The cochlear extracellular matrix gene mutations and hearing loss.
10.13201/j.issn.2096-7993.2025.08.017
- Author:
Qin WANG
1
;
Yongyi YUAN
1
;
Weiju HAN
1
Author Information
1. Senior Department of Otolaryngology Head and Neck Surgery，the 6th Medical Center of Chinese PLA General Hospital；Medical School of Chinese PLA；State Key Laboratory of Hearing and Balance Science；National Clinical Research Center for Otolaryngologic Diseases；Key Laboratory of Hearing Science，Ministry of Education；Beijing Key Laboratory of Hearing Impairment Prevention and Treatment，Beijing，100853，China.
- Publication Type:English Abstract
- Keywords:
basement membrane;
deafness gene;
extracellular matrix;
tectorial membrane
- MeSH:
Humans;
Extracellular Matrix/genetics*;
Hearing Loss/genetics*;
Mutation;
Cochlea;
Extracellular Matrix Proteins/genetics*
- From:
Journal of Clinical Otorhinolaryngology Head and Neck Surgery
2025;39(8):781-788
- CountryChina
- Language:Chinese
-
Abstract:
The extracellular matrix（ECM）is a complex network structure composed of collagen，glycoproteins，and proteoglycans.It not only provides structural support and viscoelasticity to tissues but also participates in cell signaling，responding to environmental forces and signals to mediate tissue remodeling in response to environmental cues. Due to the intricate and precise functions of the inner ear，the perception and transmission of sound rely on the complex interactions between cochlear cell structures and the ECM. In the inner ear，the ECM not only constitutes key structures such as the basilar membranes（BM）and tectorial membranes（TM），which are essential for sound perception，but also regulates cell shape，adhesion，and migration.Certain ECM components interact with cell surface receptors to activate signaling pathways that regulate gene expression.Additionally，the ECM modulates the storage and diffusion of ions and secreted factors, creating concentration gradients.These functions are critical for inner ear development，repair，and function.Thus，the ECM plays a vital role in auditory processes，and abnormalities in ECM are a cause of certain hereditary hearing loss.This review primarily summarizes the ECM genes that lead to hearing loss.
