Vohwinkel syndrome (VS) has been attributed to distinct mutations in Cx26: G59S, Y65H, D66H, and G130V (Maestrini et al., 1999; Alexandrino et al., 2005; Snoeckx et al., 2005; Bondeson et al., 2006; Iossa et al., 2009; de Zwart-Storm et al., 2011). The gene discussed is GJB2; the disease is keratoderma hereditarium mutilans.