听力损失和耳植入在查德利·麦卡洛氏综合征:一个案例系列
Ralf A Boerboom1,2, Sabine Engels3,4, Fenna A Ebbens3,4
1Department of Otorhinolaryngology, Head and Neck Surgery, University Medical Center Utrecht, Utrecht, Netherlands.
Cochlear implants international
|January 8, 2026
概括
查德利-麦克库洛氏综合征 (CMS) 听力损失是渐进的,通常涉及听力神经病变谱系障碍 (ANSD). 耳植入器 (CI) 为这些患者提供了比助听器更好的听力结果.
科学领域:
- 遗传学和听力学
- 儿科耳鼻喉科 儿科耳鼻喉科
背景情况:
- 查德利-麦卡洛氏综合征 (CMS) 是一种罕见的遗传性疾病.
- 听力损失是CMS患者常见和渐进的症状.
- 听力神经病谱系障碍 (ANSD) 经常使CMS的听力损失复杂化.
研究的目的:
- 介绍一系列患有查德利-麦克库洛氏综合征 (CMS) 的病例.
- 在CMS患者的耳植入前后分析听力测试结果.
- 评估与助听器相比,耳植入物对CMS相关听力损失的疗效.
主要方法:
- 六名患有CMS和听力损失的患者的回顾性病例系列.
- 耳植入前和后的听力测量数据的分析.
- 助听器装配和耳植入之间的结果比较.
主要成果:
- 三名患者被诊断患有ANSD; 一名被怀疑; 两名不确定.
- 助听器提供的益处有限;听力损失是渐进的.
- 耳植入器 (CI) 产生了令人满意的辅助值,但语音识别有所不同,通常比非CMS患者差.
结论:
- 诊断CMS往往是延迟的,听力损失是早期的迹象.
- 耳植入器在CMS中优于助听器,可以提高听力性能.
- 对于患有CMS和听力损失的儿童,建议早期考虑耳植入.
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