儿童先天性内耳畸形伴再发性脑膜炎26例临床分析  

Congenital Inner Ear Deformity with Recurrent Meningitis in Children:An Analysis of 26 Cases

作　　者：雷雳[1] 李永新[1] 赵守琴[1] 梁凤和[1] 郑军[1] 韩德民[1] 杨本涛[2] 闫钟钰[2] LEI Li;LI Yongxin;ZHAO Shouqin;LIANG Fenghe;ZHENG Jun;HAN Demin;YANG Bentao;YAN Zhongyu(Department of Otolaryngology Head and Neck Surgery,Beijing Tongren Hospital,Capital Medical University,Beijing 100730;Department of Radiology,Beijing Tongren Hospital,Capital Medical University,Beijing 100730)

机构地区：[1]首都医科大学附属北京同仁医院耳鼻咽喉头颈外科,北京100730 [2]首都医科大学附属北京同仁医院放射科,北京100730

出　　处：《中华耳科学杂志》2020年第2期325-328,共4页Chinese Journal of Otology

摘　　要：目的探讨儿童先天性内耳畸形伴频发脑膜炎的临床特征和治疗策略,提高诊疗水平。方法回顾性分析2008年12月至2018年7月因频发脑膜炎,颞骨CT证实内耳发育畸形,在我院接受手术治疗的26例临床资料。结果26例患者颞骨CT均存在内耳发育畸形,参照Sennaroglu分类,耳蜗未发育3例,共同腔畸形6例,耳蜗发育不良Ⅰ型1例,不完全分隔Ⅰ型4例,不完全分隔Ⅱ型12例。其中21例伴有内听道底骨质缺损,5例面神经发育畸形。脑膜炎病程1月至10年不等,脑膜炎发作至少2次。经外耳道前庭窗径路治疗14例,经乳突外半规管开窗前庭池径路治疗12例。术后随访半年至8年,除1例外耳道前庭窗径路治疗失败,再次经乳突外半规管开窗前庭池径路手术治愈,其它患者均1次手术成功,术后无脑膜炎再发。结论26例再发性脑膜炎,首次发作后1月至10年分别确诊为内耳发育畸形。为了避免漏诊和延误治疗,建议对伴有听力损伤的再发性脑膜炎患儿行颞骨CT检查。内耳畸形伴再发性脑膜炎可经外耳道前庭窗径路手术,对存在面神经畸形、初次手术失败和术中出现井喷的患者,建议选择经乳突外半规管开窗前庭池径路手术。Objective To report clinical features and treatment strategies in children with congenital inner ear malformation with recurrent meningitis.Methods Clinical data of patients who underwent surgery for inner ear malformation associated with recurrent meningitis at Beijing Tongren Hospital between December 2008 and July 2018 were retrospectively reviewed.Results Inner ear development deformities were diagnosed through temporal bone compute tomography(CT)scan in 26 patients,including cochlear aplasia(n=3),common cavity malformation(n=6),cochlear hypoplasia typeⅠ(n=1),incomplete partition typeⅠ(n=4)and incomplete partition typeⅡ(n=12)based on the Sennaroglu classification.Bony defect at fundus of the internal auditory canal was found in 21 cases and facial nerve deformity in 5 cases,respectively.Meningitis had occurred at least twice in these patients over the course of 1 month to 10 years.Surgical treatment included vestibule window packing through a transcanal approach(n=14)and labyrinthotomy and vestibular obliteration(n=12).Patients were followed for 6 months to 8 years.Transcanal vestibular window packing failed in 1 case,which was rescued by subsequent labyrinthotomy and vestibular pool obliteration with good result.Initial surgical treatment was successful with no recurrence of meningitis during following up in the remaining 25 patients.Conclusion Temporal bone CT scan should be performed for those pediatric patients suffering from recurrent meningitis with hearing impairment to avoid missed diagnosis and delay in treatment.We recommend transcanal vestibule window packing in patients with inner ear malformation for cerebrospinal fluid otorrhea,and labyrinthotomy and vestibular obliteration for patients with facial nerve deformity,intraoperative gusher,or when the initial operation has failed.

关 键 词：内耳畸形 再发性 脑膜炎 外科手术 

分 类 号：R764[医药卫生—耳鼻咽喉科]