大前庭水管综合征患者的听力特点分析

发布时间：2018-07-31 07:43

【摘要】：目的对大前庭水管综合征(large vestibular aqueduct syndrome,LVAS)患者的听力学特点进行分析,为其早期诊断和干预提供依据。方法对2010年5月~2013年10月经颞骨CT检查确诊为大前庭水管综合征的49例(96耳)患者均进行声导抗、耳声发射、听性脑干反应(ABR)及听性稳态反应(ASSR)检查,其中23例46耳(6岁以上者,均为双侧LVAS)同时行纯音听阈测试。结果 23例(46耳)6岁以上患者中33耳(71.74%,33/46)纯音听阈表现为低中频(2kHz以下)存在明显气骨导差而高频下降型的混合性听力损失,13耳(28.26%,13/46)表现为无气骨导差的感音神经性听力损失;49例96耳鼓室导抗图均为A型,其中5耳可引出声反射;36例68耳(70.83%,68/96)ABR测试在3~4ms处记录到特征性的负相波(ANSR);46例96耳DPOAE均未通过,ASSR检查多为中、重度听力损失。结论 LVAS患者中耳功能正常,纯音听阈可表现为明显的低频气骨导差而高频下降的混合性听力损失,ABR检查可引出ASNR负相波。
[Abstract]:Objective to analyze the characteristics of audiology in patients with large vestibular aqueduct syndrome (large vestibular aqueduct syndrome) and to provide evidence for early diagnosis and intervention. Methods from May 2010 to October 2013, 49 patients (96 ears) with large vestibular aqueduct syndrome diagnosed by CT examination of temporal bone were examined with acoustic conductance, otoacoustic emission, auditory brainstem response (ABR) and auditory steady-state response (ASSR). Pure-tone audiometry was performed in 46 ears of 23 patients (over 6 years old, all of whom were bilateral LVAS). Results of 23 patients (46 ears) over 6 years of age, 33 ears (71.7433 / 46) showed low and medium frequency (below 2kHz), but 13 ears (28.26g / 13 / 46) of mixed hearing loss (28.26g / 13 / 46) with low and medium frequency (below 2kHz) showed sensorineural neuropathy without air conduction difference. In 49 cases of hearing loss, 96 cases of tympanic ventricular impedance were all type A. Among them, 36 cases (70.833% 68 / 96) of acoustic reflex could be induced by ABR. The characteristic negative wave (ANSR) in 46 cases (96 ears) of DPOAE was recorded at 3~4ms. Most of them did not pass ASSR examination. Most of them had moderate or severe hearing loss. Conclusion the middle ear function of LVAS patients is normal, pure tone hearing threshold can be shown as low frequency air-bone conductance difference and high frequency decrease of mixed hearing loss. ASNR negative phase wave can be induced by ABR examination.
【作者单位】：河北大学附属医院耳鼻咽喉科;
【分类号】：R764.7

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