Most mutations in the GJB2 gene cause autosomal recessive deafness 1 (DFNB1), a leading form of NSHL [11], whereas a few mutations in the GJB2 gene have also been reported to cause autosomal dominant deafness 3 (DFNA3) [15, 16]. Here, GJB2 is linked to autosomal recessive nonsyndromic hearing loss 1A.