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Deep Phenotyping of a Mouse Model for Hearing Instability Disorders.
Otol Neurotol
December 2024
Auditory Development and Restoration Program, Neurotology Branch, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Bethesda, Maryland.
Article Synopsis
- - The study examines hearing instability (HI) in Slc26a4-insufficient mice, suggesting that differences in ion homeostasis gene expression and activated macrophages may contribute to the disorder.
- - Researchers conducted auditory tests and compared the results from mice with HI to those without, finding significant changes in gene expression and macrophage activity in the inner ear support cells of the affected mice.
- - Findings indicate that these genetic and immune factors influence endolymphatic hydrops (EH) and endocochlear potential (EP), offering insights into HI's potential causes and its relevance to human health.
Two new mouse alleles of Ocm and Slc26a5.
Hear Res
October 2024
Wolfson Sensory, Pain and Regeneration Centre, King's College London, London SE1 1UL, UK. Electronic address:
Article Synopsis
- The study investigates the roles of two genes, Ocm and Slc26a5, in hearing loss in mice, focusing on their expression in outer hair cells and their potential interaction.
- Mutations in both genes led to the absence of their respective mRNA and resulted in unique patterns of hearing dysfunction, indicating they don't regulate each other's expression.
- Ocm mutants initially had normal hearing that deteriorated over time, while Slc26a5 mutants showed consistently elevated hearing thresholds, suggesting different mechanisms of auditory impairment related to outer hair cells.
A new mutation of Sgms1 causes gradual hearing loss associated with a reduced endocochlear potential.
Hear Res
September 2024
Wolfson Sensory, Pain and Regeneration Centre, King's College London, London SE1 1UL, United Kingdom. Electronic address:
Article Synopsis
- The Sgms1 gene encodes an enzyme crucial for the sphingosine-1-phosphate signaling pathway and was linked to hearing loss in mice; however, a new allele showed normal hearing initially despite mutations.
- This mutation resulted in a reduction of Sgms1 transcript levels to 20% of normal, which was still sufficient for normal hearing at first, but led to gradual hearing loss by six months.
- Research also indicated that abnormal structures in the inner ear and reduced electrical potential contributed to the hearing issues, and findings in a human cohort suggested that variants of the SGMS1 gene might influence hearing abilities in people.
LRRC8/VRAC volume-regulated anion channels are crucial for hearing.
J Biol Chem
July 2024
Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP), Berlin, Germany; NeuroCure Cluster of Excellence, Charité Universitätsmedizin Berlin, Berlin, Germany. Electronic address:
Hearing crucially depends on cochlear ion homeostasis as evident from deafness elicited by mutations in various genes encoding cation or anion channels and transporters. Ablation of ClC‑K/barttin chloride channels causes deafness by interfering with the positive electrical potential of the endolymph, but roles of other anion channels in the inner ear have not been studied. Here we report the intracochlear distribution of all five LRRC8 subunits of VRAC, a volume-regulated anion channel that transports chloride, metabolites, and drugs such as the ototoxic anti-cancer drug cisplatin, and explore its physiological role by ablating its subunits.
View Article and Find Full Text PDFThe Stria Vascularis: Renewed Attention on a Key Player in Age-Related Hearing Loss.
Int J Mol Sci
May 2024
Department of Otorhinolaryngology/Head and Neck Surgery, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands.
Article Synopsis
- Age-related hearing loss, or presbycusis, is a complex condition affecting many older adults and involving various mechanisms, particularly metabolic dysfunction in the stria vascularis essential for hearing.
- Research has shifted focus back to the stria vascularis, highlighting its role in age-related hearing loss and examining factors like ion transport changes, pigmentation shifts, and inflammation.
- This review emphasizes the need for a comprehensive understanding of metabolic and sensorineural mechanisms in age-related hearing loss to inform future research and potential treatment strategies.
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