The tectorial membrane extracellular matrix in the cochlea controls auditory transduction, but its earliest development remains unclear. In this issue of Developmental Cell, Niazi et al. have provided insight into tectorial membrane formation, demonstrating proteolytic shedding early in development and interaction with α-tectorin and collagen.
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Different release modes of α-tectorin contribute to the development of the tectorial membrane.
Dev Cell
March 2025
Department of Translational Neurosciences, University of Arizona, College of Medicine, Phoenix, AZ 85004, USA.
The tectorial membrane extracellular matrix in the cochlea controls auditory transduction, but its earliest development remains unclear. In this issue of Developmental Cell, Niazi et al. have provided insight into tectorial membrane formation, demonstrating proteolytic shedding early in development and interaction with α-tectorin and collagen.
View Article and Find Full Text PDFPrevalence and Clinical Characteristics of -Associated Hearing Loss Identified in a Cohort of 7065 Japanese Patients with Hearing Loss.
Genes (Basel)
January 2025
Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto 390-8621, Japan.
Background/objectives: Hearing loss is one of the most common sensorineural impairments, and approximately 60% of early-onset cases are due to genetic variations. The otogelin-like protein, encoded by the gene, is a component of the acellular membranes of the inner ear, such as the tectorial membrane, and is thought to play an important role in cochlear amplification. gene variants are a rare cause of hearing loss such as DFNB84B, a mild-to-moderate sensorineural hearing loss presenting in early childhood with autosomal recessive inheritance.
View Article and Find Full Text PDFKölliker's Organ Functions as a Developmental Hub in Mouse Cochlea regulating spiral limbus and tectorial membrane development.
J Neurosci
February 2025
Anatomy Department, Midwestern University, Downers Grove, IL 60515
Kölliker's organ is a transient developmental structure in the mouse cochlea that undergoes significant remodeling postnatally. Utilizing an epithelial-specific conditional deletion mouse model of (marker and regulator of Kölliker's organ), we show that is required for interdental cell development, and thereby the development of the limbal domain of the tectorial membrane and its medial anchorage to the spiral limbus. Additionally, we show that Kölliker's organ is involved in normal tectorial membrane collagen fibril development and maturation.
View Article and Find Full Text PDFA rare haplotype of the GJD3 gene segregating in familial Meniere's disease interferes with connexin assembly.
Genome Med
January 2025
Otology & Neurotology Group CTS495, Instituto de Investigación Biosanitario, Ibs.GRANADA, Universidad de Granada, 18071, Granada, Spain.
Background: Familial Meniere's disease (FMD) is a rare polygenic disorder of the inner ear. Mutations in the connexin gene family, which encodes gap junction proteins, can also cause hearing loss, but their role in FMD is largely unknown.
Methods: We retrieved exome sequencing data from 94 individuals in 70 Meniere's disease (MD) families.
Post-Traumatic Longitudinal Atlanto-Occipital Dissociation without Neurodeficits Treated with Visor (Head-Neck-Chest ) Orthosis - a Rare Case Report and Review of the Literature.
J Orthop Case Rep
December 2024
Department of Spine Surgery, Children Hospital Westmead, Sydney, Australia.
Introduction: Death is the most common outcome of longitudinal atlanto-occipital dissociation (L-AOD). Even though rare, survival is commonly seen in the pediatric population. This study reports a successful outcome of a pediatric patient with an L-AOD without neurodeficits, immobilized in a visor (head-neck-chest) orthosis.
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