GJB2 and autosomal recessive nonsyndromic hearing loss 1A: Intriguingly, the cochlear signature genes included 35 genes responsible for nonsyndromic or syndromic hearing loss such as COCH which is responsible for autosomal dominant nonsyndroic heaing loss (DFNA9, OMIM #601369)10 and predominantly expressed in cochlear lateral wall, and GJB2 which is responsible for autosomal recessive nonsyndromic hearing loss (DFNB1A, #220290)11, the deafness gene most frequently found world wide (Table 2).