Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life
Cochlear Implantation in Patients With Usher Syndrome Type IIa Increases Performance and Quality of Life
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DOI：
10.1097/mao.0000000000001441
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发表时间：
2017-07-01
影响因子：
2.1
通讯作者：
Pennings, Ronald J. E.
中科院分区：
文献类型：
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作者：
Hartel, Bas P.;van Nierop, Josephine W. I.;Pennings, Ronald J. E.
目的：Usher综合征IIa型（USH2a）以先天性中重度听力障碍和视网膜色素变性为特征。听力康复从儿童早期开始，使用助听器。在一些USH2a患者中，听力障碍的严重进展导致使用助听器的言语清晰度不足，以及足够的沟通和安全问题。人工耳蜗植入（CI）是这类患者康复的下一步。本研究评估了CI在USH2a患者中的表现和获益。设计：回顾性病例对照研究，评估16名成年语后聋患者（8名USH2a患者和8名匹配对照）CI的表现和益处。通过语音清晰度测试和三份生活质量问卷来评估表现和效益。结果：USH2a患者植入时平均年龄为59岁，CI后表现良好。USH2a患者的音素评分从41%提高到87% (p = 0.02)，对照组的音素评分从30%提高到86% （p = 0.001）。问卷调查的结果显示了CI的明显好处。在CI前后，USH2a患者与对照患者的表现或获益没有差异。结论：CI可提高USH2a患者的言语清晰度并改善其生活质量。
Objectives: Usher syndrome type IIa (USH2a) is characterized by congenital moderate to severe hearing impairment and retinitis pigmentosa. Hearing rehabilitation starts in early childhood with the application of hearing aids. In some patients with USH2a, severe progression of hearing impairment leads to insufficient speech intelligibility with hearing aids and issues with adequate communication and safety. Cochlear implantation (CI) is the next step in rehabilitation of such patients. This study evaluates the performance and benefit of CI in patients with USH2a.Design: Retrospective case-control study to evaluate the performance and benefit of CI in 16 postlingually deaf adults (eight patients with USH2a and eight matched controls). Performance and benefit were evaluated by a speech intelligibility test and three quality-of-life questionnaires.Results: Patients with USH2a with a mean age of 59 years at implantation exhibited good performance after CI. The phoneme scores improved significantly from 41 to 87% in patients with USH2a (p = 0.02) and from 30 to 86% in the control group (p = 0.001). The results of the questionnaire survey demonstrated a clear benefit from CI. There were no differences in performance or benefit between patients with USH2a and control patients before and after CI.Conclusions: CI increases speech intelligibility and improves quality of life in patients with USH2a.