September 13, 2006
Cogan's Syndrome
DISEASE OVERVIEW
• combination of inflammatory eye disease and vestibuloauditory dysfunction
• rare autoimmune disorder affecting the inner ear
• described as Meniere's-like fluctuating hearing loss combined with impaired caloric response
• young adults commonly affected
• As many as 15% of CS patients have a large- to medium-vessel vasculitis
• As a helpful mnemonic device think of a man shooting a gun (rhymes with 'gan' in Cogan’s; see the side effects the shooter experiences in the figures below)
SIGNS / SYMPTOMS
• acute nonsyphilitic interstitial keratitis
• sensorineural hearing loss (see figure 3 below)
• Medium- and large-vessel arteritis (aortitis, aortic regurgitation, coronary artery inflammation, mesenteric vasculitis, and limb claudication)
• severe vertigo (see figure 3 below)
• nausea/ vomiting
• tinnitus
• diarrhea and/or bloody stools (10% patients with CS)
DIAGNOSIS
• clinical suspicion (make sure to consider more common diseases before committing to a diagnosis of CS)
TREATMENT
• systemic corticosteroids for inner ear disease (high doses)
• topical steroids for interstitial keratitis
• cytotoxic agents for cases refractory to corticosteroids
Posted by mmw at 09:36 PM | Comments (0)