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October 1969

Hereditary Congenital Deafness With Onychodystrophy

Author Affiliations

Columbus, Ohio; Irvine, Calif
From the Department of Medicine, Ohio State University College of Medicine, Columbus, Ohio (Dr. Goodman), and the Department of Medicine, University of California, College of Medicine Irvine, Calif (Drs. Lockareff and Gwinup).

Arch Otolaryngol. 1969;90(4):474-477. doi:10.1001/archotol.1969.00770030476012

THERE ARE numerous genetic syndromes involving deafness associated with various dermatological conditions.1 In 1961 Feinmesser and Zelig2 first reported two sisters with congenital deafness and onychodystrophy. This combination of defects was transmitted as an autosomal recessive. To the best of our knowledge no other family with such a disorder has since been recorded.

The purpose of this report is to describe and to discuss a family with similar findings, but having a different mode of inheritance.

Report of Cases  The proband is a 61-year-old white woman who was hospitalized at Orange County Medical Center in April 1968 with symptoms of congestive heart failure. Past history, physical examination, and various diagnostic procedures including cardiac catheterization supported the diagnosis of rheumatic heart disease with mitral stenosis.Further information revealed that the patient was born deaf and had abnormalities of the nails. In addition to her classical physical findings of congestive