The GJB6 gene encodes Connexin 30 (Cx30), located ~ 35 kb telomeric to GJB2. Initially identified in autosomal dominant deafness (DFNA3) GJB6 mutations were later shown to participate in digenic inheritance with GJB2 [79,82]. The gene discussed is GJB6; the disease is autosomal dominant nonsyndromic hearing loss.