Six persons in four generations had congenital severe deafness and progressive mid-life visual failure. The 53-year-old proposita and her 9-year-old son had severe neural hearing loss and optic nerve atrophy; the latter was more severe in the mother. Her visual acuity was 20/80 in each eye, while her son's visual acuity was normal. No other cause for the hearing or visual loss could be found except heredity. The proposita's father was congenitally deaf, and at the age of 68, he had progressive visual loss, with optic atrophy. Family history showed that the proposita's two paternal aunts and paternal grandmother had this same syndrome. The syndrome, transmitted by dominant mode; is unique and distinct from other familial syndromes of visual and hearing loss, including those described by Usher, Refsum, Alström, Cockayne, Norrie, Small, Sylvester, Rosenberg and Chutorian, and Tunbridge and Paley.