Complicated Cogan's syndrome with aortic insufficiency and coronary stenosis

We describe a case of Cogan's syndrome in a 19-year-old woman with tinnitus, deafness, interstitial keratitis, and complicating aortic insufficiency and coronary stenosis. Serological testing revealed IgG and IgA antibodies against Chlamydia trachomatis. In spite of very high antibody titers there was no direct evidence for C. trachomatis in her urogenital smears or in biopsies of her aortic adventitia, and therefore these findings are of uncertain significance. Reconstruction of the aortic valve and bypass surgery for an ostial stenosis of the left coronary artery were necessary. Ten months after starting cyclosporine treatment her course was stable and cochlear implant surgery was successfully performed.