Lihong Kui, Peng Ma, Wenben Zhao, Bin Yan, Xiaojing Kuang, Bo Li, Ruishuang Geng, Tihua Zheng, Qingyin Zheng
{"title":"Developmental cochlear defects are involved in early‐onset hearing loss in A/J mice","authors":"Lihong Kui, Peng Ma, Wenben Zhao, Bin Yan, Xiaojing Kuang, Bo Li, Ruishuang Geng, Tihua Zheng, Qingyin Zheng","doi":"10.1002/dvdy.741","DOIUrl":null,"url":null,"abstract":"BackgroundA/J mice exhibited a severe hearing loss (HL) at juvenile stage. Up‐to‐date, studies on HL in A/J mice have mostly focused on the damage or dysfunction of hair cells (HCs), spiral ganglion neurons (SGNs), and stereocilia. We examined A/J mice at the early postnatal stage and found that the damage and the loss of outer hair cells (OHCs) are not severe enough to explain the profound HL observed at this age, which suggests that other cochlear defects may be responsible for HL. To better understand the mechanisms of early‐onset HLin A/J mice, we characterized the pathology of the cochlea from postnatal day 3 to day 21.ResultsOur results showed defects in cochlear HC stereocilia and MET channel function as early as 3 days old. We also found abnormal localization and a significant reduction in the number of ribbon synapses in 2‐week‐old A/J mice. There are also abnormalities in the cochlear nerve innervation and terminal swellings in 3‐week‐old A/J mice.ConclusionAll of the abnormalities of cochlear existed in the A/J mice were identified in the juvenile stage and occurred before HCs or auditory nerve loss and was the initial pathological change. Our results suggest that developmental defects and subsequent cochlear degeneration are responsible for early‐onset hearing loss in A/J mice.","PeriodicalId":11247,"journal":{"name":"Developmental Dynamics","volume":"65 1","pages":""},"PeriodicalIF":2.0000,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Developmental Dynamics","FirstCategoryId":"99","ListUrlMain":"https://doi.org/10.1002/dvdy.741","RegionNum":3,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"ANATOMY & MORPHOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
BackgroundA/J mice exhibited a severe hearing loss (HL) at juvenile stage. Up‐to‐date, studies on HL in A/J mice have mostly focused on the damage or dysfunction of hair cells (HCs), spiral ganglion neurons (SGNs), and stereocilia. We examined A/J mice at the early postnatal stage and found that the damage and the loss of outer hair cells (OHCs) are not severe enough to explain the profound HL observed at this age, which suggests that other cochlear defects may be responsible for HL. To better understand the mechanisms of early‐onset HLin A/J mice, we characterized the pathology of the cochlea from postnatal day 3 to day 21.ResultsOur results showed defects in cochlear HC stereocilia and MET channel function as early as 3 days old. We also found abnormal localization and a significant reduction in the number of ribbon synapses in 2‐week‐old A/J mice. There are also abnormalities in the cochlear nerve innervation and terminal swellings in 3‐week‐old A/J mice.ConclusionAll of the abnormalities of cochlear existed in the A/J mice were identified in the juvenile stage and occurred before HCs or auditory nerve loss and was the initial pathological change. Our results suggest that developmental defects and subsequent cochlear degeneration are responsible for early‐onset hearing loss in A/J mice.
期刊介绍:
Developmental Dynamics, is an official publication of the American Association for Anatomy. This peer reviewed journal provides an international forum for publishing novel discoveries, using any model system, that advances our understanding of development, morphology, form and function, evolution, disease, stem cells, repair and regeneration.