Dominant deafness-onychodystrophy syndrome (DDOD syndrome, MIM #124480) is characterized mainly by congenital sensorineural hearing loss, accompanied by dystrophic or absent nails. In some individuals, conical and hypoplastic teeth, subtle learning and memory disorder may also be observed. It is a rare disease with the prevalence lower than 4 to 6 in 10,000 individuals. This disease follows the autosomal dominant inheritance.
ATP6V1B2