Remodeling of the Inner Hair Cell Microtubule Meshwork in a Mouse Model of Auditory Neuropathy AUNA1 [PDF]
eneuro, 2016 AbstractAuditory neuropathy 1 (AUNA1) is a form of human deafness resulting from a point mutation in the 5′ untranslated region of theDiaphanous homolog 3(DIAPH3) gene. Notably, theDIAPH3mutation leads to the overexpression of the DIAPH3 protein, a formin family member involved in cytoskeleton dynamics. Through study of diap3-overexpressing transgenic (
Surel, Clément +9 more
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C1ql1 is expressed in adult outer hair cells of the cochlea in a tonotopic gradient.
PLoS ONE, 2021 Hearing depends on the transduction of sounds into neural signals by the inner hair cells of the cochlea. Cochleae also have outer hair cells with unique electromotile properties that increase auditory sensitivity, but they are particularly susceptible ...
Joyshree Biswas +8 more
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Mechanisms of sensorineural cell damage, death and survival in the cochlea. [PDF]
, 2015 The majority of acquired hearing loss, including presbycusis, is caused by irreversible damage to the sensorineural tissues of the cochlea. This article reviews the intracellular mechanisms that contribute to sensorineural damage in the cochlea, as well ...
Ryan, Allen F, Wong, Ann CY
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Artificial induction of Sox21 regulates sensory cell formation in the embryonic chicken inner ear.
PLoS ONE, 2012 During embryonic development, hair cells and support cells in the sensory epithelia of the inner ear derive from progenitors that express Sox2, a member of the SoxB1 family of transcription factors.
Stephen D Freeman, Nicolas Daudet
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Rat Auditory Inner Hair Cell Mechanotransduction and Stereociliary Membrane Diffusivity Are Similarly Modulated by Calcium [PDF]
iScience, 2020 The lipid bilayer plays a pivotal role in force transmission to many mechanically-gated channels. We developed the technology to monitor membrane diffusivity in order to test the hypothesis positing that Ca2+ regulates open probability (P o) of cochlear hair cell mechanotransduction (MET) channels via the plasma membrane. The stereociliary membrane was
Shefin S. George +2 more
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eLife, 2021 Acoustic overexposure and aging can damage auditory synapses in the inner ear by a process known as synaptopathy. These insults may also damage hair bundles and the sensory transduction apparatus in auditory hair cells.
John Lee +3 more
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Mammalian cochlea as a physics guided evolution-optimized hearing sensor [PDF]
, 2014 Nonlinear physics plays an essential role in hearing, from sound signal generation to sound sensing to the processing of complex sound environments. We demonstrate that the evolution of the biological hearing sensors demonstrates a dramatic reduction in ...
Gomez, Florian +2 more
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Dexamethasone uptake in the murine organ of Corti with transtympanic versus systemic administration
Journal of Otolaryngology - Head and Neck Surgery, 2013 Objective To investigate glucocorticoid uptake in auditory hair cells following transtympanic versus systemic administration of dexamethasone. Study design Controlled experimental study. Setting Translational science experimental laboratory.
Amandeep S Grewal +3 more
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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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Molecular and functional characterization of gap junctions in the avian inner ear. [PDF]
, 2006 To analyze the fundamental role of gap junctions in the vertebrate inner ear, we examined molecular and functional characteristics of gap junctional communication (GJC) in the auditory and vestibular system of the chicken.
Becker, D, Forge, A, Nickel, R
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