Alcin B, Vatovec J, Zargi M
Department of Otorinolaryngology and Cervicofacial Surgery, University Medical Center, Ljubljana, Slovenia.
Pflugers Arch. 2000;439(3 Suppl):R202-3.
The goal of our work was to determine hearing thresholds in patients with hearing impairment due to hereditary motor and sensory neuropathy (HMSN I). In assessment of auditory function we used two methods: pure tone and speech audiometry. Pure tone audiometry was performed using air and bone conducted signals. Speech comprehension was defined with a test battery of monosyllabic words unknown to the patient. By comparing the results of these methods we were able to differentiate whether the hearing loss was of cochlear or retrocochlear origin. We tested 5 patients with HMSN I associated with difficulty in speech understanding. The tests showed mild to severe elevation of pure tone thresholds but no speech perception in any of tested patients. We suggest that this type of hearing impairment be due to the disorder of the auditory nerve function--a neuropathy of the auditory nerve as part of HMSN.
我们研究的目的是确定遗传性运动和感觉神经病（HMSN I型）所致听力障碍患者的听阈。在听觉功能评估中，我们采用了两种方法：纯音测听和言语测听。纯音测听使用气导和骨导信号。通过一组患者未知的单音节词测试电池来定义言语理解。通过比较这些方法的结果，我们能够区分听力损失是耳蜗性还是蜗后性起源。我们测试了5例伴有言语理解困难的HMSN I型患者。测试显示纯音阈值有轻度至重度升高，但所有受试患者均无言语感知。我们认为这种类型的听力障碍是由于听觉神经功能紊乱——作为HMSN一部分的听觉神经病变所致。
