磁共振内耳三维重建在Ⅱ型Waardenburg综合征患者中的应用

Cranium Nuclear Magnetic Resonance Three-Dimensional Reconstruction of Inner Ear in Patients with Waardenburg Syndrome Type Ⅱ

目的探讨磁共振内耳三维重建在Ⅱ型Waardenburg综合征中的应用。方法回顾性分析2008～2011年期间行人工耳蜗植入术的6例Ⅱ型Waardenburg综合征患儿术前磁共振内耳三维重建影像学检查结果,观察内耳的发育情况及人工耳蜗植入效果。结果 2例内耳无明显异常,4例有半规管畸形,3例有耳蜗畸形,术中均顺利植入全部电极,术后均顺利开机,听觉和言语水平明显提高。结论术前磁共振内耳三维重建影像学检查可清晰显示Ⅱ型Waardenburg患者的前庭、半规管及耳蜗情况,为内耳畸形特别是耳蜗畸形患者的人工耳蜗植入手术提供指导。

Objective To study nuclear magnetic resonance three--dimensional reconstruction of inner ear in patients with Waardenburg syndrome type Ⅱ Methods Cranium nuclear magnetic resonance three--dimensional re- construction of inner ear has been performed in 6 cases with Waardenburg syndrome type 1I in our department from 2008 to 2011. Results Cranium nuclear magnetic resonance in all these 5 cases with Waardenburg syndrome type Ⅱ reflect specificity, four of them the inner ear show anomaly, and one of them displays anomat--signal at second ven- tricle of cerebrum, the electrodes were totally inserted into the cochlea successfully. The sense of hearing and spoken language were improved obviously. Conclusion Preoperative nuclear magnetic resonance three-- dimensional recon- struction of inner ear have sense of guidance in cochlear implantation.