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Article
September 1965

Congenital Deafness Associated With Piebaldness

Author Affiliations

TEMPE, ARIZ; SALT LAKE CITY
From Arizona State University, Tempe, professor of zoology (Dr. Woolf); and the University of Utah, Salt Lake City; associate professor of surgery and chairman, Division of Otolaryngology (Dr. Dolowitz) and clinical assistant in surgery (Dr. Aldous).

Arch Otolaryngol. 1965;82(3):244-250. doi:10.1001/archotol.1965.00760010246005
Abstract

DURING a study of the frequency of generalized albinism in the various Indian populations occurring in the southwestern part of the United States, two Hopi male siblings with piebaldness (partial albinism) and congenital deafness were encountered.15 Since this syndrome has never been presented before, and these siblings, born in 1952 and 1956, show a remarkably similar pattern of pigment variegation, it was considered worthwhile to describe them in detail. Scientific impetus for this paper is the interest shown in recent years on the association between deafness and pigment defects.4,10,11,13

Albinism Among the Hopi Indians  The Hopi Indian Reservation is located in northeastern Arizona, where it is completely surrounded by the Navajo Indian Reservation. Hopis lived in the same region when the Spaniards first encountered them in the sixteenth century. Their origin is unknown. Today there are 12 Hopi villages, most of which are situated on top of three

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