中文摘要：

SOX10基因（性别决定区盒基因，SRY-box10）是调控神经嵴发育的关键转录因子。人类SOX10基因突变会导致神经嵴性疾病，如以耳聋及色素异常为主要表型的Waardenburg综合征。近年来发现SOX10在内耳发育早期即广泛表达于耳板及耳囊，且其突变可导致不同程度内耳畸形。本文重点讨论SOX10基因在内耳发育时期的动态表达，及其在内耳发育中的重要作用。

英文摘要：

SOX10(SRI-box10)plays an essential role as a transcription factor in neural crest development.Mutationsof SOX10can induce disorders related to neural crest cells defects.For instance,Waardenburg syndrome(WS)inhumans is an auditory-pigmentary disorder characterized by sensorineural hearing loss and abnormal pigmentation ofthe hair,skin and iris.Recently,accumulating evidence shows that SOX10is widely expressed in the inner ear duringearly development,and that its mutations can result in various types of inner ear deformities.The current review focuseson expression patterns and crucial impacts of SOX10on inner ear development.