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双侧前庭功能丧失:53例患者的临床发现

Bilateral loss of vestibular function: clinical findings in 53 patients.

作者信息

Rinne T, Bronstein A M, Rudge P, Gresty M A, Luxon L M

机构信息

MCR Human Movement and Balance Unit, Institute of Neurology, The National Hospital for Neurology and Neurosurgery, London, UK.

出版信息

J Neurol. 1998 Jun-Jul;245(6-7):314-21. doi: 10.1007/s004150050225.

DOI:10.1007/s004150050225
PMID:9669481
Abstract

The clinical presentations and aetiologies of a series of 53 cases of bilateral vestibular failure (BVF) seen by the authors over a decade were evaluated by retrospective review of the medical records. Thirty-nine per cent of patients had associated neurological disease; 13% had a progressive cerebellar syndrome with disabling gait ataxia, abnormal eye movements and cerebellar atrophy on neuro-imaging. BVF was usually unsuspected. Nine per cent had cranial or peripheral neuropathies and in this group there was no abnormality of brain stem/cerebellar oculomotor function, but hearing loss was common. Eleven per cent revealed BVF and hearing loss secondary to meningitis, and 6% had other neurological disorders. Idiopathic BVF was found in 21% of cases, characterised by paroxysmal vertigo and/or oscillopsia, but no abnormal clinical signs. Gentamicin ototoxicity accounted for a further 17%, while autoimmune disease was present in 9% of patients. Otological or neoplastic disease was diagnosed in the remaining 13% of patients. It was concluded that neurological, audiological and ocular motor assessments allow the probable cause of BVF to be defined in approximately 80% of cases. A group of BVF related to autoimmune pathologies is reported for the first time, indicating the need for immunological screening. Idiopathic BVF may present with only minor visual or vestibular symptoms, while in patients with cerebellar degeneration, BVF may be unsuspected and, thus, underdiagnosed.

摘要

作者通过对病历的回顾性分析,评估了过去十年间所诊治的53例双侧前庭功能衰竭(BVF)患者的临床表现及病因。39%的患者伴有神经系统疾病;13%患有进行性小脑综合征,伴有致残性步态共济失调、异常眼动,神经影像学检查显示小脑萎缩。BVF通常未被怀疑。9%的患者患有颅神经或周围神经病变,该组患者脑干/小脑动眼功能无异常,但听力损失常见。11%的患者因脑膜炎继发BVF和听力损失,6%患有其他神经系统疾病。21%的病例为特发性BVF,其特征为阵发性眩晕和/或视振荡,但无异常临床体征。庆大霉素耳毒性占17%,9%的患者存在自身免疫性疾病。其余13%的患者被诊断为耳科疾病或肿瘤性疾病。研究得出结论,神经学、听力学和眼动评估可在约80%的病例中确定BVF的可能病因。首次报道了一组与自身免疫性病变相关的BVF,表明需要进行免疫学筛查。特发性BVF可能仅表现为轻微的视觉或前庭症状,而在小脑变性患者中,BVF可能未被怀疑,因此诊断不足。

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