Perspectives on Auditory Neuropathy: Disorders of Inner Hair Cell, Auditory Nerve, and Their Synapse [PDF]
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 +5 more
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Mutations in ap1b1 Cause Mistargeting of the Na(+)/K(+)-ATPase Pump in Sensory Hair Cells. [PDF]
The hair cells of the inner ear are polarized epithelial cells with a specialized structure at the apical surface, the mechanosensitive hair bundle.
Kindt, Katie +14 more
core +2 more sources
Remodeling of the Inner Hair Cell Microtubule Meshwork in a Mouse Model of Auditory Neuropathy AUNA1 [PDF]
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
openaire +3 more sources
Numb is not a critical regulator of Notch-mediated cell fate decisions in the developing chick inner ear. [PDF]
The Notch signaling pathway controls differentiation of hair cells and supporting cells in the vertebrate inner ear. Here, we have investigated whether Numb, a known regulator of Notch activity in Drosophila, is involved in this process in the embryonic ...
Mark eEddison +9 more
core +1 more source
Genomic Analysis of the Function of the Transcription Factor gata3 during Development of the Mammalian Inner Ear [PDF]
We have studied the function of the zinc finger transcription factor gata3 in auditory system development by analysing temporal profiles of gene expression during differentiation of conditionally immortal cell lines derived to model specific auditory ...
Lawoko-Kerali, Grace +8 more
core +3 more sources
Artificial induction of Sox21 regulates sensory cell formation in the embryonic chicken inner ear.
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
C1ql1 is expressed in adult outer hair cells of the cochlea in a tonotopic gradient.
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
doaj +1 more source
Rat Auditory Inner Hair Cell Mechanotransduction and Stereociliary Membrane Diffusivity Are Similarly Modulated by Calcium [PDF]
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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Inner ear morphology in the Atlantic molly Poecilia mexicana - first detailed microanatomical study of the inner ear of a cyprinodontiform species [PDF]
Background: Fishes show an amazing diversity in hearing abilities, inner ear structures, and otolith morphology. Inner ear morphology, however, has not yet been investigated in detail in any member of the diverse order Cyprinodontiformes.
Heß, Martin +8 more
core +2 more sources
Inner ear morphology in the Atlantic molly Poecilia mexicana [PDF]
Fishes show an amazing diversity in hearing abilities, inner ear structures, and otolith morphology. Inner ear morphology, however, has not yet been investigated in detail in any member of the diverse order Cyprinodontiformes.
Plath, Martin +2 more
core +1 more source

