Sensorineural Hearing Loss of Suspected Autoimmune Etiology: Two Cases of Cogan’s Syndrome
10.3342/kjorl-hns.2021.00437
- Author:
Jungmin AHN
1
;
Brian KIM
;
Kyoung Rai CHO
;
Young-Soo CHANG
Author Information
1. Department of Otorhinolaryngology-Head and Neck Surgery, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Science, Seoul, Korea
- Publication Type:Case Report
- From:Korean Journal of Otolaryngology - Head and Neck Surgery
2021;64(12):943-948
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Cogan’s syndrome is a rare inflammatory disease characterized by non-syphilitic keratitis and vestibulo-auditory symptoms including hearing loss, tinnitus, and vertigo. Although its precise pathogenesis is not known, Cogan’s syndrome is generally considered an autoimmune disease. This hypothesis is supported by the frequently successful remission of hearing loss after steroid administration and the association with other autoimmune disorders such as rheumatoid arthritis. Medical treatment of Cogan’s syndrome depends on disease severity and on how extensive the disease is. The involvement of inner ear pathology requires systemic corticosteroid therapy. In cases of treatment failure or the need for a corticosteroid-sparing effect, other immunosuppressive drugs can be used. We experienced two patients with typical Cogan’s syndrome, presenting bilateral progressive sensorineural hearing loss and dizziness with ocular involvement, which we have successfully treated with systemic steroid administration and immunosuppressive therapy.