Department of Otolaryngology-Head and Neck Surgery, Johns Hopkins University, Baltimore, MD, USA.
Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
Ann Otol Rhinol Laryngol. 2021 Dec;130(12):1412-1416. doi: 10.1177/00034894211005979. Epub 2021 Apr 3.
To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation.
Case report and literature review.
A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation.
Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.
报告 1 例复发性多软骨炎致双侧感觉神经性聋和前庭功能丧失病例，并报道人工耳蜗植入后的听力转归。
病例报告和文献复习。
一名 43 岁女性突发听力及平衡丧失，数周后进展为双侧重度感音神经性聋和前庭功能丧失。类固醇治疗无效。她接受了前庭物理治疗和左侧人工耳蜗植入。初次发病约 10 个月后，她出现左耳（除耳垂外）红斑、发热、肿胀和疼痛，鼻梁扁平，以及右踝关节肿胀、发热和皮肤红斑。左侧耳廓活检显示符合复发性多软骨炎的组织病理学特征。她接受了大剂量泼尼松龙治疗。耳部炎症消退，但尽管纯音听阈有良好的听觉反应，患者在人工耳蜗植入后报告言语感知能力无改善。
复发性多软骨炎可表现为迅速进展的重度听力和前庭功能丧失。人工耳蜗植入后的听力转归可包括良好的纯音听阈检测阈值，但言语辨别率差。
