Autoimmune sensorineural hearing loss (ASHL) is a rare disease of uncertain etiology, with no established treatment strategy. The duration of morbidity is increased in refractory cases; and therefore, the preservation of hearing and the prevention of adverse effects with steroid therapy are serious long term issues to consider. Long-term follow up of patients treated for ASHL was performed retrospectively in order to elucidate the pathogenesis of ASHL, evaluate the consequences of steroid therapy, and determine a promising treatment course. The cohort in this study consists of four female patients with refractory ASHL that were followed for 16–26 years. Three patients already had profound deafness on one side, probably due to ASHL, before the initiation of steroid treatment. ASHL was managed with steroid administration and the hearing was evaluated through regular audiometric tests (173–212 times). The relationship between pure tone threshold average and steroid dose was reviewed over a long-term follow-up period for each patient. During follow-up, hearing deficit progressed rapidly several times in all patients, as did responsiveness to steroid therapy. Long-term high-dose steroid therapy was not required for hearing maintenance. Hearing thresholds were nearly maintained in three patients during the 16- to 21- year follow-up, and gradually declined over a 26-year follow-up period in one patient. Considering the progress due to presbycusis, the maintenance of hearing was considered sufficient in all patients. No serious adverse effects were observed in any of the patients. Management of patients affected by ASHL with regular audiometry allowed for hearing maintenance without the morbidity of prolonged steroid therapy. The current observations give insight into the pathogenesis of ASHL pathogenesis and establish an efficient course of treatment.

自身免疫性感觉神经性听力损失 (ASHL) 是一种病因不明的罕见疾病，目前尚无既定的治疗策略。难治性病例的发病持续时间增加；因此，保持听力和预防类固醇治疗的副作用是需要考虑的严重长期问题。回顾性地对接受 ASHL 治疗的患者进行长期随访，以阐明 ASHL 的发病机制，评估类固醇治疗的后果，并确定有希望的治疗方案。本研究中的队列由四名患有难治性 ASHL 的女性患者组成，随访时间为 16-26 年。在开始类固醇治疗之前，三名患者的一侧已经出现严重耳聋，可能是由于ASHL。使用类固醇管理 ASHL，并通过常规听力测试（173-212 次）评估听力。对每位患者的长期随访期间，对纯音阈值平均值和类固醇剂量之间的关系进行了审查。在随访期间，所有患者的听力障碍都迅速发展了数次，对类固醇治疗的反应也是如此。听力维持不需要长期大剂量类固醇治疗。在 16 至 21 年的随访期间，三名患者的听力阈值几乎保持不变，而在 26 年的随访期间，一名患者的听力阈值逐渐下降。考虑到老年性耳聋的进展，所有患者的听力维持都被认为是足够的。在所有患者中均未观察到严重的不良反应。通过定期测听对受 ASHL 影响的患者进行管理，可以维持听力，而不会出现长期类固醇治疗的并发症。目前的观察结果可以深入了解ASHL发病机制并建立有效的治疗过程。