Cogan's syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses

Cogan's syndrome of interstitial keratitis and vestibuloauditory dysfunction is rare. Systemic vasculitic manifestations occur, and 10% of patients with this syndrome have aortic valvular disease. A patient with Cogan's syndrome is presented who had aortitis of the ascending thoracic aorta, severe aortic valve regurgitation, orificial stenosis of the right coronary artery, and orificial stenoses of all three aortic arch vessels. Histopathology confirmed aortitis. Aortic valve replacement combined with coronary and aortic arch vessel reconstruction was required for correction.