神经纤维瘤病 2 型患者的人工耳蜗植入听觉康复。
Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants.
机构信息
Department of Otolaryngology, New York University School of Medicine, New York, New York 10016, USA.
出版信息
J Neurosurg. 2011 Oct;115(4):827-34. doi: 10.3171/2011.5.JNS101929. Epub 2011 Jul 15.
OBJECT
The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation.
METHODS
Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise.
RESULTS
Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15-120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants.
CONCLUSIONS
Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.
目的
本研究旨在确定是否存在神经纤维瘤病 2 型（NF2）且同侧耳蜗神经完整的患者，在接受耳蜗植入后能否实现开放式言语辨别。
方法
回顾了 7 例经证实的 NF2 患者的病历，以确定其耳蜗植入后的言语辨别结果。使用辅音-核-辅音词和音素来测量结果；在安静环境下使用 Hearing in Noise Test 句子；在安静和嘈杂环境下使用 City University of New York 句子。
结果
术前，没有患者具有开放式言语辨别能力。7 例患者中有 5 例曾行同侧前庭神经鞘瘤（VS）切除术。其中一位接受耳蜗植入的患者曾接受同侧 VS 放射治疗，另一位患者因同侧小 VS 而接受观察。在接受耳蜗植入后，7 例 NF2 患者中有 4 例在延长随访（15-120 个月）期间实现了开放式言语辨别。3 例未实现开放式言语理解的患者中有 2 例（120 和 132 个月）同侧 VS 切除与耳蜗植入之间的时间间隔较长，且在植入时存在耳蜗骨化。另一位未实现开放式言语理解的患者在植入时具有良好的对侧听力。尽管存在这些发现，7 例患者中有 6 例是耳蜗植入的日常使用者，第 7 例是偶尔使用者，这表明所有患者都从植入物中获得了一些主观收益。
结论
对于同侧耳蜗神经完整的 NF2 患者，耳蜗植入可以提供长期的听觉康复，并实现开放式言语辨别。影响植入物性能的因素包括：1）VS 切除与植入之间的时间延长；2）耳蜗骨化。
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