Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases
Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases
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先天性内耳发育不良继发脑脊液耳漏18例诊治
DOI：
10.1631/jzus.b1800224
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发表时间：
2019-02-01
影响因子：
5.1
通讯作者：
Chen, Bing
中科院分区：
文献类型：
--
作者：
Wang, Bing;Dai, Wen-jia;Chen, Bing
目的探讨先天性内耳发育不良继发耳源性脑脊液漏的临床表现、诊断、手术方法及预后特点。回顾分析2007~2017年间18例内耳发育不良继发耳源性脑脊液漏患者的临床资料，术后随访4个月以上。平均随访时间为三年。本文介绍了所有患者的临床表现特点，如自述症状、影像表现、手术途径和修复方法、术中渗漏部位、术后病程，包括手术成功率。主要表现为脑膜炎、重度听力障碍、脑脊液耳漏或鼻漏等典型症状。所有18名患者都至少有一次脑膜炎发作并伴有严重听力障碍。17例术前听力图表现为重度感音神经性聋，1例为传导性聋。12名患者以耳漏为首发症状，2名患者伴有鼻漏。6名患者主诉鼻漏，其中两名患者被误诊为脑脊液鼻漏，并在另一家医院接受了鼻内窥镜检查。高分辨率计算机断层扫描(HRCT)图像可以显示内耳的发展，如前庭囊肿扩张，半规管或耳蜗结构不清，或中耳或乳突渗出的迹象，这强烈提示脑脊液耳漏的可能性。研究中的儿童比成年人患有更严重的发育不良。所有18名患者在手术中都发现了脑脊液漏。最常见的缺损点是距骨基板(55.6%)，38.9%的患者在椭圆形窗周围有一处渗漏。1例患者术后脑脊液耳漏复发，经二次修补后未复发。先天性内耳发育不良所致的脑脊液耳漏在儿童中比成人更严重。最常见的症状是脑膜炎、听力障碍和脑脊液耳漏或鼻漏。HRCT对本病具有较高的诊断准确率。最常见的瘘口位于卵圆形窗周围，包括距骨踏板和环状韧带。
To describe the characteristics of the clinical presentation, diagnosis, surgical methods, and outcomes of patients with otogenic cerebrospinal fluid (CSF) leakage secondary to congenital inner ear dysplasia. A retrospective review was performed of 18 patients with otogenic CSF leakage secondary to inner ear dysplasia who underwent surgery in our group from 2007 to 2017 and had a follow-up of at least 4 months. The average length of follow-up was three years. The characteristics of the clinical presentations of all patients, such as self-reported symptoms, radiographic findings, surgical approaches and methods of repair, position of the leakage during surgery, and postoperative course, including the success rate of surgery, are presented. The patients presented mostly with typical symptoms of meningitis, severe hearing impairment, and CSF otorrhea or rhinorrhea. All 18 patients had at least one previous episode of meningitis accompanied by a severe hearing impairment. The preoperative audiograms of 17 patients showed profound sensorineural hearing loss, and one patient had conductive hearing loss. Twelve patients presented with an initial onset of otorrhea, and two had accompanying rhinorrhea. Six patients complained of rhinorrhea, two of whom were misdiagnosed with CSF rhinorrhea and underwent transnasal endoscopy at another hospital. High-resolution computed tomography (HRCT) images can reveal developments in the inner ear, such as expansion of a vestibular cyst, unclear structure of the semicircular canal or cochlea, or signs of effusion in the middle ear or mastoid, which strongly suggest the possibility of CSF otorrhea. The children in the study suffered more severe dysplasia than adults. All 18 patients had CSF leakage identified during surgery. The most common defect sites were in the stapes footplates (55.6%), and 38.9% of patients had a leak around the oval window. One patient had a return of CSF otorrhea during the postoperative period, which did not re-occur following a second repair. CSF otorrhea due to congenital inner ear dysplasia is more severe in children than in adults. The most common symptoms were meningitis, hearing impairment, and CSF otorrhea or rhinorrhea. HRCT has high diagnostic accuracy for this disease. The most common fistula site was around the oval window, including the stapes footplates and the annular ligament.