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, ...

potentials in the presence of normal cochlear micro- phonic and cognitive potentials suggested that synchrony). The presence of cochlear micro- phonic potentials and otoacoustic emissions indicated that cochlear function

The organ of Corti of Genetically Epilepsy Prone Rats was examined anatomically and electrophysiologically using scanning electron microscopy (SEM) and electrophysiological recording of alternating current cochlear potentials (ACCP) and N1, a volume conductor recording of the primary auditory afferent action potentials. ACCPs for GEPRs ...

The potential influence of auditory information in the production of /s/ and /integral of/ was explored for postlingually deafened adults with four-channel Ineraid cochlear implants. Analyses of the spectra of the sibilant sounds were compared for speech obtained prior to implant activation, after early implant use and after 6 months of use. In addition, ...

Objective:To report a patient with Pendred syndrome who developed life-threatening hypokalaemia as an unpredicted consequence of implant-induced imbalance and alcohol dependency, leading to multiple cardiac arrests.Setting:Addenbrooke's Hospital, Cambridge, UK.Method:Case report and review of the English language literature concerning Pendred syndrome and cochlear implantation ...

The BETA2/NeuroD1 null mouse has cochlear dysplasia. Its cochlear duct is shorter than normal, there is a lack of spiral ganglion neurons, and there is hair cell disorganization. We measured vertical movements of the tectorial membrane at acoustic frequencies in excised cochleae in response to mechanical stimulation of the stapes using laser doppler ...

ABSTRACT : Auditory neuropathy (AN) is a disorder characterized by disruption of auditory nerve activity resulting from lesions involving the auditory nerve (postsynaptic AN), inner hair cells and/or the synapses with auditory nerve terminals (presynaptic AN). Affected subjects show impairment of speech perception beyond that expected for the hearing loss, abnormality of ...

Distortion generated by the cochlea can provide a valuable indicator of its functional state. In the present study, the dependence of distortion on the operating point of the cochlear transducer and its relevance to endolymph volume disturbances has been investigated. Calculations have suggested that as the operating point moves away from zero, second harmonic distortion would ...

The sensitivity of the cochlear microphonic for the vampire bat, Desmodus, has been determined from 0.1 to 100 kc/s. The cochlear microphonics were compared with those of the less sensitive bat Myotis. A first attempt was made to evaluate the role of the ...

The structures of cochlear transduction include stereocilia at the apical surface of hair cells and their connection to the tectorial membrane. The transduction site is one of the loci for noise-induced cochlear damage. Although stereocilia are susceptible to noise, it has been found that in the inner ears of avians, this fragile structure is largely ...

The objective of the present study was to evaluate the integrity of the peripheral and central auditory systems of sickle cell disease (SCD) patients, through electrophysiological evaluation utilizing auditory evoked potentials, and comparing the results obtained in SCD patients with individuals without SCD. A total of 80 individuals were evaluated: 40 SCD patients; and 40 ...

Further measurements of cochlear potentials in the bats of the species Myotis lucifugus confirm earlier results indicating poor sensitivity for low tones and only moderate sensitivity for high tones. It appears that this bat's performance in echolocation ...

The primary aim of the present experiment was to evaluate the high and low frequency responses of the rat's ear using the cochlear potential method. Further experiments were conducted to determine the responses of the rat's ear to variations in sound inte...

... Click-evoked potentials were recorded from unanesthetized cats with electrodes chronically implanted in the auditory cortex, cochlear nucleus, and ...

... A chronic electrode is implanted in the auditory system, usually in the ... on the visual detection of a derived response of the cochlear potential (Pantev ...

This paper discusses how ion transport proteins in the hair cells of the mammalian cochlea work to produce a sensitive but stable hearing organ. The transport proteins in the inner and outer hair cells are summarized (including their current voltage characteristics), and the roles of these proteins in determining intracellular Ca(2+), membrane potential, and ultimately ...

Schairer et al. [(2003). �Effects of peripheral nonlinearity on psychometric functions for forward-masked tones,� J. Acoust. Soc. Am. 133, 1560�1573] demonstrated that cochlear nonlinearity is reflected in psychometric-function (PF) slopes for 4 kHz forward-masked tones. The goals of the current study were to use PF slopes to compare the degree of compression between ...

Summated electrical responses and action potentials of single neurons of the cochlear nuclei were studied in Vespertilionidae and Rhinolophidae. In Myotis (Vespertilionida), maximum sensitivity to ultrasound was recorded in frequency bands of 10-30 and 70...

This longitudinal study investigated how chronic gunshot noise exposure affects cochlear and saccular function in police officers who engaged in regular target shooting practice using dual protection (ear plugs plus earmuffs) for >10 years. In 1997, 20 male police officers underwent audiometry before and two weeks after shooting. Twelve of the original subjects were ...

Auditory synaptopathy/neuropathy (AS/AN) is a special subtype of sensorineural hearing disorders with heterogeneous phenotypes and underestimated incidence. AS/AN generally develops in infancy, occasionally in adulthood. Symptoms include fluctuating, mostly bilateral hearing loss and abnormally reduced speech comprehension, especially in noisy environments. Within audiological ...

Recently, significant progress has been made in understanding the contribution of the mammalian cochlear outer hair cells (OHCs) to normal auditory signal processing. In the present paper an outer hair cell model is incorporated in a complete, time-domain, one-dimensional cochlear model. The two models control each other through ...

measures in Nucleus 24 cochlear implant users. Ear and Hearing, 22(6), 471-486. PRESENTATIONS Vander Werff). Using steady state auditory evoked potentials to assist with determination of cochlear implant candidacy in electrode impedance and EAP growth functions for adult Nucleus 24 cochlear implant users. Poster ...

Alstr�m Syndrome is a life-threatening disease characterized primarily by numerous metabolic abnormalities, retinal degeneration, cardiomyopathy, kidney and liver disease, and sensorineural hearing loss. The cellular localization of the affected protein, ALMS1, has suggested roles in ciliary function and/or ciliogenesis. We have investigated the role of ALMS1 in the cochlea ...

Recent electrophysiological studies on the activity of single auditory-nerve fibres in normal animals provide a basis for parallel studies in animals with pathological conditions in the cochlea. In order to create cochlear lesions, cats were treated with ...

ObjectivesTo examine whether cochlear implantation increases the risk of meningitis in the absence of other risk factors, and to understand the pathogenesis of pneumococcal meningitis post cochlear implantation.Study Design and Setting A quantitative threshold model in rats was established to examine ...

ObjectivesTo describes a cadaveric temporal bone model of labyrinthitis ossificans and investigate the utility of intra-operative C-arm computed tomography (CBCT) in facilitating cochlear implantation.DesignCadaveric temporal bone study.MethodsFive cadaveric heads had cement introduced into the 10 cochleas. CBCT and a conventional CT scans were compared to asses the extent of ...

Bilateral cochlear implantation has recently been introduced with the aim of improving both speech perception in background noise and sound localization. Although evidence suggests that binaural perception is possible with two cochlear implants, results in humans are variable. To explore potential contributing factors to these variable ...

Bilateral cochlear implantation has recently been introduced with the aim of improving both speech perception in background noise and sound localization. Although evidence suggests that binaural perception is possible with two cochlear implants, results in humans are variable. To explore potential contributing factors to these variable ...

... The cochlear nucleus presented continuously at a rate of one per second as background electrodes were stereotaxically implanted through small ...

... Recording Cortical Potentials from Animals with Implanted Electrodes; A ... in Visual and Auditory Absolute Thresholds; Cochlear Nucleus Studies ...

... Corporate Author : CENTRAL INST FOR THE DEAF ST LOUIS MO. Personal Author(s) : TASAKI, I. ; DAVIS, H. ; ELDREDGE, DH. ...

... Cohen, LT, Burton, MJ, Clark, GM, Steady state evoked potentials: A new tool for the accurate assessment of hearing in cochlear implant candidates ...

Mutations of SLC26A4 are among the most prevalent causes of hereditary deafness. Deafness in the corresponding mouse model, Slc26a4^?/?, results from an abnormally enlarged cochlear lumen. The goal of this study was to determine whether the cochlear enlargement originates with defective ...

Common potentials used to evaluate cochlear function are the ac cochlear potential (ACCP), N1 and the positive dc endocochlear potential (EP). The ACCP is an electrical analogue of the sound stimulus; its source is the electrical activity of the cochlear hair cells. N1 is a ...

A decision aid for parents considering sequential bilateral cochlear implantation for their children with severe-to-profound hearing loss was developed using local and published evidence. Eight parents of children currently using one cochlear implant, who faced a decision regarding a second cochlear implant, and five clinicians ...

A large amount of energy produced by active aerobic metabolism is necessary for the cochlea to maintain its function. This makes the cochlea vulnerable to blockade of cochlear blood flow and interruption of the oxygen supply. Although certain forms of human idiopathic sudden sensorineural hearing loss reportedly arise from ischemic injury, the pathological mechanism of ...

... and agencies for Research Development Test and ... to Follow-up of Breast Abnormalities in Low ... with a potential breast abnormality through the ...

... and agencies for Research Development Test and ... to Follow-Up of Breast Abnormalities in Low ... with a potential breast abnormality through the ...

Intra-operative electrocochleography was undertaken during surgery for Meni�re's disease. It was found that the electrocochleogram (ECoG) provided a stable measure which accurately reflected changes within the inner ear during surgery. During salt osmosis of the round window, a rapid change in the summating potential versus action potential ratio (SP/AP) ...

Many functions have been ascribed to polyamines, but there has been no clear identification of a unique role for spermine. The Gy mouse has a deletion of part of the X chromosome that includes the SMS gene encoding spermine synthase. Tissues from male Gy mice have no spermine but increased spermidine. They have multiple abnormalities including a tendency to sudden death, small ...

Measurement were made of the cochlear potentials in three specimens of the giant sea turtle, Chelonia mydas, in response to both aerial and mechanical stimulation of the ear. The results show maximum sensitivity in the region of 300 to 400 Hz, with a rather rapid decline in sensitivity for lower and especially for higher tones. The upper limit for the ...

It is well established that an active process, the 'cochlear amplifier' increases auditory sensitivity and frequency resolution in the basal, high frequency regions of the inner ear. The amplifier depends critically on the endocochlear potential, the value of which will greatly influence hair cell transduction and amplification. In the apical, low ...

OBJECTIVES:: Hypoplasia of the auditory nerve (AN) refers to significant narrowing of the VIIIth cranial nerve which could compromise stimulation of the nerve by electrical pulses delivered from a cochlear implant (CI), thereby hindering activity in other parts of the auditory pathways. To compensate, high current levels or increased charge may be required to elicit auditory ...

Oscillatory changes in the intensity of OAEs are seen in human ears after brief loud sound exposures. The oscillations have a period of about 200s (frequency, 5mHz) and they parallel oscillations seen in hearing threshold. We propose that these fluctuations are a byproduct of a control system affecting the organ of Corti which governs cochlear amplifier gain and contributes to ...

Ninety patients who had suspected or confirmed fenestral or cochlear otosclerosis underwent CT examination. Foci of demineralization in the otic capsule were discovered in 20 ears (12 patients). Audiometric studies of the 12 patients revealed sensorineural hearing loss (SNHL) with distinct correlation of CT findings with progressivity and with involvement of the frequency ...

Despite preserved cell differentiation, the Reln(rl-Orl) phenotype comprises laminar abnormalities of cell position in auditory cortex and dorsal cochlear nucleus. The metabolic consequences of the cell ectopias were determined by estimating cytochrome oxidase (CO) activity, a marker of neuronal activity. CO activity increased in the granular cell layer of ...

Background:Gene therapy is a potentially effective therapeutic modality for treating sensorineural hearing loss. Nonviral gene delivery vectors are expected to become extremely safe and convenient, and nanoparticles are the most promising types of vectors. However, infrequent nuclear localization in the cochlear cells limits their application for gene ...

We used a combination of subtractive hybridization and differential screening strategies to identify genes that may function normally in hearing and, when mutated, result in deafness. A human fetal cochlear (membranous labyrinth) cDNA library was subtracted against total human fetal brain RNAs by an avidin-biotin-based procedure to enrich for cochlear ...

The predominant conceptual framework for understanding human age-related hearing loss (ARHL, or presbycusis) holds that three different cochlear elements (organ of Corti, afferent neurons, and stria vascularis) can degenerate independently, and exert independent influences on hearing. Within this framework, temporal bones from subjects with ARHL may be classified as ...

1. A method for perfusing the scala tympani of the cat's cochlea from basal turn to apex is described. The perfusion with modified Krebs solution did not interfere with the recording of cochlear microphonic (CM) and neural responses to sound, nor with the efferent inhibition elicited by stereotaxic stimulation of the crossed olivo-cochlear bundle (COCB) in ...

Auditory brainstem implants provide sound information by direct stimulation of the cochlear nucleus to patients with dysfunctional or absent cranial nerve VIII. In contrast to patients with cochlear implants, the use of the auditory brainstem implants is less successful. This cannot be fully explained by the difference location of stimulation but a rather ...

This paper provides an overview and a synthesis of the findings of a large, multifaceted study investigating outcomes from paediatric cochlear implantation. The study included children implanted at several Australian implant clinics and attending a variety of early intervention and educational settings across a range of locations in eastern Australia. It investigated three ...

This paper provides an overview and a synthesis of the findings of a large, multifaceted study investigating outcomes from paediatric cochlear implantation. The study included children implanted at several Australian implant clinics and attending a variety of early intervention and educational settings across a range of locations in eastern Australia. It investigated three ...

Cochlear implants (CIs) process sounds electronically and then transmit electric stimulation to the cochlea of individuals with sensorineural deafness, restoring some sensation of auditory perception. Many congenitally deaf CI recipients achieve a high degree of accuracy in speech perception and develop near-normal language skills. Post-lingually deafened implant recipients ...

The objective of the study was to investigate the potential for clinical application of neural response imaging (NRI) cochlear mapping. Cochlear mapping was performed at each fitting session up to at least six months following initial fitting. Stimulation was delivered to one electrode site. NRI was recorded from each of the remaining ...

This paper summarizes recent research on functions of the cochlea of the inner ear. The cochlea is described as the seat of the first step in the auditory sound analysis and transduction of mechanical vibration into electrochemical processes leading to the generation of neural action potentials. The cochlea is also described as a frequent seat of auditory disorders. This ...

... help him or her to understand speech. Top How does a cochlear implant work? A cochlear implant is ... months for one type of cochlear implant. Top How does someone receive a cochlear implant? Use of a ...

The main aim of this project is to develop a research interface platform which can be used by researchers interested in exploring new ideas to improve cochlear implant devices. This research platform includes a stimulator unit which can be used for electrical stimulation in animal studies, a recording unit for collecting evoked potentials from human ...

In non-mammalian, hair cell-bearing sense organs amplification is associated with mechano-electric transducer channels in the stereovilli (commonly called stereocilia). Because mammals possess differentiated outer hair cells (OHC), they also benefit from a novel electromotile process, powered by the motor protein, prestin. Here we consider new work pertaining to this protein and its ...

The main aim of this project is to develop a research interface platform which can be used by researchers interested in exploring new ideas to improve cochlear implant devices. This research platform includes a stimulator unit which can be used for electrical stimulation in animal studies, a recording unit for collecting evoked potentials from

This article reviews the current status of cochlear implantation in both adults and children, including expanding candidacy groups, bilateral implantation, advances in speech processing software, internal and external device hardware, surgical techniques and outcomes. Promising advances, novel therapies and evolving concepts are also highlighted in terms of their future impact ...

The basic helix-loop-helix (bHLH) transcription factor Math5 (Atoh7) is required for retinal ganglion cell (RGC) and optic nerve development. Using Math5-lacZ knockout mice, we have identified an additional expression domain for Math5 outside the eye, in functionally connected structures of the central auditory system. In the adult hindbrain, the cytoplasmic Math5-lacZ reporter is expressed within ...

The basic helix-loop-helix (bHLH) transcription factor Math5 (Atoh7) is required for retinal ganglion cell (RGC) and optic nerve development. Using Math5-lacZ knockout mice, we have identified an additional expression domain for Math5 outside the eye, in functionally connected structures of the central auditory system. In the adult hindbrain, the cytoplasmic Math5-lacZ reporter is expressed within ...

Epithelial cells acquire diverse shapes relating to their different functions. This is particularly relevant for the cochlear outer hair cells (OHCs), whose apical and basolateral shapes accommodate the functioning of these cells as mechano-electrical and electromechanical transducers, respectively. We uncovered a circumferential shape transition of the apical junctional ...

The combined findings of "normal evoked otoacoustic emissions (EOAE) and absent or markedly disturbed auditory evoked potentials from the brainstem and/or cortex" has been named auditory neuropathy in the English literature. The pure tone thresholds in these patients range from mild to severe loss. The loss of speech comprehension is out of proportion in comparison to the pure ...

The deaf-circling Ames waltzer (av) mouse harbors a mutation in the protocadherin 15 (Pcdh15) gene and is a model for inner ear defects associated with Usher syndrome type 1F. Earlier studies showed altered cochlear hair cell morphology in young av mice. In contrast, no structural abnormality consistent with significant vestibular dysfunction in young av ...

Preventive effects of carbogen on decreases in endocochlear potential (EP) and cochlear microcirculation induced by ischemia of the cochlea were examined in guinea pigs with intravital microscopy. The experimental model giving a severe decline in cochlear blood flow (CBF) was established by occlusion of both common carotid arteries and ...

Auditory evoked potentials are tools widely used to assess auditory cortex functions in clinical context. However, in cochlear implant users, electrophysiological measures are challenging due to implant-created artefacts in the EEG. Here, we used independent component analysis to reduce cochlear implant-related artefacts in ...

Cochlear implants work well, yet the outcome is not fully accounted by the data routinely available to the clinician, and remains unpredictable. A more in-depth understanding of the neural mechanisms that determine the clinical recovery after cochlear implantation is warranted, as they may provide the background for an accurate individual prognosis. In ...

Hyrtl's fissure is a cleft that is present in the developing fetal petrous temporal bone and extends from the area inferior to the round window to the meninges of the posterior fossa. Persistent Hyrtl's fissure, due to incomplete ossification, is considered a rare temporal bone malformation, and is a known cause of perilabyrinthine cerebrospinal fluid fistula. Very few cases are reported as being ...

SummaryA rare case of cochlear implant surgery complication is described: air collection behind the ear. A 61-year-old male with a 20-year history of progressive bilateral profound sensorineural hearing loss underwent cochlear implant surgery on the left ear with Clarion Hi-Res 90K (Advanced Bionics, Sylmar, CA, USA). Ten days after surgery, the patient ...

... biopsy using radiological guidance. This is known as stereotactic breast biopsy. The potentially abnormal area is located ...

Spiral ganglion neurons (SGNs) are the target cells of the cochlear implant, a neural prosthesis designed to provide important auditory cues to severely or profoundly deaf patients. The ongoing degeneration of SGNs that occurs following a sensorineural hearing loss is therefore considered a limiting factor in cochlear implant efficacy. We review ...

Thirty-five consecutive patients with the clinical diagnosis of fenestral otosclerosis were evaluated with high-resolution computed tomography (CT). Twenty-six were diagnosed as having this disorder by CT evidence of abnormal bony excrescences at or adjacent to the oval window. Sections were also evaluated for evidence of plaque formation elsewhere in the lateral wall of the ...

In two species of chameleons (Chamaeleo senegalensis and C. quilensis) the auditory sensitivity was measured in terms of the cochlear potentials. In these lizards there is no external ear opening or tympanic membrane and the round window of the cochlea is...

Vascular embolic and thrombotic problems postulated to be the cause of inner ear sudden deafness have been reported with decompression sickness also. Decompression sickness has been found to lead to cochlear potential loss in the guinea pig, and these los...

Middle ear muscle (MEM) activity in awake Chilonycteris p. parnelli was investigated by observing attenuations in cochlear microphonic potentials. The contracted MEMs were found to act as high pass filters that only attenuated the energy in frequencies be...

Studies of cochlear microphonic potentials during landing maneuvers were conducted with special focus on the degree of Doppler shift encountered in real flights and the exact placement of output frequencies and Doppler shifted echoes in reference to the b...

... Wever, EG and Vernon, JA Hearing in the bat, Myotis lucifugus, as shown by the cochlear potentials. J. Aud. Res., 1961b, 2, 158-175. 22 Page 35. ...

Cochlear potentials measured in several species of lizard show a close correlation between maximum electrical output and number of hair cells, whereas there is no uniform relation to sensitivity. These results are interpreted as indicating structural diff...

Some preliminary results of a research program on the effects of noise traumata on cochlear functioning are given. Measurements reported include the latency of the whole-nerve (compound) action potential (AP), the selectivity of frequency-tuning curves (e...

. ABSOLUTE Contraindications: If you have any of the following items attached or implanted within your body pacemaker (potential malfunction) Yes No __ __ Cochlear implant in ear (possibly dislodged) Yes or ventricular) Yes No __ __ Dental work done in eastern Europe Yes No __ __ Neurostimulator (Tens unit) Yes

A study of four specimens of the foothill alligator lizard, Gerrhonotus multicarinatus, by the cochlear potential method revealed an ear maximally sensitive to tones below 1000 cycles with sensitivity rapidly falling off for higher frequencies. The irregu...

In this study, a new monitoring system is developed to measure cochlear blood flow (CBF) and electrocochleography (ECochG) during transient ischemic episodes of the cochlea. A newly designed otic probe was used for the simultaneous recordings of laser-Doppler CBF and ECochG directly from the round window (RW). The probe enabled the recording of high amplitude compound action ...

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

Distortion product otoacoustic emissions (DPOAEs) evoked by low-level tones are a sensitive indicator of outer hair cell (OHC) function. High-level DPOAEs are less vulnerable to cochlear insult, and their dependence on the OHC function is more controversial. Here, the mechanism underlying high-level DPOAE generation is addressed using a mutant mouse line lacking prestin, the ...

Distortion product otoacoustic emissions (DPOAEs) evoked by low-level tones are a sensitive indicator of outer hair cell (OHC) function. High-level DPOAEs are less vulnerable to cochlear insult, and their dependence on the OHC function is more controversial. Here, the mechanism underlying high-level DPOAE generation is addressed using a mutant mouse line lacking prestin, the ...

Changing the concentration of cholesterol in the plasma membrane of isolated outer hair cells modulates electromotility and prestin-associated charge movement, suggesting that a similar manipulation would alter cochlear mechanics. We examined cochlear function before and after depletion of membrane cholesterol with methyl-?-cyclodextrin (M?CD) in an ...

OBJECTIVEThe purpose of the current study was to longitudinally assess the development of automatic sound feature discrimination and compare it to behavioral discrimination in late-implanted cochlear implant users.METHODSScalp-recorded auditory evoked potentials (AEPs) and behavioral discrimination of frequency, duration and intensity differences within an ...

Loss of ganglion cells is a common and irreversible complication of hair cell loss in the cochlea. Gene transfer could potentially be used to prevent this neuronal degeneration and other pathologies in the cochlea. Human adenoviruses should provide a feasible gene transfer vehicle for transducing the quiescent cochlear neurons and organ of Corti ...

The effect of ethacrynic acid (EA) at different blood O₂ saturations on cochlear potentials of guinea pigs was investigated. All 18 young healthy guinea pigs received 50 mg/kg/h of EA intravenously and were divided into three groups: first group, normal (90.00�6.30-86.17�4.83 mm Hg); second group, lower Po₂ ...

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 ...

Studies have shown that unilateral cochlear implant users who have residual hearing in the contralateral ear can benefit from combining a hearing aid in the nonimplanted ear with their cochlear implant. The purpose of this study was to better understand the factors influencing decision making by adults. Adults who had discontinued hearing aid use shortly ...

A cochlear implant is an electronic device designed to provide sound information for adults and children who have bilateral profound hearing loss. The task of representing speech signals as electrical stimuli is central to the design and performance of cochlear implants. Studies have shown that the current speech- processing strategies provide significant ...

Thyroid hormone plays an important role in hearing development. Hereditary hypothyroidism is frequently associated with sensorineural hearing loss as identified in both animal models and human patients. Building upon our original demonstration of congenital deafness and hair cell abnormality in a hyt/hyt mouse model which carries an autosomal recessive mutation causing ...

Congenital deafness results in synaptic abnormalities in auditory nerve endings. These abnormalities are most prominent in terminals called endbulbs of Held, which are large, axosomatic synaptic endings whose size and evolutionary conservation emphasize their importance. Transmission jitter, delay, or failures, which would corrupt the processing of timing ...