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

Background: Sensorineural hearing loss is increasingly recognized in Niemann–Pick disease type C (NPC), but the underlying cochlear lesion remains undefined. While prior work emphasized hair-cell (HC) involvement, whether auditory dysfunction instead arises from lateral-wall failure and endocochlear...

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Autores principales:	Toru Miwa, Yusei Yamada, Akira Ishii, Aina Shirakawa, Mayuko Tanaka, Yuki Kondo, Toru Takeo, Naomi Nakagata, Hiroki Takeda, Katsumi Higaki, Ryuji Ikeda, Muneaki Matsuo, Tetsumi Irie, Yoichi Ishitsuka
Formato:	Artigo
Lenguaje:	Inglês
Publicado:	Elsevier 2026-05-01
Colección:	Brain Research Bulletin
Materias:	Niemann–Pick disease type C Hearing loss Spiral ligament of cochlea Endocochlear potential Cholesterol metabolism Lysosomes
Acceso en línea:	http://www.sciencedirect.com/science/article/pii/S0361923026001322
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