PDF(Pubmed)
Abstract：
BACKGROUND: Auditory neuropathy (AN) is a hearing disorder that affects neural activity in the VIIIth cranial nerve and central auditory pathways. Progressive forms have been reported in a number of neurodegenerative diseases and may occur as a result of both the deafferentiation and desynchronisation of neuronal processes. The purpose of this study was to describe changes in auditory function over time in a patient with axonal neuropathy and to explore the effect of auditory intervention.
METHODS: We tracked auditory function in a child with progressive AN associated with Charcot-Marie-Tooth (Type 2C) disease, evaluating hearing levels, auditory-evoked potentials, and perceptual abilities over a 3-year period. Furthermore, we explored the effect of auditory intervention on everyday listening and neuroplastic development.
RESULTS: While sound detection thresholds remained constant throughout, both electrophysiologic and behavioural evidence suggested auditory neural degeneration over the course of the study. Auditory brainstem response amplitudes were reduced, and perception of auditory timing cues worsened over time. Functional hearing ability (speech perception in noise) also deteriorated through the first 1.5 years of study until the child was fitted with a \"remote-microphone\" listening device, which subsequently improved binaural processing and restored speech perception ability to normal levels.
CONCLUSIONS: Despite the deterioration of auditory neural function consistent with peripheral axonopathy, sustained experience with the remote-microphone listening system appeared to produce neuroplastic changes, which improved the patient\'s everyday listening ability-even when not wearing the device.
METHODS: We tracked auditory function in a child with progressive AN associated with Charcot-Marie-Tooth (Type 2C) disease, evaluating hearing levels, auditory-evoked potentials, and perceptual abilities over a 3-year period. Furthermore, we explored the effect of auditory intervention on everyday listening and neuroplastic development.
RESULTS: While sound detection thresholds remained constant throughout, both electrophysiologic and behavioural evidence suggested auditory neural degeneration over the course of the study. Auditory brainstem response amplitudes were reduced, and perception of auditory timing cues worsened over time. Functional hearing ability (speech perception in noise) also deteriorated through the first 1.5 years of study until the child was fitted with a \"remote-microphone\" listening device, which subsequently improved binaural processing and restored speech perception ability to normal levels.
CONCLUSIONS: Despite the deterioration of auditory neural function consistent with peripheral axonopathy, sustained experience with the remote-microphone listening system appeared to produce neuroplastic changes, which improved the patient\'s everyday listening ability-even when not wearing the device.
摘要：
背景：听觉神经病（AN）是一种听力障碍，会影响第八颅神经和中枢听觉通路的神经活动。在许多神经退行性疾病中已经报道了渐进式形式，并且可能由于神经元过程的去神经和去同步而发生。本研究的目的是描述轴索神经病变患者的听觉功能随时间的变化，并探讨听觉干预的效果。
方法：我们跟踪了一名患有与Charcot-Marie-Tooth（2C型）疾病相关的进行性AN儿童的听觉功能，评估听力水平，听觉诱发电位,和3年的感知能力。此外，我们探讨了听觉干预对日常听力和神经发育的影响。
结果：虽然声音检测阈值始终保持不变，电生理和行为学证据均提示在研究过程中听觉神经变性.听性脑干反应幅度降低，随着时间的推移，对听觉时序线索的感知会恶化。在第一个1.5年的研究中，功能性听力（在噪音中的语音感知）也有所下降，直到孩子配备了“远程麦克风”听音设备，随后改善了双耳处理并将语音感知能力恢复到正常水平。
结论：尽管听觉神经功能恶化与周围轴突病变一致，使用远程麦克风收听系统的持续经验似乎会产生神经可塑性变化，这提高了患者的日常听力能力-即使不佩戴该设备。
方法：我们跟踪了一名患有与Charcot-Marie-Tooth（2C型）疾病相关的进行性AN儿童的听觉功能，评估听力水平，听觉诱发电位,和3年的感知能力。此外，我们探讨了听觉干预对日常听力和神经发育的影响。
结果：虽然声音检测阈值始终保持不变，电生理和行为学证据均提示在研究过程中听觉神经变性.听性脑干反应幅度降低，随着时间的推移，对听觉时序线索的感知会恶化。在第一个1.5年的研究中，功能性听力（在噪音中的语音感知）也有所下降，直到孩子配备了“远程麦克风”听音设备，随后改善了双耳处理并将语音感知能力恢复到正常水平。
结论：尽管听觉神经功能恶化与周围轴突病变一致，使用远程麦克风收听系统的持续经验似乎会产生神经可塑性变化，这提高了患者的日常听力能力-即使不佩戴该设备。
