Results 31 to 40 of about 37,354 (236)

Perspectives on Auditory Neuropathy: Disorders of Inner Hair Cell, Auditory Nerve, and Their Synapse [PDF]

, 2008
The term auditory neuropathy (Starr, A. et al., 1996) was first used to describe a hearing disorder due to altered function of the auditory nerve in the presence of preserved functions of cochlear outer hair cells (OHCs; Starr, A. et al., 1991). The hearing loss has specific features reflecting impairment of auditory temporal processes that are ...
Starr, Arnold, Zeng, F. G., Michalewski, H .J., Moser, Tobias, Dallos, Peter, Oertel, Donata   +5 more
openaire   +4 more sources

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, Guillet, Marie, Lenoir, Marc, Bourien, Jérôme, Sendin, Gaston, Joly, Willy, Delprat, Benjamin, Lesperance, Marci, Puel, Jean-Luc, Nouvian, Regis   +9 more
openaire   +3 more sources

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, Charles R. Steele, Anthony J. Ricci   +2 more
openaire   +3 more sources

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
doaj   +1 more source

Sensory transduction is required for normal development and maturation of cochlear inner hair cell synapses

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, Kosuke Kawai, Jeffrey R Holt, Gwenaëlle SG Géléoc   +3 more
doaj   +1 more source

Enlargement of ribbons in zebrafish hair cells increases calcium currents, but disrupts afferent spontaneous activity and timing of stimulus onset. [PDF]

, 2017
In sensory hair cells of auditory and vestibular organs, the ribbon synapse is required for the precise encoding of a wide range of complex stimuli. Hair cells have a unique presynaptic structure-the synaptic ribbon-that organizes both synaptic vesicles ...
Beirl, A., He, X.J., Kindt, K.S., Marcotti, W., Nicolson, T., Olt, J., Petralia, R.S., Schreck, M., Sheets, L., Trapani, J.G., Wang, Y-X., Zhang, Q.   +11 more
core   +1 more source

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, Thibault Peineau, Aamir Zuberi, Cathleen Lutz, Gwénaëlle S. G. Géléoc   +4 more
doaj   +1 more source

A novel ENU-induced mutation in Myo6 causes vestibular dysfunction and deafness [PDF]

, 2016
Mouse N-ethyl-N-nitrosourea (ENU) mutagenesis has generated many useful animal models for human diseases. Here we describe the identification of a novel ENU-induced mouse mutant strain Turner (Tur) that displays circling and headtossing behavior and ...
Brahmachary, M, Wong, YME, Xu, CY, Xu, PX   +3 more
core   +1 more source

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, Xiaoling Chen, Lifeng Jia, Hai Li, Jingya Li, Wei Yuan   +5 more
doaj   +1 more source

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
core   +1 more source