Atypical Cogan's Syndrome

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 exc...

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Main Authors: João Queirós, Sofia Maia, Mariana Seca, António Friande, Maria Araújo, Angelina Meireles
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2013/476527
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author João Queirós
Sofia Maia
Mariana Seca
António Friande
Maria Araújo
Angelina Meireles
author_facet João Queirós
Sofia Maia
Mariana Seca
António Friande
Maria Araújo
Angelina Meireles
author_sort João Queirós
collection DOAJ
description 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.
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institution Kabale University
issn 2090-6722
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publishDate 2013-01-01
publisher Wiley
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series Case Reports in Ophthalmological Medicine
spelling doaj-art-780634b45ebd4d46a38764da8a71155f2025-02-03T05:45:07ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302013-01-01201310.1155/2013/476527476527Atypical Cogan's SyndromeJoão Queirós0Sofia Maia1Mariana Seca2António Friande3Maria Araújo4Angelina Meireles5Departmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalDepartmento de Oftalmologia, Hospital de Santo António, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalBackground. 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.http://dx.doi.org/10.1155/2013/476527
spellingShingle João Queirós
Sofia Maia
Mariana Seca
António Friande
Maria Araújo
Angelina Meireles
Atypical Cogan's Syndrome
Case Reports in Ophthalmological Medicine
title Atypical Cogan's Syndrome
title_full Atypical Cogan's Syndrome
title_fullStr Atypical Cogan's Syndrome
title_full_unstemmed Atypical Cogan's Syndrome
title_short Atypical Cogan's Syndrome
title_sort atypical cogan s syndrome
url http://dx.doi.org/10.1155/2013/476527
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AT angelinameireles atypicalcoganssyndrome