Keywords: Hereditary nephritís, deafness

Abstract: Alport's syndrome is a hereditary disease characterized by the association of nephropathy with symmetrical and progressive sensorineural hearing loss. Other clinical manifestations Ne ocular arcus senilis, anterior lenticonus, cataratt, retinal changes, and peripheral vestibular lesion are less common. The authors present two patients with substantial auditory and vestibular symptams despite little renal involvement. Since the vestibular disorder may be the patient's main complaint the importante of this appropriate diagnosis and management is diseussed.