Mowry Sarah E, King Sarah
a Faculty at Augusta University , GA , USA.
b Department of Otolaryngology , University Hospital and Clinic, Case Western Reserve University , Cleveland , OH , USA.
Cochlear Implants Int. 2017 Mar;18(2):116-120. doi: 10.1080/14670100.2016.1264115. Epub 2016 Dec 23.
To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation.
case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation.
A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss.
Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.
描述一例慢性炎症性脱髓鞘性多发性神经病（CDIP）合并双侧突发性感音神经性听力损失，随后单侧人工耳蜗植入获益的病例。
回顾病例史并检索文献中关于CDIP、听力损失、耳蜗前庭功能障碍和人工耳蜗植入的相关内容。
一名49岁女性在上呼吸道感染1个月后出现双侧快速进展性感音神经性听力损失（SNHL）。听力损失对大剂量类固醇无反应，且无其他实验室异常或体格检查发现。1个月内，她出现上行性运动麻痹，需要长期呼吸机支持。这种神经疾病被诊断为CDIP，她通过血浆置换和静脉注射免疫球蛋白成功治疗。她的听力从未恢复。在进行人工耳蜗植入时，她在听力计极限范围内无反应，在AzBio测试中得分为0%。术前无法记录到听觉脑干反应（ABR）。她接受了使用环模电极的顺利人工耳蜗植入手术。激活1年后，她的纯音平均听阈（PTA）为20 dB，在AzBio句子测试中得分为40%。她的电刺激听觉脑干反应（eABR）显示为神经病变模式。仅另有两例与第八神经功能障碍相关的CDIP病例被描述，且均未记录有严重听力损失。
与CDIP相关的严重SNHL罕见。尽管该患者有良好的声音接收能力，但植入后1年言语辨别能力较差。这一结果可能是由于第八神经髓鞘化未完全恢复。其他可能性包括最初病毒性上呼吸道感染导致周围神经纤维丢失，这可能导致可刺激的神经基质减少。
