To study the pathogenesis of hereditary deafness caused by Gjb2 mutations, only an R75W transgenic and some conditional knock-out mouse models (including Cx26OtogCre, Cx26Sox10Cre, P0 knockdown, etc.)have been established, but there are not typical disease models of non-syndromic deafness caused by Gjb2 mutations [18, 19, 42, 54, 55]. The gene discussed is GJB2; the disease is deafness.