OTOG and autosomal recessive nonsyndromic hearing loss 1A: Both Gjb2loxP/loxP; Otog-Cre and Gjb2loxP/loxP; Sox10-Cre mice are considered models of human DFNB1 non-syndromic hearing impairment, which is frequently associated with truncating mutations that yield non-functional Cx26 proteins (Chan and Chang, 2014; Del Castillo and Del Castillo, 2017).