Tietz syndrome is a genetic hypopigmentation and deafness syndrome characterized by congenital profound bilateral sensorineural hearing loss and generalized albino-like hypopigmentation of skin, eyes and hair. The disease is MONDO_0007077 (Tietz syndrome). Also known as: Tietz albinism-deafness syndrome, albinism-deafness of Tietz, hypopigmentation-deafness syndrome, hypopigmentation/deafness of Tietz.