The importance of central auditory evaluation in Friedreich's ataxia

ABSTRACT Objective To assess central auditory function in Friedreich's ataxia. Methods A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments. Results The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed. Conclusion In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

摘要
研究目的：评估弗里德赖希共济失调（Friedreich's ataxia）患者的中枢听觉功能。
研究方法：本研究为横断面回顾性研究，共纳入30例患者，对其开展病史采集、耳鼻咽喉科检查、纯音测听、声导抗检测及脑干听觉诱发电位（brainstem auditory evoked potential, BAEP）评估。
研究结果：检出异常情况如下：纯音测听异常率为43.3%，其中双侧异常占36.7%；BAEP检测异常率为56.6%，其中双侧异常占50%；声导抗检测异常率为46.6%。各项检测结果间差异具有统计学意义（p < 0.05）。
研究结论：听力学筛查中，4kHz频率处可见以听力图下降型构型为主的表现，且该频率下声反射消失。BAEP检测中，Ⅰ、Ⅲ、Ⅴ波潜伏期及Ⅰ-Ⅲ、Ⅰ-V、Ⅲ-V峰间期延长的情况较为常见；13.3%的患者存在Ⅴ波缺失，3.3%的患者所有波形均缺失。