Mondini畸形伴脑脊液耳鼻漏的外科处理

目的：探讨Mondini畸形伴脑脊液耳鼻漏的临床表现及外科手术方法。方法：对3例Mondini型内耳畸形伴自发性脑脊液耳鼻漏患儿，均采用经卵圆窗内耳填塞方法修补。结果：3例皆一次手术修补成功，随访半年以上无脑脊液漏或脑膜炎复发。结论：幼儿自发性脑脊液耳鼻漏应警惕Mondini型内耳畸形，颞骨薄层CT扫描可明确诊断，经鼓室进路手术修补为有效方法。     

耳内镜下修补Mondini型内耳畸形伴脑脊液耳鼻漏1例

患儿，男，11岁。自幼右耳听力极差，5岁时无明显诱因出现右耳流清水样液1次，后右鼻反复流出清水，出现咳嗽、头痛及发热等症状。每年均有7～8次，确诊为“化脓性脑膜炎”于外院住院治疗。于2005年5月来我院就诊，检查：神清，发育正常，头低位时可见右鼻流出清水，经化验糖定量检查与脑脊液相符。头颅、五官无畸形，神经系统检查阴性。双耳廓正常，外耳道通畅，鼓膜完整，右侧鼓膜浑浊、稍外隆。鼓室压图左侧正常，右侧B型。ABR检查示：左耳正常，右耳120dB peSPL。颞骨CT示：左中耳、内耳结构正常，右鼓室及乳突积液，听小骨完整，天盖完整；前庭、水平半规管扩张，耳蜗形态不正常，前庭及耳蜗与内听道之间骨性分隔缺失（图1）。诊断：右侧先天性内耳畸形Mondini型伴脑脊液耳鼻漏。     

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。     

经水平半规管前庭入路治疗Mondini畸形伴脑脊液耳鼻漏的临床探讨

目的:探讨Mondini畸形伴脑脊液耳鼻漏的临床表现及经水平半规管前庭入路修补治疗的可行性。方法:回顾性分析4例经水平半规管前庭入路进行修补的Mondini畸形伴脑脊液耳鼻漏患者。结果:4例患者均一次性修补成功,随访6个月以上无复发。讨论:反复发作的脑膜炎患者要高度警惕是由Mondini畸形所致,纯音测听及颞骨CT或MRI检查可明确诊断,经水平半规管前庭入路修补Mondini畸形伴脑脊液耳鼻漏是一种简便有效的术式,值得推广。     

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。     

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；②Ｍｏｎｄｉｎｉ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     

Mondini畸形脑脊液漏修补的围手术期护理

目的探讨Mondini畸形伴脑脊液耳漏经鼓室入路脑脊液漏修补围手术期的观察及护理要点。方法回顾性探讨17例Mondini畸形伴脑脊液耳漏患者术前后的临床特点及进行重点护理的经验。结果17例患者均恢复良好,无护理并发症。结论术前做好患者的心理疏导,对于消除因多次严重脑膜炎发作及手术失败造成的恐惧心理十分重要;做好听力障碍的护理及提高交流质量;术后除做好面瘫的观察及护理外,还应做好颅内高压及再次出现脑脊液漏的观察及护理。     

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。     

Mondini畸形的临床表现及处理(附1例报告)

目的：提高对Mondini畸形的认识。方法：对1例Mondini畸形患者的临床特征、影像学表现及手术情况等临床资料进行分析。结果：Mondini畸形形成脑脊液漏后可伴发脑膜炎,常反复发作,患者大多就诊于其他科室,不易确诊,尤其是单侧畸形,因对侧听力正常,常易漏诊。结论：反复发作不明原因的脑脊液漏伴脑膜炎者应疑及此病,CT与MRI检查是确诊的主要依据。对伴发脑脊液耳漏的患者,鼓室探查修补术是有效的方法。     

Mondini和共同腔畸形伴脑脊液漏的诊断与治疗

Mondini畸形是一种先天性内耳畸形，主要表现为听力下降，但当伴发脑脊液漏时，患者大多就诊于其他科室，不易确诊^[1]。2008年我科收治2例以脑脊液漏伴脑膜炎为主要临床表现的Mondini畸形患者并获手术成功，现报告如下。1资料与方法1.1临床资料例1患者，男，15岁。因发现左耳听力下降5年余，脑膜炎发作2次入院。     

Use of the translabyrinthine approach to repair congenital spontaneous cerebrospinal fluid leakage in five Chinese patients with Mondini dysplasia

Objectives

To introduce a more effective surgical technique for the repair of spontaneous incurable cerebrospinal fluid (CSF) leakage (CSF otorrhea).

Methods

The cases of five Chinese patients with Mondini dysplasia and CSF leakage were reviewed. All patients presented with rhinorrhea or otorrhea from childhood and recurrent meningitis attacks. CSF leakage was repaired surgically using the translabyrinthine approach.

Results

Facial paralysis occurred in the first two patients, but was cured 2 months later. The other three patients recovered well and had no facial paralysis or complication. CSF leakage did not recur in any patient after follow-up periods of 0.5–3 years.

Conclusion

Mondini dysplasia with CSF leakage is rare. Adequate diagnosis and treatment are required to avoid life-threatening complications, such as meningitis. We recommend the translabyrinthine approach as an alternative surgical technique that reduces recurrence.     

经迷路入路修补Mondini畸形合并脑脊液耳漏附2例报告

目的探讨采用经迷路入路治疗Mondini畸形合并脑脊液耳漏的外科方法。方法对2例Mondini畸形合并脑脊液耳漏患者均采用经迷路入路治疗,修补脑脊液耳漏。结果 2例患者均一次修补成功,随访6个月以上无复发。结论反复发作脑膜炎并合并耳聋患者要高度警惕Mondini畸形合并脑脊液耳漏,影像学检查和神经耳科检查可明确诊断;经迷路入路可有效治疗Mondini畸形合并脑脊液耳漏。     

The LAURA multichannel cochlear implant in a true Mondini dysplasia

A congenitally deaf child with bilateral Mondini dysplasias of the inner ear was successfully implanted with a LAURA multichannel cochlear prosthesis. This is the first report of a patient with a Mondini dysplasia in whom a LAURA multichannel cochlear prosthesis was successfully implanted. The cerebrospinal fluid leakage typically occurring after cochleostomy in similar patients was readily controlled, indicating that the deformity present was not a contra-indication for cochlear surgery. The audiological results obtained are described in detail. Since the internal unit of the LAURA prosthesis is very flexible, various speech processing strategies, such as continuous interleaved and phase-locked continuous interleaved strategies, can be tried and evaluated.     

Management of cerebrospinal fluid leaks involving the temporal bone: report on 92 patients

OBJECTIVE: To investigate the success of different surgical and nonsurgical techniques in the management of cerebrospinal fluid otorrhea or otorhinorrhea. STUDY DESIGN: Retrospective case analysis. METHODS: Ninety-two patients with confirmed diagnosis of cerebrospinal fluid otorrhea or otorhinorrhea treated at our institution between 1976 and 1998 were followed up long-term by retrospective chart review and patient interviews. RESULTS: Conservative treatment was successful in most cases of cerebrospinal fluid otorrhea or otorhinorrhea resulting from head injury (26 of 29 patients). In contrast, surgical intervention was required in all but 1 of 53 patients with cerebrospinal fluid otorrhea or otorhinorrhea caused surgically. The primary operative success rate was 76.9%. When relapse occurred, the interval ranged from 0 days to 24 months (mean interval, 3.9 mo; median, 1.3 mo). All leaks requiring surgery eventually were closed successfully. Surgical results were chronologically dependent. Before 1989, 11 failures occurred in 37 primary procedures. After 1989, only 4 failures occurred in 28 primary procedures. Of seven patients undergoing primary dural closure alone, three (43%) had recurrence of the leak. Closure rates were highest among patients in whom a multilayer technique for leak closure was used: combining a primary graft or sealing material such as bone wax, free muscle, or fascia for closure of the defect with additional autologous free grafts or allogenic materials. This resulted in a 2-year closure rate of 100% compared with a 2-year rate of 75.4% for patients whose primary closure was supported by a single layer of autologous or allogenic material (P =.034). Fibrin glue with primary closure alone did not have additional benefit. Postoperative meningitis occurred in two patients and was treated without sequelae. CONCLUSIONS: Conservative treatment should be reserved for cerebrospinal fluid otorrhea or otorhinorrhea resulting from head injury. Postoperative and nontraumatic cerebrospinal fluid otorrhea or otorhinorrhea should have early operative intervention. A multilayer technique combining allogenic materials with free autologous grafts is recommended.