Two brothers with diffuse palmoplantar keratoderma (Thost-Unna type) also were deaf. Of the 38 members of the patients' family, five had a similar disorder and ten had only hearing loss. The mode of inheritance of the dermatosis is regarded as autosomal dominant and is diagnostically distinguished from the other dermatoses associated with the disturbance of keratinization and deafness. To our knowledge, this is the second report of diffuse palmoplantar keratoderma (Thost-Unna type) with deafness, which is considered to be a new variant of the keratodermatoses.