Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System
Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System
复制标题
中枢神经系统浅表铁沉积症患者的前庭功能障碍
DOI：
10.1097/mao.0000000000001844
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发表时间：
2018
影响因子：
2.1
通讯作者：
Tsutsumi Takeshi
中科院分区：
文献类型：
--
作者：
Takeda Takamori;Kawashima Yoshiyuki;Hirai Chiaki;Makabe Ayane;Ito Taku;Fujikawa Taro;Yamamoto Katsura;Maruyama Ayako;Tsutsumi Takeshi
目的：描述中枢神经系统浅表性铁质沉着症（SSCN）患者的前庭功能。研究设计：回顾性分析。设置：三级转诊中心。患者：10例连续SSCN患者。本研究是SSCN最大的病例系列，其中详细描述了神经耳科检查结果，包括眼震电图记录、视频头脉冲测试（vHIT）和姿势图。干预措施：听力学和神经耳科检查，包括纯音测听、畸变产物耳声发射、言语测听、听性脑干反应、眼震电图记录、vHIT和姿势图。主要结果测量：纯音平均值、DP水平、最大言语辨别评分、听性脑干反应I波和V波之间的峰间潜伏期，结果：听力学检查显示耳蜗性聋3耳，耳蜗后性聋11耳，耳蜗和耳蜗后性聋合并6耳。神经耳科检查发现，8个9例小脑疾病，而所有患者也有外周前庭dysfunctions.Conclusion：除了小脑疾病，SSCN患者患有严重的外周前庭功能障碍，这可能会加剧患者的不平衡。当耳鼻喉科医生遇到明显进行性感音神经性听力损失和失衡的患者时，他们应将SSCN纳入鉴别诊断并进行神经耳科检查，包括眼震电图记录和vHIT和脑磁共振成像。
Objective:To describe the vestibular function in patients with superficial siderosis of the central nervous system (SSCN).Study Design:Retrospective analysis.Setting:Tertiary referral center.Patients:Ten consecutive patients with SSCN. This study is the largest case series of SSCN in which detailed neuro-otological findings, including electronystagmography recording, video head impulse test (vHIT), and posturography, were described.Interventions:Audiological and neuro-otological examinations, including pure-tone audiometry, distortion product otoacoustic emissions, speech audiometry, auditory brainstem responses, electronystagmography recording, vHIT, and posturography.Main Outcome Measures:Pure-tone average, DP level, maximum speech discrimination score, interpeak latency between auditory brainstem responses waves I and V, eye tracking test, examination of optokinetic nystagmus, caloric response, visual suppression, vestibulo-ocular reflex gains, total center of pressure path length, and Romberg's ratio.Results:Audiological examinations suggested that the sensorineural hearing loss was of a cochlear etiology in 3 ears, a retrocochlear etiology in 11 ears, and a combined cochlear and retrocochlear etiology in 6 ears. Neuro-otological examinations revealed that eight out of nine patients had cerebellar disorders, while all patients also had peripheral vestibular dysfunction.Conclusion:In addition to cerebellar disorders, SSCN patients suffer from severe peripheral vestibular dysfunction, which can exacerbate the patient's imbalance. When otolaryngologists encounter patients with distinctly progressive sensorineural hearing loss and imbalance, they should include SSCN in the differential diagnosis and perform neuro-otological examinations, including an electronystagmography recording and vHIT and brain magnetic resonance imaging.