Behavioral auditory thresholds and loss of ribbon synapses at inner hair cells in aged gerbils
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
openaire +2 more sources
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]
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
openaire +2 more sources
Release and elementary mechanisms of nitric oxide in hair cells
The enzyme nitric oxide (NO) synthase, that produces the signaling molecule NO, has been identified in several cell types in the inner ear. However, it is unclear whether a measurable quantity of NO is released in the inner ear to confer specific ...
Song, Haitao +12 more
core +1 more source
RAF kinase activity regulates neuroepithelial cell proliferation and neuronal progenitor cell differentiation during early inner ear development [PDF]
Background: Early inner ear development requires the strict regulation of cell proliferation, survival, migration and differentiation, coordinated by the concerted action of extrinsic and intrinsic factors.
Rapp Ulf R. +21 more
core +2 more sources
TECTB Variants Reveal Tectorial Membrane Vulnerability in Dominant Non‐Syndromic Hearing Loss
TECTB is a non‐collagenous protein of the tectorial membrane – an extracellular matrix of the cochlea. This study identifies dominant missense variants in TECTB linked to human hereditary deafness in two unrelated families. Genetically engineered mice homozygous for one of the variants are profoundly deaf, whereas heterozygous mice have normal hearing ...
Evan B. Hale +23 more
wiley +1 more source
PTEN inhibitor bisperoxovanadium protects against noise-induced hearing loss
Studies have shown that phosphatase and tensin homolog deleted on chromosome ten (PTEN) participates in the regulation of cochlear hair cell survival. Bisperoxovanadium protects against neurodegeneration by inhibiting PTEN expression.
Bei Fan +9 more
doaj +1 more source
Sleep disturbance severity closely tracks hearing loss in a clinical cohort, yet the mechanistic link remains unclear. Acute sleep deprivation is shown to trigger transient cochlear oxidative stress that switches into a self‐sustaining neuroinflammatory state, suppressing BK channels and causing irreversible synaptopathy.
Dan Chen +11 more
wiley +1 more source
Identification and characterisation of gene regulation by the POU4F3 (BRN-3C) transcription factor in the inner ear [PDF]
Hair cells are the mechanosensory cells of the inner ear whose loss causes irreversible hearing loss in mammals. POU4F3, a POU-domain transcription factor, is only expressed in hair cells in the inner ear and is essential for hair cell terminal ...
Tornari, C.
core
Clade‐wide morphological and functional variation of the sauropsid columella
Abstract The columella (=stapes) is the middle ear bone of reptiles that transmits vibrations from the environment to the inner ear. It has been shown to exhibit extensive interspecific morphological disparity in several clades; however, its morphological variation and associated functional consequences remain poorly described.
John Peacock +4 more
wiley +1 more source
Clinical features of the fragile X syndrome (FXS) phenotype include intellectual disability, repetitive behaviors, social communication deficits, and, commonly, auditory hypersensitivity to acoustic stimuli.
M. Chojnacka +2 more
doaj +1 more source

