仅有部分底回的耳蜗发育不全畸形

Cochlear Hypoplasia with Partial Cochlear Basal Turn

赵堃;王乐;郝少娟;魏楠楠;叶放蕾

1:郑州大学第一附属医院耳鼻咽喉头颈外科

2:郑州大学第一附属医院放射科

摘要
目的 根据影像学特征探讨一种有别于目前Sennaroglu分类标准的特殊耳蜗畸形种类及其临床表现。方法 分析11例(13耳)特殊耳蜗畸形患者的颞骨高分辨率CT(HRCT)、3D-核磁(3D-MRI)表现和听力学结果,归纳此类耳蜗畸形的特点。结果 此类耳蜗畸形的影像学表现为仅有部分耳蜗底回自前庭腹侧发出,无转弯或仅有部分内侧转弯,无上底回、中回及顶回(耳蜗不足0.5圈),无蜗轴及阶间隔;在HRCT轴位图像上均未见到耳蜗与内听道(IAC)产生连接。MRI均显示蜗神经发育不良(CND),听力学均表现为全聋;大多伴有发育异常的前庭(12/13)和畸形的半规管(12/13),对侧耳蜗也多伴有严重畸形(10/13)。根据影像学特点,此类耳蜗畸形仅有部分底回发育,属于耳蜗发育不全(CH),本研究将其命名为耳蜗发育不全X型(CH-X)。结论 仅有部分底回(不足0.5圈)的耳蜗发育不全(CH-X)是一类严重的内耳畸形,约占内耳畸形的2%左右,患耳表现为全聋,由于畸形的耳蜗无明显的蜗孔和蜗神经,人工耳蜗植入可能获益较小。
关键词
耳蜗畸形;耳蜗发育不全畸形;共同腔畸形;耳蜗发育不全I型
基金项目(Foundation):
中原英才计划(ZYYCYU202012117)
作者
赵堃;王乐;郝少娟;魏楠楠;叶放蕾
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