摘要
目的 分析波动性听力损失在大前庭水管综合征 (largevestibularaqueductsyndrome,LVAS)中的特征。方法 总结LVAS 13例 ( 2 3耳 )的临床资料 ,对其波动性听力损失的机理、LVAS诊断及治疗作一讨论。结果 LVAS13例 ( 2 3耳 )中双侧 10例 ,单侧 3例。平均年龄 7.9岁。多幼年起病 ,呈缓慢进行性听力减退 ,波动性突然加重 12耳 ,并伴发作性眩晕 3例。 6例有提重物、运动、乘飞机、感冒史。 3例有遗传性家族史。纯音听力多呈高频下降型感音神经性聋。眼震电图 12耳中 6耳一侧半规管麻痹。高分辨率CT显示大前庭水管扩大 2 .5～ 8mm ,有的深达前庭总脚。结论 LVAS多于幼年时出现缓慢进行性听力减退 ,可伴波动性突发性听力下降、眩晕 ,常与增加颅内压的活动有关 。
Objective To observe the characteristics of fluctuating sensorineural hearing loss(FSHL) in large vestibular aqueduct syndrome(LVAS).Methods The clinic signs in 13 cases(23 ears) of LVAS were analyzed.The machanism of FSHL,diagnosis and treatment of LVAS were discussed.Results Out of 13 LVAS patients(23 ears),10 patients were bilateral and 3 patients were unilateral.The average age was 7.9 years old.The onsets of LVAS probably were in childhood,and progressed slowly.12 ears were fluctuating hearing loss,3 patients had vertigo attack,and 6 cases were related to carrying heaving objects,doing exercise,taking airplane flights and having severe colds.3 patients had familial heredity history.6 ears were unilateral semicircular canal paralysis.High resolution CT results showed that vestibular aqueduct were large from 2.5 mm to 8 mm,and some were deep into vestibular total crus.Conclusion Besides the enlarged vestibular aqueduct comfirmed by HRCT scans,the syndrome is frequently associated with progressive sensorineural hearing loss presenting in childhood,and the onset is usually fluctuating and sudden,following an event causing increased intracranial pressure.Therefone,to avoid head trauma or activity that may increase intracranial pressure is important for prognosis and preventative counseling.
出处
《听力学及言语疾病杂志》
CAS
CSCD
2003年第2期81-84,I001,共5页
Journal of Audiology and Speech Pathology
