Lysosomal impairment and deafness
ZHAO Weihao
YUAN Yongyi
HAN Weiju
Abstract：Lysosomes are organelles found in nearly all eukaryotic cells and lysosomal impairment can cause a va-riety of diseases. Hearing deficits have been reported in several lysosome-related diseases, including Fabry disease, Gau-cher disease, Pompe disease, Alpha-mannosidosis, mucopolysaccharidosis, Niemann-Pick disease type C1, action myoc-lonus-renal failure, dominant deafness-onychodystrophy syndrome and distal renal tubular acidosis. Most of the diseas-es are inborn lysosomal diseases (also known as lysosomal storage diseases, LSDs). The lesions involve multiple re-gions and there is great clinical heterogeneity among these diseases, among which central nervous system and peripheral nervous system are the most vulnerable. This review summarizes the lysosome-related diseases with auditory system symptoms which have attracted increasing attentions in recent years.
Keywords：lysosomal impairmentinborn lysosomal diseasesdeafness
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：8( 249-256 )