大前庭水管综合征婴幼儿患者ASSR和ABR听力学特点

目的利用听觉稳态诱发反应(auditory steady state response, ASSR)与听性脑干反应(auditory brainstem response, ABR)对大前庭水管综合征(large vestibular aqueduct syndrome, LVAS) 患者进行听力测试，探讨其ASSR和ABR的听力学特点。方法对63例（121耳）确诊为LVAS和同期来我院就诊的20例非LVAS感音神经性聋患儿进行ASSR和ABR测试，分别记录结果，分析并观察其特点。结果LVAS组:ASSR 在0.5～4?kHz阈值呈上升趋势，直至4?kHz反应阈消失，近成直线关系，随低频听阈值的升高，斜率值减小；给出最大刺激频率中，1?kHz反应阈最后消失；当ASSR听阈介于50～80?dBHL时，若ABR以dBnHL为单位，其对应的反应阈数值小于ASSR听阈数值；当ASSR听阈＞100?dBHL时，ABR 109.6?dBnHL已不能引出V波；84耳ABR最大声刺激未引出V波, 其对应ASSR仍可全部或部分频率引出反应阈。非LVAS组:0.5～4?KHz阈值曲线多为平坦型, 2～4?kHz均值与ABR V波反应阈相近。结论大前庭水管综合征患者ASSR 0.5～4?kHz各频率反应阈值呈升高趋势直至消失，其数值与ABR反应阈数值大小具有阶段性改变，其改变区间在80～100?dBHL。

Audiological features of auditory steady-state response and auditory brain stem response in large vestibular aqueduct syndrome

Objective To explore characteristics of auditory steady-state response(ASSR) and auditory brainstem response(ABR) in patients with large vestibular aqueduct syndrome(LVAS).Methods Characteristics of ASSR and ABR of 63 patients(121ears) with LVAS and 20 patients(40ears) of SHL without LVAS were recorded and analyzed.Results In the LVAS group,ASSR raised in a nearly straight-line from 0.5 to 4 kHz thresholds until the threshold of response 4 kHz,with an increase of low-frequency hearing threshold,the value of...