Department of Otolaryngology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.
Department of Communications Sciences and Disorders, University of Iowa, Iowa City, IA, USA.
Int J Audiol. 2019 Dec;58(12):902-912. doi: 10.1080/14992027.2019.1633022. Epub 2019 Jul 18.
The primary goal of this study was to characterise the cochlear and neural components of hearing loss in a large cohort of people with Charcot-Marie Tooth neuropathy who reported hearing difficulties. A full complement of audiologic measures including behavioral, physiologic and subjective assessments were administered. Seventy-nine participants completed the study. Forty-four people had CMT1, 27 had CMT2 and four had CMT-INT. Pure tone average was related to age and the absence of high-frequency DPOAE energy, suggesting a strong cochlear component. Acoustic reflexes were often elevated or absent and many participants exhibited abnormal ABR waveforms, suggesting additional neural hearing loss components. Participants with an abnormal or absent ABR wave V exhibited poorer speech perception abilities. There was an association between a prolonged ABR wave I latency and an abnormal or absent ABR wave V with a higher Charcot-Marie Tooth Neuropathy Score (indicating greater disability). The hearing abilities of people with CMT are highly variable. While there were strong neural hearing loss components, speech perception abilities were not disproportionately affected in most participants. Therefore, a hearing aid trial is recommended. ABR responses may be a useful tool for monitoring the progression of CMT over time.
本研究的主要目的是描述在一组报告有听力困难的 Charcot-Marie-Tooth 神经病患者中，耳蜗和神经听力损失的特征。对他们进行了包括行为、生理和主观评估在内的全套听力测量。共有 79 名参与者完成了这项研究。44 人患有 CMT1，27 人患有 CMT2，4 人患有 CMT-INT。纯音平均值与年龄有关，高频 DPOAE 能量缺失，提示存在较强的耳蜗成分。听觉反射常升高或缺失，许多参与者表现出异常的 ABR 波型，提示存在额外的神经听力损失成分。ABR 波 V 异常或缺失的参与者言语感知能力较差。ABR 波 I 潜伏期延长与 ABR 波 V 异常或缺失与 Charcot-Marie-Tooth 神经病评分（表明残疾程度更高）之间存在关联。CMT 患者的听力能力差异很大。虽然存在较强的神经听力损失成分，但大多数参与者的言语感知能力并未受到不成比例的影响。因此，建议进行助听器试验。ABR 反应可能是随时间监测 CMT 进展的有用工具。
