Cogan’s syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan’s syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. 1. Introduction Cogan’s syndrome (CS) is a rare chronic inflammatory disorder characterized by nonsyphilitic interstitial keratitis and vestibuloauditory dysfunction [1]. Associations between CS and systemic vasculitis as well as aortitis also exist [2–4]. The peak incidence of CS occurs in the third decade of life. In the two largest series to date, the median age of onset was 22 years (range 5 to 63 years) [2, 3]. CS may also occur in children and the elderly [5–7]. There is no known gender or racial predominance. Fewer than five percent of patients initially present with systemic manifestations. In these cases, the diagnosis of CS can only be established after the development of eye or inner ear disease [8]. The predominant ocular feature of CS is interstitial keratitis (IK), which typically causes eye redness, pain, photophobia, and blurred vision. On examination of patients with IK, an irregular, granular corneal infiltration is observed, affecting particularly the posterior part of the cornea, near the limbus. Although IK is the classic eye finding, it is not essential for the diagnosis. Ocular inflammation may involve other parts of the eye and lead to iridocyclitis, conjunctivitis, episcleritis, anterior or posterior scleritis, or retinal vasculitis [3, 8–10]. The inner ear manifestations of CS are Ménière’s-like attacks consisting of vertigo, ataxia, nausea, vomiting,
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