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目的:通过对大前庭水管综合征(LVAS)与内耳其他畸形的听性脑干反应(ABR)特性进行分析,了解在进行ABR检查过程中出现声诱发短潜伏期负相波(ASNR)对诊断LVAS的特异性。方法:对2008-01-2009-09患感音神经性聋(SNHL)的患儿进行颞骨薄层CT扫描,将其中70例内耳其他畸形患儿的影像学结果进行分析,并将研究对象分组,LVAS组(包括大前庭伴其他内耳畸形)38例(76耳),内耳其他畸形组32例(64耳),在进行行为测听或纯音测听、声导抗、耳声发射听力学检查项目的基础上,均进行ABR检测。结果:ABR检查发现24例(41耳)大前庭水管,在2~3 ms处记录到特征性的负相波,检出率为54%,在这41耳中,轻度听力下降2耳、中度听力下降1耳,重度听力下降5耳,极重度听力下降33耳。内耳其他畸形组未检测到负相波。2组差异有统计学意义(P<0.05)。结论:在LVAS与内耳畸形的ABR特性分析中,LVAS无论是否伴其他内耳畸形,无论听力损失的程度如何,均有可能检测出负相波,认为ASNR是大前庭水管综合征临床听力学特征性表现之一。
OBJECTIVE: To analyze the characteristics of auditory brainstem response (ABR) in the large vestibular aqueduct syndrome (LVAS) and other deformities in the inner ear to understand the role of acoustic-induced short latency negative phase wave (ASNR) in the diagnosis of ABR LVAS specificity. Methods: The children with sensorineural deafness (SNHL) from January 2008 to September 2009 were examined by thin layer CT scan of the temporal bone. The imaging findings of 70 children with other malformations of the inner ear were analyzed and the subjects were grouped , 38 cases (76 ears) in the LVAS group (including large vestibule with other inner ear deformities) and 32 cases (64 ears) in other deformities in the inner ear. The patients underwent behavioral audiometry or pure tone audiometry, acoustic guidance and otoacoustic emission audiometry On the basis of the project, ABR tests were conducted. Results: 24 cases (41 ears) of large vestibular aqueduct were detected by ABR. The characteristic negative phase wave was recorded at 2 ~ 3 ms with a detection rate of 54%. In these 41 ears, mild hearing loss was 2 ears, Moderate hearing loss 1 ear, severe hearing loss 5 ears, very severe hearing loss 33 ears. Other deformities in the inner ear did not detect negative phase waves. The difference between the two groups was statistically significant (P <0.05). CONCLUSIONS: In the analysis of ABR characteristics of LVAS and inner ear deformity, LVAS may detect negative phase waves regardless of the degree of hearing loss, whether or not with other inner ear deformities. It is considered that ASNR is characteristic of clinical audiology of large vestibular aqueduct syndrome One of the performance.