95例大前庭水管综合征的临床分析  

Clinical analysis of 95 patients with large vestibular aqueduct syndrome

作　　者：杨伟炎[1] 张素珍[1] 赵承军[1] 冯勃[1] 

机构地区：[1]解放军总医院耳鼻咽喉-头颈外科,解放军总医院耳鼻咽喉科研究所,北京100853

出　　处：《中华耳鼻咽喉科杂志》2003年第3期191-194,T005,共5页Chinese Journal of Otorhinolaryngology

摘　　要：目的　调查大前庭水管综合征 (largevestibularaqueductsyndrome,LVAS)的发病、遗传和听觉损失情况 ,探讨LVAS之前庭水管外口宽度与听力损失的关系。方法　回溯性搜集 1 992～ 2 0 0 2年 ,95例 (1 90耳 )双侧LVAS的病史 ,听力和前庭功能检查 ,用每例LVAS颞骨CT片的标尺 ,测量前庭水管外口的宽度。结果　 95例 (1 90耳 )LVAS中 ,男 65例 ,女 30例 ,男女比为 2 2∶1。有家族史者 1 2例(1 2 6 % )。平均就诊年龄 7 6岁 ,年龄范围从 1 2～ 39岁。本组绝大多数误诊 ,因轻度外伤而致重度感音神经性聋 2 1例 (2 2 % ) ;上呼吸道感染致聋 1 3例 (1 3 6 % ) ,误诊为突发性聋 9例 (9 5 % ) ;拟诊氨基糖甙类中毒 5例 (5 3 % )。重度感音神经性聋 1 72耳 ,占 92 4%。 77例 (1 54耳 )前庭水管外口宽度( x±s)为 (7 5± 1 2 )mm ,听力损失与外口宽度不成比例。听力损害可能与富含蛋白的内淋巴从内淋巴囊倒流于耳蜗和前庭有关。结论　LVAS是独立的疾病 ,具有波动性及进行性感音神经性聋。诊断依靠颞骨CT扫描或磁共振成像 (magneticresonanceimaging ,MRI)。若听力波动后能改善可保守治疗 ,若明显听力下降 ,影响学习 。Objective To investigate the occurrence, genetic inheritance, and hearing loss conditions of large vestibular aqueduct syndrome (LVAS), and to measure the width of external aperture of vestibular aqueduct (VA) of LVAS, and to analyze the relationship between the hearing loss and the width of external aperture Methods A retrospective review was conducted by the history, auditory, and vestibular function examinations in 95 patients (190 ears) of LVAS The width of VA was measured by CT scaning criterion of each LVAS patients in the past 10 years (from 1992 to 2002) Results In 95 patients, there were male 65 cases and female 30 cases The male cases was more than the females by a ratio of 2 2∶1 Twelve cases (12 6%) would have familial genetic history The average of the consult age was 7 6 years (range from 1 2 to 39 years) The most patients were misdiagnosed, the hearing deterioration originated from mild head trauma in 21 cases (22 1%), the hearing loss after common cold in 13 cases (13 6%) Nine cases (9 5%) misdiagnosed as sudden hearing loss, 5 cases (5 3%) as ototoxic hearing loss of Aminoglycoside antibolic The profound hearing loss was found in 172 ears (92 4%) The average width of external aperture of LVAS was 7 5 mm ±1 2 mm ( ±s , 77 cases, 154 ears) The level of hearing loss did not proportional to the width of VA The hearing impairment could be derived from protein rich endolymph refluxed into the cochlear and vestibule Conclusion The LVAS is distinct clinical entity characterized by fluctuate and progressive SNHL The diagnosis depends upon the CT scan and MR 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

关 键 词：大前庭水管综合征 听力障碍 耳蜗植人 迷路疾病 感音神经性聋 内耳畸形 

分 类 号：R764[医药卫生—耳鼻咽喉科]