30例小儿大前庭导水管综合征临床分析

Diagnosis and Treatment of Children with Large Vestibular Aqueduct syndrome a Comprehensive Analysis of 30 Cases

目的探讨小儿大前庭导水管综合征的临床特点、听力学、影像学改变以及治疗方法。方法回顾性分析30例(50耳)小儿大前庭导水管综合征患者的临床资料并总结。结果小儿大前庭导水管综合征表现为小儿发作性眩晕及听力进行性下降。结论颞骨高分辩率CT扫描是小儿大前庭水管综合征的诊断不可缺少的金标准,如患者出现进行性、波动性听力下降或轻微的头颅外伤即引起严重的听力下降,CT确诊后马上进行预防和治疗,重度感音神经性聋患者考虑植入电子耳蜗。

Objective To investigate the Clinical characteristics,and hearing loss conditions of children large vestibular aqueduct syndrome（LVAS）,and the imaging changes and the treatment.Methods A retrospective review was conducted by clinical data in30 patients（50 ears）of LVAS.Results The clinical symptom of LVAS was the high frequency hearing loss or the mixed deafness of low frequency.Conclusion The LVAS diagnosis depends upon the CT image.If the hearing fluctuated and subsequently improved,the children were kept under conservation treatment.If the significantly decreased of hearing effected school performance,the cochlear implant might be considered.