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…

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.

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

Brain stem and inner ear abnormalities in children with auditory neuropathy spectrum disorder and cochlear nerve deficiency.

PubMed

Huang, B Y; Roche, J P; Buchman, C A; Castillo, M

2010-11-01

Cranial abnormalities, including CND, are common in children with ANSD. The purpose of this study was to assess whether CND is associated with brain or inner ear abnormalities in a cohort of children with ANSD. Two neuroradiologists retrospectively reviewed cranial MR imaging examinations in 103 children with ANSD. Brain, cochlear nerve, and temporal bone abnormalities were described and tabulated. Findings were stratified on the basis of the presence and laterality of CND, and differences in the presence of associated inner ear or intracranial abnormalities were assessed by using 2-tailed Fisher exact tests. CND was identified in 33.0% of children and 26.9% of ears with ANSD. Significantly more patients with bilateral CND had intracranial abnormalities than those with unilateral CND (60.0% versus 15.8%; P = .012). Forty percent of patients with bilateral CND, 0% of patients with unilateral CND, and 10.1% of those without CND demonstrated hindbrain malformations. Patients with bilateral CND were more likely to demonstrate hindbrain malformations than patients with normal nerves (P = .01) or unilateral CND (P = .004). Labyrinthine abnormalities were significantly more common in patients with bilateral CND than in those without CND (P ≤ .001). Cochlear anomalies were more common in patients with bilateral versus unilateral CND (P = .01). IAC and cochlear aperture stenosis were more common in those with unilateral and bilateral CND than those without CND (both P < .001). Cochlear and hindbrain abnormalities are significantly more common among patients with ANSD with bilateral CND compared with those with at least 1 intact cochlear nerve.

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

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

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

A Longitudinal Study in Children With Sequential Bilateral Cochlear Implants: Time Course for the Second Implanted Ear and Bilateral Performance.

PubMed

Reeder, Ruth M; Firszt, Jill B; Cadieux, Jamie H; Strube, Michael J

2017-01-01

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 abilities. A prospective longitudinal study included 24 bilaterally implanted children (mean ear surgeries at 5.11 and 14.25 years). Test intervals were every 3-6 months through 24 months postbilateral. Test conditions were each ear and bilaterally for speech recognition and localization. Overall, the rate of progress for the second implanted ear was gradual. Improvements in quiet continued through the second year of bilateral use. Improvements in noise were more modest and leveled off during the second year. On all measures, results from the second ear were poorer than the first. Bilateral scores were better than either ear alone for all measures except sentences in quiet and localization. Older sequentially implanted children with several years between surgeries may obtain speech understanding in the second implanted ear; however, performance may be limited and rate of progress gradual. Continued contralateral ear hearing aid use and reduced time between surgeries may enhance outcomes.

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.

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.

A Longitudinal Study in Adults with Sequential Bilateral Cochlear Implants: Time Course for Individual Ear and Bilateral Performance

ERIC Educational Resources Information Center

Reeder, Ruth M.; Firszt, Jill B.; Holden, Laura K.; Strube, Michael J.

2014-01-01

Purpose: The purpose of this study was to examine the rate of progress in the 2nd implanted ear as it relates to the 1st implanted ear and to bilateral performance in adult sequential cochlear implant recipients. In addition, this study aimed to identify factors that contribute to patient outcomes. Method: The authors performed a prospective…

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.

[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.

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.

Contralateral Masking in Bilateral Cochlear Implant Patients: A Model of Medial Olivocochlear Function Loss

PubMed Central

Aronoff, Justin M.; Padilla, Monica; Fu, Qian-Jie; Landsberger, David M.

2015-01-01

Contralateral masking is the phenomenon where a masker presented to one ear affects the ability to detect a signal in the opposite ear. For normal hearing listeners, contralateral masking results in masking patterns that are both sharper and dramatically smaller in magnitude than ipsilateral masking. The goal of this study was to investigate whether medial olivocochlear (MOC) efferents are needed for the sharpness and relatively small magnitude of the contralateral masking function. To do this, bilateral cochlear implant patients were tested because, by directly stimulating the auditory nerve, cochlear implants circumvent the effects of the MOC efferents. The results indicated that, as with normal hearing listeners, the contralateral masking function was sharper than the ipsilateral masking function. However, although there was a reduction in the magnitude of the contralateral masking function compared to the ipsilateral masking function, it was relatively modest. This is in sharp contrast to the results of normal hearing listeners where the magnitude of the contralateral masking function is greatly reduced. These results suggest that MOC function may not play a large role in the sharpness of the contralateral masking function but may play a considerable role in the magnitude of the contralateral masking function. PMID:25798581

Preservation of vestibular function after scala vestibuli cochlear implantation.

PubMed

Suzuki, Mitsuya; Goto, Takio; Kashio, Akinori; Yasui, Takuya; Sakamoto, Takashi; Ito, Ken; Yamasoba, Tatsuya

2011-10-01

A 58-year-old man, in whom the cochlear implant (CI) had been inserted into the left ear, had right middle-ear cancer. The CI was removed immediately before receiving subtotal removal of right temporal bone. Four months later, the CI was again inserted in his left cochlea. Because of obliterated scala tympani, the 22 active electrodes of the CI were placed into the scala vestibuli. After the surgery, the patient complained that he experienced rotary vertigo and "jumbling of vertical direction" of objects on walking. Using rotation test, we evaluated vestibular function of remaining left ear. Numerous horizontal nystagmus beats were induced during earth-vertical axis rotation, whereas vertical downbeat nystagmus was scarcely induced during off-vertical axis rotation. The horizontal vestibulo-ocular reflex (VOR) was almost normally induced by sinusoidal stimulation at 0.8Hz. These data suggest that the scala vestibuli insertion of CI would be not so invasive against the lateral semicircular canal. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Listening to the ear

NASA Astrophysics Data System (ADS)

Shera, Christopher A.

Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics-termed "tapering symmetry" after its geometric interpretation in simple models-that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical

Listening to the Ear

NASA Astrophysics Data System (ADS)

Shera, Christopher Alan

Otoacoustic emissions demonstrate that the ear creates sound while listening to sound, offering a promising acoustic window on the mechanics of hearing in awake, listening human beings. That window is clouded, however, by an incomplete knowledge of wave reflection and transmission, both forth and back within the cochlea and through the middle ear. This thesis "does windows," addressing wave propagation and scattering on both sides of the middle ear. A summary of highlights follows. Measurements of the cochlear input impedance in cat are used to identify a new symmetry in cochlear mechanics--termed "tapering symmetry" after its geometric interpretation in simple models--that guarantees that the wavelength of the traveling wave changes slowly with position near the stapes. Waves therefore propagate without reflection through the basal turns of the cochlea. Analytic methods for solving the cochlear wave equations using a perturbative scattering series are given and used to demonstrate that, contrary to common belief, conventional cochlear models exhibit negligible internal reflection whether or not they accurately represent the tapering symmetries of the inner ear. Frameworks for the systematic "deconstruction" of eardrum and middle-ear transduction characteristics are developed and applied to the analysis of noninvasive measurements of middle-ear and cochlear mechanics. A simple phenomenological model of inner-ear compressibility that correctly predicts hearing thresholds in patients with missing or disarticulated middle-ear ossicles is developed and used to establish an upper bound on cochlear compressibility several orders of magnitude smaller than that provided by direct measurements. Accurate measurements of stimulus -frequency evoked otoacoustic emissions are performed and used to determine the form and frequency variation of the cochlear traveling-wave ratio noninvasively. Those measurements are inverted to obtain the spatial distribution of mechanical

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.

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.

Cochlear implantation in adults with asymmetric hearing loss.

PubMed

Firszt, Jill B; Holden, Laura K; Reeder, Ruth M; Cowdrey, Lisa; King, Sarah

2012-01-01

Bilateral severe to profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the nonimplanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe to profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful because of limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine whether patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset, and three had pre/perilingual onset of severe to profound hearing loss. All had open-set speech recognition in the better-hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli, and a hearing handicap scale. Participants were evaluated preimplant with hearing aids and postimplant with the implant alone, the hearing aid alone in the better ear, and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at 6 mo postimplant, and pre/perilingual participants were evaluated at 6 and 12 mo postimplant. Data analysis compared the following results: (1) the poorer-hearing ear preimplant (with hearing aid) and postimplant (with cochlear implant); (2) the device(s) used for everyday listening pre- and postimplant; and (3) the hearing aid-alone and bimodal

Cochlear Implantation in Adults with Asymmetric Hearing Loss

PubMed Central

Firszt, Jill B.; Holden, Laura K.; Reeder, Ruth M.; Cowdrey, Lisa; King, Sarah

2012-01-01

Objective Bilateral severe-to-profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the non-implanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe-to-profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful due to limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine if patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Design Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset and three had pre/perilingual onset of severe-to-profound hearing loss. All had open-set speech recognition in the better hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli and a hearing handicap scale. Participants were evaluated pre-implant with hearing aids and post-implant with the implant alone, the hearing aid alone in the better ear and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at six months post-implant and pre/perilingual participants were evaluated at six and 12 months post-implant. Data analysis compared results 1) of the poorer hearing ear pre-implant (with hearing aid) and post-implant (with cochlear implant), 2) with the device(s) used for everyday listening pre- and post-implant and, 3) between the hearing

Estimation of outer-middle ear transmission using DPOAEs and fractional-order modeling of human middle ear

NASA Astrophysics Data System (ADS)

Naghibolhosseini, Maryam

Our ability to hear depends primarily on sound waves traveling through the outer and middle ear toward the inner ear. Hence, the characteristics of the outer and middle ear affect sound transmission to/from the inner ear. The role of the middle and outer ear in sound transmission is particularly important for otoacoustic emissions (OAEs), which are sound signals generated in a healthy cochlea, and recorded by a sensitive microphone placed in the ear canal. OAEs are used to evaluate the health and function of the cochlea; however, they are also affected by outer and middle ear characteristics. To better assess cochlear health using OAEs, it is critical to quantify the impact of the outer and middle ear on sound transmission. The reported research introduces a noninvasive approach to estimate outer-middle ear transmission using distortion product otoacoustic emissions (DPOAEs). In addition, the role of the outer and middle ear on sound transmission was investigated by developing a physical/mathematical model, which employed fractional-order lumped elements to include the viscoelastic characteristics of biological tissues. Impedance estimations from wideband refectance measurements were used for parameter fitting of the model. The model was validated comparing its estimates of the outer-middle ear sound transmission with those given by DPOAEs. The outer-middle ear transmission by the model was defined as the sum of forward and reverse outer-middle ear transmissions. To estimate the reverse transmission by the model, the probe-microphone impedance was calculated through estimating the Thevenin-equivalent circuit of the probe-microphone. The Thevenin-equivalent circuit was calculated using measurements in a number of test cavities. Such modeling enhances our understanding of the roles of different parts of the outer and middle ear and how they work together to determine their function. In addition, the model would be potentially helpful in diagnosing pathologies of

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.

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

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.

Chinchilla middle ear transmission matrix model and middle-ear flexibilitya)

PubMed Central

Ravicz, Michael E.; Rosowski, John J.

2017-01-01

The function of the middle ear (ME) in transforming ME acoustic inputs and outputs (sound pressures and volume velocities) can be described with an acoustic two-port transmission matrix. This description is independent of the load on the ME (cochlea or ear canal) and holds in either direction: forward (from ear canal to cochlea) or reverse (from cochlea to ear canal). A transmission matrix describing ME function in chinchilla, an animal commonly used in auditory research, is presented, computed from measurements of forward ME function: input admittance YTM, ME pressure gain GMEP, ME velocity transfer function HV, and cochlear input admittance YC, in the same set of ears [Ravicz and Rosowski (2012b). J. Acoust. Soc. Am. 132, 2437–2454; (2013a). J. Acoust. Soc. Am. 133, 2208–2223; (2013b). J. Acoust. Soc. Am. 134, 2852–2865]. Unlike previous estimates, these computations require no assumptions about the state of the inner ear, effectiveness of ME manipulations, or measurements of sound transmission in the reverse direction. These element values are generally consistent with physical constraints and the anatomical ME “transformer ratio.” Differences from a previous estimate in chinchilla [Songer and Rosowski (2007). J. Acoust. Soc. Am. 122, 932–942] may be due to a difference in ME flexibility between the two subject groups. PMID:28599566

Chinchilla middle ear transmission matrix model and middle-ear flexibility.

PubMed

Ravicz, Michael E; Rosowski, John J

2017-05-01

The function of the middle ear (ME) in transforming ME acoustic inputs and outputs (sound pressures and volume velocities) can be described with an acoustic two-port transmission matrix. This description is independent of the load on the ME (cochlea or ear canal) and holds in either direction: forward (from ear canal to cochlea) or reverse (from cochlea to ear canal). A transmission matrix describing ME function in chinchilla, an animal commonly used in auditory research, is presented, computed from measurements of forward ME function: input admittance Y TM , ME pressure gain G MEP , ME velocity transfer function H V , and cochlear input admittance Y C , in the same set of ears [Ravicz and Rosowski (2012b). J. Acoust. Soc. Am. 132, 2437-2454; (2013a). J. Acoust. Soc. Am. 133, 2208-2223; (2013b). J. Acoust. Soc. Am. 134, 2852-2865]. Unlike previous estimates, these computations require no assumptions about the state of the inner ear, effectiveness of ME manipulations, or measurements of sound transmission in the reverse direction. These element values are generally consistent with physical constraints and the anatomical ME "transformer ratio." Differences from a previous estimate in chinchilla [Songer and Rosowski (2007). J. Acoust. Soc. Am. 122, 932-942] may be due to a difference in ME flexibility between the two subject groups.

Sound pressure gain produced by the human middle ear.

PubMed

Kurokawa, H; Goode, R L

1995-10-01

The acoustic function of the middle ear is to match sound passing from the low impedance of air to the high impedance of cochlear fluid. Little information is available on the actual middle ear pressure gain in human beings. This article describes experiments on middle ear pressure gain in six fresh human temporal bones. Stapes footplate displacement and phase were measured with a laser Doppler vibrometer before and after removal of the tympanic membrane, malleus, and incus. Acoustic insulation of the round window with clay was performed. Umbo displacement was also measured before tympanic membrane removal to assess baseline tympanic membrane function. The middle ear has its major gain in the lower frequencies, with a peak near 0.9 kHz. The mean gain was 23.0 dB below 1.0 kHz, the resonant frequency of the middle ear; the mean peak gain was 26.6 dB. Above 1.0 kHz, the second pressure gain decreased at a rate of -8.6 dB/octave, with a mean gain of 6.5 dB at 4.0 kHz. Only a small amount of gain was present above 7.0 kHz. Significant individual differences in pressure gain were found between ears that appeared related to variations in tympanic membrane function and not to variations in cochlear impedance.

Modeling the measurements of cochlear microcirculation and hearing function after loud noise.

PubMed

Arpornchayanon, Warangkana; Canis, Martin; Suckfuell, Markus; Ihler, Fritz; Olzowy, Bernhard; Strieth, Sebastian

2011-09-01

Recent findings support the crucial role of microcirculatory disturbance and ischemia for hearing impairment especially after noise-induced hearing loss (NIHL). The aim of this study was to establish an animal model for in vivo analysis of cochlear microcirculation and hearing function after a loud noise to allow precise measurements of both parameters in vivo. Randomized controlled trial. Setting. Animal study. Subjects and Methods. After assessment of normacusis (0 minutes) using evoked auditory brainstem responses (ABRs), noise (106-dB sound pressure level [SPL]) was applied to both ears in 6 guinea pigs for 30 minutes while unexposed animals served as controls. In vivo fluorescence microscopy of the stria vascularis capillaries was performed after surgical exposure of 1 cochlea. ABR measurements were derived from the contralateral ear. After noise exposure, red blood cell velocity was reduced significantly by 24.3% (120 minutes) and further decreased to 44.5% at the end of the observation (210 minutes) in contrast to stable control measurements. Vessel diameters were not affected in both groups. A gradual decrease of segmental blood flow became significant (38.1%) after 150 minutes compared with controls. Hearing thresholds shifted significantly from 20.0 ± 5.5 dB SPL (0 minutes) to 32.5 ± 4.2 dB SPL (60 minutes) only in animals exposed to loud noise. With regard to novel treatments targeting the stria vascularis in NIHL, this standardized model allows us to analyze in detail cochlear microcirculation and hearing function in vivo.

Perception of Suprasegmental Speech Features via Bimodal Stimulation: Cochlear Implant on One Ear and Hearing Aid on the Other

ERIC Educational Resources Information Center

Most, Tova; Harel, Tamar; Shpak, Talma; Luntz, Michal

2011-01-01

Purpose: The purpose of the study was to evaluate the contribution of acoustic hearing to the perception of suprasegmental features by adults who use a cochlear implant (CI) and a hearing aid (HA) in opposite ears. Method: 23 adults participated in this study. Perception of suprasegmental features--intonation, syllable stress, and word…

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.

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…

The Effects of Middle Ear Pressure on Hearing.

DTIC Science & Technology

1984-04-30

Miller, J,M.: Choline salicylate : Effects on cochlear function. Arch. Otolaryngol. 99: 304-308, 1974. *12. Miller, J.M. and Holmquist, J.: An animal model...for study of eustachian tube and middle ear function. Scand. Audiol. 3: 63-68, 1974. 13. McPherson, D. and Miller, J.: Salicylate ototoxicity in the

Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls.

PubMed

Wang, Jenny R; Yuen, Heng W; Shipp, David B; Stewart, Suzanne; Lin, Vincent Y W; Chen, Joseph M; Nedzelski, Julian M

2010-12-01

Evaluate the characteristics and outcomes of patients with autoimmune inner ear disease (AIED) who have undergone cochlear implantation (CI) and compare post-CI performance in AIED to matched controls. Retrospective case control study. Study cohort was comprised of 25 adult implantees (AIED [n = 18], Cogan syndrome [n = 7]). The AIED group was defined by rapidly progressive bilateral sensorineural hearing loss leading to unusable hearing within weeks to months. Patients with Cogan syndrome, the archetypal inner ear autoimmune disease, were also examined and used for within-cohort comparison. Clinical and operative records were reviewed. Post-CI performance was assessed using open-set sentence tests. Age- and sex-matched individuals deafened by other postlingual causes were used as controls. Of 25 patients, 24 had uneventful, full electrode insertions. One AIED patient had partial insertion due to cochlear ossification and did not achieve open-set speech perception post-CI. Mean open-set sentence scores for study patients with uneventful insertions were 92.8%, 97.3%, and 96.4% at 6 months, 1 year, and ≥ 2 years, respectively. Compared to matched controls, patients deafened by autoimmune causes had significantly higher post-CI performance at all postoperative test intervals (P < .05). There was no significant difference in postimplantation performance between Cogan syndrome and AIED patients. To our knowledge this was the largest study of cochlear implantation in AIED and Cogan syndrome patients. In our experience, both groups generally attained high levels of post-CI speech perception and performed above average. Cochlear ossification affecting implantation in Cogan syndrome patients was not observed in our series, contrary to some reports.

Evaluation of cochlear function in normal-hearing young adults exposed to MP3 player noise by analyzing transient evoked otoacoustic emissions and distortion products.

PubMed

Santaolalla Montoya, Francisco; Ibargüen, Agustín Martinez; Vences, Ana Rodriguez; del Rey, Ana Sanchez; Fernandez, Jose Maria Sanchez

2008-10-01

Exposure to recreational noise may cause injuries to the inner ear, and transient evoked (TEOAEs) and distortion product otoacoustic emissions (DPOAEs) may identify these cochlear alterations. The goal of this study was to evaluate TEOAEs and DPOAEs as a method to diagnose early cochlear alterations in young adults exposed to MP3 player noise. We performed a prospective study of the cochlear function in normal-hearing MP3 player users by analyzing TEOAE and DPOAE incidence, amplitude, and spectral content. We gathered a sample of 40 ears from patients between 19 and 29 years old (mean age 24.09 years, SD 3.9 years). We compared the results with those of a control group of 232 ears not exposed to MP3 noise from patients aged 18 to 32 years (mean age 23.35 years, SD 2.7 years). Fifty percent of ears were from females and 50% were from males. Subjects who had used MP3 players for most years and for more hours each week exhibited a reduction in TEOAE and DPOAE incidence and amplitudes and an increase in DPOAE thresholds. TEOAEs showed a statistically significant lower incidence and amplitudes for normal-hearing subjects using MP3 players at frequencies of 2000, 3000, and 4000 Hz. DPOAE incidence was lower at 700, 1000, 1500, and 2000 Hz; the amplitudes were lower at frequencies between 1500 and 6000 Hz; and the thresholds were higher for all frequency bands, statistically significant at frequencies from 1500 to 6000 Hz, p < .05. Cochlear impairment caused by MP3 player noise exposure may be detectable by analyzing TEOAEs and DPOAEs before the impairment becomes clinically apparent.

Suppression tuning of distortion-product otoacoustic emissions: Results from cochlear mechanics simulation

PubMed Central

Liu, Yi-Wen; Neely, Stephen T.

2013-01-01

This paper presents the results of simulating the acoustic suppression of distortion-product otoacoustic emissions (DPOAEs) from a computer model of cochlear mechanics. A tone suppressor was introduced, causing the DPOAE level to decrease, and the decrement was plotted against an increasing suppressor level. Suppression threshold was estimated from the resulting suppression growth functions (SGFs), and suppression tuning curves (STCs) were obtained by plotting the suppression threshold as a function of suppressor frequency. Results show that the slope of SGFs is generally higher for low-frequency suppressors than high-frequency suppressors, resembling those obtained from normal hearing human ears. By comparing responses of normal (100%) vs reduced (50%) outer-hair-cell sensitivities, the model predicts that the tip-to-tail difference of the STCs correlates well with that of intra-cochlear iso-displacement tuning curves. The correlation is poorer, however, between the sharpness of the STCs and that of the intra-cochlear tuning curves. These results agree qualitatively with what was recently reported from normal-hearing and hearing-impaired human subjects, and examination of intra-cochlear model responses can provide the needed insight regarding the interpretation of DPOAE STCs obtained in individual ears. PMID:23363112

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

MEKK4 Signaling Regulates Sensory Cell Development and Function in the Mouse Inner Ear

PubMed Central

Haque, Khujista; Pandey, Atul K.; Zheng, Hong-Wei; Riazuddin, Saima; Sha, Su-Hua

2016-01-01

Mechanosensory hair cells (HCs) residing in the inner ear are critical for hearing and balance. Precise coordination of proliferation, sensory specification, and differentiation during development is essential to ensure the correct patterning of HCs in the cochlear and vestibular epithelium. Recent studies have revealed that FGF20 signaling is vital for proper HC differentiation. However, the mechanisms by which FGF20 signaling promotes HC differentiation remain unknown. Here, we show that mitogen-activated protein 3 kinase 4 (MEKK4) expression is highly regulated during inner ear development and is critical to normal cytoarchitecture and function. Mice homozygous for a kinase-inactive MEKK4 mutation exhibit significant hearing loss. Lack of MEKK4 activity in vivo also leads to a significant reduction in the number of cochlear and vestibular HCs, suggesting that MEKK4 activity is essential for overall development of HCs within the inner ear. Furthermore, we show that loss of FGF20 signaling in vivo inhibits MEKK4 activity, whereas gain of Fgf20 function stimulates MEKK4 expression, suggesting that Fgf20 modulates MEKK4 activity to regulate cellular differentiation. Finally, we demonstrate, for the first time, that MEKK4 acts as a critical node to integrate FGF20-FGFR1 signaling responses to specifically influence HC development and that FGFR1 signaling through activation of MEKK4 is necessary for outer hair cell differentiation. Collectively, this study provides compelling evidence of an essential role for MEKK4 in inner ear morphogenesis and identifies the requirement of MEKK4 expression in regulating the specific response of FGFR1 during HC development and FGF20/FGFR1 signaling activated MEKK4 for normal sensory cell differentiation. SIGNIFICANCE STATEMENT Sensory hair cells (HCs) are the mechanoreceptors within the inner ear responsible for our sense of hearing. HCs are formed before birth, and mammals lack the ability to restore the sensory deficits associated

Effects of Hearing Preservation on Psychophysical Responses to Cochlear Implant Stimulation

PubMed Central

Kang, Stephen Y.; Colesa, Deborah J.; Swiderski, Donald L.; Su, Gina L.; Raphael, Yehoash

2009-01-01

Previous studies have shown that residual acoustic hearing supplements cochlear implant function to improve speech recognition in noise as well as perception of music. The current study had two primary objectives. First, we sought to determine how cochlear implantation and electrical stimulation over a time period of 14 to 21 months influence cochlear structures such as hair cells and spiral ganglion neurons. Second, we sought to investigate whether the structures that provide acoustic hearing also affect the perception of electrical stimulation. We compared psychophysical responses to cochlear implant stimulation in two groups of adult guinea pigs. Group I (11 animals) received a cochlear implant in a previously untreated ear, while group II (ten animals) received a cochlear implant in an ear that had been previously infused with neomycin to destroy hearing. Psychophysical thresholds were measured in response to pulse-train and sinusoidal stimuli. Histological analysis of all group I animals and a subset of group II animals was performed. Nine of the 11 group I animals showed survival of the organ of Corti and spiral ganglion neurons adjacent to the electrode array. All group I animals showed survival of these elements in regions apical to the electrode array. Group II animals that were examined histologically showed complete loss of the organ of Corti in regions adjacent and apical to the electrode array and severe spiral ganglion neuron loss, consistent with previous reports for neomycin-treated ears. Behaviorally, group II animals had significantly lower thresholds than group I animals in response to 100 Hz sinusoidal stimuli. However, group I animals had significantly lower thresholds than group II animals in response to pulse-train stimuli (0.02 ms/phase; 156 to 5,000 pps). Additionally, the two groups showed distinct threshold versus pulse rate functions. We hypothesize that the differences in detection thresholds between groups are caused by the

Perception of suprasegmental speech features via bimodal stimulation: cochlear implant on one ear and hearing aid on the other.

PubMed

Most, Tova; Harel, Tamar; Shpak, Talma; Luntz, Michal

2011-04-01

The purpose of the study was to evaluate the contribution of acoustic hearing to the perception of suprasegmental features by adults who use a cochlear implant (CI) and a hearing aid (HA) in opposite ears. 23 adults participated in this study. Perception of suprasegmental features-intonation, syllable stress, and word emphasis-was assessed. All tests were administered in 2 conditions: CI alone and CI + HA (bimodal). Scores were significantly higher in the bimodal condition in comparison to scores in CI alone for all 3 tests. In both conditions, there was great variability among the individual participants. Significant negative correlations emerged between perception of suprasegmental features and the unaided pure-tone average of the contralateral ear to the CI. This study found a significant bimodal advantage for perception of all suprasegmental features, most probably due to the better low-frequency acoustic hearing that is provided by the HA. Outcomes suggest that in cases of residual hearing in the contralateral ear to the implanted ear, HA use should be encouraged.

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.

On the frequency dependence of the otoacoustic emission latency in hypoacoustic and normal ears

NASA Astrophysics Data System (ADS)

Sisto, R.; Moleti, A.

2002-01-01

Experimental measurements of the otoacoustic emission (OAE) latency of adult subjects have been obtained, as a function of frequency, by means of wavelet time-frequency analysis based on the iterative application of filter banks. The results are in agreement with previous OAE latency measurements by Tognola et al. [Hear. Res. 106, 112-122 (1997)], as regards both the latency values and the frequency dependence, and seem to be incompatible with the steep 1/f law that is predicted by scale-invariant full cochlear models. The latency-frequency relationship has been best fitted to a linear function of the cochlear physical distance, using the Greenwood map, and to an exponential function of the cochlear distance, for comparison with derived band ABR latency measurements. Two sets of ears [94 audiometrically normal and 42 impaired with high-frequency (f>3 kHz) hearing loss] have been separately analyzed. Significantly larger average latencies were found in the impaired ears in the mid-frequency range. Theoretical implications of these findings on the transmission of the traveling wave are discussed.

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.

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).…

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.

Infectious complications of pediatric cochlear implants are highly influenced by otitis media.

PubMed

Vila, Peter M; Ghogomu, Nsangou T; Odom-John, Audrey R; Hullar, Timothy E; Hirose, Keiko

2017-06-01

Determine the incidence of ear infections in cochlear implant patients, evaluate the contribution of otitis media to complications, describe the bacteriology of otitis media in the cochlear implant population, the treatment provided at our center, and the long term outcome. Data collected included age at implantation, history of otitis media or ear tubes, etiology of hearing loss, inner ear anatomy, postoperative infections, time to infection, route of antibiotic administration, and interventions for infections. Categories of infection were acute otitis media, otitis media with effusion, tube otorrhea, meningitis, scalp cellulitis, and infection at the implant site. Middle ear infections were diagnosed in 37% of implanted ears. Extension of middle ear infections into the implant site occurred in 2.8% of all implants (n = 16). Of the 16 infected devices, 10 were successfully treated with antibiotic therapy and did not require explantation. The retained implant group and explanted group both included some middle ear microbes such as Haemophilus influenzae and Streptococcus pneumoniae, as well as skin flora such as Staphylococcus aureus. Otitis media in pediatric cochlear implant patients is a common event and usually does not lead to complications of the cochlear implant. However, when the ear infection spreads to the scalp and the implant site, it is still possible to eliminate the infection using antibiotic therapy, particularly when treatment is directed to the specific organism that is recovered from the infected space and the duration and route of antibiotic treatment is carefully considered. Copyright © 2017 Elsevier B.V. All rights reserved.

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

A Fully-Implantable Cochlear Implant SoC with Piezoelectric Middle-Ear Sensor and Arbitrary Waveform Neural Stimulation.

PubMed

Yip, Marcus; Jin, Rui; Nakajima, Hideko Heidi; Stankovic, Konstantina M; Chandrakasan, Anantha P

2015-01-01

A system-on-chip for an invisible, fully-implantable cochlear implant is presented. Implantable acoustic sensing is achieved by interfacing the SoC to a piezoelectric sensor that detects the sound-induced motion of the middle ear. Measurements from human cadaveric ears demonstrate that the sensor can detect sounds between 40 and 90 dB SPL over the speech bandwidth. A highly-reconfigurable digital sound processor enables system power scalability by reconfiguring the number of channels, and provides programmable features to enable a patient-specific fit. A mixed-signal arbitrary waveform neural stimulator enables energy-optimal stimulation pulses to be delivered to the auditory nerve. The energy-optimal waveform is validated with in-vivo measurements from four human subjects which show a 15% to 35% energy saving over the conventional rectangular waveform. Prototyped in a 0.18 μ m high-voltage CMOS technology, the SoC in 8-channel mode consumes 572 μ W of power including stimulation. The SoC integrates implantable acoustic sensing, sound processing, and neural stimulation on one chip to minimize the implant size, and proof-of-concept is demonstrated with measurements from a human cadaver ear.

Human middle-ear model with compound eardrum and airway branching in mastoid air cells

PubMed Central

Keefe, Douglas H.

2015-01-01

An acoustical/mechanical model of normal adult human middle-ear function is described for forward and reverse transmission. The eardrum model included one component bound along the manubrium and another bound by the tympanic cleft. Eardrum components were coupled by a time-delayed impedance. The acoustics of the middle-ear cleft was represented by an acoustical transmission-line model for the tympanic cavity, aditus, antrum, and mastoid air cell system with variable amounts of excess viscothermal loss. Model parameters were fitted to published measurements of energy reflectance (0.25–13 kHz), equivalent input impedance at the eardrum (0.25–11 kHz), temporal-bone pressure in scala vestibuli and scala tympani (0.1–11 kHz), and reverse middle-ear impedance (0.25–8 kHz). Inner-ear fluid motion included cochlear and physiological third-window pathways. The two-component eardrum with time delay helped fit intracochlear pressure responses. A multi-modal representation of the eardrum and high-frequency modeling of the middle-ear cleft helped fit ear-canal responses. Input reactance at the eardrum was small at high frequencies due to multiple modal resonances. The model predicted the middle-ear efficiency between ear canal and cochlea, and the cochlear pressures at threshold. PMID:25994701

Human middle-ear model with compound eardrum and airway branching in mastoid air cells.

PubMed

Keefe, Douglas H

2015-05-01

An acoustical/mechanical model of normal adult human middle-ear function is described for forward and reverse transmission. The eardrum model included one component bound along the manubrium and another bound by the tympanic cleft. Eardrum components were coupled by a time-delayed impedance. The acoustics of the middle-ear cleft was represented by an acoustical transmission-line model for the tympanic cavity, aditus, antrum, and mastoid air cell system with variable amounts of excess viscothermal loss. Model parameters were fitted to published measurements of energy reflectance (0.25-13 kHz), equivalent input impedance at the eardrum (0.25-11 kHz), temporal-bone pressure in scala vestibuli and scala tympani (0.1-11 kHz), and reverse middle-ear impedance (0.25-8 kHz). Inner-ear fluid motion included cochlear and physiological third-window pathways. The two-component eardrum with time delay helped fit intracochlear pressure responses. A multi-modal representation of the eardrum and high-frequency modeling of the middle-ear cleft helped fit ear-canal responses. Input reactance at the eardrum was small at high frequencies due to multiple modal resonances. The model predicted the middle-ear efficiency between ear canal and cochlea, and the cochlear pressures at threshold.

Inner-ear sound pressures near the base of the cochlea in chinchilla: Further investigation

PubMed Central

Ravicz, Michael E.; Rosowski, John J.

2013-01-01

The middle-ear pressure gain GMEP, the ratio of sound pressure in the cochlear vestibule PV to sound pressure at the tympanic membrane PTM, is a descriptor of middle-ear sound transfer and the cochlear input for a given stimulus in the ear canal. GMEP and the cochlear partition differential pressure near the cochlear base ΔPCP, which determines the stimulus for cochlear partition motion and has been linked to hearing ability, were computed from simultaneous measurements of PV, PTM, and the sound pressure in scala tympani near the round window PST in chinchilla. GMEP magnitude was approximately 30 dB between 0.1 and 10 kHz and decreased sharply above 20 kHz, which is not consistent with an ideal transformer or a lossless transmission line. The GMEP phase was consistent with a roughly 50-μs delay between PV and PTM. GMEP was little affected by the inner-ear modifications necessary to measure PST. GMEP is a good predictor of ΔPCP at low and moderate frequencies where PV ⪢ PST but overestimates ΔPCP above a few kilohertz where PV ≈ PST. The ratio of PST to PV provides insight into the distribution of sound pressure within the cochlear scalae. PMID:23556590

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.

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.

Cochlear Implants (For Parents)

MedlinePlus

... nerve, and send it to the brain. The cochlear implant package is made up of: a receiver-stimulator that contains all of the electronic circuits that control the flow of electrical pulses into the ear an antenna ...

Central masking with bilateral cochlear implants

PubMed Central

Lin, Payton; Lu, Thomas; Zeng, Fan-Gang

2013-01-01

Across bilateral cochlear implants, contralateral threshold shift has been investigated as a function of electrode difference between the masking and probe electrodes. For contralateral electric masking, maximum threshold elevations occurred when the position of the masker and probe electrode was approximately place-matched across ears. The amount of masking diminished with increasing masker-probe electrode separation. Place-dependent masking occurred in both sequentially implanted ears, and was not affected by the masker intensity or the time delay from the masker onset. When compared to previous contralateral masking results in normal hearing, the similarities between place-dependent central masking patterns suggest comparable mechanisms of overlapping excitation in the central auditory nervous system. PMID:23363113

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.

Perilymph pharmacokinetics of marker applied through a cochlear implant in guinea pigs

PubMed Central

Hartsock, Jared; Gill, Ruth; Smyth, Daniel; Kirk, Jonathon; Verhoeven, Kristien

2017-01-01

Patients undergoing cochlear implantation could benefit from a simultaneous application of drugs into the ear, helping preserve residual low-frequency hearing and afferent nerve fiber populations. One way to apply drugs is to incorporate a cannula into the implant, through which drug solution is driven. For such an approach, perilymph concentrations achieved and the distribution in the ear over time have not previously been documented. We used FITC-labeled dextran as a marker, delivering it into perilymph of guinea pigs at 10 or 100 nL/min though a cannula incorporated into a cochlear implant with the outlet in the mid basal turn. After injections of varying duration (2 hours, 1 day or 7 days) perilymph was collected from the cochlear apex using a sequential sampling technique, allowing dextran levels and gradients along scala tympani to be quantified. Data were interpreted quantitatively using computer simulations of the experiments. For injections of 2 hours duration, dextran levels were critically influenced by the presence or absence of fluid leakage at the cochleostomy site. When the cochleostomy was fluid-tight, substantially higher perilymph levels were achieved at the injection site, with concentration declining along scala tympani towards the apex. Contrary to expectations, large dextran gradients along scala tympani persisted after 24 hours of sustained injection and were still present in some animals after 7 days injection. Functional changes associated with implantation and dextran delivery, and the histological state of the implant and cannula were also documented. The persistent longitudinal gradients of dextan along the ear were not readily explained by computer simulations of the experiments based on prior pharmacokinetic data. One explanation is that inner ear pharmacokinetics are altered in the period after cochlear implantation, possibly by a permeabilization of the blood-labyrinth barrier as part of the immune response to the implant. PMID

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

3D finite element model of the chinchilla ear for characterizing middle ear functions

PubMed Central

Wang, Xuelin; Gan, Rong Z.

2016-01-01

Chinchilla is a commonly used animal model for research of sound transmission through the ear. Experimental measurements of the middle ear transfer function in chinchillas have shown that the middle ear cavity greatly affects the tympanic membrane (TM) and stapes footplate (FP) displacements. However, there is no finite element (FE) model of the chinchilla ear available in the literature to characterize the middle ear functions with the anatomical features of the chinchilla ear. This paper reports a recently completed 3D FE model of the chinchilla ear based on X-ray micro-computed tomography images of a chinchilla bulla. The model consisted of the ear canal, TM, middle ear ossicles and suspensory ligaments, and the middle ear cavity. Two boundary conditions of the middle ear cavity wall were simulated in the model as the rigid structure and the partially flexible surface, and the acoustic-mechanical coupled analysis was conducted with these two conditions to characterize the middle ear function. The model results were compared with experimental measurements reported in the literature including the TM and FP displacements and the middle ear input admittance in chinchilla ear. An application of this model was presented to identify the acoustic role of the middle ear septa - a unique feature of chinchilla middle ear cavity. This study provides the first 3D FE model of the chinchilla ear for characterizing the middle ear functions through the acoustic-mechanical coupled FE analysis. PMID:26785845

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.

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

Cochlear transducer operating point adaptation.

PubMed

Zou, Yuan; Zheng, Jiefu; Ren, Tianying; Nuttall, Alfred

2006-04-01

The operating point (OP) of outer hair cell (OHC) mechanotransduction can be defined as any shift away from the center position on the transduction function. It is a dc offset that can be described by percentage of the maximum transduction current or as an equivalent dc pressure in the ear canal. The change of OP can be determined from the changes of the second and third harmonics of the cochlear microphonic (CM) following a calibration of its initial value. We found that the initial OP was dependent on sound level and cochlear sensitivity. From CM generated by a lower sound level at 74 dB SPL to avoid saturation and suppression of basal turn cochlear amplification, the OHC OP was at constant 57% of the maximum transduction current (an ear canal pressure of -0.1 Pa). To perturb the OP, a constant force was applied to the bony shell of the cochlea at the 18 kHz best frequency location using a blunt probe. The force applied over the scala tympani induced an OP change as if the organ of Corti moved toward the scala vestibuli (SV) direction. During an application of the constant force, the second harmonic of the CM partially recovered toward the initial level, which could be described by two time constants. Removing the force induced recovery of the second harmonic to its normal level described by a single time constant. The force applied over the SV caused an opposite result. These data indicate an active mechanism for OHC transduction OP.

Concise Review: Inner Ear Stem Cells—An Oxymoron, But Why?

PubMed Central

Ronaghi, Mohammad; Nasr, Marjan; Heller, Stefan

2012-01-01

Hearing loss, caused by irreversible loss of cochlear sensory hair cells, affects millions of patients worldwide. In this concise review, we examine the conundrum of inner ear stem cells, which obviously are present in the inner ear sensory epithelia of nonmammalian vertebrates, giving these ears the ability to functionally recover even from repetitive ototoxic insults. Despite the inability of the mammalian inner ear to regenerate lost hair cells, there is evidence for cells with regenerative capacity because stem cells can be isolated from vestibular sensory epithelia and from the neonatal cochlea. Challenges and recent progress toward identification of the intrinsic and extrinsic signaling pathways that could be used to re-establish stemness in the mammalian organ of Corti are discussed. PMID:22102534

Concise review: Inner ear stem cells--an oxymoron, but why?

PubMed

Ronaghi, Mohammad; Nasr, Marjan; Heller, Stefan

2012-01-01

Hearing loss, caused by irreversible loss of cochlear sensory hair cells, affects millions of patients worldwide. In this concise review, we examine the conundrum of inner ear stem cells, which obviously are present in the inner ear sensory epithelia of nonmammalian vertebrates, giving these ears the ability to functionally recover even from repetitive ototoxic insults. Despite the inability of the mammalian inner ear to regenerate lost hair cells, there is evidence for cells with regenerative capacity because stem cells can be isolated from vestibular sensory epithelia and from the neonatal cochlea. Challenges and recent progress toward identification of the intrinsic and extrinsic signaling pathways that could be used to re-establish stemness in the mammalian organ of Corti are discussed. Copyright © 2011 AlphaMed Press.

PCB exposure and cochlear function at age 6 years.

PubMed

Palkovičová Murínová, Ľubica; Moleti, Arturo; Sisto, Renata; Wimmerová, Soňa; Jusko, Todd A; Tihányi, Juraj; Jurečková, Dana; Kováč, Ján; Koštiaková, Vladimíra; Drobná, Beata; Trnovec, Tomáš

2016-11-01

Epidemiological studies have documented adverse associations between exposure to polychlorinated biphenyls (PCBs) and otological outcomes. Previously, we documented decreased distortion product otoacoustic emission (DPOAE) levels in children exposed to PCBs, up to the age of 45 months, amongst a cohort of children in eastern Slovakia. The objective of the present study is to evaluate cochlear dysfunction at 72 months of age in 214 children from this same cohort and to compare the otoacoustic test sensitivity to that of pure tone audiometry (PTA). The association between DPOAE, PTA, and PCBs was estimated by means of multivariate ANOVA (MANOVA) and linear regression models. ROC curves were computed to estimate the DPOAE-test power in children. The DPOAE level at 72 months was related to PCB-153 serum levels. The DPOAE Input/Output function test at mid-frequency (2kHz) has shown instead nonmonotonic dependence on PCB exposure, for the left ears of children, over the whole growth curve. No significant association was found between PTA hearing levels and PCB-153 concentration. High diagnostic power of the DPOAE-test was found in children, similar to that found by the same authors in adults. In conclusions the DPOAE-PCB correlation obtained at 72 months is similar to that at 45 months suggesting a permanent and stable ototoxic effect of the PCB exposure. The lack of statistical significance of the PCB-PTA correlation suggests that DPOAEs are sensitive biomarkers of cochlear damage. Copyright © 2016 Elsevier Inc. All rights reserved.

Assessment of auditory skills in 140 cochlear implant children using the EARS protocol.

PubMed

Sainz, Manuel; Skarzynski, Henryk; Allum, John H J; Helms, Jan; Rivas, Adriana; Martin, Jane; Zorowka, Patrick Georg; Phillips, Lucy; Delauney, Joseph; Brockmeyer, Steffi Johanna; Kompis, Martin; Korolewa, Inna; Albegger, Klaus; Zwirner, Petra; Van De Heyning, Paul; D'Haese, Patrick

2003-01-01

Auditory performance of cochlear implant (CI) children was assessed with the Listening Progress Profile (LiP) and the Monosyllabic-Trochee-Polysyllabic-Word Test (MTP) following the EARS protocol. Additionally, the 'initial drop' phenomenon, a recently reported decrease of auditory performance occurring immediately after first fitting, was investigated. Patients were 140 prelingually deafened children from various clinics and centers worldwide implanted with a MEDEL COMBI 40/40+. Analysis of LiP data showed a significant increase after 1 month of CI use compared to preoperative scores (p < 0.01). No initial decrease was observed with this test. Analysis of MTP data revealed a significant improvement of word recognition after 6 months (p < 0.01), with a significant temporary decrease after initial fitting (p < 0.01). With both tests, children's auditory skills improved up to 2 years. Amount of improvement was negatively correlated with age at implantation. Copyright 2003 S. Karger AG, Basel

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.

Compensating for ear-canal acoustics when measuring otoacoustic emissions

PubMed Central

Charaziak, Karolina K.; Shera, Christopher A.

2017-01-01

Otoacoustic emissions (OAEs) provide an acoustic fingerprint of the inner ear, and changes in this fingerprint may indicate changes in cochlear function arising from efferent modulation, aging, noise trauma, and/or exposure to harmful agents. However, the reproducibility and diagnostic power of OAE measurements is compromised by the variable acoustics of the ear canal, in particular, by multiple reflections and the emergence of standing waves at relevant frequencies. Even when stimulus levels are controlled using methods that circumvent standing-wave problems (e.g., forward-pressure-level calibration), distortion-product otoacoustic emission (DPOAE) levels vary with probe location by 10–15 dB near half-wave resonant frequencies. The method presented here estimates the initial outgoing OAE pressure wave at the eardrum from measurements of the conventional OAE, allowing one to separate the emitted OAE from the many reflections trapped in the ear canal. The emitted pressure level (EPL) represents the OAE level that would be recorded were the ear canal replaced by an infinite tube with no reflections. When DPOAEs are expressed using EPL, their variation with probe location decreases to the test–retest repeatability of measurements obtained at similar probe positions. EPL provides a powerful way to reduce the variability of OAE measurements and improve their ability to detect cochlear changes. PMID:28147590

Pharmacokinetics of Drug Entry into Cochlear Fluids

ERIC Educational Resources Information Center

Salt, Alec N.

2005-01-01

The inner ear is exposed to aminoglycosides or other drugs either intentionally or as a side effect of clinical treatments directed at other regions of the body. An understanding of the effects of drugs on the inner ear requires knowledge of the pharmacokinetics of the drug once it reaches the cochlear fluids, specifically how much of it reaches…

Study of tonotopic brain changes with functional MRI and FDG-PET in a patient with unilateral objective cochlear tinnitus.

PubMed

Guinchard, A-C; Ghazaleh, Naghmeh; Saenz, M; Fornari, E; Prior, J O; Maeder, P; Adib, S; Maire, R

2016-11-01

We studied possible brain changes with functional MRI (fMRI) and fluorodeoxyglucose positron emission tomography (FDG-PET) in a patient with a rare, high-intensity "objective tinnitus" (high-level SOAEs) in the left ear of 10 years duration, with no associated hearing loss. This is the first case of objective cochlear tinnitus to be investigated with functional neuroimaging. The objective cochlear tinnitus was measured by Spontaneous Otoacoustic Emissions (SOAE) equipment (frequency 9689 Hz, intensity 57 dB SPL) and is clearly audible to anyone standing near the patient. Functional modifications in primary auditory areas and other brain regions were evaluated using 3T and 7T fMRI and FDG-PET. In the fMRI evaluations, a saturation of the auditory cortex at the tinnitus frequency was observed, but the global cortical tonotopic organization remained intact when compared to the results of fMRI of healthy subjects. The FDG-PET showed no evidence of an increase or decrease of activity in the auditory cortices or in the limbic system as compared to normal subjects. In this patient with high-intensity objective cochlear tinnitus, fMRI and FDG-PET showed no significant brain reorganization in auditory areas and/or in the limbic system, as reported in the literature in patients with chronic subjective tinnitus. Copyright © 2016 Elsevier B.V. All rights reserved.

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

A new computed tomography method to identify meningitis-related cochlear ossification and fibrosis before cochlear implantation.

PubMed

Ichikawa, Kazunori; Kashio, Akinori; Mori, Harushi; Ochi, Atushi; Karino, Shotaro; Sakamoto, Takashi; Kakigi, Akinobu; Yamasoba, Tatsuya

2014-04-01

To develop a new method to determine the presence of intracochlear ossification and/or fibrosis in cochlear implantation candidates with bilateral profound deafness following meningitis. Diagnostic test assessment. A university hospital. This study involved 15 ears from 13 patients with profound deafness following meningitis who underwent cochlear implantation. These ears showed normal structures, soft tissue, partial bony occlusion, and complete bony occlusion in 4, 3, 2, and 6 ears, respectively. We measured radiodensity in Hounsfield units (HU) using 0.5-mm-thick axial high-resolution computed tomography image slices at 3 different levels in the basal turn, the fenestration, and inferior and ascending segment sites, located along the electrode-insertion path. Pixel-level analysis on the DICOM viewer yielded actual computed tomography values of intracochlear soft tissues by eliminating the partial volume effect. The values were compared with the intraoperative findings. Values for ossification (n = 12) ranged from +547 HU to +1137 HU; for fibrosis (n = 11), from +154 HU to +574 HU; and for fluid (n = 22), from -49 HU to +255 HU. From these values, we developed 2 presets of window width (WW) and window level (WL): (1) WW: 1800, WL: 1100 (200 HU to 2000 HU) and (2) WW: 1500, WL: 1250 (500 HU to 2000 HU). The results using these 2 presets corresponded well to the intraoperative findings. Our new method is easy and feasible for preoperative determination of the presence of cochlear ossification and/or fibrosis that develops following meningitis.

Rhesus Cochlear and Vestibular Functions Are Preserved After Inner Ear Injection of Saline Volume Sufficient for Gene Therapy Delivery.

PubMed

Dai, Chenkai; Lehar, Mohamed; Sun, Daniel Q; Rvt, Lani Swarthout; Carey, John P; MacLachlan, Tim; Brough, Doug; Staecker, Hinrich; Della Santina, Alexandra M; Hullar, Timothy E; Della Santina, Charles C

2017-08-01

Sensorineural losses of hearing and vestibular sensation due to hair cell dysfunction are among the most common disabilities. Recent preclinical research demonstrates that treatment of the inner ear with a variety of compounds, including gene therapy agents, may elicit regeneration and/or repair of hair cells in animals exposed to ototoxic medications or other insults to the inner ear. Delivery of gene therapy may also offer a means for treatment of hereditary hearing loss. However, injection of a fluid volume sufficient to deliver an adequate dose of a pharmacologic agent could, in theory, cause inner ear trauma that compromises functional outcome. The primary goal of the present study was to assess that risk in rhesus monkeys, which closely approximates humans with regard to middle and inner ear anatomy. Secondary goals were to identify the best delivery route into the primate ear from among two common surgical approaches (i.e., via an oval window stapedotomy and via the round window) and to determine the relative volumes of rhesus, rodent, and human labyrinths for extrapolation of results to other species. We measured hearing and vestibular functions before and 2, 4, and 8 weeks after unilateral injection of phosphate-buffered saline vehicle (PBSV) into the perilymphatic space of normal rhesus monkeys at volumes sufficient to deliver an atoh1 gene therapy vector. To isolate effects of injection, PBSV without vector was used. Assays included behavioral observation, auditory brainstem responses, distortion product otoacoustic emissions, and scleral coil measurement of vestibulo-ocular reflexes during whole-body rotation in darkness. Three groups (N = 3 each) were studied. Group A received a 10 μL transmastoid/trans-stapes injection via a laser stapedotomy. Group B received a 10 μL transmastoid/trans-round window injection. Group C received a 30 μL transmastoid/trans-round window injection. We also measured inner ear fluid space volume via 3D

Cochlear implant – state of the art

PubMed Central

Lenarz, Thomas

2018-01-01

Cochlear implants are the treatment of choice for auditory rehabilitation of patients with sensory deafness. They restore the missing function of inner hair cells by transforming the acoustic signal into electrical stimuli for activation of auditory nerve fibers. Due to the very fast technology development, cochlear implants provide open-set speech understanding in the majority of patients including the use of the telephone. Children can achieve a near to normal speech and language development provided their deafness is detected early after onset and implantation is performed quickly thereafter. The diagnostic procedure as well as the surgical technique have been standardized and can be adapted to the individual anatomical and physiological needs both in children and adults. Special cases such as cochlear obliteration might require special measures and re-implantation, which can be done in most cases in a straight forward way. Technology upgrades count for better performance. Future developments will focus on better electrode-nerve interfaces by improving electrode technology. An increased number of electrical contacts as well as the biological treatment with regeneration of the dendrites growing onto the electrode will increase the number of electrical channels. This will give room for improved speech coding strategies in order to create the bionic ear, i.e. to restore the process of natural hearing by means of technology. The robot-assisted surgery will allow for high precision surgery and reliable hearing preservation. Biological therapies will support the bionic ear. Methods are bio-hybrid electrodes, which are coded by stem cells transplanted into the inner ear to enhance auto-production of neurotrophins. Local drug delivery will focus on suppression of trauma reaction and local regeneration. Gene therapy by nanoparticles will hopefully lead to the preservation of residual hearing in patients being affected by genetic hearing loss. Overall the cochlear implant

[Magnetic resonance imaging study and cochlear implantation in post-meningitic deaf patients].

PubMed

Liu, Xiuli; Yao, Yiwen; He, Guili; Zhai, Lijie

2004-07-01

To investigate the clinical application of magnetic resonance imaging (MRI) in post-meningitic patients and its impact on surgical decision. The pre-operative MRI data and auditory brainstem response (ABR) examination of five post-meningitic patients were studied. They were implanted with cochleas. The interval between the onset of bacterial meningitis and the hearing loss was (15.8 +/- 15.0)d and it was longer in children than adults. Five ears showed membranous cochlear labyrinth abnormality; 3 ears had vestibule vestibule abnormality; 8 ears demonstrated semicircular canal abnormality on MRI examinations in totally 10 ears. The mean hearing threshold of 10 ears was (102.0 +/- 7.1)dB HL,that of the operated ears was (98.0 +/- 5.7)dB HL and that of the un-operated ears was (106.0 +/- 6.5)dB HL. It was (15.8 +/- 15.0)d from the bacterial meningitis onset to hearing loss. The interval is longer in children than adults. There were 3 ears that electrodes could not be inserted completely. The bacterial meningitis may cause the abnormalities of inner ears and the MRI before surgery is essential for the pre-operative planning of cochlear implant.

Dichotic listening and otoacoustic emissions: shared variance between cochlear function and dichotic listening performance in adults with normal hearing.

PubMed

Markevych, Vladlena; Asbjørnsen, Arve E; Lind, Ola; Plante, Elena; Cone, Barbara

2011-07-01

The present study investigated a possible connection between speech processing and cochlear function. Twenty-two subjects with age range from 18 to 39, balanced for gender with normal hearing and without any known neurological condition, were tested with the dichotic listening (DL) test, in which listeners were asked to identify CV-syllables in a nonforced, and also attention-right, and attention-left condition. Transient evoked otoacoustic emissions (TEOAEs) were recorded for both ears, with and without the presentation of contralateral broadband noise. The main finding was a strong negative correlation between language laterality as measured with the dichotic listening task and of the TEOAE responses. The findings support a hypothesis of shared variance between central and peripheral auditory lateralities, and contribute to the attentional theory of auditory lateralization. The results have implications for the understanding of the cortico-fugal efferent control of cochlear activity. 2011 Elsevier Inc. All rights reserved.

Restudy of malformations of the internal auditory meatus, cochlear nerve canal and cochlear nerve.

PubMed

Li, Youjin; Yang, Jun; Liu, Jinfen; Wu, Hao

2015-07-01

The present study aims to restudy the correlation between the internal auditory meatus (IAM), the cochlear nerve canal (CNC), the cochlear nerve (CN) and inner ear malformations. In this retrospective study design, the abnormal diameter of the IAM, CNC and CN in patients with any kind of inner ear malformations was evaluated using multi-slice spiral computed tomography (MSCT) (37 patients) and magnetic resonance imaging (MRI) (18 patients). Of 37 MSCT-diagnosed patients, 2 had IAM atresia, 11 IAM stenosis, 22 enlarged IAM, and 2 normal IAM with an abnormal CN. MRI diagnoses of 18 patients revealed 8 cases of aplastic CN, 6 hypoplastic CN, and 4 normal CN. CNC stenosis was associated with CN hypoplasia (P < 0.001). Patients with absent or stenotic IAM had less CN development than those with normal or enlarged IAM (P = 0.001). We propose a modification of the existing classification systems with a view to distinguishing malformations of the IAM, CNC and CN.

Bacterial invasion of the inner ear in association with pneumococcal meningitis.

PubMed

Møller, Martin Nue; Brandt, Christian; Østergaard, Christian; Caye-Thomasen, Per

2014-06-01

To examine the pathways of bacterial invasion and subsequent spreading in the inner ear during pneumococcal meningitis. A well-established adult rat model of Streptococcus pneumoniae meningitis was used. Thirty rats were inoculated intrathecally with S. pneumoniae serotype 1, 3 or 9 V and received no additional treatment. The rats were sacrificed when reaching terminal illness or on Day 7 and then prepared for serial sectioning and PAS-Alcian blue staining for light microscopy. During the first few days after inoculation, bacteria invade the inner ear through the cochlear aqueduct, into the scala tympani of the cochlea (perilymphatic space). From here, bacteria spreads apically toward the helicotrema and subsequently basally through the scala vestibuli, toward the vestibule and the vestibular system. When the bacteria after 5 to 6 days had reached scala vestibuli of the basal turn of the cochlea, hematogenous spreading occurred to the spiral ligament and into the cochlear endolymph, subsequently to the vestibular endolymph. We found no evidence of alternative routes for bacterial invasion in the inner ear. Several internal barriers to bacterial spreading were found within the inner ear. Bacterial elimination was evidenced by engulfment by macrophages within the inner ear. From the meninges, pneumococci invade the inner ear through the cochlear aqueduct during the first days of infection, whereas hematogenous invasion via the spiral ligament capillary bed occur at later stages. Although internal barriers exist within the inner ear, the spreading of bacteria occurs via the natural pathways of the fluid compartments. Bacterial elimination occurs by local macrophage engulfment.

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

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

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.

Bilateral cochlear implants in infants: a new approach--Nucleus Hybrid S12 project.

PubMed

Gantz, Bruce J; Dunn, Camille C; Walker, Elizabeth A; Kenworthy, Maura; Van Voorst, Tanya; Tomblin, Bruce; Turner, Chris

2010-10-01

The purpose of this feasibility study was to evaluate whether the use of a shorter-length cochlear implant (10 mm) on one ear and a standard electrode (24 mm) on the contralateral ear is a viable bilateral option for children with profound bilateral sensorineural hearing loss. A secondary purpose of this study was to determine whether the ear with the shorter-length electrode performs similarly to the standard-length electrode. Our goal was to provide an option of electrical stimulation that theoretically might preserve the structures of the scala media and organ of Corti. The study is being conducted as a repeated-measure, single-subject experiment. University of Iowa-Department of Otolaryngology. Eight pediatric patients with profound bilateral sensorineural hearing loss between the ages of 12 and 24 months. Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in the contralateral ear. The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) parent questionnaire, Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children's Vowel tests will be used to evaluate speech perception and the Minnesota Child Development Inventory and Preschool Language Scales 3 test will be used to evaluate language growth. Preliminary results for 8 children have been collected before and after the operation using the IT-MAIS. All 3 children showed incremental improvements in their IT-MAIS scores overtime. Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children's Vowel word perception results indicated no difference between the individual ears for the 2 children tested. Performance compared with age-matched children implanted with standard bilateral cochlear implants showed similar results to the children implanted with Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in contralateral ears. The use of a shorter-length cochlear implant on one ear and a standard-length electrode on the

A Comparative Study of Drug Delivery Methods Targeted to the Mouse Inner Ear: Bullostomy Versus Transtympanic Injection

PubMed Central

Cediel, Rafael; Celaya, Adelaida M.; Lassaletta, Luis; Varela-Nieto, Isabel; Contreras, Julio

2017-01-01

We present two minimally invasive microsurgical techniques in rodents for specific drug delivery into the middle ear so that it may reach the inner ear. The first procedure consists of perforation of the tympanic bulla, termed bullostomy; the second one is a transtympanic injection. Both emulate human clinical intratympanic procedures. Chitosan-glycerophosphate (CGP) and Ringer´s Lactate buffer (RL) were used as biocompatible vehicles for local drug delivery. CGP is a nontoxic biodegradable polymer widely used in pharmaceutical applications. It is a viscous liquid at RT but it congeals to a semi solid phase at body temperature. RL is an isotonic solution used for intravenous administrations in humans. A small volume of this vehicle is precisely placed on the Round Window (RW) niche by means of a bullostomy. A transtympanic injection fills the middle ear and allows less control but broader access to the inner ear. The safety profiles of both techniques were studied and compared by using functional and morphological tests. Hearing was evaluated by registering the Auditory Brainstem Response (ABR) before and several times after microsurgery. The cytoarchitecture and preservation level of cochlear structures were studied by conventional histological techniques in paraformaldehyde-fixed and decalcified cochlear samples. In parallel, unfixed cochlear samples were taken and immediately frozen to analyze gene expression profiles of inflammatory markers by quantitative Reverse Transcriptase Polymerase Chain Reaction (qRT-PCR). Both procedures are suitable as drug delivery methods into the mouse middle ear, although transtympanic injection proved to be less invasive compared to bullostomy. PMID:28362376

A cochlear-bone wave can yield a hearing sensation as well as otoacoustic emission

PubMed Central

Tchumatchenko, Tatjana; Reichenbach, Tobias

2014-01-01

A hearing sensation arises when the elastic basilar membrane inside the cochlea vibrates. The basilar membrane is typically set into motion through airborne sound that displaces the middle ear and induces a pressure difference across the membrane. A second, alternative pathway exists, however: stimulation of the cochlear bone vibrates the basilar membrane as well. This pathway, referred to as bone conduction, is increasingly used in headphones that bypass the ear canal and the middle ear. Furthermore, otoacoustic emissions, sounds generated inside the cochlea and emitted therefrom, may not involve the usual wave on the basilar membrane, suggesting that additional cochlear structures are involved in their propagation. Here we describe a novel propagation mode within the cochlea that emerges through deformation of the cochlear bone. Through a mathematical and computational approach we demonstrate that this propagation mode can explain bone conduction as well as numerous properties of otoacoustic emissions. PMID:24954736

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.

In Vivo Analysis of Lrig Genes Reveals Redundant and Independent Functions in the Inner Ear

PubMed Central

del Rio, Tony; Nishitani, Allison M.; Yu, Wei-Ming; Goodrich, Lisa V.

2013-01-01

Lrig proteins are conserved transmembrane proteins that modulate a variety of signaling pathways from worm to humans. In mammals, there are three family members – Lrig1, Lrig2, and Lrig3 – that are defined by closely related extracellular domains with a similar arrangement of leucine rich repeats and immunoglobulin domains. However, the intracellular domains show little homology. Lrig1 inhibits EGF signaling through internalization and degradation of ErbB receptors. Although Lrig3 can also bind ErbB receptors in vitro, it is unclear whether Lrig2 and Lrig3 exhibit similar functions to Lrig1. To gain insights into Lrig gene functions in vivo, we compared the expression and function of the Lrigs in the inner ear, which offers a sensitive system for detecting effects on morphogenesis and function. We find that all three family members are expressed in the inner ear throughout development, with Lrig1 and Lrig3 restricted to subsets of cells and Lrig2 expressed more broadly. Lrig1 and Lrig3 overlap prominently in the developing vestibular apparatus and simultaneous removal of both genes disrupts inner ear morphogenesis. This suggests that these two family members act redundantly in the otic epithelium. In contrast, although Lrig1 and Lrig2 are frequently co-expressed, Lrig1−/−;Lrig2−/− double mutant ears show no enhanced structural abnormalities. At later stages, Lrig1 expression is sustained in non-sensory tissues, whereas Lrig2 levels are enhanced in neurons and sensory epithelia. Consistent with these distinct expression patterns, Lrig1 and Lrig2 mutant mice exhibit different forms of impaired auditory responsiveness. Notably, Lrig1−/−;Lrig2−/− double mutant mice display vestibular deficits and suffer from a more severe auditory defect that is accompanied by a cochlear innervation phenotype not present in single mutants. Thus, Lrig genes appear to act both redundantly and independently, with Lrig2 emerging as the most functionally distinct family

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.

Inner Ear Drug Delivery for Auditory Applications

PubMed Central

Swan, Erin E. Leary; Mescher, Mark J.; Sewell, William F.; Tao, Sarah L.; Borenstein, Jeffrey T.

2008-01-01

Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the development of local methods of drug delivery to the inner ear. Intratympanic approaches, which deliver therapeutics to the middle ear, rely on permeation through tissue for access to the structures of the inner ear, whereas intracochlear methods are able to directly insert drugs into the inner ear. Innovative drug delivery systems to treat various inner ear ailments such as ototoxicity, sudden sensorineural hearing loss, autoimmune inner ear disease, and for preserving neurons and regenerating sensory cells are being explored. PMID:18848590

[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.

Further studies on the effects of magnetic resonance imaging fields on middle ear implants.

PubMed

Applebaum, E L; Valvassori, G E

1990-10-01

We investigated the effects of magnetic resonance imaging (MRI) fields on 21 stapedectomy prostheses and other middle ear implants and two different receiver-stimulator modules from 22-channel cochlear implants. None of the middle ear implants was displaced by the magnetic field, except for one platinum-stainless steel stapedectomy piston. Magnetism was not induced in any of the middle ear implants subjected to prolonged exposure in the MRI scanner. We conclude that MRI could pose a hazard to patients who have had stapedectomy using certain platinum-stainless steel piston prostheses and to patients with cochlear implants. Magnetic resonance imaging should pose no hazard to patients who have had the other middle ear implants reported on in this and our previous investigation.

The Relationship between Binaural Benefit and Difference in Unilateral Speech Recognition Performance for Bilateral Cochlear Implant Users

PubMed Central

Yoon, Yang-soo; Li, Yongxin; Kang, Hou-Yong; Fu, Qian-Jie

2011-01-01

Objective The full benefit of bilateral cochlear implants may depend on the unilateral performance with each device, the speech materials, processing ability of the user, and/or the listening environment. In this study, bilateral and unilateral speech performances were evaluated in terms of recognition of phonemes and sentences presented in quiet or in noise. Design Speech recognition was measured for unilateral left, unilateral right, and bilateral listening conditions; speech and noise were presented at 0° azimuth. The “binaural benefit” was defined as the difference between bilateral performance and unilateral performance with the better ear. Study Sample 9 adults with bilateral cochlear implants participated. Results On average, results showed a greater binaural benefit in noise than in quiet for all speech tests. More importantly, the binaural benefit was greater when unilateral performance was similar across ears. As the difference in unilateral performance between ears increased, the binaural advantage decreased; this functional relationship was observed across the different speech materials and noise levels even though there was substantial intra- and inter-subject variability. Conclusions The results indicate that subjects who show symmetry in speech recognition performance between implanted ears in general show a large binaural benefit. PMID:21696329

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 pharmacokinetics with local inner ear drug delivery using a three-dimensional finite-element computer model.

PubMed

Plontke, Stefan K; Siedow, Norbert; Wegener, Raimund; Zenner, Hans-Peter; Salt, Alec N

2007-01-01

Cochlear fluid pharmacokinetics can be better represented by three-dimensional (3D) finite-element simulations of drug dispersal. Local drug deliveries to the round window membrane are increasingly being used to treat inner ear disorders. Crucial to the development of safe therapies is knowledge of drug distribution in the inner ear with different delivery methods. Computer simulations allow application protocols and drug delivery systems to be evaluated, and may permit animal studies to be extrapolated to the larger cochlea of the human. A finite-element 3D model of the cochlea was constructed based on geometric dimensions of the guinea pig cochlea. Drug propagation along and between compartments was described by passive diffusion. To demonstrate the potential value of the model, methylprednisolone distribution in the cochlea was calculated for two clinically relevant application protocols using pharmacokinetic parameters derived from a prior one-dimensional (1D) model. In addition, a simplified geometry was used to compare results from 3D with 1D simulations. For the simplified geometry, calculated concentration profiles with distance were in excellent agreement between the 1D and the 3D models. Different drug delivery strategies produce very different concentration time courses, peak concentrations and basal-apical concentration gradients of drug. In addition, 3D computations demonstrate the existence of substantial gradients across the scalae in the basal turn. The 3D model clearly shows the presence of drug gradients across the basal scalae of guinea pigs, demonstrating the necessity of a 3D approach to predict drug movements across and between scalae with larger cross-sectional areas, such as the human, with accuracy. This is the first model to incorporate the volume of the spiral ligament and to calculate diffusion through this structure. Further development of the 3D model will have to incorporate a more accurate geometry of the entire inner ear and

Cochlear Pharmacokinetics with Local Inner Ear Drug Delivery Using a Three-Dimensional Finite-Element Computer Model

PubMed Central

Plontke, Stefan K.; Siedow, Norbert; Wegener, Raimund; Zenner, Hans-Peter; Salt, Alec N.

2006-01-01

Hypothesis: Cochlear fluid pharmacokinetics can be better represented by three-dimensional (3D) finite-element simulations of drug dispersal. Background: Local drug deliveries to the round window membrane are increasingly being used to treat inner ear disorders. Crucial to the development of safe therapies is knowledge of drug distribution in the inner ear with different delivery methods. Computer simulations allow application protocols and drug delivery systems to be evaluated, and may permit animal studies to be extrapolated to the larger cochlea of the human. Methods: A finite-element 3D model of the cochlea was constructed based on geometric dimensions of the guinea pig cochlea. Drug propagation along and between compartments was described by passive diffusion. To demonstrate the potential value of the model, methylprednisolone distribution in the cochlea was calculated for two clinically relevant application protocols using pharmacokinetic parameters derived from a prior one-dimensional (1D) model. In addition, a simplified geometry was used to compare results from 3D with 1D simulations. Results: For the simplified geometry, calculated concentration profiles with distance were in excellent agreement between the 1D and the 3D models. Different drug delivery strategies produce very different concentration time courses, peak concentrations and basal-apical concentration gradients of drug. In addition, 3D computations demonstrate the existence of substantial gradients across the scalae in the basal turn. Conclusion: The 3D model clearly shows the presence of drug gradients across the basal scalae of guinea pigs, demonstrating the necessity of a 3D approach to predict drug movements across and between scalae with larger cross-sectional areas, such as the human, with accuracy. This is the first model to incorporate the volume of the spiral ligament and to calculate diffusion through this structure. Further development of the 3D model will have to incorporate a more

Adenovirus Vectors Target Several Cell Subtypes of Mammalian Inner Ear In Vivo

PubMed Central

Li, Wenyan; Shen, Jun

2016-01-01

Mammalian inner ear harbors diverse cell types that are essential for hearing and balance. Adenovirus is one of the major vectors to deliver genes into the inner ear for functional studies and hair cell regeneration. To identify adenovirus vectors that target specific cell subtypes in the inner ear, we studied three adenovirus vectors, carrying a reporter gene encoding green fluorescent protein (GFP) from two vendors or with a genome editing gene Cre recombinase (Cre), by injection into postnatal days 0 (P0) and 4 (P4) mouse cochlea through scala media by cochleostomy in vivo. We found three adenovirus vectors transduced mouse inner ear cells with different specificities and expression levels, depending on the type of adenoviral vectors and the age of mice. The most frequently targeted region was the cochlear sensory epithelium, including auditory hair cells and supporting cells. Adenovirus with GFP transduced utricular supporting cells as well. This study shows that adenovirus vectors are capable of efficiently and specifically transducing different cell types in the mammalian inner ear and provides useful tools to study inner ear gene function and to evaluate gene therapy to treat hearing loss and vestibular dysfunction. PMID:28116172

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.

Inverse solution of ear-canal area function from reflectance

PubMed Central

Rasetshwane, Daniel M.; Neely, Stephen T.

2011-01-01

A number of acoustical applications require the transformation of acoustical quantities, such as impedance and pressure that are measured at the entrance of the ear canal, to quantities at the eardrum. This transformation often requires knowledge of the shape of the ear canal. Previous attempts to measure ear-canal area functions were either invasive, non-reproducible, or could only measure the area function up to a point mid-way along the canal. A method to determine the area function of the ear canal from measurements of acoustic impedance at the entrance of the ear canal is described. The method is based on a solution to the inverse problem in which measurements of impedance are used to calculate reflectance, which is then used to determine the area function of the canal. The mean ear-canal area function determined using this method is similar to mean ear-canal area functions measured by other researchers using different techniques. The advantage of the proposed method over previous methods is that it is non- invasive, fast, and reproducible. PMID:22225043

Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

PubMed

Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

2015-01-01

Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

[The Heidelberg CI database module : Quality control in hearing restoration with cochlear implants].

PubMed

Herisanu, I T; Hoth, S; Praetorius, M

2016-12-01

Cochlear implants (CI) have been established as the therapy of choice for functional deafness. The number of CI-rehabilitated patients is continuously growing. The resulting data can provide important information for physicians, health insurance companies, and scientists. Assessment and structuring of data becomes more feasible with the application of modern database systems. In collaboration with Innoforce Est., Liechtenstein, the authors developed a database module for the specific needs of CI patients. Data of 100 patients were included and evaluated. The main features of the module and an example data analysis are presented. Analysis of data from these 100 patients reveals 50 men and 50 women aged between 1 and 87 years, with a maximum value in the 51-60-years age group. More than 50% of the patients were also severely hearing impaired in the contralateral ear and fitted the CI indication criteria. Functional deafness in the ear subsequently fitted with CI in most of the patients had arisen more than 20 years previously. Preoperative diagnostic electrical stimulation of the cochlear nerve was positive in 67 patients. All 100 patients perceived auditory sensations with the CI. The presented patient cohort is representative of patients at the Department of Otorhinolaryngology, University of Heidelberg Medical Center, Germany, and the demographic distribution is in accordance with the literature. The state of the contralateral ear, often also fitting the CI indication, is not surprising, as cochlear implantation is a comparatively new procedure. Preoperative electrical stimulation turned out not to be significant by itself. The hearing results and overview of complications were easy to calculate in comparison to a pure data storage system such as i.s.h.med.

Sheep as a large animal ear model: Middle-ear ossicular velocities and intracochlear sound pressure.

PubMed

Péus, Dominik; Dobrev, Ivo; Prochazka, Lukas; Thoele, Konrad; Dalbert, Adrian; Boss, Andreas; Newcomb, Nicolas; Probst, Rudolf; Röösli, Christof; Sim, Jae Hoon; Huber, Alexander; Pfiffner, Flurin

2017-08-01

Animals are frequently used for the development and testing of new hearing devices. Dimensions of the middle ear and cochlea differ significantly between humans and commonly used animals, such as rodents or cats. The sheep cochlea is anatomically more like the human cochlea in size and number of turns. This study investigated the middle-ear ossicular velocities and intracochlear sound pressure (ICSP) in sheep temporal bones, with the aim of characterizing the sheep as an experimental model for implantable hearing devices. Measurements were made on fresh sheep temporal bones. Velocity responses of the middle ear ossicles at the umbo, long process of the incus and stapes footplate were measured in the frequency range of 0.25-8 kHz using a laser Doppler vibrometer system. Results were normalized by the corresponding sound pressure level in the external ear canal (P EC ). Sequentially, ICSPs at the scala vestibuli and tympani were then recorded with custom MEMS-based hydrophones, while presenting identical acoustic stimuli. The sheep middle ear transmitted most effectively around 4.8 kHz, with a maximum stapes velocity of 0.2 mm/s/Pa. At the same frequency, the ICSP measurements in the scala vestibuli and tympani showed the maximum gain relative to the P EC (24 dB and 5 dB, respectively). The greatest pressure difference across the cochlear partition occurred between 4 and 6 kHz. A comparison between the results of this study and human reference data showed middle-ear resonance and best cochlear sensitivity at higher frequencies in sheep. In summary, sheep can be an appropriate large animal model for research and development of implantable hearing devices. Copyright © 2017 Elsevier B.V. All rights reserved.

Continued Expression of GATA3 Is Necessary for Cochlear Neurosensory Development

PubMed Central

Duncan, Jeremy S.; Fritzsch, Bernd

2013-01-01

Hair cells of the developing mammalian inner ear are progressively defined through cell fate restriction. This process culminates in the expression of the bHLH transcription factor Atoh1, which is necessary for differentiation of hair cells, but not for their specification. Loss of several genes will disrupt ear morphogenesis or arrest of neurosensory epithelia development. We previously showed in null mutants that the loss of the transcription factor, Gata3, results specifically in the loss of all cochlear neurosensory development. Temporal expression of Gata3 is broad from the otic placode stage through the postnatal ear. It therefore remains unclear at which stage in development Gata3 exerts its effect. To better understand the stage specific effects of Gata3, we investigated the role of Gata3 in cochlear neurosensory specification and differentiation utilizing a LoxP targeted Gata3 line and two Cre lines. Foxg1Cre∶Gata3f/f mice show recombination of Gata3 around E8.5 but continue to develop a cochlear duct without differentiated hair cells and spiral ganglion neurons. qRT-PCR data show that Atoh1 was down-regulated but not absent in the duct whereas other hair cell specific genes such as Pou4f3 were completely absent. In addition, while Sox2 levels were lower in the Foxg1Cre:Gata3f/f cochlea, Eya1 levels remained normal. We conclude that Eya1 is unable to fully upregulate Atoh1 or Pou4f3, and drive differentiation of hair cells without Gata3. Pax2-Cre∶Gata3f/f mice show a delayed recombination of Gata3 in the ear relative to Foxg1Cre:Gata3f/f. These mice exhibited a cochlear duct containing patches of partially differentiated hair cells and developed only few and incorrectly projecting spiral ganglion neurons. Our conditional deletion studies reveal a major role of Gata3 in the signaling of prosensory genes and in the differentiation of cochlear neurosenory cells. We suggest that Gata3 may act in combination with Eya1, Six1, and Sox2 in cochlear prosensory

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.)

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.

Evaluation of cochlear involvement by transient evoked otoacoustic emission test in children with Crimean-Congo hemorrhagic fever.

PubMed

Uysal, İsmail Önder; Kaya, Ali; Güven, Ahmet Sami; Altuntaş, Emine Elif; Müderris, Suphi

2011-06-01

The aim of this study was to investigate cochlear involvement in child patients with Crimean-Congo hemorrhagic fever (CCHF) disease. Twenty-eight CCHF disease patients (56 ears) and 26 sex- and age-matched healthy control subjects (52 ears) were included in the study. Pure-tone audiometry at frequencies 0.25, 0.5, 1, 2, 4, and 6 kHz, immittance measures including tympanometry and acoustic reflex testing, and transient evoked otoacoustic emission (TEOAE) testing were performed in the patients and controls. The proportion with a result of 'fail' for the TEOAE test in the CCHF patients was not statistically significant from the control group (p>0.05). CCHF disease does not impair cochlear function in children. The clinical course of CCHF among children seems to be milder than in adults. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

Bilateral Cochlear Implants in Infants: A New Approach—Nucleus Hybrid S12 Project

PubMed Central

Gantz, Bruce J.; Dunn, Camille C.; Walker, Elizabeth A.; Kenworthy, Maura; Van Voorst, Tanya; Tomblin, Bruce; Turner, Chris

2010-01-01

Objective The purpose of this feasibility study was to evaluate whether the use of a shorter-length cochlear implant (10 mm) on one ear and a standard electrode (24 mm) on the contralateral ear is a viable bilateral option for children with profound bilateral sensorineural hearing loss. A secondary purpose of this study was to determine whether the ear with the shorter-length electrode performs similarly to the standard-length electrode. Our goal was to provide an option of electrical stimulation that theoretically might preserve the structures of the scala media and organ of Corti. Study Design The study is being conducted as a repeated-measure, single-subject experiment. Setting University of Iowa—Department of Otolaryngology. Patients Eight pediatric patients with profound bilateral sensorineural hearing loss between the ages of 12 and 24 months. Interventions Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in the contralateral ear. Main Outcome Measures The Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) parent questionnaire, Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children’s Vowel tests will be used to evaluate speech perception and the Minnesota Child Development Inventory and Preschool Language Scales 3 test will be used to evaluate language growth. Results Preliminary results for 8 children have been collected before and after the operation using the IT-MAIS. All 3 children showed incremental improvements in their IT-MAIS scores overtime. Early Speech Perception, Glendonald Auditory Screening Procedure word test, and Children’s Vowel word perception results indicated no difference between the individual ears for the 2 children tested. Performance compared with age-matched children implanted with standard bilateral cochlear implants showed similar results to the children implanted with Nucleus Hybrid S12 10-mm electrode and a Nucleus Freedom implant in contralateral ears

Transmission matrix analysis of the chinchilla middle ear

PubMed Central

Songer, Jocelyn E.; Rosowski, John J.

2008-01-01

Despite the common use of the chinchilla as an animal model in auditory research, a complete characterization of the chinchilla middle ear using transmission matrix analysis has not been performed. In this paper we describe measurements of middle-ear input admittance and stapes velocity in ears with the middle-ear cavity opened under three conditions: intact tympano-ossicular system and cochlea, after the cochlea has been drained, and after the stapes has been fixed. These measurements, made with stimulus frequencies of 100–8000 Hz, are used to define the transmission matrix parameters of the middle ear and to calculate the cochlear input impedance as well as the middle-ear output impedance. This transmission characterization of the chinchilla middle ear will be useful for modeling auditory sensitivity in the normal and pathological chinchilla ear. PMID:17672642

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.

Determination of the position of nucleus cochlear implant electrodes in the inner ear.

PubMed

Skinner, M W; Ketten, D R; Vannier, M W; Gates, G A; Yoffie, R L; Kalender, W A

1994-09-01

Accurate determination of intracochlear electrode position in patients with cochlear implants could provide a basis for detecting migration of the implant and could aid in the selection of stimulation parameters for sound processor programming. New computer algorithms for submillimeter resolution and 3-D reconstruction from spiral computed tomographic (CT) scans now make it possible to accurately determine the position of implanted electrodes within the cochlear canal. The accuracy of these algorithms was tested using an electrode array placed in a phantom model. Measurements of electrode length and interelectrode distance from spiral CT scan reconstructions were in close agreement with those from stereo microscopy. Although apparent electrode width was increased on CT scans due to partial volume averaging, a correction factor was developed for measurements from conventional radiographs and an expanded CT absorption value scale added to detect the presence of platinum electrodes and wires. The length of the cochlear canal was calculated from preoperative spiral CT scans for one patient, and the length of insertion of the electrode array was calculated from her postoperative spiral CT scans. The cross-sectional position of electrodes in relation to the outer bony wall and modiolus was measured and plotted as a function of distance with the electrode width correction applied.

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.

Preoperative vestibular assessment protocol of cochlear implant surgery: an analytical descriptive study.

PubMed

Bittar, Roseli Saraiva Moreira; Sato, Eduardo Setsuo; Ribeiro, Douglas Jósimo Silva; Tsuji, Robinson Koji

Cochlear implants are undeniably an effective method for the recovery of hearing function in patients with hearing loss. To describe the preoperative vestibular assessment protocol in subjects who will be submitted to cochlear implants. Our institutional protocol provides the vestibular diagnosis through six simple tests: Romberg and Fukuda tests, assessment for spontaneous nystagmus, Head Impulse Test, evaluation for Head Shaking Nystagmus and caloric test. 21 patients were evaluated with a mean age of 42.75±14.38 years. Only 28% of the sample had all normal test results. The presence of asymmetric vestibular information was documented through the caloric test in 32% of the sample and spontaneous nystagmus was an important clue for the diagnosis. Bilateral vestibular areflexia was present in four subjects, unilateral arreflexia in three and bilateral hyporeflexia in two. The Head Impulse Test was a significant indicator for the diagnosis of areflexia in the tested ear (p=0.0001). The sensitized Romberg test using a foam pad was able to diagnose severe vestibular function impairment (p=0.003). The six clinical tests were able to identify the presence or absence of vestibular function and function asymmetry between the ears of the same individual. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Computational modeling of the generation and propagation of distortion products in the inner ear

NASA Astrophysics Data System (ADS)

Bowling, Thomas; Wen, Haiqi; Meaud, Julien

2018-05-01

Distortion product otoacoustic emissions are used in both clinical and research settings to assess cochlear function although there are still questions for how the distortion products propagate in the cochlea from their generation location to the middle ear. Here, a physiologically based computational model of the gerbil ear is used to investigate distortion product propagation. The fluid is modeled in three dimensions and includes two ducts. Simulations of the distortion products in the cochlear fluid pressure and basilar membrane are compared with published experimental data. Model results are consistent with measurements from Ren and colleagues which indicated that the intracochlear distortion product is dominated by a forward traveling wave at a low primary frequency ratio, although backward traveling waves become apparent when other ratios are considered. The magnitude and phase of both basilar membrane and spatial variations of the distortion product fluid pressure are qualitatively similar to the expected response of a slowly propagating backward traveling wave. These results combined suggest that distortion products propagate primarily as a slow wave both when the cochlea is driven by intracochlear sources and an acoustic stimulus in the ear canal.

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.

[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.

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

Finite element modelling of sound transmission from outer to inner ear.

PubMed

Areias, Bruno; Santos, Carla; Natal Jorge, Renato M; Gentil, Fernanda; Parente, Marco Pl

2016-11-01

The ear is one of the most complex organs in the human body. Sound is a sequence of pressure waves, which propagates through a compressible media such as air. The pinna concentrates the sound waves into the external auditory meatus. In this canal, the sound is conducted to the tympanic membrane. The tympanic membrane transforms the pressure variations into mechanical displacements, which are then transmitted to the ossicles. The vibration of the stapes footplate creates pressure waves in the fluid inside the cochlea; these pressure waves stimulate the hair cells, generating electrical signals which are sent to the brain through the cochlear nerve, where they are decoded. In this work, a three-dimensional finite element model of the human ear is developed. The model incorporates the tympanic membrane, ossicular bones, part of temporal bone (external auditory meatus and tympanic cavity), middle ear ligaments and tendons, cochlear fluid, skin, ear cartilage, jaw and the air in external auditory meatus and tympanic cavity. Using the finite element method, the magnitude and the phase angle of the umbo and stapes footplate displacement are calculated. Two slightly different models are used: one model takes into consideration the presence of air in the external auditory meatus while the other does not. The middle ear sound transfer function is determined for a stimulus of 60 dB SPL, applied to the outer surface of the air in the external auditory meatus. The obtained results are compared with previously published data in the literature. This study highlights the importance of external auditory meatus in the sound transmission. The pressure gain is calculated for the external auditory meatus.

Bilateral Sequential Cochlear Implantation in Patients With Enlarged Vestibular Aqueduct (EVA) Syndrome.

PubMed

Manzoor, Nauman F; Wick, Cameron C; Wahba, Marian; Gupta, Amit; Piper, Robin; Murray, Gail S; Otteson, Todd; Megerian, Cliff A; Semaan, Maroun T

2016-02-01

To analyze audiometric outcomes after bilateral cochlear implantation in patients with isolated enlarged vestibular aqueduct (EVA) syndrome and associated incomplete partition (IP) malformations. Secondary objective was to analyze rate of cerebrospinal fluid (CSF) gusher in patients with IP-EVA spectrum deformities and compare this with the existing literature. Retrospective chart review. Thirty-two patients with EVA syndrome who received unilateral or bilateral cochlear implants between June 1999 and January 2014 were identified in the University Hospitals Case Medical Center cochlear implant database. Isolated EVA (IEVA) and Incomplete Partition Type II (IP-II) malformations were identified by reviewing high-resolution computed tomography (HRCT) imaging. Demographic information, age at implantation, surgical details, postimplantation audiometric data including speech reception thresholds (SRT), word, and sentence scores were reviewed and analyzed. Intra- and postoperative complications were analyzed as well and compared with the literature. Seventeen patients (32 implanted ears) had pediatric cochlear implantation for EVA-associated hearing loss. Data from 16 controls (32 implanted ears) were used to compare audiometric and speech outcomes of EVA cohort. Mean age at implantation was 6.8 years for EVA cohort and 6.0 years for controls. There was no statistically significant difference in long-term postoperative SRT, monaurally aided word scores, and binaurally tested word scores between pediatric EVA group and controls. The EVA patients had a long-term mean sentence score of 85.92%. A subset of EVA patients implanted at mean age of 3.18 years (n = 15 ears) had similar audiometric outcomes to another control group with Connexin 26 mutations (n = 20 ears) implanted at a similar age. Further subset analysis revealed no significant differences in age at implantation, SRT, and word scores in patients with IEVA and IP-II malformation. There was no significant

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.

Surgical factors in pediatric cochlear implantation and their early effects on electrode activation and functional outcomes.

PubMed

Francis, Howard W; Buchman, Craig A; Visaya, Jiovani M; Wang, Nae-Yuh; Zwolan, Teresa A; Fink, Nancy E; Niparko, John K

2008-06-01

To assess the impact of surgical factors on electrode status and early communication outcomes in young children in the first 2 years of cochlear implantation. Prospective multicenter cohort study. Six tertiary referral centers. Children 5 years or younger before implantation with normal nonverbal intelligence. Cochlear implant operations in 209 ears of 188 children. Percent active channels, auditory behavior as measured by the Infant Toddler Meaningful Auditory Integration Scale/Meaningful Auditory Integration Scale and Reynell receptive language scores. Stable insertion of the full electrode array was accomplished in 96.2% of ears. At least 75% of electrode channels were active in 88% of ears. Electrode deactivation had a significant negative effect on Infant Toddler Meaningful Auditory Integration Scale/Meaningful Auditory Integration Scale scores at 24 months but no effect on receptive language scores. Significantly fewer active electrodes were associated with a history of meningitis. Surgical complications requiring additional hospitalization and/or revision surgery occurred in 6.7% of patients but had no measurable effect on the development of auditory behavior within the first 2 years. Negative, although insignificant, associations were observed between the need for perioperative revision of the device and 1) the percent of active electrodes and 2) the receptive language level at 2-year follow-up. Activation of the entire electrode array is associated with better early auditory outcomes. Decrements in the number of active electrodes and lower gains of receptive language after manipulation of the newly implanted device were not statistically significant but may be clinically relevant, underscoring the importance of surgical technique and the effective placement of the electrode array.

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…

Isolation of sphere-forming stem cells from the mouse inner ear.

PubMed

Oshima, Kazuo; Senn, Pascal; Heller, Stefan

2009-01-01

The mammalian inner ear has very limited ability to regenerate lost sensory hair cells. This deficiency becomes apparent when hair cell loss leads to hearing loss as a result of either ototoxic insult or the aging process. Coincidently, with this inability to regenerate lost hair cells, the adult cochlea does not appear to harbor cells with a proliferative capacity that could serve as progenitor cells for lost cells. In contrast, adult mammalian vestibular sensory epithelia display a limited ability for hair cell regeneration, and sphere-forming cells with stem cell features can be isolated from the adult murine vestibular system. The neonatal inner ear, however, does harbor sphere-forming stem cells residing in cochlear and vestibular tissues. Here, we provide protocols to isolate sphere-forming stem cells from neonatal vestibular and cochlear sensory epithelia as well as from the spiral ganglion. We further describe procedures for sphere propagation, cell differentiation, and characterization of inner ear cell types derived from spheres. Sphere-forming stem cells from the mouse inner ear are an important tool for the development of cellular replacement strategies of damaged inner ears and are a bona fide progenitor cell source for transplantation studies.

[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.

Auditory plasticity in deaf children with bilateral cochlear implants

NASA Astrophysics Data System (ADS)

Litovsky, Ruth

2005-04-01

Human children with cochlear implants represent a unique population of individuals who have undergone variable amounts of auditory deprivation prior to being able to hear. Even more unique are children who received bilateral cochlear implants (BICIs), in sequential surgical procedures, several years apart. Auditory deprivation in these individuals consists of a two-stage process, whereby complete deafness is experienced initially, followed by deafness in one ear. We studied the effects of post-implant experience on the ability of deaf children to localize sounds and to understand speech in noise. These are two of the most important functions that are known to depend on binaural hearing. Children were tested at time intervals ranging from 3-months to 24-months following implantation of the second ear, while listening with either implant alone or bilaterally. Our findings suggest that the period during which plasticity occurs in human binaural system is protracted, extending into middle-to-late childhood. The rate at which benefits from bilateral hearing abilities are attained following deprivation is faster for speech intelligibility in noise compared with sound localization. Finally, the age at which the second implant was received may play an important role in the acquisition of binaural abilities. [Work supported by NIH-NIDCD.

Petrosal anatomy and inner ear structures of the Late Jurassic Henkelotherium (Mammalia, Cladotheria, Dryolestoidea): insight into the early evolution of the ear region in cladotherian mammals

PubMed Central

Ruf, Irina; Luo, Zhe-Xi; Wible, John R; Martin, Thomas

2009-01-01

The petrosal anatomy and inner ear structure of Jurassic cladotherian mammals represent the ancestral morphological conditions (groundplan) from which modern therian mammals (marsupials and placentals) have evolved. We present the reconstruction of the petrosal and inner ear features of the Late Jurassic dryolestoid mammal Henkelotherium guimarotae from high-resolution computed tomography and three-dimensional imaging analysis. This study of Henkelotherium revealed a combination of derived and primitive features, including: cladotherian apomorphies, such as the promontorial sulcus for the internal carotid artery and reduced lateral trough; trechnotherian characters, such as an enclosed cochlear canaliculus for the perilymphatic duct, post-promontorial tympanic sinus and caudal tympanic process; in addition to plesiomorphic mammalian features, such as the cavum supracochleare and prootic canal. The inner ear of Henkelotherium shows a division between the utricle and saccule, a cochlear canal coiled through at least 270°, a distinctive primary bony lamina for the basilar membrane, and a secondary bony lamina. The development of the primary and secondary bony laminae in the cochlear canal is suggested here to be correlated with the concurrent coiling of the bony canal and membranous duct of the inner ear cochlea, apomorphies of the more inclusive cladotherian clade that also represent the ancestral morphotype of modern therian mammals. Because these features are crucial for high-frequency hearing in extant therian mammals, their early appearance in Late Jurassic cladotherians suggests a more ancient origination for high-frequency hearing in mammalian history than previously thought. PMID:19438763

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

The Electrical Activity of a Denervated Ear 1

PubMed Central

Rawdon-Smith, A. F.; Hawkins, J. E.

1939-01-01

The electrical response from the cochlea of a cat which had previously been denervated by intracranial crushing of the auditory nerve was submitted to a lengthy study, the results of which may be summarized as follows:- The responses to acoustical stimulation derived from electrodes placed on the round window margin and in the chin muscles were studied by means of an amplifier and cathode ray oscillograph, in the usual way. Transient stimuli whose polarity could be reversed were employed to demonstrate the absence of any electrical component of neural origin such as is invariably present in a normal ear. In all other respects, however, the responses were unaffected, and both threshold contours (the so-called “electrical audiogram”) and equal response contours for approximately pure-tone stimuli demonstrated close comparability with those for normal ears. Harmonic analysis of the cochlear response yielded results departing from the normal only in such respects as would be expected in view of the complete absence of nervous component in the analysed wave. From these data, it is argued that this animal presented a case in which normal electrical responses were obtained from the peripheral organ, despite virtually complete degeneration of the auditory nerve, and, it follows, complete unilateral deafness. Subsequent histological examination confirmed these observations, and it is urged, therefore, that the validity of the view that the cochlear response provides an index of the hearing ability of an animal, as is sometimes stated, is open to question. Additionally, this experiment finally discredits the hypothesis that the cochlear response itself is, in any sense, neural in origin; it further indicates the necessity for caution in the interpretation of results obtained from normal ears, where the cochlear response, however derived, is in some degree adulterated by the simultaneous presence of an action potential component. ImagesFig. 8 PMID:19991849

Physiological functioning of the ear and masking

NASA Technical Reports Server (NTRS)

1984-01-01

The physiological functions of the ear and the role masking plays in speech communication are examined. Topics under investigation include sound analysis of the ear, the aural reflex, and various types of noise masking.

Measurement of cochlear length using the 'A' value for cochlea basal diameter: A feasibility study.

PubMed

Deep, Nicholas L; Howard, Brittany E; Holbert, Sarah O; Hoxworth, Joseph M; Barrs, David M

2017-07-01

To determine whether the cochlea basal diameter (A value) measurement can be consistently and precisely obtained from high-resolution temporal bone imaging for use in cochlear length estimation. A feasibility study at a tertiary referral center was performed using the temporal bone CTs of 40 consecutive patients. The distance from the round window to the lateral wall was measured for each cochlea by two independent reviewers, a neuroradiologist and an otolaryngologist. The interrater reliability was calculated using the intraclass correlation coefficient (ICC) and the Bland-Altman plot. Forty patients (19 males, 21 females) for a total of 80 cochleae were included. Interrater reliability on the same ear had a high level of agreement by both the ICC and the Bland-Altman plot. ICCs were 0.90 (95% CI: 0.82, 0.94) for the left ear and 0.96 (95% CI: 0.92, 0.98) for the right ear. Bland-Altman plot confirmed interrater reliability with all 96% of measurements falling within the 95% limits of agreement. Measurement between the round window and lateral cochlear wall can be consistently and reliably obtained from high-resolution temporal bone CT scans. Thus, it is feasible to utilize this method to estimate the cochlear length of patients undergoing cochlear implantation.

Malformation of stria vascularis in the developing inner ear of the German waltzing guinea pig.

PubMed

Jin, Zhe; Mannström, Paula; Järlebark, Leif; Ulfendahl, Mats

2007-05-01

Auditory function and cochlear morphology have previously been described in the postnatal German waltzing guinea pig, a strain with recessive deafness. In the present study, cochlear histopathology was further investigated in the inner ear of the developing German waltzing guinea pig (gw/gw). The lumen of the cochlear duct diminished progressively from embryonic day (E) 35 to E45 and was absent at E50 because of the complete collapse of Reissner's membrane onto the hearing organ. The embryonic stria vascularis, consisting of a simple epithelium, failed to transform into the complex trilaminar tissue seen in normal animals and displayed signs of degeneration. Subsequent degeneration of the sensory epithelium was observed from E50 and onwards. Defective and insufficient numbers of melanocytes were observed in the developing gw/gw stria vascularis. A gene involved in cochlear melanocyte development, Pax3, was markedly reduced in lateral wall tissue of the cochlea of both E40 and adult gw/gw individuals, whereas its expression was normal in the skin and diaphragm muscle of adult gw/gw animals. The Pax3 gene may thus be involved in the pathological process but is unlikely to be the primary mutated gene in the German waltzing guinea pig. TUNEL assay showed no signs of apoptotic cell death in the developing stria vascularis of this type of guinea pig. Thus, malformation of the stria vascularis appears to be the primary defect in the inner ear of the German waltzing guinea pig. Defective and insufficient numbers of melanocytes might migrate to the developing stria vascularis but fail to provide the proper support for the subsequent development of marginal and basal cells, thereby leading to stria vascularis malformation and dysfunction in the inner ear of the German waltzing guinea pig.

Chinchilla middle-ear admittance and sound power: High-frequency estimates and effects of inner-ear modifications

PubMed Central

Ravicz, Michael E.; Rosowski, John J.

2012-01-01

The middle-ear input admittance relates sound power into the middle ear (ME) and sound pressure at the tympanic membrane (TM). ME input admittance was measured in the chinchilla ear canal as part of a larger study of sound power transmission through the ME into the inner ear. The middle ear was open, and the inner ear was intact or modified with small sensors inserted into the vestibule near the cochlear base. A simple model of the chinchilla ear canal, based on ear canal sound pressure measurements at two points along the canal and an assumption of plane-wave propagation, enables reliable estimates of YTM, the ME input admittance at the TM, from the admittance measured relatively far from the TM. YTM appears valid at frequencies as high as 17 kHz, a much higher frequency than previously reported. The real part of YTM decreases with frequency above 2 kHz. Effects of the inner-ear sensors (necessary for inner ear power computation) were small and generally limited to frequencies below 3 kHz. Computed power reflectance was ∼0.1 below 3.5 kHz, lower than with an intact ME below 2.5 kHz, and nearly 1 above 16 kHz. PMID:23039439

Inner ear dysplasia is common in children with Down syndrome (trisomy 21).

PubMed

Blaser, Susan; Propst, Evan J; Martin, Daniel; Feigenbaum, Annette; James, Adrian L; Shannon, Patrick; Papsin, Blake C

2006-12-01

Middle and external ear anomalies are well recognized in Down syndrome (DS, trisomy 21). Inner ear anomalies are much less frequently described. This study reviews inner ear morphology on imaging to determine the prevalence of cochlear and vestibular anomalies in children with DS. The authors conducted a retrospective review of imaging features of (DS) inner ear structures. Fifty-nine sequential patients with DS with imaging of the inner ear were identified by a radiology report text search program. Quantitative biometric assessment of the inner ear was performed on patients with high-resolution computed tomography or magnetic resonance images of the petrous bone. Petrous imaging was performed for evaluation of inflammatory disease or hearing loss. Spinal imaging, which included petrous views, was performed in most cases to exclude C1 to 2 dislocation, a potential complication of DS. Measurements were compared with normative data. Inner ear dysplasia is much more common in DS than previously reported. Inner ear structures are universally hypoplastic. Vestibular malformations are particularly common and a small bony island of the lateral semicircular canal (<3 mm in diameter) appears highly typical. Additional findings in some patients were persistent lateral semicircular anlage with fusion of the lateral semicircular canal and vestibule into a single cavity, vestibular aqueduct and endolymphatic sac fossa enlargement, cochlear nerve canal hypoplasia, and stenosis or duplication of the internal auditory canal. Stenosis of the external meatus, poor mastoid pneumatization, middle ear and mastoid opacification, and cholesteatoma were common, as expected.

No effect of prolonged pulsed high frequency ultrasound imaging of the basilar membrane on cochlear function or hair cell survival found in an initial study.

PubMed

Landry, Thomas G; Bance, Manohar L; Adamson, Robert B; Brown, Jeremy A

2018-06-01

Miniature high frequency ultrasound devices show promise as tools for clinical middle ear and basal cochlea imaging and vibrometry. However, before clinical use it is important to verify that the ultrasound exposure does not damage the cochlea. In this initial study, electrophysiological responses of the cochlea were measured for a range of stimulus frequencies in both ears of anesthetized chinchillas, before and after exposing the organ of Corti region of one ear to pulsed focused ultrasound for 30 min. Measurements were again taken after an 11 day survival period. Cochlear tissue was examined with a confocal microscope for signs of damage to the cochlear hair cells. No significant change in response thresholds due to exposure was found, and no signs of ultrasound-induced tissue damage were observed, although one animal (out of ten) did have a region of extensive tissue damage in the exposed cochlea. However, after further analysis this was concluded to be not likely a result of the ultrasound exposure. Copyright © 2017 Elsevier B.V. All rights reserved.

Modeling microstructure of incudostapedial joint and the effect on cochlear input

NASA Astrophysics Data System (ADS)

Gan, Rong Z.; Wang, Xuelin

2015-12-01

The incudostapedial joint (ISJ) connects the incus to stapes in human ear and plays an important role for sound transmission from the tympanic membrane (TM) to cochlea. ISJ is a synovial joint composed of articular cartilage on the lenticular process and stapes head with the synovial fluid between them. However, there is no study on how the synovial ISJ affects the middle ear and cochlear functions. Recently, we have developed a 3-dimensinal finite element (FE) model of synovial ISJ and connected the model to our comprehensive FE model of the human ear. The motions of TM, stapes footplate, and basilar membrane and the pressures in scala vestibule and scala tympani were derived over frequencies and compared with experimental measurements. Results show that the synovial ISJ affects sound transmission into cochlea and the frequency-dependent viscoelastic behavior of ISJ provides protection for cochlea from high intensity sound.

Investigation of Notch Signaling during Spontaneous Regeneration of Cochlear Hair Cells

DTIC Science & Technology

2016-10-01

AWARD NUMBER: W81XWH-15-1-0475 TITLE: Investigation of Notch Signaling during Spontaneous Regeneration of Cochlear Hair Cells PRINCIPAL...Sep 2016 4. TITLE AND SUBTITLE 5a. CONTRACT NUMBER Investigation of Notch Signaling during Spontaneous Regeneration of Cochlear Hair Cells 5b...inherent to military settings. These noise exposures damage and kill sensory hair cells (HCs) found in the cochlea of the inner ear, resulting in permanent

Effects of carbogen on cochlear blood flow and hearing function following acute acoustic trauma in guinea pigs.

PubMed

Zhao, Jing; Sun, Jianjun; Liu, Yang

2012-10-01

Disturbances of microcirculation and hemorheological changes in the inner ear are the results of noise-induced hearing loss (NIHL). Both the disturbances of microcirculation and hemorheological changes are the etiologies of NIHL development, but they are also the results. Although previous reports that inhalation of high concentration of CO(2) may increase cochlear blood flow (CoBF), the effects of carbogen on the cochlear microcirculation and NIHL remain unclear. Changes induced by noise, carbogen and pure oxygen within the cochlear lateral wall microvasculature and in hearing thresholds were observed in guinea pigs using intravital microscopy and the auditory brainstem response. At the same time, arterial oxygen saturation and morphologic changes of cochlear hair cells were observed. Carbogen inhalation increased vessel diameters and blood flow velocities. Hearing thresholds elevation in the carbogen group was smaller than those in the control and oxygen group (p <0.05). Carbogen inhalation produced a trend toward less threshold shift after noise exposure, which reached statistical significance after day 3 (p <0.01). Respiratory acidosis was not found in our study. The segmented basal membranes of Corti in three groups indicated that no losses or discorders of hair cells were found. Carbogen inhalation can preserve hearing in animal models after acute acoustic trauma. Copyright © 2012 IMSS. All rights reserved.

Evidence for a Right-Ear Advantage in Newborn Hearing Screening Results

PubMed Central

Hildesheimer, Minka; Roziner, Ilan; Henkin, Yael

2016-01-01

The aim of the present study was to investigate the effect of ear asymmetry, order of testing, and gender on transient-evoked otoacoustic emission (TEOAE) pass rates and response levels in newborn hearing screening. The screening results of 879 newborns, of whom 387 (study group) passed screening successfully in only one ear in the first TEOAE screening, but passed screening successfully in both ears thereafter, and 492 (control group) who passed screening successfully in both ears in the first TEOAE, were retrospectively examined for pass rates and TEOAE characteristics. Results indicated a right-ear advantage, as manifested by significantly higher pass rates in the right ear (61% and 39% for right and left ears, respectively) in the study group, and in 1.75 dB greater TEOAE response amplitudes in the control group. The right-ear advantage was enhanced when the first tested ear was the right ear (76%). When the left ear was tested first, pass rates were comparable in both ears. The right-ear advantage in pass rates was similar in females versus males, but manifested in 1.5 dB higher response amplitudes in females compared with males, regardless of the tested ear and order of testing in both study and control groups. The study provides further evidence for the functional lateralization of the auditory system at the cochlear level already apparent soon after birth in both males and females. While order of testing plays a significant role in the asymmetry in pass rates, the innate right-ear advantage seems to be a more dominant contributor. PMID:27927982

Demonstration of a longitudinal concentration gradient along scala tympani by sequential sampling of perilymph from the cochlear apex.

PubMed

Mynatt, Robert; Hale, Shane A; Gill, Ruth M; Plontke, Stefan K; Salt, Alec N

2006-06-01

Local applications of drugs to the inner ear are increasingly being used to treat patients' inner ear disorders. Knowledge of the pharmacokinetics of drugs in the inner ear fluids is essential for a scientific basis for such treatments. When auditory function is of primary interest, the drug's kinetics in scala tympani (ST) must be established. Measurement of drug levels in ST is technically difficult because of the known contamination of perilymph samples taken from the basal cochlear turn with cerebrospinal fluid (CSF). Recently, we reported a technique in which perilymph was sampled from the cochlear apex to minimize the influence of CSF contamination (J. Neurosci. Methods, doi: 10.1016/j.jneumeth.2005.10.008 ). This technique has now been extended by taking smaller fluid samples sequentially from the cochlear apex, which can be used to quantify drug gradients along ST. The sampling and analysis methods were evaluated using an ionic marker, trimethylphenylammonium (TMPA), that was applied to the round window membrane. After loading perilymph with TMPA, 10 1-muL samples were taken from the cochlear apex. The TMPA content of the samples was consistent with the first sample containing perilymph from apical regions and the fourth or fifth sample containing perilymph from the basal turn. TMPA concentration decreased in subsequent samples, as they increasingly contained CSF that had passed through ST. Sample concentration curves were interpreted quantitatively by simulation of the experiment with a finite element model and by an automated curve-fitting method by which the apical-basal gradient was estimated. The study demonstrates that sequential apical sampling provides drug gradient data for ST perilymph while avoiding the major distortions of sample composition associated with basal turn sampling. The method can be used for any substance for which a sensitive assay is available and is therefore of high relevance for the development of preclinical and clinical

Demonstration of a Longitudinal Concentration Gradient Along Scala Tympani by Sequential Sampling of Perilymph from the Cochlear Apex

PubMed Central

Mynatt, Robert; Hale, Shane A.; Gill, Ruth M.; Plontke, Stefan K.

2006-01-01

ABSTRACT Local applications of drugs to the inner ear are increasingly being used to treat patients' inner ear disorders. Knowledge of the pharmacokinetics of drugs in the inner ear fluids is essential for a scientific basis for such treatments. When auditory function is of primary interest, the drug's kinetics in scala tympani (ST) must be established. Measurement of drug levels in ST is technically difficult because of the known contamination of perilymph samples taken from the basal cochlear turn with cerebrospinal fluid (CSF). Recently, we reported a technique in which perilymph was sampled from the cochlear apex to minimize the influence of CSF contamination (J. Neurosci. Methods, doi: http://10.1016/j.jneumeth.2005.10.008). This technique has now been extended by taking smaller fluid samples sequentially from the cochlear apex, which can be used to quantify drug gradients along ST. The sampling and analysis methods were evaluated using an ionic marker, trimethylphenylammonium (TMPA), that was applied to the round window membrane. After loading perilymph with TMPA, 10 1-μL samples were taken from the cochlear apex. The TMPA content of the samples was consistent with the first sample containing perilymph from apical regions and the fourth or fifth sample containing perilymph from the basal turn. TMPA concentration decreased in subsequent samples, as they increasingly contained CSF that had passed through ST. Sample concentration curves were interpreted quantitatively by simulation of the experiment with a finite element model and by an automated curve-fitting method by which the apical–basal gradient was estimated. The study demonstrates that sequential apical sampling provides drug gradient data for ST perilymph while avoiding the major distortions of sample composition associated with basal turn sampling. The method can be used for any substance for which a sensitive assay is available and is therefore of high relevance for the development of preclinical

Localization and interaural time difference (ITD) thresholds for cochlear implant recipients with preserved acoustic hearing in the implanted ear

PubMed Central

Gifford, René H.; Grantham, D. Wesley; Sheffield, Sterling W.; Davis, Timothy J.; Dwyer, Robert; Dorman, Michael F.

2014-01-01

The purpose of this study was to investigate horizontal plane localization and interaural time difference (ITD) thresholds for 14 adult cochlear implant recipients with hearing preservation in the implanted ear. Localization to broadband noise was assessed in an anechoic chamber with a 33-loudspeaker array extending from −90 to +90°. Three listening conditions were tested including bilateral hearing aids, bimodal (implant + contralateral hearing aid) and best aided (implant + bilateral hearing aids). ITD thresholds were assessed, under headphones, for low-frequency stimuli including a 250-Hz tone and bandpass noise (100–900 Hz). Localization, in overall rms error, was significantly poorer in the bimodal condition (mean: 60.2°) as compared to both bilateral hearing aids (mean: 46.1°) and the best-aided condition (mean: 43.4°). ITD thresholds were assessed for the same 14 adult implant recipients as well as 5 normal-hearing adults. ITD thresholds were highly variable across the implant recipients ranging from the range of normal to ITDs not present in real-world listening environments (range: 43 to over 1600 μs). ITD thresholds were significantly correlated with localization, the degree of interaural asymmetry in low-frequency hearing, and the degree of hearing preservation related benefit in the speech reception threshold (SRT). These data suggest that implant recipients with hearing preservation in the implanted ear have access to binaural cues and that the sensitivity to ITDs is significantly correlated with localization and degree of preserved hearing in the implanted ear. PMID:24607490

Localization and interaural time difference (ITD) thresholds for cochlear implant recipients with preserved acoustic hearing in the implanted ear.

PubMed

Gifford, René H; Grantham, D Wesley; Sheffield, Sterling W; Davis, Timothy J; Dwyer, Robert; Dorman, Michael F

2014-06-01

The purpose of this study was to investigate horizontal plane localization and interaural time difference (ITD) thresholds for 14 adult cochlear implant recipients with hearing preservation in the implanted ear. Localization to broadband noise was assessed in an anechoic chamber with a 33-loudspeaker array extending from -90 to +90°. Three listening conditions were tested including bilateral hearing aids, bimodal (implant + contralateral hearing aid) and best aided (implant + bilateral hearing aids). ITD thresholds were assessed, under headphones, for low-frequency stimuli including a 250-Hz tone and bandpass noise (100-900 Hz). Localization, in overall rms error, was significantly poorer in the bimodal condition (mean: 60.2°) as compared to both bilateral hearing aids (mean: 46.1°) and the best-aided condition (mean: 43.4°). ITD thresholds were assessed for the same 14 adult implant recipients as well as 5 normal-hearing adults. ITD thresholds were highly variable across the implant recipients ranging from the range of normal to ITDs not present in real-world listening environments (range: 43 to over 1600 μs). ITD thresholds were significantly correlated with localization, the degree of interaural asymmetry in low-frequency hearing, and the degree of hearing preservation related benefit in the speech reception threshold (SRT). These data suggest that implant recipients with hearing preservation in the implanted ear have access to binaural cues and that the sensitivity to ITDs is significantly correlated with localization and degree of preserved hearing in the implanted ear. Copyright © 2014. Published by Elsevier B.V.

Probing the Xenopus laevis inner ear transcriptome for biological function

PubMed Central

2012-01-01

Background The senses of hearing and balance depend upon mechanoreception, a process that originates in the inner ear and shares features across species. Amphibians have been widely used for physiological studies of mechanotransduction by sensory hair cells. In contrast, much less is known of the genetic basis of auditory and vestibular function in this class of animals. Among amphibians, the genus Xenopus is a well-characterized genetic and developmental model that offers unique opportunities for inner ear research because of the amphibian capacity for tissue and organ regeneration. For these reasons, we implemented a functional genomics approach as a means to undertake a large-scale analysis of the Xenopus laevis inner ear transcriptome through microarray analysis. Results Microarray analysis uncovered genes within the X. laevis inner ear transcriptome associated with inner ear function and impairment in other organisms, thereby supporting the inclusion of Xenopus in cross-species genetic studies of the inner ear. The use of gene categories (inner ear tissue; deafness; ion channels; ion transporters; transcription factors) facilitated the assignment of functional significance to probe set identifiers. We enhanced the biological relevance of our microarray data by using a variety of curation approaches to increase the annotation of the Affymetrix GeneChip® Xenopus laevis Genome array. In addition, annotation analysis revealed the prevalence of inner ear transcripts represented by probe set identifiers that lack functional characterization. Conclusions We identified an abundance of targets for genetic analysis of auditory and vestibular function. The orthologues to human genes with known inner ear function and the highly expressed transcripts that lack annotation are particularly interesting candidates for future analyses. We used informatics approaches to impart biologically relevant information to the Xenopus inner ear transcriptome, thereby addressing the

Auditory Brainstem Circuits That Mediate the Middle Ear Muscle Reflex

PubMed Central

Mukerji, Sudeep; Windsor, Alanna Marie; Lee, Daniel J.

2010-01-01

The middle ear muscle (MEM) reflex is one of two major descending systems to the auditory periphery. There are two middle ear muscles (MEMs): the stapedius and the tensor tympani. In man, the stapedius contracts in response to intense low frequency acoustic stimuli, exerting forces perpendicular to the stapes superstructure, increasing middle ear impedance and attenuating the intensity of sound energy reaching the inner ear (cochlea). The tensor tympani is believed to contract in response to self-generated noise (chewing, swallowing) and nonauditory stimuli. The MEM reflex pathways begin with sound presented to the ear. Transduction of sound occurs in the cochlea, resulting in an action potential that is transmitted along the auditory nerve to the cochlear nucleus in the brainstem (the first relay station for all ascending sound information originating in the ear). Unknown interneurons in the ventral cochlear nucleus project either directly or indirectly to MEM motoneurons located elsewhere in the brainstem. Motoneurons provide efferent innervation to the MEMs. Although the ascending and descending limbs of these reflex pathways have been well characterized, the identity of the reflex interneurons is not known, as are the source of modulatory inputs to these pathways. The aim of this article is to (a) provide an overview of MEM reflex anatomy and physiology, (b) present new data on MEM reflex anatomy and physiology from our laboratory and others, and (c) describe the clinical implications of our research. PMID:20870664

Microanatomy of the cochlear hook

NASA Astrophysics Data System (ADS)

Kwan, Changyow Claire; Tan, Xiaodong; Stock, Stuart R.; Soriano, Carmen; Xiao, Xianghui; Richter, Claus-Peter

2017-09-01

Communication among humans occurs through coding and decoding of acoustic information. The inner ear or cochlea acts as a frequency analyzer and divides the acoustic signal into small frequency bands, which are processed at different sites along the cochlea. The mechano-electrical conversion is accomplished by the soft tissue structures in the cochlea. While the anatomy for most of the cochlea has been well described, a detailed description of the very high frequency and vulnerable cochlear hook region is missing. To study the cochlear hook, mice cochleae were imaged with synchrotron radiation and high-resolution reconstructions have been made from the tomographic scans. This is the first detailed description of the bony and soft tissues of the hook region of the mammalian cochlea.

Regeneration and replacement in the vertebrate inner ear.

PubMed

Matsui, Jonathan I; Parker, Mark A; Ryals, Brenda M; Cotanche, Douglas A

2005-10-01

Deafness affects more than 40 million people in the UK and the USA, and many more world-wide. The primary cause of hearing loss is damage to or death of the sensory receptor cells in the inner ear, the hair cells. Birds can readily regenerate their cochlear hair cells but the mammalian cochlea has shown no ability to regenerate after damage. Current research efforts are focusing on gene manipulation, gene therapy and stem cell transplantation for repairing or replacing damaged mammalian cochlear hair cells, which could lead to therapies for treating deafness in humans.

Optoacoustic induced vibrations within the inner ear.

PubMed

Zhang, K Y; Wenzel, G I; Balster, S; Lim, H H; Lubatschowski, H; Lenarz, T; Ertmer, W; Reuter, G

2009-12-07

An acoustic transient can be generated inside an absorbing tissue as a result of laser-tissue interaction after pulsed laser irradiation. Herein we report a novel application of this physical process, the optoacoustic wave generation in the inner ear and subsequently the induction of basilar membrane vibrations. These laser induced vibrations show a direct correlation to the laser energy and an indirect correlation to the distance from the irradiation focus. Through these characteristics they may be used, in a new generation of cochlear implants, to improve the frequency specific cochlear activation and consequently improve speech perception in hearing impaired patients with residual hearing.

An Investigation of Spatial Hearing in Children with Normal Hearing and with Cochlear Implants and the Impact of Executive Function

NASA Astrophysics Data System (ADS)

Misurelli, Sara M.

The ability to analyze an "auditory scene"---that is, to selectively attend to a target source while simultaneously segregating and ignoring distracting information---is one of the most important and complex skills utilized by normal hearing (NH) adults. The NH adult auditory system and brain work rather well to segregate auditory sources in adverse environments. However, for some children and individuals with hearing loss, selectively attending to one source in noisy environments can be extremely challenging. In a normal auditory system, information arriving at each ear is integrated, and thus these binaural cues aid in speech understanding in noise. A growing number of individuals who are deaf now receive cochlear implants (CIs), which supply hearing through electrical stimulation to the auditory nerve. In particular, bilateral cochlear implants (BICIs) are now becoming more prevalent, especially in children. However, because CI sound processing lacks both fine structure cues and coordination between stimulation at the two ears, binaural cues may either be absent or inconsistent. For children with NH and with BiCIs, this difficulty in segregating sources is of particular concern because their learning and development commonly occurs within the context of complex auditory environments. This dissertation intends to explore and understand the ability of children with NH and with BiCIs to function in everyday noisy environments. The goals of this work are to (1) Investigate source segregation abilities in children with NH and with BiCIs; (2) Examine the effect of target-interferer similarity and the benefits of source segregation for children with NH and with BiCIs; (3) Investigate measures of executive function that may predict performance in complex and realistic auditory tasks of source segregation for listeners with NH; and (4) Examine source segregation abilities in NH listeners, from school-age to adults.

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.

Utility of bilateral acoustic hearing in combination with electrical stimulation provided by the cochlear implant.

PubMed

Plant, Kerrie; Babic, Leanne

2016-01-01

The aim of the study was to quantify the benefit provided by having access to amplified acoustic hearing in the implanted ear for use in combination with contralateral acoustic hearing and the electrical stimulation provided by the cochlear implant. Measures of spatial and non-spatial hearing abilities were obtained to compare performance obtained with different configurations of acoustic hearing in combination with electrical stimulation. In the combined listening condition participants had access to bilateral acoustic hearing whereas the bimodal condition used acoustic hearing contralateral to the implanted ear only. Experience was provided with each of the listening conditions using a repeated-measures A-B-B-A experimental design. Sixteen post-linguistically hearing-impaired adults participated in the study. Group mean benefit was obtained with use of the combined mode on measures of speech recognition in coincident speech in noise, localization ability, subjective ratings of real-world benefit, and musical sound quality ratings. Access to bilateral acoustic hearing after cochlear implantation provides significant benefit on a range of functional measures.

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.

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.

Evidence for a Right-Ear Advantage in Newborn Hearing Screening Results.

PubMed

Ari-Even Roth, Daphne; Hildesheimer, Minka; Roziner, Ilan; Henkin, Yael

2016-12-06

The aim of the present study was to investigate the effect of ear asymmetry, order of testing, and gender on transient-evoked otoacoustic emission (TEOAE) pass rates and response levels in newborn hearing screening. The screening results of 879 newborns, of whom 387 (study group) passed screening successfully in only one ear in the first TEOAE screening, but passed screening successfully in both ears thereafter, and 492 (control group) who passed screening successfully in both ears in the first TEOAE, were retrospectively examined for pass rates and TEOAE characteristics. Results indicated a right-ear advantage, as manifested by significantly higher pass rates in the right ear (61% and 39% for right and left ears, respectively) in the study group, and in 1.75 dB greater TEOAE response amplitudes in the control group. The right-ear advantage was enhanced when the first tested ear was the right ear (76%). When the left ear was tested first, pass rates were comparable in both ears. The right-ear advantage in pass rates was similar in females versus males, but manifested in 1.5 dB higher response amplitudes in females compared with males, regardless of the tested ear and order of testing in both study and control groups. The study provides further evidence for the functional lateralization of the auditory system at the cochlear level already apparent soon after birth in both males and females. While order of testing plays a significant role in the asymmetry in pass rates, the innate right-ear advantage seems to be a more dominant contributor. © The Author(s) 2016.

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.

Cochlear Implant Electrode Effect on Sound Energy Transfer within the Cochlea during Acoustic Stimulation

PubMed Central

Greene, Nathaniel T.; Mattingly, Jameson K.; Jenkins, Herman A.; Tollin, Daniel J.; Easter, James R.; Cass, Stephen P.

2015-01-01

Hypothesis Cochlear implants (CI) designed for hearing preservation will not alter mechanical properties of the middle and inner ear as measured by intracochlear pressure (PIC) and stapes velocity (Vstap). Background CIs designed to provide combined electrical and acoustic stimulation (EAS) are now available. To maintain functional acoustic hearing, it is important to know if a CI electrode can alter middle or inner ear mechanics, as any alteration could contribute to elevated low-frequency thresholds in EAS patients. Methods Seven human cadaveric temporal bones were prepared, and pure-tone stimuli from 120Hz–10kHz were presented at a range of intensities up to 110 dB SPL. PIC in the scala vestibuli (PSV) and tympani (PST) were measured with fiber-optic pressure sensors concurrently with VStap using laser Doppler vibrometry. Five CI electrodes from two different manufacturers, with varying dimensions were inserted via a round window approach at six different depths (16–25 mm). Results The responses of PIC and VStap to acoustic stimulation were assessed as a function of stimulus frequency, normalized to SPL in the external auditory canal (EAC), in baseline and electrode inserted conditions. Responses measured with electrodes inserted were generally within ~5 dB of baseline, indicating little effect of cochlear implant electrode insertion on PIC and VStap. Overall, mean differences across conditions were small for all responses, and no substantial differences were consistently visible across electrode types. Conclusions Results suggest that the influence of a CI electrode on middle and inner ear mechanics is minimal, despite variation in electrode lengths and configurations. PMID:26333018

Effects of the intensity of masking noise on ear canal recorded low-frequency cochlear microphonic waveforms in normal hearing subjects.

PubMed

Zhang, Ming

2014-07-01

Compared to auditory brainstem responses (ABRs), cochlear microphonics (CMs) may be more appropriate to serve as a supplement to the test of otoacoustic emissions (OAEs). Researchers have shown that low-frequency CMs from the apical cochlea are measurable at the tympanic membrane using high-pass masking noise. Our objective is to study the effect of such noise at different intensities on low-frequency CMs recorded at the ear canal, which is not completely known. Six components were involved in this CM measurement including an ear canal electrode (1), a relatively long and low-frequency toneburst (2), and high-pass masking noise at different intensities (3). The rest components include statistical analysis based on multiple human subjects (4), curve modeling based on amplitudes of CM waveforms (CMWs) and noise intensity (5), and a technique based on electrocochleography (ECochG or ECoG) (6). Results show that low-frequency CMWs appeared clearly. The CMW amplitude decreased with an increase in noise level. It decreased first slowly, then faster, and finally slowly again. In conclusion, when masked with high-pass noise, the low-frequency CMs are measurable at the human ear canal. Such noise reduces the low-frequency CM amplitude. The reduction is noise-intensity dependent but not completely linear. The reduction may be caused by the excited basal cochlea which the low-frequency has to travel and pass through. Although not completely clear, six mechanisms related to such reduction are discussed. Copyright © 2014 Elsevier B.V. All rights reserved.

Effects of Stimulus Intensity on Low-Frequency Toneburst Cochlear Microphonic Waveforms.

PubMed

Zhang, Ming

2013-01-02

This study investigates changes in amplitude and delays in low-frequency toneburst cochlear microphonic (CM) waveforms recorded at the ear canal in response to different stimulus intensities. Ten volunteers aged 20-30 were recruited. Low-frequency CM waveforms at 500 Hz in response to a 14-ms toneburst were recorded from an ear canal electrode using electrocochleography techniques. The data was statistically analyzed in order to confirm whether the differences were significant in the effects of stimulus intensity on the amplitudes and delays of the low-frequency CM waveforms. Electromagnetic interference artifacts can jeopardize CM measurements but such artifacts can be avoided. The CM waveforms can be recorded at the ear canal in response to a toneburst which is longer than that used in ABR measurements. The CM waveforms thus recorded are robust, and the amplitude of CM waveforms is intensity-dependent. In contrast, the delay of CM waveforms is intensity-independent, which is different from neural responses as their delay or latency is intensity-dependent. These findings may be useful for development of the application of CM measurement as a supplementary approach to otoacoustic emission (OAE) measurement in the clinic which is severely affected by background acoustic noise. The development of the application in the assessment of low-frequency cochlear function may become possible if a further series of studies can verify the feasibility, but it is not meant to be a substitute for audiometry or OAE measurements. The measurement of detection threshold of CM waveform responses using growth function approach may become possible in the clinic. The intensity-independent nature of CMs with regards to delay measurements may also become an impacting factor for differential diagnoses and for designing new research studies.

The role of intracochlear drug delivery devices in the management of inner ear disease.

PubMed

Ayoob, Andrew M; Borenstein, Jeffrey T

2015-03-01

Diseases of the inner ear include those of the auditory and vestibular systems, and frequently result in disabling hearing loss or vertigo. Despite a rapidly expanding pipeline of potential cochlear therapeutics, the inner ear remains a challenging organ for targeted drug delivery, and new technologies are required to deliver these therapies in a safe and efficacious manner. In addition to traditional approaches for direct inner ear drug delivery, novel microfluidics-based systems are under development, promising improved control over pharmacokinetics over longer periods of delivery, ultimately with application towards hair cell regeneration in humans. Advances in the development of intracochlear drug delivery systems are reviewed, including passive systems, active microfluidic technologies and cochlear prosthesis-mediated delivery. This article provides a description of novel delivery systems and their potential future clinical applications in treating inner ear disease. Recent progresses in microfluidics and miniaturization technologies are enabling the development of wearable and ultimately implantable drug delivery microsystems. Progress in this field is being spurred by the convergence of advances in molecular biology, microfluidic flow control systems and models for drug transport in the inner ear. These advances will herald a new generation of devices, with near-term applications in preclinical models, and ultimately with human clinical use for a range of diseases of the inner ear.

Probing cochlear tuning and tonotopy in the tiger using otoacoustic emissions.

PubMed

Bergevin, Christopher; Walsh, Edward J; McGee, JoAnn; Shera, Christopher A

2012-08-01

Otoacoustic emissions (sound emitted from the ear) allow cochlear function to be probed noninvasively. The emissions evoked by pure tones, known as stimulus-frequency emissions (SFOAEs), have been shown to provide reliable estimates of peripheral frequency tuning in a variety of mammalian and non-mammalian species. Here, we apply the same methodology to explore peripheral auditory function in the largest member of the cat family, the tiger (Panthera tigris). We measured SFOAEs in 9 unique ears of 5 anesthetized tigers. The tigers, housed at the Henry Doorly Zoo (Omaha, NE), were of both sexes and ranged in age from 3 to 10 years. SFOAE phase-gradient delays are significantly longer in tigers--by approximately a factor of two above 2 kHz and even more at lower frequencies--than in domestic cats (Felis catus), a species commonly used in auditory studies. Based on correlations between tuning and delay established in other species, our results imply that cochlear tuning in the tiger is significantly sharper than in domestic cat and appears comparable to that of humans. Furthermore, the SFOAE data indicate that tigers have a larger tonotopic mapping constant (mm/octave) than domestic cats. A larger mapping constant in tiger is consistent both with auditory brainstem response thresholds (that suggest a lower upper frequency limit of hearing for the tiger than domestic cat) and with measurements of basilar-membrane length (about 1.5 times longer in the tiger than domestic cat).

Cochlear implantation with hearing preservation yields significant benefit for speech recognition in complex listening environments

PubMed Central

Gifford, René H.; Dorman, Michael F.; Skarzynski, Henryk; Lorens, Artur; Polak, Marek; Driscoll, Colin L. W.; Roland, Peter; Buchman, Craig A.

2012-01-01

Objective The aim of this study was to assess the benefit of having preserved acoustic hearing in the implanted ear for speech recognition in complex listening environments. Design The current study included a within subjects, repeated-measures design including 21 English speaking and 17 Polish speaking cochlear implant recipients with preserved acoustic hearing in the implanted ear. The patients were implanted with electrodes that varied in insertion depth from 10 to 31 mm. Mean preoperative low-frequency thresholds (average of 125, 250 and 500 Hz) in the implanted ear were 39.3 and 23.4 dB HL for the English- and Polish-speaking participants, respectively. In one condition, speech perception was assessed in an 8-loudspeaker environment in which the speech signals were presented from one loudspeaker and restaurant noise was presented from all loudspeakers. In another condition, the signals were presented in a simulation of a reverberant environment with a reverberation time of 0.6 sec. The response measures included speech reception thresholds (SRTs) and percent correct sentence understanding for two test conditions: cochlear implant (CI) plus low-frequency hearing in the contralateral ear (bimodal condition) and CI plus low-frequency hearing in both ears (best aided condition). A subset of 6 English-speaking listeners were also assessed on measures of interaural time difference (ITD) thresholds for a 250-Hz signal. Results Small, but significant, improvements in performance (1.7 – 2.1 dB and 6 – 10 percentage points) were found for the best-aided condition vs. the bimodal condition. Postoperative thresholds in the implanted ear were correlated with the degree of EAS benefit for speech recognition in diffuse noise. There was no reliable relationship among measures of audiometric threshold in the implanted ear nor elevation in threshold following surgery and improvement in speech understanding in reverberation. There was a significant correlation between ITD

[The effect of OSAHS on middle ear and inner ear vestibule function advances].

PubMed

Li, K L; Li, J R

2016-05-20

Obstructive sleep apnea hypopnea syndrome(OSAHS) as a common frequentlyoccurring disease, it can cause repeated episodes of hypoxaemia and hypercapnia during sleep. With long period of hypoxaemia, obvious pathological changes and dysfunction emerged in heart，brain and lung then all kinds of clinical symptoms appear. Because of the middle ear and inner ear themselves anatomical characteristics and blood supply of regulating mechanism， they often has been damaged before the other important organ damage. As scholars have indepth study of the auditory system complications in patients with OSAHS, various influence of OSAHS on the middle ear，inner ear also gradually be known.This paper will review the effect of OSAHS on middle ear， inner ear and vestibule function, hope to have some application value for clinical work. Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.

The Role of Zic Genes in Inner Ear Development in the Mouse: Exploring Mutant Mouse Phenotypes

PubMed Central

Chervenak, Andrew P.; Bank, Lisa M.; Thomsen, Nicole; Glanville-Jones, Hannah C; Skibo, Jonathan; Millen, Kathleen J.; Arkell, Ruth M.; Barald, Kate F.

2014-01-01

Background Murine Zic genes (Zic1-5) are expressed in the dorsal hindbrain and in periotic mesenchyme (POM) adjacent to the developing inner ear. Zic genes are involved in developmental signaling pathways in many organ systems, including the ear, although their exact roles haven't been fully elucidated. This report examines the role of Zic1, Zic2, and Zic4 during inner ear development in mouse mutants in which these Zic genes are affected Results Zic1/Zic4 double mutants don't exhibit any apparent defects in inner ear morphology. By contrast, inner ears from Zic2kd/kd and Zic2Ku/Ku mutants have severe but variable morphological defects in endolymphatic duct/sac and semicircular canal formation and in cochlear extension in the inner ear. Analysis of otocyst patterning in the Zic2Ku/Ku mutants by in situ hybridization showed changes in the expression patterns of Gbx2 and Pax2. Conclusions The experiments provide the first genetic evidence that the Zic genes are required for morphogenesis of the inner ear. Zic2 loss-of-function doesn't prevent initial otocyst patterning but leads to molecular abnormalities concomitant with morphogenesis of the endolymphatic duct. Functional hearing deficits often accompany inner ear dysmorphologies, making Zic2 a novel candidate gene for ongoing efforts to identify the genetic basis of human hearing loss. PMID:25178196

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.

New HRCT-based measurement of the human outer ear canal as a basis for acoustical methods.

PubMed

Grewe, Johanna; Thiele, Cornelia; Mojallal, Hamidreza; Raab, Peter; Sankowsky-Rothe, Tobias; Lenarz, Thomas; Blau, Matthias; Teschner, Magnus

2013-06-01

As the form and size of the external auditory canal determine its transmitting function and hence the sound pressure in front of the eardrum, it is important to understand its anatomy in order to develop, optimize, and compare acoustical methods. High-resolution computed tomography (HRCT) data were measured retrospectively for 100 patients who had received a cochlear implant. In order to visualize the anatomy of the auditory canal, its length, radius, and the angle at which it runs were determined for the patients’ right and left ears. The canal’s volume was calculated, and a radius function was created. The determined length of the auditory canal averaged 23.6 mm for the right ear and 23.5 mm for the left ear. The calculated auditory canal volume (Vtotal) was 0.7 ml for the right ear and 0.69 ml for the left ear. The auditory canal was found to be significantly longer in men than in women, and the volume greater. The values obtained can be employed to develop a method that represents the shape of the auditory canal as accurately as possible to allow the best possible outcomes for hearing aid fitting.

Gerbil middle-ear sound transmission from 100 Hz to 60 kHz1

PubMed Central

Ravicz, Michael E.; Cooper, Nigel P.; Rosowski, John J.

2008-01-01

Middle-ear sound transmission was evaluated as the middle-ear transfer admittance HMY (the ratio of stapes velocity to ear-canal sound pressure near the umbo) in gerbils during closed-field sound stimulation at frequencies from 0.1 to 60 kHz, a range that spans the gerbil’s audiometric range. Similar measurements were performed in two laboratories. The HMY magnitude (a) increased with frequency below 1 kHz, (b) remained approximately constant with frequency from 5 to 35 kHz, and (c) decreased substantially from 35 to 50 kHz. The HMY phase increased linearly with frequency from 5 to 35 kHz, consistent with a 20–29 μs delay, and flattened at higher frequencies. Measurements from different directions showed that stapes motion is predominantly pistonlike except in a narrow frequency band around 10 kHz. Cochlear input impedance was estimated from HMY and previously-measured cochlear sound pressure. Results do not support the idea that the middle ear is a lossless matched transmission line. Results support the ideas that (1) middle-ear transmission is consistent with a mechanical transmission line or multiresonant network between 5 and 35 kHz and decreases at higher frequencies, (2) stapes motion is pistonlike over most of the gerbil auditory range, and (3) middle-ear transmission properties are a determinant of the audiogram. PMID:18646983

[Facial nerve monitoring during middle ear surgery: Results of a French survey].

PubMed

Mazzaschi, O; Juvanon, J-M; Mondain, M; Lavieile, J-P; Ayache, D

2014-01-01

Facial nerve injury is a rare complication of middle ear surgery. To date there is no widely accepted consensus on the use of intraoperative facial nerve monitoring during middle ear surgery, whereas its use has been proved as a valuable adjunct in neurotologic surgery. The purpose of our study was to identify introperative facial nerve monitoring practice patterns in France for middle ear surgery. A 19-item survey has been made up by three experienced otologists under the auspices of the French Otology and Neurotology Association. With the support of the French Society of Otolaryngology--Head and Neck Surgery, the survey was electronically sent by email to 1249 practicing ENT with a valid email address. Answers were analyzed two months later. Among 1249 email sent, 299 were opened (24%) and 83 answers were collected (6,6%). Of the respondents, 66% had access to intraoperative facial nerve monitoring. Otolaryngologists involved in academic setting were influenced by their teaching duty in 27%. Intraoperative facial nerve monitoring should not be required for stapes surgery, ossiculoplasty, myringoplasty for, respectively, 92%, 93 % and 98% of the respondents. In cochlear implantation, 78% of ear surgeons used facial nerve monitoring. Answers were more controversial for chronic ear surgery, ear atresia and middle ear implant. Revision surgery and CT scan can influence answers. Despite a low response rate, results of this national survey revealed interesting findings. For most of the respondents, intraoperative facial nerve monitoring was not indicated in stapes surgery, myringoplasty and ossiculoplasty. The use of intraoperative facial nerve monitoring for cochlear implantation was supported by the majority of respondents. Variations in response rate were more significant for chronic ear surgery, including middle ear cholesteatoma, and for ear atresia surgery.

Inner ear changes in mucopolysaccharidosis type I/Hurler syndrome.

PubMed

Kariya, Shin; Schachern, Patricia A; Nishizaki, Kazunori; Paparella, Michael M; Cureoglu, Sebahattin

2012-10-01

Mucopolysaccharidosis type I/Hurler syndrome is an autosomal recessive disease caused by a deficiency of α-L-iduronidase activity. Recurrent middle ear infections and hearing loss are common complications in Hurler syndrome. Although sensorineural and conductive components occur, the mechanism of sensorineural hearing loss has not been determined. The purpose of this study is to evaluate the quantitative inner ear histopathology of the temporal bones of patients with Hurler syndrome. Eleven temporal bones from 6 patients with Hurler syndrome were examined. Age-matched healthy control samples consisted of 14 temporal bones from 7 cases. Temporal bones were serially sectioned in the horizontal plane and stained with hematoxylin and eosin. The number of spiral ganglion cells, loss of cochlear hair cells, area of stria vascularis, and cell density of spiral ligament were evaluated using light microscopy. There was no significant difference between Hurler syndrome and healthy controls in the number of spiral ganglion cells, area of stria vascularis, or cell density of spiral ligament. The number of cochlear hair cells in Hurler syndrome was significantly decreased compared with healthy controls. Auditory pathophysiology in the central nerve system in Hurler syndrome remains unknown; however, decreased cochlear hair cells may be one of the important factors for the sensorineural component of hearing loss.

Resistance of Gerbil Auditory Function to Reversible Decrease in Cochlear Blood Flow.

PubMed

El Afia, Fahd; Giraudet, Fabrice; Gilain, Laurent; Mom, Thierry; Avan, Paul

2017-01-01

The objective was to design in gerbils a model of reversible decrease in cochlear blood flow (CBF) and analyze its influence on cochlear function. In Mongolian gerbils injected with ferromagnetic microbeads, a magnet placed near the porus acusticus allowed CBF to be manipulated. The cochlear microphonic potential (CM) from the basal cochlea was monitored by a round-window electrode. In 13 of the 20 successfully injected gerbils, stable CBF reduction was obtained for 11.5 min on average. The CM was affected only when CBF fell to less than 60% of its baseline, yet remained >40% of its initial level in about 2/3 of such cases. After CBF restoration, CM recovery was fast and usually complete. Reduced CM came with a 35- to 45-dB threshold elevation of neural responses determined by compound action potentials. This method allowing reversible changes of CBF confirms the robustness of cochlear function to decreased CBF. It can be used to study whether a hypovascularized cochlea is abnormally sensitive to stress. © 2017 S. Karger AG, Basel.

[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.

Transcanal endoscopic ear surgery for perilymphatic fistula after electric acoustic stimulation.

PubMed

Omichi, Ryotaro; Kariya, Shin; Maeda, Yukihide; Nishizaki, Kazunori

2018-06-01

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant. Copyright © 2017 Elsevier B.V. All rights reserved.

Cochlear implantation in Waardenburg syndrome: The Indian scenario.

PubMed

Deka, Ramesh Chandra; Sikka, Kapil; Chaturvedy, Gaurav; Singh, Chirom Amit; Venkat Karthikeyan, C; Kumar, Rakesh; Agarwal, Shivani

2010-10-01

Children with Waardenburg syndrome (WS) exhibiting normal inner ear anatomy, like those included in our cohort, derive significant benefit from cochlear implantation and results are comparable to those reported for the general population of implanted children. The patient population of WS accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with WS who have undergone cochlear implantation. On retrospective chart review, there were four cases with WS who underwent cochlear implantation. These cases were assessed for age at implantation, clinical and radiological features, operative and perioperative course, and performance outcomes. Auditory perception and speech production ability were evaluated using categories of auditory performance (CAP), meaningful auditory integration scales (MAIS), and speech intelligibility rating (SIR) during the follow-up period. In this group of children with WS, with a minimum follow-up of 12 months, the CAP score ranged from 3 to 5, MAIS from 25 to 30, and SIR was 3. These scores are comparable with those of other cochlear implantees.

TNF-α inhibition using etanercept prevents noise-induced hearing loss by improvement of cochlear blood flow in vivo.

PubMed

Arpornchayanon, Warangkana; Canis, Martin; Ihler, Friedrich; Settevendemie, Claudia; Strieth, Sebastian

2013-08-01

Exposure to loud noise can impair cochlear microcirculation and cause noise-induced hearing loss (NIHL). TNF-α signaling has been shown to be activated in NIHL and to control spiral modiolar artery vasoconstriction that regulates cochlear microcirculation. It was the aim of this experimental study to analyse the effects of the TNF-α inhibitor etanercept on cochlear microcirculation and hearing threshold shift in NIHL in vivo. After assessment of normacusis using ABR, loud noise (106 dB SPL, 30 minutes) was applied on both ears in guinea pigs. Etanercept was administered systemically after loud noise exposure while control animals received a saline solution. In vivo fluorescence microscopy of strial capillaries was performed after surgical exposure of the cochlea for microcirculatory analysis. ABR measurements were derived from the contralateral ear. Guinea pigs (n = 6, per group). Compared to controls, cochlear blood flow in strial capillary segments was significantly increased in etanercept-treated animals. Additionally, hearing threshold was preserved in animals receiving the TNF-α inhibitor in contrast to a significant threshold raising in controls. TNF-α inhibition using etanercept improves cochlear microcirculation and protects hearing levels after loud noise exposure and appears as a promising treatment strategy for human NIHL.

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.

PVDF-Based Piezoelectric Microphone for Sound Detection Inside the Cochlea: Toward Totally Implantable Cochlear Implants.

PubMed

Park, Steve; Guan, Xiying; Kim, Youngwan; Creighton, Francis Pete X; Wei, Eric; Kymissis, Ioannis John; Nakajima, Hideko Heidi; Olson, Elizabeth S

2018-01-01

We report the fabrication and characterization of a prototype polyvinylidene fluoride polymer-based implantable microphone for detecting sound inside gerbil and human cochleae. With the current configuration and amplification, the signal-to-noise ratios were sufficiently high for normally occurring sound pressures and frequencies (ear canal pressures >50-60 dB SPL and 0.1-10 kHz), though 10 to 20 dB poorer than for some hearing aid microphones. These results demonstrate the feasibility of the prototype devices as implantable microphones for the development of totally implantable cochlear implants. For patients, this will improve sound reception by utilizing the outer ear and will improve the use of cochlear implants.

PVDF-Based Piezoelectric Microphone for Sound Detection Inside the Cochlea: Toward Totally Implantable Cochlear Implants

PubMed Central

Guan, Xiying; Kim, Youngwan; Creighton, Francis (Pete) X.; Wei, Eric; Kymissis, Ioannis(John); Nakajima, Hideko Heidi; Olson, Elizabeth S.

2018-01-01

We report the fabrication and characterization of a prototype polyvinylidene fluoride polymer-based implantable microphone for detecting sound inside gerbil and human cochleae. With the current configuration and amplification, the signal-to-noise ratios were sufficiently high for normally occurring sound pressures and frequencies (ear canal pressures >50–60 dB SPL and 0.1–10 kHz), though 10 to 20 dB poorer than for some hearing aid microphones. These results demonstrate the feasibility of the prototype devices as implantable microphones for the development of totally implantable cochlear implants. For patients, this will improve sound reception by utilizing the outer ear and will improve the use of cochlear implants. PMID:29732950

Morphological variation among the inner ears of extinct and extant baleen whales (Cetacea: Mysticeti).

PubMed

Ekdale, Eric G

2016-12-01

Living mysticetes (baleen whales) and odontocetes (toothed whales) differ significantly in auditory function in that toothed whales are sensitive to high-frequency and ultrasonic sound vibrations and mysticetes to low-frequency and infrasonic noises. Our knowledge of the evolution and phylogeny of cetaceans, and mysticetes in particular, is at a point at which we can explore morphological and physiological changes within the baleen whale inner ear. Traditional comparative anatomy and landmark-based 3D-geometric morphometric analyses were performed to investigate the anatomical diversity of the inner ears of extinct and extant mysticetes in comparison with other cetaceans. Principal component analyses (PCAs) show that the cochlear morphospace of odontocetes is tangential to that of mysticetes, but odontocetes are completely separated from mysticetes when semicircular canal landmarks are combined with the cochlear data. The cochlea of the archaeocete Zygorhiza kochii and early diverging extinct mysticetes plot within the morphospace of crown mysticetes, suggesting that mysticetes possess ancestral cochlear morphology and physiology. The PCA results indicate variation among mysticete species, although no major patterns are recovered to suggest separate hearing or locomotor regimes. Phylogenetic signal was detected for several clades, including crown Cetacea and crown Mysticeti, with the most clades expressing phylogenetic signal in the semicircular canal dataset. Brownian motion could not be excluded as an explanation for the signal, except for analyses combining cochlea and semicircular canal datasets for Balaenopteridae. J. Morphol. 277:1599-1615, 2016. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Stem Cell Therapy for the Inner Ear

PubMed Central

Okano, Takayuki

2012-01-01

In vertebrates, perception of sound, motion, and balance is mediated through mechanosensory hair cells located within the inner ear. In mammals, hair cells are only generated during a short period of embryonic development. As a result, loss of hair cells as a consequence of injury, disease, or genetic mutation, leads to permanent sensory deficits. At present, cochlear implantation is the only option for profound hearing loss. However, outcomes are still variable and even the best implant cannot provide the acuity of a biological ear. The recent emergence of stem cell technology has the potential to open new approaches for hair cell regeneration. The goal of this review is to summarize the current state of inner ear stem cell research from a viewpoint of its clinical application for inner ear disorders to illustrate how complementary studies have the potential to promote and refine stem cell therapies for inner ear diseases. The review initially discusses our current understanding of the genetic pathways that regulate hair cell formation from inner ear progenitors during normal development. Subsequent sections discuss the possible use of endogenous inner ear stem cells to induce repair as well as the initial studies aimed at transplanting stem cells into the ear. PMID:22514095

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.

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.

Bilateral Cochlear Implantation in Children: Experiences and Considerations

ERIC Educational Resources Information Center

Bohnert, Andrea; Spitzlei, Vera; Lippert, Karl L.; Keilmann, Annerose

2006-01-01

Between 2000 and 2006, the University Clinic for Ear Nose and Throat and Communication Disorders in Mainz, Germany, performed 41 bilateral cochlear implantations in children. This article addresses some of the factors to be considered in a decision to bilaterally implant a child, including the age of the child at the first implant, the length of…

Chirp-Evoked Otoacoustic Emissions and Middle Ear Absorbance for Monitoring Ototoxicity in Cystic Fibrosis Patients.

PubMed

Garinis, Angela C; Keefe, Douglas H; Hunter, Lisa L; Fitzpatrick, Denis F; Putterman, Daniel B; McMillan, Garnett P; Gold, Jeffrey A; Feeney, M Patrick

The goal of this study was to investigate the use of transient-evoked otoacoustic emissions (TEOAEs) and middle ear absorbance measurements to monitor auditory function in patients with cystic fibrosis (CF) receiving ototoxic medications. TEOAEs were elicited with a chirp stimulus using an extended bandwidth (0.71 to 8 kHz) to measure cochlear function at higher frequencies than traditional TEOAEs. Absorbance over a wide bandwidth (0.25 to 8 kHz) provides information on middle ear function. The combination of these time-efficient measurements has the potential to identify early signs of ototoxic hearing loss. A longitudinal study design was used to monitor the hearing of 91 patients with CF (median age = 25 years; age range = 15 to 63 years) who received known ototoxic medications (e.g., tobramycin) to prevent or treat bacterial lung infections. Results were compared to 37 normally hearing young adults (median age = 32.5 years; age range = 18 to 65 years) without a history of CF or similar treatments. Clinical testing included 226-Hz tympanometry, pure-tone air-conduction threshold testing from 0.25 to 16 kHz and bone conduction from 0.25 to 4 kHz. Experimental testing included wideband absorbance at ambient and tympanometric peak pressure and TEOAEs in three stimulus conditions: at ambient pressure and at tympanometric peak pressure using a chirp stimulus with constant incident pressure level across frequency and at ambient pressure using a chirp stimulus with constant absorbed sound power across frequency. At the initial visit, behavioral audiometric results indicated that 76 of the 157 ears (48%) from patients with CF had normal hearing, whereas 81 of these ears (52%) had sensorineural hearing loss for at least one frequency. Seven ears from four patients had a confirmed behavioral change in hearing threshold for ≥3 visits during study participation. Receiver operating characteristic curve analyses demonstrated that all three TEOAE conditions were

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.

Differential Effects of AAV.BDNF and AAV.Ntf3 in the Deafened Adult Guinea Pig Ear

PubMed Central

Budenz, Cameron L.; Wong, Hiu Tung; Swiderski, Donald L.; Shibata, Seiji B.; Pfingst, Bryan E.; Raphael, Yehoash

2015-01-01

Cochlear hair cell loss results in secondary regression of peripheral auditory fibers (PAFs) and loss of spiral ganglion neurons (SGNs). The performance of cochlear implants (CI) in rehabilitating hearing depends on survival of SGNs. Here we compare the effects of adeno-associated virus vectors with neurotrophin gene inserts, AAV.BDNF and AAV.Ntf3, on guinea pig ears deafened systemically (kanamycin and furosemide) or locally (neomycin). AAV.BDNF or AAV.Ntf3 was delivered to the guinea pig cochlea one week following deafening and ears were assessed morphologically 3 months later. At that time, neurotrophins levels were not significantly elevated in the cochlear fluids, even though in vitro and shorter term in vivo experiments demonstrate robust elevation of neurotrophins with these viral vectors. Nevertheless, animals receiving these vectors exhibited considerable re-growth of PAFs in the basilar membrane area. In systemically deafened animals there was a negative correlation between the presence of differentiated supporting cells and PAFs, suggesting that supporting cells influence the outcome of neurotrophin over-expression aimed at enhancing the cochlear neural substrate. Counts of SGN in Rosenthal's canal indicate that BDNF was more effective than NT-3 in preserving SGNs. The results demonstrate that a transient elevation in neurotrophin levels can sustain the cochlear neural substrate in the long term. PMID:25726967

[European Portuguese EARS test battery adaptation].

PubMed

Alves, Marisa; Ramos, Daniela; Oliveira, Graça; Alves, Helena; Anderson, Ilona; Magalhães, Isabel; Martins, Jorge H; Simões, Margarida; Ferreira, Raquel; Fonseca, Rita; Andrade, Susana; Silva, Luís; Ribeiro, Carlos; Ferreira, Pedro Lopes

2014-01-01

The use of adequate assessment tools in health care is crucial for the management of care. The lack of specific tools in Portugal for assessing the performance of children who use cochlear implants motivated the translation and adaptation of the EARS (Evaluation of Auditory Responses to Speech) test battery into European Portuguese. This test battery is today one of the most commonly used by (re)habilitation teams of deaf children who use cochlear implants worldwide. The goal to be achieved with the validation of EARS was to provide (re)habilitation teams an instrument that enables: (i) monitoring the progress of individual (re)habilitation, (ii) managing a (re)habilitation program according to objective results, comparable between different (re)habilitation teams, (iii) obtaining data that can be compared with the results of international teams, and (iv) improving engagement and motivation of the family and other professionals from local teams. For the test battery translation and adaptation process, the adopted procedures were the following: (i) translation of the English version into European Portuguese by a professional translator, (ii) revision of the translation performed by an expert panel, including doctors, speech-language pathologists and audiologists, (iii) adaptation of the test stimuli by the team's speechlanguage pathologist, and (iv) further review by the expert panel. For each of the tests that belong to the EARS battery, the introduced adaptations and adjustments are presented, combining the characteristics and objectives of the original tests with the linguistic and cultural specificities of the Portuguese population. The difficulties that have been encountered during the translation and adaptation process and the adopted solutions are discussed. Comparisons are made with other versions of the EARS battery. We defend that the translation and the adaptation process followed for the EARS test battery into European Portuguese was correctly conducted

Hearing Preservation Outcomes With a Mid-Scala Electrode in Cochlear Implantation.

PubMed

Hunter, Jacob B; Gifford, René H; Wanna, George B; Labadie, Robert F; Bennett, Marc L; Haynes, David S; Rivas, Alejandro

2016-03-01

To evaluate hearing preservation (HP) outcomes in adult cochlear implant recipients with a mid-scala electrode. Tertiary academic center. Adult patients implanted with a mid-scala electrode between May 2013 and July 2015. Cochlear implantation. Age, sex, surgical approach, residual hearing changes post cochlear implantation, HP rates using different published classifications, and speech perception scores. Fifty ears for 47 patients (mean age, 58.2 yr; range, 23-86) were implanted with the electrode. Recognizing that not all patients were true HP candidates and/or underwent generally accepted HP surgical techniques, 39 ears had preoperative low-frequency hearing (audiometric threshold ≤ 85dB HL at 250Hz), 24 preserved acoustic hearing postoperatively (75.0%). Patients who had preserved acoustic hearing were implanted via round window (N = 18), extended round window (N = 4), or via cochleostomy (N = 2) approaches. Mean threshold elevation for low-frequency pure-tone average (125, 250, and 500  Hz) was 20.2  dB after surgery. 43.8% of patients had aidable low-frequency hearing at activation, 30.0% at 6-months postoperatively, and 30.8% 1-year postopera tively. Using a formula outlined by Skarzynski and colleagues, at 6-months postoperatively, 15.0% of patients had complete HP, whereas 40.0% had partial HP. At 1-year, these percentages decreased to 0% and 38.5%, respectively. Age, type of approach, and perioperative steroid use were not correlated with HP outcomes at activation and 6-months postoperatively (p > 0.05). The mid-scala electrode evaluated allows preservation of low-frequency hearing in patients undergoing cochlear implantation at rates and degrees of preservation close to other reports in the cochlear implant literature.

Management of Children Using Cochlear Implants and Hearing Aids.

ERIC Educational Resources Information Center

Ching, Teresa Y. C.; Psarros, Colleen; Incerti, Paula; Hill, Mandy

2001-01-01

Four case studies identify six factors affecting successful use of a hearing aid with a cochlear implant: duration of hearing aid use prior to implantation, amount of residual hearing in the non-implanted ear, educational and listening demands, cosmetic issues, hearing aid rejection, and extended period of non-use of hearing aid. (Contains…

Labyrinthectomy with cochlear implantation.

PubMed

Zwolan, T A; Shepard, N T; Niparko, J K

1993-05-01

Numerous reports indicate that the cochlea remains responsive to electrical stimulation following labyrinthectomy. We report a case of a 47-year-old woman with a severe to profound sensorineural hearing loss from birth, who developed episodic vertigo with symptoms suggestive of delayed onset endolymphatic hydrops. Following 8 months of failed medical and vestibular rehabilitation management, a right-sided labyrinthectomy combined with cochlear implantation was performed without complication. Postoperatively the patient was free of vertigo. Attempts to activate the patient's device between 4 to 12 weeks after surgery were unsuccessful as stimulation of the electrodes resulted in discomfort. However, all 20 electrodes elicited comfortable hearing sensations 16 weeks postsurgery. One year after the successful activation, the patient demonstrated improved sound awareness and speech recognition with the implant when compared with preoperative performance with a hearing aid. This case study suggests that electrical detection thresholds with prosthetic stimulation may be unstable in the recently labyrinthectomized ear but supports and extends prior observations of preserved cochlear responsiveness after labyrinthectomy.

Comparative effects of glycerol and Urografin on cochlear blood flow and serum osmolarity.

PubMed

Noi, O; Makimoto, K

1998-09-01

Glycerol, an osmotic diuretic, has been used for the diagnosis and treatment of endolymphatic hydrops. Hearing improvements in hydropic ears are attributed to its dehydrating effect. In addition to this effect, glycerol also increases cochlear blood flow. Urografin, another hyperosmotic agent used for vasography, is similarly known to increase local blood flow. The present study compared these two hyperosmotic agents, glycerol and Urografin, in their effects on cochlear blood flow and serum osmolarity. Laser Doppler flowmetry on the lateral wall of the cochlea revealed that the increase in cochlear blood flow with a 30-min infusion (0.025 ml/min) of 76% Urografin continued for a longer time than with a 30-min infusion (0.025 ml/min) of 50% (v/v) glycerol. The significant increases appeared at 20 and 30 min after the infusion with the former; 10, 20, 30, 40, 50 and 60 min after the infusion with the latter. Intravenous infusion of these agents also caused elevation in serum osmolarity. This elevation was appreciably greater with Urografin infusion (maximal increase: about 30 mOsm on average) than with glycerol infusion (maximal increase: about 6 mOsm on average), and the former elevation appeared to be longer lasting than the latter. These differences were ascribed to differences between glycerol and Urografin with respect to the creation of an osmotic gradient across the capillary walls of cochlear blood vessels. Since glycerol penetrates the interstitial space and moves into inner ear fluids, the gradient may decline faster. It would be assumed that a higher concentration of the hyperosmotic agent in the capillary blood causes more vasodilatation and lowering of blood viscosity. Alternatively, direct action of these agents on the vascular wall may affect some biological processes, leading to vasodilatation in different degrees and durations with different agents. Hearing improvement with glycerol administration in hydropic ears was also discussed from the

Surgery of the ear and the lateral skull base: pitfalls and complications

PubMed Central

Schick, Bernhard; Dlugaiczyk, Julia

2013-01-01

Surgery of the ear and the lateral skull base is a fascinating, yet challenging field in otorhinolaryngology. A thorough knowledge of the associated complications and pitfalls is indispensable for the surgeon, not only to provide the best possible care to his patients, but also to further improve his surgical skills. Following a summary about general aspects in pre-, intra-and postoperative care of patients with disorders of the ear/lateral skull base, this article covers the most common pitfalls and complications in stapes surgery, cochlear implantation and surgery of vestibular schwannomas and jugulotympanal paragangliomas. Based on these exemplary procedures, basic “dos and don’ts” of skull base surgery are explained, which the reader can easily transfer to other disorders. Special emphasis is laid on functional aspects, such as hearing, balance and facial nerve function. Furthermore, the topics of infection, bleeding, skull base defects, quality of life and indication for revision surgery are discussed. An open communication about complications and pitfalls in ear/lateral skull base surgery among surgeons is a prerequisite for the further advancement of this fascinating field in ENT surgery. This article is meant to be a contribution to this process. PMID:24403973

In Vitro and In Vivo Evaluation of a Hydrogel Reservoir as a Continuous Drug Delivery System for Inner Ear Treatment

PubMed Central

Hessler, Roland; Stöver, Timo; Esser, Karl-Heinz; Möller, Martin; Lenarz, Thomas; Jolly, Claude; Groll, Jürgen; Scheper, Verena

2014-01-01

Fibrous tissue growth and loss of residual hearing after cochlear implantation can be reduced by application of the glucocorticoid dexamethasone-21-phosphate-disodium-salt (DEX). To date, sustained delivery of this agent to the cochlea using a number of pharmaceutical technologies has not been entirely successful. In this study we examine a novel way of continuous local drug application into the inner ear using a refillable hydrogel functionalized silicone reservoir. A PEG-based hydrogel made of reactive NCO-sP(EO-stat-PO) prepolymers was evaluated as a drug conveying and delivery system in vitro and in vivo. Encapsulating the free form hydrogel into a silicone tube with a small opening for the drug diffusion resulted in delayed drug release but unaffected diffusion of DEX through the gel compared to the free form hydrogel. Additionally, controlled DEX release over several weeks could be demonstrated using the hydrogel filled reservoir. Using a guinea-pig cochlear trauma model the reservoir delivery of DEX significantly protected residual hearing and reduced fibrosis. As well as being used as a device in its own right or in combination with cochlear implants, the hydrogel-filled reservoir represents a new drug delivery system that feasibly could be replenished with therapeutic agents to provide sustained treatment of the inner ear. PMID:25105670

Off the ear with no loss in speech understanding: comparing the RONDO and the OPUS 2 cochlear implant audio processors.

PubMed

Dazert, Stefan; Thomas, Jan Peter; Büchner, Andreas; Müller, Joachim; Hempel, John Martin; Löwenheim, Hubert; Mlynski, Robert

2017-03-01

The RONDO is a single-unit cochlear implant audio processor, which omits the need for a behind-the-ear (BTE) audio processor. The primary aim was to compare speech perception results in quiet and in noise with the RONDO and the OPUS 2, a BTE audio processor. Secondary aims were to determine subjects' self-assessed levels of sound quality and gather subjective feedback on RONDO use. All speech perception tests were performed with the RONDO and the OPUS 2 behind-the-ear audio processor at 3 test intervals. Subjects were required to use the RONDO between test intervals. Subjects were tested at upgrade from the OPUS 2 to the RONDO and at 1 and 6 months after upgrade. Speech perception was determined using the Freiburg Monosyllables in quiet test and the Oldenburg Sentence Test (OLSA) in noise. Subjective perception was determined using the Hearing Implant Sound Quality Index (HISQUI 19 ), and a RONDO device-specific questionnaire. 50 subjects participated in the study. Neither speech perception scores nor self-perceived sound quality scores were significantly different at any interval between the RONDO and the OPUS 2. Subjects reported high levels of satisfaction with the RONDO. The RONDO provides comparable speech perception to the OPUS 2 while providing users with high levels of satisfaction and comfort without increasing health risk. The RONDO is a suitable and safe alternative to traditional BTE audio processors.

The hearing benefit of cochlear implantation for individuals with unilateral hearing loss, but no tinnitus.

PubMed

Skarzynski, Henryk; Lorens, Artur; Kruszynska, Marika; Obrycka, Anita; Pastuszak, Dorota; Skarzynski, Piotr Henryk

2017-07-01

Cochlear implants improve the hearing abilities of individuals with unilateral hearing loss and no tinnitus. The benefit is no different from that seen in patients with unilateral hearing loss and incapacitating tinnitus. To evaluate hearing outcomes after cochlear implantation in individuals with unilateral hearing loss and no tinnitus and compare them to those obtained in a similar group who had incapacitating tinnitus. Six cases who did not experience tinnitus before operation and 15 subjects with pre-operative tinnitus were evaluated with a structured interview, a monosyllabic word test under difficult listening situations, a sound localization test, and an APHAB (abbreviated profile of hearing aid benefit) questionnaire. All subjects used their cochlear implant more than 8 hours a day, 7 days a week. In 'no tinnitus' patients, mean benefit of cochlear implantation was 19% for quiet speech, 15% for speech in noise (with the same signal-to-noise ratio in the implanted and non-implanted ear), and 16% for a more favourable signal-to-noise ratio at the implanted ear. Sound localization error improved by an average of 19°. The global score of APHAB improved by 16%. The benefits across all evaluations did not differ significantly between the 'no tinnitus' and 'tinnitus' groups.

Anisotropic yield function capable of predicting eight ears

NASA Astrophysics Data System (ADS)

Yoon, J. H.; Cazacu, O.

2011-08-01

Deep drawing of a cylindrical cup from a rolled sheet is one of the typical forming operations where the effect of this anisotropy is most evident. Indeed, it is well documented in the literature that the number of ears and the shape of the earing pattern correlate with the r-values profile. For the strongly textured aluminum alloy AA 5042 (Numisheet Benchmark 2011), the experimental r-value distribution has two minima between the rolling and transverse direction data provided for this show that the r-value along the transverse direction (TD) is five times larger than the value corresponding to the rolling direction. Therefore, it is expected that there are more that the earing profile has more than four ears. The main objective of this paper is to assess whether a new form of CPB06ex2 yield function (Plunkett et al. (2008)) tailored for metals with no tension-compression asymmetry is capable of predicting more than four ears for this material.

Moments of click-evoked otoacoustic emissions in human ears: group delay and spread, instantaneous frequency and bandwidth.

PubMed

Keefe, Douglas H

2012-11-01

A click-evoked otoacoustic emission (CEOAE) has group delay and spread as first- and second-order temporal moments varying over frequency, and instantaneous frequency and bandwidth as first- and second-order spectral moments varying over time. Energy-smoothed moments were calculated from a CEOAE database over 0.5-15 kHz bandwidth and 0.25-20 ms duration. Group delay and instantaneous frequency were calculated without phase unwrapping using a coherence synchrony measure that accurately classified ears with hearing loss. CEOAE moment measurements were repeatable in individual ears. Group delays were similar for CEOAEs and stimulus-frequency OAEs. Group spread is a frequency-specific measure of temporal spread in an emission, related to spatial spread across tonotopic generation sites along the cochlea. In normal ears, group delay and spread increased with frequency and decreased with level. A direct measure of cochlear tuning above 4 kHz was analyzed using instantaneous frequency and bandwidth. Synchronized spontaneous OAEs were present in most ears below 4 kHz, and confounded interpretation of moments. In ears with sensorineural hearing loss, group delay and spread varied with audiometric classification and amount of hearing loss; group delay differed between older males and females. CEOAE moments reveal clinically relevant information on cochlear tuning in ears with normal and impaired hearing.

A 3D-printed functioning anatomical human middle ear model.

PubMed

Kuru, Ismail; Maier, Hannes; Müller, Mathias; Lenarz, Thomas; Lueth, Tim C

2016-10-01

The middle ear is a sophisticated and complex structure with a variety of functions, yet a delicate organ prone to injuries due to various reasons. Both, understanding and reconstructing its functions has always been an important topic for researchers from medical and technical background. Currently, human temporal bones are generally used as model for tests, experiments and validation of the numerical results. However, fresh human preparations are not always easily accessible and their mechanical properties vary with time and between individuals. Therefore we have built an anatomically based and functional middle ear model to serve as a reproducible test environment. Our middle ear model was manufactured with the aid of 3D-printing technology. We have segmented the essential functional elements from micro computed tomography data (μCT) of a single temporal bone. The ossicles were 3D-printed by selective laser melting (SLM) and the soft tissues were casted with silicone rubber into 3D-printed molds. The ear canal, the tympanic cavity and the inner ear were artificially designed, but their design ensured the anatomically correct position of the tympanic membrane, ossicular ligaments and the oval window. For the determination of their auditory properties we have conducted two kinds of tests: measurement of the stapes footplate response to sound and tympanometry of the model. Our experiments regarding the sound transmission showed that the model has a similar behavior to a human middle ear. The transfer function has a resonance frequency at around 1 kHz, the stapes' response is almost constant for frequencies below the resonance and a roll-off is observed above the resonance. The tympanometry results show that the compliance of the middle ear model is similar to the compliance of a healthy human middle ear. We also present that we were able to manipulate the transmission behavior, so that healthy or pathological scenarios can be created. For this purpose we have

[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.

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.

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.

Customized acoustic transform functions and their accuracy at predicting real-ear hearing aid performance.

PubMed

Munro, K J; Hatton, N

2000-02-01

The purpose of the study was to evaluate the validity of predicting the real-ear aided response by adding customized acoustic transform functions to the performance of a hearing aid in a 2-cc coupler. The real-ear hearing aid response, the real-ear-to-coupler difference (RECD/HA2), and field to behind-the-ear microphone transfer functions were measured in both ears of 24 normally hearing subjects using probe-tube microphone equipment. The RECD/HA2 transform function was obtained using both insert earphones and with the hearing aid/ pressure comparison method. An RECD/HA2 transfer function was also obtained with a customized earmold, ER-3A foam tip, and an oto-admittance tip. Validity estimates were calculated as the difference between the derived and measured real-ear response. The derived response was generally within 5 dB of the measured real-ear response when it incorporated an RECD/HA2 transform function obtained with a customized earmold for the specific ear in question. Discrepancies increased when the RECD/HA2 transfer function was obtained from the same subject but the opposite ear. There were significant differences between the RECD/HA2 transform function obtained with customized and temporary earmolds. As a result, the derived response incorporating these transforms differed significantly from the measured real-ear response obtained with the customized earmold. The insert earphone and the hearing aid RECD/HA2 transfer function were equally valid. The derived response may be used as a substitute for in situ hearing aid response procedures when it incorporates acoustic transform functions obtained with a customized earmold from the specific ear in question.

Psychometric validity of the Cochlear Implant Function Index (CIFI): a quality of life assessment tool for adult cochlear implant users.

PubMed

Coelho, Daniel H; Hammerschlag, Paul E; Bat-Chava, Yael; Kohan, Darius

2009-06-01

The Cochlear Implant Function Index (CIFI) is created to assess adult cochlear implant (CI) auditory effectiveness in real world situations. Our objective is to evaluate the CIFI as a reliable psychometric tool to assess 1) reliance on visual assistance, 2) telephone use, 3) communication at work, 4) 'hearing' in noise, 5) in groups, and 6) in large room settings. Based upon Guttman scaling properties, the CIFI elicits implanted respondent's functional level with auditory independence from Level 1 (still requiring signing) to level 4 (without any help beyond CI). A blinded, retrospective questionnaire is anonymously answered by cochlear implant recipients. CI centers of tertiary care medical centers, CI support group, and an interactive web page of a hearing and speech center in a large metropolitan region. 245 respondents from a varied adult CI population implanted for one month to 19 years prior to answering the questionnaire. An assessment tool of CI function. A coefficient of reproducibility (CR) for the Guttman scale format equal or greater than 0.90, indicating good scalability. CR in the CIFI was above 0.90. Effective scalability and mean scores from 2.5 to 3.5 for the six areas examined (1.00-4.00) were achieved. The psychometric properties of this user friendly survey demonstrate consistently good scalability. Based on these findings, the CIFI provides a validated tool that can be used for systematic comparisons between groups of patients or for follow-up outcomes in patients who use cochlear implants. Further study is indicated to correlate CIFI scores with sound and speech perception scores. Copyright 2009 John Wiley & Sons, Ltd.

[Complications and pitfalls in surgery of the ear/lateral skull base].

PubMed

Schick, B; Dlugaiczyk, J

2013-04-01

Surgery of the ear and the lateral skull base is a fascinating, yet challenging field in otorhinolaryngology. A thorough knowledge of the associated complications and pitfalls is indispensable for the surgeon, not only to provide the best possible care to his patients, but also to further improve his surgical skills.Following a summary about general aspects in pre-, intra- and postoperative care of patients with disorders of the ear/lateral skull base, this article covers the most common pitfalls and complications in stapes surgery, cochlear implantation, surgery of vestibular schwannomas, and jugulotympanal paragangliomas. Based on these exemplary procedures, basic "do's and don'ts" of skull base surgery are explained, which the reader can easily transfer to other disorders. Special emphasis is laid on functional aspects, such as hearing, balance and facial nerve function. Furthermore, the topics of infection, bleeding, skull base defects, quality of life and indication for revision surgery are discussed.An open communication about complications and pitfalls in ear/lateral skull base surgery among surgeons is a prerequisite for the further advancement of this fascinating field in ENT surgery. This article is meant to be a contribution to this process. © Georg Thieme Verlag KG Stuttgart · New York.

Combined Effect of Fluid and Pressure on Middle Ear Function

PubMed Central

Dai, Chenkai; Wood, Mark W.; Gan, Rong Z.

2008-01-01

In our previous studies, the effects of effusion and pressure on sound transmission were investigated separately. The aim of this study is to investigate the combined effect of fluid and pressure on middle ear function. An otitis media with effusion model was created by injecting saline solution and air pressure simultaneously into the middle ear of human temporal bones. Tympanic membrane displacement in response to 90 dB SPL sound input was measured by a laser vibrometer and the compliance of the middle ear was measured by a tympanometer. The movement of the tympanic membrane at the umbo was reduced up to 17 dB by the combination of fluid and pressure in the middle ear over the auditory frequency range. The fluid and pressure effects on the umbo movement in the fluid-pressure combination are not additive. The combined effect of fluid and pressure on the umbo movement is different compared with that of only fluid or pressure change in the middle ear. Negative pressure in fluid-pressure combination had more effect on middle ear function than positive pressure. Tympanometry can detect the middle ear pressure of the fluid-pressure combination. This study provides quantitative information for analysis of the combined effect of fluid and pressure on tympanic membrane movement. PMID:18162348

Effects of obstructive sleep apnea surgery on middle ear function.

PubMed

Lin, Hsin-Ching; Friedman, Michael; Chang, Hsueh-Wen; Shao, Chi-Hsin; Pulver, Tanya M; Chen, Yung-Che

2011-04-01

To study the effect of Z-palatopharyngoplasty plus radiofrequency of the base of the tongue on middle ear function. A retrospective review of a prospective data set at a tertiary care center. University-affiliated medical center. The study population included 47 patients (42 men and 5 women; mean age, 40.8 years) who underwent Z-palatopharyngoplasty plus radiofrequency of the base of the tongue for obstructive sleep apnea/hypopnea syndrome. All patients had healthy eardrums and no previous history of chronic ear disease. Pure-tone audiometric and tympanometric assessments were performed preoperatively and at 3 days, 7 days, 1 month, and 3 months postoperatively. Levels of baseline and postoperative middle ear pressure were compared. Twelve patients (26%) reported otologic concerns, such as ear pressure and/or otalgia, within 1 week postoperatively. No permanent otologic discomfort occurred. A trend toward reduced middle ear pressure was noted in this study. The decrease in middle ear pressure became apparent on day 3. However, mean pressure changes were no longer significantly different than preoperative values by 1 week after surgery. We found that Z-palatopharyngoplasty plus radiofrequency of the base of the tongue for obstructive sleep apnea/hypopnea syndrome induces changes in middle ear function. However, the changes were temporary and not significant after 3 months of follow-up.

a Middle-Ear Reverse Transfer Function Computed from Vibration Measurements of Otoacoustic Emissions on the Ear Drum of the Guinea PIG

NASA Astrophysics Data System (ADS)

Dalhoff, Ernst; Turcanu, Diana; Gummer, Anthony W.

2009-02-01

Using distortion products measured as vibration of the umbo and as sound pressure in the ear canal of guinea pigs, we calculated the corresponding reverse transfer function. We compare the measurements with a middle-ear model taken from the literature and adapted to the guinea pig. A reasonable fit could be achieved. We conclude that the reverse transfer function will be useful to aid fitting a middle-ear model to measured transfer functions of human subjects.

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.

Comparisons of auditory brainstem response and sound level tolerance in tinnitus ears and non-tinnitus ears in unilateral tinnitus patients with normal audiograms.

PubMed

Shim, Hyun Joon; An, Yong-Hwi; Kim, Dong Hyun; Yoon, Ji Eun; Yoon, Ji Hyang

2017-01-01

Recently, "hidden hearing loss" with cochlear synaptopathy has been suggested as a potential pathophysiology of tinnitus in individuals with a normal hearing threshold. Several studies have demonstrated that subjects with tinnitus and normal audiograms show significantly reduced auditory brainstem response (ABR) wave I amplitudes compared with control subjects, but normal wave V amplitudes, suggesting increased central auditory gain. We aimed to reconfirm the "hidden hearing loss" theory through a within-subject comparison of wave I and wave V amplitudes and uncomfortable loudness level (UCL), which might be decreased with increased central gain, in tinnitus ears (TEs) and non-tinnitus ears (NTEs). Human subjects included 43 unilateral tinnitus patients (19 males, 24 females) with normal and symmetric hearing thresholds and 18 control subjects with normal audiograms. The amplitudes of wave I and V from the peak to the following trough were measured twice at 90 dB nHL and we separately assessed UCLs at 500 Hz and 3000 Hz pure tones in each TE and NTE. The within-subject comparison between TEs and NTEs showed no significant differences in wave I and wave V amplitude, or wave V/I ratio in both the male and female groups. Individual data revealed increased V/I amplitude ratios > mean + 2 SD in 3 TEs, but not in any control ears. We found no significant differences in UCL at 500 Hz or 3000 Hz between the TEs and NTEs, but the UCLs of both TEs and NTEs were lower than those of the control ears. Our ABR data do not represent meaningful evidence supporting the hypothesis of cochlear synaptopathy with increased central gain in tinnitus subjects with normal audiograms. However, reduced sound level tolerance in both TEs and NTEs might reflect increased central gain consequent on hidden synaptopathy that was subsequently balanced between the ears by lateral olivocochlear efferents.

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.

Comparison of the benefits of cochlear implantation versus contra-lateral routing of signal hearing aids in adult patients with single-sided deafness: study protocol for a prospective within-subject longitudinal trial.

PubMed

Kitterick, Pádraig T; O'Donoghue, Gerard M; Edmondson-Jones, Mark; Marshall, Andrew; Jeffs, Ellen; Craddock, Louise; Riley, Alison; Green, Kevin; O'Driscoll, Martin; Jiang, Dan; Nunn, Terry; Saeed, Shakeel; Aleksy, Wanda; Seeber, Bernhard U

2014-01-01

Individuals with a unilateral severe-to-profound hearing loss, or single-sided deafness, report difficulty with listening in many everyday situations despite having access to well-preserved acoustic hearing in one ear. The standard of care for single-sided deafness available on the UK National Health Service is a contra-lateral routing of signals hearing aid which transfers sounds from the impaired ear to the non-impaired ear. This hearing aid has been found to improve speech understanding in noise when the signal-to-noise ratio is more favourable at the impaired ear than the non-impaired ear. However, the indiscriminate routing of signals to a single ear can have detrimental effects when interfering sounds are located on the side of the impaired ear. Recent published evidence has suggested that cochlear implantation in individuals with a single-sided deafness can restore access to the binaural cues which underpin the ability to localise sounds and segregate speech from other interfering sounds. The current trial was designed to assess the efficacy of cochlear implantation compared to a contra-lateral routing of signals hearing aid in restoring binaural hearing in adults with acquired single-sided deafness. Patients are assessed at baseline and after receiving a contra-lateral routing of signals hearing aid. A cochlear implant is then provided to those patients who do not receive sufficient benefit from the hearing aid. This within-subject longitudinal design reflects the expected care pathway should cochlear implantation be provided for single-sided deafness on the UK National Health Service. The primary endpoints are measures of binaural hearing at baseline, after provision of a contra-lateral routing of signals hearing aid, and after cochlear implantation. Binaural hearing is assessed in terms of the accuracy with which sounds are localised and speech is perceived in background noise. The trial is also designed to measure the impact of the interventions on

Translabyrinthine vestibular neurectomy and simultaneous cochlear implant for Ménière's disease.

PubMed

Canzi, Pietro; Manfrin, Marco; Perotti, Marco; Aprile, Federico; Quaglieri, Silvia; Rebecchi, Elisabetta; Locatelli, Giulia; Benazzo, Marco

2017-01-01

Surgical management of Ménière's disease (MD) is recommended in case of medical and intratympanic treatment failures. Translabyrinthine vestibular nerve section has been considered the gold standard for denervation procedures in order to control vertigo attacks, although at the cost of sacrificing residual hearing. To the best of our knowledge, no work has been published with regard to a group of patients submitted to translabyrinthine vestibular neurectomy and simultaneous cochlear implant for MD. The aim of the present study was to assess the effectiveness of translabyrinthine vestibular nerve section and simultaneous cochlear implant in a prospective study. All adult patients (over 18 years of age) with a diagnosis of intractable unilateral definite MD and useless residual hearing function were enrolled after medical and intratympanic treatment failures. Pre- and postoperative otoneurological evaluation concerned: frequency of vertigo attacks, head impulse test and caloric testing, pure tone average and speech perception audiometry in quiet conditions, tinnitus handicap inventory test, functional level scale and rate of vertigo control, dizziness handicap inventory test, and MD patient-oriented severity index. At least 6 months of follow-up were needed to be enrolled in the study. Four patients were included in the study. Translabyrinthine vestibular nerve section and simultaneous cochlear implant seemed to considerably improve the disabling effects of MD, achieving a good control of vestibular symptoms (mean pre/postoperative vertigo attacks per month: 16.5/0), resolving hearing loss (mean pre/postoperative pure tone average in the affected ear: 86.2/32.5 dB), improving the tinnitus (mean pre/postoperative tinnitus handicap inventory test: 77.2/6), and finally increasing the overall quality-of-life parameters. In our preliminary report, translabyrinthine vestibular nerve section and simultaneous cochlear implant showed encouraging results in order to

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.

Cochlear Implant Using Neural Prosthetics

NASA Astrophysics Data System (ADS)

Gupta, Shweta; Singh, Shashi kumar; Dubey, Pratik Kumar

2012-10-01

This research is based on neural prosthetic device. The oldest and most widely used of these electrical, and often computerized, devices is the cochlear implant, which has provided hearing to thousands of congenitally deaf people in this country. Recently, the use of the cochlear implant is expanding to the elderly, who frequently suffer major hearing loss. More cutting edge are artificial retinas, which are helping dozens of blind people see, and ìsmartî artificial arms and legs that amputees can maneuver by thoughts alone, and that feel more like real limbs.Research, which curiosity led to explore frog legs dancing during thunderstorms, a snail shapedorgan in the inner ear, and how various eye cells react to light, have fostered an understanding of how to ìtalkî to the nervous system. That understanding combined with the miniaturization of electronics and enhanced computer processing has enabled prosthetic devices that often can bridge the gap in nerve signaling that is caused by disease or injury.

Qualities of Single Electrode Stimulation as a Function of Rate and Place of Stimulation with a Cochlear Implant

PubMed Central

Landsberger, David M.; Vermeire, Katrien; Claes, Annes; Van Rompaey, Vincent; Van de Heyning, Paul

2015-01-01

Objectives Although it has been previously shown that changes in temporal coding produce changes in pitch in all cochlear regions, research has suggested that temporal coding might be best encoded in relatively apical locations. We hypothesized that although temporal coding may provide useable information at any cochlear location, low rates of stimulation might provide better sound quality in apical regions that are more likely to encode temporal information in the normal ear. In the present study, sound qualities of single electrode pulse trains were scaled to provide insight into the combined effects of cochlear location and stimulation rate on sound quality. Design Ten long term users of MED-EL cochlear implants with 31 mm electrode arrays (Standard or FLEXSOFT) were asked to scale the sound quality of single electrode pulse trains in terms of how “Clean”, “Noisy”, “High”, and “Annoying” they sounded. Pulse trains were presented on most electrodes between 1 and 12 representing the entire range of the long electrode array at stimulation rates of 100, 150, 200, 400, or 1500 pulses per second. Results While high rates of stimulation are scaled as having a “Clean” sound quality across the entire array, only the most apical electrodes (typically 1 through 3) were considered “Clean” at low rates. Low rates on electrodes 6 through 12 were not rated as “Clean” while the low rate quality of electrodes 4 and 5 were typically in between. Scaling of “Noisy” responses provided an approximately inverse pattern as “Clean” responses. “High” responses show the trade-off between rate and place of stimulation on pitch. Because “High” responses did not correlate with “Clean” responses, subjects were not rating sound quality based on pitch. Conclusions If explicit temporal coding is to be provided in a cochlear implant, it is likely to sound better when provided apically. Additionally, the finding that low rates sound clean only at

Neurotrophic factor intervention restores auditory function in deafened animals

NASA Astrophysics Data System (ADS)

Shinohara, Takayuki; Bredberg, Göran; Ulfendahl, Mats; Pyykkö, Ilmari; Petri Olivius, N.; Kaksonen, Risto; Lindström, Bo; Altschuler, Richard; Miller, Josef M.

2002-02-01

A primary cause of deafness is damage of receptor cells in the inner ear. Clinically, it has been demonstrated that effective functionality can be provided by electrical stimulation of the auditory nerve, thus bypassing damaged receptor cells. However, subsequent to sensory cell loss there is a secondary degeneration of the afferent nerve fibers, resulting in reduced effectiveness of such cochlear prostheses. The effects of neurotrophic factors were tested in a guinea pig cochlear prosthesis model. After chemical deafening to mimic the clinical situation, the neurotrophic factors brain-derived neurotrophic factor and an analogue of ciliary neurotrophic factor were infused directly into the cochlea of the inner ear for 26 days by using an osmotic pump system. An electrode introduced into the cochlea was used to elicit auditory responses just as in patients implanted with cochlear prostheses. Intervention with brain-derived neurotrophic factor and the ciliary neurotrophic factor analogue not only increased the survival of auditory spiral ganglion neurons, but significantly enhanced the functional responsiveness of the auditory system as measured by using electrically evoked auditory brainstem responses. This demonstration that neurotrophin intervention enhances threshold sensitivity within the auditory system will have great clinical importance for the treatment of deaf patients with cochlear prostheses. The findings have direct implications for the enhancement of responsiveness in deafferented peripheral nerves.

Current Status and Prospects of Gene Therapy for the Inner Ear

PubMed Central

Huang, Aji

2011-01-01

Abstract Inner ear diseases are common and often result in hearing disability. Sensorineural hearing loss is the main cause of hearing disability. So far, no effective treatment is available although some patients may benefit from a hearing aid equipped with a hearing amplifier or from cochlear implantation. Inner ear gene therapy has become an emerging field of study for the treatment of hearing disability. Numerous new discoveries and tremendous advances have been made in inner ear gene therapy including gene vectors, routes of administration, and therapeutic genes and targets. Gene therapy may become a treatment option for inner ear diseases in the near future. In this review, we summarize the current state of inner ear gene therapy including gene vectors, delivery routes, and therapeutic genes and targets by examining and analyzing publications on inner ear gene therapy from the literature and patent documents, and identify promising patents, novel techniques, and vital research projects. We also discuss the progress and prospects of inner ear gene therapy, the advances and shortcomings, with possible solutions in this field of research. PMID:21338273

Tobacco smoke exposure during childhood: effect on cochlear physiology.

PubMed

Durante, Alessandra S; Pucci, Beatriz; Gudayol, Nicolly; Massa, Beatriz; Gameiro, Marcella; Lopes, Cristiane

2013-10-24

The rate of smoking in Brazil is about 18.8%. Exposure to environmental tobacco smoke is one of the major factors predisposing children to several hazardous health problems. The objective of the present research was to analyze the effect of tobacco smoke exposure during childhood on cochlear physiology by measuring the transient evoked otoacoustic emissions (TEOAE) response levels. Cotinine, the main metabolite of nicotine, was measured in 145 students' (8-10 years old) urine. Sixty students indicated tobacco smoke exposure (TSE) (cotinine urine levels ≥ 5.0 ng/mL) and 85 did not. The evaluation of TEOAE of TSE students showed lower response levels, mainly on frequencies of 2.8 kHz on the right and left ears and 2.0 kHz on left ear and lower signal noise response levels, mainly on the 1.0 kHz and 1.4 kHz frequencies, when compared to controls that were not exposed to tobacco. The mean reduction observed in TEOAE of tobacco smoke exposure children was 2.1 dB SPL. These results have important implications on the damage to the cochlear structures and indicate a possible loss in hearing and hearing ability development.

Comparative Anatomy of the Bony Labyrinth (Inner Ear) of Placental Mammals

PubMed Central

Ekdale, Eric G.

2013-01-01

Background Variation is a naturally occurring phenomenon that is observable at all levels of morphology, from anatomical variations of DNA molecules to gross variations between whole organisms. The structure of the otic region is no exception. The present paper documents the broad morphological diversity exhibited by the inner ear region of placental mammals using digital endocasts constructed from high-resolution X-ray computed tomography (CT). Descriptions cover the major placental clades, and linear, angular, and volumetric dimensions are reported. Principal Findings The size of the labyrinth is correlated to the overall body mass of individuals, such that large bodied mammals have absolutely larger labyrinths. The ratio between the average arc radius of curvature of the three semicircular canals and body mass of aquatic species is substantially lower than the ratios of related terrestrial taxa, and the volume percentage of the vestibular apparatus of aquatic mammals tends to be less than that calculated for terrestrial species. Aspects of the bony labyrinth are phylogenetically informative, including vestibular reduction in Cetacea, a tall cochlear spiral in caviomorph rodents, a low position of the plane of the lateral semicircular canal compared to the posterior canal in Cetacea and Carnivora, and a low cochlear aspect ratio in Primatomorpha. Significance The morphological descriptions that are presented add a broad baseline of anatomy of the inner ear across many placental mammal clades, for many of which the structure of the bony labyrinth is largely unknown. The data included here complement the growing body of literature on the physiological and phylogenetic significance of bony labyrinth structures in mammals, and they serve as a source of data for future studies on the evolution and function of the vertebrate ear. PMID:23805251

Great Ears: Low-Frequency Sensitivity Correlates in Land and Marine Leviathans.

PubMed

Ketten, D R; Arruda, J; Cramer, S; Yamato, M

2016-01-01

Like elephants, baleen whales produce low-frequency (LF) and even infrasonic (IF) signals, suggesting they may be particularly susceptible to underwater anthropogenic sound impacts. Analyses of computerized tomography scans and histologies of the ears in five baleen whale and two elephant species revealed that LF thresholds correlate with basilar membrane thickness/width and cochlear radii ratios. These factors are consistent with high-mass, low-stiffness membranes and broad spiral curvatures, suggesting that Mysticeti and Proboscidea evolved common inner ear adaptations over similar time scales for processing IF/LF sounds despite operating in different media.

Visualization of human inner ear anatomy with high-resolution MR imaging at 7T: initial clinical assessment.

PubMed

van der Jagt, M A; Brink, W M; Versluis, M J; Steens, S C A; Briaire, J J; Webb, A G; Frijns, J H M; Verbist, B M

2015-02-01

In many centers, MR imaging of the inner ear and auditory pathway performed on 1.5T or 3T systems is part of the preoperative work-up of cochlear implants. We investigated the applicability of clinical inner ear MR imaging at 7T and compared the visibility of inner ear structures and nerves within the internal auditory canal with images acquired at 3T. Thirteen patients with sensorineural hearing loss eligible for cochlear implantation underwent examinations on 3T and 7T scanners. Two experienced head and neck radiologists evaluated the 52 inner ear datasets. Twenty-four anatomic structures of the inner ear and 1 overall score for image quality were assessed by using a 4-point grading scale for the degree of visibility. The visibility of 11 of the 24 anatomic structures was rated higher on the 7T images. There was no significant difference in the visibility of 13 anatomic structures and the overall quality rating. A higher incidence of artifacts was observed in the 7T images. The gain in SNR at 7T yielded a more detailed visualization of many anatomic structures, especially delicate ones, despite the challenges accompanying MR imaging at a high magnetic field. © 2015 by American Journal of Neuroradiology.

Commissural axons of the mouse cochlear nucleus.

PubMed

Brown, M Christian; Drottar, Marie; Benson, Thane E; Darrow, Keith

2013-05-01

The axons of commissural neurons that project from one cochlear nucleus to the other were studied after labeling with anterograde tracer. Injections were made into the dorsal subdivision of the cochlear nucleus in order to restrict labeling only to the group of commissural neurons that gave off collaterals to, or were located in, this subdivision. The number of labeled commissural axons in each injection was correlated with the number of labeled radiate multipolar neurons, suggesting radiate neurons as the predominant origin of the axons. The radiate commissural axons are thick and myelinated, and they exit the dorsal acoustic stria of the injected cochlear nucleus to cross the brainstem in the dorsal half, near the crossing position of the olivocochlear bundle. They enter the opposite cochlear nucleus via the dorsal and ventral acoustic stria and at its medial border. Reconstructions of single axons demonstrate that terminations are mostly in the core and typically within a single subdivision of the cochlear nucleus. Extents of termination range from narrow to broad along both the dorsoventral (i.e., tonotopic) and the rostrocaudal dimensions. In the electron microscope, labeled swellings form synapses that are symmetric (in that there is little postsynaptic density), a characteristic of inhibitory synapses. Our labeled axons do not appear to include excitatory commissural axons that end in edge regions of the nucleus. Radiate commissural axons could mediate the broadband inhibition observed in responses to contralateral sound, and they may balance input from the two ears with a quick time course. Copyright © 2012 Wiley Periodicals, Inc.

Commissural Axons of the Mouse Cochlear Nucleus

PubMed Central

Brown, M. Christian; Drottar, Marie; Benson, Thane E.; Darrow, Keith

2012-01-01

The axons of commissural neurons that project from one cochlear nucleus to the other were studied after labeling with anterograde tracer. Injections were made into the dorsal subdivision of the cochlear nucleus in order to restrict labeling only to the group of commissural neurons that gave off collaterals to, or were located in, this subdivision. The number of labeled commissural axons in each injection was correlated with the number of labeled radiate multipolar neurons, suggesting radiate neurons as the predominant origin of the axons. The radiate commissural axons are thick and myelinated, and they exit the dorsal acoustic stria of the injected cochlear nucleus to cross the brainstem in the dorsal half, near the crossing position of the olivocochlear bundle. They enter the opposite cochlear nucleus via the dorsal and ventral acoustic stria and at its medial border. Reconstructions of single axons demonstrate that terminations are mostly in the core and typically within a single subdivision of the cochlear nucleus. Extents of termination range from narrow to broad along both the dorso-ventral (i.e. tonotopic) and rostro-caudal dimensions. In the electron microscope, labeled swellings form synapses that are symmetric (in that there is little postsynaptic density), a characteristic of inhibitory synapses. Our labeled axons do not appear to include excitatory commissural axons that end in edge regions of the nucleus. Radiate commissural axons could mediate the broad-band inhibition observed in responses to contralateral sound, and they may balance input from the two ears on a quick time course. PMID:23124982

Preoperative steroids for hearing preservation cochlear implantation: A review.

PubMed

Kuthubutheen, Jafri; Smith, Leah; Hwang, Euna; Lin, Vincent

2016-01-01

Preoperative steroids have been shown to be beneficial in reducing the hearing loss associated with cochlear implantation. This review article discusses the mechanism of action, effects of differing routes of administration, and side effects of steroids administered to the inner ear. Studies on the role of preoperative steroids in animal and human studies are also examined and future directions for research in this area are discussed.

A new retractor for cochlear implantation.

PubMed

Monksfield, Peter; Proops, David

2008-03-01

In cochlear implantation and other major ear surgeries a post auricular incision is often used. Following this, fascia, muscle and fat is divided, and then a periosteal flap is raised. This creates different tissue planes which need to be held back and protected from the surgeon and the drill. We present a new device which provides excellent retraction for this procedure, does not put undue pressure on the pinna, while at the same time it allowing the surgeon an unobstructed view. (c) 2008 John Wiley & Sons, Ltd.

Eustachian tube function and middle ear barotrauma associated with extremes in atmospheric pressure.

PubMed

Miyazawa, T; Ueda, H; Yanagita, N

1996-11-01

Eustachian tube (ET) function was studied by means of sonotubometry and tubotympano-aerodynamography (TTAG) prior to and following exposure to hypobaric or hyperbaric conditions. Forty normal adults were subjected to hypobaric pressure. Fifty adults who underwent hyperbaric oxygen (HBO) therapy also were studied. Following hypobaric exposure, 14 of 80 ears (17.5%) exhibited middle ear barotrauma. Following hyperbaric exposure, 34 of 100 ears (34%) exhibited middle ear barotrauma. Dysfunction of the ET, characterized by altered active and passive opening capacity, was more prevalent following exposure to extremes in atmospheric pressure compared to baseline. The ET function, which was impaired after the first HBO treatment, improved gradually over the next 2 hours. Overall, however, ET function was worse after the seventh treatment. The patients who developed barotrauma exhibited worse ET function prior to hypobaric or hyperbaric exposure. Thus, abnormal ET function can be used to predict middle ear barotrauma prior to exposure to hypobaric or hyperbaric atmospheric pressure.

Identification of Adeno-Associated Viral Vectors That Target Neonatal and Adult Mammalian Inner Ear Cell Subtypes

PubMed Central

Shu, Yilai; Tao, Yong; Wang, Zhengmin; Tang, Yong; Li, Huawei; Dai, Pu; Gao, Guangping; Chen, Zheng-Yi

2016-01-01

The mammalian inner ear consists of diverse cell types with important functions. Gene mutations in these diverse cell types have been found to underlie different forms of genetic hearing loss. Targeting these mutations for gene therapy development represents a future therapeutic strategy to treat hearing loss. Adeno-associated viral (AAV) vectors have become the vector of choice for gene delivery in animal models in vivo. To identify AAV vectors that target inner ear cell subtypes, we systemically screened 12 AAV vectors with different serotypes (AAV1, 2, 5, 6, 6.2, 7, 8, 9, rh.8, rh.10, rh.39, and rh.43) that carry a reporter gene GFP in neonatal and adult mice by microinjection in vivo. We found that most AAVs infect both neonatal and adult inner ear, with different specificities and expression levels. The inner ear cochlear sensory epithelial region, which includes auditory hair cells and supporting cells, is most frequently targeted for gene delivery. Expression of the transgene is sustained, and neonatal inner ear delivery does not adversely affect hearing. Adult inner ear injection of AAV has a similar infection pattern as the younger inner ear, with the exception that outer hair cell death caused by the injection procedure can lead to hearing loss. In the adult, more so than in the neonatal mice, cell types infected and efficiency of infection are correlated with the site of injection. Most infected cells survive in neonatal and adult inner ears. The study adds to the list of AAV vectors that transduce the mammalian inner ear efficiently, providing the tools that are important to study inner ear gene function and for the development of gene therapy to treat hearing loss. PMID:27342665

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

Single-unit labeling of medial olivocochlear neurons: the cochlear frequency map for efferent axons.

PubMed

Brown, M Christian

2014-06-01

Medial olivocochlear (MOC) neurons are efferent neurons that project axons from the brain to the cochlea. Their action on outer hair cells reduces the gain of the "cochlear amplifier," which shifts the dynamic range of hearing and reduces the effects of noise masking. The MOC effects in one ear can be elicited by sound in that ipsilateral ear or by sound in the contralateral ear. To study how MOC neurons project onto the cochlea to mediate these effects, single-unit labeling in guinea pigs was used to study the mapping of MOC neurons for neurons responsive to ipsilateral sound vs. those responsive to contralateral sound. MOC neurons were sharply tuned to sound frequency with a well-defined characteristic frequency (CF). However, their labeled termination spans in the organ of Corti ranged from narrow to broad, innervating between 14 and 69 outer hair cells per axon in a "patchy" pattern. For units responsive to ipsilateral sound, the midpoint of innervation was mapped according to CF in a relationship generally similar to, but with more variability than, that of auditory-nerve fibers. Thus, based on CF mappings, most of the MOC terminations miss outer hair cells involved in the cochlear amplifier for their CF, which are located more basally. Compared with ipsilaterally responsive neurons, contralaterally responsive neurons had an apical offset in termination and a larger span of innervation (an average of 10.41% cochlear distance), suggesting that when contralateral sound activates the MOC reflex, the actions are different than those for ipsilateral sound. Copyright © 2014 the American Physiological Society.

Single-unit labeling of medial olivocochlear neurons: the cochlear frequency map for efferent axons

PubMed Central

2014-01-01

Medial olivocochlear (MOC) neurons are efferent neurons that project axons from the brain to the cochlea. Their action on outer hair cells reduces the gain of the “cochlear amplifier,” which shifts the dynamic range of hearing and reduces the effects of noise masking. The MOC effects in one ear can be elicited by sound in that ipsilateral ear or by sound in the contralateral ear. To study how MOC neurons project onto the cochlea to mediate these effects, single-unit labeling in guinea pigs was used to study the mapping of MOC neurons for neurons responsive to ipsilateral sound vs. those responsive to contralateral sound. MOC neurons were sharply tuned to sound frequency with a well-defined characteristic frequency (CF). However, their labeled termination spans in the organ of Corti ranged from narrow to broad, innervating between 14 and 69 outer hair cells per axon in a “patchy” pattern. For units responsive to ipsilateral sound, the midpoint of innervation was mapped according to CF in a relationship generally similar to, but with more variability than, that of auditory-nerve fibers. Thus, based on CF mappings, most of the MOC terminations miss outer hair cells involved in the cochlear amplifier for their CF, which are located more basally. Compared with ipsilaterally responsive neurons, contralaterally responsive neurons had an apical offset in termination and a larger span of innervation (an average of 10.41% cochlear distance), suggesting that when contralateral sound activates the MOC reflex, the actions are different than those for ipsilateral sound. PMID:24598524

An animal model for the analysis of cochlear blood flow [corrected] disturbance and hearing threshold in vivo.

PubMed

Canis, Martin; Arpornchayanon, Warangkana; Messmer, Catalina; Suckfuell, Markus; Olzowy, Bernhard; Strieth, Sebastian

2010-02-01

Impairment of cochlear blood flow (CBF) is considered to be important in inner ear pathology. However, direct measurement of CBF is difficult and has not been investigated in combination with hearing function. Six guinea pigs were used to show feasibility of an animal model for the analysis of cochlear microcirculation by intravital microscopy in combination with investigation of the hearing threshold by brainstem response audiometry (ABR). By the application of sodium nitroprusside (SNP), CBF was increased over 30 min. Reproducibility of measurements was shown by retest measurements. Mean baseline velocity of CBF was 109 +/- 19 mum/s. Vessel diameters had a mean value of 9.4 +/- 2.7 mum. Mean hearing threshold was 19 +/- 6 dB. In response to SNP, CBF velocity increased significantly to 161 +/- 26 mum/s. Mean arterial pressure decreased significantly to 36 +/- 11 mmHg. After the end of the application, CBF velocity recovered to a minimum of 123 +/- 17 microm/s. Within the retest, CBF velocity significantly increased to a maximum of 160 +/- 31 microm/s. Second recovery of CBF velocity was 125 +/- 14 mum/s. Within the second retest, CBF increased significantly to 157 +/- 25 microm/s. ABR thresholds did not change significantly. The increase in blood flow velocity occurred in spite of substantial hypotension as induced by a vasodilator. This may explain the fact that ABR threshold remained unchanged reflecting a maintained blood supply in this part of the brain. This technique can be used to evaluate effects of treatments aimed at cochlear microcirculation in inner ear pathologies.

Revisiting gender, race, and ear differences in peripheral auditory function

NASA Astrophysics Data System (ADS)

Boothalingam, Sriram; Klyn, Niall A. M.; Stiepan, Samantha M.; Wilson, Uzma S.; Lee, Jungwha; Siegel, Jonathan H.; Dhar, Sumitrajit

2018-05-01

Various measures of auditory function are reported to be superior in females as compared to males, in African American compared to Caucasian individuals, and in right compared to left ears. We re-examined the influence of these subject variables on hearing thresholds and otoacoustic emissions (OAEs) in a sample of 887 human participants between 10 and 68 years of age. Even though the variables of interest here have been examined before, previous attempts have largely been limited to frequencies up to 8 kHz. We used state-of-the-art signal delivery and recording techniques that compensated for individual differences in ear canal acoustics, allowing us to measure hearing thresholds and OAEs up to 20 kHz. The use of these modern calibration and recording techniques provided the motivation for re-examining these commonly studied variables. While controlling for age, noise exposure history, and general health history, we attempted to isolate the effects of gender, race, and ear (left versus right) on hearing thresholds and OAEs. Our results challenge the notion of a right ear advantage and question the existence of a significant gender and race differences in both hearing thresholds and OAE levels. These results suggest that ear canal anatomy and acoustics should be important considerations when evaluating the influence of gender, race, and ear on peripheral auditory function.

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

Comparison of the benefits of cochlear implantation versus contra-lateral routing of signal hearing aids in adult patients with single-sided deafness: study protocol for a prospective within-subject longitudinal trial

PubMed Central

2014-01-01

Background Individuals with a unilateral severe-to-profound hearing loss, or single-sided deafness, report difficulty with listening in many everyday situations despite having access to well-preserved acoustic hearing in one ear. The standard of care for single-sided deafness available on the UK National Health Service is a contra-lateral routing of signals hearing aid which transfers sounds from the impaired ear to the non-impaired ear. This hearing aid has been found to improve speech understanding in noise when the signal-to-noise ratio is more favourable at the impaired ear than the non-impaired ear. However, the indiscriminate routing of signals to a single ear can have detrimental effects when interfering sounds are located on the side of the impaired ear. Recent published evidence has suggested that cochlear implantation in individuals with a single-sided deafness can restore access to the binaural cues which underpin the ability to localise sounds and segregate speech from other interfering sounds. Methods/Design The current trial was designed to assess the efficacy of cochlear implantation compared to a contra-lateral routing of signals hearing aid in restoring binaural hearing in adults with acquired single-sided deafness. Patients are assessed at baseline and after receiving a contra-lateral routing of signals hearing aid. A cochlear implant is then provided to those patients who do not receive sufficient benefit from the hearing aid. This within-subject longitudinal design reflects the expected care pathway should cochlear implantation be provided for single-sided deafness on the UK National Health Service. The primary endpoints are measures of binaural hearing at baseline, after provision of a contra-lateral routing of signals hearing aid, and after cochlear implantation. Binaural hearing is assessed in terms of the accuracy with which sounds are localised and speech is perceived in background noise. The trial is also designed to measure the impact of

NANOCI-Nanotechnology Based Cochlear Implant With Gapless Interface to Auditory Neurons.

PubMed

Senn, Pascal; Roccio, Marta; Hahnewald, Stefan; Frick, Claudia; Kwiatkowska, Monika; Ishikawa, Masaaki; Bako, Peter; Li, Hao; Edin, Fredrik; Liu, Wei; Rask-Andersen, Helge; Pyykkö, Ilmari; Zou, Jing; Mannerström, Marika; Keppner, Herbert; Homsy, Alexandra; Laux, Edith; Llera, Miguel; Lellouche, Jean-Paul; Ostrovsky, Stella; Banin, Ehud; Gedanken, Aharon; Perkas, Nina; Wank, Ute; Wiesmüller, Karl-Heinz; Mistrík, Pavel; Benav, Heval; Garnham, Carolyn; Jolly, Claude; Gander, Filippo; Ulrich, Peter; Müller, Marcus; Löwenheim, Hubert

2017-09-01

: Cochlear implants (CI) restore functional hearing in the majority of deaf patients. Despite the tremendous success of these devices, some limitations remain. The bottleneck for optimal electrical stimulation with CI is caused by the anatomical gap between the electrode array and the auditory neurons in the inner ear. As a consequence, current devices are limited through 1) low frequency resolution, hence sub-optimal sound quality and 2), large stimulation currents, hence high energy consumption (responsible for significant battery costs and for impeding the development of fully implantable systems). A recently completed, multinational and interdisciplinary project called NANOCI aimed at overcoming current limitations by creating a gapless interface between auditory nerve fibers and the cochlear implant electrode array. This ambitious goal was achieved in vivo by neurotrophin-induced attraction of neurites through an intracochlear gel-nanomatrix onto a modified nanoCI electrode array located in the scala tympani of deafened guinea pigs. Functionally, the gapless interface led to lower stimulation thresholds and a larger dynamic range in vivo, and to reduced stimulation energy requirement (up to fivefold) in an in vitro model using auditory neurons cultured on multi-electrode arrays. In conclusion, the NANOCI project yielded proof of concept that a gapless interface between auditory neurons and cochlear implant electrode arrays is feasible. These findings may be of relevance for the development of future CI systems with better sound quality and performance and lower energy consumption. The present overview/review paper summarizes the NANOCI project history and highlights achievements of the individual work packages.

Wideband acoustic reflex test in a test battery to predict middle-ear dysfunction

PubMed Central

Keefe, Douglas H.; Fitzpatrick, Denis; Liu, Yi-Wen; Sanford, Chris A.; Gorga, Michael P.

2013-01-01

A wideband (WB) aural acoustical test battery of middle-ear status, including acoustic-reflex thresholds (ARTs) and acoustic-transfer functions (ATFs, i.e., absorbance and admittance) was hypothesized to be more accurate than 1-kHz tympanometry in classifying ears that pass or refer on a newborn hearing screening (NHS) protocol based on otoacoustic emissions. Assessment of middle-ear status may improve NHS programs by identifying conductive dysfunction and cases in which auditory neuropathy exists. Ipsilateral ARTs were assessed with a stimulus including four broadband-noise or tonal activator pulses alternating with five clicks presented before, between and after the pulses. The reflex shift was defined as the difference between final and initial click responses. ARTs were measured using maximum likelihood both at low frequencies (0.8–2.8 kHz) and high (2.8–8 kHz). The median low-frequency ART was elevated by 24 dB in NHS refers compared to passes. An optimal combination of ATF and ART tests performed better than either test alone in predicting NHS outcomes, and WB tests performed better than 1-kHz tympanometry. Medial olivocochlear efferent shifts in cochlear function may influence ARs, but their presence would also be consistent with normal conductive function. Baseline clinical and WB ARTs were also compared in ipsilateral and contralateral measurements in adults. PMID:19772907

Real-time loudness normalisation with combined cochlear implant and hearing aid stimulation

PubMed Central

Van Eeckhoutte, Maaike; Van Deun, Lieselot; Francart, Tom

2018-01-01

Background People who use a cochlear implant together with a contralateral hearing aid—so-called bimodal listeners—have poor localisation abilities and sounds are often not balanced in loudness across ears. In order to address the latter, a loudness balancing algorithm was created, which equalises the loudness growth functions for the two ears. The algorithm uses loudness models in order to continuously adjust the two signals to loudness targets. Previous tests demonstrated improved binaural balance, improved localisation, and better speech intelligibility in quiet for soft phonemes. In those studies, however, all stimuli were preprocessed so spontaneous head movements and individual head-related transfer functions were not taken into account. Furthermore, the hearing aid processing was linear. Study design In the present study, we simplified the acoustical loudness model and implemented the algorithm in a real-time system. We tested bimodal listeners on speech perception and on sound localisation, both in normal loudness growth configuration and in a configuration with a modified loudness growth function. We also used linear and compressive hearing aids. Results The comparison between the original acoustical loudness model and the new simplified model showed loudness differences below 3% for almost all tested speech-like stimuli and levels. We found no effect of balancing the loudness growth across ears for speech perception ability in quiet and in noise. We found some small improvements in localisation performance. Further investigation with a larger sample size is required. PMID:29617421

A genome-wide linkage and association study of musical aptitude identifies loci containing genes related to inner ear development and neurocognitive functions

PubMed Central

Oikkonen, J.; Huang, Y.; Onkamo, P.; Ukkola-Vuoti, L.; Raijas, P.; Karma, K.; Vieland, V. J.; Järvelä, I.

2014-01-01

Humans have developed the perception, production and processing of sounds into the art of music. A genetic contribution to these skills of musical aptitude has long been suggested. We performed a genome-wide scan in 76 pedigrees (767 individuals) characterized for the ability to discriminate pitch (SP), duration (ST) and sound patterns (KMT), which are primary capacities for music perception. Using the Bayesian linkage and association approach implemented in program package KELVIN, especially designed for complex pedigrees, several SNPs near genes affecting the functions of the auditory pathway and neurocognitive processes were identified. The strongest association was found at 3q21.3 (rs9854612) with combined SP, ST and KMT test scores (COMB). This region is located a few dozen kilobases upstream of the GATA binding protein 2 (GATA2) gene. GATA2 regulates the development of cochlear hair cells and the inferior colliculus (IC), which are important in tonotopic mapping. The highest probability of linkage was obtained for phenotype SP at 4p14, located next to the region harboring the protocadherin 7 gene, PCDH7. Two SNPs rs13146789 and rs13109270 of PCDH7 showed strong association. PCDH7 has been suggested to play a role in cochlear and amygdaloid complexes. Functional class analysis showed that inner ear and schizophrenia related genes were enriched inside the linked regions. This study is the first to show the importance of auditory pathway genes in musical aptitude. PMID:24614497

A genome-wide linkage and association study of musical aptitude identifies loci containing genes related to inner ear development and neurocognitive functions.

PubMed

Oikkonen, J; Huang, Y; Onkamo, P; Ukkola-Vuoti, L; Raijas, P; Karma, K; Vieland, V J; Järvelä, I

2015-02-01

Humans have developed the perception, production and processing of sounds into the art of music. A genetic contribution to these skills of musical aptitude has long been suggested. We performed a genome-wide scan in 76 pedigrees (767 individuals) characterized for the ability to discriminate pitch (SP), duration (ST) and sound patterns (KMT), which are primary capacities for music perception. Using the Bayesian linkage and association approach implemented in program package KELVIN, especially designed for complex pedigrees, several single nucleotide polymorphisms (SNPs) near genes affecting the functions of the auditory pathway and neurocognitive processes were identified. The strongest association was found at 3q21.3 (rs9854612) with combined SP, ST and KMT test scores (COMB). This region is located a few dozen kilobases upstream of the GATA binding protein 2 (GATA2) gene. GATA2 regulates the development of cochlear hair cells and the inferior colliculus (IC), which are important in tonotopic mapping. The highest probability of linkage was obtained for phenotype SP at 4p14, located next to the region harboring the protocadherin 7 gene, PCDH7. Two SNPs rs13146789 and rs13109270 of PCDH7 showed strong association. PCDH7 has been suggested to play a role in cochlear and amygdaloid complexes. Functional class analysis showed that inner ear and schizophrenia-related genes were enriched inside the linked regions. This study is the first to show the importance of auditory pathway genes in musical aptitude.

Temporal and spatial expression patterns of Hedgehog receptors in the developing inner and middle ear.

PubMed

Shin, Jeong-Oh; Ankamreddy, Harinarayana; Jakka, Naga Mahesh; Lee, Seokwon; Kim, Un-Kyung; Bok, Jinwoong

2017-01-01

The mammalian inner ear is a complex organ responsible for balance and hearing. Sonic hedgehog (Shh), a member of the Hedgehog (Hh) family of secreted proteins, has been shown to play important roles in several aspects of inner ear development, including dorsoventral axial specification, cochlear elongation, tonotopic patterning, and hair cell differentiation. Hh proteins initiate a downstream signaling cascade by binding to the Patched 1 (Ptch1) receptor. Recent studies have revealed that other types of co-receptors can also mediate Hh signaling, including growth arrest-specific 1 (Gas1), cell-adhesion molecules-related/down-regulated by oncogenes (Cdon), and biregional Cdon binding protein (Boc). However, little is known about the role of these Hh co-receptors in inner ear development. In this study, we examined the expression patterns of Gas1, Cdon, and Boc, as well as that of Ptch1, in the developing mouse inner ear from otocyst (embryonic day (E) 9.5) until birth and in the developing middle ear at E15.5. Ptch1, a readout of Hh signaling, was expressed in a graded pattern in response to Shh signaling throughout development. Expression patterns of Gas1, Cdon, and Boc differed from that of Ptch1, and each Hh co-receptor was expressed in specific cells and domains in the developing inner and middle ear. These unique and differential expression patterns of Hh co-receptors suggest their roles in mediating various time- and space-specific functions of Shh during ear development.

Conductive Hearing Loss Caused by Third-Window Lesions of the Inner Ear

PubMed Central

Merchant, Saumil N.; Rosowski, John J.

2008-01-01

Background Various authors have described conductive hearing loss (CHL), defined as an air-bone gap on audiometry, in patients without obvious middle ear pathologic findings. Recent investigations have suggested that many of these cases are due to disorders of the inner ear, resulting in pathologic third windows. Objective To provide an overview of lesions of the inner ear resulting in a CHL due to a third-window mechanism. The mechanism of the CHL is explained along with a classification scheme for these disorders. We also discuss methods for diagnosis of these disorders. Data Sources The data were compiled from a review of the literature and recent published research on middle and inner ear mechanics from our laboratory. Conclusion A number of disparate disorders affecting the labyrinth can produce CHL by acting as a pathologic third window in the inner ear. The common denominator is that these conditions result in a mobile window on the scala vestibuli side of the cochlear partition. The CHL results by the dual mechanism of worsening of air conduction thresholds and improvement of bone conduction thresholds. Such lesions may be anatomically discrete or diffuse. Anatomically discrete lesions may be classified by location: semicircular canals (superior, lateral, or posterior canal dehiscence), bony vestibule (large vestibular aqueduct syndrome, other inner ear malformations), or the cochlea (carotid-cochlear dehiscence, X-linked deafness with stapes gusher, etc.). An example of an anatomically diffuse lesion is Paget disease, which may behave as a distributed or diffuse third window. Third-window lesions should be considered in the differential diagnosis of CHL in patients with an intact tympanic membrane and an aerated, otherwise healthy, middle ear. Clues to suspect such a lesion include a low-frequency air-bone gap with supranormal thresholds for bone conduction, and presence of acoustic reflexes, vestibular evoked myogenic responses, or otoacoustic emission

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.

Comparisons of auditory brainstem response and sound level tolerance in tinnitus ears and non-tinnitus ears in unilateral tinnitus patients with normal audiograms

PubMed Central

An, Yong-Hwi; Kim, Dong Hyun; Yoon, Ji Eun; Yoon, Ji Hyang

2017-01-01

Objective Recently, “hidden hearing loss” with cochlear synaptopathy has been suggested as a potential pathophysiology of tinnitus in individuals with a normal hearing threshold. Several studies have demonstrated that subjects with tinnitus and normal audiograms show significantly reduced auditory brainstem response (ABR) wave I amplitudes compared with control subjects, but normal wave V amplitudes, suggesting increased central auditory gain. We aimed to reconfirm the “hidden hearing loss” theory through a within-subject comparison of wave I and wave V amplitudes and uncomfortable loudness level (UCL), which might be decreased with increased central gain, in tinnitus ears (TEs) and non-tinnitus ears (NTEs). Subjects and methods Human subjects included 43 unilateral tinnitus patients (19 males, 24 females) with normal and symmetric hearing thresholds and 18 control subjects with normal audiograms. The amplitudes of wave I and V from the peak to the following trough were measured twice at 90 dB nHL and we separately assessed UCLs at 500 Hz and 3000 Hz pure tones in each TE and NTE. Results The within-subject comparison between TEs and NTEs showed no significant differences in wave I and wave V amplitude, or wave V/I ratio in both the male and female groups. Individual data revealed increased V/I amplitude ratios > mean + 2 SD in 3 TEs, but not in any control ears. We found no significant differences in UCL at 500 Hz or 3000 Hz between the TEs and NTEs, but the UCLs of both TEs and NTEs were lower than those of the control ears. Conclusions Our ABR data do not represent meaningful evidence supporting the hypothesis of cochlear synaptopathy with increased central gain in tinnitus subjects with normal audiograms. However, reduced sound level tolerance in both TEs and NTEs might reflect increased central gain consequent on hidden synaptopathy that was subsequently balanced between the ears by lateral olivocochlear efferents. PMID:29253030

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.

Ear Structures of the Naked Mole-Rat, Heterocephalus glaber, and Its Relatives (Rodentia: Bathyergidae)

PubMed Central

Mason, Matthew J.; Cornwall, Hannah L.; Smith, Ewan St. J.

2016-01-01

Although increasingly popular as a laboratory species, very little is known about the peripheral auditory system of the naked mole-rat, Heterocephalus glaber. In this study, middle and inner ears of naked mole-rats of a range of ages were examined using micro-computed tomography and dissection. The ears of five other bathyergid species (Bathyergus suillus, Cryptomys hottentotus, Fukomys micklemi, Georychus capensis and Heliophobius argenteocinereus) were examined for comparative purposes. The middle ears of bathyergids show features commonly found in other members of the Ctenohystrica rodent clade, including a fused malleus and incus, a synovial stapedio-vestibular articulation and the loss of the stapedius muscle. Heterocephalus deviates morphologically from the other bathyergids examined in that it has a more complex mastoid cavity structure, poorly-ossified processes of the malleus and incus, a ‘columelliform’ stapes and fewer cochlear turns. Bathyergids have semicircular canals with unusually wide diameters relative to their radii of curvature. How the lateral semicircular canal reaches the vestibule differs between species. Heterocephalus has much more limited high-frequency hearing than would be predicted from its small ear structures. The spongy bone forming its ossicular processes, the weak incudo-stapedial articulation, the columelliform stapes and (compared to other bathyergids) reduced cochlear coiling are all potentially degenerate features which might reflect a lack of selective pressure on its peripheral auditory system. Substantial intraspecific differences were found in certain middle and inner ear structures, which might also result from relaxed selective pressures. However, such interpretations must be treated with caution in the absence of experimental evidence. PMID:27926945

Influence of contralateral acoustic hearing on adult bimodal outcomes after cochlear implantation.

PubMed

Plant, Kerrie; van Hoesel, Richard; McDermott, Hugh; Dawson, Pamela; Cowan, Robert

2016-08-01

To examine post-implantation benefit and time taken to acclimate to the cochlear implant for adult candidates with more hearing in the contralateral non-implanted ear than has been previously considered within local candidacy guidelines. Prospective, within-subject experimental design. Forty postlingual hearing-impaired adult subjects with a contralateral ear word score in quiet ranging from 27% to 100% (median 67%). Post-implantation improvement of 2.4 dB and 4.0 dB was observed on a sentence in coincident babble test at presentation levels of 65 and 55 dB SPL respectively, and a 2.1 dB benefit in spatial release from masking (SRM) advantage observed when the noise location favoured the implanted side. Significant post-operative group mean change of between 2.1 and 3.0 was observed on the sub-scales of the speech, spatial, and qualities (SSQ) questionnaire. Degree of post-implantation speech reception threshold (SRT) benefit on the coincident babble test and on perception of soft speech and sounds in the environment was greater for subjects with less contralateral hearing. The degree of contralateral acoustic hearing did not affect time taken to acclimate to the device. The findings from this study support cochlear implantation for candidates with substantial acoustic hearing in the contralateral ear, and provide guidance regarding post-implantation expectations.

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.

Language and Psychosocial Functioning among Deaf Learners with and without Cochlear Implants

ERIC Educational Resources Information Center

Marschark, Marc; Machmer, Elizabeth; Spencer, Linda J.; Borgna, Georgianna; Durkin, Andreana; Convertino, Carol

2018-01-01

Various studies have examined psychosocial functioning and language abilities among deaf children with and without cochlear implants (CIs). Few, however, have explored how relations among those abilities might change with age and setting. Most relevant studies also have failed to consider that psychosocial functioning among both CI users and…

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.

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.

Automatic detection of the inner ears in head CT images using deep convolutional neural networks

NASA Astrophysics Data System (ADS)

Zhang, Dongqing; Noble, Jack H.; Dawant, Benoit M.

2018-03-01

Cochlear implants (CIs) use electrode arrays that are surgically inserted into the cochlea to stimulate nerve endings to replace the natural electro-mechanical transduction mechanism and restore hearing for patients with profound hearing loss. Post-operatively, the CI needs to be programmed. Traditionally, this is done by an audiologist who is blind to the positions of the electrodes relative to the cochlea and relies on the patient's subjective response to stimuli. This is a trial-and-error process that can be frustratingly long (dozens of programming sessions are not unusual). To assist audiologists, we have proposed what we call IGCIP for image-guided cochlear implant programming. In IGCIP, we use image processing algorithms to segment the intra-cochlear anatomy in pre-operative CT images and to localize the electrode arrays in post-operative CTs. We have shown that programming strategies informed by image-derived information significantly improve hearing outcomes for both adults and pediatric populations. We are now aiming at deploying these techniques clinically, which requires full automation. One challenge we face is the lack of standard image acquisition protocols. The content of the image volumes we need to process thus varies greatly and visual inspection and labelling is currently required to initialize processing pipelines. In this work we propose a deep learning-based approach to automatically detect if a head CT volume contains two ears, one ear, or no ear. Our approach has been tested on a data set that contains over 2,000 CT volumes from 153 patients and we achieve an overall 95.97% classification accuracy.

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.

Modeling Sound Processing in Cochlear Nuclei

NASA Astrophysics Data System (ADS)

Meddis, Ray

2003-03-01

The cochlear nucleus is an obligatory relay nucleus between the ear and the rest of the brain. It consists of many different types of neurons each responding differently to the same stimulus. Much is known about the wiring diagram of the system but it has so far proved difficult to characterise the signal processing that is going on or what purpose it serves. The solution to this problem is a pre-requisite of any attempt to produce a practical electronic simulation that exploits the brain's unique capacity to recognise the significance of acoustic events and generate appropriate responses. This talk will explain the different types of neural cell and specify hypotheses as to their various functions. Cell-types vary in terms of their size and shape as well as the number and type of minute electrical currents that flow across the cell membranes. Computer models will also be used to illustrate how the physical substrate (the wet-ware) is used to achieve its signal-processing goals.

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.

Unilateral Hearing Loss: Understanding Speech Recognition and Localization Variability-Implications for Cochlear Implant Candidacy.

PubMed

Firszt, Jill B; Reeder, Ruth M; Holden, Laura K

the open/hearing side. In contrast, UHLs localized better than the normal-hearing unilateral listeners for stimuli on the open/hearing side but not the blocked/deaf side. This suggests that UHLs had learned strategies for improved localization on the side of the intact ear. The UHL and unilateral normal-hearing participant groups were not significantly different for speech in noise measures. UHL participants with childhood rather than recent hearing loss onset localized significantly better; however, these two groups did not differ for speech recognition in noise. Age at onset in UHL adults appears to affect localization ability differently than understanding speech in noise. Hearing thresholds were significantly correlated with speech recognition for UHL participants but not the other two groups. Auditory abilities of UHLs varied widely and could be explained only in part by hearing threshold levels. Age at onset and length of hearing loss influenced performance on some, but not all measures. Results support the need for a revised and diverse set of clinical measures, including sound localization, understanding speech in varied environments, and careful consideration of functional abilities as individuals with severe to profound UHL are being considered potential cochlear implant candidates.

Unilateral Hearing Loss: Understanding Speech Recognition and Localization Variability - Implications for Cochlear Implant Candidacy

PubMed Central

Firszt, Jill B.; Reeder, Ruth M.; Holden, Laura K.

2016-01-01

the blocked/deaf side but not the open/hearing side. In contrast, UHLs localized better than the normal hearing unilateral listeners for stimuli on the open/hearing side but not the blocked/deaf side. This suggests that UHLs had learned strategies for improved localization on the side of the intact ear. The UHL and unilateral normal hearing participant groups were not significantly different for speech-in-noise measures. UHL participants with childhood rather than recent hearing loss onset localized significantly better; however, these two groups did not differ for speech recognition in noise. Age at onset in UHL adults appears to affect localization ability differently than understanding speech in noise. Hearing thresholds were significantly correlated with speech recognition for UHL participants but not the other two groups. Conclusions Auditory abilities of UHLs varied widely and could be explained only in part by hearing threshold levels. Age at onset and length of hearing loss influenced performance on some, but not all measures. Results support the need for a revised and diverse set of clinical measures, including sound localization, understanding speech in varied environments and careful consideration of functional abilities as individuals with severe to profound UHL are being considered potential cochlear implant candidates. PMID:28067750

The zinc finger transcription factor Gfi1, implicated in lymphomagenesis, is required for inner ear hair cell differentiation and survival

NASA Technical Reports Server (NTRS)

Wallis, Deeann; Hamblen, Melanie; Zhou, Yi; Venken, Koen J T.; Schumacher, Armin; Grimes, H. Leighton; Zoghbi, Huda Y.; Orkin, Stuart H.; Bellen, Hugo J.

2003-01-01

Gfi1 was first identified as causing interleukin 2-independent growth in T cells and lymphomagenesis in mice. Much work has shown that Gfi1 and Gfi1b, a second mouse homolog, play pivotal roles in blood cell lineage differentiation. However, neither Gfi1 nor Gfi1b has been implicated in nervous system development, even though their invertebrate homologues, senseless in Drosophila and pag-3 in C. elegans are expressed and required in the nervous system. We show that Gfi1 mRNA is expressed in many areas that give rise to neuronal cells during embryonic development in mouse, and that Gfi1 protein has a more restricted expression pattern. By E12.5 Gfi1 mRNA is expressed in both the CNS and PNS as well as in many sensory epithelia including the developing inner ear epithelia. At later developmental stages, Gfi1 expression in the ear is refined to the hair cells and neurons throughout the inner ear. Gfi1 protein is expressed in a more restricted pattern in specialized sensory cells of the PNS, including the eye, presumptive Merkel cells, the lung and hair cells of the inner ear. Gfi1 mutant mice display behavioral defects that are consistent with inner ear anomalies, as they are ataxic, circle, display head tilting behavior and do not respond to noise. They have a unique inner ear phenotype in that the vestibular and cochlear hair cells are differentially affected. Although Gfi1-deficient mice initially specify inner ear hair cells, these hair cells are disorganized in both the vestibule and cochlea. The outer hair cells of the cochlea are improperly innervated and express neuronal markers that are not normally expressed in these cells. Furthermore, Gfi1 mutant mice lose all cochlear hair cells just prior to and soon after birth through apoptosis. Finally, by five months of age there is also a dramatic reduction in the number of cochlear neurons. Hence, Gfi1 is expressed in the developing nervous system, is required for inner ear hair cell differentiation, and its loss

Physiopathology of the Cochlear Microcirculation

PubMed Central

Shi, Xiaorui

2011-01-01

Normal blood supply to the cochlea is critically important for establishing the endocochlear potential and sustaining production of endolymph. Abnormal cochlear microcirculation has long been considered an etiologic factor in noise-induced hearing loss, age-related hearing loss (presbycusis), sudden hearing loss or vestibular function, and Meniere's disease. Knowledge of the mechanisms underlying the pathophysiology of cochlear microcirculation is of fundamental clinical importance. A better understanding of cochlear blood flow (CoBF) will enable more effective management of hearing disorders resulting from aberrant blood flow. This review focuses on recent discoveries and findings related to the physiopathology of the cochlear microvasculature. PMID:21875658

Executive Functioning Skills in Preschool-Age Children with Cochlear Implants

ERIC Educational Resources Information Center

Beer, Jessica; Kronenberger, William G.; Castellanos, Irina; Colson, Bethany G.; Henning, Shirley C.; Pisoni, David B.

2014-01-01

Purpose: The purpose of this study was to determine whether deficits in executive functioning (EF) in children with cochlear implants (CIs) emerge as early as the preschool years. Method: Two groups of children ages 3 to 6 years participated in this cross-sectional study: 24 preschoolers who had CIs prior to 36 months of age and 21 preschoolers…

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.

Selective attention reduces physiological noise in the external ear canals of humans. I: Auditory attention

PubMed Central

Walsh, Kyle P.; Pasanen, Edward G.; McFadden, Dennis

2014-01-01

In this study, a nonlinear version of the stimulus-frequency OAE (SFOAE), called the nSFOAE, was used to measure cochlear responses from human subjects while they simultaneously performed behavioral tasks requiring, or not requiring, selective auditory attention. Appended to each stimulus presentation, and included in the calculation of each nSFOAE response, was a 30-ms silent period that was used to estimate the level of the inherent physiological noise in the ear canals of our subjects during each behavioral condition. Physiological-noise magnitudes were higher (noisier) for all subjects in the inattention task, and lower (quieter) in the selective auditory-attention tasks. These noise measures initially were made at the frequency of our nSFOAE probe tone (4.0 kHz), but the same attention effects also were observed across a wide range of frequencies. We attribute the observed differences in physiological-noise magnitudes between the inattention and attention conditions to different levels of efferent activation associated with the differing attentional demands of the behavioral tasks. One hypothesis is that when the attentional demand is relatively great, efferent activation is relatively high, and a decrease in the gain of the cochlear amplifier leads to lower-amplitude cochlear activity, and thus a smaller measure of noise from the ear. PMID:24732069

How do the medial olivocochlear efferents influence the biomechanics of the outer hair cells and thereby the cochlear amplifier? Simulation results

NASA Astrophysics Data System (ADS)

Saremi, Amin; Stenfelt, Stefan; Verhulst, Sarah

2015-12-01

The bottom-up signal pathway, which starts from the outer ear and leads to the brain cortices, gives the classic image of the human sound perception. However, there have been growing evidences in the last six decades for existence of a functional descending network whereby the central auditory system can modulate the early auditory processing, in a top-down manner. The medial olivocochlear efferent fibers project from the superior olivary complex at the brainstem into the inner ear. They are linked to the basal poles of the hair cells by forming synaptic cisterns. This descending network can activate nicotinic cholinergic receptors (nAChR) that increase the membrane conductance of the outer hair cells and thereby modify the magnitude of the active force generated inside the cochlea. The aim of the presented work is to quantitatively investigate how the changes in the biomechanics of the outer hair cells, caused by the efferent activation, manipulate the cochlear responses. This is done by means of a frequency-domain biophysical model of the cochlea [12] where the parameters of the model convey physiological interpretations of the human cochlear structures. The simulations manifest that a doubling of the outer hair cell conductance, due to efferent activation, leads to a frequency-dependent gain reduction along the cochlear duct with its highest effect at frequencies between 1 kHz and 3.5 kHz and a maximum of approximately 10 dB gain reduction at 2 kHz. This amount of the gain inhibition and its frequency dependence reasonably agrees with the experimental data recorded from guinea pig, cat and human cochleae where the medial olivococlear efferents had been elicited by broad-band stimuli. The simulations also indicate that the efferent-induced increase of the outer hair cell conductance increases the best frequency of the cochlear responses, in the basal region. The presented simulations quantitatively confirm that activation of the medial olivocochlear efferents can

Outcomes and special considerations of cochlear implantation in waardenburg syndrome.

PubMed

Kontorinis, Georgios; Lenarz, Thomas; Giourgas, Alexandros; Durisin, Martin; Lesinski-Schiedat, Anke

2011-08-01

The objective of this study was a state-of-the-art analysis of cochlear implantation in patients with Waardenburg syndrome (WS). Twenty-five patients with WS treated with cochlear implants in our department from 1990 to 2010. The 25 patients with WS underwent 35 cochlear implantations. Hearing outcome was evaluated using HSM sentence test in 65 dB in quiet, Freiburg Monosyllabic Test, and categories of auditory performance for children and compared with that of a control group. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging and computed tomography of the temporal bones. The mean follow-up time was 8.3 years (range, 0.3-18.3 yr). The majority achieved favorable postimplantation performance with mean HSM scores of 75.3% (range, 22.6%-99%) and Freiburg Monosyllabic Test scores of 67.8% (range, 14%-95%). However, in 4 cases, the results were less satisfactory. The comparison with the control group did not reveal any statistical significance (p = 0.56). In 6 patients (24%), behavioral disorders caused temporary difficulties during the rehabilitation procedure. Except of isolated large vestibule in 1 patient, the radiological assessment of the 50 temporal bones did not reveal any temporal bone abnormalities. Most patients with WS performed well with cochlear implants. However, WS is related to behavioral disorders that may cause temporary rehabilitation difficulties. Finally, temporal bone malformations that could affect cochlear implantation are notcharacteristic of WS.

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.

Post-treatment effects of local GDNF administration to the inner ears of deafened guinea pigs.

PubMed

Fransson, Anette; Maruyama, Jun; Miller, Josef M; Ulfendahl, Mats

2010-09-01

For patients with profound hearing loss, a cochlear implant is the only treatment available today. The function of a cochlear implant depends in part on the function and survival of spiral ganglion neurons. Following deafferentation, glial cell-derived neurotrophic factor (GDNF) is known to affect spiral ganglion neuron survival. The purpose of this study was to assess delayed GDNF treatment after deafening, the effects of cessation of GDNF treatment, and the effects of subsequent antioxidants on responsiveness and survival of the spiral ganglion neurons. Three-week deafened (by local neomycin administration) guinea pigs were implanted in the scala tympani with a combined cochlear implant electrode and cannula. GDNF (1 μg/mL) or artificial perilymph was then delivered for 4 weeks, following which the animals received systemic ascorbic acid  +  Trolox or saline for an additional 4 weeks. Thresholds for electrically-evoked auditory brain stem responses (eABRs) were significantly elevated at 3 weeks with deafness, stabilized with GDNF, and showed no change with GDNF cessation and treatment with antioxidants or saline. The populations of spiral ganglion neurons were reduced with deafness (by 40% at 3 weeks and 70% at 11 weeks), and rescued from cell death by GDNF with no further reduction at 8 weeks following 4 weeks of cessation of GDNF treatment equally in both the antioxidant- and saline-treated groups. Local growth factor treatment of the deaf ear may prevent deterioration in electrical responsiveness and rescue auditory nerve cells from death; these effects outlast the period of treatment, and may enhance the benefits of cochlear implant therapy for the deaf.

Physiopathology of the cochlear microcirculation.

PubMed

Shi, Xiaorui

2011-12-01

Normal blood supply to the cochlea is critically important for establishing the endocochlear potential and sustaining production of endolymph. Abnormal cochlear microcirculation has long been considered an etiologic factor in noise-induced hearing loss, age-related hearing loss (presbycusis), sudden hearing loss or vestibular function, and Meniere's disease. Knowledge of the mechanisms underlying the pathophysiology of cochlear microcirculation is of fundamental clinical importance. A better understanding of cochlear blood flow (CoBF) will enable more effective management of hearing disorders resulting from aberrant blood flow. This review focuses on recent discoveries and findings related to the physiopathology of the cochlear microvasculature. Published by Elsevier B.V.

Cochlear implantation outcomes in children with common cavity deformity; a retrospective study.

PubMed

Zhang, Li; Qiu, Jianxin; Qin, Feifei; Zhong, Mei; Shah, Gyanendra

2017-09-01

A common cavity deformity (CCD) is a deformed inner ear in which the cochlea and vestibule are confluent forming a common rudimentary cystic cavity that results in profound hearing loss. There are few studies paying attention to common cavity. Our group is engrossed in observing the improvement of auditory and verbal abilities in children who have received cochlear implantation (CI), and comparing these targets between children with common cavity and normal inner ear structure. A retrospective study was conducted in 12 patients with profound hearing loss that were divided into a common cavity group and a control group, six in each group matched in sex, age and time of implantation, based on inner ear structure. Categories of Auditory Performance (CAP) and speech intelligibility rating (SIR) scores and aided hearing thresholds were collected and compared between the two groups. All patients wore CI for more than 1 year at the Cochlear Center of Anhui Medical University from 2011 to 2015. Postoperative CAP and SIR scores were higher than before operation in both groups (p < 0.05), although the scores were lower in the CCD group than in the control group (p < 0.05). The aided threshold was also lower in the control group than in the CCD group (p < 0.05). Even though audiological improvement in children with CCD was not as good as in those without CCD, CI provides benefits in auditory perception and communication skills in these children.

Early postnatal virus inoculation into the scala media achieved extensive expression of exogenous green fluorescent protein in the inner ear and preserved auditory brainstem response thresholds.

PubMed

Wang, Yunfeng; Sun, Yu; Chang, Qing; Ahmad, Shoeb; Zhou, Binfei; Kim, Yeunjung; Li, Huawei; Lin, Xi

2013-01-01

Gene transfer into the inner ear is a promising approach for treating sensorineural hearing loss. The special electrochemical environment of the scala media raises a formidable challenge for effective gene delivery at the same time as keeping normal cochlear function intact. The present study aimed to define a suitable strategy for preserving hearing after viral inoculation directly into the scala media performed at various postnatal developmental stages. We assessed transgene expression of green fluorescent protein (GFP) mediated by various types of adeno-associated virus (AAV) and lentivirus (LV) in the mouse cochlea. Auditory brainstem responses were measured 30 days after inoculation to assess effects on hearing. Patterns of GFP expression confirmed extensive exogenous gene expression in various types of cells lining the endolymphatic space. The use of different viral vectors and promoters resulted in specific cellular GFP expression patterns. AAV2/1 with cytomegalovirus promoter apparently gave the best results for GFP expression in the supporting cells. Histological examination showed normal cochlear morphology and no hair cell loss after either AAV or LV injections. We found that hearing thresholds were not significantly changed when the injections were performed in mice younger than postnatal day 5, regardless of the type of virus tested. Viral inoculation and expression in the inner ear for the restoration of hearing must not damage cochlear function. Using normal hearing mice as a model, we have achieved this necessary step, which is required for the treatment of many types of congenital deafness that require early intervention. Copyright © 2013 John Wiley & Sons, Ltd.

Advances in Cochlear Implant Telemetry: Evoked Neural Responses, Electrical Field Imaging, and Technical Integrity

PubMed Central

Mens, Lucas H. M.

2007-01-01

During the last decade, cochlear implantation has evolved into a well-established treatment of deafness, predominantly because of many improvements in speech processing and the controlled excitation of the auditory nerve. Cochlear implants now also feature telemetry, which is highly useful to monitor the proper functioning of the implanted electronics and electrode contacts. Telemetry can also support the clinical management in young children and difficult cases where neural unresponsiveness is suspected. This article will review recent advances in the telemetry of the electrically evoked compound action potential that have made these measurements simple and routine procedures in most cases. The distribution of the electrical stimulus itself sampled by “electrical field imaging” reveals general patterns of current flow in the normal cochlea and gross abnormalities in individual patients; models have been developed to derive more subtle insights from an individual electrical field imaging. Finally, some thoughts are given to the extended application of telemetry, for example, in monitoring the neural responses or in combination with other treatments of the deaf ear. PMID:17709572

Localization and proteomic characterization of cholesterol-rich membrane microdomains in the inner ear.

PubMed

Thomas, Paul V; Cheng, Andrew L; Colby, Candice C; Liu, Liqian; Patel, Chintan K; Josephs, Lydia; Duncan, R Keith

2014-05-30

Biological membranes organize and compartmentalize cell signaling into discrete microdomains, a process that often involves stable, cholesterol-rich platforms that facilitate protein-protein interactions. Polarized cells with distinct apical and basolateral cell processes rely on such compartmentalization to maintain proper function. In the cochlea, a variety of highly polarized sensory and non-sensory cells are responsible for the early stages of sound processing in the ear, yet little is known about the mechanisms that traffic and organize signaling complexes within these cells. We sought to determine the prevalence, localization, and protein composition of cholesterol-rich lipid microdomains in the cochlea. Lipid raft components, including the scaffolding protein caveolin and the ganglioside GM1, were found in sensory, neural, and glial cells. Mass spectrometry of detergent-resistant membrane (DRM) fractions revealed over 600 putative raft proteins associated with subcellular localization, trafficking, and metabolism. Among the DRM constituents were several proteins involved in human forms of deafness including those involved in ion homeostasis, such as the potassium channel KCNQ1, the co-transporter SLC12A2, and gap junction proteins GJA1 and GJB6. The presence of caveolin in the cochlea and the abundance of proteins in cholesterol-rich DRM suggest that lipid microdomains play a significant role in cochlear physiology. Although mechanisms underlying cholesterol synthesis, homeostasis, and compartmentalization in the ear are poorly understood, there are several lines of evidence indicating that cholesterol is a key modulator of cochlear function. Depletion of cholesterol in mature sensory cells alters calcium signaling, changes excitability during development, and affects the biomechanical processes in outer hair cells that are responsible for hearing acuity. More recently, we have established that the cholesterol-modulator beta-cyclodextrin is capable of

Early uneven ear input induces long-lasting differences in left-right motor function.

PubMed

Antoine, Michelle W; Zhu, Xiaoxia; Dieterich, Marianne; Brandt, Thomas; Vijayakumar, Sarath; McKeehan, Nicholas; Arezzo, Joseph C; Zukin, R Suzanne; Borkholder, David A; Jones, Sherri M; Frisina, Robert D; Hébert, Jean M

2018-03-01

How asymmetries in motor behavior become established normally or atypically in mammals remains unclear. An established model for motor asymmetry that is conserved across mammals can be obtained by experimentally inducing asymmetric striatal dopamine activity. However, the factors that can cause motor asymmetries in the absence of experimental manipulations to the brain remain unknown. Here, we show that mice with inner ear dysfunction display a robust left or right rotational preference, and this motor preference reflects an atypical asymmetry in cortico-striatal neurotransmission. By unilaterally targeting striatal activity with an antagonist of extracellular signal-regulated kinase (ERK), a downstream integrator of striatal neurotransmitter signaling, we can reverse or exaggerate rotational preference in these mice. By surgically biasing vestibular failure to one ear, we can dictate the direction of motor preference, illustrating the influence of uneven vestibular failure in establishing the outward asymmetries in motor preference. The inner ear-induced striatal asymmetries identified here intersect with non-ear-induced asymmetries previously linked to lateralized motor behavior across species and suggest that aspects of left-right brain function in mammals can be ontogenetically influenced by inner ear input. Consistent with inner ear input contributing to motor asymmetry, we also show that, in humans with normal ear function, the motor-dominant hemisphere, measured as handedness, is ipsilateral to the ear with weaker vestibular input.

Cochlear Implantation in Patients With CHARGE Syndrome.

PubMed

Rah, Yoon Chan; Lee, Ji Young; Suh, Myung-Whan; Park, Moo Kyun; Lee, Jun Ho; Chang, Sun O; Oh, Seung-Ha

2016-11-01

To determine the optimal surgical approach for cochlear implantation (CI) preoperatively based on the spatial relation of a displaced facial nerve (FN) and middle ear structures and to analyze clinical outcomes of CHARGE syndrome. Facial nerve displacement and associated deviation of inner ear structures were analyzed in 13 patients (17 ears) with CHARGE syndrome who underwent CI. Surgical accessibility through the facial recess was assessed based on anatomical landmarks. Postoperative speech performance and associated clinical characteristics were analyzed. The most consistently identified ear anomalies were semicircular canal aplasia (100%), ossicular anomaly (100%), and vestibular hypoplasia (88%). Facial nerve displacement was found in 77% of cases (anteroinferior: 47%, anterior: 24%, inferior: 6%). The width of available surgical space around facial recess was significantly greater in cases of facial recess approach (2.85 ± 0.9 mm) than those of alternative approach (0.12 ± 0.29 mm, P = .02). Postoperatively, 53% achieved better than category 4 on the categories of auditory perception (CAP) scale. The CAP category was significantly correlated with internal auditory canal diameter (P = .025) and did not differ according to the applied surgical approach. Preoperative determination of surgical accessibility through facial recess would be useful for safe surgical approach, and successful hearing rehabilitation was achievable by applying appropriate surgical approaches. © The Author(s) 2016.

Investigation of middle ear anatomy and function with combined video otoscopy-phase sensitive OCT

PubMed Central

Park, Jesung; Cheng, Jeffrey T.; Ferguson, Daniel; Maguluri, Gopi; Chang, Ernest W.; Clancy, Caitlin; Lee, Daniel J.; Iftimia, Nicusor

2016-01-01

We report the development of a novel otoscopy probe for assessing middle ear anatomy and function. Video imaging and phase-sensitive optical coherence tomography are combined within the same optical path. A sound stimuli channel is incorporated as well to study middle ear function. Thus, besides visualizing the morphology of the middle ear, the vibration amplitude and frequency of the eardrum and ossicles are retrieved as well. Preliminary testing on cadaveric human temporal bone models has demonstrated the capability of this instrument for retrieving middle ear anatomy with micron scale resolution, as well as the vibration of the tympanic membrane and ossicles with sub-nm resolution. PMID:26977336

A Binaural Cochlear Implant Sound Coding Strategy Inspired by the Contralateral Medial Olivocochlear Reflex

PubMed Central

Eustaquio-Martín, Almudena; Stohl, Joshua S.; Wolford, Robert D.; Schatzer, Reinhold; Wilson, Blake S.

2016-01-01

Objectives: In natural hearing, cochlear mechanical compression is dynamically adjusted via the efferent medial olivocochlear reflex (MOCR). These adjustments probably help understanding speech in noisy environments and are not available to the users of current cochlear implants (CIs). The aims of the present study are to: (1) present a binaural CI sound processing strategy inspired by the control of cochlear compression provided by the contralateral MOCR in natural hearing; and (2) assess the benefits of the new strategy for understanding speech presented in competition with steady noise with a speech-like spectrum in various spatial configurations of the speech and noise sources. Design: Pairs of CI sound processors (one per ear) were constructed to mimic or not mimic the effects of the contralateral MOCR on compression. For the nonmimicking condition (standard strategy or STD), the two processors in a pair functioned similarly to standard clinical processors (i.e., with fixed back-end compression and independently of each other). When configured to mimic the effects of the MOCR (MOC strategy), the two processors communicated with each other and the amount of back-end compression in a given frequency channel of each processor in the pair decreased/increased dynamically (so that output levels dropped/increased) with increases/decreases in the output energy from the corresponding frequency channel in the contralateral processor. Speech reception thresholds in speech-shaped noise were measured for 3 bilateral CI users and 2 single-sided deaf unilateral CI users. Thresholds were compared for the STD and MOC strategies in unilateral and bilateral listening conditions and for three spatial configurations of the speech and noise sources in simulated free-field conditions: speech and noise sources colocated in front of the listener, speech on the left ear with noise in front of the listener, and speech on the left ear with noise on the right ear. In both bilateral and

A Binaural Cochlear Implant Sound Coding Strategy Inspired by the Contralateral Medial Olivocochlear Reflex.

PubMed

Lopez-Poveda, Enrique A; Eustaquio-Martín, Almudena; Stohl, Joshua S; Wolford, Robert D; Schatzer, Reinhold; Wilson, Blake S

2016-01-01

In natural hearing, cochlear mechanical compression is dynamically adjusted via the efferent medial olivocochlear reflex (MOCR). These adjustments probably help understanding speech in noisy environments and are not available to the users of current cochlear implants (CIs). The aims of the present study are to: (1) present a binaural CI sound processing strategy inspired by the control of cochlear compression provided by the contralateral MOCR in natural hearing; and (2) assess the benefits of the new strategy for understanding speech presented in competition with steady noise with a speech-like spectrum in various spatial configurations of the speech and noise sources. Pairs of CI sound processors (one per ear) were constructed to mimic or not mimic the effects of the contralateral MOCR on compression. For the nonmimicking condition (standard strategy or STD), the two processors in a pair functioned similarly to standard clinical processors (i.e., with fixed back-end compression and independently of each other). When configured to mimic the effects of the MOCR (MOC strategy), the two processors communicated with each other and the amount of back-end compression in a given frequency channel of each processor in the pair decreased/increased dynamically (so that output levels dropped/increased) with increases/decreases in the output energy from the corresponding frequency channel in the contralateral processor. Speech reception thresholds in speech-shaped noise were measured for 3 bilateral CI users and 2 single-sided deaf unilateral CI users. Thresholds were compared for the STD and MOC strategies in unilateral and bilateral listening conditions and for three spatial configurations of the speech and noise sources in simulated free-field conditions: speech and noise sources colocated in front of the listener, speech on the left ear with noise in front of the listener, and speech on the left ear with noise on the right ear. In both bilateral and unilateral listening

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.

A missense mutation in myosin VIIA prevents aminoglycoside accumulation in early postnatal cochlear hair cells.

PubMed

Richardson, G P; Forge, A; Kros, C J; Marcotti, W; Becker, D; Williams, D S; Thorpe, J; Fleming, J; Brown, S D; Steel, K P

1999-11-28

Myosin VIIA is expressed by sensory hair cells in the inner ear and proximal tubule cells in the kidney, the two primary targets of aminoglycoside antibiotics. Using cochlear cultures prepared from early postnatal Myo7a6J mice with a missense mutation in the head region of the myosin VIIA molecule we show that this myosin is required for aminoglycoside accumulation in cochlear hair cells. Hair cells in homozygous mutant Myo7a6J cochlear cultures have disorganized hair bundles, but are otherwise morphologically normal and transduce. However, and in contrast to hair cells from heterozygous Myo7a6J cultures, the homozygous Myo7a6J hair cells do not accumulate [3H]gentamicin and do not exhibit an ototoxic response on exposure to aminoglycoside. Possible roles for myosin VIIA in the process of aminoglycoside accumulation are discussed.

Electric-acoustic pitch comparisons in single-sided-deaf cochlear implant users: frequency-place functions and rate pitch.

PubMed

Schatzer, Reinhold; Vermeire, Katrien; Visser, Daniel; Krenmayr, Andreas; Kals, Mathias; Voormolen, Maurits; Van de Heyning, Paul; Zierhofer, Clemens

2014-03-01

Eight cochlear implant users with near-normal hearing in their non-implanted ear compared pitch percepts for pulsatile electric and acoustic pure-tone stimuli presented to the two ears. Six subjects were implanted with a 31-mm MED-EL FLEX(SOFT) electrode, and two with a 24-mm medium (M) electrode, with insertion angles of the most apical contacts ranging from 565° to 758°. In the first experiment, frequency-place functions were derived from pure-tone matches to 1500-pps unmodulated pulse trains presented to individual electrodes and compared to Greenwood's frequency position map along the organ of Corti. While the overall median downward shift of the obtained frequency-place functions (-0.16 octaves re. Greenwood) and the mean shifts in the basal (<240°; -0.33 octaves) and middle (-0.35 octaves) regions were statistically significant, the shift in the apical region (>480°; 0.26 octaves) was not. Standard deviations of frequency-place functions were approximately half an octave at electrode insertion angles below 480°, increasing to an octave at higher angular locations while individual functions were gradually leveling off. In a second experiment, subjects matched the rates of unmodulated pulse trains presented to individual electrodes in the apical half of the array to low-frequency pure tones between 100 Hz and 450 Hz. The aim was to investigate the influence of electrode place on the salience of temporal pitch cues, for coding strategies that present temporal fine structure information via rate modulations on select apical channels. Most subjects achieved reliable matches to tone frequencies from 100 Hz to 300 Hz only on electrodes at angular insertion depths beyond 360°, while rate-matches to 450-Hz tones were primarily achieved on electrodes at shallower insertion angles. Only for electrodes in the second turn the average slopes of rate-pitch functions did not differ significantly from the pure-tone references, suggesting their use for the encoding

The Association between Cardiovascular Disease and Cochlear Function in Older Adults

ERIC Educational Resources Information Center

Torre, Peter, III; Cruickshanks, Karen J.; Klein, Barbara E.K.; Klein, Ronald; Nondahl, David M.

2005-01-01

The purpose of this research was to evaluate the relation between self-reported cardiovascular disease (CVD) and cochlear function in older adults. The Epidemiology of Hearing Loss Study (EHLS) is an ongoing population-based study of hearing loss and its risk factors in Beaver Dam, Wisconsin. As part of the EHLS questionnaire, participants were…

Frequency specificity and left-ear advantage of medial olivocochlear efferent modulation: a study based on stimulus frequency otoacoustic emission.

PubMed

Xing, Dongjia; Gong, Qin

2017-09-06

The medial olivocochlear (MOC) bundle is an auditory nucleus that projects efferent nerve fibers to the outer hair cells (OHCs) for synaptic innervation. The aim of the present study was to investigate the possible existence of frequency and ear specificity in MOC efferent modulation, as well as how MOC activation influences cochlear tuning. Stimulus frequency otoacoustic emissions (SFOAEs) were used to study MOC efferent modulation. Therefore, the current experiment was designed to compare the degree of SFOAE suppression in the both ears of 20 individuals at 1, 2, 4, and 8 kHz. We also compared changes in Q10 values of SFOAE suppression tuning curves at 1, 2, and 4 kHz under contralateral acoustic stimulation (CAS) and no-CAS conditions. We observed a significant reduction in SFOAE magnitude in the CAS condition compared with the no-CAS condition at 1 and 2 kHz in the left ear. A significant difference in CAS suppression was also found between the left and right ears at 1 and 2 kHz, with larger CAS suppression in the left ear. CAS further produced a statistically significant increase in the Q10 value at 1 kHz and a significant reduction in Q10 values at 2 and 4 kHz. These findings suggest a left-ear advantage in terms of CAS-induced MOC efferent SFOAE suppression, with larger MOC efferent modulation for lower frequencies, and cochlear tuning was sharpened by means of MOC activation at lower frequencies and broadened at higher frequencies.

An image guidance system for positioning robotic cochlear implant insertion tools

NASA Astrophysics Data System (ADS)

Bruns, Trevor L.; Webster, Robert J.

2017-03-01

Cochlear implants must be inserted carefully to avoid damaging the delicate anatomical structures of the inner ear. This has motivated several approaches to improve the safety and efficacy of electrode array insertion by automating the process with specialized robotic or manual insertion tools. When such tools are used, they must be positioned at the entry point to the cochlea and aligned with the desired entry vector. This paper presents an image guidance system capable of accurately positioning a cochlear implant insertion tool. An optical tracking system localizes the insertion tool in physical space while a graphical user interface incorporates this with patient- specific anatomical data to provide error information to the surgeon in real-time. Guided by this interface, novice users successfully aligned the tool with an mean accuracy of 0.31 mm.

Specification of absorbed-sound power in the ear canal: Application to suppression of stimulus frequency otoacoustic emissions

PubMed Central

Keefe, Douglas H.; Schairer, Kim S.

2011-01-01

An insert ear-canal probe including sound source and microphone can deliver a calibrated sound power level to the ear. The aural power absorbed is proportional to the product of mean-squared forward pressure, ear-canal area, and absorbance, in which the sound field is represented using forward (reverse) waves traveling toward (away from) the eardrum. Forward pressure is composed of incident pressure and its multiple internal reflections between eardrum and probe. Based on a database of measurements in normal-hearing adults from 0.22 to 8 kHz, the transfer-function level of forward relative to incident pressure is boosted below 0.7 kHz and within 4 dB above. The level of forward relative to total pressure is maximal close to 4 kHz with wide variability across ears. A spectrally flat incident-pressure level across frequency produces a nearly flat absorbed power level, in contrast to 19 dB changes in pressure level. Calibrating an ear-canal sound source based on absorbed power may be useful in audiological and research applications. Specifying the tip-to-tail level difference of the suppression tuning curve of stimulus frequency otoacoustic emissions in terms of absorbed power reveals increased cochlear gain at 8 kHz relative to the level difference measured using total pressure. PMID:21361437

Distinct capacity for differentiation to inner ear cell types by progenitor cells of the cochlea and vestibular organs

PubMed Central

McLean, Will J.; McLean, Dalton T.; Eatock, Ruth Anne

2016-01-01

Disorders of hearing and balance are most commonly associated with damage to cochlear and vestibular hair cells or neurons. Although these cells are not capable of spontaneous regeneration, progenitor cells in the hearing and balance organs of the neonatal mammalian inner ear have the capacity to generate new hair cells after damage. To investigate whether these cells are restricted in their differentiation capacity, we assessed the phenotypes of differentiated progenitor cells isolated from three compartments of the mouse inner ear – the vestibular and cochlear sensory epithelia and the spiral ganglion – by measuring electrophysiological properties and gene expression. Lgr5+ progenitor cells from the sensory epithelia gave rise to hair cell-like cells, but not neurons or glial cells. Newly created hair cell-like cells had hair bundle proteins, synaptic proteins and membrane proteins characteristic of the compartment of origin. PLP1+ glial cells from the spiral ganglion were identified as neural progenitors, which gave rise to neurons, astrocytes and oligodendrocytes, but not hair cells. Thus, distinct progenitor populations from the neonatal inner ear differentiate to cell types associated with their organ of origin. PMID:27789624

Development of a finite element model of the middle ear.

PubMed

Williams, K R; Blayney, A W; Rice, H J

1996-01-01

A representative finite element model of the healthy ear is developed commencing with a description of the decoupled isotropic tympanic membrane. This model was shown to vibrate in a manner similar to that found both numerically (1, 2) and experimentally (8). The introduction of a fibre system into the membrane matrix significantly altered the modes of vibration. The first mode "remains as a piston like movement as for the isotropic membrane. However, higher modes show a simpler vibration pattern similar to the second mode but with a varying axis of movement and lower amplitudes. The introduction of a malleus and incus does not change the natural frequencies or mode shapes of the membrane for certain support conditions. When constraints are imposed along the ossicular chain by simulation of a cochlear impedance term then significantly altered modes can occur. More recently a revised model of the ear has been developed by the inclusion of the outer ear canal. This discretisation uses geometries extracted from a Nuclear Magnetic resonance scan of a healthy subject and a crude inner ear model using stiffness parameters ultimately fixed through a parameter tuning process. The subsequently tuned model showed behaviour consistent with previous findings and should provide a good basis for subsequent modelling of diseased ears and assessment of the performance of middle ear prostheses.

Comparison of forward (ear-canal) and reverse (round-window) sound stimulation of the cochlea.

PubMed

Stieger, Christof; Rosowski, John J; Nakajima, Hideko Heidi

2013-07-01

The cochlea is normally driven with "forward" stimulation, in which sound is introduced to the ear canal. Alternatively, the cochlea can be stimulated at the round window (RW) using an actuator. During RW "reverse" stimulation, the acoustic flow starting at the RW does not necessarily take the same path as during forward stimulation. To understand the differences between forward and reverse stimulation, we measured ear-canal pressure, stapes velocity, RW velocity, and intracochlear pressures in scala vestibuli (SV) and scala tympani (ST) of fresh human temporal bones. During forward stimulation, the cochlear drive (differential pressure across the partition) results from the large difference in magnitude between the pressures of SV and ST, which occurs due to the high compliance of the RW. During reverse stimulation, the relatively high impedance of the middle ear causes the pressures of SV and ST to have similar magnitudes, and the differential pressure results primarily from the difference in phase of the pressures. Furthermore, the sound path differs between forward and reverse stimulation, such that motion through a third window is more significant during reverse stimulation. Additionally, we determined that although stapes velocity is a good estimate of cochlear drive during forward stimulation, it is not a good measure during reverse stimulation. This article is part of a special issue entitled "MEMRO 2012". Copyright © 2012 Elsevier B.V. All rights reserved.

Improvement in cochlear flow in patients with tinnitus with the complex supplement Acustop: a product evaluation.

PubMed

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

2011-09-01

Patients with tinnitus constitute a very large group without a real, specific therapeutic solution. With noninvasive, color duplex it is possible to measure flow in the cochlear artery and to follow duplex flow changes due to treatments in most patients. The aim of this preliminary evaluation was to study flow variations in patients with "mild-to-moderate" tinnitus, possibly associated to cochlear hypo-perfusion, after administration of Acustop (used as a food supplement). The aim was to improve cochlear flow decreasing the level of tinnitus. Patients with "mild-to-moderate", "idiopathic", monolateral tinnitus, present for at least 4 weeks were included; no vertigo or important hearing loss had been observed. The origin of tinnitus had been sudden (hours or days). The tinnitus was associated to a decrease in cochlear flow measured by color Duplex at the affected ear. A group of 42 patients was evaluated; 25 used Acustop; there were 17 controls (follow-up only). Groups were comparable for their clinical problem and other details. The average duration of treatment was 4 weeks. No side effects were observed and no drop-outs were recorded. Flow velocity at the level of the affected inner ear was significantly lower (both the diastolic and systolic components; P<0.05) in comparison with the other ear. This was considered an indication of the vascular origin of the tinnitus. With Acustop treatment there was a significant improvement in systolic (P<0.05) and diastolic flow velocity (P<0.05). The increase in flow velocity was not significant in controls. An analogue scale line was used to measure symptoms in the Acustop group: it was 8.2;2 at inclusion; it decreased to 3.1;1.5 at 4 weeks (P<0.05). The score was 8.4;2 in controls at inclusion; at 4 weeks the score was 7.1;2.2 (not significant). Tinnitus scale: the value at inclusion of the tinnitus scale in the Acustop group a was 8.5;1.1 versus 8.3;1.2 in controls. After 4 weeks the score was 3.1;1.1 (P<0.05) in the

Basilar-membrane interference patterns from multiple internal reflection of cochlear traveling waves.

PubMed

Shera, Christopher A; Cooper, Nigel P

2013-04-01

At low stimulus levels, basilar-membrane (BM) mechanical transfer functions in sensitive cochleae manifest a quasiperiodic rippling pattern in both amplitude and phase. Analysis of the responses of active cochlear models suggests that the rippling is a mechanical interference pattern created by multiple internal reflection within the cochlea. In models, the interference arises when reverse-traveling waves responsible for stimulus-frequency otoacoustic emissions (SFOAEs) reflect off the stapes on their way to the ear canal, launching a secondary forward-traveling wave that combines with the primary wave produced by the stimulus. Frequency-dependent phase differences between the two waves then create the rippling pattern measurable on the BM. Measurements of BM ripples and SFOAEs in individual chinchilla ears demonstrate that the ripples are strongly correlated with the acoustic interference pattern measured in ear-canal pressure, consistent with a common origin involving the generation of SFOAEs. In BM responses to clicks, the ripples appear as temporal fine structure in the response envelope (multiple lobes, waxing and waning). Analysis of the ripple spacing and response phase gradients provides a test for the role of fast- and slow-wave modes of reverse energy propagation within the cochlea. The data indicate that SFOAE delays are consistent with reverse slow-wave propagation but much too long to be explained by fast waves.

Adult Human Nasal Mesenchymal-Like Stem Cells Restore Cochlear Spiral Ganglion Neurons After Experimental Lesion

PubMed Central

Bas, Esperanza; Van De Water, Thomas R.; Lumbreras, Vicente; Rajguru, Suhrud; Goss, Garrett; Hare, Joshua M.

2014-01-01

A loss of sensory hair cells or spiral ganglion neurons from the inner ear causes deafness, affecting millions of people. Currently, there is no effective therapy to repair the inner ear sensory structures in humans. Cochlear implantation can restore input, but only if auditory neurons remain intact. Efforts to develop stem cell-based treatments for deafness have demonstrated progress, most notably utilizing embryonic-derived cells. In an effort to bypass limitations of embryonic or induced pluripotent stem cells that may impede the translation to clinical applications, we sought to utilize an alternative cell source. Here, we show that adult human mesenchymal-like stem cells (MSCs) obtained from nasal tissue can repair spiral ganglion loss in experimentally lesioned cochlear cultures from neonatal rats. Stem cells engraft into gentamicin-lesioned organotypic cultures and orchestrate the restoration of the spiral ganglion neuronal population, involving both direct neuronal differentiation and secondary effects on endogenous cells. As a physiologic assay, nasal MSC-derived cells engrafted into lesioned spiral ganglia demonstrate responses to infrared laser stimulus that are consistent with those typical of excitable cells. The addition of a pharmacologic activator of the canonical Wnt/β-catenin pathway concurrent with stem cell treatment promoted robust neuronal differentiation. The availability of an effective adult autologous cell source for inner ear tissue repair should contribute to efforts to translate cell-based strategies to the clinic. PMID:24172073

Of mice, moles and guinea pigs: functional morphology of the middle ear in living mammals.

PubMed

Mason, Matthew J

2013-07-01

The middle ear apparatus varies considerably among living mammals. Body size, phylogeny and acoustic environment all play roles in shaping ear structure and function, but experimental studies aimed ultimately at improving our understanding of human hearing can sometimes overlook these important species differences. This review focuses on three groups of mammals, bringing together anatomical, zoological and physiological information in order to highlight unusual features of their middle ears and attempt to interpret their function. "Microtype" ears, found in species such as mice and bats, are associated with high-frequency hearing. The orbicular apophysis, the focus of some recent developmental studies on mouse ears, is characteristic of microtype mallei but is not found in humans or other "freely mobile" species. The apophysis increases ossicular inertia about the anatomical axis of rotation: its adaptive purpose in a high-frequency ear is still not clear. Subterranean mammals have convergently evolved a "freely mobile" ossicular morphology which appears to favour lower-frequency sound transmission. More unusual features found in some of these animals include acoustically coupled middle ear cavities, the loss of middle ear muscles and hypertrophied ossicles which are believed to subserve a form of inertial bone conduction. Middle ears of the rodent group Ctenohystrica (which includes guinea pigs and chinchillas, important models in hearing research) show some striking characteristics which together comprise a unique type of auditory apparatus requiring a classification of its own, referred to here as the "Ctenohystrica type". These characteristics include a distinctive malleus morphology, fusion of the malleus and incus, reduction or loss of the stapedius muscle, a synovial stapedio-vestibular articulation and, in chinchillas, enormously expanded middle ear cavities. These characteristics may be functionally linked and associated with the excellent low

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

Exploring the Relationship between Physiological Measures of Cochlear and Brainstem Function

PubMed Central

Dhar, S.; Abel, R.; Hornickel, J.; Nicol, T.; Skoe, E.; Zhao, W.; Kraus, N.

2009-01-01

Objective Otoacoustic emissions and the speech-evoked auditory brainstem response are objective indices of peripheral auditory physiology and are used clinically for assessing hearing function. While each measure has been extensively explored, their interdependence and the relationships between them remain relatively unexplored. Methods Distortion product otoacoustic emissions (DPOAE) and speech-evoked auditory brainstem responses (sABR) were recorded from 28 normal-hearing adults. Through correlational analyses, DPOAE characteristics were compared to measures of sABR timing and frequency encoding. Data were organized into two DPOAE (Strength and Structure) and five brainstem (Onset, Spectrotemporal, Harmonics, Envelope Boundary, Pitch) composite measures. Results DPOAE Strength shows significant relationships with sABR Spectrotemporal and Harmonics measures. DPOAE Structure shows significant relationships with sABR Envelope Boundary. Neither DPOAE Strength nor Structure is related to sABR Pitch. Conclusions The results of the present study show that certain aspects of the speech-evoked auditory brainstem responses are related to, or covary with, cochlear function as measured by distortion product otoacoustic emissions. Significance These results form a foundation for future work in clinical populations. Analyzing cochlear and brainstem function in parallel in different clinical populations will provide a more sensitive clinical battery for identifying the locus of different disorders (e.g., language based learning impairments, hearing impairment). PMID:19346159

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.

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.

Inner ear anatomy in Waardenburg syndrome: radiological assessment and comparison with normative data.

PubMed

Kontorinis, Georgios; Goetz, Friedrich; Lanfermann, Heinrich; Luytenski, Stefan; Giesemann, Anja M

2014-08-01

As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations. A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values. Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others. Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Two Ears and Two (or More?) Devices: A Pediatric Case Study of Bilateral Profound Hearing Loss

PubMed Central

Uchanski, Rosalie M.; Davidson, Lisa S.; Quadrizius, Sharon; Reeder, Ruth; Cadieux, Jamie; Kettel, Jerrica; Chole, Richard A.

2009-01-01

Advances in technology and expanding candidacy guidelines have motivated many clinics to consider children with precipitously sloping high-frequency hearing loss as candidates for cochlear implants (CIs). A case study is presented of a pediatric CI patient whose hearing thresholds were preserved within 10 dB of preimplant levels (125–750 Hz) after receiving a fully inserted 31.5-mm electrode array at one ear. The primary goal of this study was to explore the possible benefit of using both a hearing aid (HA) and a CI at one ear while using a HA at the opposite ear. The authors find that although the use of bilateral hearing aids with a CI may only provide a slight benefit, careful attention must be paid to the coordinated fitting of devices, especially at the ear with two devices. PMID:19447765

Two ears and two (or more?) devices: a pediatric case study of bilateral profound hearing loss.

PubMed

Uchanski, Rosalie M; Davidson, Lisa S; Quadrizius, Sharon; Reeder, Ruth; Cadieux, Jamie; Kettel, Jerrica; Chole, Richard A

2009-06-01

Advances in technology and expanding candidacy guidelines have motivated many clinics to consider children with precipitously sloping high-frequency hearing loss as candidates for cochlear implants (CIs). A case study is presented of a pediatric CI patient whose hearing thresholds were preserved within 10 dB of preimplant levels (125-750 Hz) after receiving a fully inserted 31.5-mm electrode array at one ear. The primary goal of this study was to explore the possible benefit of using both a hearing aid (HA) and a CI at one ear while using a HA at the opposite ear. The authors find that although the use of bilateral hearing aids with a CI may only provide a slight benefit, careful attention must be paid to the coordinated fitting of devices, especially at the ear with two devices.

Preserved Acoustic Hearing in Cochlear Implantation Improves Speech Perception

PubMed Central

Sheffield, Sterling W.; Jahn, Kelly; Gifford, René H.

2015-01-01

Background With improved surgical techniques and electrode design, an increasing number of cochlear implant (CI) recipients have preserved acoustic hearing in the implanted ear, thereby resulting in bilateral acoustic hearing. There are currently no guidelines, however, for clinicians with respect to audio-metric criteria and the recommendation of amplification in the implanted ear. The acoustic bandwidth necessary to obtain speech perception benefit from acoustic hearing in the implanted ear is unknown. Additionally, it is important to determine if, and in which listening environments, acoustic hearing in both ears provides more benefit than hearing in just one ear, even with limited residual hearing. Purpose The purposes of this study were to (1) determine whether acoustic hearing in an ear with a CI provides as much speech perception benefit as an equivalent bandwidth of acoustic hearing in the non-implanted ear, and (2) determine whether acoustic hearing in both ears provides more benefit than hearing in just one ear. Research Design A repeated-measures, within-participant design was used to compare performance across listening conditions. Study Sample Seven adults with CIs and bilateral residual acoustic hearing (hearing preservation) were recruited for the study. Data Collection and Analysis Consonant-nucleus-consonant word recognition was tested in four conditions: CI alone, CI + acoustic hearing in the nonimplanted ear, CI + acoustic hearing in the implanted ear, and CI + bilateral acoustic hearing. A series of low-pass filters were used to examine the effects of acoustic bandwidth through an insert earphone with amplification. Benefit was defined as the difference among conditions. The benefit of bilateral acoustic hearing was tested in both diffuse and single-source background noise. Results were analyzed using repeated-measures analysis of variance. Results Similar benefit was obtained for equivalent acoustic frequency bandwidth in either ear. Acoustic

Minimally invasive surgical method to detect sound processing in the cochlear apex by optical coherence tomography

NASA Astrophysics Data System (ADS)

Ramamoorthy, Sripriya; Zhang, Yuan; Petrie, Tracy; Fridberger, Anders; Ren, Tianying; Wang, Ruikang; Jacques, Steven L.; Nuttall, Alfred L.

2016-02-01

Sound processing in the inner ear involves separation of the constituent frequencies along the length of the cochlea. Frequencies relevant to human speech (100 to 500 Hz) are processed in the apex region. Among mammals, the guinea pig cochlear apex processes similar frequencies and is thus relevant for the study of speech processing in the cochlea. However, the requirement for extensive surgery has challenged the optical accessibility of this area to investigate cochlear processing of signals without significant intrusion. A simple method is developed to provide optical access to the guinea pig cochlear apex in two directions with minimal surgery. Furthermore, all prior vibration measurements in the guinea pig apex involved opening an observation hole in the otic capsule, which has been questioned on the basis of the resulting changes to cochlear hydrodynamics. Here, this limitation is overcome by measuring the vibrations through the unopened otic capsule using phase-sensitive Fourier domain optical coherence tomography. The optically and surgically advanced method described here lays the foundation to perform minimally invasive investigation of speech-related signal processing in the cochlea.

Verbal Processing Speed and Executive Functioning in Long-Term Cochlear Implant Users

ERIC Educational Resources Information Center

AuBuchon, Angela M.; Pisoni, David B.; Kronenberger, William G.

2015-01-01

Purpose: The purpose of this study was to report how "verbal rehearsal speed" (VRS), a form of covert speech used to maintain verbal information in working memory, and another verbal processing speed measure, perceptual encoding speed, are related to 3 domains of executive function (EF) at risk in cochlear implant (CI) users: verbal…

A mathematical model of vowel identification by users of cochlear implants

PubMed Central

Sagi, Elad; Meyer, Ted A.; Kaiser, Adam R.; Teoh, Su Wooi; Svirsky, Mario A.

2010-01-01

A simple mathematical model is presented that predicts vowel identification by cochlear implant users based on these listeners’ resolving power for the mean locations of first, second, and∕or third formant energies along the implanted electrode array. This psychophysically based model provides hypotheses about the mechanism cochlear implant users employ to encode and process the input auditory signal to extract information relevant for identifying steady-state vowels. Using one free parameter, the model predicts most of the patterns of vowel confusions made by users of different cochlear implant devices and stimulation strategies, and who show widely different levels of speech perception (from near chance to near perfect). Furthermore, the model can predict results from the literature, such as Skinner, et al. [(1995). Ann. Otol. Rhinol. Laryngol. 104, 307–311] frequency mapping study, and the general trend in the vowel results of Zeng and Galvin’s [(1999). Ear Hear. 20, 60–74] studies of output electrical dynamic range reduction. The implementation of the model presented here is specific to vowel identification by cochlear implant users, but the framework of the model is more general. Computational models such as the one presented here can be useful for advancing knowledge about speech perception in hearing impaired populations, and for providing a guide for clinical research and clinical practice. PMID:20136228

Surgical considerations and safety of cochlear implantation in otitis media with effusion.

PubMed

Cevizci, Rasit; Dilci, Alper; Celenk, Fatih; Karamert, Recep; Bayazit, Yildirim

2018-06-01

To evaluate the effects of otitis media with effusion on surgical parameters, patient safety, perioperative and postoperative complications. Total 890 children who underwent cochlear implantation between 2006 and 2015 were included. The ages ranged from 12 months to 63 months (mean: 32 months). The patients were divided into two groups according to the presence or absence of otitis media with effusion; otitis media with effusion group and non-otitis media group. Of 890 children, 105 had otitis media with effusion prior to surgery. In non-otitis media with group, there were 785 children. The average duration of surgery was 60min (ranged from 28 to 75min) in non-otitis media group, and 90min (ranged from 50 to 135min) in otitis media with effusion group (p<0.05). Granulation tissue and edematous middle ear and mastoid mucosa were observed in all cases of otitis media with effusion during the surgery. There was no significant difference between the complications of groups with or without otitis media with effusion (p>0.05). In 5 of 105 patients, there was a ventilation tube inserted before cochlear implantation, which did not change the outcome of implantation. There is no need for surgical treatment for otitis media with effusion before implantation since otitis media with effusion does not increase the risks associated with cochlear implantation. Operation duration is longer in the presence of otitis media with effusion. However, otitis media with effusion leads to intraoperative difficulties like longer operation duration, bleeding, visualization of the round window membrane, cleansing the middle ear granulations as well as mastoid and petrous air cells. Copyright © 2017 Elsevier B.V. All rights reserved.

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…

Characteristics and pathogenesis of facial nerve stimulation after cochlear implant surgeries: A single-center retrospective analysis from 1,151 patients.

PubMed

Kim, Y R; Yoo, M H; Lee, J Y; Yang, C J; Park, J W; Kang, B C; Kang, W S; Ahn, J H; Chung, J W; Park, H J

2018-05-29

Incidence of facial nerve stimulation (FNS) was 2.8% (32 out of 1151) and higher in ears with cochlear anomaly (6.4%, 25 out of 391) than without cochlear anomaly (0.9%, 7 out of 760). FNS occurred at various current levels and locations of electrodes by different mechanisms related to incomplete insertion of electrodes, cochleo-facial dehiscence, and types of cochlear anomalies. FNS at apical electrodes related to cochleo-facial dehiscence with low current levels, and FNS at basal electrodes with high current levels and partial insertion of electrodes. FNS at most electrodes with high current levels was the most common type in patients with a common cavity or narrowing of the bony cochlear nerve canal. Understanding the mechanisms of FNS might provide insight for preventing FNS when performing CI surgeries. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

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

Importance of age and postimplantation experience on speech perception measures in children with sequential bilateral cochlear implants.

PubMed

Peters, B Robert; Litovsky, Ruth; Parkinson, Aaron; Lake, Jennifer

2007-08-01

Clinical trials in which children received bilateral cochlear implants in sequential operations were conducted to analyze the extent to which bilateral implantation offers benefits on a number of measures. The present investigation was particularly focused on measuring the effects of age at implantation and experience after activation of the second implant on speech perception performance. Thirty children aged 3 to 13 years were recipients of 2 cochlear implants, received in sequential operations, a minimum of 6 months apart. All children received their first implant before 5 years of age and had acquired speech perception capabilities with the first device. They were divided into 3 age groups on the basis of age at time of second ear implantation: Group I, 3 to 5 years; Group II, 5.1 to 8 years; and Group III, 8.1 to 13 years. Speech perception measures in quiet included the Multisyllabic Lexical Neighborhood Test (MLNT) for Group I, the Lexical Neighborhood Test (LNT) for Groups II and III, and the Hearing In Noise Test for Children (HINT-C) sentences in quiet for Group III. Speech perception in noise was assessed using the Children's Realistic Intelligibility and Speech Perception (CRISP) test. Testing was performed preoperatively and again postactivation of the second implant at 3, 6, and 12 months (CRISP at 3 and 9 mo) in both the unilateral and bilateral conditions in a repeated-measures study design. Two-way repeated-measures analysis of variance was used to analyze statistical significance among device configurations and performance over time. US Multicenter. Results for speech perception in quiet show that children implanted sequentially acquire open-set speech perception in the second ear relatively quickly (within 6 mo). However, children younger than 8 years do so more rapidly and to a higher level of speech perception ability at 12 months than older children (mean second ear MLNT/LNT scores at 12 months: Group I, 83.9%; range, 71-96%; Group II, 59

Fibro-vascular coupling in the control of cochlear blood flow.

PubMed

Dai, Min; Shi, Xiaorui

2011-01-01

Transduction of sound in the cochlea is metabolically demanding. The lateral wall and hair cells are critically vulnerable to hypoxia, especially at high sound levels, and tight control over cochlear blood flow (CBF) is a physiological necessity. Yet despite the importance of CBF for hearing, consensus on what mechanisms are involved has not been obtained. We report on a local control mechanism for regulating inner ear blood flow involving fibrocyte signaling. Fibrocytes in the super-strial region are spatially distributed near pre-capillaries of the spiral ligament of the albino guinea pig cochlear lateral wall, as demonstrably shown in transmission electron microscope and confocal images. Immunohistochemical techniques reveal the inter-connected fibrocytes to be positive for Na+/K+ ATPase β1 and S100. The connected fibrocytes display more Ca(2+) signaling than other cells in the cochlear lateral wall as indicated by fluorescence of a Ca(2+) sensor, fluo-4. Elevation of Ca(2+) in fibrocytes, induced by photolytic uncaging of the divalent ion chelator o-nitrophenyl EGTA, results in propagation of a Ca(2+) signal to neighboring vascular cells and vasodilation in capillaries. Of more physiological significance, fibrocyte to vascular cell coupled signaling was found to mediate the sound stimulated increase in cochlear blood flow (CBF). Cyclooxygenase-1 (COX-1) was required for capillary dilation. The findings provide the first evidence that signaling between fibrocytes and vascular cells modulates CBF and is a key mechanism for meeting the cellular metabolic demand of increased sound activity.

Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias.

PubMed

Syal, Rajan; Tyagi, Isha; Goyal, Amit

2005-07-01

Cochlear dysplasia associated with defect in stapes footplate can be a cause of cerebrospinal fluid leak. Repair of cerebrospinal fluid leak in these cases is usually done by packing the vestibule with muscle or fascia. This traditional method of repair has 30-60% failure rate. Cerebrospinal fluid leak in four such patients was successfully repaired using multiple layer packing of vestibule, reinforced by pedicle temporalis muscle graft. Intraoperatively continuous lumbar drain was done. Magnetic resonance imaging of inner ear using 3D FSE T2WI and 3D FIESTA sequences was found helpful noninvasive investigation to localize site and route of cerebrospinal fluid leak.

Historical Aspects of Inner Ear Anatomy and Biology that Underlie the Design of Hearing and Balance Prosthetic Devices.

PubMed

Van De Water, Thomas R

2012-11-01

This review presents some of the major historical events that advanced the body of knowledge of the anatomy of the inner ear and its sensory receptors as well as the biology of these receptors that underlies the sensory functions of hearing and balance. This knowledge base of the inner ear's structure/function has been an essential factor for the design and construction of prosthetic devices to aid patients with deficits in their senses of hearing and balance. Prosthetic devices are now available for severely hearing impaired and deaf patients to restore hearing and are known as cochlear implants and auditory brain stem implants. A prosthetic device for patients with balance disorders is being perfected and is in an animal model testing phase with another prosthetic device for controlling intractable dizziness in Meniere's patients currently being evaluated in clinical testing. None of this would have been possible without the pioneering studies and discoveries of the investigators mentioned in this review and with the work of many other talented investigators to numerous to be covered in this review. Copyright © 2012 Wiley Periodicals, Inc.

Improved imaging of cochlear nerve hypoplasia using a 3-Tesla variable flip-angle turbo spin-echo sequence and a 7-cm surface coil.

PubMed

Giesemann, Anja M; Raab, Peter; Lyutenski, Stefan; Dettmer, Sabine; Bültmann, Eva; Frömke, Cornelia; Lenarz, Thomas; Lanfermann, Heinrich; Goetz, Friedrich

2014-03-01

Magnetic resonance imaging of the temporal bone has an important role in decision making with regard to cochlea implantation, especially in children with cochlear nerve deficiency. The purpose of this study was to evaluate the usefulness of the combination of an advanced high-resolution T2-weighted sequence with a surface coil in a 3-Tesla magnetic resonance imaging scanner in cases of suspected cochlear nerve aplasia. Prospective study. Seven patients with cochlear nerve hypoplasia or aplasia were prospectively examined using a high-resolution three-dimensional variable flip-angle turbo spin-echo sequence using a surface coil, and the images were compared with the same sequence in standard resolution using a standard head coil. Three neuroradiologists evaluated the magnetic resonance images independently, rating the visibility of the nerves in diagnosing hypoplasia or aplasia. Eight ears in seven patients with hypoplasia or aplasia of the cochlear nerve were examined. The average age was 2.7 years (range, 9 months-5 years). Seven ears had accompanying malformations. The inter-rater reliability in diagnosing hypoplasia or aplasia was greater using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence (fixed-marginal kappa: 0.64) than with the same sequence in lower resolution (fixed-marginal kappa: 0.06). Examining cases of suspected cochlear nerve aplasia using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence in combination with a surface coil shows significant improvement over standard methods. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

Consequences of Stimulus Type on Higher-Order Processing in Single-Sided Deaf Cochlear Implant Users.

PubMed

Finke, Mareike; Sandmann, Pascale; Bönitz, Hanna; Kral, Andrej; Büchner, Andreas

2016-01-01

Single-sided deaf subjects with a cochlear implant (CI) provide the unique opportunity to compare central auditory processing of the electrical input (CI ear) and the acoustic input (normal-hearing, NH, ear) within the same individual. In these individuals, sensory processing differs between their two ears, while cognitive abilities are the same irrespectively of the sensory input. To better understand perceptual-cognitive factors modulating speech intelligibility with a CI, this electroencephalography study examined the central-auditory processing of words, the cognitive abilities, and the speech intelligibility in 10 postlingually single-sided deaf CI users. We found lower hit rates and prolonged response times for word classification during an oddball task for the CI ear when compared with the NH ear. Also, event-related potentials reflecting sensory (N1) and higher-order processing (N2/N4) were prolonged for word classification (targets versus nontargets) with the CI ear compared with the NH ear. Our results suggest that speech processing via the CI ear and the NH ear differs both at sensory (N1) and cognitive (N2/N4) processing stages, thereby affecting the behavioral performance for speech discrimination. These results provide objective evidence for cognition to be a key factor for speech perception under adverse listening conditions, such as the degraded speech signal provided from the CI. © 2016 S. Karger AG, Basel.

Altered Expression of Middle and Inner Ear Cytokines in Mouse Otitis Media

PubMed Central

MacArthur, Carol J.; Pillers, De-Ann M.; Pang, Jiaqing; Kempton, J. Beth; Trune, Dennis R.

2010-01-01

Objectives/Hypothesis The inner ear is at risk for sensorineural hearing loss in both acute and chronic otitis media (OM), but the underlying mechanisms underlying sensorineural hearing loss are unknown. Previous gene expression array studies showed cytokine genes might be upregulated in the cochleas of mice with acute and chronic otitis media. This implies that the inner ear could manifest a direct inflammatory response to OM that may cause sensorineural damage. Therefore, to better understand inner ear cytokine gene expression during OM, quantitative RT-PCR and immunohistochemistry were performed on mouse models to evaluate middle and inner ear inflammatory and remodeling cytokines. Study Design Basic science experiment. Methods An acute OM model was created in Balb/c mice by a transtympanic injection of S. pneumoniae in one ear; the other ear used as a control. C3H/HeJ mice were screened for unilateral chronic OM with the non-infected ear serving as control. Results Both acute and chronic OM caused both the middle ear and inner tissues in these two mouse models to over express numerous cytokine genes related to tissue remodeling (TNFα, FGF, BMP) and angiogenesis (VEGF), as well as inflammatory cell proliferation (IL-1α,β, IL-2, IL-6). Immunohistochemistry confirmed that both the middle ear and inner ear tissues expressed these cytokines. Conclusion Cochlear tissues are capable of expressing cytokine mRNA that contributes to the inflammation and remodeling that occur in association with middle ear disease. This provides a potential molecular basis for the transient and permanent sensorineural hearing loss often reported with acute and chronic OM. Level of Evidence N/A PMID:21271590

The Relationship Between Intensity Coding and Binaural Sensitivity in Adults With Cochlear Implants.

PubMed

Todd, Ann E; Goupell, Matthew J; Litovsky, Ruth Y

Many bilateral cochlear implant users show sensitivity to binaural information when stimulation is provided using a pair of synchronized electrodes. However, there is large variability in binaural sensitivity between and within participants across stimulation sites in the cochlea. It was hypothesized that within-participant variability in binaural sensitivity is in part affected by limitations and characteristics of the auditory periphery which may be reflected by monaural hearing performance. The objective of this study was to examine the relationship between monaural and binaural hearing performance within participants with bilateral cochlear implants. Binaural measures included dichotic signal detection and interaural time difference discrimination thresholds. Diotic signal detection thresholds were also measured. Monaural measures included dynamic range and amplitude modulation detection. In addition, loudness growth was compared between ears. Measures were made at three stimulation sites per listener. Greater binaural sensitivity was found with larger dynamic ranges. Poorer interaural time difference discrimination was found with larger difference between comfortable levels of the two ears. In addition, poorer diotic signal detection thresholds were found with larger differences between the dynamic ranges of the two ears. No relationship was found between amplitude modulation detection thresholds or symmetry of loudness growth and the binaural measures. The results suggest that some of the variability in binaural hearing performance within listeners across stimulation sites can be explained by factors nonspecific to binaural processing. The results are consistent with the idea that dynamic range and comfortable levels relate to peripheral neural survival and the width of the excitation pattern which could affect the fidelity with which central binaural nuclei process bilateral inputs.

Inner ear dysfunction in caspase-3 deficient mice

PubMed Central

2011-01-01

Background Caspase-3 is one of the most downstream enzymes activated in the apoptotic pathway. In caspase-3 deficient mice, loss of cochlear hair cells and spiral ganglion cells coincide closely with hearing loss. In contrast with the auditory system, details of the vestibular phenotype have not been characterized. Here we report the vestibular phenotype and inner ear anatomy in the caspase-3 deficient (Casp3-/-) mouse strain. Results Average ABR thresholds of Casp3-/- mice were significantly elevated (P < 0.05) compared to Casp3+/- mice and Casp3+/+ mice at 3 months of age. In DPOAE testing, distortion product 2F1-F2 was significantly decreased (P < 0.05) in Casp3-/- mice, whereas Casp3+/- and Casp3+/+ mice showed normal and comparable values to each other. Casp3-/- mice were hyperactive and exhibited circling behavior when excited. In lateral canal VOR testing, Casp3-/- mice had minimal response to any of the stimuli tested, whereas Casp3+/- mice had an intermediate response compared to Casp3+/+ mice. Inner ear anatomical and histological analysis revealed gross hypomorphism of the vestibular organs, in which the main site was the anterior semicircular canal. Hair cell numbers in the anterior- and lateral crista, and utricle were significantly smaller in Casp3-/- mice whereas the Casp3+/- and Casp3+/+ mice had normal hair cell numbers. Conclusions These results indicate that caspase-3 is essential for correct functioning of the cochlea as well as normal development and function of the vestibule. PMID:21988729

A Protocol for Decellularizing Mouse Cochleae for Inner Ear Tissue Engineering.

PubMed

Neal, Christopher A; Nelson-Brantley, Jennifer G; Detamore, Michael S; Staecker, Hinrich; Mellott, Adam J

2018-01-01

In mammals, mechanosensory hair cells that facilitate hearing lack the ability to regenerate, which has limited treatments for hearing loss. Current regenerative medicine strategies have focused on transplanting stem cells or genetic manipulation of surrounding support cells in the inner ear to encourage replacement of damaged stem cells to correct hearing loss. Yet, the extracellular matrix (ECM) may play a vital role in inducing and maintaining function of hair cells, and has not been well investigated. Using the cochlear ECM as a scaffold to grow adult stem cells may provide unique insights into how the composition and architecture of the extracellular environment aids cells in sustaining hearing function. Here we present a method for isolating and decellularizing cochleae from mice to use as scaffolds accepting perfused adult stem cells. In the current protocol, cochleae are isolated from euthanized mice, decellularized, and decalcified. Afterward, human Wharton's jelly cells (hWJCs) that were isolated from the umbilical cord were carefully perfused into each cochlea. The cochleae were used as bioreactors, and cells were cultured for 30 days before undergoing processing for analysis. Decellularized cochleae retained identifiable extracellular structures, but did not reveal the presence of cells or noticeable fragments of DNA. Cells perfused into the cochlea invaded most of the interior and exterior of the cochlea and grew without incident over a duration of 30 days. Thus, the current method can be used to study how cochlear ECM affects cell development and behavior.

Pre- and post-operative dizziness, tinnitus, and taste disturbances among cochlear implant recipients.

PubMed

Mikkelsen, K S; Ovesen, T; Swan, C Z

2017-04-01

To determine the pre- and post-operative prevalence of dizziness, tinnitus and taste disturbances in adult cochlear implant recipients. A questionnaire regarding pre- and post-operative dizziness, tinnitus and taste disturbances was sent to 170 cochlear implant recipients implanted between January 2003 and March 2009. Seventy-seven patients (41 per cent) responded. Pre-operatively, 20 per cent of the participants experienced dizziness, 52 per cent experienced tinnitus and 3 per cent experienced taste disturbances. Post-operative dizziness developed in 46 per cent of patients and resolved in the majority of these; however, 15 per cent reported dizziness more than six months after implantation. Tinnitus worsened in 25 per cent of patients, whereas 73 per cent reported attenuation or termination of tinnitus. Post-operatively, tinnitus developed in 12 per cent and taste disturbances developed in 17 per cent of the patients. The high prevalence of dizziness, tinnitus and taste disturbances reported by cochlear implant recipients necessitates that assessment of symptoms related to inner ear and chorda tympani damage are included when evaluating operative results.

Simultaneous cochlear implantation as a therapeutic option in vestibular schwannoma surgery: case report.

PubMed

Dos Santos Neto, Pedro Helo; Zamponi, Johnni Oswaldo; Hamerschmidt, Rogério; Wiemes, Gislaine Richter Minhoto; Rassi, Marcio S; Borba, Luis A B

2018-03-01

Hearing loss is the most common symptom of vestibular schwannomas (VSs). The management of these lesions includes observation, radiosurgery, and microsurgical resection. Hearing preservation and rehabilitation are the major challenges after the tumor treatment. A 43-year-old man with previous left-sided profound hearing loss and tinnitus presented with a 2-mm left-sided intracanalicular VS. The decision was made to perform a simultaneous cochlear implantation (CI) and microsurgical resection of the tumor. The patient did well postoperatively, with significant improvement of tinnitus, sound localization, and speech recognition in noise. Previous reports of simultaneous CI and VS resection in patients with neurofibromatosis type 2 and sporadic VS in the only hearing ear have been described. The role of CI in patients with VS and normal contralateral hearing has been recently described, showing positive outcomes due to the binaural benefits. Tinnitus also can be treated by the implantation of the cochlear device. The simultaneous microsurgical removal of VS and implantation of a cochlear device is a feasible approach in patients with unilateral hearing loss and severe tinnitus.

Executive Functioning and Speech-Language Skills Following Long-Term Use of Cochlear Implants

ERIC Educational Resources Information Center

Kronenberger, William G.; Colson, Bethany G.; Henning, Shirley C.; Pisoni, David B.

2014-01-01

Neurocognitive processes such as executive functioning (EF) may influence the development of speech-language skills in deaf children after cochlear implantation in ways that differ from normal-hearing, typically developing children. Conversely, spoken language abilities and experiences may also exert reciprocal effects on the development of EF.…

Pediatric cochlear implants: additional disabilities prevalence, risk factors, and effect on language outcomes.

PubMed

Birman, Catherine S; Elliott, Elizabeth J; Gibson, William P R

2012-10-01

To determine the prevalence of additional disabilities in a pediatric cochlear population, to identify medical and radiologic conditions associated with additional disabilities, and to identify the effect of additional disabilities on speech perception and language at 12 months postoperatively. Retrospective case review. Tertiary referral center and cochlear implant program. Records were reviewed for children 0 to 16 years old inclusive, who had cochlear implant-related operations over a 12-month period. Diagnostic and rehabilitative. Additional disabilities prevalence; medical history and radiologic abnormalities; and the effect on Categories of Auditory Performance (CAP) score at 12 months postoperatively. Eighty-eight children having 96 operations were identified. The overall prevalence of additional disabilities (including developmental delay, cerebral palsy, visual impairment, autism and attention deficit disorder) was 33%. The main conditions associated with additional disabilities were syndromes and chromosomal abnormalities (87%), jaundice (86%), prematurity (62%), cytomegalovirus (60%), and inner ear abnormalities including cochlea nerve hypoplasia or aplasia (75%) and semicircular canal anomalies (56%). At 12 months postoperatively, almost all (96%) of the children without additional disabilities had a CAP score of 5 or greater (speech), compared with 52% of children with additional disabilities. Children with developmental delay had a median CAP score of 4, at 12 months compared with 6 for those without developmental delay. Additional disabilities are prevalent in approximately a third of pediatric cochlear implant patients. Additional disabilities significantly affect the outcomes of cochlear implants.

Quantification of solute entry into cochlear perilymph through the round window membrane.

PubMed

Salt, A N; Ma, Y

2001-04-01

The administration of drugs to the inner ear via the round window membrane is becoming more widely used for both clinical and experimental purposes. The actual drug levels achieved in different regions of the inner ear by this method have not been established. The present study has made use of simulations of solute movements in the cochlear fluids to describe the distribution of a marker solute in the guinea pig cochlear fluid spaces. Simulation parameters were derived from experimental measurements using a marker ion, trimethylphenylammonium (TMPA). The distribution of this ion in the cochlea was monitored without volume disturbance using TMPA-selective microelectrodes sealed into the first and second turns of scala tympani (ST). TMPA was applied to perilymph by irrigation of the intact round window membrane with 2 mM solution. At the end of a 90 min application period, TMPA in the first turn, 1.4 mm from the base of ST, reached an average concentration of 330 microM (standard deviation (S.D.) 147 microM, n = 8). TMPA in the second turn, 7.5 mm from the base of ST reached a concentration of 15 microM (S.D. 33 microM, n = 5). The measured time courses of TMPA concentration change were interpreted using the Washington University Cochlear Fluids Simulator (V 1.4), a public-domain program available on the internet at http ://oto.wustl.edu/cochlea/. Simulations with parameters producing concentration time courses comparable to those measured were: (1) round window permeability: 1.9 x 10(-80 cm/s; (2) ST clearance half-time: 60 min; (3) longitudinal perilymph flow rate: 4.4 nl/min, directed from base to apex. Solute concentrations in apical regions of the cochlea were found to be determined primarily by the rate at which the solute diffuses, balanced by the rate of clearance of the solute from perilymph. Longitudinal perilymph flow was not an important factor in solute distribution unless the bony otic capsule was perforated, which rapidly caused substantial changes to

[Functional anatomy of the cochlear nerve and the central auditory system].

PubMed

Simon, E; Perrot, X; Mertens, P

2009-04-01

The auditory pathways are a system of afferent fibers (through the cochlear nerve) and efferent fibers (through the vestibular nerve), which are not limited to a simple information transmitting system but create a veritable integration of the sound stimulus at the different levels, by analyzing its three fundamental elements: frequency (pitch), intensity, and spatial localization of the sound source. From the cochlea to the primary auditory cortex, the auditory fibers are organized anatomically in relation to the characteristic frequency of the sound signal that they transmit (tonotopy). Coding the intensity of the sound signal is based on temporal recruitment (the number of action potentials) and spatial recruitment (the number of inner hair cells recruited near the cell of the frequency that is characteristic of the stimulus). Because of binaural hearing, commissural pathways at each level of the auditory system and integration of the phase shift and the difference in intensity between signals coming from both ears, spatial localization of the sound source is possible. Finally, through the efferent fibers in the vestibular nerve, higher centers exercise control over the activity of the cochlea and adjust the peripheral hearing organ to external sound conditions, thus protecting the auditory system or increasing sensitivity by the attention given to the signal.

Cochlear third window in the scala vestibuli: an animal model.

PubMed

Preis, Michal; Attias, Joseph; Hadar, Tuvia; Nageris, Ben I

2009-08-01

Pathologic third window has been investigated in both animals and humans, with a third window located in the vestibular apparatus, specifically, dehiscence of the superior semicircular canal, serving as the clinical model. The present study sought to examine the effect of a cochlear third window in the scala vestibuli on the auditory thresholds in fat sand rats that have a unique anatomy of the inner ear that allows for easy surgical access. The experiment included 7 healthy 6-month-old fat sand rats (a total of 10 ears). A pathologic third window was induced by drilling a hole in the bony labyrinth over the scala vestibuli, with preservation of the membranous labyrinth. Auditory brainstem responses to high- and low-frequency acoustic stimuli delivered via air and bone conduction were recorded before and after the procedure. In the preoperative auditory brainstem response recordings, air-conduction thresholds (ACTs) to clicks and tone bursts averaged 9 and 10 dB, respectively, and bone-conduction thresholds averaged 4.5 and 2.9 dB, respectively. Postfenestration ACTs averaged 41 and 42.2 dB, and bone-conduction thresholds averaged 1.1 and 4.3 dB. The change in ACT was statistically significant (p < 0.01). The presence of a cochlear third window in the scala vestibuli affects auditory thresholds by causing a decrease in sensitivity to air-conducted sound stimuli. These findings agree with the theoretical model and clinical findings.

Changes in immunostaining of inner ears after antigen challenge into the scala tympani.

PubMed

Ichimiya, I; Kurono, Y; Hirano, T; Mogi, G

1998-04-01

To study the mechanisms of immune responses and immune injuries in inner ears, labyrinthitis was induced by inoculation of keyhole limpet hemocyanin (KLH) into the scala tympani of systemically sensitized guinea pigs. Inner ears were then immunostained for KLH, immunoglobulin G (IgG), albumin, connexin26 (Cx26), and sodium-potassium adenosine triphosphate (Na,K-ATPase). Inflammatory cells containing KLH were observed in the scala tympani and in the collecting venule of the spiral modiolar vein (SMV). Spiral ligament, spiral limbus, and blood vessels including the SMV were diffusely positive for IgG and albumin. Immunoreactivity for Cx26 and Na,K-ATPase was decreased compared with the normal ears in the fibrocytes of the spiral ligament. These results suggest that inflammatory cells and blood constituents could extravasate into the cochlea from blood vessels and that fibrocyte damage in the spiral ligament could cause cochlear dysfunction.

The plastic ear and perceptual relearning in auditory spatial perception

PubMed Central

Carlile, Simon

2014-01-01

The auditory system of adult listeners has been shown to accommodate to altered spectral cues to sound location which presumably provides the basis for recalibration to changes in the shape of the ear over a life time. Here we review the role of auditory and non-auditory inputs to the perception of sound location and consider a range of recent experiments looking at the role of non-auditory inputs in the process of accommodation to these altered spectral cues. A number of studies have used small ear molds to modify the spectral cues that result in significant degradation in localization performance. Following chronic exposure (10–60 days) performance recovers to some extent and recent work has demonstrated that this occurs for both audio-visual and audio-only regions of space. This begs the questions as to the teacher signal for this remarkable functional plasticity in the adult nervous system. Following a brief review of influence of the motor state in auditory localization, we consider the potential role of auditory-motor learning in the perceptual recalibration of the spectral cues. Several recent studies have considered how multi-modal and sensory-motor feedback might influence accommodation to altered spectral cues produced by ear molds or through virtual auditory space stimulation using non-individualized spectral cues. The work with ear molds demonstrates that a relatively short period of training involving audio-motor feedback (5–10 days) significantly improved both the rate and extent of accommodation to altered spectral cues. This has significant implications not only for the mechanisms by which this complex sensory information is encoded to provide spatial cues but also for adaptive training to altered auditory inputs. The review concludes by considering the implications for rehabilitative training with hearing aids and cochlear prosthesis. PMID:25147497

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

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.

Increasing spatial resolution and comparison of MR imaging sequences for the inner ear

NASA Astrophysics Data System (ADS)

Snyder, Carl J.; Bolinger, Lizann; Rubinstein, Jay T.; Wang, Ge

2002-04-01

The size and location of the cochlea and cochlear nerve are needed to assess the feasibility of cochlea implantation, provide information for surgical planning, and aid in construction of cochlear models. Models of implant stimulation incorporating anatomical and physiological information are likely to provide a better understanding of the biophysics of information transferred with cochlear implants and aid in electrode design and arrangement on cochlear implants. Until recently MR did not provide the necessary image resolution and suffered from long acquisition times. The purpose of this study was to optimize both Fast Spin Echo (FSE) and Steady State Free Precession (FIESTA) imaging scan parameters for the inner ear and comparatively examine both for improved image quality and increased spatial resolution. Image quality was determined by two primary measurements, signal to noise ratio (SNR), and image sharpness. Optimized parameters for FSE were 120ms, 3000ms, 64, and 32.25kHz for the TE, TR, echo train length, and bandwidth, respectively. FIESTA parameters were optimized to 2.7, 5.5ms, 70 degree(s), and 62.5kHz, for TE, TR, flip angle, and bandwidth, respectively. While both had the same in-plane spatial resolution, 0.625mm, FIESTA data shows higher SNR per acquisition time and better edge sharpness.

Low-resolution electromagnetic tomography (LORETA) in children with cochlear implants: a preliminary report.

PubMed

Henkin, Yael; Kishon-Rabin, Liat; Tatin-Schneider, Simona; Urbach, Doron; Hildesheimer, Minka; Kileny, Paul R

2004-12-01

The current preliminary report describes the utilization of low-resolution electromagnetic tomography (LORETA) in a small group of highly performing children using the Nucleus 22 cochlear implant (CI) and in normal-hearing (NH) adults. LORETA current density estimations were performed on an averaged target P3 component that was elicited by non-speech and speech oddball discrimination tasks. The results indicated that, when stimulated with tones, patients with right implants and NH adults (regardless of stimulated ear) showed enhanced activation in the right temporal lobe, whereas patients with left implants showed enhanced activation in the left temporal lobe. When stimulated with speech, patients with right implants showed bilateral activation of the temporal and frontal lobes, whereas patients with left implants showed only left temporal lobe activation. NH adults (regardless of stimulated ear) showed enhanced bilateral activation of the temporal and parietal lobes. The differences in activation patterns between patients with CI and NH subjects may be attributed to the long-term exposure to degraded input conditions which may have resulted in reorganization in terms of functional specialization. The difference between patients with right versus left implants, however, is intriguing and requires further investigation.

Auditory Implant Research at the House Ear Institute 1989–2013

PubMed Central

Shannon, Robert V.

2014-01-01

The House Ear Institute (HEI) had a long and distinguished history of auditory implant innovation and development. Early clinical innovations include being one of the first cochlear implant (CI) centers, being the first center to implant a child with a cochlear implant in the US, developing the auditory brainstem implant, and developing multiple surgical approaches and tools for Otology. This paper reviews the second stage of auditory implant research at House – in-depth basic research on perceptual capabilities and signal processing for both cochlear implants and auditory brainstem implants. Psychophysical studies characterized the loudness and temporal perceptual properties of electrical stimulation as a function of electrical parameters. Speech studies with the noise-band vocoder showed that only four bands of tonotopically arrayed information were sufficient for speech recognition, and that most implant users were receiving the equivalent of 8–10 bands of information. The noise-band vocoder allowed us to evaluate the effects of the manipulation of the number of bands, the alignment of the bands with the original tonotopic map, and distortions in the tonotopic mapping, including holes in the neural representation. Stimulation pulse rate was shown to have only a small effect on speech recognition. Electric fields were manipulated in position and sharpness, showing the potential benefit of improved tonotopic selectivity. Auditory training shows great promise for improving speech recognition for all patients. And the Auditory Brainstem Implant was developed and improved and its application expanded to new populations. Overall, the last 25 years of research at HEI helped increase the basic scientific understanding of electrical stimulation of hearing and contributed to the improved outcomes for patients with the CI and ABI devices. PMID:25449009

Structure and function of the mammalian middle ear. I: Large middle ears in small desert mammals.

PubMed

Mason, Matthew J

2016-02-01

Many species of small desert mammals are known to have expanded auditory bullae. The ears of gerbils and heteromyids have been well described, but much less is known about the middle ear anatomy of other desert mammals. In this study, the middle ears of three gerbils (Meriones, Desmodillus and Gerbillurus), two jerboas (Jaculus) and two sengis (elephant-shrews: Macroscelides and Elephantulus) were examined and compared, using micro-computed tomography and light microscopy. Middle ear cavity expansion has occurred in members of all three groups, apparently in association with an essentially 'freely mobile' ossicular morphology and the development of bony tubes for the middle ear arteries. Cavity expansion can occur in different ways, resulting in different subcavity patterns even between different species of gerbils. Having enlarged middle ear cavities aids low-frequency audition, and several adaptive advantages of low-frequency hearing to small desert mammals have been proposed. However, while Macroscelides was found here to have middle ear cavities so large that together they exceed brain volume, the bullae of Elephantulus are considerably smaller. Why middle ear cavities are enlarged in some desert species but not others remains unclear, but it may relate to microhabitat. © 2015 Anatomical Society.

Sheep as a large animal model for middle and inner ear implantable hearing devices: a feasibility study in cadavers.

PubMed

Schnabl, Johannes; Glueckert, Rudolf; Feuchtner, Gudrun; Recheis, Wolfgang; Potrusil, Thomas; Kuhn, Volker; Wolf-Magele, Astrid; Riechelmann, Herbert; Sprinzl, Georg M

2012-04-01

Currently, no large animal model exists for surgical-experimental exploratory analysis of implantable hearing devices. In a histomorphometric study, we sought to investigate whether sheep or pig cochleae are suitable for this purpose and whether device implantation is feasible. Skulls of pig and sheep cadavers were examined using high-resolution 128-slice computed tomography (CT) to study anatomic relationships. A cochlear implant and an active middle ear implant could be successfully implanted into the sheep's inner and middle ear, respectively. Correct device placement was verified by CT and histology. The cochlear anatomy of the sheep was further studied by micro-CT and histology. Our investigations indicate that the sheep is a suitable animal model for implantation of implantable hearing devices. The implantation of the devices was successfully performed by access through a mastoidectomy. The histologic, morphologic, and micro-CT study of the sheep cochlea showed that it is highly similar to the human cochlea. The temporal bone of the pig was not suitable for these microsurgical procedures because the middle and inner ear were not accessible owing to distinct soft and fatty tissue coverage of the mastoid. The sheep is an appropriate large animal model for experimental studies with implantable hearing devices, whereas the pig is not.

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.

Functional evaluation of a cell replacement therapy in the inner ear

PubMed Central

Hu, Zhengqing; Ulfendahl, Mats; Prieskorn, Diane M.; Olivius, N. Petri; Miller, Josef M.

2015-01-01

Hypothesis Cell replacement therapy in the inner ear will contribute to the functional recovery of hearing loss. Background Cell replacement therapy is a potentially powerful approach to replace degenerated or severely damaged spiral ganglion neurons. This study aimed at stimulating the neurite outgrowth of the implanted neurons and enhancing the potential therapeutic of inner ear cell implants. Methods Chronic electrical stimulation (CES) and exogenous neurotrophic growth factor (NGF) were applied to 46 guinea pigs transplanted with embryonic dorsal root ganglion (DRG) neurons four days post deafening. The animals were evaluated with the electrically-evoked auditory brain stem responses (EABRs) at experimental day 7, 11, 17, 24, 31. The animals were euthanized at day 31 and the inner ears were dissected out for immunohistochemistry investigation. Results Implanted DRG cells, identified by EGFP fluorescence and a neuronal marker, were found close to Rosenthal's canal in the adult inner ear for up to four weeks following transplantation. Extensive neurite projections clearly, greater than in non-treated animals, were observed to penetrate the bony modiolus and reach the spiral ganglion region in animals supplied with CES and/or NGF. There was, however, no significant difference in the thresholds of EABRs between DRG-transplanted-animals supplied with CES and/or NGF and DRG-transplanted animals without CES or NGF supplement. Conclusions The results suggest that CES and/or NGF can stimulate neurite outgrowth from implanted neurons, although based on EABR measurement these interventions did not induce functional connections to the central auditory pathway. Additional time or novel approaches may enhance functional responsiveness of implanted cells in the adult cochlea. PMID:19395986

Sequential bilateral cochlear implantation improves working performance, quality of life, and quality of hearing.

PubMed

Härkönen, Kati; Kivekäs, Ilkka; Rautiainen, Markus; Kotti, Voitto; Sivonen, Ville; Vasama, Juha-Pekka

2015-05-01

This prospective study shows that working performance, quality of life (QoL), and quality of hearing (QoH) are better with two compared with a single cochlear implant (CI). The impact of the second CI on the patient's QoL is as significant as the impact of the first CI. To evaluate the benefits of sequential bilateral cochlear implantation in working, QoL, and QoH. We studied working performance, work-related stress, QoL, and QoH with specific questionnaires in 15 patients with unilateral CI scheduled for sequential CI of another ear. Sound localization performance and speech perception in noise were measured with specific tests. All questionnaires and tests were performed before the second CI surgery and 6 and 12 months after its activation. Bilateral CIs increased patients' working performance and their work-related stress and fatigue decreased. Communication with co-workers was easier and patients were more active in their working environment. Sequential bilateral cochlear implantation improved QoL, QoH, sound localization, and speech perception in noise statistically significantly.

Mechanisms of Hearing Loss after Blast Injury to the Ear

PubMed Central

Cho, Sung-Il; Gao, Simon S.; Xia, Anping; Wang, Rosalie; Salles, Felipe T.; Raphael, Patrick D.; Abaya, Homer; Wachtel, Jacqueline; Baek, Jongmin; Jacobs, David; Rasband, Matthew N.; Oghalai, John S.

2013-01-01

Given the frequent use of improvised explosive devices (IEDs) around the world, the study of traumatic blast injuries is of increasing interest. The ear is the most common organ affected by blast injury because it is the body’s most sensitive pressure transducer. We fabricated a blast chamber to re-create blast profiles similar to that of IEDs and used it to develop a reproducible mouse model to study blast-induced hearing loss. The tympanic membrane was perforated in all mice after blast exposure and found to heal spontaneously. Micro-computed tomography demonstrated no evidence for middle ear or otic capsule injuries; however, the healed tympanic membrane was thickened. Auditory brainstem response and distortion product otoacoustic emission threshold shifts were found to be correlated with blast intensity. As well, these threshold shifts were larger than those found in control mice that underwent surgical perforation of their tympanic membranes, indicating cochlear trauma. Histological studies one week and three months after the blast demonstrated no disruption or damage to the intra-cochlear membranes. However, there was loss of outer hair cells (OHCs) within the basal turn of the cochlea and decreased spiral ganglion neurons (SGNs) and afferent nerve synapses. Using our mouse model that recapitulates human IED exposure, our results identify that the mechanisms underlying blast-induced hearing loss does not include gross membranous rupture as is commonly believed. Instead, there is both OHC and SGN loss that produce auditory dysfunction. PMID:23840874

Mammalian spontaneous otoacoustic emissions are amplitude-stabilized cochlear standing waves.

PubMed

Shera, Christopher A

2003-07-01

Mammalian spontaneous otoacoustic emissions (SOAEs) have been suggested to arise by three different mechanisms. The local-oscillator model, dating back to the work of Thomas Gold, supposes that SOAEs arise through the local, autonomous oscillation of some cellular constituent of the organ of Corti (e.g., the "active process" underlying the cochlear amplifier). Two other models, by contrast, both suppose that SOAEs are a global collective phenomenon--cochlear standing waves created by multiple internal reflection--but differ on the nature of the proposed power source: Whereas the "passive" standing-wave model supposes that SOAEs are biological noise, passively amplified by cochlear standing-wave resonances acting as narrow-band nonlinear filters, the "active" standing-wave model supposes that standing-wave amplitudes are actively maintained by coherent wave amplification within the cochlea. Quantitative tests of key predictions that distinguish the local-oscillator and global standing-wave models are presented and shown to support the global standing-wave model. In addition to predicting the existence of multiple emissions with a characteristic minimum frequency spacing, the global standing-wave model accurately predicts the mean value of this spacing, its standard deviation, and its power-law dependence on SOAE frequency. Furthermore, the global standing-wave model accounts for the magnitude, sign, and frequency dependence of changes in SOAE frequency that result from modulations in middle-ear stiffness. Although some of these SOAE characteristics may be replicable through artful ad hoc adjustment of local-oscillator models, they all arise quite naturally in the standing-wave framework. Finally, the statistics of SOAE time waveforms demonstrate that SOAEs are coherent, amplitude-stabilized signals, as predicted by the active standing-wave model. Taken together, the results imply that SOAEs are amplitude-stabilized standing waves produced by the cochlea acting as a

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

Music Perception with Cochlear Implants: A Review

PubMed Central

McDermott, Hugh J.

2004-01-01

The acceptance of cochlear implantation as an effective and safe treatment for deafness has increased steadily over the past quarter century. The earliest devices were the first implanted prostheses found to be successful in compensating partially for lost sensory function by direct electrical stimulation of nerves. Initially, the main intention was to provide limited auditory sensations to people with profound or total sensorineural hearing impairment in both ears. Although the first cochlear implants aimed to provide patients with little more than awareness of environmental sounds and some cues to assist visual speech-reading, the technology has advanced rapidly. Currently, most people with modern cochlear implant systems can understand speech using the device alone, at least in favorable listening conditions. In recent years, an increasing research effort has been directed towards implant users’ perception of nonspeech sounds, especially music. This paper reviews that research, discusses the published experimental results in terms of both psychophysical observations and device function, and concludes with some practical suggestions about how perception of music might be enhanced for implant recipients in the future. The most significant findings of past research are: (1) On average, implant users perceive rhythm about as well as listeners with normal hearing; (2) Even with technically sophisticated multiple-channel sound processors, recognition of melodies, especially without rhythmic or verbal cues, is poor, with performance at little better than chance levels for many implant users; (3) Perception of timbre, which is usually evaluated by experimental procedures that require subjects to identify musical instrument sounds, is generally unsatisfactory; (4) Implant users tend to rate the quality of musical sounds as less pleasant than listeners with normal hearing; (5) Auditory training programs that have been devised specifically to provide implant users with

Music perception with cochlear implants: a review.

PubMed

McDermott, Hugh J

2004-01-01

The acceptance of cochlear implantation as an effective and safe treatment for deafness has increased steadily over the past quarter century. The earliest devices were the first implanted prostheses found to be successful in compensating partially for lost sensory function by direct electrical stimulation of nerves. Initially, the main intention was to provide limited auditory sensations to people with profound or total sensorineural hearing impairment in both ears. Although the first cochlear implants aimed to provide patients with little more than awareness of environmental sounds and some cues to assist visual speech-reading, the technology has advanced rapidly. Currently, most people with modern cochlear implant systems can understand speech using the device alone, at least in favorable listening conditions. In recent years, an increasing research effort has been directed towards implant users' perception of nonspeech sounds, especially music. This paper reviews that research, discusses the published experimental results in terms of both psychophysical observations and device function, and concludes with some practical suggestions about how perception of music might be enhanced for implant recipients in the future. The most significant findings of past research are: (1) On average, implant users perceive rhythm about as well as listeners with normal hearing; (2) Even with technically sophisticated multiple-channel sound processors, recognition of melodies, especially without rhythmic or verbal cues, is poor, with performance at little better than chance levels for many implant users; (3) Perception of timbre, which is usually evaluated by experimental procedures that require subjects to identify musical instrument sounds, is generally unsatisfactory; (4) Implant users tend to rate the quality of musical sounds as less pleasant than listeners with normal hearing; (5) Auditory training programs that have been devised specifically to provide implant users with

Effect of low level laser (LLL) on cochlear and vestibular inner ear including tinnitus

NASA Astrophysics Data System (ADS)

Rhee, Chung-Ku; Lim, Eun-Seok; Kim, Young-Saeng; Chung, Yong-Won; Jung, Jae-Yun; Chung, Phil-Sang

2006-02-01

Objectives: 1. To investigate preventive effect of LLL on gentamicin-induced vestibular ototoxicity. 2. To evaluate the effectiveness of lower level laser (LLL) in the treatment of tinnitus. Methods: 1. Twenty guinea pigs were divided into control and laser groups. Vestibular ototoxicity was induced by intratympanic injection of gentamicin into left ear. LLL was irradiated into left ear canal of animals in laser group. Vestibular function of the animals was evaluated with vertical and off-vertical axis rotation testing. 2. Forty patients with tinnitus were treated with ginkgo biloba orally and randomly divided into control and laser groups. The 20 patients of laser group received 80.4 J/cm2 of 830 nm laser, 3 times per week for 4 weeks, via transmeatal irradiation. Tinnitus was evaluated by visual analogue scale (VAS) and tinnitus handicap inventory (THI). Results: 1. Preventive effect of LLL to gentamicin induced vestibular ototoxicity was demonstrated by preventing reduction of gain in slow harmonic acceleration test and modulation in the off-vertical axis rotation test. 2. Eleven of 20 laser group patients have shown significant improvement in VAS and THI compared to those of the control group. Conclusions: 1. LLL therapy may have preventive effect to vestibular ototoxicity. 2. LLL therapy in combination with ginkgo biloba seems to be worth trying on patients with tinnitus.

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.

The Relationship Between Intensity Coding and Binaural Sensitivity in Adults With Cochlear Implants

PubMed Central

Todd, Ann E.; Goupell, Matthew J.; Litovsky, Ruth Y.

2016-01-01

Objectives Many bilateral cochlear implant users show sensitivity to binaural information when stimulation is provided using a pair of synchronized electrodes. However, there is large variability in binaural sensitivity between and within participants across stimulation sites in the cochlea. It was hypothesized that within-participant variability in binaural sensitivity is in part affected by limitations and characteristics of the auditory periphery which may be reflected by monaural hearing performance. The objective of this study was to examine the relationship between monaural and binaural hearing performance within participants with bilateral cochlear implants. Design Binaural measures included dichotic signal detection and interaural time difference discrimination thresholds. Diotic signal detection thresholds were also measured. Monaural measures included dynamic range and amplitude modulation detection. In addition, loudness growth was compared between ears. Measures were made at three stimulation sites per listener. Results Greater binaural sensitivity was found with larger dynamic ranges. Poorer interaural time difference discrimination was found with larger difference between comfortable levels of the two ears. In addition, poorer diotic signal detection thresholds were found with larger differences between the dynamic ranges of the two ears. No relationship was found between amplitude modulation detection thresholds or symmetry of loudness growth and the binaural measures. Conclusions The results suggest that some of the variability in binaural hearing performance within listeners across stimulation sites can be explained by factors non-specific to binaural processing. The results are consistent with the idea that dynamic range and comfortable levels relate to peripheral neural survival and the width of the excitation pattern which could affect the fidelity with which central binaural nuclei process bilateral inputs. PMID:27787393

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.

Prenatal and postnatal serum PCB concentrations and cochlear function in children at 45 months of age.

PubMed

Jusko, Todd A; Sisto, Renata; Iosif, Ana-Maria; Moleti, Arturo; Wimmerová, Sonˇa; Lancz, Kinga; Tihányi, Juraj; Sovčiková, Eva; Drobná, Beata; Palkovičová, L'ubica; Jurečková, Dana; Thevenet-Morrison, Kelly; Verner, Marc-André; Sonneborn, Dean; Hertz-Picciotto, Irva; Trnovec, Tomáš

2014-11-01

Some experimental and human data suggest that exposure to polychlorinated biphenyls (PCBs) may induce ototoxicity, though results of previous epidemiologic studies are mixed and generally focus on either prenatal or postnatal PCB concentrations exclusively. Our aim was to evaluate the association between pre- and postnatal PCB concentrations in relation to cochlear status, assessed by distortion product otoacoustic emissions (DPOAEs), and to further clarify the critical periods in development where cochlear status may be most susceptible to PCBs. A total of 351 children from a birth cohort in eastern Slovakia underwent otoacoustic testing at 45 months of age. Maternal pregnancy, cord, and child 6-, 16-, and 45-month blood samples were collected and analyzed for PCB concentrations. At 45 months of age, DPOAEs were assessed at 11 frequencies in both ears. Multivariate, generalized linear models were used to estimate the associations between PCB concentrations at different ages and DPOAEs, adjusting for potential confounders. Maternal and cord PCB-153 concentrations were not associated with DPOAEs at 45 months. Higher postnatal PCB concentrations at 6-, 16-, and 45-months of age were associated with lower (poorer) DPOAE amplitudes. When all postnatal PCB exposures were considered as an area-under-the-curve metric, an increase in PCB-153 concentration from the 25th to the 75th percentile was associated with a 1.6-dB SPL (sound pressure level) decrease in DPOAE amplitude (95% CI: -2.6, -0.5; p = 0.003). In this study, postnatal rather than maternal or cord PCB concentrations were associated with poorer performance on otoacoustic tests at age 45 months.

Impedances of the ear estimated with intracochlear pressures in normal human temporal bones

NASA Astrophysics Data System (ADS)

Frear, Darcy; Guan, Xiying; Stieger, Christof; Nakajima, Hideko Heidi

2018-05-01

We have measured intracochlear pressures and velocities of stapes and round window (RW) evoked by air conduction (AC) stimulation in many fresh human cadaveric specimens. Our techniques have improved through the years to ensure reliable pressure sensor measurements in the scala vestibuli and scala tympani. Using these measurements, we have calculated impedances of the middle and inner ear (cochlear partition, RW, and physiological leakage impedance in scala vestibuli) to create a lumped element model. Our model simulates our data and allows us to understand the mechanisms involved in air-conducted sound transmission. In the future this model will be used as a tool to understand transmission mechanisms of various stimuli and to help create more sophisticated models of the ear.

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

Trimodal speech perception: how residual acoustic hearing supplements cochlear-implant consonant recognition in the presence of visual cues.

PubMed

Sheffield, Benjamin M; Schuchman, Gerald; Bernstein, Joshua G W

2015-01-01

As cochlear implant (CI) acceptance increases and candidacy criteria are expanded, these devices are increasingly recommended for individuals with less than profound hearing loss. As a result, many individuals who receive a CI also retain acoustic hearing, often in the low frequencies, in the nonimplanted ear (i.e., bimodal hearing) and in some cases in the implanted ear (i.e., hybrid hearing) which can enhance the performance achieved by the CI alone. However, guidelines for clinical decisions pertaining to cochlear implantation are largely based on expectations for postsurgical speech-reception performance with the CI alone in auditory-only conditions. A more comprehensive prediction of postimplant performance would include the expected effects of residual acoustic hearing and visual cues on speech understanding. An evaluation of auditory-visual performance might be particularly important because of the complementary interaction between the speech information relayed by visual cues and that contained in the low-frequency auditory signal. The goal of this study was to characterize the benefit provided by residual acoustic hearing to consonant identification under auditory-alone and auditory-visual conditions for CI users. Additional information regarding the expected role of residual hearing in overall communication performance by a CI listener could potentially lead to more informed decisions regarding cochlear implantation, particularly with respect to recommendations for or against bilateral implantation for an individual who is functioning bimodally. Eleven adults 23 to 75 years old with a unilateral CI and air-conduction thresholds in the nonimplanted ear equal to or better than 80 dB HL for at least one octave frequency between 250 and 1000 Hz participated in this study. Consonant identification was measured for conditions involving combinations of electric hearing (via the CI), acoustic hearing (via the nonimplanted ear), and speechreading (visual cues

[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.

[Correlation between the thresholds acquired with ASSR and sbjective thresholds in cochlear implants in prelingually deaf children].

PubMed

Wang, Z; Gu, J; Jiang, X J

2017-04-20

Objective: To learn the relationship between the auditory steady state responses(ASSR)threshold and C-level and behavior T-level in cochlear implants in prelingually deaf children. Method: One hundred and twelve children with Nucleus CI24R(CA) cochlear implants were divided into residual hearing group and no residual hearing group on the basis of the results of ASSR before operation in this study.Compare the difference between the two groups in C-level and behavior T-level one year after operation. Result: There was difference in C-level and behavior T-level between residual hearing group and no residual hearing group( P <0.05 or P <0.01). Conclusion: According to the results of ASSR before operation,we can estimate the effect of cochlear implants,providing reference for the selection of choosing operating ears,and providing a reasonable expectation for physicians and parents of the patients. Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.

Anatomy, Physiology and Function of the Auditory System

NASA Astrophysics Data System (ADS)

Kollmeier, Birger

The human ear consists of the outer ear (pinna or concha, outer ear canal, tympanic membrane), the middle ear (middle ear cavity with the three ossicles malleus, incus and stapes) and the inner ear (cochlea which is connected to the three semicircular canals by the vestibule, which provides the sense of balance). The cochlea is connected to the brain stem via the eighth brain nerve, i.e. the vestibular cochlear nerve or nervus statoacusticus. Subsequently, the acoustical information is processed by the brain at various levels of the auditory system. An overview about the anatomy of the auditory system is provided by Figure 1.

Functional recovery in the avian ear after hair cell regeneration.

PubMed

Smolders, J W

1999-01-01

Trauma to the inner ear in birds, due to acoustic overstimulation or ototoxic aminoglycosides, can lead to hair cell loss which is followed by regeneration of new hair cells. These processes are paralleled by hearing loss followed by significant functional recovery. After acoustic trauma, functional recovery is rapid and nearly complete. The early and major part of functional recovery after sound trauma occurs before regenerated hair cells become functional. Even very intense sound trauma causes loss of only a proportion of the hair cell population, mainly so-called short hair cells residing on the abneural mobile part of the avian basilar membrane. Uncoupling of the tectorial membrane from the hair cells during sound overexposure may serve as a protection mechanism. The rapid functional recovery after sound trauma appears not to be associated with regeneration of the lost hair cells, but with repair processes involving the surviving hair cells. Small residual functional deficits after recovery are most likely associated with the missing upper fibrous layer of the tectorial membrane which fails to regenerate after sound trauma. After aminoglycoside trauma, functional recovery is slower and parallels the structural regeneration more closely. Aminoglycosides cause damage to both types of hair cells, starting at the basal (high frequency) part of the basilar papilla. However, functional hearing loss and recovery also occur at lower frequencies, associated with areas of the papilla where hair cells survive. Functional recovery in these low frequency areas is complete, whereas functional recovery in high frequency areas with complete hair cell loss is incomplete, despite regeneration of the hair cells. Permanent residual functional deficits remain. This indicates that in low frequency regions functional recovery after aminoglycosides involves repair of nonlethal injury to hair cells and/or hair cell-neural synapses. In the high frequency regions functional recovery

A cochlear implant fabricated using a bulk silicon-surface micromachining process

NASA Astrophysics Data System (ADS)

Bell, Tracy Elizabeth

1999-11-01

This dissertation presents the design and fabrication of two generations of a silicon microelectrode array for use in a cochlear implant. A cochlear implant is a device that is inserted into the inner ear and uses electrical stimulation to provide sound sensations to the profoundly deaf. The first-generation silicon cochlear implant is a passive device fabricated using silicon microprobe technology developed at the University of Michigan. It contains twenty-two iridium oxide (IrO) stimulating sites that are 250 mum in diameter and spaced at 750 mum intervals. In-vivo recordings were made in guinea pig auditory cortex in response to electrical stimulation with this device, verifying its ability to electrically evoke an auditory response. Auditory thresholds as low as 78 muA were recorded. The second-generation implant is a thirty-two site, four-channel device with on-chip CMOS site-selection circuitry and integrated position sensing. It was fabricated using a novel bulk silicon surface micromachining process which was developed as a part of this dissertation work. While the use of semiconductor technology offers many advantages in fabricating cochlear implants over the methods currently used, it was felt that even further advantages could be gained by developing a new micromachining process which would allow circuitry to be distributed along the full length of the cochlear implant substrate. The new process uses electropolishing of an n+ bulk silicon sacrificial layer to undercut and release n- epitaxial silicon structures from the wafer. An extremely abrupt etch-stop between the n+ and n- silicon is obtained, with no electropolishing taking place in the n-type silicon that is doped lower than 1 x 1017 cm-3 in concentration. Lateral electropolishing rates of up to 50 mum/min were measured using this technique, allowing one millimeter-wide structures to be fully undercut in as little as 10 minutes. The new micromachining process was integrated with a standard p

Advantage of bimodal fitting in prosody perception for children using a cochlear implant and a hearing aid.

PubMed

Straatman, L V; Rietveld, A C M; Beijen, J; Mylanus, E A M; Mens, L H M

2010-10-01

Cochlear implants are largely unable to encode voice pitch information, which hampers the perception of some prosodic cues, such as intonation. This study investigated whether children with a cochlear implant in one ear were better able to detect differences in intonation when a hearing aid was added in the other ear ("bimodal fitting"). Fourteen children with normal hearing and 19 children with bimodal fitting participated in two experiments. The first experiment assessed the just noticeable difference in F0, by presenting listeners with a naturally produced bisyllabic utterance with an artificially manipulated pitch accent. The second experiment assessed the ability to distinguish between questions and affirmations in Dutch words, again by using artificial manipulation of F0. For the implanted group, performance significantly improved in each experiment when the hearing aid was added. However, even with a hearing aid, the implanted group required exaggerated F0 excursions to perceive a pitch accent and to identify a question. These exaggerated excursions are close to the maximum excursions typically used by Dutch speakers. Nevertheless, the results of this study showed that compared to the implant only condition, bimodal fitting improved the perception of intonation.

Persistent Language Delay Versus Late Language Emergence in Children With Early Cochlear Implantation

PubMed Central

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 midelementary grades and to identify risk factors for persistent language delay following early cochlear implantation. Materials and Method Children receiving unilateral CIs at young ages (12–38 months) were tested longitudinally and classified with normal language emergence (n = 19), late language emergence (n = 22), or persistent language delay (n = 19) on the basis of their test scores at 4.5 and 10.5 years of age. Relative effects of demographic, audiological, linguistic, and academic characteristics on language emergence were determined. Results Age at CI was associated with normal language emergence but did not differentiate late emergence from persistent delay. Children with persistent delay were more likely to use left-ear implants and older speech processor technology. They experienced higher aided thresholds and lower speech perception scores. Persistent delay was foreshadowed by low morphosyntactic and phonological diversity in preschool. Logistic regression analysis predicted normal language emergence with 84% accuracy and persistent language delay with 74% accuracy. Conclusion CI characteristics had a strong effect on persistent versus resolving language delay, suggesting that right-ear (or bilateral) devices, technology upgrades, and improved audibility may positively influence long-term language outcomes. PMID:26501740

Inner ear anomalies and conductive hearing loss in children with Apert syndrome: an overlooked otologic aspect.

PubMed

Zhou, Guangwei; Schwartz, Lynn Thomas; Gopen, Quinton

2009-02-01

To identify the occurrence of inner ear structural anomalies and conductive hearing loss (CHL) in children with Apert syndrome. Retrospective review. Pediatric tertiary referral center. Twenty pediatric patients with Apert syndrome were found; all patients (38/40 ears) had inner ear anomalies. Computerized tomography of the head/temporal bone, pure-tone (including air and bone conduction) audiometry, and tympanometry. Imaging demonstrating inner ear anomalies, including malformations of the cochlea, dilated vestibule, and/or semicircular canal; audiologic findings of air-bone gap(s). Hearing loss was found in 90% of the patients with Apert syndrome, and 80% of them had CHL. Air-bone gaps were found at all frequencies, with larger gaps at low frequencies. Fifty percent (20/40) of the ears had better than 0 dB hearing level bone conduction thresholds at 250 and/or 500 Hz. Normal middle ear pressure and mobility were found in all ears with intact eardrum. Inner ear anomalies were found in all patients, and 90% of them had bilateral involvement. Most frequently observed inner ear anomalies were dilated vestibule, malformed lateral semicircular canal, and cochlear dysplasia. Children with Apert syndrome may present with significant CHL that cannot be explained by minor middle ear pathologies alone. This conductive loss may be, at least partially, attributed to the inner ear anomalies; however, these structural anomalies are usually not recognized in these patients. Failure to close air-bone gap after surgical intervention may raise the suspicion of inner ear anomalies, and computed tomographic scan of the temporal bone can provide definitive proof.

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

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.

Improvement in symptoms and cochlear flow with pycnogenol in patients with Meniere's disease and tinnitus.

PubMed

Luzzi, R; Belcaro, G; Hu, S; Dugall, M; Hosoi, M; Cacchio, M; Ippolito, E; Corsi, M

2014-06-01

The aim of this supplement registry was to evaluate the efficacy of the Pycnogenol® in improving cochlear flow and symptoms in a 6-month follow-up for patients with Meniere's disease (MD), tinnitus and cochlear hypoperfusion. Main signs/symptoms were considered: Spontaneous vertigo, positional vertigo, hearing loss, tinnitus, pressure in the ear, unsteady gait, associated clinical problems, alterations in daily life. All subjects were managed with the best available management (BM); one group used the supplement Pycnogenol (150 mg/day). Cochlear flow and tinnitus were also evaluated. Out of 120 patients incuded in the registry, 55 used Pycnogenol and 52 (controls) were managed only with BM. There was a more significant improvement in all registry items at 3 and 6 months in the Pycnogenol group (P<0.05). The number of lost working days was lower in the Pycnogenol group. At 3 months, 45.4% of subjects using Pycnogenol were completely asymptomatic in comparison with 23.07% of controls. At 6 months 87.3% of the Pycnogenol subjects were asymptomatic compared with 34.6% of controls. Cochlear flow velocity was significantly better (higher flow, higher diastolic component) in the Pycnogenol group (P<0.05). The subjective tinnitus scale decreased in both groups (P<0.05); the decrease was more significant in Pycnogenol subjects (P<0.05) at 3 and 6 months. Symptoms of Meniere's disease, flow at cochlear level and tinnitus improved in Pycnogenol subjects in comparison with best management.

Generation of inner ear organoids containing functional hair cells from human pluripotent stem cells.

PubMed

Koehler, Karl R; Nie, Jing; Longworth-Mills, Emma; Liu, Xiao-Ping; Lee, Jiyoon; Holt, Jeffrey R; Hashino, Eri

2017-06-01

The derivation of human inner ear tissue from pluripotent stem cells would enable in vitro screening of drug candidates for the treatment of hearing and balance dysfunction and may provide a source of cells for cell-based therapies of the inner ear. Here we report a method for differentiating human pluripotent stem cells to inner ear organoids that harbor functional hair cells. Using a three-dimensional culture system, we modulate TGF, BMP, FGF, and WNT signaling to generate multiple otic-vesicle-like structures from a single stem-cell aggregate. Over 2 months, the vesicles develop into inner ear organoids with sensory epithelia that are innervated by sensory neurons. Additionally, using CRISPR-Cas9, we generate an ATOH1-2A-eGFP cell line to detect hair cell induction and demonstrate that derived hair cells exhibit electrophysiological properties similar to those of native sensory hair cells. Our culture system should facilitate the study of human inner ear development and research on therapies for diseases of the inner ear.

Cochlear implantation using a custom guide catheter in fourteen patients with incomplete partition type III.

PubMed

Tian, Hao; Wang, Line; Gao, Fenqi; Liang, Wenqi; Peng, Kevin A

2018-05-22

Incomplete partition type III (IP-III), also termed X-linked deafness with stapes gusher, is a heterogeneous condition that predominantly affects males; however, females demonstrating the phenotype also exist. The absence of a bony partition between the fundus of the internal auditory canal (IAC) and cochlea predisposes these patients to cerebrospinal fluid leak or electrode passage into the IAC when performing cochlear implantation. We describe a surgeon-fabricated guide catheter, made intraoperatively from a 16-gauge intravenous catheter, that helps avoid electrode passage into the IAC during CI for patients with IP-III. Acceptable cochlear implant outcomes were attainable in IP-III patients, but these patients scored worse than matched CI patients with normal inner ear structures. This article is protected by copyright. All rights reserved. This article is protected by copyright. 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

Inner ear development: building a spiral ganglion and an organ of Corti out of unspecified ectoderm.

PubMed

Fritzsch, Bernd; Pan, Ning; Jahan, Israt; Elliott, Karen L

2015-07-01

The mammalian inner ear develops from a placodal thickening into a complex labyrinth of ducts with five sensory organs specialized to detect position and movement in space. The mammalian ear also develops a spiraled cochlear duct containing the auditory organ, the organ of Corti (OC), specialized to translate sound into hearing. Development of the OC from a uniform sheet of ectoderm requires unparalleled precision in the topological developmental engineering of four different general cell types, namely sensory neurons, hair cells, supporting cells, and general otic epithelium, into a mosaic of ten distinctly recognizable cell types in and around the OC, each with a unique distribution. Moreover, the OC receives unique innervation by ear-derived spiral ganglion afferents and brainstem-derived motor neurons as efferents and requires neural-crest-derived Schwann cells to form myelin and neural-crest-derived cells to induce the stria vascularis. This transformation of a sheet of cells into a complicated interdigitating set of cells necessitates the orchestrated expression of multiple transcription factors that enable the cellular transformation from ectoderm into neurosensory cells forming the spiral ganglion neurons (SGNs), while simultaneously transforming the flat epithelium into a tube, the cochlear duct, housing the OC. In addition to the cellular and conformational changes forming the cochlear duct with the OC, changes in the surrounding periotic mesenchyme form passageways for sound to stimulate the OC. We review molecular developmental data, generated predominantly in mice, in order to integrate the well-described expression changes of transcription factors and their actions, as revealed in mutants, in the formation of SGNs and OC in the correct position and orientation with suitable innervation. Understanding the molecular basis of these developmental changes leading to the formation of the mammalian OC and highlighting the gaps in our knowledge might guide in

Inner ear development: Building a spiral ganglion and an organ of Corti out of unspecified ectoderm

PubMed Central

Fritzsch, Bernd; Pan, Ning; Jahan, Israt; Elliott, Karen L.

2014-01-01

The mammalian inner ear develops from a placodal thickening into a complex labyrinth of ducts with five sensory organs specialized to detect position and movement in space. In addition, the mammalian ear develops a spiraled cochlear duct containing the auditory organ, the organ of Corti (OC), specialized to translate sound into hearing. Developing the OC out of a uniform sheet of ectoderm requires an unparalleled precision in topological developmental engineering of four different general cell types, sensory neurons, hair cells, supporting cells, and general otic epithelium, into a mosaic of ten distinctly recognizable cell types in and around the OC, each with a unique distribution. In addition, the OC receives a unique innervation by ear-derived spiral ganglion afferents and brainstem-derived motor neurons as efferents, and requires neural crest-derived Schwann cells to form myelin and neural crest-derived cells to induce the stria vascularis. To achieve this transformation of a sheet of cells into a complicated interdigitating set of cells necessitates the orchestrated expression of multiple transcription factors that enable the cellular transformation from ectoderm into neurosensory cells forming the spiral ganglion neurons (SGN) while simultaneously transforming the flat epithelium into a tube, the cochlear duct housing the OC. In addition to the cellular and conformational changes to make the cochlear duct with the OC, additional changes in the surrounding periotic mesenchyme form passageways for sound to stimulate the OC. This article reviews molecular developmental data generated predominantly in mice. The available data are ordered into a plausible scenario that integrates the well described expression changes of transcription factors and their actions revealed in mouse mutants for formation of SGNs and OC in the right position and orientation with the right kind of innervation. Understanding the molecular basis of these developmental changes leading to

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

External ear anomalies and hearing impairment in Noonan Syndrome.

PubMed

van Trier, Dorothée C; van Nierop, Josephine; Draaisma, Jos M Th; van der Burgt, Ineke; Kunst, Henricus; Croonen, Ellen A; Admiraal, Ronald J C

2015-06-01

This is the first cohort in which hearing impairment and external ear anomalies in Noonan Syndrome are described extensively. Retrospective analysis of the otorhinolaryngological and clinical genetic data from 97 Noonan Syndrome (NS) patients. Forty-four NS patients were seen by an otorhinolaryngologist for the analysis of hearing impairment. In our cohort 80 of the 97 patients were genetically tested. In 71 of these mutations were found: in 48 patients a mutation in PTPN11, in 10 patients in SOS1, in 5 patients in SHOC2, in 5 patients in RAF1, in 1 patient in MAP2K2, in 1 patient in KRAS and in 1 patient in A2ML1. External ear anomalies were reported in 75 NS patients (77%). In 69 patients the ears were low-set, 28 patients had posteriorly rotated ears, 14 patients showed protruding ears and 18 had thickened helices. Hearing impairment was detected in 34 NS patients. Nine patients had sensorineural hearing impairment, two a permanent conductive hearing impairment, two other patients had mixed hearing impairment and 20 patients had conductive hearing impairment in the past, caused by otitis media with effusion. Their temporary conductive hearing impairment resolved between the ages of 2 and 18 years. Sensorineural hearing impairment varied between mild high-frequency hearing impairment and profound (uni- and bilateral) hearing impairment and was progressive in three patients. Four NS patients received cochlear implants for their severe sensorineural hearing impairment. The cohort is small for genotype-phenotype correlations, but sensorineural hearing impairment, especially the bilateral severe hearing impairment, was only seen in patients with a PTPN11 mutation. NS is characterized by dysmorphic external ear anomalies and both sensorineural and conductive hearing impairment. Audiological examinations are recommended in all patients with Noonan Syndrome. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

A prediction of the minke whale (Balaenoptera acutorostrata) middle-ear transfer function.

PubMed

Tubelli, Andrew A; Zosuls, Aleks; Ketten, Darlene R; Yamato, Maya; Mountain, David C

2012-11-01

The lack of baleen whale (Cetacea Mysticeti) audiograms impedes the assessment of the impacts of anthropogenic noise on these animals. Estimates of audiograms, which are difficult to obtain behaviorally or electrophysiologically for baleen whales, can be made by simulating the audiogram as a series of components representing the outer, middle, and inner ear (Rosowski, 1991; Ruggero and Temchin, 2002). The middle-ear portion of the system can be represented by the middle-ear transfer function (METF), a measure of the transmission of acoustic energy from the external ear to the cochlea. An anatomically accurate finite element model of the minke whale (Balaenoptera acutorostrata) middle ear was developed to predict the METF for a mysticete species. The elastic moduli of the auditory ossicles were measured by using nanoindentation. Other mechanical properties were estimated from experimental stiffness measurements or from published values. The METF predicted a best frequency range between approximately 30 Hz and 7.5 kHz or between 100 Hz and 25 kHz depending on stimulation location. Parametric analysis found that the most sensitive parameters are the elastic moduli of the glove finger and joints and the Rayleigh damping stiffness coefficient β. The predicted hearing range matches well with the vocalization range.

Function and expression pattern of nonsyndromic deafness genes

PubMed Central

Hilgert, Nele; Smith, Richard J.H.; Van Camp, Guy

2010-01-01

Hearing loss is the most common sensory disorder, present in 1 of every 500 newborns. To date, 46 genes have been identified that cause nonsyndromic hearing loss, making it an extremely heterogeneous trait. This review provides a comprehensive overview of the inner ear function and expression pattern of these genes. In general, they are involved in hair bundle morphogenesis, form constituents of the extracellular matrix, play a role in cochlear ion homeostasis or serve as transcription factors. During the past few years, our knowledge of genes involved in hair bundle morphogenesis has increased substantially. We give an up-to-date overview of both the nonsyndromic and Usher syndrome genes involved in this process, highlighting proteins that interact to form macromolecular complexes. For every gene, we also summarize its expression pattern and impact on hearing at the functional level. Gene-specific cochlear expression is summarized in a unique table by structure/cell type and is illustrated on a cochlear cross-section, which is available online via the Hereditary Hearing Loss Homepage. This review should provide auditory scientists the most relevant information for all identified nonsyndromic deafness genes. PMID:19601806

Binaural fusion and listening effort in children who use bilateral cochlear implants: a psychoacoustic and pupillometric study.

PubMed

Steel, Morrison M; Papsin, Blake C; Gordon, Karen A

2015-01-01

Bilateral cochlear implants aim to provide hearing to both ears for children who are deaf and promote binaural/spatial hearing. Benefits are limited by mismatched devices and unilaterally-driven development which could compromise the normal integration of left and right ear input. We thus asked whether children hear a fused image (ie. 1 vs 2 sounds) from their bilateral implants and if this "binaural fusion" reduces listening effort. Binaural fusion was assessed by asking 25 deaf children with cochlear implants and 24 peers with normal hearing whether they heard one or two sounds when listening to bilaterally presented acoustic click-trains/electric pulses (250 Hz trains of 36 ms presented at 1 Hz). Reaction times and pupillary changes were recorded simultaneously to measure listening effort. Bilaterally implanted children heard one image of bilateral input less frequently than normal hearing peers, particularly when intensity levels on each side were balanced. Binaural fusion declined as brainstem asymmetries increased and age at implantation decreased. Children implanted later had access to acoustic input prior to implantation due to progressive deterioration of hearing. Increases in both pupil diameter and reaction time occurred as perception of binaural fusion decreased. Results indicate that, without binaural level cues, children have difficulty fusing input from their bilateral implants to perceive one sound which costs them increased listening effort. Brainstem asymmetries exacerbate this issue. By contrast, later implantation, reflecting longer access to bilateral acoustic hearing, may have supported development of auditory pathways underlying binaural fusion. Improved integration of bilateral cochlear implant signals for children is required to improve their binaural hearing.

Using Stimulus Frequency Emissions to Characterize Cochlear Function in Mice

NASA Astrophysics Data System (ADS)

Cheatham, M. A.; Katz, E. D.; Charaziak, K.; Dallos, P.; Siegel, J. H.

2011-11-01

Stimulus frequency otoacoustic emissions (SFOAE) were used to assay cochlear function in wildtype and prestin knockin (KI) mice. The latter contain a mutated form of the outer hair cell (OHC) motor protein (V499G/Y501H) with significantly reduced activity. Because several genetic mutations cause accelerated OHC death, it is beneficial to perform experiments in young mice without surgical intervention. Inasmuch as SFOAE thresholds are elevated by only 30 dB in KIs, it is possible to obtain SFOAE tuning functions in these animals. This approach allows sensitivity/frequency selectivity to be assayed within the basilar membane-OHC-tectorial membrane feedback loop, thereby providing information about signal processing prior to inner hair cell stimulation and auditory nerve activation.

Neurotrophins differentially stimulate the growth of cochlear neurites on collagen surfaces and in gels☆

PubMed Central

Xie, Joanna; Pak, Kwang; Evans, Amaretta; Kamgar-Parsi, Andy; Fausti, Stephen; Mullen, Lina; Ryan, Allen Frederic

2013-01-01

The electrodes of a cochlear implant are located far from the surviving neurons of the spiral ganglion, which results in decreased precision of neural activation compared to the normal ear. If the neurons could be induced to extend neurites toward the implant, it might be possible to stimulate more discrete subpopulations of neurons, and to increase the resolution of the device. However, a major barrier to neurite growth toward a cochlear implant is the fluid filling the scala tympani, which separates the neurons from the electrodes. The goal of this study was to evaluate the growth of cochlear neurites in three-dimensional extracellular matrix molecule gels, and to increase biocompatibility by using fibroblasts stably transfected to produce neurotrophin-3 and brain-derived neurotrophic factor. Spiral ganglion explants from neonatal rats were evaluated in cultures. They were exposed to soluble neurotrophins, cells transfected to secrete neurotrophins, and/or collagen gels. We found that cochlear neurites grew readily on collagen surfaces and in three-dimensional collagen gels. Co-culture with cells producing neurotrophin-3 resulted in increased numbers of neurites, and neurites that were longer than when explants were cultured with control fibroblasts stably transfected with green fluorescent protein. Brain-derived neurotrophic factor-producing cells resulted in a more dramatic increase in the number of neurites, but there was no significant effect on neurite length. It is suggested that extracellular matrix molecule gels and cells transfected to produce neurotrophins offer an opportunity to attract spiral ganglion neurites toward a cochlear implant. PMID:24459465

Magnetic resonance volumetric measurement of endolymphatic space in patients without vertiginous or cochlear symptoms.

PubMed

Inui, Hiroshi; Sakamoto, Tsuyoshi; Ito, Taeko; Kitahara, Tadashi

2016-12-01

Magnetic resonance volumetric measurement of inner ear endolymphatic space (ELS) was performed in patients without vertiginous or cochlear symptoms. The existence of the ELS in patients with chronic rhinosinusitis (CRS) was shown. The ELS in the cochlea and vestibule was classified into four categories. These findings could be useful as a standard reference for further research. To identify normal values of the ELS in the cochlea and vestibule. Twenty-four patients with CRS were enrolled. Inner ear fluid space images and positive perilymph/positive endolymph images were acquired using a 3.0-tesla unit. Three-dimensional (3-D) images were constructed semi-automatically using both anatomical and tissue information by fusing the 3-D images of the inner ear fluid space and the ELS. Among all patients, the mean ELS/the total fluid space (TFS) ratio in the cochlea was 8.8% and that in the vestibule was 16.2%. The ELS in the cochlea and vestibule was classified into four categories. Age-related differences were found in the TFS, ELS, and ELS/TFS ratio in the inner ear and the ELS and ELS/TFS ratio in the vestibule.

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.

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

Immune-Mediated Inner Ear Disease: Diagnostic and therapeutic approaches.

PubMed

Penêda, José Ferreira; Lima, Nuno Barros; Monteiro, Francisco; Silva, Joana Vilela; Gama, Rita; Condé, Artur

2018-03-07

Immune Mediated Inner Ear Disease (IMIED) is a rare form of sensorineural bilateral hearing loss, usually progressing in weeks to months and responsive to immunosuppressive treatment. Despite recent advances, there is no consensus on diagnosis and optimal treatment. A review of articles on IMIED from the last 10 years was conducted using PubMed ® database. IMIED is a rare disease, mostly affecting middle aged women. It may be a primary ear disease or secondary to autoimmune systemic disease. A dual immune response (both cellular and humoral) seems to be involved. Cochlin may be the inner ear protein targeted in this disease. Distinction from other (core common) forms of neurosensory hearing loss is a challenge. Physical examination is mandatory for exclusion of other causes of hearing loss; audiometry identifies characteristic hearing curves. Laboratory and imaging studies are controversial since no diagnostic marker is available. Despite recent research, IMIED diagnosis remains exclusive. Steroids are the mainstay treatment; other therapies need further investigation. For refractory cases, cochlear implantation is an option and with good relative outcome. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Activation of NLRP3 inflammasome in human middle ear cholesteatoma and chronic otitis media.

PubMed

Kariya, Shin; Okano, Mitsuhiro; Zhao, Pengfei; Kataoka, Yuko; Yoshinobu, Junko; Maeda, Yukihide; Ishihara, Hisashi; Higaki, Takaya; Nishizaki, Kazunori

2016-01-01

The nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome plays an important role in the pathogenesis of middle ear diseases. Modulation of inflammasome-mediated inflammation may be a novel therapeutic strategy for cholesteatoma and chronic otitis media. NLRP3 inflammasome is a critical molecule mediating interleukin (IL)-1β responses. However, the expression of NLRP3 in the pathogenesis of cholesteatoma and chronic otitis media has not been fully examined. This study sought to assess the expression of NLRP3, ASC (apoptosis-associated speck-like protein containing a caspase recruitment domain and a pyrin domain), and caspase-1 in middle ear tissues in patients with cholesteatoma or chronic otitis media. Middle ear tissue samples were obtained from patients with cholesteatoma or chronic otitis media. Control middle ear samples were collected during cochlear implant surgery of patients without middle ear inflammation. The expression of NLRP3, ASC, and caspase-1 were examined by reverse transcription polymerase chain reaction (RT-PCR) assay and immunohistochemical study. The levels of mRNA of NLRP3, ASC, and caspase-1 were significantly elevated in cholesteatoma and chronic otitis media as compared with that of normal controls. The proteins of NLRP3, ASC, and caspase-1 were observed in infiltrating inflammatory cells in cholesteatoma and chronic otitis media.

BAAV mediated GJB2 gene transfer restores gap junction coupling in cochlear organotypic cultures from deaf Cx26Sox10Cre mice.

PubMed

Crispino, Giulia; Di Pasquale, Giovanni; Scimemi, Pietro; Rodriguez, Laura; Galindo Ramirez, Fabian; De Siati, Romolo Daniele; Santarelli, Rosa Maria; Arslan, Edoardo; Bortolozzi, Mario; Chiorini, John A; Mammano, Fabio

2011-01-01

The deafness locus DFNB1 contains GJB2, the gene encoding connexin26 and GJB6, encoding connexin30, which appear to be coordinately regulated in the inner ear. In this work, we investigated the expression and function of connexin26 and connexin30 from postnatal day 5 to adult age in double transgenic Cx26(Sox10Cre) mice, which we obtained by crossing connexin26 floxed mice with a deleter Sox10-Cre line. Cx26(Sox10Cre) mice presented with complete connexin26 ablation in the epithelial gap junction network of the cochlea, whereas connexin30 expression was developmentally delayed; immunolabeling patterns for both connexins were normal in the cochlear lateral wall. In vivo electrophysiological measurements in Cx26(Sox10Cre) mice revealed profound hearing loss accompanied by reduction of endocochlear potential, and functional experiments performed in postnatal cochlear organotypic cultures showed impaired gap junction coupling. Transduction of these cultures with a bovine adeno associated virus vector restored connexin26 protein expression and rescued gap junction coupling. These results suggest that restoration of normal connexin levels by gene delivery via recombinant adeno associated virus could be a way to rescue hearing function in DFNB1 mouse models and, in future, lead to the development of therapeutic interventions in humans.

Animal model of cochlear third window in the scala vestibuli or scala tympani.

PubMed

Attias, Joseph; Preis, Michal; Shemesh, Rafi; Hadar, Tuvia; Nageris, Ben I

2010-08-01

The auditory impact of a cochlear third window differs by its location in the scala vestibuli or scala tympani. Pathologic third window has been investigated primarily in the vestibular apparatus of animals and humans. Dehiscence of the superior semicircular canal is the clinical model. Fat sand rats (n = 11) have a unique inner-ear anatomy that allows easy surgical access. A window was drilled in the bony labyrinth over the scala vestibuli in 1 group (12 ears) and over the scala tympani in another (7 ears) while preserving the membranous labyrinth. Auditory brain stem responses to high- and low-frequency stimuli delivered by air and bone conduction were recorded before and after the procedure. Scala vestibuli group: preoperative air-conduction thresholds to clicks and tone-bursts averaged 8.3 and 9.6 dB, respectively, and bone-conduction thresholds, 4.6 and 3.3 dB, respectively; after fenestration, air-conduction thresholds averaged 40.4 and 41.8 dB, respectively, and bone-conduction thresholds, -1 and 5.6 dB, respectively. Scala tympani group: preoperative air-conduction thresholds to clicks and tone-bursts averaged 8.6 dB each, and bone-conduction thresholds, 7.9 dB and 7.1 dB, respectively; after fenestration, air-conduction thresholds averaged 11.4 and 9.3 dB, respectively, and bone-conduction thresholds, 9.3 and 4.2 dB, respectively. The changes in air- (p = 0.0001) and bone-conduction (p = 0.04) thresholds were statistically significant only in the scala vestibuli group. The presence of a cochlear third window over the scala vestibuli, but not over the scala tympani, causes a significant increase in air-conduction auditory thresholds. These results agree with the theoretic model and clinical findings and contribute to our understanding of vestibular dehiscence.

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.

Connecting the ear to the brain: molecular mechanisms of auditory circuit assembly

PubMed Central

Appler, Jessica M.; Goodrich, Lisa V.

2011-01-01

Our sense of hearing depends on precisely organized circuits that allow us to sense, perceive, and respond to complex sounds in our environment, from music and language to simple warning signals. Auditory processing begins in the cochlea of the inner ear, where sounds are detected by sensory hair cells and then transmitted to the central nervous system by spiral ganglion neurons, which faithfully preserve the frequency, intensity, and timing of each stimulus. During the assembly of auditory circuits, spiral ganglion neurons establish precise connections that link hair cells in the cochlea to target neurons in the auditory brainstem, develop specific firing properties, and elaborate unusual synapses both in the periphery and in the CNS. Understanding how spiral ganglion neurons acquire these unique properties is a key goal in auditory neuroscience, as these neurons represent the sole input of auditory information to the brain. In addition, the best currently available treatment for many forms of deafness is the cochlear implant, which compensates for lost hair cell function by directly stimulating the auditory nerve. Historically, studies of the auditory system have lagged behind other sensory systems due to the small size and inaccessibility of the inner ear. With the advent of new molecular genetic tools, this gap is narrowing. Here, we summarize recent insights into the cellular and molecular cues that guide the development of spiral ganglion neurons, from their origin in the proneurosensory domain of the otic vesicle to the formation of specialized synapses that ensure rapid and reliable transmission of sound information from the ear to the brain. PMID:21232575

Inner ear decompression sickness in compressed-air diving.

PubMed

Klingmann, Christoph

2012-01-01

Inner ear decompression sickness (IEDCS) has become more frequently reported in recreational diving. We examined 34 divers after IEDCS and analyzed their dive profiles, pattern of symptoms, time of symptom onset and the association with a right-to left shunt (r/l shunt). Four divers used mixed gas and were excluded from the analysis. Of the remaining 30 divers, 25 presented with isolated IEDCS alone, while five divers had additional skin and neurological symptoms. All divers presented with vertigo (100%), and 12 divers reported additional hearing loss (40%). All symptoms occurred within 120 minutes (median 30 minutes) of ascent. Twenty-two of 30 divers (73.3%) showed a r/l shunt. A possible explanation for the frequent association of a r/l shunt and the dominance of vestibular rather than cochlear symptoms could be attributed to the different blood supply of the inner ear structures and the different size of the labyrinthine compartments. The cochlea has a blood supply up to four times higher than the vestibular part of the inner ear, whereas the vestibular fluid space is 30% larger. The higher prevalence of symptoms referrable to the less well-perfused vestibular organ provides further evidence that persistent local inert gas supersaturation may cause growth of incoming arterial bubbles and may therefore be an important pathophysiological factor in IEDCS.

Cochlear implantation with hearing preservation yields significant benefit for speech recognition in complex listening environments.

PubMed

Gifford, René H; Dorman, Michael F; Skarzynski, Henryk; Lorens, Artur; Polak, Marek; Driscoll, Colin L W; Roland, Peter; Buchman, Craig A

2013-01-01

The aim of this study was to assess the benefit of having preserved acoustic hearing in the implanted ear for speech recognition in complex listening environments. The present study included a within-subjects, repeated-measures design including 21 English-speaking and 17 Polish-speaking cochlear implant (CI) recipients with preserved acoustic hearing in the implanted ear. The patients were implanted with electrodes that varied in insertion depth from 10 to 31 mm. Mean preoperative low-frequency thresholds (average of 125, 250, and 500 Hz) in the implanted ear were 39.3 and 23.4 dB HL for the English- and Polish-speaking participants, respectively. In one condition, speech perception was assessed in an eight-loudspeaker environment in which the speech signals were presented from one loudspeaker and restaurant noise was presented from all loudspeakers. In another condition, the signals were presented in a simulation of a reverberant environment with a reverberation time of 0.6 sec. The response measures included speech reception thresholds (SRTs) and percent correct sentence understanding for two test conditions: CI plus low-frequency hearing in the contralateral ear (bimodal condition) and CI plus low-frequency hearing in both ears (best-aided condition). A subset of six English-speaking listeners were also assessed on measures of interaural time difference thresholds for a 250-Hz signal. Small, but significant, improvements in performance (1.7-2.1 dB and 6-10 percentage points) were found for the best-aided condition versus the bimodal condition. Postoperative thresholds in the implanted ear were correlated with the degree of electric and acoustic stimulation (EAS) benefit for speech recognition in diffuse noise. There was no reliable relationship among measures of audiometric threshold in the implanted ear nor elevation in threshold after surgery and improvement in speech understanding in reverberation. There was a significant correlation between interaural time

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

Association of microRNA 146 with middle ear hyperplasia in pediatric otitis media.

PubMed

Samuels, Tina L; Yan, Justin; Khampang, Pawjai; MacKinnon, Alexander; Hong, Wenzhou; Johnston, Nikki; Kerschner, Joseph E

2016-09-01

Toll-like receptor signaling activated by bacterial otitis media pathogens in the middle ear has been shown to play a key role in OM susceptibility, pathogenesis and recovery. Recent studies implicate microRNA 146 (miR-146) in regulation of inflammation via negative feedback of toll-like receptor signaling (TLR) in a wide variety of tissues, however its involvement in otitis media is unknown. Human middle ear epithelial cells were stimulated with proinflammatory cytokines, interleukin 1 beta or tumor necrosis factor alpha, for two to twenty-four hours. Middle ear biopsies were collected from children with otitis media with effusion (n = 20), recurrent otitis media (n = 9), and control subjects undergoing cochlear implantation (n = 10). miR-146a, miR-146b expression was assayed by quantitative PCR (qPCR). Expression of miR-146 targets involved in TLR signaling, IRAK1 and TRAF6, was assayed by qPCR in middle ear biopsies. Middle ear biopsies were cryosectioned and epithelial thickness measured by a certified pathologist. Proinflammatory cytokines induced expression of miR-146 in middle ear epithelial cells in vitro. Middle ear miR-146a and miR-146b expression was elevated in otitis media patients relative to control subjects and correlated with middle ear epithelial thickness. A trend towards inverse correlation was observed between miR-146 and TRAF6 expression in the clinical population. This report is the first to assess miRNA expression in a clinical population with OM. Findings herein suggest miR-146 may play a role in OM. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

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

Pathophysiology of the inner ear after blast injury caused by laser-induced shock wave.

PubMed

Niwa, Katsuki; Mizutari, Kunio; Matsui, Toshiyasu; Kurioka, Takaomi; Matsunobu, Takeshi; Kawauchi, Satoko; Satoh, Yasushi; Sato, Shunichi; Shiotani, Akihiro; Kobayashi, Yasushi

2016-08-17

The ear is the organ that is most sensitive to blast overpressure, and ear damage is most frequently seen after blast exposure. Blast overpressure to the ear results in sensorineural hearing loss, which is untreatable and is often associated with a decline in the quality of life. In this study, we used a rat model to demonstrate the pathophysiological and structural changes in the inner ear that replicate pure sensorineural hearing loss associated with blast injury using laser-induced shock wave (LISW) without any conductive hearing loss. Our results indicate that threshold elevation of the auditory brainstem response (ABR) after blast exposure was primarily caused by outer hair cell dysfunction induced by stereociliary bundle disruption. The bundle disruption pattern was unique; disturbed stereocilia were mostly observed in the outermost row, whereas those in the inner and middle rows stereocilia remained intact. In addition, the ABR examination showed a reduction in wave I amplitude without elevation of the threshold in the lower energy exposure group. This phenomenon was caused by loss of the synaptic ribbon. This type of hearing dysfunction has recently been described as hidden hearing loss caused by cochlear neuropathy, which is associated with tinnitus or hyperacusis.

Biomechanical Modeling and Measurement of Blast Injury and Hearing Protection Mechanisms

DTIC Science & Technology

2015-10-01

12 software into Workbench V. 15 in CFX/ANSYS; 2) building the geometry of the ear model with ossicular chain and cochlear load in CFX; 3...the ear canal to middle ear. The model consists of the ear canal, TM, middle ear ossicles and suspensory ligaments, middle ear cavity, and cochlear ...the TM, ossicles, and ligaments/muscle tendons with the cochlear load applied on the stapes footplate. 17 Fig. 21. Time-history plots of

Otic ablation of smoothened reveals direct and indirect requirements for Hedgehog signaling in inner ear development

PubMed Central

Brown, Alexander S.; Epstein, Douglas J.

2011-01-01

In mouse embryos lacking sonic hedgehog (Shh), dorsoventral polarity within the otic vesicle is disrupted. Consequently, ventral otic derivatives, including the cochlear duct and saccule, fail to form, and dorsal otic derivatives, including the semicircular canals, endolymphatic duct and utricle, are malformed or absent. Since inner ear patterning and morphogenesis are heavily dependent on extracellular signals derived from tissues that are also compromised by the loss of Shh, the extent to which Shh signaling acts directly on the inner ear for its development is unclear. To address this question, we generated embryos in which smoothened (Smo), an essential transducer of Hedgehog (Hh) signaling, was conditionally inactivated in the otic epithelium (Smoecko). Ventral otic derivatives failed to form in Smoecko embryos, whereas vestibular structures developed properly. Consistent with these findings, we demonstrate that ventral, but not dorsal, otic identity is directly dependent on Hh. The role of Hh in cochlear-vestibular ganglion (cvg) formation is more complex, as both direct and indirect signaling mechanisms are implicated. Our data suggest that the loss of cvg neurons in Shh–/– animals is due, in part, to an increase in Wnt responsiveness in the otic vesicle, resulting in the ectopic expression of Tbx1 in the neurogenic domain and subsequent repression of Ngn1 transcription. A mitogenic role for Shh in cvg progenitor proliferation was also revealed in our analysis of Smoecko embryos. Taken together, these data contribute to a better understanding of the intrinsic and extrinsic signaling properties of Shh during inner ear development. PMID:21831920

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.

Adeno-associated virus-mediated gene delivery into the scala media of the normal and deafened adult mouse ear.

PubMed

Kilpatrick, L A; Li, Q; Yang, J; Goddard, J C; Fekete, D M; Lang, H

2011-06-01

Murine models are ideal for studying cochlear gene transfer, as many hearing loss-related mutations have been discovered and mapped within the mouse genome. However, because of the small size and delicate nature, the membranous labyrinth of the mouse is a challenging target for the delivery of viral vectors. To minimize injection trauma, we developed a procedure for the controlled release of adeno-associated viruses (AAVs) into the scala media of adult mice. This procedure poses minimal risk of injury to structures of the cochlea and middle ear, and allows for near-complete preservation of low and middle frequency hearing. In this study, transduction efficiency and cellular specificity of AAV vectors (serotypes 1, 2, 5, 6 and 8) were investigated in normal and drug-deafened ears. Using the cytomegalovirus promoter to drive gene expression, a variety of cell types were transduced successfully, including sensory hair cells and supporting cells, as well as cells in the auditory nerve and spiral ligament. Among all five serotypes, inner hair cells were the most effectively transduced cochlear cell type. All five serotypes of AAV vectors transduced cells of the auditory nerve, though serotype 8 was the most efficient vector for transduction. Our findings indicate that efficient AAV inoculation (via the scala media) can be performed in adult mouse ears, with hearing preservation a realistic goal. The procedure we describe may also have applications for intra-endolymphatic drug delivery in many mouse models of human deafness.

Adeno-associated virus-mediated gene delivery into the scala media of the normal and deafened adult mouse ear

PubMed Central

Kilpatrick, Lauren A.; Li, Qian; Yang, John; Goddard, John C; Fekete, Donna M.; Lang, Hainan

2010-01-01

Murine models are ideal for studying cochlear gene transfer as many hearing loss-related mutations have been discovered and mapped within the mouse genome. However, due to its small size and delicate nature, the membranous labyrinth of the mouse is a challenging target for delivery of viral vectors. To minimize injection trauma, we developed a procedure for the controlled release of adeno-associated viruses (AAV) into the scala media of adult mice. This procedure poses minimal risk of injury to structures of the cochlea and middle ear and allows for near-complete preservation of low and middle frequency hearing. In the present study, transduction efficiency and cellular specificity of AAV vectors (serotypes 1, 2, 5, 6, and 8) were investigated in normal and drug-deafened ears. Using the cytomegalovirus (CMV) promoter to drive gene expression, a variety of cell types were transduced successfully, including sensory hair cells and supporting cells, as well as cells in the auditory nerve and spiral ligament. Among all five serotypes, inner hair cells (IHCs) were the most effectively transduced cochlear cell type. All five serotypes of AAV vectors transduced cells of the auditory nerve, though serotype 8 was the most efficient vector for transduction. Our findings indicate that efficient AAV inoculation (via the scala media) can be performed in adult mouse ears, with hearing preservation a realistic goal. The procedure we describe may also have applications for intra-endolymphatic drug delivery in many mouse models of human deafness. PMID:21209625

Contralateral-noise effects on cochlear responses in anesthetized mice are dominated by feedback from an unknown pathway

PubMed Central

Usubuchi, Hajime; Vetter, Douglas E.; Elgoyhen, A. Bélen; Thomas, Steven A.; Liberman, M. Charles

2012-01-01

Suppression of ipsilateral distortion product otoacoustic emissions (DPOAEs) by contralateral noise is used in humans and animals to assay the strength of sound-evoked negative feedback from the medial olivocochlear (MOC) efferent pathway. However, depending on species and anesthesia, contributions of other feedback systems to the middle or inner ear can cloud the interpretation. Here, contributions of MOC and middle-ear muscle reflexes, as well as autonomic feedback, to contra-noise suppression in anesthetized mice are dissected by selectively eliminating each pathway by surgical transection, pharmacological blockade, or targeted gene deletion. When ipsilateral DPOAEs were evoked by low-level primaries, contra-noise suppression was typically ∼1 dB with contra-noise levels around 95 dB SPL, and it always disappeared upon contralateral cochlear destruction. Lack of middle-ear muscle contribution was suggested by persistence of contra-noise suppression after paralysis with curare, tensor tympani cauterization, or section of the facial nerve. Contribution of cochlear sympathetics was ruled out by studying mutant mice lacking adrenergic signaling (dopamine β-hydroxylase knockouts). Surprisingly, contra-noise effects on low-level DPOAEs were also not diminished by eliminating the MOC system pharmacologically (strychnine), surgically, or by deletion of relevant cholinergic receptors (α9/α10). In contrast, when ipsilateral DPOAEs were evoked by high-level primaries, the contra-noise suppression, although comparable in magnitude, was largely eliminated by MOC blockade or section. Possible alternate pathways are discussed for the source of contra-noise-evoked effects at low ipsilateral levels. PMID:22514298

Magnetic stem cell targeting to the inner ear

NASA Astrophysics Data System (ADS)

Le, T. N.; Straatman, L.; Yanai, A.; Rahmanian, R.; Garnis, C.; Häfeli, U. O.; Poblete, T.; Westerberg, B. D.; Gregory-Evans, K.

2017-12-01

Severe sensorineural deafness is often accompanied by a loss of auditory neurons in addition to injury of the cochlear epithelium and hair cell loss. Cochlear implant function however depends on a healthy complement of neurons and their preservation is vital in achieving optimal results. We have developed a technique to target mesenchymal stem cells (MSCs) to a deafened rat cochlea. We then assessed the neuroprotective effect of systematically delivered MSCs on the survival and function of spiral ganglion neurons (SGNs). MSCs were labeled with superparamagnetic nanoparticles, injected via the systemic circulation, and targeted using a magnetized cochlea implant and external magnet. Neurotrophic factor concentrations, survival of SGNs, and auditory function were assessed at 1 week and 4 weeks after treatments and compared against multiple control groups. Significant numbers of magnetically targeted MSCs (>30 MSCs/section) were present in the cochlea with accompanied elevation of brain-derived neurotrophic factor and glial cell-derived neurotrophic factor levels (p < 0.001). In addition we saw improved survival of SGNs (approximately 80% survival at 4 weeks). Hearing threshold levels in magnetically targeted rats were found to be significantly better than those of control rats (p < 0.05). These results indicate that magnetic targeting of MSCs to the cochlea can be accomplished with a magnetized cochlear permalloy implant and an external magnet. The targeted stem cells release neurotrophic factors which results in improved SGN survival and hearing recovery. Combining magnetic cell-based therapy and cochlear implantation may improve cochlear implant function in treating deafness.