Hearing, Cochlea, Tinnitus, Genetics

The paper shows that purinergic signaling via ATP release from inner supporting cells drives spontaneous activity in the auditory system from birth to hearing onset, promoting coordinated neural firing in frequency-specific circuits.

This study shows that MOC efferent nerves also innervate cochlear supporting cells, modulating gap junctions and affecting OHC electromotility, which impacts hearing sensitivity and noise protection.

This study develops an optimized stimulus envelope to enhance the sensitivity of envelope-following responses (EFRs) for detecting cochlear synaptopathy in humans through computational modeling and experimental validation.

The paper shows that tip links in hair bundles can recover rapidly after disruption by restoring calcium ions and re-establishing mechanical contact.

This study compares optogenetic and acoustic stimulation in guinea pigs, finding similar auditory responses to light and sound, with increased amplitude at higher light intensity.

The paper identifies a new inhibitory neuron class, L-Stellate cells, in the ventral cochlear nucleus using transgenic mice and imaging techniques, showing their distinct morphology and functional role in auditory processing.

This study shows that Jagged1 is essential for forming and maintaining Hensen cells in the mouse cochlea, with its loss leading to cell death or conversion into Claudius cells.

This study investigates neural mechanisms of misophonia using fMRI, finding increased orbitofrontal connectivity and auditory-limbic pathway interactions in response to trigger sounds.

The paper identifies NCOA3 as a candidate gene for autosomal dominant progressive hearing loss through linkage analysis and functional studies in zebrafish.

This study shows ISRIB protects against noise-induced cochlear synaptopathy in male mice but not females, highlighting sex-dependent effects of UPR inhibition.

The paper investigates the role of tectorial membrane mechanics in the cochlear amplifier's phase shift and power generation, showing that radial impedance minima align with experimental data.

This study shows that shear forces and local repulsion drive the formation of the checkerboard pattern of hair cells in the mammalian inner ear.

The paper investigates how cochlear place coding mediates frequency modulation perception across different hearing conditions and finds that FM detection thresholds correlate with place-coding fidelity regardless of modulation rate.

This paper develops an automated 3D inner ear modeling method to assess morphological similarities across species, finding mini pigs closely resemble humans in key structures.

This study demonstrates that antisense oligonucleotides targeting the c.151C>T mutation in COCH can reduce mutant transcript levels in transgenic cells, suggesting potential as a therapy for DFNA9.

The paper identifies 31 new genomic risk loci for hearing difficulty using GWAS and multi-omic analysis, highlighting gene regulation in cochlear cells.

This study uses a novel stimulus and EEG to assess neural function at multiple stages of the auditory pathway simultaneously in normal-hearing adults, revealing distinct subcortical and cortical responses to frequency modulation.

This study investigates a homozygous frameshift variant in the COCH gene, showing it causes loss-of-function through reduced cochlin translation, impacting genetic counseling for carriers.

This study investigates how contextual information affects aversive responses to sounds in individuals with misophonia, finding that the same sound can be perceived as more or less aversive based on context.

The paper models LSO neurons to replicate human sensitivity to envelope interaural time difference (ITDENV) across varying frequencies and modulation rates.

This study shows that loud noise exposure differentially affects firing properties of type A and B pyramidal cells in the auditory cortex of mice one week post-exposure.

This study shows that LIN28B maintains supporting cell plasticity in the cochlea by activating mTOR signaling, enabling hair cell regeneration in immature mice.

The paper identifies ETV4 and ETV5 as downstream regulators of mesenchymal FGF signaling that promote cochlear lengthening during development.

This study shows that the MOC efferent system couples bilateral spontaneous activity in the developing auditory system, and its absence reduces auditory sensitivity at hearing onset.

This study identifies a THOC1 variant causing late-onset nonsyndromic hearing loss via p53-mediated hair cell apoptosis in zebrafish.

The paper identifies pathogenic mutations in GJB2, GJB3, and GJB6 genes in an Indian cohort with non-syndromic sensorineural hearing loss and finds lower mutation rates compared to other populations.

This study shows that pterostilbene reduces cochlear damage in diabetic rats by decreasing apoptosis and improving metabolic parameters.

The paper investigates the mechanisms of cochlear duct morphogenesis by analyzing cellular dynamics and signaling patterns during development.

The paper identifies genetic causes of inherited hearing loss in the Israeli Jewish population, finding a novel deafness gene ATOH1 and demonstrating the effectiveness of targeted genomic sequencing.