人工耳蜗植入的研究

人工耳蜗植入的研究曹克利用耳蜗植入（电子耳蜗）的方法使全自病人恢复听觉，是近二十年来生物医学工程领域内的又一进展，它是涉及耳科学、听大学、听生理学、心理物理学、电子学及高分子材料学等多学科综合研究的产物。随着微电子学和计算机科学等高技术的出现，近年来...     

人工耳蜗植入相关的内耳解剖异常的诊断及处理

人工耳蜗植入是双耳重度感音神经性聋和全聋最有效的康复手段。为了提高人工耳蜗植入手术的成功率，我们对拟接受人工耳蜗植入的患者应进行全面的术前评估与筛选，对内耳解剖异常的类型要有详细的了解。本文就与人工耳蜗植入相关的内耳解剖异常的诊断和处理及效果进行综述。     

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。     

聋儿早期人工耳蜗植入的分析

人工耳蜗装置和人工耳蜗植入手术近三十年的广泛应用．使越来越多的聋人改善听觉。随着科技的发展和经验的积累．早期诊断婴幼儿听力障碍和判断人工耳蜗适应症的可行性越来越高。人工耳蜗装置越来越适合小年龄聋儿。只要经过全面的医学检查和康复．使用合适的人工耳蜗装置进行手术．聋儿早期可以在听觉的发展．语言的获得等各方面取得实效．尽快地回归主流社会。给个体、家庭及社会减轻负担。早期人工耳蜗植入将逐渐成为聋儿早期干预和康复的常规手段之一。     

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.     

人工耳蜗植入后并发脑膜炎一例

人工耳蜗是目前重度及极重度感音神经性聋患者重新感受有声世界的最有效方法之一，目前已发展得相当成熟，而与之相关的并发症发生率已降到相当低的水平。但是，作为一种异体植入和有创手术，其相关并发症仍然不容忽视。我院人工耳蜗中心自开展人工耳蜗植入手术以来至2006年2月底已为866例患者实施了手术。这些患者中有1例术后2个月出现脑膜炎。报道如下。     

人工耳蜗植入工作指南（2003年，长沙）

人工耳蜗是一种可以帮助聋人恢复听力和语言交流能力的生物医学工程装置。由于人工耳蜗植入是医学和康复领域中的一项新技术，因此在适应证的选择、手术前后的评估、手术、术后调机和听觉言语康复方面都需要一份可供参考的标准。本指南旨在为从事此项工作的临床医生、听力和言语康复工作者提供指导性的意见，使我国的人工耳蜗植入工作达到规范化和标准化，从而提高治疗效果，避免不必要的风险。     

人工耳蜗植入工作指南的试行必将推动学科发展

对依靠扩大或选择性扩大音响刺激强度改善听觉功能的设备无效的重度或极重度感音神经性聋病例，选用人工耳蜗植入治疗，已为耳科学界和患者普遍认同。全世界已有60000多例，我国已有1600多例接受了电子耳蜗。越来越多的医院和医师将参加此项工作，将有更多患者因此受益，前景是很美好     

先天性内耳畸形患者多通道人工耳蜗植入的效果观察

目的探讨多通道人工耳蜗在先天性内耳畸形患者植入的效果.方法对16例先天性内耳畸形患者(畸形组,Mondini畸形12例,大前庭导水管综合征4例),2例经前庭窗植入电极,3例经鼓岬植入电极,11例经圆窗龛前上缘植入电极.2例术中发生镫井喷.以10例耳蜗发育正常的植入者作为对照组,进行术后效果对比.结果畸形组与对照组术中和术后并发症差异无显著性意义.术后听阈畸形组多数患者达到30～40 dB HL,与正常组相似,仅少数患者听阈稍高.经统计学分析差异无显著性意义(P>0.05).听觉语言康复效果畸形组多数与对照组接近.结论多通道人工耳蜗植入适用于绝大多数先天性内耳畸形的患者,术后听力及语言康复效果满意.     

中国多道人工耳蜗植入现状

近年来，随着多道人工耳蜗技术的不断发展，有越来越多的重度感音性聋患者接受了人工耳蜗植入术，提高了生活质量，使他们能重新参与社会生活。自1995年我国引进此技术以来，截止到2003年8月已有1488例患者接受了手术。现对我国人工耳蜗植入现状、存在问题及解决方法进行探讨。     

先天性内耳畸形的人工耳蜗植入

目的　探讨先天性内耳畸形引起重度感音神经性聋者人工耳蜗植入的有关问题。方法　 2 0 0 1年 1月至 2 0 0 3年 4月间对内耳畸形引起极重度感音神经性聋 18例进行了人工耳蜗植入术。结果　 18例中前庭水管 11例 ,Waardenburg综合征 3例 ,Mondini畸形 3例 ,Usher综合征 1例。全部病例采用Nucleus 2 4型人工耳蜗 ,其中对前庭水管 5例采用Contour植入体 ,其余病例采用直电极植入体。术中发现前庭水管 11例开骨窗后仅有外淋巴搏动 ,但无井喷 (脑脊液漏 ) ,电极植入顺利。Waardenburg综合征 3例和Mondini畸形 3例中各有 1例伴发圆窗骨性封闭畸形。 结论　前庭水管者人工耳蜗植入手术顺利 ,术后效果与耳蜗发育正常者相同。如Mondini和CommonCavity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 ,要充分估计手术难度和避免术后可能出现的脑脊液耳鼻漏及其颅内感染     

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.     

先天性内耳畸形并中耳畸形患者多通道人工耳蜗植入术3例报告

目的：探讨先天性内耳畸形并中耳畸形患者行多通道人工耳蜗植入术的方法及效果。方法：1995年5月-2002年5月我院为3例罕见的先天性内耳畸形并中耳畸形患者经乳突进路行人工耳蜗植入术,分别植入27、28、32个电极,植入后3个月行声场测听。结果：3例患者均成功地行人工耳蜗植入,术中、术后无严重并发症发生,术后声场测听听阈达35-40dBHL。结论：罕见的先天性内耳畸形并中耳畸形患者也可行人工耳蜗植入术,术后效果满意。     

Cochlear implantation in the Mondini inner ear malformation

We report the case of a profoundly deaf 4-year-old boy with congenital deafness as a result of Mondini's dysplasia. The Mondini inner ear malformation is the result of arrested labyrinthine development during embryogenesis and is characterized by both bony and membranous anomalies of the inner ear. The dysplastic cochlear anatomy does not preclude successful cochlear implantation, and electrical threshold measurements are similar to those recorded in pediatric subjects deafened as a result of other causes.     

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.     

Cochlear implant and inner ear malformation. Proposal for an hyperosmolar therapy at surgery

The objective of this retrospective study is to evaluate the efficiency of hyperosmolar therapy for cerebrum spinal fluid (CSF) leakage in cochlear implant (CI) surgery in children with inner ear malformations. Between 1991 and 2006, 490 cochlear implantations were performed in Armand Trousseau Children's Hospital. Thirty-seven patients (7.5%) had inner ear malformation. They were classified as isolated enlargement of the vestibular aqueduct (EVA) (18 cases), incomplete partition (IP) (11 cases), common cavity (CC) (1 case) and variable canal and vestibular malformations (VSCC) (7 cases). A hyperosmolar protocol was applied during surgery to 13 patients after 2003 (Gp) to be compared to the 24 patients without treatment previously to this date (G0). Mean age at implant CI was 8.1 years (1-20 years), mean follow up was 3.9 years (1 month-15 years). Per operative observations were collected for all patients with an empiric method of evaluation of the leakage. A grading using five steps ranged from Grade 0 (no leak) to Grade 4 (gusher). Grading, complications and perceptive results in closed and open set word (Lafon lists) at respectively preoperatively, at 3 and 24 months were gathered and compared between the two groups. Important per operative leak was observed (Grade 4) in 24.3% cases (9/37) of Grade 4, 88.8% of them in G0 (8/9). In 66.6% cases there was a severe dysplasia (CC or IP) (6/9), to be compared to the 21.4% of cases of severe dysplasia with Grade<3 (6/28) (p=0.02). Grade 4 was seen in 45% cases of IP (5/11); it represented 33.3% of the IP in Gp (1/3), and 50% of the IP in G0 (4/8) (p>0.05). Grade 4 was seen in 16.6% cases of EVA (3/18); there were no Grade 4 observed in Gp (0/10), and 37.5% cases of EVA in G0 (3/8) (p=0.04). Grade 4 was observed in 100% case in CC in the G0 (1/1). Severe complications were misplacement of the electrode in one case (G0), persistent leakage in one case (G0) and meningitis in one case (Gp). Vertigo was observed in 29.7% of cases (11/37) in this population, 72.7% of them in G0 (8/11). Vertigo was associated to severe dysplasia in 75% cases in G0 (6/8), and to EVA in all cases in Gp. In G0, mean perceptive scores showed for G0, preoperatively and at 3 months, respectively, 1.3% and 50.6% in closed set word (CSW), and 65.9% in open set words at 2 years. In Gp mean perceptive scores showed preoperatively and at 3 months, respectively 6.1% and 69.8% in CSW, and 81% in open set words at 2 years. The differences between the two groups are not significant (p>0.05). Osmotherapy is known to be effective for cerebral oedema and regularly used in neurological surgery. In inner ear malformation, gusher at surgery is directly related to the intra-cerebral pressure (ICP). Corresponding to neurosurgical practice, the mainstay of our protocol rests on hyperosmolar treatment, to reduce the ICP the time of the surgery. Our results suggest that this treatment is effective for a better control of leakage at cochleostomy on EVA, and could be effective on more severe malformations. No severe complication related to surgery was seen in Gp. Its good tolerance could allow its use in most patients with inner ear malformation. Vertigo was a frequent complication. The possibility of vertigo depends on the initial vestibular status and on the course of the surgery. The protocol could protect the vestibular function, lowering the pressure and quantity of the liquid issue. The treatment does not seem to influence the perceptive results.     

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.