Page 382 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

356 Sarah Conway and David S. Younger

Cogan Syndrome

Morgan and Baumgartner [178] described a 26-year-old man with recurrent pain, spasm
and redness of the left eye with photophobia, excessive tearing, and marked conjunctival
injection, followed by severe attack of dizziness, tinnitus, vertigo, nausea, vomiting, ringing
in the ears, profuse perspiration, and deafness. A diagnosis of recurrent interstitial keratitis
and explosive Meniere disease was made however in retrospect this patient may have been
the first reported case of CS associated with nonsyphilitic interstitial keratitis (IK).
Vestibuloauditory symptoms were later described by Cogan [179]. Haynes and colleagues
[180] set forth diagnostic criteria for typical CS according to the definitions established by
Cogan [179] in a review of 30 patients seen at the National Eye Institute of the
National Institutes of Health (NIH) by Cogan [179, 181] with symptoms of IK that developed
abruptly and gradually resolved, associated with photophobia, lacrimation, and unilateral or
bilateral eye pain. Such symptoms tended to recur periodically for years before becoming
quiescent. Vestibuloauditory dysfunction was manifested by sudden onset of Meniere-like
attacks of nausea, vomiting, tinnitus, vertigo, and frequently progressive hearing loss that
characteristically occurred before or after the onset of IK. However within one to six months
of the onset of eye symptoms, auditory symptoms progressed to deafness over a period of one
to three months, certainly no longer than two years.

Gluth [182] reviewed a cohort of CS seen at the Mayo Clinic between 1940 and 2002.
The commonest symptoms at presentation were sudden hearing loss in 50%, balance
disturbance in 40%, ocular irritation I n32%, photophobia in 23%, tinnitus in 13%,
and blurred vision in 10%). Specifically noted inflammatory eye findings that occurred in the
course of disease included interstitial keratitis in 77%, iritis or uveitis in 37%, oscillopsia in
25%, scleritis or episcleritis in 23%, and conjunctivitis in 10%.

Most patients with CS (58%) were treated with corticosteroids with an overall favorable
response in both vestibuloauditory and ophthalmologic manifestations, with the remainder
demonstrating only ophthalmologic (23%) or vestibuloauditory improvement (19%)
alone (98). Other therapies included methotrexate, cyclophosphamide, azathioprine,
entanercept, hydroxchloroquine, and IVIg therapy. Surgical cochlear implantation can led to
objective and subjective benefits with improved hearing recognition.

Single Organ Vasculitis

Primary CNS Vasculitis

Adult [183] and childhood isolated CNS angiitis [184], primary angiitis of the CNS
[185], adult [186] and childhood PCNSV [187]; granulomatous angiitis of the brain (GAB)
(188) and granulomatous angiitis of the nervous system (GANS) [189], are equivalent terms
for a prototypical primary vasculitic disorder restricted to the CNS of diverse cause and
clinicopathologic expression.

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