Dominant variants in the gap junction beta-2 (<i>GJB2</i>) gene may lead to various degrees of syndromic hearing loss (SHL) which is manifest as sensorineural hearing impairment and hyperproliferative epidermal disorders, including palmoplantar keratoderma with deafness (PPKDFN). Here, GJB2 is linked to Sensorineural hearing impairment.