摘要
目的:探讨大前庭水管综合征(LVAS)的发病、临床表现及防治措施。方法:回顾性分析2002～2008年确诊的76例(152耳)LVAS患者的病史、听力、前庭功能检查及治疗过程。结果:93.4%的患者表现为感音神经性聋,61.8%的患者在低频处存在不同程度的气骨导差,听力损失>40～60dB HL43耳,>60～80dB HL47耳,>80dB HL62耳。听力图以下降型曲线为特征,高频下降型112耳,平坦型29耳,岛状11耳。46例患者行前庭功能检查,显示前庭功能低下。鼓室导抗图141耳A型,11耳C型。高分辨率颞骨CT扫描示:前庭水管管径最小2.2mm,最大6.2mm,表现为开口较宽,深部较狭窄,呈"三角形"或"喇叭形"。本组76例均为单纯前庭水管扩大,无大前庭及半规管畸形或耳蜗畸形,均无智力及其他发育障碍。根据听力损失程度,20例表现为听力突然下降的患者经药物治疗后听力恢复好,11例验配了适宜的助听器,并进行听觉语言康复,45例极重度聋患者行人工耳蜗植入术,术后1个月开始编程调试效果好。结论:LVAS临床主要表现为波动性进行性听力下降,患者应尽早行颞骨高分辨CT检查,该病目前尚无确切有效的治疗方法,对有残余听力的聋儿应尽早选配助听器进行语言康复训练,对极重度聋而助听器无法达到有效补偿者,应尽早行人工耳蜗植入术。
Objective： To explore the disease incidence, clinical symptoms, prevention and treatment measures of the large vestibular aqueduct syndrome （LVAS）. Method： Retrospective analyse the medical history, hearing, vestibular function examination and treatment of 76 LVAS patients who were diagnosed in our department of Otolaryngology from 2002 to 2008. Result：Most patients（93.4%）showed sensorineural hearing loss. Part of patients （61.8%）showed air-bone conduction gap in low frequency . The hearing loss of 43 ears is 2〉40-60 dB HL, 2〉60-80 dB HL 47 ears, 2〉80 dB HL 62 ears. Decline curve is the characteristic of the Audiogram. The decline in high-frequency 112 ears, flat curve in 29 ears, island hearing in 11 ears. Forty-six patients were conducted the vestibular function examination, which showed low vestibular function. Tympanogram showed that 141 ears are type A, 11 ears are type C. High-resolution CT scan revealed that vestibular aqueduct minimum diameter is 2.2 mm and the largest is 6.2 mm, with a wide opening and deep narrower, and showed the “triangle” or “flared”. Forty-two cases of this group were simple dilatation of the vestibular aqueduct, and no large vestibular semicircular canal malformation or cochlear malformation. There was no intellectual and other development disorders. In accordance with the degree of hearing loss, 20 cases of patients restored hearing after drug treatment. Eleven were cases fit a suitable hearing aid and carried out the language rehabilitation training. Forty-five very severe patients were implanted the cochlear and mapping one month later. Conclusion： Fluctuative and progressive hearing loss is the main clinical symptoms of large vestibular aqueduct syndrome. The patients should be examined by high reso-lution CT scan of the temporal bone. There is no precise and effective treatment for the disease. It is very important for the deaf children who have residual hearing to fit hearing aids and carry out the language rehabilitation training as soon as possible. As for the patients who suffer from hearing loss severely and the hearing aid cannot a-chieve effective compensation, the cochlear implant should be considered.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
CSCD
北大核心
2009年第13期594-596,共3页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
关键词
大前庭水管综合征
听觉丧失
人工耳蜗植入术
large vestibular aqueduct syndrome
hearing loss
cochlear implantation
