Home About us Contact
|
Home About us Contact | ||
|
|
||
Inner Hair Cells (inner + hair_cell)
Selected AbstractsThe deaf mouse mutant whirler suggests a role for whirlin in actin filament dynamics and stereocilia developmentCYTOSKELETON, Issue 7 2007Mette M. Mogensen Abstract Stereocilia, finger-like projections forming the hair bundle on the apical surface of sensory hair cells in the cochlea, are responsible for mechanosensation and ultimately the perception of sound. The actin cytoskeleton of the stereocilia contains hundreds of tightly cross-linked parallel actin filaments in a paracrystalline array and it is vital for their function. Although several genes have been identified and associated with stereocilia development, the molecular mechanisms responsible for stereocilia growth, maintenance and organisation of the hair bundle have not been fully resolved. Here we provide further characterisation of the stereocilia of the whirler mouse mutant. We found that a lack of whirlin protein in whirler mutants results in short stereocilia with larger diameters without a corresponding increase in the number of actin filaments in inner hair cells. However, a decrease in the actin filament packing density was evident in the whirler mutant. The electron-density at the tip of each stereocilium was markedly patchy and irregular in the whirler mutants compared with a uniform band in controls. The outer hair cell stereocilia of the whirler homozygote also showed an increase in diameter and variable heights within bundles. The number of outer hair cell stereocilia was significantly reduced and the centre-to-centre spacing between the stereocilia was greater than in the wildtype. Our findings suggest that whirlin plays an important role in actin filament packing and dynamics during postnatal stereocilium elongation. Cell Motil. Cytoskeleton 2007. © 2007 Wiley-Liss, Inc. [source] Cysteine-string protein in inner hair cells of the organ of Corti: synaptic expression and upregulation at the onset of hearingEUROPEAN JOURNAL OF NEUROSCIENCE, Issue 9 2002Michel Eybalin Abstract Cysteine-string protein is a vesicle-associated protein that plays a vital function in neurotransmitter release. We have studied its expression and regulation during cochlear maturation. Both the mRNA and the protein were found in primary auditory neurons and the sensory inner hair cells. More importantly, cysteine-string protein was localized on synaptic vesicles associated with the synaptic ribbon in inner hair cells and with presynaptic differentiations in lateral and medial olivocochlear terminals , the cell bodies of which lie in the auditory brainstem. No cysteine-string protein was expressed by the sensory outer hair cells suggesting that the distinct functions of the two cochlear hair cell types imply different mechanisms of neurotransmitter release. In developmental studies in the rat, we observed that cysteine-string protein was present beneath the inner hair cells at birth and beneath outer hair cells by postnatal day 2 only. We found no expression in the inner hair cells before about postnatal day 12, which corresponds to the period during which the first cochlear action potentials could be recorded. In conclusion, the close association of cysteine-string protein with synaptic vesicles tethered to synaptic ribbons in inner hair cells and its synchronized expression with the appearance and maturation of the cochlear potentials strongly suggest that this protein plays a fundamental role in sound-evoked glutamate release by inner hair cells. This also suggests that this role may be common to ribbon synapses and conventional central nervous system synapses. [source] Developmental regulation of neuron-specific P2X3 receptor expression in the rat cochleaTHE JOURNAL OF COMPARATIVE NEUROLOGY, Issue 2 2005Lin-Chien Huang Abstract 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. J. Comp. Neurol. 484:133,143, 2005. © 2005 Wiley-Liss, Inc. [source] Presbycusis: A Human Temporal Bone Study of Individuals With Downward Sloping Audiometric Patterns of Hearing Loss and Review of the LiteratureTHE LARYNGOSCOPE, Issue S112 2006Erik G. Nelson MD Abstract Objective: The purpose of this retrospective case review was to identify patterns of cochlear element degeneration in individuals with presbycusis exhibiting downward sloping audiometric patterns of hearing loss and to correlate these findings with those reported in the literature to clarify conflicting concepts regarding the association between hearing loss and morphologic abnormalities. Methods: Archival human temporal bones from individuals with presbycusis were selected on the basis of strict audiometric criteria for downward-sloping audiometric thresholds. Twenty-one temporal bones that met these criteria were identified and compared with 10 temporal bones from individuals with normal hearing. The stria vascularis volumes, spiral ganglion cell populations, inner hair cells, and outer hair cells were quantitatively evaluated. The relationship between the severity of hearing loss and the degeneration of cochlear elements was analyzed using univariate linear regression models. Results: Outer hair cell loss and ganglion cell loss was observed in all individuals with presbycusis. Inner hair cell loss was observed in 18 of the 21 individuals with presbycusis and stria vascularis loss was observed in 10 of the 21 individuals with presbycusis. The extent of degeneration of all four of the cochlear elements evaluated was highly associated with the severity of hearing loss based on audiometric thresholds at 8,000 Hz and the pure-tone average at 500, 1,000, and 2,000 Hz. The extent of ganglion cell degeneration was associated with the slope of the audiogram. Conclusions: Individuals with downward-sloping audiometric patterns of presbycusis exhibit degeneration of the stria vascularis, spiral ganglion cells, inner hair cells, and outer hair cells that is associated with the severity of hearing loss. This association has not been previously reported in studies that did not use quantitative methodologies for evaluating the cochlear elements and strict audiometric criteria for selecting cases. [source] Evidence for surviving outer hair cell function in congenitally deaf ears,THE LARYNGOSCOPE, Issue 11 2003FRCS (London), FRCS (ORL-HNS), Peter A. Rea MA Abstract Objective/Hypothesis: The hypotheses of the study were that congenital hearing impairment in infants can result from the isolated loss of inner hair cells of the cochlea and that this is shown by the presence of abnormal positive summating potentials on round window electrocochleography. The objectives were to establish the proportion of infants with hearing loss affected, the nature of the cochlear lesion, and its etiology. And to highlight the important implications for otoacoustic emissions testing and universal neonatal screening. Study Design: A prospectively conducted consecutive cohort study with supplemental review of notes was performed. Methods: Four hundred sixty-four children underwent round window electrocochleography and auditory brainstem response testing under general anesthesia to assess suspected hearing loss. The presence of abnormal positive potentials was recorded. Otoacoustic emissions data were collected separately and retrospectively. Results: Three hundred forty-two children had significant bilateral congenital hearing loss. All results were from hearing-impaired children. Abnormal positive potentials were recorded in 73 of 342 children (21%). Eighty-three percent of children with otoacoustic emissions also had abnormal positive potentials, but only 14% of children without otoacoustic emissions had abnormal positive potentials (P < .001). In the neonatal intensive care unit setting, 43% of infants were found to have abnormal positive potentials, whereas only 10% had abnormal positive potentials if not in the neonatal intensive care unit setting (P < .001). Abnormal positive potentials were present in 63% of infants born before 30 weeks gestation and in 14% of infants born at term (P < .001). Abnormal positive potentials were identified in 57% of infants with documented hypoxia and 11% of children with no episodes (P < .001). Otoacoustic emissions were present in 48% of infants from the neonatal intensive care unit, despite their hearing loss. Conclusion: Both otoacoustic emissions and abnormal positive potentials may originate from outer hair cell activity following inner hair cell loss. This may occur in more than 40% of hearing-impaired children in the neonatal intensive care unit setting. Chronic hypoxia is the most likely cause. Otoacoustic emissions testing may not be a suitable screening tool for such infants. [source] Selective Vestibular Ablation by KTP Laser in Endolymphatic Hydrops,,THE LARYNGOSCOPE, Issue 6 2001Melanie Adamczyk MD Abstract Objectives and Hypothesis Vertigo, the cause of disability in many patients with Ménière's disease, may be the result of the effects of endolymphatic hydrops on the semicircular canals. We hypothesize that intractable vertigo may be controlled by destruction of the semicircular canal neuroepithelium using visible light lasers without the need for extensive fenestration of the bony labyrinth. This study was designed to assess the cochlear effects of potassium titanyl phosphate (KTP) laser-assisted triple semicircular canal ablation (TSCA) in endolymphatic hydrops. Study Design Randomized, prospective, and controlled. Methods Forty-one adult guinea pigs underwent either a unilateral endolymphatic duct occlusion to induce hydrops or a sham procedure. Ten weeks after induction of the hydrops, a KTP laser-assisted TSCA or a sham surgery was performed. Results Electrocochleographic thresholds to clicks and tone-bursts (2,20 kHz) did not change significantly up to 4 weeks after TSCA in hydropic ears. Cross-sectional histology confirmed the presence of hydrops and the ablation of the semicircular canals. Cochlear whole-mounts for hair cell counts showed no significant loss of outer or inner hair cells in hydropic ears treated with TSCA. Conclusion KTP laser-assisted TSCA can be performed in the guinea pig model of endolymphatic hydrops without significant loss of hearing. Evaluation of this technique may be warranted in patients with intractable Ménière's disease. [source] | ||||||||||