仅有部分底回的耳蜗发育不全畸形  

Cochlear Hypoplasia with Partial Cochlear Basal Turn

作　　者：赵堃[1] 王乐[1] 郝少娟[1] 魏楠楠 叶放蕾[1] Zhao Kun;Wang Le;Hao Shaojuan;Wei Nannan;Ye Fanglei(Department of Otolaryngology-Head and Neck Surgery,the First Affiliated Hospital of Zhengzhou University,Zhengzhou,450052,China;不详)

机构地区：[1]郑州大学第一附属医院耳鼻咽喉头颈外科,郑州450052 [2]郑州大学第一附属医院放射科

出　　处：《听力学及言语疾病杂志》2024年第3期221-227,共7页Journal of Audiology and Speech Pathology

基　　金：中原英才计划(ZYYCYU202012117)。

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。Objective To study a distinct type of cochlear malformation,differentiated by its radiological feature,from the current Sennaroglu's classification criteria along with its clinical manifestations.Methods By analyzing the radiological findings from high-resolution CT(HRCT)of the temporal bone and 3D-dimensional magnetic resonance imaging(3D-MRI)of the inner ear in 11 cases(13 ears)with special cochlear malformations,we summarized the characteristics of this type of cochlear deformity.Results①The radiological features of this type of cochlear malformation showed that only a part of the cochlear basal turn originated ventrally from the vestibule,with no or only partial inward turning.There were no upper,middle,or apical turns(cochlea with less than 0.5 turns),and the modiolus and interscalar septa were absent.On HRCT axial images,there was no connection observed between the cochlea and the internal auditory canal(IAC).MRI results consistently showed a cochlear nerve deficiency(CND),with audiometric findings indicating complete hearing loss.Most cases were accompanied by abnormally developed vestibules and malformed semicircular canals,and the cochlea on the opposite side often had severe malformations as well.②Based on the imaging characteristics,this type of cochlear malformation,with only partial development of the basal turn(cochlea with less than 0.5 turns),was categorized as cochlear hypoplasia(CH).We provisionally designated it as cochlear hypoplasia type X(CH-X).Conclusion Cochlear hypoplasia(CH-X),characterized by cochlea with less than 0.5 turns,represents a severe type of inner ear malformation,accounting for approximately 2%of such deformities.Due to the malformed cochlea lacking a distinct bony cochlear nerve canal(BCNC)and cochlear nerve(CN),the potential benefits of cochlear implant surgery may be relatively limited.

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 

分 类 号：R764.35[医药卫生—耳鼻咽喉科]