Sensorineural hearing loss in conjunction with aortic insufficiency in systemic lupus erythematosus

Sensorineural hearing loss may occur in SLE, but aortic insufficiency has b een very rarely reported. We are describing two patients with well-establis hed SLE who developed bilateral hearing loss and aortic insufficiency, asso ciated with serological evidence of active lupus. Neither patient had evide nce of keratitis, and thus did not satisfy criteria for Cogan's syndrome. T he aortic insufficiency in one patient stabilized after treatment with high doses of steroids while in the second patient, who refused medical treatme nt, it progressed requiring surgical valve replacement. Our observations su ggest that the aortic valve and the inner ear may share some antigenic cros sreactivity not shared by the cornea. In SLE patients, with sensorineural h earing loss, echocardiography should be performed looking for evidence of a ortic insufficiency, which may be steroid responsive.