Mondini畸形伴脑脊液耳漏的外科治疗

目的：探讨Mondini畸形伴脑脊液耳漏患者的外科治疗方法及预防措施。方法：对我院确诊为Mondini畸形伴脑脊液耳漏的15例患者采用鼓室探查并经卵圆窗、圆窗及其他漏孔进行填塞修补。结果：手术探查发现卵圆窗处有脑脊液漏出，圆窗及附近其他部位也有漏孔发生。15例患者中，10例经漏孔填塞修补1次治愈；5例经2次或3次填塞术后治愈。术后随访10个月～7年无脑脊液漏或脑膜炎复发。结论：经鼓室进路手术修补为该病的有效治疗方法，避免致畸因素的影响是预防本病发生的重要措施，佩带助听器是治疗手段之一，部分患者可考虑电子耳蜗植入。     

自发性脑脊液耳鼻漏伴内耳畸形的诊断及治疗

目的 探讨自发性脑脊液耳鼻漏伴内耳畸形患者的临床表现及外科治疗方法.方法 对我院10例自发性脑脊液耳鼻漏伴内耳畸形患者资料进行回顾.有两种手术方法:一种为后鼓室探查、镫骨底板切除后行前庭窗填塞术;另一种是经乳突面隐窝径路探查后鼓室、水平半规管开窗(迷路开窗)后行前庭池填塞术.结果 术前所有患者患耳听力完全丧失,10例均...     

内耳畸形伴脑脊液耳漏的高分辨率CT特征

目的:探讨先天性内耳畸形伴脑脊液耳漏的高分辨率CT(HRCT) 特征.方法:回顾性分析11例先天性内耳畸形伴脑脊液耳漏患者的HRCT表现.结果:共11例患者,耳蜗未发育4例,共腔畸形2例,耳蜗未未分隔型3例,耳蜗不全分隔型2例.内耳道底骨质缺损8例.所有患者患侧乳突气化和发育程度与对侧一致,前庭窗处都有软组织影.结论:内耳畸形伴脑脊液耳漏患者常存在内耳道底骨质缺损.前庭窗是脑脊液由内耳进入中耳腔的好发部位.HRCT能为先天性内耳畸形伴脑脊液耳漏患者的诊断及治疗提供可靠依据.     

先天性内耳畸形伴发脑脊液耳鼻漏

目的探讨先天性内耳畸形合并化脓性脑膜炎后出现脑脊液耳鼻漏的诊断、治疗方法。方法回顾性分析1例临床病例。患者脑膜炎痊愈后发现左侧鼻腔清亮液体流出,经检验为脑脊液,结合颞骨CT发现左内耳畸形,包括共同腔、半规管及内听道扩大三种畸形,确诊为先天性内耳畸形伴脑脊液耳鼻漏。于全麻下行鼓室探查,修补瘘口,脑脊液漏停止。结果患者术后恢复良好,随访半年无复发。结论对于仅有一次脑膜炎发作,伴单侧或双侧感音神经性聋的患者出现鼻腔清亮分泌物,需警惕先天性内耳畸形合并脑脊液鼻漏,一旦发现,需根据听力情况选择手术方法,尽早封闭瘘口。     

自发性脑脊液耳漏4例临床分析

目的探讨自发性脑脊液耳漏的临床特点及治疗。方法回顾性分析2015年7月—2016年12月手术治疗的4例自发性脑脊液耳漏患者的临床病例资料，4例患者均有细菌性脑膜炎病史，2例为内耳畸形（内耳不完全分隔Ⅰ型），漏点均位于镫骨足板；行耳后切口鼓室探查术，术中见镫骨足板缺损有清亮液体涌出，去除镫骨及砧骨，以颞肌筋膜、耳廓软骨及纤维蛋白胶封堵前庭窗。另2例漏点位于后颅窝硬脑膜，其中1例位于弓状隆起至总角水平，1例位于圆窗至外耳道下壁水平；此2例患者均行完壁式乳突开放术，显露后颅窝骨质缺损处，可见脑脊液流出，以颞肌筋膜、耳廓软骨、乳突皮质骨骨粉及纤维蛋白胶封堵缺损。结果4例患者术后随访23~39个月，脑脊液耳漏及脑膜炎均无复发。结论自发性脑脊液耳漏发病率低，容易漏诊、误诊，以致脑膜炎反复发作。听力下降伴脑膜炎的患者应高度怀疑自发性脑脊液耳漏的可能。     

Mondini内耳畸形的诊断与治疗

为总结内耳畸形的诊断与治疗特点，报告５例（６耳）Ｍｏｎｄｉｎｉ内耳畸形。４例（５耳）伴发脑脊液耳、鼻漏（其中３例伴发Ｋｌｉｐｐｅｌ－Ｆｅｉｌ综合症）经手术补漏成功，１例伴发耳硬化症。结合病理诊断及手术处理特点重点讨论：①诊断依据除听力检查外，特别强调ＣＴ为确诊提供的影像学资料；②Ｍｏｎ－ｄｉｎｉ内耳畸形所致外淋巴瘘主要在前庭窗或其附近，镫骨底板及圆窗部位；③自发性脑脊液耳、鼻漏处理可采取耳内切口的鼓室探查及瘘孔修补术，瘘孔周围粘膜应彻底搔刮，选用中胚叶组织填塞前庭窗，填塞物应呈嵌顿状。     

17例脑脊液耳漏的临床诊治分析

目的 本文旨在分析脑脊液耳漏的病因、临床表现、探讨其诊断及治疗方法。方法 回顾性分析自2012年以来南昌大学第一附属医院耳鼻咽喉头颈外科收治的17例脑脊液耳漏患者的临床资料,其中男10例,女7例;成人15例,儿童2例;年龄4~85岁;右耳9例,左耳8例。17例中自发性脑脊液耳漏4例,内耳畸形2例,有头部外伤史6例,有中耳乳突手术史3例,有头颈部恶性肿瘤放疗史2例。其中采用岩骨次全切除术者7例,鼓室探查+前庭封闭术者2例,乳突切开+脑脊液耳漏修补术者7例。结果 1例患者经保守治疗成功,其余16例均一次性手术修补成功;16例患者随访1年以上,1例患者随访3个月以上,均无复发;2例患者术后切口愈合不良,1例为耳道切口,另1例为耳后切口,均经过局部换药治愈。结论 手术是治疗脑脊液耳漏的主要方法。手术成功的关键首先在于准确定位瘘口的部位,其次正确处理封闭瘘口,术后抗炎,降低颅内压对瘘口的修复也十分重要。     

自发性脑脊液耳漏临床诊治分析

目的探讨自发性脑脊液耳漏的早期临床表现、诊断及手术方法。方法结合文献分析一例4岁儿童(病例1)和一例68岁成人(病例2)自发性脑脊液耳漏患者的临床表现、听力及影像学等检查结果、手术方法及预后。结果病例1表现为反复发作化脓性脑膜炎;影像学检查见中耳低密度影,未见明显骨质缺损;中耳积液检测符合脑脊液;该病例经耳道鼓室入路手术探查发现漏口位于镫骨底板,表现为裂隙样骨质缺损,采用哑铃状肌肉筋膜填塞后修补成功。病例2主要表现为耳闷和听力下降;影像学及中耳积液检查同病例1;经耳道鼓室联合乳突入路手术探查发现漏口位于鼓室天盖,通过多层填塞法修补成功。病例1随访1年、病例2随访6个月均无复发。结论自发性脑脊液耳漏在儿童和成人的临床表现差异大且特异性差,易误诊,可通过影像学及中耳积液的实验室检查辅助诊断;手术是最有效的治疗方法且效果可靠;需综合临床表现及影像学检查后选择合适手术入路。     

侧颅底术后持续性脑脊液耳漏的治疗与预防

目的 :总结侧颅底手术后持续性脑脊液耳漏的发病原因、手术处理和预防措施。方法 :根据原发病及原手术类型 ,本次手术采用不同的方法 :乳突入路探查 3例 ,耳后联合迷路入路及耳后联合颅中窝入路岩尖胆脂瘤切除并脑脊液漏修补术各 1例 ,乙状窦后入路探查 2例 ,颞部原切口入路肿瘤切除并脑脊液漏修补术 3例。结果 :外漏口位于乳突尖 3例 ,内听道 3例 ,颅中窝硬脑膜缺损表现为外耳道内口漏 3例 ,外耳道后壁肿瘤残留破坏 1例。 9例一次修补成功 ,1例行两次修补。全部病例均经手术治愈。结论 :颅底手术方式不同 ,术中预防和处理耳漏的侧重点应有不同。乙状窦后入路术中应注意保护乳突后及乳突尖气房 ,如有破损应及时封堵 ;迷路入路应处理好内听道、鼓室及咽鼓管黏膜 ,视情况刮除并封堵中鼓室 ;颞骨全切及次全切除术中应修补缺损的硬脑膜和缝合封堵咽鼓管咽口     

颅脑外伤后顽固性脑脊液耳漏的手术策略和技巧研究

目的 探讨颅脑外伤后顽固性脑脊液耳漏手术治疗的技巧和策略.方法 采用颞下中颅底-乳突后联合入路,对11例颅脑外伤后中颅窝底缺损所致脑脊液耳漏且保守治疗无效的患者施行手术修补.结果 ①无需行乳突探查5例.因乳突为粉碎性骨折,在取下骨瓣前先行钛板固定2例.另外4例乳突粉碎骨折严重,术中将事先收集的颅骨碎屑回植到乳突缺损内,取头最长肌加生物胶加固;②术中发现有颅底广泛骨质缺损6例,脑组织疝入缺损中或乳突黏膜暴露广泛;③修补探查过程发现听骨链完整者3例,缺失2例,部分听小骨(锤骨)缺失2例,术中未探查听骨链者4例.术后随访2~11年,无复发.结论 ①当合并复杂性骨折时,通过幕上幕下联合,经颞下中颅底-乳突后联合入路,无疑是明智的选择,尤其是不稳定骨折累及乳突的患者;②对颅底骨折复杂而听力完全丧失的脑脊液耳漏患者,除了封闭外耳道外,尚需进行鼓室及咽鼓管填塞封闭,以防止假鼻漏的情况发生;③颈内动脉岩段与鼓室及咽鼓管关系密切,脑脊液耳漏修补手术中应避免手术误伤或骨折片意外伤及颈内动脉.     

Ohrmißbildung als Ursache rezidivierender Meningitis

Summary 2 cases of spontaneous cerebrospinal otorrhea, causing recurrent meningitis, are reported. In both children with congenital deafness of the injured ear, nearly identical malformations of the middle and inner ear, were found. A hole in the footplate of the stapes was discovered, as a fistula abundant by discharging cerebrospinal fluid from the labyrinthine cavities. After stopping the leakage with fascia of the temporal muscle, and long-term application of antibiotics, both patients recovered.     

Use of porcine small intestinal submucosa for pediatric endoscopic tympanic membrane repair

Tympanic membrane perforationsoccur frequently in children, and can result in hearing loss, otorrhea, pain, and cholesteatoma. Due to the narrower ear canal in children, a postauricular incision is often needed to access the tympanic membrane for surgical repair. Endoscopic approaches are increasingly being used for tympanic membrane repair, reducing the need for postauricular incisions. As the need for a postauricular incision decreases, the demand for non-autologous grafting material has increased. Acellular porcine small intestinal submucosa (SIS) has been described in the literature as an alternative to commonly used autologous grafts, and is well suited for use with transcanal endoscopic ear surgery as a minimally invasive approach. This paper describes techniques for use of SIS in endoscopic tympanic membrane repair in children.     

Elimination of Temporal Bone Cerebrospinal Fluid Otorrhea Using Hydroxyapatite Cement

Objective This report introduces a new method to control cerebrospinal fluid (CSF) otorrhea using hydroxyapatite cement (HAC) via a transmastoid approach. This technique eliminates the need for a transmastoid or middle cranial fossa approach with soft tissue repair and prolonged hospitalization caused by lumbar drainage. Study Design Retrospective review. Methods Thirteen cases of transmastoid repairs of CSF otorrhea using HAC from August 1996 to February 1999 were reviewed. Results The CSF leak was controlled in every patient using HAC through a transmastoid approach. The reconstruction involved eight tegmen defects, three posterior fossa dural plate defects, and two congenital inner ear fistula secondary to Mondini malformation. Postoperative wound infection in one patient was the only complication that occurred. The average hospital stay was 48 hours. Follow‐up ranged from 12 to 44 months with no recurrence of CSF otorrhea. Conclusion The successful use of HAC to control CSF otorrhea through a transmastoid approach reduces patient morbidity by obviating the need for middle cranial fossa approaches, donor soft tissue sites, and spinal drainage.     

内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例,对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170,18.8%)明显高于其他文献报道;②32例内耳畸形中,大前庭导水管23例(占全部畸形数71.3%),大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例,并发外半规管未发育1例),Mondini畸形2例,Mondini畸形并发外半规管未发育前庭腔扩大1例,耳蜗CT影像疑似为“三叉”无法分类1例;③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例,及大前庭导水管并发Mondini畸形1例);④耳蜗影像疑似为“三叉”患者,术中发生严重井喷,电极植入困难,4个电极不能植入,术后听力未改善,半年后行对侧耳植入成功;⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作,术后1年行手术探查,后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括,大前庭导水管综合征及其相伴发或单发的各类内耳畸形;②内耳畸形非人工耳蜗植入术的绝对禁忌证,但术中严重井喷多见,电极植入不完全多见,术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗,术前详细的影像学检查可以对各类畸形进行详细分类,并在术前对手术难度有充分的准备,可以减少相关并发症的发生。     

自发性脑脊液耳漏的临床分析

目的 总结归纳并探讨自发性脑脊液耳漏(SCSFO)的临床特点、手术方式及术后疗效。方法 回顾性分析 2015—2020年收治的SCSFO患者的临床资料,总结病例的临床特点,分析归纳病例的漏口、手术修补方式、术后反应,并随访手术效果。结果 共收集10例SCSFO病例资料,其中男4例,女6例;儿童患者3例,成人患者7例。病史25 d至20余年,单侧发病9例,双侧发病1例。3例儿童患者均为先天性极重度感音神经性耳聋,均因发现鼻漏或脑膜炎就诊。7例成人患者中,因耳闷伴听力下降就诊5例,其中1例同时合并耳漏;因鼻漏就诊1例;因耳痛合并脑膜炎就诊1例。外院曾行鼓膜穿刺或置管史4例。3例儿童患者中,缺损部位均位于镫骨足板处和镫骨周围,其中2例患者伴镫骨畸形;7例成人患者中,2例漏口位于镫骨足板,3例漏口位于鼓室天盖,2例漏口位于乳突天盖。1例术后出现癫痫及颅内压升高。10例患者随访周期为5个月至 6年,均无再发脑脊液耳漏。结论 早期识别脑脊液耳漏的不典型症状,掌握确诊脑脊液耳漏的定位、定性诊断方法非常重要。SCSFO保守治疗效果差,一旦确诊,建议积极手术探查,精准寻找漏点,确切修补漏口是关键。     

Recurrent meningitis secondary to idiopathic oval window csf leak

Bacterial meningitis remains a life-threatening infection even in the present antibiotic era; thus, any abnormality which predisposes a patient to a recurrence of this serious disease, must be identified and corrected. This report describes the history of a 12-year-old boy with a profound neurosensory hearing loss, a related absence of vestibular function and a Monclini-type of temporal bone dysplasia who developed recurrent episodes of meningitis which were due to an idiopathic cerebrospinal fluid otorrhea. Even though the meningitis was labyrinthogenic in origin, the patient did not experience the associated symptoms of hearing loss and/or vertigo since the affected inner ear was clinically unreactive. By surgically exploring the middle ear, the presence of a cerebrospinal fluid otorrhea was confirmed. The leak was observed to be coming from a defect in the stapes footplate, and it was controlled by firmly packing the inner ear vestibule with muscle. A remarkable similarity exists between the patient described above and the 15 previously reported cases of meningitis due to a spontaneous cerebrospinal fluid otorrhea. Generally, the problem occurred in young children, the average age being 6.4 years; male and female were equally afflicted. All 15 previously reported cases had a severe neurosensory hearing loss which was unilateral in 10 individuals and bilateral in the other five. In 11 of the case reports, the vestibular function was evaluated, and the labyrinth was noted to be unreactive in the affected ear. An associated congenital abnormality of the inner ear was described in 11 of the patients reviewed. Anatomically, in 13 cases, the leak was observed to be coming from the oval window area. Other affected sites included one report of a fissure of the promontory and one report of a defect in the roof of the eustachian tube. Multiple surgical procedures were required in 11 of the 15 patients in order to identify the exact source of the otorrhea and to seal it permanently. In three cases, the successful procedure was a middle ear exploration with stapedectomy and packing of the inner ear vestibule. Overall, a total of 36 operations was performed in the 15 patients reviewed. In conclusion, when the physician is confronted by a case of meningitis in a patient with a unilateral or bilateral total loss of hearing and vestibular function, the possible presence of an idiopathic cerebrospinal fluid leak should be considered, especially if radiographic studies demonstrate a temporal bone dysplasia. In these selected cases, if the etiology of the meningitis is obscure, a middle ear exploration should be performed both for diagnostic purposes as a means to ascertain definitely the presence of a leak and for therapeutic purposes to seal it effectively.     

内耳畸形与耳蜗骨化患者的耳蜗植入

目的探讨先天性内耳畸形与耳蜗骨化患者耳蜗植入术的有关问题.方法对我院2002年10月～2004年2月间行耳蜗植入术的8例内耳畸形和2例耳蜗骨化的患者进行回顾性分析.结果4例大前庭水管综合征患者术中有外淋巴液搏动;3例Mondini畸形和1例共同腔畸形患者术中出现井喷;1例耳蜗部分骨化患者术中误将电极插入内听道后纠正;另一例耳蜗部分骨化患者植人短电极.1例Mondini畸形患者术后发生少量脑脊液耳鼻漏,保守治疗3月后痊愈,其余患者无并发症.所有患者均成功开机.结论对于内耳畸形或耳蜗骨化的患者,耳蜗植入术前详细的影像学评估,对术中困难的充分估计和正确、规范处理是手术成功的基本条件.