세포독성약제에 반응한 자가면역성 감각신경성 난청 1예
A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent
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Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineuralhearing 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 innerear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in somecases, 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, aftercontinuous steroid treatment development of corticosteroids, resistance is common in manyAIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but alsoa criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presentinga fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agentin suspicion of autoimmune sensorineural hearing loss. We reviewed this case with referenceto relevant publications in the medical literature.
Abstract
Autoimmune sensorineural hearing loss is a rare disease characterized by bilateral sensorineuralhearing 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 innerear disease (AIED), which primarily involves the vestibulo-cochlear system. However, in somecases, 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, aftercontinuous steroid treatment development of corticosteroids, resistance is common in manyAIED. To patients with AIED, response to steroids is not only a criterion for diagnosis but alsoa criterion to test steroid-sparing therapies such as methotrexate. A 10 year-old boy presentinga fluctuating pattern of bilateral sensorineural hearing loss was treated with a cytotoxic agentin suspicion of autoimmune sensorineural hearing loss. We reviewed this case with referenceto relevant publications in the medical literature.
참고문헌 (21)
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