Developmental cochlear defects are involved in early-onset hearing loss in A/J mice.

Autor: Kui L; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Ma P; Department of Medical Genetics and Cell Biology, Binzhou Medical University, Yantai, China., Zhao W; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Yan B; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Kuang X; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Li B; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Geng R; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Zheng T; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China., Zheng Q; Hearing and Speech Rehabilitation Institute, College of Special Education and Rehabilitation, Binzhou Medical University, Yantai, China.
Jazyk: angličtina
Zdroj: Developmental dynamics : an official publication of the American Association of Anatomists [Dev Dyn] 2024 Sep 18. Date of Electronic Publication: 2024 Sep 18.
DOI: 10.1002/dvdy.741
Abstrakt: Background: A/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.
Results: Our 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.
Conclusion: All 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.
(© 2024 American Association for Anatomy.)
Databáze: MEDLINE
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