幕下浅表性铁沉着导致双侧耳蜗前庭功能丧失1例

Gyuman Lee, Youngmin Mun, Dae Bo Shim
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摘要

浅表性黄素沉着症(SS)是一种罕见的神经退行性疾病,其特征是含铁血黄素沉积在中枢神经系统,有时会导致感音神经性听力损失和前庭功能障碍。本病例报告详细介绍了一名63岁女性患者的诊断和治疗,该患者表现为长达一年的姿势不稳定史和最近的突然右侧听力丧失和失衡症状。体格检查和影像学检查显示双侧耳聋和幕下SS。治疗包括药物治疗、前庭运动、大剂量类固醇和高压氧治疗,导致头晕明显减轻,但听力没有改善。本病例强调临床医生需要考虑突发性听力丧失和头晕患者的SS,即使之前没有任何病史或创伤,以准确确定潜在原因。
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A Case of Patient with Bilateral Cochleovestibular Function Loss due to Infratentorial Superficial Siderosis
Superficial siderosis (SS) is a rare neurodegenerative condition characterized by hemosiderin deposition in the central nervous system, which sometimes leads to sensorineural hearing loss and vestibular dysfunction. This case report details the diagnosis and treatment of a 63-year-old female patient presenting with a yearlong history of postural instability and recent symptoms of sudden right-sided hearing loss and imbalance. Physical examination and imaging revealed bilateral deafness and infratentorial SS. Treatment, including medication, vestibular exercises, high-dose steroids, and hyperbaric oxygen therapy, led to a significant alleviation of dizziness but no improvement in hearing. This case emphasizes the need for clinicians to consider SS in patients presenting with sudden-onset hearing loss and dizziness, even without prior any medical history or trauma, to accurately identify the underlying cause.
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