%0 Journal Article
%T Atypical Cogan's Syndrome
%A Jo£¿o Queir¨®s
%A Sofia Maia
%A Mariana Seca
%A Ant¨®nio Friande
%A Maria Ara¨²jo
%A Angelina Meireles
%J Case Reports in Ophthalmological Medicine
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/476527
%X Background. Cogan¡¯s syndrome is a rare clinical entity whose etiopathology is still unknown, and the treatment strategies are not clearly defined. Case. A 23-year-old male presented with symptoms of headache, peripheral facial palsy, persistent right hearing loss and bilateral papillitis. Workup excluded all infectious, granulomatous, neoplastic, and immune causes. The diagnosis of atypical Cogan¡¯s syndrome was established, and the patient was treated with systemic corticosteroids and later on with cyclophosphamide and methotrexate. There were improvement of visual symptoms and stabilisation of left hearing. Conclusion. Cogan¡¯s syndrome is a very rare disease with no specific biological tests for the diagnosis. The diagnostic exams are mostly important to exclude other etiologies. The atypical ocular and audiovestibular manifestations make the diagnosis difficult, delaying the institution of appropriate therapy which may result in profound bilateral deafness. 1. Introduction Cogan's syndrome (CS) is a very rare disorder of unknown etiology, most commonly observed in young Caucasian adults of either gender. It was described in 1945 by Cogan as an association of nonsyphilitic interstitial keratitis (IK) and M¨¦ni¨¨re-like audiovestibular symptoms [1]. Later, in 1980, Haynes and colleagues suggested that the condition should include other ocular and audiovestibular symptoms and proposed diagnostic criteria for typical and atypical CS [2]. In the typical form, the ocular involvement is characterized by IK, sometimes associated with conjunctivitis, subconjunctival haemorrhage, or iritis. On examination, an irregular, granular, perilimbic corneal infiltrate is observed. In most cases, both eyes are affected during the disease course. In the atypical form, many ocular lesions have been described, either isolated or associated with IK, including episcleritis/scleritis, retinitis, choroiditis, optic neuritis, papillitis, and central retinal artery occlusion, among others. The typical ear involvement resembles a picture of M¨¦ni¨¨re disease with a rapid onset of vertigo, nausea, vomiting, or tinnitus, accompanied with progressive hearing loss, usually evolving to deafness in 3 months. The hearing loss is often bilateral from the onset of the disease, but in some patients it may be unilateral, becoming bilateral later on [3]. In the atypical form, the audiovestibular symptoms are not characteristic of a M¨¦ni¨¨re-like disease. We report a case of an atypical Cogan¡¯s syndrome, the workup of the diagnosis, and treatment results. 2. Case Report A 23-year-old male was
%U http://www.hindawi.com/journals/criopm/2013/476527/