Diseases of the kidney have been associated with hearing loss. It is considered that the disorders of fluid and electrolyte metabolism resulting from the renal disease are the cause of the hearing loss. In 1927, Alport described a syndrome of chronic nephritis, hematuria, and progressive renal failure characterized by nephritis accompanied by sensorineural hearing loss and various disorders of the eye. Males are affected more frequently and more severely than females and in males the disease is more likely to progress to renal failure and deafness. The pathogenesis of the inner ear hearing loss is not completely understood. The authors recently experienced a case of Alport's syndrome in 20 year old male patient with slowly progressive symmetrical sensorineural hearing loss, hematuria, proteinuria, anterior lenticonus and anterior polar cataract, and report this case with review of the literature.