Results 91 to 100 of about 15,676 (196)

Defective Tmprss3-Associated Hair Cell Degeneration in Inner Ear Organoids

Stem Cell Reports, 2019
Summary: Mutations in the gene encoding the type II transmembrane protease 3 (TMPRSS3) cause human hearing loss, although the underlying mechanisms that result in TMPRSS3-related hearing loss are still unclear.
Pei-Ciao Tang, Alpha L. Alex, Jing Nie, Jiyoon Lee, Adam A. Roth, Kevin T. Booth, Karl R. Koehler, Eri Hashino, Rick F. Nelson   +8 more
doaj   +1 more source

A Novel Missense Variant in LMX1A Leads to Autosomal Dominant Nonsyndromic Hearing Loss

American Journal of Medical Genetics Part A, Volume 200, Issue 2, Page 531-534, February 2026.
ABSTRACT Hereditary nonsyndromic hearing loss (NSHL) is a prevalent entity associated with over 150 known causative genes, including LMX1A, which has fewer than 10 reported pathogenic variants. Here we present a novel missense variant in LMX1A in a family of European descent with hereditary hearing loss. Clinical and family histories were obtained, and
Ryan Chen, Dayna Morel Swols, Guney Bademci, Mustafa Tekin   +3 more
wiley   +1 more source

An efficient strategy for establishing a model of sensorineural deafness in rats

Neural Regeneration Research, 2015
Ototoxic drugs can be used to produce a loss of cochlear hair cells to create animal models of deafness. However, to the best of our knowledge, there is no report on the establishment of a rat deafness model through the combined application of ...
Long Ma, Hai-jin Yi, Fen-qian Yuan, Wei-wei Guo, Shi-ming Yang   +4 more
doaj   +1 more source

Distribution of Voltage‐Gated Sodium Channels and Scaffolding Proteins on Vestibular Calyx Ending Delineates the Axon Initial Segment

Journal of Comparative Neurology, Volume 534, Issue 2, February 2026.
Various sodium channel isoforms and their scaffolding proteins are found in four different microdomains in vestibular calyx‐bearing afferents. These microdomains serve different purposes, analogous to the heminode, axon initial segment, and synaptic domains of other types of sensory afferents.
Anna Lysakowski, Aravind Chenrayan Govindaraju, Steven D. Price, Sophie Gaboyard‐Niay, Irina Calin‐Jageman, Robstein L. Chidavaenzi, Ruth Anne Eatock, Robert M. Raphael, Jay M. Goldberg   +8 more
wiley   +1 more source

Cell type-specific transcriptome analysis reveals a major role for Zeb1 and miR-200b in mouse inner ear morphogenesis.

PLoS Genetics, 2011
Cellular heterogeneity hinders the extraction of functionally significant results and inference of regulatory networks from wide-scale expression profiles of complex mammalian organs.
Ronna Hertzano, Ran Elkon, Kiyoto Kurima, Annie Morrisson, Siaw-Lin Chan, Michelle Sallin, Andrew Biedlingmaier, Douglas S Darling, Andrew J Griffith, David J Eisenman, Scott E Strome   +10 more
doaj   +1 more source

The Key Transcription Factor Expression in the Developing Vestibular and Auditory Sensory Organs: A Comprehensive Comparison of Spatial and Temporal Patterns

Neural Plasticity, 2018
Inner ear formation requires that a series of cell fate decisions and morphogenetic events occur in a precise temporal and spatial pattern. Previous studies have shown that transcription factors, including Pax2, Sox2, and Prox1, play important roles ...
Shaofeng Liu, Yunfeng Wang, Yongtian Lu, Wen Li, Wenjing Liu, Jun Ma, Fuqin Sun, Mao Li, Zheng-Yi Chen, Kaiming Su, Wenyan Li   +10 more
doaj   +1 more source

Therapeutic potential of stem cells in auditory hair cell repair

Journal of Stem Cells and Regenerative Medicine, 2009
The prevalence of acquired hearing loss is very high. About 10% of the total population and more than one third of the population over 65 years suffer from debilitating hearing loss.
Ryuji Hata
doaj  

Cochlear Homeostasis in Sensorineural Hearing Loss: Mechanisms, Implications, and Therapeutic Prospects. [PDF]

Int J Mol Sci
Vlajkovic SM, Suzuki-Kerr H, Nayagam BA.
europepmc   +1 more source

A novel minimally invasive stereotaxic technique to target inner ear neurons in the mouse. [PDF]

Front Neurol
Al-Asad RK, Luu NC, Edge AS, Kempfle JS.
europepmc   +1 more source

GPR156 is required in sensory hair cells for proper auditory and vestibular function. [PDF]

Sci Rep
Jarysta A, Verdone BM, Hartig EI, Cullen KE, Tarchini B.   +4 more
europepmc   +1 more source