Dominant mutations in Cx26 have been described in several syndromic deafness associated with skin diseases with varying phenotypes, including KID, palmoplantar keratoderma with deafness, Bart-Pumphrey syndrome, Vohwinkel syndrome, and Hystrix-like ichtyosis-deafness syndrome [13]. The gene discussed is GJB2; the disease is keratoderma hereditarium mutilans.