Hereditary Hematuria, Nephropathy and Deafness

Abstract

THIS report is concerned with a familial renal disease associated with nerve deafness. Familial renal disease without associated nerve deafness, which has been adequately reviewed by Eason,¹ Rinkoff² and Reyersbach³ and their associates, is not discussed further here.We are of the opinion that the three following criteria are necessary for the diagnosis of this syndrome: a family history of progressive renal disease in males affected; the presence or history of hematuria, which may be intermittent; and nerve deafness in most of the males and in some of the females affected. We have thus termed this entity "hereditary hematuria, nephropathy . . .