Congenital Hereditary Kidney Disease and Hearing Loss: A Case History

Abstract

This paper is presented to acquaint the otolaryngologist with a relatively rare syndrome consisting of congenital familial kidney disease and nerve hearing loss and to report a case that was seen in the Winthrop Foundation of the Massachusetts Eye and Ear Infirmary. In reviewing the literature it is possible to find a relatively abundant amount of material pertaining to hereditary kidney disease, but very little reporting an associated deafness. Hereditary kidney disease in any form is very unusual.¹ Guthrie² in 1902 described a family in which 12 members had hematuria, either gross or microscopic. These members were reevaluated by Kendall and Hertz, Hurst, and Alport,³ who observed that the hematuria was often intermittent. They also added some more members, seven males and eight females. An associated nerve deafness was usually found in the males, and they died of chronic renal disease before the age of 20. Only