The SOX10(SRY-box 10)gene belongs to the SOX(SRY-related HMG-box)transcription factor fami-ly,and is characterized by HMG(high mobility group)boxes that mediate the binding of Sox proteins to DNA.Neural crest cells(NCCs)are migratory pluripotent cells that differentiate into many cell types throughout their development,including vestibular cochlear glial cells in the inner ear,glial cells of the nervous system,melanocytes in the skin,bone and connective tissue cells in the craniofacial complex,and smooth muscle cells in the cardiovascular system.The SOX10 is necessary for the maintenance of the pluripotency of NCCs and the survival of neural crest-derived cells.The SOX10 gene regulates inner ear development in a variety of ways by participating in the development of neural crest-de-rived cells.This paper reviews relevant literatures on the roles of SOX10 in the development of neural crest-derived cells,in order to further understand its regulatory mechanisms in inner ear development,so as to better intervene in syn-dromic deafness caused by SOX10 mutations.