人工耳蜗植入术在内耳畸形治疗中的进展

先天性内耳畸形是儿童感音神经性聋的主要病因,临床上多采用配戴助听器及手术进行治疗,而人工耳蜗植入术是目前治疗先天性内耳畸形导致重度感音神经性聋的最佳方法。本文就人工耳蜗植入术在先天性内耳畸形导致的感音神经性聋中的应用及价值做一综述。     

先天性重度、极重度感音神经性聋患儿内耳及颅脑影像学评估

目的探讨先天性重度、极重度感音神经性聋患儿中内耳畸形及颅脑异常的发病情况,为人工耳蜗植入适应证的选择提供参考。方法对确诊为先天性重度、极重度感音神经性聋的245例患儿进行影像学检查,其中232例行颞骨高分辨率CT检查,204例行内听道MRI检查,196例行常规颅脑MRI检查,分析内耳结构异常及颅脑异常患儿的影像表现。结果行颞骨高分辨率CT扫描的232例中,195例内耳正常,37例内耳异常,占15.95%;行内听道MRI检查的204例中,167例正常,37例异常,占18.14%;行颅脑MRI平扫的196例中,110例正常,86例异常,占43.88%。耳蜗畸形包括:Michel畸形3例4耳,耳蜗未发育4例7耳,耳蜗发育不全2例3耳,不完全分隔Ⅰ型5例8耳,不完全分隔Ⅱ型5例10耳。前庭畸形16例26耳,半规管畸形14例24耳;内听道畸形7例12耳;前庭导水管畸形16例29耳。颅脑异常者包括:脑白质病61例,颅内发育变异9例,脑先天畸形7例,脑损伤、破坏性疾病6例,颅骨异常2例及松果体囊肿1例。结论本组先天性重度或极重度感音神经性聋患儿内耳畸形发生率为15.10%(37/245),颅脑异常发生率为43.88%(86/196),术前常规进行颞骨高分辨率CT、内听道MRI及颅脑MRI检查能够发现内耳及颅脑病变,可作为人工耳蜗植入适应人群的筛选及植入耳选择的参考。     

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     

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。     

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 2001年1月至2003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Nucleus24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Common Cavity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。     

内耳畸形的分类与人工耳蜗植入研究

内耳畸形是导致先天性感音神经性聋的主要病因之一。伴随着医学影像技术的发展，大量内耳畸形被发现，而不同内耳畸形进行人工耳蜗植入的术后效果明显存在差异。本文重点对内耳畸形的分类及内耳畸形与人工耳蜗植入的相关问题做一综述。     

语后聋成人人工耳蜗植入对于音乐感知的影响的研究

语后聋成人耳聋患者在接受人工耳蜗植入后在音乐感知方面远不如其听力及言语方面的提高令人满意。人工耳蜗植入是目前治疗重度-极重度感音神经性聋的重要手段,其在音乐感知方面的不理想在近年来人工耳蜗的研究中比较深入。本文通过综述近年来国内外相关文献,探讨语后聋成人接受人工耳蜗植入后对音乐感知的现况及分析其可能影响因素,以及可能的方法以助于音乐感知的提高。     

MRI内耳水成像在人工耳蜗植入电极的应用价值

目的 了解耳蜗的膜迷路结构。为人工耳蜗手术檀入电极做好术前准备。方法 分析11例双耳极重度感音神经性耳聋的MRI内耳水成像圈像。使用GE Signa Infinity TwinSpeed 1．5T扫描仪，采集内耳所在区域内水信号。送AW4．0工作站进行圈像的后处理。结果 双耳极重度感音神经性耳聋10例显示耳蜗的膜迷路正常，1例双侧耳蜗只显示一圈半，且耳蜗直径缩小。诊断为耳蜗骨化。结论 MRI内耳水成像能清晰显示耳蜗的膜迷路，为人工耳蜗植入电极提供保障。     

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。     

梅尼埃病致双侧极重度感音神经性聋行人工耳蜗植入一例

由于梅尼埃病具有发作性眩晕、波动性进行性听力下降、耳闷塞感及不同程度耳鸣等症状，人工耳蜗植入后不仅要解决听觉恢复的问题，还必须考虑前庭功能恢复，特别是眩晕的情况。另外临床上双侧极重度感音神经性聋的双侧严重梅尼埃病发生率也较低。我们介绍1例梅尼埃病致双侧极重度感音神经性聋的患者行人工耳蜗植入的情况，探讨梅尼埃病行人工耳蜗植入的适应证、术前术后处理等情况。     

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。     

Contraindication for cochlear implantation in Wildervanck's syndrome

A case of 4-year-old boy with Wildervanck's syndrome and associated profound sensorineural hearing loss is presented. The child was disqualified from cochlear implantation following the CT scan of petrous pyramids which revealed inner ear malformation. The authors emphasize role of CT imaging in the management of profound sensorineural hearing loss in children before final qualification for cochlear implantation.     

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。     

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.     

"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。     

Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome

This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.     

Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome

This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.     

Intraoperative CT-guided cochlear implantation in congenital ear deformity

Abstract Conclusions: Intraoperative computed tomography (iCT)-guided cochlear implantation is practical and effective for correct electrode placement in the cochlea of patients with congenital inner ear and/or complex middle ear malformation. Objectives: The operation in patients with inner ear and/or complex middle ear malformation including abnormal facial nerve course is difficult. This study evaluated the efficacy of cochlear implantation under the guidance of iCT to insure correct electrode placement. Methods: This was a prospective interventional case series. Ten patients with severe to profound sensorineural hearing loss due to ear malformations were enrolled, and iCT was used to confirm the right placement of electrodes. Results: Intraoperative CT was performed three times in one patient, twice in two, and once in the others. Interruption of the surgical process for each iCT until resumption of surgery was 9.64 ± 0.63 min. iCT revealed incorrectly positioned cochlear implants in two patients, which were immediately corrected. There were no reoperations due to misplacement of electrodes. iCT helped locate the cochlea in the middle ear of one patient with an abnormal facial nerve course. The overall intervention rate based on iCT findings was 30%. Level of evidence: level 4.     

Successful cochlear implantation in a child with Keratosis, Icthiosis and Deafness (KID) Syndrome and Dandy-Walker malformation

Keratosis, Icthiosis, and Deafness (KID) Syndrome is a rare congenital disorder associated with dominant connexin 26 mutations, affecting epidermis, corneal epithelium, and inner ear. Given eventual visual impairment, cochlear implantation is an important consideration despite an increased risk of wound complications. We present a child with KID Syndrome and bilateral profound sensorineural hearing loss associated with a novel heterozygous missense D50A connexin 26 mutation (c.149A > C). Imaging revealed mild cochlear malformation and Dandy-Walker malformation. She received a cochlear implant at the age of 12 months, using a small incision approach. Following an immediate minor wound infection, implantation has been successful without further complication over 4 years.     

Cochlear implantation in children

Cochlear implantation has revolutionized the treatment and prognosis of children with severe to profound sensorineural hearing loss who receive limited benefits from hearing aids. Children who receive cochlear implantation at young age, in particular before 2 years of age, can be expected to reach their normal age-equivalent developmental milestones and have higher chance to integrate into the mainstream educational settings. With the positive outcomes after cochlear implantation and the improvements in technology and surgical techniques, candidacy for cochlear implantation in children has been expanding to include hearing-impaired children with significant residual hearing, severe inner ear malformations, multiple handicaps such as mental retardation or visual impairment, and auditory neuropathy. Furthermore, there is growing interest in offering bilateral cochlear implantation to give children the benefits of binaural hearing. As the candidacy criteria expand, cochlear implant programs including preoperative evaluation, surgery, and habilitation have become more complex. Therefore, candidates should be selected prudently by multidisciplinary approach and cochlear implantation in children is much better to be provided by experienced cochlear implant team consisting of experts in relevant fields for the best results.