortical auditory development in ANSD. That CAEP responses were correlated with behavioral outcome highlights their clinical decision-making utility. PMID:23819618

Binaural release from masking with single- and multi-electrode stimulation in children with cochlear implantsa)

PubMed Central

Todd, Ann E.; Goupell, Matthew J.; Litovsky, Ruth Y.

2016-01-01

Cochlear implants (CIs) provide children with access to speech information from a young age. Despite bilateral cochlear implantation becoming common, use of spatial cues in free field is smaller than in normal-hearing children. Clinically fit CIs are not synchronized across the ears; thus binaural experiments must utilize research processors that can control binaural cues with precision. Research to date has used single pairs of electrodes, which is insufficient for representing speech. Little is known about how children with bilateral CIs process binaural information with multi-electrode stimulation. Toward the goal of improving binaural unmasking of speech, this study evaluated binaural unmasking with multi- and single-electrode stimulation. Results showed that performance with multi-electrode stimulation was similar to the best performance with single-electrode stimulation. This was similar to the pattern of performance shown by normal-hearing adults when presented an acoustic CI simulation. Diotic and dichotic signal detection thresholds of the children with CIs were similar to those of normal-hearing children listening to a CI simulation. The magnitude of binaural unmasking was not related to whether the children with CIs had good interaural time difference sensitivity. Results support the potential for benefits from binaural hearing and speech unmasking in children with bilateral CIs. PMID:27475132

Binaural release from masking with single- and multi-electrode stimulation in children with cochlear implants.

PubMed

Todd, Ann E; Goupell, Matthew J; Litovsky, Ruth Y

2016-07-01

Cochlear implants (CIs) provide children with access to speech information from a young age. Despite bilateral cochlear implantation becoming common, use of spatial cues in free field is smaller than in normal-hearing children. Clinically fit CIs are not synchronized across the ears; thus binaural experiments must utilize research processors that can control binaural cues with precision. Research to date has used single pairs of electrodes, which is insufficient for representing speech. Little is known about how children with bilateral CIs process binaural information with multi-electrode stimulation. Toward the goal of improving binaural unmasking of speech, this study evaluated binaural unmasking with multi- and single-electrode stimulation. Results showed that performance with multi-electrode stimulation was similar to the best performance with single-electrode stimulation. This was similar to the pattern of performance shown by normal-hearing adults when presented an acoustic CI simulation. Diotic and dichotic signal detection thresholds of the children with CIs were similar to those of normal-hearing children listening to a CI simulation. The magnitude of binaural unmasking was not related to whether the children with CIs had good interaural time difference sensitivity. Results support the potential for benefits from binaural hearing and speech unmasking in children with bilateral CIs.

[Applied anatomy of scala tympani inlet related to cochlear implantation].

PubMed

Zou, Tuanming; Guo, Menghe; Zhang, Hongzheng; Shu, Fan; Xie, Nanping

2012-06-01

To investigate the related parameters of the temporal bone structure for determining the position of implanting electrode into the scala tympani in cochlear implantation surgery through the facial recess and epitympanum approach. In a surgical simulation experiment, 20 human temporal bones were studied and measured to determine the related parameters of the temporal bone structure. The distance 5.91∓0.29 mm between the short process of the incus and the round window niche, 2.11∓0.18 mm between the stapes and the round window niche, 6.70∓0.19 mm between the facial nerve in the perpendicular paragraph and the round window niche, 2.22∓0.21 mm from the pyramidal eminence to the round window, and 2.16∓0.14 mm between the stapes and the round window. The minimal distance between the implanting electrode and the vestibular window was 2.12∓0.19 mm. The distance between the cochleariform process and the round window niche was 3.79∓0.17 mm. The position of the cochlear electrode array insertion into the second cochlear turn was 2.25∓0.13 mm under the stapes. The location of the cochlear electrode array insertion into the second cochlear turn was 2.28∓0.20 mm inferior to the pyramidal eminence. These parameters provide a reference value to determine the different positions of cochlear electrode array insertion into the scale tympani in different patients.

Sequential Bilateral Cochlear Implantation in a Patient with Bilateral Meniere’s Disease

PubMed Central

Holden, Laura K.; Neely, J. Gail; Gotter, Brenda D.; Mispagel, Karen M.; Firszt, Jill B.

2012-01-01

This case study describes a 45 year old female with bilateral, profound sensorineural hearing loss due to Meniere’s disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient’s ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Meniere’s disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work. PMID:22463939

Electrical resistivity measurements in the mammalian cochlea after neural degeneration.

PubMed

Micco, Alan G; Richter, Claus-Peter

2006-08-01

In the present series of experiments, the effect of neural degeneration on the cochlear structure electrical resistivities was evaluated to test if it alters the current flow in the cochlea and if increased current levels are needed to stimulate the impaired cochlea. In cochlear implants, frequency information is encoded in part by stimulating discrete populations of spiral ganglion cells along the cochlea. However, electrical properties of the cochlear structures result in shunting of the current away from the auditory neurons. This consumes energy, makes cochlear implants less efficient, and drastically reduces battery life. Models of the electrically stimulated cochlea serve to make predictions on current paths using modified and improved cochlear implant electrodes. However, one of the model's shortcomings is that most of the values for tissue impedances are not direct measurements. They are derived from bulk impedance measurements, which are fitted to lumped-element models. The four-electrode reflection-coefficient technique was used to measure resistivities in the gerbil cochlea. In vivo and in vitro (the hemicochlea) models were used. Measurements were made in normal and in deafened animals. Cochlear damage was induced by neomycin injection into the animals' middle ears. Neural degeneration was allowed to occur over 2 months before performing the measurements in the deafened animals. The resistivity values in deafened animals were smaller than in the normal-hearing animals, thus altering the current flow within the cochlea. Resistivity changes and subsequent changes in current path should be considered in future designs of cochlear implants.

Cochlear Processes: A Research Report.

ERIC Educational Resources Information Center

Zwislocki, Jozef J.

This paper summarizes recent research on functions of the cochlea of the inner ear. The cochlea is described as the seat of the first step in the auditory sound analysis and transduction of mechanical vibration into electrochemical processes leading to the generation of neural action potentials. The cochlea is also described as a frequent seat of…

The Auditory System of the Minke Whale (Balaenoptera Acutorostrata): A Potential Fatty Sound Reception Pathway in a Mysticete Cetacean

DTIC Science & Technology

2012-09-01

thermoregulate their acoustic fats through increased blood flow to both the melon and peri-mandibular fats (Houser et al., 2004). Cetaceans inhabiting...Fleischer G. 1976. Hearing in Extinct Cetaceans as Determined by Cochlear Structure. Journal of Paleontology 50:133-152. Fraser FC, Purves PE

A novel method for extraction of neural response from single channel cochlear implant auditory evoked potentials.

PubMed

Sinkiewicz, Daniel; Friesen, Lendra; Ghoraani, Behnaz

2017-02-01

Cortical auditory evoked potentials (CAEP) are used to evaluate cochlear implant (CI) patient auditory pathways, but the CI device produces an electrical artifact, which obscures the relevant information in the neural response. Currently there are multiple methods, which attempt to recover the neural response from the contaminated CAEP, but there is no gold standard, which can quantitatively confirm the effectiveness of these methods. To address this crucial shortcoming, we develop a wavelet-based method to quantify the amount of artifact energy in the neural response. In addition, a novel technique for extracting the neural response from single channel CAEPs is proposed. The new method uses matching pursuit (MP) based feature extraction to represent the contaminated CAEP in a feature space, and support vector machines (SVM) to classify the components as normal hearing (NH) or artifact. The NH components are combined to recover the neural response without artifact energy, as verified using the evaluation tool. Although it needs some further evaluation, this approach is a promising method of electrical artifact removal from CAEPs. Copyright © 2016 IPEM. Published by Elsevier Ltd. All rights reserved.

Association of intracellular and synaptic organization in cochlear inner hair cells revealed by 3D electron microscopy.

PubMed

Bullen, Anwen; West, Timothy; Moores, Carolyn; Ashmore, Jonathan; Fleck, Roland A; MacLellan-Gibson, Kirsty; Forge, Andrew

2015-07-15

The ways in which cell architecture is modelled to meet cell function is a poorly understood facet of cell biology. To address this question, we have studied the cytoarchitecture of a cell with highly specialised organisation, the cochlear inner hair cell (IHC), using multiple hierarchies of three-dimensional (3D) electron microscopy analyses. We show that synaptic terminal distribution on the IHC surface correlates with cell shape, and the distribution of a highly organised network of membranes and mitochondria encompassing the infranuclear region of the cell. This network is juxtaposed to a population of small vesicles, which represents a potential new source of neurotransmitter vesicles for replenishment of the synapses. Structural linkages between organelles that underlie this organisation were identified by high-resolution imaging. Taken together, these results describe a cell-encompassing network of membranes and mitochondria present in IHCs that support efficient coding and transmission of auditory signals. Such techniques also have the potential for clarifying functionally specialised cytoarchitecture of other cell types. © 2015. Published by The Company of Biologists Ltd.

Speech Perception for Adults Who Use Hearing Aids in Conjunction with Cochlear Implants in Opposite Ears

ERIC Educational Resources Information Center

Mok, Mansze; Grayden, David; Dowell, Richard C.; Lawrence, David

2006-01-01

This study aimed to (a) investigate the effect of using a hearing aid in conjunction with a cochlear implant in opposite ears on speech perception in quiet and in noise, (b) identify the speech information obtained from a hearing aid that is additive to the information obtained from a cochlear implant, and (c) explore the relationship between…

Sirviendo a los estudiantes sordos que tienen Los implantes cocleares. Hoja de consejos de PEPNet (Serving Deaf Students Who Have Cochlear Implants. PEPNet Tipsheet)

ERIC Educational Resources Information Center

Clark, Catherine

2010-01-01

This version of "Serving Deaf Students Who Have Cochlear Implants. PEPNet Tipsheet," written in Spanish, describes how cochlear implants (CIs) work. CIs are complex electronic devices surgically implanted under the skin behind the ear. These devices utilize electrodes placed in the inner ear (the cochlea) to stimulate the auditory nerve of…