185例大前庭水管综合征患者的听力学特点分析

张波;沈芳;李萍;孙敬武;许为青;孙家强;

1:安徽省立医院耳鼻咽喉头颈外科

摘要
目的探讨大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者的听力学特点。方法对185例(370耳)LVAS患者进行纯音测听及声导抗、ABR、DPOAE、颞骨高分辨CT、头颅MRI检查,分析其特点。结果 183例(366耳)患者CT检查均提示双侧前庭水管扩大,2例(2耳)提示单侧前庭水管扩大;120例(240耳)行MRI检查,238耳均提示内淋巴囊扩大;112例(224耳)行纯音听阈测试,1例(1耳)听力正常,28例(55耳)为感音神经性聋,85例(168耳)呈混合性聋,其中152耳鼓室导抗图为A型的混合性聋患者,其低频气骨导差较大,中高频气骨导差较小;105例(210耳)行声导抗检测显示鼓室导抗图为A型192耳(其中低频引出声反射7耳),As型1耳,Ad型1耳,B型7耳,C型9耳;176例(352耳)行ABR检测,出现声诱发短潜伏期负反应波(acoustically evoked short latency negative response,ASNR)89例(124耳),占50.6%(89/176),ASNR平均波潜伏期在95dB SPL刺激声强时为3.04±0.29ms,在90dB SPL时为3.12±0.28ms;185例(370耳)DPOAE均未引出。结论 LVAS患者在中耳功能正常时其纯音听阈显示低频气骨导差较大、中高频气骨导差较小的混合性聋,ABR测试可引出特征性的ASNR。
关键词
大前庭水管综合征;气骨导差;短潜伏期负反应波
基金项目(Foundation):
作者
张波;沈芳;李萍;孙敬武;许为青;孙家强;
参考文献

1 Mafong DD,Shin EJ,Lalwani AK.Use of laboratory evaluation and radiologic imaging in the diagnostic evaluation of children with sensorineural hearing loss[J].Laryngoscope,2002,112:1.

2 Griffith AJ,Arts A,Downs C,et al.Familial large vestibular aqueduct syndrome[J].Laryngoscope,1996,106:960.

3 Ellis MA,Audra W.Audiometric findings in children with a large vestibular aqueduct[J].Arch Otolaryngol Head Neck Surg,2004,130:1169.

4 Bemettomo S,Forli F,Bogazzi F,et al.Large vestibular aqueduct syndrome:audiological,radiological,clinical,and genetic features[J].Am J Otolaryngol,2005,26:363.

5 Govaerts PJ,Casselman J,Daemers K,et al.Audiological findings in large vestibular aqueduct syndrome[J].Int J Pediatr Otohinolaryngol,1999,51:157.

6 刘辉,董玉云,莫玲燕,等.大前庭水管综合征患者的听力学特点[J].听力学及言语疾病杂志,2006,14:31.

7 Mafee MF,Charletta D,Kumar A,et al.Large vestibular aqueduct syndrome and congenital sensorineural hearingloss[J].AJNR,1992,13:805.

8 刘韵,张道行.大前庭导水管畸形患者的耳蜗发育状况观察[J].中国中西医结合耳鼻咽喉科杂志,2012,20:365.

9 Freeman S,Sichel JY,Sohmer H.Bone conduction experiments in animals-evidence for a non-osseous mechanism[J].Hear Res,2000,146:72.

10 Shirazi A,Fenton JE,Fagan PA,et al.Large vestigbular aqueduct syndrome and stapes fixation[J].J Laryngol Otol,1994,108:989.

11 Ranke OF,Keidelwd,Weschke HG.Hearing with closure of the fenestra ovalis[J].J Laryngol Rhinol Otol,1952,31:467.

12 Minor LB,Carey JP,Cremer PD,et al.Dehiscence of bone overlying the superior canal as a cause of apparet conductive hearing loss[J].Otol Neurotol,2003,24:270.

13 Mason S,Garnham C,hudson B.Electric response audiometry in young children before cochlear implantation:a short latency component[J].Ear Hear,1996,17:537.

14 Nong DX,Ura M,Owa T,et al.An acoustically evoked short latency negative response in profoung hearing loss antients[J].Acta Otolaryngol,2000,120:960.

15 刘玉欣,周得,崔鑫.儿童大前庭导水管综合征听力特点分析(附6例报告)[J].青岛大学医学院学报,2010,46:508.

16 周娜,于黎明,刘传莲,等.重度聋的声诱发短潜伏期负反应[J].听力学及言语疾病杂志,2003,11:169.

17 兰兰,于黎明,陈之慧,等.短潜伏期负反应诊断前庭水管扩大的意义[J].听力学及言语疾病杂志,2006,14:241.