Concomitant Progressive Deafness, Chronic Nephritis, and Ocular Lens Disease

Ocular lens defects and inner ear type hearing loss are seen individually in juveniles quite frequently. Concomitance of these abnormalities or association with other anomalies is attested to by the multiplicity of syndromes, diseases, and symptom complexes which have been described. Juvenile renal disease associated with hearing loss or ocular disease has also been reported,¹⁻⁷but the combination of simultaneous chronic renal disease, inner ear hearing loss, and lens abnormality is infrequent. Goldbloom and associates⁸,⁹described the clinical findings in a boy with chronic nephritis who developed bilateral nerve deafness at 11 years of age and later was noted to have "an unusual type of bilateral anterior subcapsular cataract." Two brothers, one of whom had inner ear hearing loss, died of chronic renal disease, and the mother had chronic nephritis. Although this was postulated to have been an inherited characteristic, an investigation of four generations of the family