The pathogenicity of GJB3 mutations has not been defined, and it isstill unclear whether these mutations are related to autosomal dominant or recessivenonsyndromic hearing loss, especially in the case of mutation c.538C > T. Themutation in GJB3 (c.538C > T) has been first identified to beassociated with autosomal dominant hearing impairment in two Chinese families (Xia et al., 1998). This evidence concerns the gene GJB3 and hearing loss disorder.