An auditory pigmentary syndrome characterized by
hypopigmentation of the skin, iris, hair and stria vascularis of the
cochlea. Most patients present with two different colored eyes, white
forelock and eyelashes, and premature graying of the hair. Moderate
acrocephaly, lack of osseous fusion of the short tubular bones, oligodactyly
of the feet, short stature, pericardial cysts, rectal prolapse, and deformed
ears are reported. Other features include hypoplastic maxilla, asymmetry of
hands, deformity of the first phalanx of the head, absence of the first
digit of the foot, and bifid distal phalanges of the second and third toes.