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AbstractP 242 A severe onset of an atypical Cogan syndrome in a mature adult: a case report Aliyeva S., Hoffmann E. M., Pfeiffer N. Department of Ophthalmology, University of Mainz Objective: Cogan syndrome is a rare systemic vascular disease characterised by the combination of ocular inflammation with audiovestibular dysfunction. So far fewer than 250 cases have been reported in the literature. Patients with a delay of more than 2 years between eye and ear onsets, and with presence of other inflammatory lesions in addition of keratitis are classified as having atypical Cogan syndrome. Although it was mostly described in young adults, the first manifestation in mature age has been previously reported. Methods: We present an uncommon case of a late severe onset of an atypical Cogan syndrome in an 84-year-old man who developed a deep stromal keratitis, an advanced anterior uveitis with hypopion, and a massive vitreous haemorrhage probably caused by an expulsive bleeding from a choroidal neovascularisation. An acute tinnitus with sudden marked hearing loss was diagnosed in his right ear four weeks previously. Results: Despite intensive treatment with corticosteroids and antibiotics, an initial central diffuse corneal infiltration rapidly progressed to a perforated ulcer. An urgent right penetrating keratoplasty was performed because of corneal perforation. Conclusions: The present case confirms the necessity to strongly suspect Cogan syndrome in a patient at any age with association of ocular inflammatory diseases with vestibuloauditory symptoms. The delay in immunosuppressive treatment may lead to severe complications including blindness and hearing loss.
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