微创人工耳蜗植入

摘要：人工耳蜗植入已经成为重度感音神经性耳聋的有效治疗手段,微创理念已被医患广泛接受并重视。本文通过文献回顾微创理念的不同层面探讨人工耳蜗植入效果影响因素,希望通过良好的设计和规范化实施,显著降低手术并发症、保留残余听力,寻找最合理和微创的手术方法,推动人工耳蜗植入工作不断发展。     

人工耳蜗及相关技术的进展

人工耳蜗植入(cochlear implantation, CI)是重度-极重度感音神经性聋患者的听觉和言语康复的主要方法, 随着人们对听觉系统和中枢可塑性的深入研究,手术技术及辅助工具的开发,以及人工耳蜗植入相关电子科技和材料科学的发展,人工耳蜗及相关技术有了很大的进步。本文分析人工耳蜗植入体,尤其是电极设计和言语处理器的升级、人工耳蜗植入适应证的拓宽、术前评估手段的完善及精准微创人工耳蜗植入和残余听力保留的开展,并对人工耳蜗植入相关技术进行展望。     

脑膜炎后耳蜗骨化患者人工耳蜗植入的临床分析

目的总结因脑膜炎而致严重感音神经性听力损失的人工耳蜗植入经验,探讨其诊疗策略。方法回顾性分析2010年9月—2020年9月于首都医科大学附属北京同仁医院耳鼻咽喉头颈外科因脑膜炎致重度及极重度感音神经性听力损失进行人工耳蜗植入的22例患者资料,其中成人13例,儿童9例。对其术前影像学、手术所见进行分析。结果颞骨HRCT检查中16例(72.7%)诊断耳蜗骨化;6例(27.3%)未诊断,但经手术探查证实存在耳蜗骨化。18例内耳MRI检查中,3例(16.7%)未发现耳蜗信号改变,而经手术探查存在耳蜗骨化。两者结合后检出率为90.9%(20/22),20例(90.9%)可见合并半规管等其他迷路病变。术中未见耳蜗骨化者1例(4.5%),圆窗骨化7例(31.8%),耳蜗底转骨化14例(63.6%)。电极完全植入者18例(81.8%),部分植入者4例（18.2%）。结论脑膜炎后耳蜗骨化可致严重感音神经性听力损失,人工耳蜗是理想的治疗策略,建议尽早植入。术前颞骨HRCT和MRI对确定是否适合耳蜗植入和术前计划至关重要,诊断存在假阴性,但联合检查可大大提高耳蜗骨化的术前诊断阳性率。     

人工耳蜗在前庭水管扩大患者中的应用

目的 研究前庭水管扩大患者人工耳蜗植入手术的经验。方法 对15例(15耳)双侧前庭水管扩大伴重度感音神经性聋的患者行多导人工耳蜗植入术，并对术后结果进行随访。经面隐窝径路行人工耳蜗植入术，在圆窗龛前下方行鼓岬钻孔，植入多导人工耳蜗电极。结果 ①术中电极完全插入耳蜗，其中6耳出现轻微井喷；②术后无面瘫、脑脊液耳漏等并发症；③所有患者人工耳蜗植入术后都有听觉；④术后听阈与耳蜗结构正常植入患者无明显区别(自由声场平均听阈30dBHL)；⑤术后语训在北京同仁医院或中国聋儿康复中心进行，听力及言语能力均有不同程度提高，其中2例患者已经进人正常学校就读。结论 前庭水管扩大伴重度感音神经性聋的人工耳蜗植入与耳蜗结构正常患者植入术后效果基本一致，人工耳蜗植入可以作为前庭水管扩大伴有重度感音神经性聋的治疗和康复手段。     

人工耳蜗在老年性聋患者中的应用

目的随着社会人口老龄化,听力障碍在老年人群中的发病率也逐年增加,老年性聋引起的沟通障碍会给老年人群带来生活及心理上的变化,严重影响了老年人的生活质量。目前老年性聋的干预措施较多,其中重度及极重度感音神经性老年性聋患者可选择人工耳蜗植入,本文就老年性聋人工耳蜗植入的选择标准、术前评估、术后并发症、术后康复及效果评估等进行综述。     

感音神经性聋并分泌性中耳炎患者一期人工耳蜗植入

目的探讨感音神经性聋并慢性分泌性中耳炎患者一期人工耳蜗植入的可行性。方法对就诊于中山大学孙逸仙纪念医院耳鼻咽喉科的2例慢性分泌性中耳炎并双侧极重度感音神经性聋患者行一期人工耳蜗植入术,观察植入效果及听力言语康复情况。结果 2例患者术中均顺利植入电极,术后随访均无分泌性中耳炎复发,听力言语能力恢复效果好。结论对于合并慢性分泌性中耳炎的感音神经性聋患者,在清除病变的基础上,同期行人工耳蜗植入是可行的。     

老年性聋人工耳蜗植入进展

目的耳聋是全球第三大导致老年人残疾障碍的原因，不仅仅影响声音的感知，还会引发一系列日常交流和社交障碍。目前，人工耳蜗植入是重度 极重度感音性聋且助听器效果不佳老年人的听觉和言语康复的最有效方法，但众多原因导致我国老年耳聋患者人工耳蜗植入开展得不够，并且老年人本身耳聋发生及其听力学和听觉康复有其特殊性。本文从老年耳聋的发生率、老年性聋的病因、人工耳蜗植入的适应证及疗效特点等方面进行分析和讨论，并对老年耳聋患者人工耳蜗植入的未来前景做展望。     

人工耳蜗植入效果的相关因素分析

人工耳蜗植入（cochlear implantation,CI） 是治疗重度或极重度感音神经性聋的有效方法,然而手术效果受到诸多因素影响,这些影响因素一直以来都是关注焦点。在植入前对人工耳蜗植入术后效果进行准确的预测,可为患儿家长、听力师及临床耳外科医师选择治疗方式及时机提供重要的参考依据。     

人工耳蜗植入患者双耳聆听的相关研究进展

人工耳蜗植入被广泛应用于治疗助听器无效或效果不理想的双侧重度或极重度感音神经性耳聋患者。近年来随着人们对聆听质量要求的提高,患者常采用非植入侧佩戴助听器的双耳双模式(Binaural-Bimodal Fitting, BIM)或双侧人工耳蜗植入(Bilateral cochlear implantation, BCI)等方法以获得双耳聆听。同时对于单侧聋(Single-sided deafness, SSD)患者来说,人们也开始尝试为其植入人工耳蜗以达到双耳聆听的效果。本文在回顾近些年文献的基础上,对于双耳聆听的优势、单侧聋患者的人工耳蜗植入、双侧感音神经性耳聋的双耳双模式聆听以及双侧人工耳蜗植入的研究进展进行综述。     

伴有中枢神经系统疾病的聋儿人工耳蜗植入

随着人工耳蜗植入适应范围的逐步扩大,越来越多伴有中枢神经系统疾病的极重度感音神经性聋患者接受人工耳蜗植入术。这类患者的术后康复效果与疾病的严重程度等因素密切相关,因此需要经过充分的术前评估和全面观察,从而保证实施的人工耳蜗植入可以获得满意的效果。     

Cochlear nerve size evaluation in children with sensorineural hearing loss by high-resolution magnetic resonance imaging

PURPOSE: To determine differences in size of cochlear nerves among subjects with deafness due to connexin 26 (Cx26) mutations, subjects with deafness of unknown origin, and normal hearing subjects by sagittal high-resolution magnetic resonance (HRMR) imaging of the temporal bone. MATERIALS AND METHODS: Cross-sectional and surface areas and volumetric measurements of the cochlear nerve and modiolus were made on HRMR images of the internal auditory canal (IAC) and inner ear in the 3 groups of children (groups 1, 2, and 3). Three-way comparisons of in vivo cochlear nerve measurements on HRMR imaging were made among 17 children with sensorineural hearing loss (SNHL) and no obvious etiology for the hearing loss (group 1), 7 children with profound SNHL due to a Cx26 mutation (group 2), and 10 normal hearing children (group 3). RESULTS: Children with profound SNHL of unknown cause and children with profound SNHL due to a connexin mutation displayed hypoplastic cochlear nerves as compared with normal controls. HRMR imaging of the temporal bone was accurately delineated potential problems with cochlear nerves in 2 of 17 instances where high-resolution computed tomography did not do so. CONCLUSIONS: Accurate and specific measurements of the cochlear nerve and related structures is possible on HRMR imaging of the temporal bone. The size of the cochlear nerve is mildly hypoplastic in children with profound SNHL of unknown causes or children with a deafness-causing Cx26 mutation. HRMR imaging is superior to high-resolution computed tomography in the investigation of profound SNHL in children.     

Recent epidemiology of pediatric cochlear implantation in the United States: disparity among children of different ethnicity and socioeconomic status

OBJECTIVES/HYPOTHESIS: Congenital severe to profound sensorineural hearing loss (SNHL) is found in higher proportions of children with minority and/or lower socioeconomic status (SES). Cochlear implants were approved by the U.S. Food and Drug Administration for use in children with bilateral severe to profound SNHL in 1990. The objectives of the study were as follows: 1) to study the epidemiology of pediatric cochlear implantation, assessing whether cochlear implant technology is provided to children with severe to profound SNHL in proportion to their racial/ethnic or SES, and 2) to compare data provided by a national health care database with data provided by cochlear implant manufacturers. STUDY DESIGN: Patients aged 0 to 18 years who underwent cochlear implantation in 1997 using a cross-sectional study design. METHODS: Analyses were made of pediatric cochlear implant patients, using data from the 1997 Health Care and Utilization Project/Kids' Inpatient Database. Relative rates of implantation compared with rates of severe to profound SNHL were calculated using national estimates generated from census and Galludet Research Institution data. Logistic regression analysis was carried out to compare implanted children of different racial/ethnic backgrounds. A surrogate measure of socioeconomic status was used based on the median household income of the patient's home ZIP code. Information was also obtained from the two companies producing U.S. Food and Drug Administration-approved cochlear implants in 1997 and used to determine whether the data obtained from the Health Care and Utilization Project/Kids' Inpatient Database were representative of the national cohort of implanted children. RESULTS: The Health Care and Utilization Project/Kids' Inpatient Database identified 124 children who underwent cochlear implant surgery in 1997. White and Asian children were implanted at higher rates than Hispanic and black children. Furthermore, white and Asian children received implants at greater rates than would be expected based on prevalence of severe to profound SNHL. The relative rate (RR) of implantation, defined as the proportion of children who received cochlear implants divided by the proportion of children with severe to profound SNHL (in each race/ethnicity group compared with the same ratio in white children), was similar in white (RR = 1.00) and Asian (RR = 0.93) children but markedly different in Hispanic (RR = 0.28) and black (RR = 0.10) children. Comparison of SES information from the Health Care and Utilization Project/Kids' Inpatient Database population with the manufacturers' database suggested that the Health Care and Utilization Project/Kids' Inpatient Database is representative of all implanted children in the United States. Both sources of information suggested that children receiving cochlear implants in the United States in 1997 resided in above-average SES areas. CONCLUSION: White and Asian children with severe to profound SNHL had higher proportionate rates of cochlear implantation than black and Hispanic children in 1997. Implanted children were more likely to live in areas (represented by ZIP codes) with higher median incomes. Although there was a disparity in rate of cochlear implantation based on race/ethnicity and surrogate measures of SES, these data did not allow the authors to determine the causes for these differences.     

Cochlear implantation in the chronically diseased ear

PURPOSE OF REVIEW: The presence of chronic ear disease in patients with profound SNHL presents a challenge to the otologic surgeon and was considered, in the past, a contraindication to cochlear implantation. This review discusses options for cochlear implantation in patients with chronically diseased ears. RECENT FINDINGS: Several management options are available for cochlear implantation in patients with chronic suppurative otitis media (CSOM) and severe to profound sensorineural hearing loss (SNHL). CONCLUSION: Cochlear implantation can be safely achieved in patients with CSOM. The approach chosen should be individualized based on clinical findings.     

Cochlear implantation in a human immunodeficiency virus-infected patient

OBJECTIVES/HYPOTHESIS: Patients infected with HIV have an increased risk of developing sensorineural hearing loss (SNHL), yet pathogenesis of SNHL in HIV infection is still poorly understood. In subjects affected by bilateral profound or total SNHL, cochlear implantation may be the only possibility to restore a hearing level that allows them to have an acceptable quality of life. STUDY DESIGN: Case report. METHODS: A retrospective chart review of a HIV type 1-seropositive profoundly deafened patient who underwent cochlear implantation. RESULTS: To date, with a follow-up of 4 years, the patient has not experienced any complication and has regained useful open-set speech perception. CONCLUSIONS: Cochlear impairment with preserved auditory pathways can be responsible for profound SNHL in HIV-infected patients. Cochlear implantation can restore a social hearing in these patients, dramatically improving their quality of life. The surgical procedure can be safely performed when keeping in mind that the general condition of the patient is the decisive factor for or against surgery.     

耳蜗死区对感音神经性听力损失患者言语识别能力的影响

目的 探究耳蜗死区在感音神经性听力损失(sensorineural hearing loss,SNHL)患者中的存在情况及其对言语识别能力的影响.方法 采用纯音听阈测试筛选出41例(81耳)感音神经性听力损失患者,经均衡噪声阈值测试将患者分为有耳蜗死区组(35耳)和无耳蜗死区组(46耳),分别进行言语识别阈(SRT)和言语识别率(SDS)测试,分析81耳耳蜗死区的分布及其对言语识别能力的影响.结果 41例(81耳)感音神经性听力损失患耳中有35耳(43.21％,35/81)存在耳蜗死区,其中轻度SNHL患耳耳蜗死区检出率为0(0/11),中度SNHL患耳耳蜗死区检出率为24.1％(7/29),重度SNHL患耳耳蜗死区检出率为66.7％(24/36),极重度SNHL患耳耳蜗死区检出率为80.0％(4/5),不同听力损失程度耳耳蜗死区检出率差异有统计学意义(P＜0.05);高频耳蜗死区(16耳)明显多于低频耳蜗死区(8耳),但两者的言语识别能力差异无统计学意义(P＞0.05);有耳蜗死区患者的言语识别阈及言语识别率分别为61.63±16.76 dB HL,86.35％±12.03％,无耳蜗死区的患者分别为75.54±9.56 dBHL,64.97％±20.84％,二者间差异有统计学意义(P＜0.05).结论 听力损失越重,耳蜗死区检出率越高;高频耳蜗死区较低频常见,且存在耳蜗死区的感音神经性听力损失患者言语识别能力明显低于无耳蜗死区的患者.     

Contemporary surgical issues in paediatric cochlear implantation

Abstract

Objective: To review the contemporary surgical issues in paediatric cochlear implantation (CI) based on published evidence. Design: Narrative literature review. Results: Surgical challenges in paediatric CI are discussed, with respect to post meningitic labyrinthitis ossificans; cochlear malformation; cochlear implantation in infants; auditory neuropathy and cochlear nerve deficiency; bilateral cochlear implantation; hearing preservation; otitis media; and device failure. Conclusion: Early CI is recommended if bacterial meningitis causes profound sensorineural hearing loss (SNHL). CI in cochleovestibular malformation requires pre-operative imaging to plan surgical technique, choice of electrode, and to anticipate complication. Children with congenital severe to profound SNHL should undergo early bilateral simultaneous implantation, preferably before 12 months of age, except those with auditory neuropathy spectrum disorder who should be implanted after one year. Soft surgical technique should be deployed in an attempt to preserve any residual hearing. Otitis media with effusion is not a contraindication to cochlear implantation, but active or recurrent acute otitis media requires resolution of infection with grommet insertion pre-operatively. Device failure in CI recipients requires a stepwise audiological, medical, radiological, and device integrity assessment to determine the need for reimplantation.     

Inner ear anomalies in cochlear implantees: importance of radiologic measurements in the classification.

OBJECTIVE: To classify the inner ear anomalies of cochlear implantees with profound sensorineural hearing loss. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: For the normative data of the inner ear structures, the temporal bone computed tomography of 60 patients (120 ears) with normal bone-conduction threshold (< 15 dB) were used, and the data were applied to 570 ears of 285 cochlear implantees. INTERVENTIONS: Predesignated inner ear structures were measured in temporal bone computed tomography images from the normal and cochlear implantation groups using a computer-based caliper that formed part of a picture archiving and communication system. MAIN OUTCOME MEASURES: The inner ear anomalies were defined when the structures presented visually obvious malformations or the measurements deviated 2 standard deviations from the means in the normative data. RESULTS: The application of normative data to 570 profound SNHL ears resulted in the identification of 293 individual anomalies in 127 anomalous ears. An enlarged vestibular aqueduct was the most common individual anomaly (49 cases), followed by vestibular enlargement (38 cases) and other semicircular canal dysplasia (37 cases). When the individual anomalies were reaccounted according to the more prominent anomaly where multiple anomalies were present in each ear, incomplete partition type II was the most common (34 ears), followed by cochlear hypoplasia (22 ears) and incomplete partition type I (20 ears). CONCLUSION: We suggested a measurement technique for the inner ear structures using computed tomography and derived normative measurements helpful for diagnosing inner ear anomalies. Using these normative data, we classified the inner ear anomalies of profound SNHL ears in cochlear implantees.     

1例耳蜗前庭神经畸形患者耳蜗植入术前的功能性磁共振评估并文献复习

目的:探讨功能性磁共振(fMRI)评价耳蜗前庭神经畸形患者是否有人工耳蜗植入指征的可能性。方法:对1例15岁双侧耳聋患者,在人工耳蜗植入前进行听力学、影像学检查及fMRI综合评估。结果:纯音测听和听觉脑干反应显示患者为双侧极重度感音神经性聋,瞬态诱发性耳声发射双侧均未引出。颞骨薄层CT显示内耳畸形(不全分隔Ⅰ型),MRI斜矢状位重建显示耳蜗前庭神经畸形。fMRI显示左耳给声,1000Hz100dBHL,右侧听皮层被激活;右耳给声,双侧听皮层均未见激活。左侧人工耳蜗植入术后1个月开机,患者对声音有反应。结论:结合听力学和影像学检查,fMRI可评估人工耳蜗植入候选者,尤其是在影像学检查显示患者耳蜗前庭神经发育畸形时,更是一种有效的评估方法。     

儿童人工耳蜗植入年龄下限降至9月龄的启示与应对

食品与药品管理局(FDA)批准将儿童人工耳蜗植入年龄下限降至9月龄表明：有必要更早地植入人工耳蜗，以收获聋儿植入后更佳的听力、言语和语言康复成效。在低龄婴幼儿中进行人工耳蜗植入，离不开一批训练有素的医护及麻醉团队提供围手术期支持，对诊断、麻醉风险、外科技术、术中测试和术后编程、长期安全等方面提出了更高要求。同时也对婴幼儿听力学诊断、干预和评估带来了挑战。9~24月龄儿童植入人工耳蜗的指征仍为双侧极重度感音神经性听力损失，围绕“先天聋儿植入人工耳蜗的指征和时机”“植入前是否试配助听器”等话题，耳外科专家与听力学专家应携起手来，审慎、稳妥、积极地开展低龄儿童人工耳蜗植入的循证医学研究。