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大前庭导水管综合征23例报告 |
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韩睿 李炬 陈振声
摘要： 报告23例大前庭导水管综合征。全部为感音神经性聋，听力损失中中重度 占23.91％，重度占54.35％，极重度占21.74％。听力曲线高频下降型74％，平坦型13％， 岛状听力13％。部分有进行性听力下降或突发性听力下降，2例行前庭功能检查，显示前庭 功能低下。高分辩内耳CT检查，单纯前庭小管扩大者27耳，伴有大前庭及半规管畸形12耳， 伴耳蜗畸形者7耳。认为该病为胚胎发育性疾病，其发生与胚胎早期内淋巴管发育障碍有关 ，感染是其主要诱因。该病目前尚无确切有效的治疗方法，我们认为对有残余听力的聋儿应 尽早选配助听器进行听觉、语言训练，可取得较为满意的效果，而听力损失极重，助听器无 法达到有效补偿者，或渐进性听力下降至极重度聋者，可考虑选择电子耳蜗植入。
关键词： 大前庭导水管综合征；耳聋；听力康复
中图分类号：R493 文献标识码：A 文章编号：1006-9771(2000)02-0059-03
Large vestibular aqueduct syndrome:23 cases report
HAN Rui,LI J u,CHEN Zhensheng
(Chinese Journal of Rehabilitation Theory & Practice)
Abstract： This paper reports 23 cases of the large vestibular aqueduct syndrome.All cases are sensori-neural hearing loss,23.91％ is moderately severe hearing loss,54.35％ is severe hearing loss,and 21.74％ is profound hearing loss.The contour of audiogram in high frequency dctcrioration i s 74％,che flat contour of audiogram is 13％,the islanded contour audiogram is 1 3％.Some cases are progressive hearing loss,and the others are sudden hearing lo ss,two cases among whole cases were found vestibular dysfunction after examinati on.After high resolution CT scan in inner ear,27 ears were found pathological ch anges only in vestibular aqueduct enlargement,in addition to these changes,12 ea rs were found malformation of vestibule and semicircular canals,and 7 ears were showed cochlea malformation.It is said that the disease is caused by abnormal de velopment on endolymphatic duct in early embryonic stage,and the principal induc ement is infectious factor.There is not an effective treatment,but the better re sults can be reached in deaf children with residual hearing,after they wearing h earing aids and getting the auditory-verbal training in the early period of tim e.If the hearing aid can not reach effective hearing compensation for these pati ents,cochlea implantation is considered and suggested.
Key words： large vestibular aqueduct[1] [2] 下一页
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