Hearing, Cochlea, Tinnitus, Genetics

Auditory Cellular Cooperativity Probed Via Spontaneous Otoacoustic Emissions

This study examines fluctuations in spontaneous otoacoustic emissions (SOAE) across three vertebrates to investigate cellular cooperativity in the inner ear's active system.

The relationship between distortion product otoacoustic emissions and audiometric thresholds in the extended high-frequency range

This study examines the correlation between distortion product otoacoustic emissions and audiometric thresholds in extended high frequencies using calibrated measurements.

CIB2 function is distinct from Whirlin in the development of cochlear stereocilia staircase pattern

The paper investigates the distinct roles of CIB2 and whirlin in cochlear stereocilia development and shows that CIB2 has independent functions from whirlin in maintaining the staircase pattern.

Genome-wide association study meta-analysis brings monogenic hearing loss genes into the polygenic realm

This paper conducts a GWAS meta-analysis of sensorineural hearing loss, identifying 108 loci, including 54 novel genes, and shows substantial genetic overlap between cohorts despite differing phenotypes.

Inducible, virus-free direct lineage reprogramming enhances scalable generation of human inner ear hair cell-like cells

This study develops a virus-free, inducible system to generate human inner ear hair cell-like cells from iPS cells, achieving higher efficiency and faster conversion than viral methods.

A Myosin Nanomotor Essential for Stereocilia Maintenance Expands the Etiology of Hereditary Hearing Loss DFNB3

The paper identifies a new myosin isoform, MYO15A-3, essential for maintaining stereocilia structure in postnatal hair cells, contributing to DFNB3 hearing loss.

GABAergic synapses between auditory efferent neurons and type II spiral ganglion afferent neurons in the mouse cochlea

The paper demonstrates a GABAergic synapse between MOC efferent neurons and type II spiral ganglion afferent neurons in the mouse cochlea, mediated by GABAARs, bypassing outer hair cells.

Confirmatory insights into ELMOD3-associated autosomal dominant non-syndromic hearing loss

The paper identifies a novel ELMOD3 variant causing autosomal dominant hearing loss and shows its pathogenicity through structural and functional analyses.

TMEM145 is a key component in stereociliary link structures of outer hair cells

This study identifies TMEM145 as a critical transmembrane protein in OHC stereociliary structures, showing its necessity for structural integrity and auditory function.

The genomic landscape of syndromic and non-syndromic hearing loss within the 100,000 Genomes Project cohort

This study analyzes genetic causes of syndromic and non-syndromic hearing loss in 5,488 individuals from the 100,000 Genomes Project, identifying 273 genes and a 27.5% diagnostic yield.

Unique SLC26A4 Mutation Spectrum in Mongolian Patients with Enlarged Vestibular Aqueduct: A Whole-Exome Sequencing Study

This study identifies biallelic SLC26A4 pathogenic variants in all Mongolian EVA patients, revealing a unique mutation spectrum across East, North, and Southwest Asian populations.

Hair Bundle Degeneration is a Key Contributor to Age-Related Vestibular Dysfunction

The paper identifies age-related molecular and cellular changes in vestibular hair cells that contribute to vestibular dysfunction by impairing mechanotransduction and hair bundle integrity.

A modiolar-pillar gradient in auditory-nerve dendritic length: a novel post-synaptic contribution to dynamic range?

The paper identifies a modiolar-pillar gradient in auditory-nerve dendritic length in guinea pigs, suggesting it contributes to differences in threshold sensitivity among auditory-nerve fibers.

Limiting hearing loss in transgenic mouse models

This paper presents a method to reduce hearing loss in transgenic mice by crossing them with congenic B6.CAST-Cdh23Ahl+mice, improving auditory research accuracy.

Lateral olivocochlear neurons modulate cochlear responses to noise exposure

This study investigates how lateral olivocochlear neurons modulate cochlear responses to noise exposure and finds that their ablation leads to worsened auditory function after recovery.

Molecular differences between neonatal and adult stria vascularis from organotypic explants and transcriptomics

The paper develops an in vitro model of neonatal and adult stria vascularis and uses single-cell RNA sequencing to identify key genes and pathways involved in SV proliferation and maintenance.

Outer hair cells stir cochlear fluids

The paper investigates how active outer hair cells drive cochlear fluid circulation using experimental and computational methods.

Auditory decision-making deficits after permanent noise-induced hearing loss

This study shows that permanent noise-induced hearing loss impairs auditory decision-making by affecting perceptual acuity, attention, choice bias, and motor execution in gerbils.

Molecular logic for cellular specializations that initiate the auditory parallel processing pathways

The paper identifies molecular logic underlying cellular specializations in the cochlear nuclear complex using single-nucleus RNA sequencing and Patch-seq.

Deciphering compromised speech-in-noise intelligibility in older listeners: the role of cochlear synaptopathy

This study investigates how cochlear synaptopathy affects speech intelligibility in older adults using an EEG-based marker and finds that CS contributes to speech coding deficits in noise beyond hearing sensitivity.

Complexes of vertebrate TMC1/2 and CIB2/3 proteins form hair-cell mechanotransduction cation channels

The paper shows that CIB2/3 and TMC1/2 form complexes essential for hair-cell mechanotransduction in vertebrates, supported by structural and functional analyses.

Notch1 is required to maintain supporting cell identity and vestibular function during maturation of the mammalian balance organs

This study shows that Notch1 is critical for maintaining supporting cell identity in the vestibular organs, and its deletion leads to supporting cell conversion to hair cells and vestibular dysfunction.

Auditory evoked-potential abnormalities in a mouse model of 22q11.2 Deletion Syndrome and their interactions with hearing impairment

The paper investigates how hearing impairment interacts with genetic vulnerability in a mouse model of 22q11.2 deletion syndrome to affect auditory brain function.

Noise-induced hearing loss enhances Ca2+-dependent spontaneous bursting activity in lateralcochlear efferents

This study investigates how noise-induced hearing loss affects Ca2+-dependent spontaneous bursting activity in lateral olivocochlear neurons, finding increased burst duration and altered ion channel function.

Hair cells in the cochlea must tune resonant modes to the edge of instability without destabilizing collective modes

The paper investigates how hair cells in the cochlea tune resonant modes to the edge of instability without destabilizing collective modes using a computational model.

CaBP1 and 2 enable sustained CaV1.3 calcium currents and synaptic transmission in inner hair cells

This study shows that CaBP1 and CaBP2 suppress CaV1.3 channel inactivation in inner hair cells, enabling sustained synaptic transmission and hearing.

Assessment of Contralateral Efferent Effects in Human Via ECochG

This study investigates contralateral efferent effects in humans using ECochG, finding that contralateral noise suppresses neural responses and elevates detection thresholds, suggesting frequency-specific efferent influences.

Bridging the gap between presynaptic hair cell function and neural sound encoding

This study investigates how synaptic heterogeneity in inner hair cells contributes to the diversity of spiral ganglion neuron firing by analyzing ex vivo IHC-SGN synapse recordings.

Placode and neural crest origins of congenital deafness in mouse models of Waardenburg-Shah syndrome

This study investigates the role of endothelin signaling in auditory development using mouse models of Waardenburg-Shah syndrome, revealing distinct contributions from neural crest and placode lineages.

Myosin XVA isoforms participate in the mechanotransduction-dependent remodeling of the actin cytoskeleton in auditory stereocilia

This study investigates the role of MYO15A isoforms in mechanotransduction-dependent remodeling of actin cytoskeleton in auditory stereocilia, finding that they regulate this process and contribute to the stability of the tallest row.