Sensorineural hearing loss may occur in SLE, but aortic insufficiency has been very rarely reported. We are describing two patients with well-established SLE who developed bilateral hearing loss and aortic insufficiency, associated with serological evidence of active lupus. Neither patient had evidence of keratitis, and thus did not satisfy criteria for Cogan's syndrome. The aortic insufficiency in one patient stabilized after treatment with high doses of steroids while in the second patient, who refused medical treatment, it progressed requiring surgical valve replacement. Our observations suggest that the aortic valve and the inner ear may share some antigenic crossreactivity not shared by the cornea. In SLE patients, with sensorineural hearing loss, echocardiography should be performed looking for evidence of aortic insufficiency, which may be steroid responsive.