关键词: 大前庭导水管综合征, 耳聋, 听力康复

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 is 74%,che flat contour of audiogram is 13%,the islanded contour audiogram is 13%.Some cases are progressive hearing loss,and the others are sudden hearing loss,two cases among whole cases were found vestibular dysfunction after examination.After high resolution CT scan in inner ear,27 ears were found pathological changes only in vestibular aqueduct enlargement,in addition to these changes,12 ears 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 development on endolymphatic duct in early embryonic stage,and the principal inducement is infectious factor.There is not an effective treatment,but the better results can be reached in deaf children with residual hearing,after they wearing hearing aids and getting the auditory-verbal training in the early period of time.If the hearing aid can not reach effective hearing compensation for these patients,cochlea implantation is considered and suggested.

Key words: large vestibular aqueduct syndrome, hearing loss, hearing rehabilitation