Journal of Otology & RhinologyISSN: 2324-8785

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Review Article, J Otol Rhinol Vol: 4 Issue: 5

Cogan's Syndrome: A Retrospective Study of 22 Years

Sandra Agostinho1*, Marta Canas Marques1,2, Marco Alveirinho Simão1,2 and Óscar Dias1,2
1Department of Otorhinolaryngology, Voice and Communication Disorders, Santa Maria Hospital, Lisbon, Portugal
2Otorhinolaringology Clinic University, Faculty of Medicine, University of Lisbon, Hospital of Santa Maria, Lisbon, Portugal
Corresponding author :Sandra Filomena da Silva Agostinho MD
Department of Otolaryngology, Voice and Communication Disorders, Hospital of Santa Maria, Avenida Professor Egas Moniz, 1649-035, Lisboa – Portugal
Tel: 21 780 5000; Fax: 21 780 5610
E-mail: [email protected]
Received: February 12, 2015 Accepted: June 09, 2015 Published: June 15,2015
Citation: Agostinho S, Marques MC, Simão MA, Dias O (2015) Cogan’s Syndrome: A Retrospective Study of 22 Years. J Otol Rhinol 4:4. doi:10.4172/2324-8785.1000233

Abstract

Introduction: Cogan’s syndrome (CS) is a rare clinical entity, of presumptive autoimmune etiology, typically characterized by a non-syphilitic interstitial keratitis (IK) associated with a Menière-like vestibuloauditory dysfunction.

Objective: Since only few cases have been published to current date and considering the variable presentation, clinical course and absence of specific diagnostic tests, we aim to potentially bring further insight for diagnosis and management.

Materials and Methods: We conducted a retrospective study of all cases of CS followed at the Department of Otorhinolaryngology, Voice and Communication Disorders in Santa Maria Hospital, Lisbon, from 1992 to 2013. Patient’s demographics, otolaryngologic, ophthalmologic and systemic manifestations, presenting symptoms, audiovestibular and laboratory testing, imaging assessment, performed treatment, disease course and hearing outcomes were analyzed.

Results: We review a group of six patients, two with typical and four with atypical CS. Five patients evolved to profound hearing loss, bilateral in two cases. No patient had permanent visual deficit. The Erythrocyte Sedimentation Rate (ESR) increase was the most consistent laboratory finding. Only one patient had positive anti- Heat Shock Protein (HSP) 70 antibodies. All patients were treated with corticosteroids. Methotrexate was associated in three, with some hearing loss stabilization. Three patients underwent cochlear implantation.

Discussion and Conclusions: In our series, morbidity was mainly related to the vestibuloauditory dysfunction. In implanted patients, there was a significant improvement of hearing function. We highlight the need of a multidisciplinary approach to this pathology for better therapeutic outcomes and quality of life.

Keywords: Cogan’s syndrome; Autoimmune hearing loss; Interstitial keratitis

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