Spiral ligament dysfunction and endocochlear potential loss drive hearing impairment in Niemann–Pick C1 mice

NPC-related hearing loss arises primarily from SLi dysfunction and EP failure, with secondary HC compromise, rather than degeneration. These findings reposition NPC hearing loss as a disorder of cochlear homeostasis and identify EP preservation and correction of cholesterol trafficking as rational therapeutic targets. Early auditory monitoring may improve clinical outcomes.

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Publication Details

Journal
Brain Research Bulletin
Published
2026-03-01
DOI
https://doi.org/10.1016/j.brainresbull.2026.111846
Primary Topic
Hearing, Cochlea, Tinnitus, Genetics
Type
article
Field-Weighted Citation Impact
0.00
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article

Spiral ligament dysfunction and endocochlear potential loss drive hearing impairment in Niemann–Pick C1 mice

Ryuji Ikeda, Yoichi Ishitsuka, Naomi Nakagata, Yusei Yamada et al.
Brain Research Bulletin
Hearing, Cochlea, Tinnitus, Genetics
article

Spiral ligament dysfunction and endocochlear potential loss drive hearing impairment in Niemann–Pick C1 mice

Ryuji Ikeda, Yoichi Ishitsuka, Naomi Nakagata, Yusei Yamada, Muneaki Matsuo, Aina Shirakawa, Mayuko Tanaka, Katsumi Higaki, Akira Ishii, Yuki Kondo, Hiroki Takeda, Tetsumi Irie, Toru Miwa, Toru Takeo
article en

Abstract

NPC-related hearing loss arises primarily from SLi dysfunction and EP failure, with secondary HC compromise, rather than degeneration. These findings reposition NPC hearing loss as a disorder of cochlear homeostasis and identify EP preservation and correction of cholesterol trafficking as rational therapeutic targets. Early auditory monitoring may improve clinical outcomes.

Brain Research Bulletin
University of Miyazaki (JP), Saga University (JP), Kumamoto University Hospital (JP), University of Miyazaki Hospital (JP), Tottori University (JP), Teikyo University (JP), Kumamoto University (JP)
Openalex Percentile: Top 7%
Hearing, Cochlea, Tinnitus, Genetics
DOIOpen Access PDFOpenAlex
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Spiral ligament dysfunction and endocochlear potential loss drive hearing impairment in Niemann–Pick C1 mice — Ryuji Ikeda, Yoichi Ishitsuka, et al. · Brain Research Bulletin (2026) | TGRS Research Map | TGRS