Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by bo th renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula a nd intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has b een included in the definition of Alport's syndrome. However, other types o f nephropathy have been occasionally associated with hereditary deafness. T he association of ADPKD with hereditary SNHL has not been previously docume nted. We report a family with ADPKD associated with bilateral sensorineural dearness in a pedigree of four affected members in four generations.