Cogan's Syndrome
Cogan's syndrome is a rare disorder of unknown origin characterized by
inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer
from intestitial keratitis and sudden onset of tinnitus and hearing loss. Few cases
(around 150) have been published and thus it is difficult to determine the percentage of
patients with underlying systemic disorders such as systemic vasculitis. The variety of
systemic manifestations is large and includes fever splenomegaly, lymphadenopathy, and
musculoskeletal complaints. Systemic vasculitis can be seen in around 10% of cases and may
involve the large vessels, appearing as Takayasu-like vasculitis with affection of the
aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating
the exact extension of the systemic features determines the choice of treatment. While
corticosteroids have proved to be of short-term benefit long-term treatment with
immunosuppressive drugs is controversial. Auditory function in deaf patients has often
been restored successfully with cochlear implants.
To illustrate the nature of the syndrome, we present two patients with a wide clinical
spectrum of symptoms from local disease restricted to the eyes and ears to a widespread
vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We
then review the typical aspects as well as the etiology of the disease.
Modified 3-12-04
Information compiled from the National Institutes of Health |
