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Case Reports in Ophthalmological Medicine
Volume 2013 (2013), Article ID 476527, 4 pages
http://dx.doi.org/10.1155/2013/476527
Case Report

Atypical Cogan's Syndrome

Departmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, Portugal

Received 11 February 2013; Accepted 5 March 2013

Academic Editors: S. M. Johnson, S. Machida, N. Rosa, M. Rosner, and G. Savini

Copyright © 2013 João Queirós et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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.