"井喷"在内耳畸形人工耳蜗植入术中的发生率

目的：探讨“井喷”在内耳畸形人工耳蜗植入术中的发生率，为有“井喷”现象的人工耳蜗植入术积累临床经验。方法：对680例行人工耳蜗植入术患者行术前高分辨CT检查，显示80例双耳有内耳畸形，其中20例并发内耳道底骨性缺损与内耳相通，行人工耳蜗植入术。结果：20例（25％）并发内耳道底骨性缺损与内耳相通者，术中发生了“井喷”，其中大前庭导水管畸形者5例，Mondini畸形者11例，耳蜗前庭与内耳道共同腔畸形者4例；耳蜗、前庭、外半规管共同腔和前庭、外半规管共同腔畸形者无“井喷”发生。结论：①80例内耳畸形人工耳蜗植入术患者“井喷”的发生率为25％；②内耳畸形人工耳蜗植入术中“井喷”的发生率依次为：耳蜗、前庭与内耳道共同腔畸形，Mondini畸形，大前庭导水管畸形；③单纯前庭、耳蜗、外半规管共同腔畸形及半规管畸形者，人工耳蜗植入术中未发生“井喷”；④80例内耳畸形人工耳蜗植入术中，发生“井喷”者均存在内耳道底骨缺损，与内耳形成共同开放的通道。     

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。     

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。     

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。     

人工耳蜗植入"井喷"的判断与术中处理的体会

目的：探讨人工耳蜗植入发生“井喷”的术前判断与术中处理的方法。方法：327例人工耳蜗植入患者术前均行颞骨高分辨CT（HRCT）筛查，并行术前准备及术中处理。结果：11例术前颞骨HRCT显示内耳畸形并发内听道底骨性缺损与耳蜗、前庭相通，术中均有脑脊液喷出，经过术中对植入口的严密封闭，术后未见脑脊液耳鼻漏等并发症的发生。结论：人工耳蜗植入发生“井喷”的原因为内耳与蛛网膜腔有异常开放的通道。术前行颞骨HRCT检查不仅能对内耳畸形的患者作出准确的判断，还能为术中进行正确的处理提供准确的科学依据，从而避免并发症的发生。     

人工耳蜗植入术中发生脑脊液“井喷”病例的影像学分析

目的分析人工耳蜗植入术中发生脑脊液“井喷”的原因及影像学特点。方法回顾性分析1998年1月～2011年7月2238例人工耳蜗植入患者中,发生脑脊液井喷的134例患者术前的影像学特征。结果134例人工耳蜗植入术中发生脑脊液“井喷”的病例中,大前庭导水管合并与内耳有骨性交通11例,大前庭导水管伴Mondini畸形合并内听道与内耳骨性交通28例,Mondini畸形伴内听道底与内耳骨性交通88例,耳蜗与内听道共同腔畸形7例。结论伴有内听道底骨缺损与内耳相通的任何内耳畸形患者,人工耳蜗植入术中必然发生脑脊液“井喷”。     

45例先天性内耳畸形的高分辨率CT特征

目的：探讨先天性内耳畸形的高分辨率CT(HRCT)特征及其在先天性内耳畸形诊治中的意义。方法：总结45例先天性内耳畸形的临床资料．分析颞骨HRCT所见。结果：45例耳聋多起于幼儿．呈缓慢进行性感音神经性听力减退，波动性者15耳；17耳半规管麻痹。HRCT显示：Michel型3例(4耳)；Mondini型25例(39耳)；不伴内耳畸形的前庭水管扩大13例(23耳)；内耳道发育畸形4例(5耳)。伴中、外耳畸形13耳。结论：HRCT对先天性内耳畸形具有重要的诊断价值，并为临床治疗、特别是人工耳蜗植入术适应证的选择提供了重要的依据。     

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。     

3D-FIESTA序列在先天性内耳畸形中应用

目的评估3D-FIESTA序列在先天性内耳畸形中的应用价值。方法采用3D-FIESTA序列对38例内耳畸形患者进行扫描,并进行多平面重组。结果 38例（62耳）内耳畸形中耳蜗畸形31耳,前庭畸形28耳,半规管畸形32耳,前庭水管及内淋巴囊扩大21耳,内耳道神经发育异常29耳。结论 3D-FIESTA序列可以清晰显示内耳道及内耳迷路的先天发育异常,具有较高的临床应用价值。     

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。     

高分辨率CT容积重建对内耳畸形的诊断价值

目的：通过高分辨率CT（HRCT）容积重建（VR）对正常内耳形态的观察,探讨其对先天性内耳畸形的诊断价值。方法：对10例（20耳）无耳部疾患者（对照组）和7例（11耳）先天性内耳畸形患者（病变组）行HRCT扫描,利用容积漫游技术对内耳骨迷路重建。对照组观察内耳骨迷路的正常结构,病变组观察内耳畸形情况。结果：正常耳VR图像不但显示了内耳骨迷路的细微结构,而且还反映了各结构之间的关系;病变耳立体显示了畸形部位及程度。11耳患耳中Mondini型7耳次;前庭及半规管畸形3耳次;前庭导水管扩大7耳次,其中6耳次伴随其他畸形;内耳道畸形2耳次且均伴随其他畸形。11耳畸形中9耳HRCT横断面图像和平面重建（MPR）冠状位图像、VR图像均可以清晰地显示畸形的部位和程度,其中VR图像可以直观、立体地显示畸形的空间形态结构;2耳水平半规管短小畸形患者VR图像较断面图像更好地显示了畸形的部位和程度。结论：VR三维重建可以立体显示正常内耳骨迷路的形态,直观显示内耳畸形的程度及病变位置,对内耳畸形的诊断具有重要的辅助价值。     

先天性耳廓畸形与中耳畸形的相关性分析

目的 探讨先天性耳廓畸形与中耳畸形的相关性及其意义.方法 收集自2008年1月至2009年12月在复旦大学附属眼耳鼻喉科医院就诊的86例先天性外中耳畸形患者的资料,其中男51例,女35例;单侧58例(右侧34例,左侧24例),双侧28例;共计114耳.收集所有患者颞骨高分辨率CT资料,参照Jahrsdoerfer评分标准对中耳畸形情况进行评分,同时按照Marx H分度标准对耳廓畸形进行分度,并将耳廓畸形分度与中耳畸形评分进行Spearman秩相关分析.结果 86例先天性外中耳畸形患者中耳廓畸形Marx H分度结果为:Ⅰ度12耳,Ⅱ度25耳,Ⅲ度77耳,相应的Jahrsdoerfer评分((-x)±s)分别为(7.8±2.4)、(6.8±2.6)、(6.0±2.8)分.耳廓畸形Marx H分度与中耳畸形Jahrsdoerfer评分呈现出负相关趋势(r=-0.2386,P=0.0106).结论 先天性外中耳畸形患者的耳廓畸形程度与中耳畸形程度具有一定的相关性.     

Cochlear implantation in children with congenital inner ear malformations

OBJECTIVE/HYPOTHESIS: To assess the audiologic and surgical outcomes for pediatric cochlear implant patients with inner ear malformations. STUDY DESIGN: Retrospective review of 315 pediatric cochlear implant cases from 1994 to 2002. METHODS: Twenty-eight pediatric cochlear implant patients with known inner ear malformations determined on high-resolution computed tomography (HRCT) of the temporal bone were the subjects of review. Results of HRCT findings, intraoperative findings, postoperative complications, and objective measures of both closed- and open-set testing of speech perception were analyzed. RESULTS: Patients with the constellation of an incompletely partitioned (IP) cochlea, enlarged vestibular aqueduct (EVA), and a dilated vestibule (i.e., Mondini's malformation) as well as those with an isolated EVA or partial semicircular canal aplasia have relatively good levels of speech perception. Patients with total semicircular canal aplasia, isolated IP, cochlear hypoplasia, or common cavity demonstrated lower levels of performance. Poor performance may be related to associated developmental delays rather than labyrinthine anatomy alone. Complications of surgery were relatively limited. CONCLUSIONS: Cochlear implantation can be successfully performed in children with inner ear malformations. These children and their parents can expect significant auditory benefits from this intervention. The various types of inner ear malformations may have quite different prognoses for good auditory performance.     

Inner ear anatomy in Waardenburg syndrome: Radiological assessment and comparison with normative data

Objective

As patients with Waardenburg syndrome (WS) represent potential candidates for cochlear implantation, their inner ear anatomy is of high significance. There is an ongoing debate whether WS is related to any inner ear dysplasias. Our objective was to evaluate radiologically the inner ear anatomy in patients with WS and identify any temporal bone malformations.

Methods

A retrospective case review was carried out in a tertiary, referral center. The high resolution computed tomography (HRCT) scans of the temporal bone from 20 patients (40 ears) with WS who were managed for deafness in a tertiary referral center from 1995 to 2012 were retrospectively examined. Measurements of 15 different inner ear dimensions, involving the cochlea, the vestibule, the semicircular canals and the internal auditory meatus, as well as measurements of the vestibular aqueduct, were performed independently by two neuroradiologists. Finally, we compared the results from the WS group with a control group consisting of 50 normal hearing subjects (100 ears) and with previously reported normative values.

Results

Inner ear malformations were not found in any of the patients with WS. All measured inner ear dimensions were within the normative values compiled by our study group as well as by others.

Conclusions

Inner ear malformations are not characteristic for all types of WS; however, certain rare subtypes might be related to inner ear deformities. Normative cochleovestibular dimensions that can help in assessing the temporal bone anatomy are provided.     

不同内耳畸形人工耳蜗植入效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHODS: Among 410 patients who received multi-channel cochlear implantations from 1996 to 2004 in Beijing Tongren Hospital, 82 patients were diagnosed with inner ear malformations and implanted. A retrospective analysis was performed about the surgical characteristics and mapping characteristics after implantation. RESULTS: (1) All patients had auditory sensations. (2) Gusher was more common than the normal cochlear implantation. (3) The electrodes were inserted in the "cochleostomy" in full length of 80 Patients, but 2 pairs of electrodes remained outside of "cochleostomy" in 2 patients. (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, the T level and C level were similar with the normal cochlear implantation. The results had no significant difference in compare with normal cochlear group (P > 0. 05). (6) The abilities of speech discrimination and spoken language were improved through rehabilitation. CONCLUSIONS: The cochlear implantation can be performed safely in inner ear malformations. The outcome of hearing rehabilitation for patients with inner ear malformations are similar to those children with normal cochlear structure followed the multi-channel cochlear implantation.     

HRCT imaging characterized of congenital abnormalities of the inner ear in 45 cases]

OBJECTIVE: To explore the high resolution CT (HRCT) image characterized of congenital abnormalities of the inner ear(CAIE), and its value in the diagnosis and treatment of CAIE. METHOD: The clinic data and axial HRCT scans of CAIE in 45 cases were analyzed. RESULT: In 45 CAIE patients, most of them were frequently associated with slowly progressive sensorineural hearing loss in childhood, 15 ears were fluctuating hearing loss. Seventeen ears were unilateral semicircular canal paralysis. HRCT showed that Michel type 3 cases(4 ears), Mondini type 25 cases(39 ears). Large vestibular aqueduct malformation not associated with anomalies of inner ears 13 cases(23 ears), anomalies of internal auditory canal 4 cases (5 ears). Thirteen ears were associated with outer and middle ear malformation. CONCLUSION: HRCT image has the important value in the diagnosis and treatment of CAIE, especially for the excerpt of indication of cochlear implantation.     

内耳畸形相关SLC26A4基因的研究

目的:研究内耳畸形相关SLC26A4基因在大前庭水管综合征(LVAS)、Mondini畸形及不伴前庭水管扩大的耳蜗畸形耳聋人群中的突变情况,在分子水平上探讨内耳畸形的发病机制,为基因诊断提供理论基础。方法:收集14例散发LVAS、6例Mondini畸形(伴前庭水管扩大)及7例不伴前庭水管扩大的耳蜗畸形耳聋患者的外周血DNA样本及临床资料,利用PCR扩增目的基因后直接测序的方法对所有患者进行SLC26A4基因全编码序列检测,同时进行GJB2、线粒体12SrRNA 1555/1494位点排除性检测。结果:14例LVAS患者中12例(85.7%)具有SLC26A4双等位基因(纯合或复合杂合)突变,2例(14.3%)有单等位基因突变。6例Mondini畸形患者均具有SLC26A4双等位基因(纯合或复合杂合)突变。7例不伴前庭水管扩大的耳蜗畸形耳聋患者中均未查出SLC26A4基因突变。27例患者均未发现GJB2、线粒体12SrRNA 1555/1494致病突变。结论:LVAS及Mondini畸形与SLC26A4基因突变密切相关,且Mondini畸形比单纯LVAS基因突变率更高,而不伴前庭水管扩大的耳蜗畸形耳聋患者未发现SLC26A4基因突变,尚需对该部分耳聋患者进行深入的分子病因学研究。     

先天性外中耳畸形的Ⅰ期手术整复

目的 :探讨 期整复术治疗先天性外耳、中耳畸形和外耳道闭锁并存的患者的疗效。方法 :耳廓成形术 :取自体肋软骨作支架 ,患耳颞部超薄皮瓣及皮下组织瓣包裹软骨支架形成再造耳廓。鼓室成形术 :根据鼓室畸形情况作 型或大鼓室 型成形术 ;外耳道再造 :用全厚皮片移植。结果 :术后随访 4～ 6年 ,全部病例外耳道宽畅 ,成形鼓膜完整。语言频率听阈降低均达到应用水平 ;再造耳 11耳成活 ,8耳外形满意。结论 : 期整复术是治疗外耳、中耳畸形和外耳道闭锁并存的有效方法。     

先天性中耳畸形的临床分型及其与耳聋的相关性

目的 探讨先天性中耳畸形的临床分型，以及不同类型的先天性中耳畸形与耳聋的相关性，以利于术前中耳畸形的诊断和术式的选择。方法 回顾性分析解放军总医院1995年3月-2004年5月收治的经手术证实为单纯先天性中耳畸形的病例（64例，82耳）。根据中耳组织胚胎学发育及手术探查中耳畸形情况进行临床分型，统计学检验各型先天性中耳畸形听阈的差异。结果 根据中耳结构组织胚胎学的发育，将先天性中耳畸形分为：A1：先天性锤砧骨畸形；A2：先天性砧镫骨畸形；B型：先天性镫骨固定；C型：先天性前庭窗或蜗窗发育不全或闭锁。听力学检查显示A、B、C3组间在语言频率上差异无统计学意义（P=0．1617），而高频（〉2kHz）的听阈B型及C型与A型均具有统计学意义（P〈0．05），并且B、C型先天性中耳畸形存在骨导下降及混合聋。结论 中耳结构的组织发育来源不同，在临床上不同类型的先天性中耳畸形累及的范围及程度存在很大差异，从而导致不同程度的传导性聋或混合性聋，其听阈不仅决定于听骨链是否完整，还在于畸形的部位和累及的范围。骨导听阈与气导高频听阈有助于鉴别不同类型的先天性中耳畸形。