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.