Cogan Syndrome

Extremely rare: approximately 250 reported cases in the literature

Mean presentation 30–40 y; however, cases in children and elderly reported

No predilection for gender, race, or ethnicity

Possible association with IBD

Hemorrhage

Thrombosis and organ/limb ischemia

Adrenal insufficiency and immunosuppression due to chronic treatment

Postop N/V with vestibuloauditory dysfunction

Activity state of disease and hemorrhage/extension of pathologic vasculitis

Coexisting vasculitis affecting cerebral, cardiac, mesenteric, and renal perfusion

Sepsis with immunosuppression

Heterogeneous presentation of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms within 2 y of each other; note an atypical version allows exceptions to these criteria

10–15% of pts develop large cell vasculitis, usually aortitis

Coronary involvement: often asymptomatic

Typically sudden severe bilateral hearing loss; distinct from unilateral Meniere disease; deafness develops in ∼50% of pts.

Recurrent flares for majority of pts

Mean long-term survival: 20+ y after diagnosis

No definitive cause, but an autoimmune process is suspected; often preceded by a viral prodrome.

Proposed mechanisms include antibodies to an inner ear peptide, Cogan peptide, and HSP70.

Rheumatoid factor and ANA are not consistently associated with diagnosis, but a small percent of pts are ANCA+.

Approximately 50% have a history of daily smoking, and approximately 33% have or develop IBD.