大前庭导水管综合征的临床特点

<title>udy on the clinical characteristics of patients with large vestibular aqueduct syndrome

目的 分析大前庭导水管综合征的临床特点,为指导医师和家长采取适宜的防范措施提供依据。方法应用听力学检查(包括纯音测听／儿童游戏测听、声阻抗、听觉脑干诱发电位、耳声发射等)、眼震电图检查和影像学检查(CT和／或MRI检查),对130例(男72例,女58例)进行分析。结果 ①母孕期感冒和出生时有异常者占患病总人数的79％;②有外伤史的占63％;③有3个家族7例发病,占5％;④有耳毒性药物使用史占23.4％;⑤6岁以前首次发病的占83%;⑥占总人数80%的儿童有波动性听力变化,8.5%出现混合性听力下降,69.1％以重度耳聋为首次发病的特点;⑦67％呈现单纯的前庭导水管扩大,且多为双侧畸形;⑧首次发病时间与畸形类型无关;⑨26例可配合眼震电图检查的患者中,稳定期患者半规管功能均对称正常,但在听力下降期进行检查的8例患者中,4例出现了前庭功能异常。结论 尽量避免孕期感染可能有助于减少疾病的出现;预防幼童头部外伤和感冒是减少发病的措施之一;听力波动性变化与前庭功能下降有关联但首次发病时间与畸形类型无关。

<abstract>JECTIVE To analyze the clinical characteristics of 130 patients with Large Vestibular Aqueduct Syndrome (LVAS) and identify the prognostic factors. METHODS 130 patients with LVAS were tested at Beijing Tongren Hospital between 1992 and 2003. The records of serial hearing test (PTA, ABR, OAE, ENG) and high-resolution computer tomography were collected. RESULTS In 130 patients, there were male 72 cases and female 58 cases. The male cases was more than the females. 82 cases (63 %) have head trauma; 7 cases (5 %) have familial history; 30 cases (23.4 %) as ototoxic hearing loss of Aminoglycoside antibolic. The average of the consult age was less than 6 years. 69.1 % cases showed as the profound hearing loss. And there is a fluctuating hearing loss (80 %) and a conductive component often accompany (8.5 %) LVAS. The lower vestibular function was presented in 4 cases by 26 cases who can be tested. CONCLUSION LVAS, a congenital disease, is charade rized by fluctuating sensorineural hearing loss. During pregnancy to avoid infection is very important and it is useful for keeping the good hearing level to avoid head trauma. There is maybe a few relationship between the fluctuate of hearing and vestibular function, but the consult age and type of monstrosity is irrelative.