Korean J Otorhinolaryngol-Head Neck Surg.  2019 Aug;62(8):470-474. 10.3342/kjorl-hns.2018.00549.

A Case of Autoimmune Sensorineural Hearing Loss Responding to Cytotoxic Agent

Affiliations
  • 1Department of Otolaryngology-Head and Neck Surgery, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea. minbeom.kim@gmail.com

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.

Keyword

Autoimmune sensorineural hearing loss; Cytotoxins; Methotrexate

MeSH Terms

Adrenal Cortex Hormones
Cytotoxins
Diagnosis
Hearing Loss, Sensorineural*
Humans
Labyrinth Diseases
Male
Methotrexate
Rare Diseases
Steroids
Adrenal Cortex Hormones
Cytotoxins
Methotrexate
Steroids
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