内耳畸形语前聋患儿人工耳蜗术后康复效果分析

目的　评估内耳畸形语前聋患儿人工耳蜗植入术后康复结果。方法　回顾性分析术前影像学检查为内耳畸形语前聋人工耳蜗21例,并筛选背景接近的21例正常耳蜗结构语前聋人工耳蜗植入患儿配对组合。对患者术前、术后6个月及术后1年3个阶段听力言语康复效果进行评估。结果　9例前庭导水管扩大综合征和6例Mondini 畸形患者与耳蜗结构正常者相比,听觉言语结果无明显差异。畸形比较严重的1例Mondini和5例共同腔患者与耳蜗正常者相比,听觉言语结果有明显的差异。结论　前庭导水管扩大综合征患者人工耳蜗术后效果与耳蜗发育正常者相似,但对严重的Mondini 畸形和共同腔等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形,以助于术后制定康复方案。     

内耳畸形人工耳蜗植入术后听力言语康复效果分析

目的：使用听觉和言语问卷分级及术后产生听觉的最小电流值（T值）的方法,评估并比较内耳畸形与正常解剖结构语前聋患儿人工耳蜗植入术后的听觉言语康复效果。方法：按术前影像学检查将语前聋人工耳蜗植入患儿分为正常结构组和内耳畸形组,并配对组合,对思儿家长进行问卷形式调查随访,对术后听力及言语康复效果进行评估分析,记录术后1年凋机T值。用秩和检验比较2组听觉行为分级标准（CAP）、言语可懂度分级标准（SIR）结果及T值。结果：人工耳蜗植入患儿家长术前主要担心术后效果不理想及手术并发症的发生,多数认为听力言语康复训练应由医疗机构进行;秩和检验显示：2组CAP及SIR均无显著差异,术后1年调机时产生听觉的T值无显著差异。结论：①内耳畸形患儿人工耳蜗植入术后,经正规康复训练,听力言语康复效果与内耳解剖结构正常植入者相同,人工耳蜗植入术可帮助伴耳蜗畸形的极重度感音神经性聋患者重建听力,重返主流社会;②听力言语康复训练尚有很多方面需要改进。     

人工耳蜗咨询问题解答（3）

为什么人工耳蜗术前要进行听力康复训练？ 人工耳蜗植入后可使聋人获得听觉,但要达到识别语言,运用语言（语前聋儿童）,需要逐渐熟悉和学习人工耳蜗所提供的新的听觉信息,尤其是对于聋哑儿童需要一个长期的听力语言康复过程。因此,为了选择患者是否适合接受人工耳蜗植入,以及为使人工耳蜗植入后发挥更大的作用,需要进行术前听力康复训练。术前听力康复的重要内容就是佩带助听器训练。对于语前聋患儿,学会配合听力检测尤其重要。人工耳蜗手术有危险吗？ 人工耳蜗的手术主要是将人工耳蜗内植部件植入耳后的颅骨外皮下,将电极组插入耳…     

皮层听觉诱发电位评估人工耳蜗植入语前聋儿童的中枢听觉系统发育

目的研究人工耳蜗植入语前聋儿童的皮层听觉诱发电位(cortical auditory evoked potential,CAEP)P1波潜伏期的发展规律,评估不同年龄段植入人工耳蜗语前聋儿童的中枢听觉系统的发育。方法以人工耳蜗植入语前聋儿童50例(植入年龄15~66月,平均38.7±15.2月,其中小于42月龄者27例,大于42月龄者23例)和年龄相匹配的正常听力儿童50例为研究对象,采用500、1000、2000、4000 Hz四种频率的短纯音(tone burst,TB)刺激声分别对受试者进行皮层听觉诱发电位测试,并对人工耳蜗植入语前聋儿童进行婴幼儿有意义听觉整合量表或有意义听觉整合量表(infant-toddler meaningful auditory integration scale/meamingful auditory integration scale,IT-MAIS/MAIS)评分,比较两组P1波潜伏期,对P1波潜伏期和IT-MAIS/MAIS评分进行相关性分析。结果正常听力儿童四种不同频率短纯音刺激记录的CAEP P1波潜伏期与年龄均呈负相关(P<0.01);42月龄前植入人工耳蜗的语前聋儿童P1波潜伏期与正常听力儿童无统计学差异(P>0.05);42月龄以后植入人工耳蜗的语前聋儿童的P1波潜伏期较正常儿童显著延长,差异有显著统计学意义(P<0.01)。人工耳蜗植入儿童的CAEP P1波潜伏期与IT-MAIS/MAIS量表评分均呈负相关(P<0.01),不同频率TB刺激声下的P1波潜伏期无统计学差异(P>0.05)。结论大于42月龄植入人工耳蜗的语前聋儿童的中枢听觉系统发育较正常听力儿童延迟。     

复杂内耳畸形患儿人工耳蜗植入术后疗效分析

目的 探讨复杂内耳畸形患儿人工耳蜗植入术后的听觉言语发育效果.方法 回顾性分析2018年1月至2019年6月于西京医院行人工耳蜗植入术的17例复杂内耳畸形语前聋(内耳畸形组)患儿的临床资料,其中双侧植入1例,右侧植入8例,左侧植入8例,植入年龄1～10岁(平均3.49岁),男6例,女11例;IP-1畸形2例,IP-3畸...     

不同内耳畸形人工耳蜗植入效果分析

目的 就不同内耳畸形人工耳蜗植入特点及效果进行分析,积累经验。方法 1996年1月～2004年5月北京同仁医院共开展多道人工耳蜗植入术410例(410耳),对其中双侧内耳畸形患者82例(82耳)进行回顾性分析,就不同原因内耳畸形的人工耳蜗植入手术特点及术后开机调试特点与耳蜗形态正常的人工耳蜗植入者进行了比较。结果 ①所有内耳畸形患者人工耳蜗植入术后都有听觉;②内耳畸形人工耳蜗术中容易出现井喷;③82例中除2例患者各遗留2对蜗外电极外,其余电极均完全植入蜗内;④术后无面瘫、脑脊液漏等并发症;⑤术后开机调试数值与结构正常植入者接近,无统计学差异(P>0.05);⑥术后经过语训,1年后听力及言语能力均有不同程度的提高。结论 伴有内耳发育畸形的深度感音性聋患者可以行人工耳蜗植入手术,植入效果与耳蜗发育正常患者的人工耳蜗植入效果基本一致,人工耳蜗植入术可以作为伴有内耳畸形的深度感音性聋患者的治疗和康复手段。     

多道人工耳蜗植入533例临床分析

目的 分析人工耳蜗植入患者的手术适应证、手术方法和术后效果。方法 533例(534耳)重度和极重度聋患者接受人工耳蜗植入手术。其中语前聋495例,语后聋38例。植入时年龄:1～3岁167例,～5岁77例,-7岁73例,-14岁136例,～17岁28例,>17岁52例。内耳中耳畸形共76例,其中Mondini畸形26例、共同腔畸形10例、前庭水管扩大综合征20例。术前纯音听阈(听力级)为105.5 dB,听性脑干反应阈>95 dB,40 Hz相关电位(500 Hz)>101.7 dB。使用装置:Nucleus 22M 27耳,24M 308耳,24R Contour 131耳,24RST 21耳;Med El C40 44耳;Clarion CI 3耳。常规手术采用面隐窝进路方法。共同腔畸形病例采用经水平半规管进路。部分病例行术后声场测听和听觉言语评估。结果 Mondini畸形26例中有20例术中出现脑脊液井喷。电极平均植入深度:耳蜗正常病例中Nucleus 30环,Med El为31 mm。Mondini畸形病例中Nucleus 28环。共同腔畸形病例中Necleus 26环。语后聋患者的平均开放言语识别率为71％,17岁以下语前聋的问卷调查满意度为94.7％。结论 人工耳蜗是使重度和极重度聋患者恢复听觉功能的有效方法,术前评估,手术和术后康复都是治疗效果的关键。     

Mondini内耳畸形患者的多通道人工耳蜗植入

目的探讨Mondini内耳畸形患者人工耳蜗植入方法及手术前后听力评估和术后言语康复效果。方法回顾性分析经乳突面隐窝进路行人工耳蜗植入的19例Mondini内耳畸形患者的临床资料，并将其中10例患者植入后听力情况与10例耳蜗发育正常的人工耳蜗植入者(对照组)进行比较。结果19例Mondini内耳畸形患者中，32个电极全部植入者12例，28个电极植入者2例，26个电极植入者2例，22个电极植入者2例，20个电极植入者l例；术中7例发生多量脑脊液流出(ozze)，5例发生脑脊液井喷(gusher)，7例无脑脊液外溢。两组患者术后听力接近，均在30～40dB HL左右，无严重并发症发生，17例Mondini内耳畸形患者术后听觉言语康复效果与耳蜗发育正常者接近，2例稍差。长期效果有待进一步观察。结论人工耳蜗植入适用于Mondini内耳畸形患者，但手术前应进行全面的听力学及影像学评估。     

影响语前聋患儿人工耳蜗植入术后前语言交流能力的因素分析

目的 探讨影响语前聋患儿人工耳蜗植入术后前语言交流能力的相关因素.方法 对接受人工耳蜗植入术的语前聋患儿 31例进行随访调查,平均植入年龄为46.71±24.78月,术后1个月开机进行康复训练;采用视频分析法于开机后12个月对患儿的轮流交流、主动交流、视觉交流及听觉注意等进行分析,并根据以下方面分组:按植入年龄分为≤36个月组16例,>36个月组15例;按术前有无正规康复训练(时间超过3个月)分为康复组14例,未康复组17例;按术前残余听力分为≤80 dB HL组6例,>80 dB HL组25例;按术前是否使用助听器(时间超过3个月)分为使用组21例,未使用组10例.应用Wilcoxon秩和检验分析各组前语言交流能力的差别.结果 术前进行康复训练、术前佩戴助听器、术前残余听力好的人工耳蜗植入患儿的前语言交流能力得分高于术前未进行康复训练、术前未佩戴助听器、术前残余听力差的人工耳蜗植入患儿(P<0.05);植入年龄≤36个月组与>36个月组间比较差异无统计学意义(P>0.05).结论 术前残余听力好、佩戴助听器、进行过正规康复训练的语前聋患儿人工耳蜗植入术后其前语言交流能力较好,而植入年龄对其无明显影响.     

磁共振内耳三维重建在Ⅱ型Waardenburg综合征患者中的应用

目的探讨磁共振内耳三维重建在Ⅱ型Waardenburg综合征中的应用。方法回顾性分析2008～2011年期间行人工耳蜗植入术的6例Ⅱ型Waardenburg综合征患儿术前磁共振内耳三维重建影像学检查结果,观察内耳的发育情况及人工耳蜗植入效果。结果 2例内耳无明显异常,4例有半规管畸形,3例有耳蜗畸形,术中均顺利植入全部电极,术后均顺利开机,听觉和言语水平明显提高。结论术前磁共振内耳三维重建影像学检查可清晰显示Ⅱ型Waardenburg患者的前庭、半规管及耳蜗情况,为内耳畸形特别是耳蜗畸形患者的人工耳蜗植入手术提供指导。     

Cochlear implantation in children with inner ear malformation and postoperative performance

Cochlear implantation in children with cochlear malformation is on the rise. However, only one case has been reported in Japan. The objective of this paper is to report the authors' experiences with cochlear implants in children with cochlear malformations and, to investigate the morphological classification of cochlear malformation, surgical procedure, complications, and speech perceptions after implantation. Five children (6 ears, including 1 Usher's syndrome and 1 CHARGE association) with congenital inner ear malformations were implanted with multichannel cochlear implants (Nucleus Mini 22 device) between 1994 and 1998. Malformations included common cavity deformity 2, and incomplete partition 4. On the other hand, 4 cases with incomplete partition deformity underwent cochlear implantation by the transmastoid facial recess approach, 2 patients with common cavity deformity received implantation by the transmastoid labyrinthotomy approach. Complications such as facial twitching, cerebrospinal fluid (CSF) gusger, and flap-related problems were encountered. Facial twitching occurred in 1 of 2 patients with common cavity where the electrode array extended into the internal auditory canal. CSF gusher was encountered in 1 of 4 patients with incomplete partition, and revision surgery was performed one week later. One patient had delayed cochlear implant infection and a split pericranial flap was used to cover the defect in the skin flap. Within 6 months, however, the revised pedestal site broke down because of recurrent flap infection. Finally, the cochlear implant was explanted, and the patient underwent a new cochlear implantation in the contralateral ear. All the patients had satisfactory speech reception and word acquisition, but still poor phonetic expression.     

共同腔畸形人工耳蜗手术适应证及手术方法的探讨

目的：探讨共同腔畸形人工耳蜗手术适应证以及人工耳蜗电极植入人路的选择。方法：在对重度或全聋患者进行人工耳蜗植入术前影像掌检查中,发现了6例耳蜗、前庭、外半规管呈共同腔畸形,其中5例有残留听力,1例未查到残留听力。结果：6例影像学检查呈共同腔畸形患者中,对5例有残留听力患者进行了人工耳蜗植入,其中3例选择了常规入路植入电极,2例选择了经乳突侧入路植入电极,术后均建立了人工耳蜗的听觉反应。1例因未查到残留听力,放弃了人工耳蜗手术治疗。结论：有残留听力的共同腔畸形患者,如果能够接受术后听觉言语识别效果差的事实,可以进行人工耳蜗手术。无残留听力或无法了解到有听觉反应的共同腔畸形患者,在现有技术条件下应放弃人工耳蜗植入手术。     

Cochlear Implants for Patients With Common Cavity Deformities and the Impact of Electrode Positioning

ObjectivesCommon cavity deformity is a rare congenital bony labyrinth malformation associated with profound hearing loss. Cochlear implants are widely used for hearing rehabilitation for common cavity deformities; however, the reported prognosis is poor. Due to the deformed anatomical structure, it is important to consider the position of the electrodes to maximize the performance of the cochlear implant. The present study discusses the impact of electrode placement on hearing outcomes.MethodsA retrospective medical chart review of eight common cavity deformity patients (10 cochlear implants) who received cochlear implants was performed at a single university hospital. In all eight patients, implant surgery was performed using single-slit labyrinthotomy. Electrodes wer e manually bent before insertion to prevent misplacement and to reduce physical damage to the neuroepithelium.ResultsFour of the 10 electrodes were misplaced, with their tips placed in the anterior semicircular canal or internal auditory canal. However, after implant surgery, all patients—including those with misplaced electrodes—gained auditory perception and improved hearing function. One patient who had electrodes that did not contact the inner wall of the cavity showed limited activity of the electrodes (27%) compared to others (64%–100%).ConclusionProper contact of the electrode with the inner wall was more likely to be important for cochlear implant success in cases of common cavity deformity than appropriate placement of the electrode tip.     

Congenital malformation of the inner ear and pediatric cochlear implantation.

OBJECTIVES: To study the surgical aspects and performance outcome of cochlear implantation in children with malformed inner ears. STUDY DESIGN: Clinical and audiometric evaluation in 13 patients. METHODS: Patient data concerning surgery, postoperative follow-up, and pre- and postimplantation audiometry were obtained from the cochlear implant center's database and evaluated. A review of the literature has been included. SETTING: Tertiary referral center. PATIENTS: The patients had a variety of inner ear malformations and profound hearing loss. One patient with recurrent meningitis had a severe cochlear malformation (common cavity). RESULTS: Major complications did not occur. In one patient with an abnormal position of the cochlea and concurring middle ear disease, it was difficult to find the scala tympani during surgery. A cerebrospinal fluid gusher was encountered in two patients and an aberrant facial nerve in another, which did not lead to any complications. The patients with mild cochlear malformation such as an incomplete partition demonstrated a good performance in speech perception tests. Even the child with the common cavity deformity had some open-set speech perception 1 year after implantation. CONCLUSIONS: Viewing the patients from this study and patients from a review of the literature concerning cochlear implantation in children with malformed inner ears including severe cochlear malformations, the occurrence of an aberrant facial nerve was 17%, which increases to 27% if one reviews the surgical findings in children with severe malformed cochleae such as a common cavity or a severe cochlear hypoplasia. In the latter patients, results in speech perception vary. Although the result of cochlear implantation may be promising, as in our patient with a common cavity, during preoperative counseling the child's parents must be informed that the result is uncertain.     

内耳结构异常患者的人工耳蜗植入术

目的探讨对内耳结构异常患者行人工耳蜗植入的方法并评估其术后效果。方法回顾性分析2000年1月~2010年5月行人工耳蜗植入术的24例内耳结构异常患者的临床资料,包括耳蜗骨化2例、Waarden-burg综合征Ⅱ型8例、Mondini畸形12例、共同腔畸形1例、半规管缺如1例;随机选择30例内耳结构正常的相同年龄段接受了人工耳蜗植入的极重度感音性聋患者作为对照组;分析并比较两组患者的术前听力和影像学资料以及手术植入方法和术后听觉言语康复情况。结果 1例共同腔畸形和2例Mondini畸形患者术中出现井喷,另有10例Mondini畸形、2例Waardenburg综合征Ⅱ型病例在耳蜗钻孔后见外淋巴液搏动性涌出,经抬高头位降低液压并以筋膜粒严密封堵开窗口等处理完成电极植入;2例耳蜗骨化者经扩大开窗范围并剔除骨化组织后分别植入8个和12个电极;其余病例均经常规方法完成电极植入。内耳结构异常组患者人工耳蜗植入术后均获得听觉,所有患者均未出现严重并发症。术后6个月内耳结构异常组啭音声场测听平均听阈35.4±4.9 dB HL,闭合式词表言语识别率平均为75.18%±3.1%,开放式词表言语识别率平均为70.32%±2.5%;对照组上述三项结果分别为31.4±5.5 dB HL、81.63%±3.7%、76.77%±5.4%,两组间比较差异无统计学意义(P>0.05)。结论对内耳结构异常的极重度感音性聋患者行人工耳蜗植入安全有效,但针对不同的内耳畸形需采取不同的处理方法。     

Cochlear implantation in cases with incomplete partition type III (X-linked anomaly)

Incomplete partition-type III anomaly (X-linked deformity) is no common finding in a prospective candidate for cochlear implantation. In this paper, the problems about the cochlear implantation in cases with incomplete partition-type III anomaly (X-linked deformity) and profound sensorineural hearing loss is discussed. High-resolution multidedector computed tomography (MDCT) and magnetic resonance imaging were performed preoperatively in all patients. MDCT revealed that there was bulbous dilatation at the lateral ends of internal auditory canals (IAC) in all patients. There were also enlargements of labyrinthine segments of facial and superior vestibular nerve canals. Patients with the basal turns of cochlea incompletely separated from IAC were also presented. Patients with IP-type III (X-linked deformity) and profound SNHL were implanted. Standard transmastoid-facial recess approach was used and cerebrospinal gusher was encountered after the cochleostomy in all cases. Postoperative performance was very good in all patients. Two patients had complications, which are facial nerve stimulation and device failure. Both patients were reimplanted. Cochlear implantation is a good choice in the patients with IP-type III. However, this anomaly may have special potential risk than the other inner ear abnormalities. Therefore, the surgeon should be aware of them and must be ready to inform the patient and parents. An erratum to this article can be found at     

A new classification for cochleovestibular malformations

OBJECTIVE: The report proposes a new classification system for inner ear malformations, based on radiological features of inner ear malformations reviewed in 23 patients. STUDY DESIGN: The investigation took the form of a retrospective review of computerized tomography findings relating to the temporal bone in 23 patients (13 male and 10 female patients) with inner ear malformations. The subjects were patients with profound bilateral sensorineural hearing loss who had all had high-resolution computed tomography (CT) with contiguous 1-mm-thick images obtained through the petrous bone in axial sections. METHODS: The CT results were reviewed for malformations of bony otic capsule under the following subgroups: cochlear, vestibular, semicircular canal, internal auditory canal (IAC), and vestibular and cochlear aqueduct malformations. Cochlear malformations were classified as Michel deformity, common cavity deformity, cochlear aplasia, hypoplastic cochlea, incomplete partition types I (IP-I) and II (IP-II) (Mondini deformity). Incomplete partition type I (cystic cochleovestibular malformation) is defined as a malformation in which the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance, and there is an accompanying large cystic vestibule. In IP-II (the Mondini deformity), there is a cochlea consisting of 1.5 turns (in which the middle and apical turns coalesce to form a cystic apex) accompanied by a dilated vestibule and enlarged vestibular aqueduct. RESULTS: Four patients demonstrated anomalies involving only one inner ear component. All the remaining patients had diseases or conditions affecting more than one inner ear component. Eight ears had IP-I, and 10 patients had IP-II. Ears with IP-I had large cystic vestibules, whereas the amount of dilation was minimal in patients with IP-II. The majority of the semicircular canals (67%) were normal. Semicircular canal aplasia accompanied cases of Michel deformity, cochlear hypoplasia, and common cavity. In 14 ears, the IAC had a defective fundus at the lateral end. In two ears the IAC was absent. In all seven cases of common cavity malformations, there was a bony defect at the lateral end of the IAC. In five of them the IAC was enlarged, whereas in two the IAC was narrow. All patients with IP-I had an enlarged IAC, whereas in patients with type II disease, four had a normal IAC and 10 had an enlarged IAC. All cases of IP-II had an enlarged vestibular aqueduct, whereas this finding was not present in any of the cases of IP-I. In all cases, the vestibular aqueduct findings were symmetrical on both sides (simultaneously normal or enlarged). No patient demonstrated enlargement or any other abnormalities involving the cochlear aqueduct. CONCLUSIONS: Radiological findings of congenital malformations in the present study suggested two different types of incomplete partition. Cystic cochleovestibular malformation (IP-I) and the classic Mondini deformity (IP-II). The type I malformation is less differentiated than the type II malformation. Classic Mondini deformity has three components (a cystic apex, dilated vestibule, and large vestibular aqueduct), whereas type I malformation has an empty, cystic cochlea and vestibule without an enlarged vestibular aqueduct. Mondini deformity represents a later malformation, so the amount of dysplasia is much less than in type II. Therefore, it is more accurate and useful for clinical purposes to classify these malformations (in descending order of severity) as follows: Michel deformity, cochlear aplasia, common cavity, IP-I (cystic cochleovestibular malformation), cochlear hypoplasia, and IP-II (Mondini deformity). Only in this way can these complex malformations be grouped precisely and the results of cochlear implantation compared.     

Double posterior labyrinthotomy technique: results in three Med-El patients with common cavity.

HYPOTHESIS: This study reports on the use of the double posterior labyrinthotomy surgical technique and a custom-designed electrode to ensure better positioning of stimulating electrodes within the common cavity and thus demonstrate suitable outcomes in patients. BACKGROUND: Cochlear implantation has proven beneficial for numerous children with congenital malformations of the inner ear. Several studies show good auditory perception outcomes in children with common cavity. However, there have been risks involved with surgical techniques used in the actual implantation. These include possible aberrant facial nerve and the strong potential for a cerebrospinal fluid gusher. Improved surgical techniques and electrode design could allow for better electrode contact and avoid electrode placement in the internal auditory meatus. METHOD: The double posterior labyrinthotomy technique was carried out in three cases using a custom made MED-EL COMBI 40+ electrode. RESULTS: Surgery was carried out with no complications and is no more technically demanding than other standard surgical approaches. The speech processor program remains stable over time, and auditory perception results are similar to those obtained from children with no cochlear abnormalities. CONCLUSION: These results demonstrate the success of the double posterior labyrinthotomy approach with modified cochlear implant, and this could be recommended as the procedure of choice in children presenting to an implant team with a common cavity.     

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.     

Surgical results of cochlear implantation in malformed cochlea.

OBJECTIVE: To report the surgical aspects of cochlear implantation in malformed cochlea. SETTING: Tertiary care center. STUDY DESIGN: Retrospective case review. METHODS: Between November 1997 and October 2004, 20 patients with inner ear malformations were implanted in our department. The age range was between 2 and 37 years (average, 8.8 yr). The anomalies were classified according to Sennaroglu and Saatci classification. There were two patients with common cavity deformity, four cases of incomplete partition (IP) type I (cystic cochleovestibular malformation), four cases of IP type II (classical Mondini's deformity), nine patients with large vestibular aqueduct (LVA) syndrome, and one patient with X-linked deafness. RESULTS: Standard transmastoid facial recess approach was used in 17 patients (three patients with IP I, four patients with IP II, and nine patients with LVA syndrome). In the remaining patient with IP I, because of the dehiscent and anteriorly located facial nerve, the surgical approach had to be modified, and an anteroposterior approach was used. After elevating the tympanomeatal flap, the electrode was inserted through the ear canal and then transferred to the mastoid through a full-length cut produced in the ear canal. The flap then returned to its place. In the patients with common cavity deformity, the electrode was inserted by the transmastoid labyrinthotomy approach. Facial nerve had an abnormal course in four patients, but no patient had facial weakness postoperatively. Cerebrospinal fluid gusher was encountered in four patients, whereas oozing was present in five patients. It seems that a slightly larger cochleostomy may reduce postoperative rhinorrhea. The patient with common cavity deformity showed abnormal vestibular stimulation which decreased and was totally abolished during a 3-month period. CONCLUSION: Based on these findings, cochlear implantation is surgically feasible in patients with common cavity, IP types I and II, and LVA. The surgeon should be ready to make modifications in the surgical approach because of the abnormal course of the facial nerve and be ready to produce special precautions to cerebrospinal fluid gusher.