经外耳道水下耳内镜修复先天性内耳畸形伴脑脊液耳漏1例并文献复习

目的探讨先天性内耳畸形并脑脊液耳漏患者的外科手术治疗方法。方法对1例先天性内耳畸形并伴有脑脊液耳漏患者采用经外耳道径路常规耳内镜鼓室探查再改用水下耳内镜行脑脊液耳漏填塞修补术。结果手术探查见镫骨底板有缺失漏口,用自体脂肪和颞肌及肌筋膜填塞内耳,封闭前庭窗,1次手术修补成功。术后随访5个月未见有脑脊液耳漏及脑膜炎复发。结论采用水下耳内镜修复内耳畸形伴脑脊液耳漏是有优势的手术方式。     

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。     

不完全分隔Ⅰ型内耳畸形并脑脊液耳鼻漏的诊治体会北大核心CSCD

目的探讨不完全分隔I型(incomplete partition type Ⅰ,IP-Ⅰ)内耳畸形并脑脊液耳鼻漏的临床表现、诊断及手术方法。方法结合3例IP-Ⅰ型内耳畸形并脑脊液耳鼻漏患者的临床资料,分析其表现及颞骨高分辨率CT(HRCT)特点,3例手术均经前庭窗入路手术,术中用约2倍前庭池直径的颞肌填塞前庭池修补脑脊液漏口。结果 3例患者均表现为患耳极重度感音神经性聋,反复发作脑膜炎,2例间断流清水样涕;3例HRCT均显示右侧内耳畸形(符合IP-Ⅰ型),术中见第1例和第3例镫骨底板边缘呈半月形缺损,第2例镫骨底板呈中央穿孔,均一次手术修补成功,随访1年以上未见复发。结论 IP-Ⅰ型内耳畸形并脑脊液耳鼻漏者可表现为患耳听力下降,间断流清涕,反复发作的化脓性脑膜炎,纯音听阈及HRCT有助于诊断;可通过手术经前庭窗入路用颞肌填塞前庭池治疗脑脊液耳鼻漏。     

Mondini内耳畸形合并反复化脓性脑膜炎5例报告

目的探讨Mondini内耳畸形合并脑脊液耳漏、耳鼻漏同时并发脑膜炎时的诊断、治疗方法。方法早期2例,在行中耳探查时发现瘘孔在畸形的镫骨足板,采用肌肉、筋膜等材料从中耳腔侧覆盖前庭窗瘘孔。后期3例则在术中摘除镫骨,用筋膜等组织由前庭窗向前庭池填塞。结果采用从中耳腔侧覆盖瘘孔的早期2例术后失败,改用前庭池填塞后未再复发。后期由前庭窗向前庭池作填塞的3例皆一次手术成功。结论Mondini内耳畸形合并脑脊液耳漏、耳鼻漏时需及时行中耳探查术、封闭瘘管。术中摘除畸形镫骨,以筋膜等材料从前庭窗向前庭池填塞则术后不易复发。     

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...     

伴内耳畸形的人工耳蜗植入术后自发性脑脊液耳漏的治疗北大核心CSCD

先天性内耳畸形患者可伴有自发性脑脊液耳漏,反复脑膜炎发作。对于已行人工耳蜗植入的迟发性脑脊液耳漏患儿,如何既保留耳蜗电极,又修复脑脊液耳漏,是耳科医师面临的难题。本文报告一例伴内耳畸形的人工耳蜗植入术后自发性脑脊液耳漏的患者,在保留电极的前提下经水平半规管开窗后前庭池填塞术修补脑脊液耳漏,术后随访1年,未见脑膜炎及脑脊液耳漏复发。     

镫骨手术并发镫井喷3例报告

目的报告镫骨手术中并发镫井喷3例及其处理方法。方法回顾性分析镫骨手术中发生镫井喷3例患者的处理经过。首先吸除术野中脑脊液至不再有液体流出，然后取填塞物进行填塞，可用填塞物包括动物胶、骨腊、筋膜、肌肉、静脉瓣等。结果经及时处理，3例患者均止住了脑脊液溢出。术后随访2年，2例未再发生继发性脑膜炎，其中1例眩晕和耳呜均消失。结论对准备行镫骨手术或内耳开放的患者，术前必须作高分辨CT，确定有无内耳畸形，并仔细询问家族史。     

用耳神经外科联合径路治疗慢性耳病及脑脊液耳漏

作者报告三例。第1例为51岁男性患者,因蛛网膜囊肿引起脑膜炎、囊肿经硬膜及岩骨进入中耳,使用耳神经外科联合径路才最终确定了病变的性质,切除病变并修补硬脑膜,术后随访10个月良好。第2例为33岁女患者,25年前因颞叶脓肿经乳突凿开引流,以后发生三次脑膜炎,一次自发性脑脊液耳漏,一次耳后脓肿,因持续性耳漏,做过三次乳突清创术均未能控制,这次因脑膜炎及第二次脑脓肿做颞顶部开颅、切除了颞叶巨大     

内耳畸形中脑脊液搏动致镫骨底板瘘

脑脊液（ＣＳＦ）耳漏起因多见于颞骨骨折、慢性破坏性耳部疾病、经乳突颅内手术、先天性内耳畸形。前三者诊断较易，而内耳畸形中脑膜炎常是骨折缺损的首发病症。早在１７９１年Ｃａｒｌｏｍｏｎｄｉｎｉ描述了Ｍｏｎｄｉｎｉ畸形的特征为耳聋，耳蜗仅有１．５转，前庭及前庭导水管膨大。该文报告１例双侧Ｍｏｎｄｉｎｉ伴镫骨畸形致单侧ＣＳＦ耳漏的病例。患者女性，１０个月，出生后二度发生脑膜炎，双中耳腔已置通气管，有耳聋家族史。耳镜检查见左耳通气管内有大量清水样液溢出（液体性质与ＣＳＦ一致），右耳除通气管外余正常。听力测…     

改良乳突根治结合鼓室成形的结果

本文报告266个流脓中耳做了改良乳突根治并结合鼓室成形术的结果,其中54耳松弛部穿孔。术中发现听骨链完整者54例、砧骨豆状突缺损18例、砧骨长脚缺损134例、砧骨完全缺损47例、镫骨部分缺损14例、镫骨完全缺损87例、锤骨部分缺损65例、锤骨完全缺损19例。术中107例保留鼓环,大多数都比较狭窄。鼓室肉芽及鼓岬粘膜肉芽均去除,鼓岬粘膜缺损多者可在镫骨底板两边放上硅胶片,鼓膜大穿孔和完全穿孔者用颞筋膜修补并越过桥部贴到上鼓室上壁。如桥部去除,上鼓室可用明胶海绵填充,颞筋膜也同样贴在上鼓室上壁,乳突腔用上方带     

Spontaneous cerebrospinal fluid otorrhea in a normal inner ear. A case report]

A 49-year-old male with spontaneous cerebrospinal fluid (CSF) otorrhea, an extremely rare condition, is presented as the third reported case in Japan. The patient visited us complaining of sudden hearing loss and fullness of the ear on the left side. A tegmen defect of the posterior cranial fossa and CSF leakage from the fistula into the mastoid air cells, but a normal inner ear, were found by means of metrizamide CT. The fistula was surgically occluded with fascia temporalis and an artificial bone with fibrin glue. The CSF otorrhea from the tegmen defect was successfully stopped by this procedure and no recurrence has been observed. We discuss this extremely rare disease in this paper.     

Hearing preservation in perilymphatic fistula due to a congenital fistula in an adult

Congenital fistula in the stapedial footplate usually can be diagnosed by the recurrence of meningitis caused by spontaneous cerebrospinal fluid (CSF) in infants or young children. We report the case of a 65-year-old woman, who was initially diagnosed as having an acquired perilymphatic fistula caused by aural barotrauma and demonstrated episodic vertigo and fluctuant sensorineural hearing loss in the right ear after air travel. Surgical exploration showed a congenital circular defect in the peripheral part of the stapedial footplate with leakage of CSF. The fistula was closed by inserting a tiny piece of fascia attached to both the tympanic and perilymphatic side of the stapedial footplate utilizing the back-pressure of perilymphatic fluid and fibrin glue; hearing was preserved.     

Diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea

OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.     

Diagnosis and management of spontaneous cerebrospinal fluid otorrhea.

OBJECTIVE: To describe the clinical presentation, diagnosis, surgical management, and outcome of patients with spontaneous cerebrospinal fluid otorrhea. STUDY DESIGN: Retrospective case series. SETTING: Tertiary referral center. METHODS: The authors conducted a chart review of all previously unreported cases of surgically confirmed cerebrospinal fluid otorrhea at their institution between September 1996 and February 2005. Acquired cases were excluded from this study. Eleven cases of spontaneous cerebrospinal fluid otorrhea were identified among 10 patients. RESULTS: Nine of the 10 patients presenting with spontaneous cerebrospinal fluid otorrhea were women. Ages ranged from 34 to 79 years. Eight patients presented with serous otitis media, and two women presented with meningitis. High-resolution computed tomography demonstrated a tegmen defect with a sensitivity of 80%. Nine tegmen defects were repaired using a transmastoid approach without recurrence. One patient with a contracted mastoid and a meningoencephalocele herniating from the tegmen tympani into the attic required the temporal craniotomy approach for definitive repair. Another patient with a tegmen tympani defect developed a recurrence of cerebrospinal fluid otorrhea 8 years after a transmastoid repair using only fascia and fibrin glue. A recurrent tegmen defect in this patient was repaired using a transmastoid approach and a multilayered closure technique. CONCLUSION: The diagnosis of spontaneous cerebrospinal fluid otorrhea requires clinical suspicion in the setting of persistent serous otitis media. High-resolution computed tomography can confirm the diagnosis. The authors' findings indicate that repair through a transmastoid approach is effective if the tegmen defect can be widely visualized. The authors advocate a multilayered closure technique.     

Recurrent meningitis due to spontaneous cerebrospinal fluid otorrhea. A case report

Bilateral congenital deafness was observed in a 9-year-old girl with recurrent meningitis. A careful workup, including polytomograph, CT scanning and Radioisotope (RI)-cisternography revealed an inner ear dysplasia of the Mondini type, viz. dilation of the lateral semicircular canal, vestibule and cystic degeneration of cochlea on both ears. RI-cisternography demonstrated an abnormal accumulation of radioisotope in the left mastoid region, suggesting spontaneous cerebrospinal fluid (CSF) otorrhea. At exploratory tympanotomy of the left ear, the CSF leak was found from a round defect in the center of the stapedial foot plate. The CSF leak was treated successfully by packing the vestibule with temporal fascia. Spontaneous CSF otorrhea is seldom the cause of meningitis but has to be considered especially if associated with a dead ear and with recurrent meningitis. The diagnosis and management are discussed.     

Spontaneous cerebrospinal fluid leakage and middle ear encephalocele in seven patients

Isolated cases of spontaneous cerebrospinal fluid (CSF) leakage with and without middle ear encephalocele have been reported. These leaks are usually accompanied by episodes of recurrent meningitis, hearing loss, or chronic headache. In this article, we report seven new cases of spontaneous CSF leakage. Six of these patients had conductive hearing loss and serous otitis media, and three had recurrent meningitis. Prior to a definitive diagnosis, six patients had received myringotomy tubes, which produced profuse clear otorrhea. Three patients had positive beta-2 transferrin assays. Computed tomography and magnetic resonance imaging confirmed a defect in the temporal bone tegmen. A combined transmastoid and middle fossa surgical approach with a three-layer closure was used to repair the tegmen defect. All patients had a lumbar drain placed prior to surgery. In addition to describing the seven new cases, we review the history of CSF leakage and discuss diagnostic methods, surgical findings, and our recommendations for management.     

Middle cranial fossa approach for the repair of spontaneous cerebrospinal fluid otorrhoea using autologous bone pate

Tegmen plate defects causing cerebrospinal fluid (CSF) leaks and brain hernias have conventionally been repaired using soft tissue grafts by a transmastoid approach. A review of the literature reveals that the results of transmastoid repairs have been less than satisfactory. We present here four patients with spontaneous CSF otorrhoea who had tegmen defects repaired using the middle cranial fossa approach. An autologous bone pate slab mixed with fibrin glue with additional temporalis fascia reinforcement was used for the repairs. All four patients had uneventful postoperative periods and have had no subsequent CSF leak (follow-up range: 1-3 years). We believe that the middle cranial fossa approach is more effective than the transmastoid approach for the repair of CSF leaks and brain hernias through tegmen plate defects. An important additional benefit is hearing preservation. We also recommend the use of bone pate along with soft tissue for the repair in order to achieve a secure seal. Bone pate can effectively seal multiple defects in the tegmen plate without any risk of migration.     

Association of spontaneous anterior fossa CSF rhinorrhea and congenital perilymphatic fistula in a patient with recurrent meningitis

A case of recurrent meningitis associated with spontaneous cerebrospinal fluid (CSF) rhinorrhea and left, sensorineural hearing loss in a 4-year-old boy was found to be due to simultaneous congenital defects. High resolution CT examination clearly showed an anterior fossa defect and an inner ear malformation, including demineralization in the region of the footplate of the stapes, and this provide clear guidance for the surgeon.     

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.