内耳畸形相关性镫骨底板瘘的影像学表现  

The imaging appearances of stapical footplate fistula related to inner ear malformation

作　　者：王林省 张丽红 胡娜 刘善凤 李进叶 魏平 孙立新 巩若箴 Wang Linsheng;Zhang Lihong;Hu Na;Liu Shanfeng;Li Jinye;Wei Ping;Sun Lixin;Gong Ruozhen(Department of Medical Imaging Center,Shandong Second Provincial General Hospital,Jinan 250022,China;Department of Medical Imaging Center,Central Hospital Affiliated to Shandong First Medical University,Jinan 250013,China)

机构地区：[1]山东省第二人民医院影像中心,济南250022 [2]山东第一医科大学附属中心医院影像中心,济南250013

出　　处：《中华耳鼻咽喉头颈外科杂志》2024年第8期803-811,共9页Chinese Journal of Otorhinolaryngology Head and Neck Surgery

摘　　要：目的总结内耳畸形相关性镫骨底板瘘(stapical footplate fistula related to inner ear malformation,SFF-Re-IEM)的CT和MRI表现特征。方法回顾性分析山东省第二人民医院2014年8月至2023年10月期间48例(53耳)SFF-Re-IEM患者的CT和MRI资料,其中男35例、女13例,年龄1~55岁;53耳中有25耳经手术证实。记录患耳伴发内耳畸形的类型、内听道发育情况、镫骨底板及鼓岬完整性、鼓室内积液的密度、信号特征等。结果53耳SFF-Re-IEM中伴发内耳畸形不全分隔Ⅰ型17耳(32.1%,17/53)、共同腔畸形13耳(24.5%,13/53)、耳蜗未发育13耳(24.5%,13/53)、耳蜗发育不良7耳(13.2%,7/53)、Mondini畸形3耳(5.7%,3/53)。94.3%(50/53)患耳伴发内听道底部发育不良或缺损。根据镫骨底板骨质完整性及伴发脑脊液耳漏情况分为4类:镫骨底板骨质缺损、镫骨底板疝、镫骨底板漏和镫骨底板旁漏。镫骨底板漏22耳,其中2耳可能由镫骨底板骨质缺损发展而来,6耳由镫骨底板疝发展而来;镫骨底板旁漏1耳;孤立性镫骨底板疝30耳。2耳镫骨底板骨质缺损患耳缺损区在CT和MRI上不能显示。36耳镫骨底板疝在CT上表现为镫骨底板局部骨质缺损、缺损区见半球形软组织密度影,其在MRI水成像序列上呈脑脊液样信号影,外缘光整,鼓室内无积液信号。22耳镫骨底板漏中,患侧镫骨底板除在CT上显示局限性骨质缺损外,患侧鼓室内显示多少不一脑脊液样密度或信号影;17耳大量漏出患耳显示鼓室内高信号经缺损区与畸形内耳内高信号相连续,5耳少量脑脊液漏出患耳表现为患侧足弓间隙内囊状脑脊液信号影,与鼓室内少量积液不连续。1耳镫骨底板旁漏表现患侧镫骨底板骨质完整,其后下方鼓岬骨质局限性缺损,鼓室内脑脊液样信号通过缺损区与内耳内高信号相连续。结论SFF-Re-IEM因类型不同其影像学表现具有多样性,了解其影像学表现特征,有助于其诊断。ObjectiveTo summarize the HRCT and MRI appearances of stapical footplate fistula related to inner ear malformation(SFF-Re-IEM).MethodsThe HRCT and MRI materials of 48 cases(53 ears)SFF-Re-IEM were retrospectively analyzed.Among them,25 SFF-Re-IEM ears were confirmed by surgery.Their CT and MRI findings including associated IEM type,internal auditory canal(IAC)malformation,tympanic fluid,its density and signal features,and accompanied labyrinthitis were recorded.ResultsAmong 48 cases(53 ears)with SFF-Re-IEM,17 ears with incomplete partition typeⅠ,accounting for 32.1%,13 ears with common cavity for 24.5%,13 ears with cochlear aplasia for 24.5%,7 ears with cochlear dysplasiaⅡfor 13.2%,and 3 ears with Mondini for 5.7%,were found respectively.94.3%of them were associated with a defect or dysplasia in the found of the IAC.They were divided into 4 types according to the intact of the stapical footplate and accompanied CSF otorrhea:22 ears were diagnosed as the stapical footplate leaking,of them,2 ears might come from the stapical footplate bony defect,6 ears were from the stapical footplate hernia.1 ear belonged to the peristapical footplate leaking.30 ears with the isolated the stapical footplate hernia were another found.The bony defect in 2 ears with the stapical footplate bony defect were not presented on CT and MRI.The focal bony defect of the affected stapical footplate of 36 ears with the stapical footplate hernia were demonstrated,which presented the hemispherical protruding into the tympana,the soft-tissue density on CT,and CSF-like signal on the MR heaved-T2WI images.Among 22 ears with the stapical footplate leaking,their imaging appearances varied from the different amount of the leaking CSF.Besides the focal bony defects of the affected stapical footplates,there were much more CSF-like density or signal in the ipsilateral tympanic cavity in 17 affected ears connecting with the vestibule through the defect area.In the CSF leaking ears with less CSF leaking in 5 ears,the CSF-like cysts like SFH were shown

关 键 词：镫骨底板瘘 内耳 畸形 体层摄影术 X线计算机 磁共振成像 

分 类 号：R764.3[医药卫生—耳鼻咽喉科]