Back to Journals » Journal of Multidisciplinary Healthcare » Volume 11

Optimal management of Cogan’s syndrome: a multidisciplinary approach

Authors D'Aguanno V, Ralli M, de Vincentiis M, Greco A

Received 22 October 2017

Accepted for publication 25 November 2017

Published 22 December 2017 Volume 2018:11 Pages 1—11

DOI https://doi.org/10.2147/JMDH.S150940

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Amy Norman

Peer reviewer comments 2

Editor who approved publication: Dr Scott Fraser

Vittorio D’Aguanno,1 Massimo Ralli,2 Marco de Vincentiis,2 Antonio Greco1

1Department of Sense Organs, Sapienza University of Rome, Rome, Italy; 2Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Rome, Italy

Abstract: Cogan’s syndrome (CS) is a rare disorder characterized by nonsyphilitic interstitial keratitis (IK) and audio-vestibular symptoms. CS affects mainly young Caucasian adults, mostly during their first three decades of age, and may develop into typical and atypical variants. Typical CS manifests primarily with IK and hearing loss, whereas atypical CS usually presents with inflammatory ocular manifestations in association with audio-vestibular symptoms but mostly different Ménière-like symptoms and, more frequently, with systemic inflammation (70%), of which vasculitis is the pathogenic mechanism. CS is considered as an autoimmune- or immune-mediated disease supported mainly by the beneficial response to corticosteroids. Using well-developed assays, antibodies to inner ear antigens, anti-Hsp70, and antineutrophil cytoplasmic antibodies were found to be associated with CS. Corticosteroids represent the first line of treatment, and multiple immunosuppressive drugs have been tried with variable degrees of success. Tumor necrosis factor-alpha blockers and other biological agents are a recent novel therapeutic option in CS. Cochlear implantation is a valuable rescue surgical strategy in cases with severe sensorineural hearing loss unresponsive to intensive and/or innovative immunosuppressive regimens.

Keywords: Cogan’s syndrome, hearing loss, multidisciplinary approach, autoimmunity

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]