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PubMed1988

The actin filament content of hair cells of the bird cochlea is nearly constant even though the length, width, and number of stereocilia vary depending on the hair cell location.

By direct counts off scanning electron micrographs, we determined the number of stereocilia per hair cell of the chicken cochlea as a function of the position of the hair cell on the cochlea. Micrographs of thin cross sections of stereociliary bundles located at known positions on the cochlea were enlarged and the total number of actin filaments per stereocilium was counted and recorded. By comparing the counts of filament number with measurements of actin filament bundle width of the same stereocilium, we were able to relate actin filament bundle width to filament number with an error margin (r2) of 16%. Combining this data with data already published or in the process of publication from our laboratory on the length and width of stereocilia, we were able to calculate the total length of actin filaments present in stereociliary bundles of hair cells located at a variety of positions on the cochlea. We found that stereociliary bundles of hair cells contain 80,000-98,000 micron of actin filament, i.e., the concentration of actin is constant in all hair cells with a range of values that is less than our error in measurement and/or biological variation, the greatest variation being in relating the diameters of the stereocilia to filament number. We also calculated the membrane surface needed to cover the stereocilia of hair cells located throughout the cochlea. The values (172-192 micron 2) are also constant. The implications of our observation that the total amount of actin is constant even though the length, width, and number of stereocilia per hair cell vary are discussed.

Actins↗

PubMed1998

Surgical technique for implantation of the totally ossified cochlea.

OBJECTIVES: Among the technical challenges of cochlear implant surgery is electrode insertion into the fully ossified cochlea. Earlier drill-out techniques have two significant drawbacks: 1. up to one half fail when the electrode pulls away from the cochlea; and 2. extended radical mastoidectomy, abdominal fat graft for obliteration, and closure of the external auditory meatus are required. A simplified technique is described that allows positive fixation of the cochlear implant electrode and in selected cases avoids a radical cavity with obliteration. STUDY DESIGN: Technical description with case reports and hearing outcomes. METHODS: Ten cadaver temporal bones were dissected to determine middle ear landmarks that overlie the basal turn of the cochlea from the transcanal approach and to establish the feasibility of the intact canal wall procedure. Surgery was performed on four patients who had preoperative imaging evidence of full ossification, two with the canal wall down, and two with canal wall up. Standard measures of speech recognition were used to evaluate hearing. RESULTS: Critical surgical landmarks are the round window, carotid artery, cochleariform process, and oval window. The procedure was successfully performed on four patients and open-set speech recognition is present in each. CONCLUSIONS: This canal wall up procedure allows long electrode insertion without radical cavity/obliteration in patients with fully ossified cochleas and prevents distraction of the electrode from the cochlea. While open-set word recognition was achieved by all subjects, results are poorer than expected for patients with limited or no ossification.

Audiometry, Speech↗

PubMed2000

Long-term follow-up of cochlear implant users with ossified cochlea.

OBJECTIVE: Cochlear implantation surgery in ossified cochlea is a challenge, even for the experienced otologist. Short-term assessments of auditory perception show that implantation in partial or even extensive ossified cochlea could be achieved with varying success, but no long-term follow-up results have been published yet. DESIGN AND METHODS: This paper proposes a retrospective review of eight Nucleus cochlear implant users with ossified cochlea who have been followed on a 12- to 60-month period. MAIN OUTCOME MEASURE: Auditory performances of users are reported at each control on a scale of 100 units divided into 4 skill zones of 25 units based on Erber's proposition, that is, detection, discrimination, identification, and recognition. RESULTS: Three of the eight subjects showed some progression in their auditory performances during the follow-up. The five other subjects showed no long-term progress in their auditory performance. CONCLUSIONS: Open-set comprehension could be achieved with the insertion of 9 to 10 electrodes of the Nucleus device. Auditory performance in users with ossified cochlea seems to be influenced by the same factors as in users with patent cochlea.

Adult↗

PubMed1991

Flexible fiberoptic endoscopy of the cochlea: human temporal bone studies.

New bone formation within the cochlea has been found more frequently than expected during cochlear implantation. Although implantation of partially ossified cochleas with long multichannel electrodes is possible, in practicality it may be difficult to determine whether electrode insertion is feasible prior to attempting it. In spite of use of high resolution computerized tomography, the presence, nature, and extent of new bone formation in the scala tympani is often difficult to ascertain. Flexible fiberoptic otologic endoscopes, 0.7 mm and 1.0 mm in diameter, were developed for the purpose of visualizing the fluid-containing scalae of the cochlea. In order to determine the efficacy of the device in visualizing the interior of the cochlea, as well as determining possible damage caused by its use, ten formalin fixed human temporal bones were studied. The flexible fiberoptic otologic endoscope was found to be capable of passage through the scala tympani of the cochlea in the inferior segment and of identifying normal and abnormal structures. Passage beyond this point resulted in damage to the spiral ligament. We conclude that human trials of use of the endoscope during cochlear implantation on previously deafened individuals is feasible.

Cochlear Implants↗

PubMed2005

Distribution of type IV collagen in the cochlea in Alport syndrome.

OBJECTIVE: To determine the distribution of alpha1, alpha3, and alpha5 chains of type IV collagen in the cochlea in Alport syndrome. DESIGN: Case-control study. PATIENTS: Two patients with sensorineural hearing loss due to Alport syndrome. Both patients had known mutations in the COL4A5 gene. MAIN OUTCOME MEASURES: Immunostaining was used to study the distribution of type IV collagen (alpha1, alpha3, and alpha5 chains) within the cochlea. Immunostaining was also performed in the cochlear tissues of an unaffected individual used as a control. RESULTS: In the control ear, alpha1 staining was observed in the basement membrane overlying the basilar membrane, in the basement membrane of cochlear blood vessels and Schwann cells, and within the spiral limbus. In the control ear, we also observed strong staining for alpha3 and alpha5 chains in the basement membrane overlying the basilar membrane and within the spiral ligament. In both cases with Alport syndrome, no immunostaining was observed for alpha3 or alpha5 chains within the cochlea, whereas alpha1 staining was present in locations similar to that seen in the control ear. CONCLUSIONS: The results indicate that isotype switching does not occur within the cochlea in Alport syndrome. The results are also consistent with the hypothesis that the sensorineural hearing loss in Alport syndrome may be due to alterations in cochlear micromechanics and/or dysfunction of the spiral ligament.

Adult↗

PubMed1996

Flexible fiberoptic endoscopy and laser surgery in obliterated cochleas: human temporal bone studies.

BACKGROUND AND OBJECTIVE: The use of conventional drilling procedures in cochlear implant surgery of ossified cochleae poses special risks to the facial nerve and the carotid artery. This study evaluated the alternate use of flexible fiberoptic endoscopy and mid-infrared laser surgery for recanalization of partially and artificially obliterated cochleae in freshly dissected human cadavers. STUDY DESIGN/MATERIALS AND METHODS: A pulsed Holmium:YAG-laser (lambda = 2120 nm) was used in the free-running mode (1180 mJ, 250 microseconds pulse, 5 Hz). A 660 microns optic quartz fiber was positioned in the center of the round window niche and slowly--endoscopically guided--advanced in contact shooting over 1.5 cm, creating by vaporization and photoablation a passage through the artificial bony occlusion in the basal segment of the cochlea. RESULTS: In all experiments, laser application (110-130 pulses) resulted in complete recanalization of the bony occlusions without damaging surrounding structures. The microendoscopy proved capable of guiding the laser fiber through the curved segment of the basal turn allowing identification of normal bone, bone cement, and laser-treated bone cement. CONCLUSION: If partial ossification of the basal turn is present, this technique could give access to place analog as well as digital implants deep within the cochlea.

Cochlea↗

PubMed2005

Developmental regulation of neuron-specific P2X3 receptor expression in the rat cochlea.

ATP-gated ion channels assembled from P2X3 receptor (P2X3R) subunits contribute to neurotransmission and neurotrophic signaling, associated with neurite development and synaptogenesis, particularly in peripheral sensory neurons. Here, P2X3R expression was characterized in the rat cochlea from embryonic day 16 (E16) to adult (P49-56), using RT-PCR and immunohistochemistry. P2X3R mRNA was strongly expressed in the cochlea prior to birth, declined to a minimal level at P14, and was absent in adult tissue. P2X3R protein expression was confined to spiral ganglion neurons (SGN) within Rosenthal's canal of the cochlea. At E16, immunolabeling was detected in the SGN neurites, but not the distal neurite projection within the developing sensory epithelium (greater epithelial ridge). From E18, the immunolabeling was observed in the peripheral neurites innervating the inner hair cells but was reduced by P6. However, from P2-8, immunolabeling of the SGN neurites extended to include the outer spiral bundle fiber tract beneath the outer hair cells. This labeling of type II SGN afferent fiber declined after P8. By P14, all synaptic terminal immunolabeling in the organ of Corti was absent, and SGN cell body labeling was minimal. In adult cochlear tissue, P2X3R immunolabeling was not detected. Noise exposure did not induce P2X3R expression in the adult cochlea. These data indicate that ATP-gated ion channels incorporating P2X3R subunit expression are specifically targeted to the afferent terminals just prior to the onset of hearing, and likely contribute to the neurotrophic signaling which establishes functional auditory neurotransmission.

Animals↗

PubMed1987

Morphology of labeled afferent fibers in the guinea pig cochlea.

Cochlear afferent and efferent fibers in the guinea pig were labeled by focal extracellular injections of horseradish peroxidase into the spiral ganglion of the basal turn. The morphology and pattern of termination of these fibers were studied by light microscopy. Fibers labeled by injections into the peripheral side of the ganglion could be grouped on the basis of their courses and terminations in the cochlea into two classes of afferent fibers, two classes of efferent (olivocochlear) fibers, and other presumably autonomic fibers. This paper describes the characteristics of labeled afferent fibers and their parent ganglion cells. Peripheral afferent fibers were grouped into two major classes: thick (mean diameter 1.7 micron) radial fibers projecting in a primarily radial fashion from the spiral ganglion and terminating on single inner hair cells and thin (mean diameter 0.5 micron) outer spiral fibers that spiral basalward in the organ of Corti to terminate on outer hair cells, usually in one row. For outer spiral fibers, the number of outer hair cells contacted and the length of the terminal region depend on the row of outer hair cells contacted, with third-row fibers forming, on the average, the most extensive region of termination. Within the spiral ganglion, two types of ganglion cells could be distinguished: type-I ganglion cells of large size (mean soma area = 216 microns 2) with a ratio of central process diameter to peripheral process diameter greater than one and type-II ganglion cells of smaller size (mean soma area = 100 microns 2) and a central to peripheral process ratio near one. In three cochleae in which injections were made central to the ganglion, 11 type-I ganglion cells have been traced to radial fibers contacting inner hair cells and eight type-II ganglion cells have been traced to outer spiral fibers contacting outer hair cells. Thus the afferent innervation of the guinea pig cochlea is similar to the pattern described in other mammals, in which there is separate innervation of the inner and outer hair cells by the two types of ganglion cells. The central axons of both types of ganglion cells were traced individually through serial sections of a block of tissue containing the cochlea, the auditory nerve, and the cochlear nucleus. They followed similar courses in the auditory nerve, and the axons followed into the cochlear nucleus bifurcated in similar regions of the interstitial portion.(ABSTRACT TRUNCATED AT 400 WORDS)

Afferent Pathways↗

PubMed1994

Astrocyte proliferation in the chick auditory brainstem following cochlea removal.

Astrocytes in the central nervous system (CNS) respond to injury and disease by proliferating and extending processes. The intermediate filament protein of astrocytes, glial fibrillary acidic protein (GFAP) also increases in astrocytes. These cells are called "reactive astrocytes" and are thought to play a role in CNS repair. We have previously demonstrated rapid increases (< 6 hours) in GFAP-immunoreactive and silver-impregnated glial processes in the chick cochlear nucleus, nucleus magnocellularis (NM), following cochlea removal or activity blockade of the eighth nerve. It was not known whether these changes were the result of glial proliferation, glial hypertrophy, or both. The present study examined the time course of astrocyte proliferation in NM following cochlea removal. Postnatal chicks received unilateral cochlea removal and survived for 6, 12, 18, 24, 36, 48, and 72 hours. Bromodeoxyuridine was used to label proliferating cells. The volume and number of labeled cells in NM was calculated for both the experimental and control sides of the brains for experimental animals was well as for unoperated control animals. A subset of astrocytes continuously divide in the normal posthatch chick brainstem. The percentage of labeled nuclei increases within NM 36 hours following cochlea removal and is robust by 48 hours. This increase is due to astrocyte proliferation within, rather than migration to, NM. These results indicate that rapid increases in GFAP following reduced activity are independent of cell proliferation. The time course of astrocyte proliferation suggests that cellular degeneration within the nucleus may play a role in upregulating astrocyte proliferation.

Animals↗

PubMed1995

Afferent influences on brainstem auditory nuclei of the chicken: regulation of transcriptional activity following cochlea removal.

Neuronal survival in the cochlear nucleus of young animals is regulated by afferent activity. Removal or blockade of nerve VIII input results in the death of 20-40% of neurons in the cochlear nucleus, nucleus magnocellularis (NM), of the 10-14 days posthatch chick. Neuronal death in NM is preceded by complete failure of protein synthesis and degradation of ribosomes. In addition, there is a biphasic change in the immunoreactivity of ribosomes for a monoclonal antiribosomal RNA antibody, Y10B. Initially, the entire population of afferent-deprived NM neurons loses Y10B immunoreactivity, but, after 6 or 12 hours of afferent deprivation, lack of Y10B immunoreactivity specifically marks dying NM neurons. Whether RNA synthesis is also altered in afferent-deprived NM neurons has not previously been studied. To determine whether RNA synthesis in NM neurons is regulated by loss of afferent activity, we injected chicks with 3H-uridine following unilateral cochlea removal and measured the incorporation of RNA precursor with tissue autoradiography. As early as 1 hour after cochlea removal, there was a significant decrease in 3H-uridine incorporation by afferent-deprived NM neurons. After longer periods of afferent deprivation (6 or 12 hours), the majority of dying NM neurons (marked by loss of Y10B immunoreactivity) fail to incorporate RNA precursor. Six or 12 hours following cochlea removal, the subpopulation of surviving NM neurons incorporates 3H-uridine at increased levels over those observed 1 or 3 hours after cochlea removal. These findings suggest that nuclear function is regulated by afferent synaptic activity and that failure of RNA synthesis occurs early in the cell death process.

Afferent Pathways↗

PubMed1988

Cochlea in old world mice and rats (Muridae).

Morphometric analysis of the cochlea was performed in wild and laboratory murids: Mus musculus, Apodemus sylvaticus, Rattus rattus, R. norvegicus, NMRI mouse, and Wistar rat. Results are based on light microscopic examination of surface specimens and serial sections and on three-dimensional computer reconstruction. The cochleae have 1.75-2.2 coils. The length of the basilar membrane varies from 6.0 to 12.1 mm. Mean density of outer hair cells ranges between 363 and 411, inner hair cells 98 and 121, neurons 1,230 and 1,760 per 1 mm. Following parameters change from base to apex: basilar membrane width 66.0 (+/- 8.2) to 175.0 (+/- 24.7) microns, basilar membrane thickness 17.0 (+/- 2.6) to 1.9 (+/- 0.1) microns, width of triad of outer hair cells 13.2 (+/- 0.7) to 28.8 (+/- 4.4) microns. The given numbers are mean "murid" values (with respective standard deviations). Maximum of dimensions of scalae is located at 10-15%, that of density of outer hair cells at 65%, density of inner hair cells at 2.8 mm, maximum of innervation density at 40-60% from the base. The following parameters are correlated with pinna size: length and maximum width of basilar membrane, dimensions of scalae, total number of receptors, and probably resolution capabilities. The following parameters are correlated with body size: maximum width of triad of outer hair cells, density and total number of neurons, ratio of neurons to receptors, apicobasal difference in basilar membrane stiffness and width of triad of outer hair cells; inversely proportional is receptor density and ratio of outer to inner hair cells and probably low-frequency cut-off. Thickness, and minimum width of basilar membrane and triad of outer hair cells and probably high-frequency cutoff are species-specific and independent of pinna or body size. The parameters mentioned indicate that the examined murids are acoustically unspecialized mammals and their cochleae approximate the generalized plan for a mammalian cochlea. Differences between domesticated and wild murids are stated.

Animals↗

PubMed1998

Endolymphatic hydrops reduces retrograde labeling of trigeminal innervation to the cochlea.

This paper reports that endolymphatic hydrops causes a significant reduction of retrogradely labeled cell bodies of the ipsilateral trigeminal ganglion following application of horseradish peroxidase in the cochlea. We previously showed that the trigeminal ganglion is a source of primary sensory innervation to the cochlear blood vessels. The innervation of the cochlea from the trigeminal ganglion may provide the basis of an alternative mechanism for Ménière's syndrome (imbalance, hearing loss, tinnitus, and a sensation of fullness in the ear) for which a central neural basis has been speculated. Innervation patterns of sensory nerves from the trigeminal ganglion to the cochlear blood vessels were studied using retrograde transport of wheat germ agglutinin conjugated to horseradish peroxidase (WGA-HRP). Healthy and hydropic guinea pigs were unilaterally implanted with an osmotic pump and a cannula for cochlear delivery of 2% WGA-HRP or vehicle alone. In other guinea pigs the cochlea was pretreated with 100 micromol capsaicin before administering 2% WGA-HRP. Histological sections of the ipsi- and contralateral trigeminal ganglia were obtained 48 h after WGA-HRP infusion. In the hydropic guinea pig, the number of labeled nerve cell bodies observed in the anteriomedial portion of the trigeminal ganglion at the origin of the ophthalmic nerve was reduced by 70% relative to normal animals. Capsaicin pretreatment nearly eliminated the labeled sensory fibers as expected. These data indicate that the trigeminal innervation to the cochlea could be involved in inner ear homeostatic disturbances, including the hydrops that is symptomatic of Ménière's disease.

Animals↗

PubMed1990

Laser light transmission and laser Doppler blood flow measurements on the human, rat and guinea pig cochlea.

In order to test the applicability of laser-Doppler flowmetry in monitoring cochlear blood flow clinically, the thickness and the helium-neon laser light transmission of specimens of human, rat and guinea pig promontory bone and human skin were determined. Furthermore, comparative laser-Doppler measurements were taken from the promontory in patients, rats and guinea pigs. Due to the different thicknesses of the promontory bone in different species, the light transmission was found to be considerably higher for the animal cochlea (rat, 15%; guinea pig, 6.6%) than the human cochlea (1.7%). However, a clearly higher laser-Doppler signal was recorded from both the human and the rat cochleas as compared with the guinea pig. The relative laser light attenuation by the human skin specimens corresponded to that of the human promontory bone. The findings are discussed with regard to the suitability of the laser-Doppler method for blood flow measurements in the human cochlea.

Aged↗

PubMed1987

Brainstem organization of efferent projections to the guinea pig cochlea studied using the fluorescent tracers fast blue and diamidino yellow.

Intracochlear injection of the fluorescent retrograde neuronal tracers fast blue and diamidino yellow was used to investigate the distribution within the brainstem of neurones projecting to the cochlea in the guinea pig. The overall pattern of distribution of cells within the brainstem auditory nuclei was the same for both tracers and was also in broad agreement with recent studies in this species using horseradish peroxidase as the neuronal tracer. However, the total number of neurones found (mean of 1234 projecting to each cochlea) was significantly greater than that reported using horseradish peroxidase, largely as a result of more small labelled neurones being detected within the lateral superior olivary nucleus ipsilateral to the injected cochlea. The yield of labelled cells was greatest in animals in which care was taken to perfuse the whole length of the cochlear epithelium. After bilateral injections of both tracers, double-labelled cells were found in small numbers within all the large neurone medial system nuclei and the ventral nucleus of the lateral lemniscus. It was concluded that between 1-5% of the medial system neurones project to both cochleas.

Amidines↗

PubMed1994

Antidiuretic hormone restores the endolymphatic longitudinal K+ gradient in the Brattleboro rat cochlea.

In the cochlea, endolymph is hyperosmotic to plasma and perilymph. To test the hypothesis that antidiuretic hormone is involved in the modulation of endolymph secretion, the electrochemical composition of cochlear fluids, endolymph and perilymph, was studied in three groups of anaesthetized rats: control Long Evans rats, homozygous Brattleboro rats that are genetically deprived of antidiuretic hormone, and Brattleboro rats that were treated with antidiuretic hormone (dDAVP, 0.5 microgram/100 g body weight/24 h during 8 days). Endolymph was sampled from the scala media at each turn of the cochlea and perilymph from the scala vestibuli. In Long Evans rats, the endocochlear potential, the endolymphatic K+ and Cl- concentrations decreased from base to apex of the cochlea as previously reported in guinea pigs and Sprague Dawley rats. In Brattleboro rats, the endocochlear potential and the Cl- concentration gradients were still present, whereas the K+ concentration gradient were still present, whereas the K+ concentration gradient was absent. This K+ gradient was restored by the administration of dDAVP, which increased the K+ concentration at the base of the cochlea. This work indicates that the K+ secretion in endolymph, and thus the osmolality, may be locally modulated by the antidiuretic hormone, probably via V2 receptors.

Animals↗

PubMed1980

Unusual blood vessels in the cochlea of the squirrel monkey.

Unusual blood vessels in the cochlea of the squirrel monkey are reported. The blood vessels in Reissner's membrane and basilar membrane are occasionally found in the course of the experimental middle ear study. The blood vessel of Reissner's membrane derives from a radiating arteriole in the upper spiral ligament and takes a straight course down to the inner surface of the spiral limbus, running on the scala vestibuli side of Reissner's membrane, and finally joins the venous vessel of the spiral limbus. Two blood vessels are noted in the basilar membrane of basal turn, running fairly parallel to each other from the tympanic lip to the basilar crest of the spiral ligament in the same cochlea. It is confirmed that these unusual blood vessels derive from the radiating arterioles in the osseous spiral lamina and connect with the venules of the basilar crest in the spiral ligament. Although unusual blood vessels in both Reissner's membrane and basilar membrane are thought to be vestigial structures, they contain a remarkable amount of alkaline phosphatase in their walls. This suggests that these unusual vessels have actually functioned until their death. Multiple occurrence of unusual blood vessels in both Reissner's membrane and basilar membrane of the cochlea of various animals, including man, has not yet been reported in the literature as far as we have been able to ascertain. Incomplete devascularization in the developing process of the cochlea may possibly be the cause of such unusual conditions after birth.

Animals↗

PubMed2004

The expression of VIP and SP in the cochlea of spontaneously hypertensive rats and its implication.

To investigate the expression of vasoactive intestinal peptide (VIP) and substance P (SP) in the cochlea of spontaneously hypertensive rat (SHR), and to assess the function of VIP and SP in the cochlea following the damage of hypertension, hearing thresholds of ABR were observed and the fixative (4% paraformaldehyde) was pumped through the circulatory system. Adult Wistar rats (3 months, n=20) served as the control group and SHRs (3 months, n=20) as the hypertension group. Bullas were taken out and cochleas were irrigated in vitro with the same fixative. The number of base turn's spiral ganglions in the sections was counted. The expression of VIP and SP were detected by SABC method and the images of the sections were analyzed. The number of base turn's spiral ganglsons in the hypertension group was significantly less than in the normal group (P<0.01). VIP and SP were expressed in the spiral ganglion cytoplasma and stria vascularis of the two groups. There were no significant difference in the expression of VIP and SP in spiral ganglion cytoplasma (P>0.05) between the two groups. However, in stria vascularis the expression of VIP in the hypertension group was higher than in the normal group (P<0.05), and no significant difference in SP was found between the two groups. It was suggested that VIP not only contributed to the regulation of the cochlea microcirculation, but also made the neurotransmitter in the pathway of the auditory system. However, SP made only the neurotransmitter in the pathway of the auditory system.

Animals↗

PubMed2000

Potassium ion recycling pathway via gap junction systems in the mammalian cochlea and its interruption in hereditary nonsyndromic deafness.

In the mammalian cochlea, there are two independent gap junction systems, the epithelial cell gap junction system and the connective tissue cell gap junction system. Thus far, four different connexin molecules, including connexin 26, 30, 31, and 43, have been reported in the cochlea. The two networks of gap junctions form the route by which K+ ions that pass through the sensory cells during mechanosensory transduction can be recycled back to the endolymphatic space, from which they reenter the sensory cells. Activation of hair cells by acoustic stimuli induces influx of K+ ions from the endolymph to sensory hair cells. These K+ ions are released basolaterally to the extracellular space of the organ of Corti, from which they enter the cochlear supporting cells. Once inside the supporting cells they move via the epithelial cell gap junction system laterally to the lower part of the spiral ligament. The K+ ions are released into the extracellular space of the spiral ligament by root cells and taken up by type II fibrocytes. This uptake incorporates K+ into the connective tissue gap junction system. Within this system, the K+ ions pass through the tight junctional barrier of the stria vascularis and are released within the intrastrial extracellular space. The marginal cells of the stria vascularis then take up K+ and return it to the endolymphatic space, where it can be used again in sensory transduction. It is highly probable that mutations of connexin genes that result in human nonsyndromic deafness cause dysfunction of cochlear gap junctions and thereby interrupt K+ ion recirculation pathways. In addition to connexin mutations, other conditions may disrupt gap junctions within the ear. For example, mice with a functionally significant mutation of Brain-4, which is expressed in the connective tissue cells within the cochlea, show marked depression of the endolymphatic potential and profound sensorineural hearing loss. It seems likely that disruption of connective tissue cells by this mutation disrupts K+ ion entry into the stria vascularis and thereby results in loss of endolymphatic potential. The association of sensorineural hearing loss with these genetic disorders provides strong evidence for the necessity of gap junction systems for the normal functioning of the cochlea.

Animals↗