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Korean Journal of Otorhinolaryngology-Head and Neck Surgery > Volume 62(8); 2019 > Article
Korean Journal of Otorhinolaryngology-Head and Neck Surgery 2019;62(8): 470-474.
doi: https://doi.org/10.3342/kjorl-hns.2018.00549
A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent
Yong Woo Lee, Jin Lee, Min-Beom Kim, Sun O Chang
Department of Otolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
세포독성약제에 반응한 자가면역성 감각신경성 난청 1예
이용우, 이진, 김민범, 장선오
성균관대학교 의과대학 강북삼성병원 이비인후과학교실
Correspondence  Min-Beom Kim ,Tel: +82-2-2001-2264, Fax: +82-2-2001-2273, Email: minbeom.kim@gmail.com
Received: 31 July 2018; Revised: September 20, 2018   Accepted: 2 October 2018.  Published online: 26 February 2019.
ABSTRACT
Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineural hearing loss, some in a progressive pattern and others in idiopathic or fluctuating pattern, often accompanied by vestibular symptoms. This disease is also known as autoimmune inner ear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in some cases, it occurs in the context of systemic autoimmune disorder such as wegner granulomatosis, or the Bechet disease. Response to steroids has been the requisite clinical criterion for diagnosis. Treatment usually includes corticosteroids and immunosuppressive drugs. However, after continuous steroid treatment development of corticosteroids, resistance is common in many AIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but also a criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presenting a fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agent in suspicion of autoimmune sensorineural hearing loss. We reviewed this case with reference to relevant publications in the medical literature.
Keywords: Autoimmune sensorineural hearing lossCytotoxinsMethotrexate
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