Autosomal dominant onychodystrophy and congenital sensorineural deafness

The disease "deafness and onychodystrophy" (DOD) is characterized by congen ital hearing impairment and dystrophic or absent nails and teeth. The autos omal dominant form of the disorder has been previously reported only in one family. We describe here another family in which three members in three ge nerations (a girl, her mother, and her maternal grandfather) were affected with DOD. Our finding is consistent with an autosomal dominant mode of inhe ritance and confirms autosomal dominant DOD (DDOD, MIM *124480) as a recogn izable clinical entity.