Hearing, Cochlea, Tinnitus, Genetics

This study shows that loss of Esr1 does not affect auditory function or balance performance in normal-hearing mice, suggesting compensation by ESR2 or other receptors.

This study identifies PKHD1L1 as a cause of autosomal recessive nonsyndromic hearing loss through exome sequencing and functional analysis in human families and animal models.

The paper functionally characterizes deafness-associated pendrin and prestin variants using in vitro assays to assess their pathogenic impacts.

This study investigates immune responses in deafened rats, finding that both innate and adaptive immune mechanisms contribute to spiral ganglion neuron degeneration without requiring complement activation.

This study shows that female GluA3-KO mice develop early hearing loss and afferent swellings when exposed to ambient sound, suggesting sex-dependent vulnerability to AMPAR-mediated excitotoxicity.

This study characterizes hearing loss in mice with human MIR96 mutations and identifies a therapeutic candidate that delays hearing loss progression in heterozygous mice.

This study identifies Nrxn3 as essential for ribbon-synapse assembly in hair cells across mouse and zebrafish models, showing its critical role in synaptic function without affecting acoustic startle behavior.

This study shows that AC102, a pyridoindole, protects residual hearing during cochlear implantation by reducing inflammation and apoptosis.

This study compares simple and complex peripheral auditory models for estimating subcortical EEG TRFs in continuous speech, finding simple models yield similar results faster.

This study identifies parvalbumin and somatostatin as biomarkers for two distinct tectothalamic pathways in the auditory midbrain, revealing functional differences in their connectivity and electrophysiological properties.

This study investigates the role of Jag1 in repressing Notch activation in lateral supporting cells and its impact on outer hair cell development in the cochlea using a mouse model of Alagille syndrome.

The paper uses single-cell sequencing to analyze the cellular heterogeneity of mouse inner ear organoids and compares it to in vivo data, identifying distinct hair cell subtypes and developmental trajectories.

This study shows that CHD4 regulates axon guidance in spiral ganglion neurons by modulating chromatin at cis-regulatory elements to control axon guidance molecules.

This study shows chloride binding does not affect very high-frequency nonlinear capacitance in mouse outer hair cells, suggesting mechanical loads, not anion interactions, govern high-frequency prestin function.

This study shows cisplatin accumulates in hair cell mitochondria and causes dysregulation, linking mitochondrial dysfunction to cisplatin-induced hearing loss.

This paper estimates cochlear frequency selectivity using a convolution model of forward-masked compound action potentials in chinchillas, showing close agreement with auditory nerve tuning curves.

The paper identifies a rare GJD3 haplotype associated with familial Meniere Disease, showing it disrupts connexin assembly and affects inner ear function.

This study shows that EBF1 is crucial for inner ear development, regulating cochlear structure and cell numbers, and is necessary for normal hearing function.

The paper identifies two distinct release modes of alpha-tectorin (TECTA) mediated by microvilli and proteolytic shedding, which determine domain-specific matrix organization in the tectorial membrane.

The paper investigates how attention affects Mismatch Negativity (MMN) responses to intensity deviants in tinnitus patients and controls, finding differential effects based on attention type and deviant direction.

The paper assesses ERP responses to intensity deviant stimuli in groups with different combinations of tinnitus and hyperacusis, revealing distinct profiles and interactions between the conditions.

The paper investigates how different frequency paradigms affect Mismatch Negativity responses in healthy controls using three experimental designs with varying frequency differences.

This study measures hearing ability in prairie voles, finding sex-specific differences in monaural hearing but not binaural hearing or morphology.

This study measures subcortical responses to continuous speech in sound-field conditions and finds that 16 minutes of data is sufficient to reliably estimate TRFs with clear wave V peaks.

The paper identifies objective signatures of tinnitus and auditory hypersensitivity disorders through pupil dilation and facial movement analysis in response to sound valence.

This study shows that zebrafish lateral line hair cells use anion efflux, not cation influx, for mechanotransduction due to low external cation concentrations.

This study shows that Kremen1 inhibits Wnt signaling and hair cell formation in zebrafish lateral line, and its absence leads to excessive regeneration through increased support cells when Wnt is overactivated.

The paper uses optogenetics to study the role of supporting cells in force transmission to and from outer hair cells in the mouse cochlea, finding that hyperpolarizing these cells suppresses cochlear amplification and alters mechanical responses.

The paper develops an auditory model with efferent gain control that simulates increased firing rates in inferior colliculus neurons in response to amplitude-modulated noise.