Loss of Pex1 in Inner Ear Hair Cells Contributes to Cochlear Synaptopathy and Hearing Loss
Cells, 2022 Peroxisome Biogenesis Disorders (PBD) and Zellweger syndrome spectrum disorders (ZSD) are rare genetic multisystem disorders that include hearing impairment and are associated with defects in peroxisome assembly, function, or both.
Stephanie A. Mauriac +4 more
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Zinc-finger protein A20 protects hair cells from damage made by high-power microwave
Journal of Bio-X Research, 2019 . Inner ear hair cells are important for maintaining hearing. Irreversible damage to hair cells is an important cause of sensorineural deafness. Electromagnetic radiation, especially high-power microwave, is an important threat to human health in modern ...
Feng Tang +5 more
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Striatin Is Required for Hearing and Affects Inner Hair Cells and Ribbon Synapses
Frontiers in Cell and Developmental Biology, 2020 Striatin, a subunit of the serine/threonine phosphatase PP2A, is a core member of the conserved striatin-interacting phosphatase and kinase (STRIPAK) complexes. The protein is expressed in the cell junctions between epithelial cells, which play a role in
Prathamesh T. Nadar-Ponniah +14 more
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Age-related changes in auditory nerve–inner hair cell connections, hair cell numbers, auditory brain stem response and gap detection in UM-HET4 mice [PDF]
Neuroscience, 2015 This study compared the timing of appearance of three components of age-related hearing loss that determine the pattern and severity of presbycusis: the functional and structural pathologies of sensory cells and neurons and changes in gap detection (GD), the latter as an indicator of auditory temporal processing.
R A, Altschuler +9 more
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Frontiers in Molecular Neuroscience, 2020 Hair cells sense and transmit auditory, vestibular, and hydrodynamic information by converting mechanical stimuli into electrical signals. This process of mechano-electrical transduction (MET) requires a mechanically gated channel localized in the apical
Timothy Erickson +5 more
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A Reversal in Hair Cell Orientation Organizes Both the Auditory and Vestibular Organs
Frontiers in Neuroscience, 2021 Sensory hair cells detect mechanical stimuli with their hair bundle, an asymmetrical brush of actin-based membrane protrusions, or stereocilia. At the single cell level, stereocilia are organized in rows of graded heights that confer the hair bundle with
Basile Tarchini +2 more
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Differences in auditory nerve fiber responses in regard to inner and outer hair cells [PDF]
The Journal of the Acoustical Society of America, 1978 Single unit recordings were made from auditory nerve fibers in the monkey to pure tone stimuli. Extensive studies of frequency response areas suggested the existence of two types of auditory nerve fibers. One type showed a flat-bottomed response area and the other showed a conventional sharp one.
M. Nomoto, M. Maekawa
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Frontiers in Cellular Neuroscience, 2018 During development, the sensory cells of the cochlea, the inner hair cells (IHCs), fire spontaneous calcium action potentials. This activity at the pre-hearing stage allows the IHCs to autonomously excite the auditory nerve fibers and hence, represents ...
Anne-Gabrielle Harrus +5 more
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Behavioral auditory thresholds and loss of ribbon synapses at inner hair cells in aged gerbils
Experimental Gerontology, 2016 The potential contribution of auditory synaptopathy to age dependent hearing loss was studied in groups of young and old gerbils. The analysis of the number of inner hair cell ribbon synapses in aged gerbils (37.9±3.3months of age) revealed only a relatively small (11-17%) loss in the basal two thirds of the cochlea, while a more pronounced reduction ...
Otto, Gleich +2 more
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Dual-AAV mediated transfer of full-length otoferlin cDNA into auditory inner hair cells and the effects of different mutations in the OTOF gene on the protein levels and cellular distribution of otoferlin in auditory inner hair cells [PDF]
, 2022 Otoferlin is a large multi-C2 domain protein indispensable for hearing and synaptic transmission in auditory inner hair cells (IHCs). Mutations within the OTOF gene, coding for otoferlin, cause non-syndromic recessive hearing loss DFNB9. The severity of the hearing impairment can range from profound deafness to moderate hearing loss depending on the ...
Al-Moyed, Hanan +8 more
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