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.

Optimal management of Cogan’s syndrome: a multidisciplinary approach / D'Aguanno, Vittorio; Ralli, Massimo; de Vincentiis, Marco; Greco, Antonio. - In: JOURNAL OF MULTIDISCIPLINARY HEALTHCARE. - ISSN 1178-2390. - 2018:11(2018), pp. 1-11. [10.2147/JMDH.S150940]

Optimal management of Cogan’s syndrome: a multidisciplinary approach

D'Aguanno, Vittorio
Primo
;
Ralli, Massimo
Secondo
;
de Vincentiis, Marco
Penultimo
;
Greco, Antonio
Ultimo
2018

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.
2018
Cogan’s syndrome; hearing loss; multidisciplinary approach; autoimmunity
01 Pubblicazione su rivista::01g Articolo di rassegna (Review)
Optimal management of Cogan’s syndrome: a multidisciplinary approach / D'Aguanno, Vittorio; Ralli, Massimo; de Vincentiis, Marco; Greco, Antonio. - In: JOURNAL OF MULTIDISCIPLINARY HEALTHCARE. - ISSN 1178-2390. - 2018:11(2018), pp. 1-11. [10.2147/JMDH.S150940]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1044604
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