Goswamy Jay, Bruce Iain A, Green Kevin M J, O'Driscoll Martin P
Manchester Royal Infirmary, Manchester, UK.
Cochlear Implants Int. 2012 Aug;13(3):184-7. doi: 10.1179/1754762811Y.0000000021. Epub 2011 Sep 23.
Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT.
A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%.
A Med-el Flex(SOFT) cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively.
This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.
夏科-马里-图斯（CMT）病是最常见的遗传性运动和感觉神经病变，可导致严重的感音神经性听力损失，其言语感知缺陷程度超过了若听力损失源于耳蜗时所预期的程度。本研究调查通过人工耳蜗植入重新引入听觉同步性是否会改善那些因CMT继发的时间处理能力受损而存在同步性障碍者的言语感知。
一名67岁男性因缓慢进展的脱髓鞘性周围神经病变，出现听力逐渐但显著下降。在开放式言语辨别测试中，他的得分是0%。
将一个Med-el Flex（SOFT）人工耳蜗完全植入左耳，无手术并发症。人工耳蜗言语处理器在植入后1个月安装，在初次安装后的1周、3个月、9个月和21个月进行标准化言语评估，开放式言语辨别得分分别为0%、0%、53%和54%。
本报告表明，人工耳蜗植入是一种可用于使继发于CMT病且存在听觉不同步的成人患者恢复重度至极重度听力损失的选择。植入后的进展可能比普通人工耳蜗使用者更慢。
