内耳畸形相关性脑脊液耳漏的临床和影像学表现  

The clinical and imaging appearance of cerebrospinal fluid otorrhea related to inner ear malformation

作　　者：郝淑珍 王林省 HAO Shuzhen;WANG Linsheng(Clinical College,Jining Medical University,Jining 272067,China;Department of Radiology,The People’s Hospital of Zoucheng,Jining 273599,China;Department of Medical Imaging Center,Shandong Second Provincial General Hospital(Shandong Provincial ENT Hospital),Jinan 250023,China;Department of Medical Imaging Center,The Affiliated Hospital of Jining Medical University,Jining 272029,China)

机构地区：[1]济宁医学院临床学院,山东济宁272067 [2]山东省邹城市人民医院放射科,山东济宁273599 [3]山东省第二人民医院(山东省耳鼻喉医院)影像中心,山东济南250023 [4]济宁医学院附属医院影像中心,山东济宁272029

出　　处：《医学影像学杂志》2023年第10期1760-1764,共5页Journal of Medical Imaging

基　　金：山东省医药卫生科技发展计划项目(编号202109010701);山东省济宁市重点研发项目(编号2020YXSN016)。

摘　　要：目的探讨总结内耳畸形相关性脑脊液耳漏(CFO-Re-IEM)的临床和影像学表现特征。方法选取经手术证实CFO-Re-IEM 20例(21耳)患者的临床、HRCT和MRI资料。结果CFO-Re-IEM患耳表现为中度至极重度耳聋;伴有头痛和/或发热9例,脑脊液耳漏或鼻漏6例,听力下降4例,反复肺炎就诊1例。20例(21耳)CFO-Re-IEM伴发内耳畸形(IEM),不全分隔I型10耳、共同腔6耳、耳蜗未发育1耳、耳蜗发育不良-II型3耳、Mondini畸形1耳。在HRCT表现患耳均有患侧镫骨底板骨质缺损,其中1耳伴有鼓室盖骨质缺损。16耳大量脑脊液漏,患耳除表现镫骨底板骨质缺损外,患侧鼓室内在MR水成像呈近似脑脊液样信号,均经缺损区与内耳淋巴液相连续。5耳脑脊液少量漏出患耳在MR水成像鼓室内信号不均,其镫骨底板骨质缺损处可见半球形脑脊液样高信号影,其外缘不规则,与鼓室内高信号不连续。结论CFO-Re-IEM患者临床表现多为重度耳聋、脑膜炎及脑脊液耳鼻漏等,影像学以患耳伴发严重IEM、镫骨底板骨质缺损及鼓室积液为特征。Objective To summarize the clinical and imaging presentations of the cerebrospinal fluid otorrhea related to inner ear malformation(CFO-Re-IEM).Methods The clinical and imaging materials of 20 patients(21 ears)with CFO-Re-IEM confirmed by surgery were retrospectively analyzed.Results All of the CFO-Re-IEM ears presented hearing loss ranging from mederate to profound.Besides this,headache and or fever in 9 cases,cerebrospinal fluid(CSF)otorrhea or rhinorrhea in 6 cases,hearing loss in 4 cases,and recurrent pneumona in one case were found.Among 20 cases(21 ears)with CFO-Re-IEM,10 ears with incomplete Partition type I,6 ears with common cavity,1 ears with cochlear aplasia,3 ears with cochlear dysplasia II,and 1 ears with Mondini malfomation were found,respectively.The stapes floor(SF)defects were presented in all affected ears on HRCT.There were lots of CSF-like signal intensity in the tympanic cavity connecting with the vestibule through the bony defect of the SF in 16 affected ears on MRI.For the 5 affected ears with a small amount of leaking,the hemispheric,CSF-like structures were shown at the SF defect on MRI,their outer margins were irregular,which did not connect with the heterogeneous signal in the tympanic cavity.Conclusion The CFO-Re-IEM patients usually present with severe hearing loss,meningitis,and cerebrospinal fluid otorhinorrhea in clinical practice,and its imaging features include severe IEM,bone defects in the SF,and tympanic effusion on the affected side.

关 键 词：脑脊液耳漏 内耳畸形 体层摄影术 X线计算机 磁共振成像 

分 类 号：R764.33[医药卫生—耳鼻咽喉科] R445[医药卫生—临床医学]