Young Ting-Chia, Wang Wen-Hsiu, Chu Chia-Huei, Lin Hung-Ching, Chen Pey-Yu
Department of Medicine, Mackay Medical College, New Taipei City, Taiwan.
Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Mackay Memorial Hospital, Taipei City, Taiwan.
Laryngoscope. 2025 Apr;135(4):1496-1498. doi: 10.1002/lary.31870. Epub 2024 Oct 23.
Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high index of suspicion. Secondary AIED, which accompanies other autoimmune diseases, occurs in up to 30% of AIED cases. Secondary AIED is a rare manifestation of paraneoplastic syndrome, and the causal malignancy may be occult initially. Detecting and treating associated diseases, along with the use of immunosuppressants, are the mainstay for the management of AIED. Herein, we presented a woman with bilateral fluctuating and progressive SNHL, partially responding to steroid or immunosuppressant treatment. Her hearing eventually stabilized after completing the treatment for diffuse large B cell lymphoma, which was diagnosed 6 months after the onset of hearing loss. This case serves as a reminder to physicians to be aware of AIED in cases of fluctuating SNHL and the possibility of associated malignancy alongside autoimmune disturbance. Laryngoscope, 135:1496-1498, 2025.
自身免疫性内耳疾病(AIED)是波动性感音神经性听力损失(SNHL)的一种罕见病因,通过排除法诊断,需要高度怀疑指数。继发性AIED与其他自身免疫性疾病相关,在高达30%的AIED病例中出现。继发性AIED是副肿瘤综合征的一种罕见表现,且因果性恶性肿瘤最初可能隐匿。检测和治疗相关疾病以及使用免疫抑制剂是AIED管理的主要手段。在此,我们报告了一名患有双侧波动性和进行性SNHL的女性,其对类固醇或免疫抑制剂治疗有部分反应。在完成弥漫性大B细胞淋巴瘤的治疗后,她的听力最终稳定下来,该淋巴瘤在听力损失发作6个月后被诊断出来。这个病例提醒医生,在波动性SNHL的病例中要意识到AIED,以及在自身免疫紊乱的同时存在相关恶性肿瘤的可能性。《喉镜》,135:1496 - 1498,2025年。