THE COGAN syndrome is characterized by sudden onset of photophobia, ocular pain, blurring of vision, and blepharospasm. Usually, within a few hours to a few weeks evidence of eighth nerve involvement appears, manifested by tinnitus, nausea, vomiting, vertigo, and bilateral progressive loss of hearing. The vestibular symptoms are almost identical with those found in Ménière's syndrome, may precede the ocular manifestations, and usually resolve when the deafness is complete. The evolution of the complete syndrome is usually rapid. Interstitial keratitis appears early. It is always bilateral, fluctuates in its severity, and is not associated with retinal disease. Visual acuity diminishes and vascularization of the cornea finally eventuates. Serological and clinical evidence of syphilis is consistently lacking.
The disease is considered rare, with the usual implied qualification that many instances are missed or incorrectly diagnosed. Cogan in his original account in 1945,1 collected four cases in one year and subsequently
STEVENS H. COGAN'S SYNDROME: (Nonsyphilitic Interstitial Keratitis with Deafness). AMA Arch NeurPsych. 1954;71(3):337–343. doi:10.1001/archneurpsyc.1954.02320390067006
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