Poissant Sarah F, Megerian Cliff A, Hume Dayna
Department of Communication Disorders, University of Massachusetts, Amherst, MA 01003, USA.
Otol Neurotol. 2003 Sep;24(5):751-6. doi: 10.1097/00129492-200309000-00011.
To determine the efficacy of cochlear implantation in an individual with neurofibromatosis type 1 and profound hearing loss and to determine, to the extent possible in a living subject, the site of lesion of the hearing loss in an individual with neurofibromatosis type 1.
Postoperative assessment of an adult male with neurofibromatosis type 1.
The study was completed in the Departments of Otolaryngology and Audiology, University of Massachusetts Medical Center, Massachusetts.
One adult male with neurofibromatosis type 1 and bilateral, chiefly profound, sensorineural hearing loss.
The subject underwent cochlear implantation as treatment of bilateral, chiefly profound sensorineural hearing loss.
Outcome measures include results of electrical auditory brainstem response testing, electrical compound action potential measures, and speech recognition scores.
Results of testing revealed expected latencies of waves II, III, and V of the electrical auditory brainstem response, measurable compound action potentials across virtually the entire length of the electrode array, and high levels of open-set, auditory-only speech understanding.
This is the first case report describing the outcome of cochlear implantation in a patient with neurofibromatosis type 1. Composite test results support the absence of VIIIth nerve degeneration or other anomalies, indicating a cochlear site of lesion for the hearing loss in this patient. Based on the results of this investigation, standard cochlear implantation should be considered a viable option and the first line of therapy for the treatment of bilateral, severe to profound sensorineural hearing loss in patients with neurofibromatosis type 1 and normal, VIIIth nerve radiographic findings.
确定1型神经纤维瘤病伴极重度听力损失患者人工耳蜗植入的疗效，并在活体受试者中尽可能确定1型神经纤维瘤病患者听力损失的病变部位。
对一名成年男性1型神经纤维瘤病患者进行术后评估。
该研究在马萨诸塞州马萨诸塞大学医学中心耳鼻喉科和听力学系完成。
一名成年男性，患有1型神经纤维瘤病，双侧主要为极重度感音神经性听力损失。
该受试者接受人工耳蜗植入，以治疗双侧主要为极重度感音神经性听力损失。
观察指标包括电听性脑干反应测试结果、电复合动作电位测量结果和言语识别分数。
测试结果显示电听性脑干反应的II、III和V波潜伏期正常，几乎在整个电极阵列长度上均可测量到复合动作电位，以及高水平的开放式纯听觉言语理解能力。
这是首例描述1型神经纤维瘤病患者人工耳蜗植入结果的病例报告。综合测试结果支持第VIII对脑神经无变性或其他异常，表明该患者听力损失的病变部位在耳蜗。基于本研究结果，对于1型神经纤维瘤病且第VIII对脑神经影像学检查结果正常的双侧重度至极重度感音神经性听力损失患者，标准人工耳蜗植入应被视为一种可行的选择和一线治疗方法。
