Hartel Bas P, van Nierop Josephine W I, Huinck Wendy J, Rotteveel Liselotte J C, Mylanus Emmanuel A M, Snik Ad F, Kunst Henricus P M, Pennings Ronald J E
*Department of Otorhinolaryngology †Radboud Institute for Health Sciences, Radboud University Medical Center ‡Donders Institute for Brain, Cognition and Behaviour, Radboud University Nijmegen, Nijmegen §Department of Otorhinolaryngology, Leiden University Medical Center, Leiden, the Netherlands.
Otol Neurotol. 2017 Jul;38(6):e120-e127. doi: 10.1097/MAO.0000000000001441.
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.
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.
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.
CI increases speech intelligibility and improves quality of life in patients with USH2a.
IIa型Usher综合征（USH2a）的特征为先天性中度至重度听力障碍和色素性视网膜炎。听力康复始于幼儿期，通过佩戴助听器进行。在一些USH2a患者中，听力障碍的严重进展导致使用助听器时言语清晰度不足，以及沟通和安全方面的问题。人工耳蜗植入（CI）是此类患者康复的下一步。本研究评估了CI在USH2a患者中的效果和益处。
回顾性病例对照研究，以评估CI在16名语后聋成年人（8名USH2a患者和8名匹配对照）中的效果和益处。通过言语清晰度测试和三份生活质量问卷评估效果和益处。
植入时平均年龄为59岁的USH2a患者在CI后表现良好。USH2a患者的音素得分从41%显著提高到87%（p = 0.02），对照组从30%提高到86%（p = 0.001）。问卷调查结果表明CI带来了明显益处。USH2a患者和对照患者在CI前后的表现或益处没有差异。
CI可提高USH2a患者的言语清晰度并改善生活质量。
