听神经发育异常合并中耳内耳畸形及面神经畸形的人工耳蜗植入术1例北大核心CSCD

听神经发育异常患者人工耳蜗植入效果个体差异较大,同时合并中耳内耳畸形及面神经畸形者较少见,可对手术造成一定困难。本文介绍1例听神经发育异常合并中耳内耳畸形及面神经畸形患者的人工耳蜗植入,通过其临床表现、听力学、影像学检查,以及手术经过,结合文献分析听力学、影像学等检查对于听神经发育异常患者的术前评估作用。     

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。     

人工耳蜗植入术25例报告

目的：探讨人工耳蜗植入术的方法及疗效。方法：行人工耳蜗植入术25例,男16例,女9例,1—36岁,其中语前聋24例,语后聋1例,语前聋中经CT和MRI证实Mondini畸形1例,前庭导水管扩大3例,耳蜗部分骨化1例。术前行听力学检查,短声ABR阈值均〉100dB,畸变产物耳声发射未引出,经乳突后鼓室径路行人工耳蜗植入术,植入24型K、M型人工耳蜗（澳大利亚产）11例,24contour型14例,术后1月开机调试及跟踪随访。结果：25例均一次植入成功,2例术中脑脊液涌出,包括Mondini畸形、前庭导水管扩大各1例,3例术后出现轻微眩晕,5d后消失,出院前行X线耳蜗电极定位,电极均在耳蜗内,开机调试后患者均能听到声音。结论：为保证植入手术的成功,除手术前严格掌握手术指征外,术中正确处理相关情况也极为重要。     

人工耳蜗植入术中因内耳畸形发生“井喷”的危险因素分析

目的 分析人工耳蜗植入术治疗内耳畸形中发生“井喷”的危险因素。方法 回顾性分析湘南学院附属医院耳鼻咽喉头颈外科及中南大学湘雅二医院耳鼻咽喉科2010年1月～2015年10月收治的25例内耳畸形行人工耳蜗植入术患者的病例资料。结果 8例患者术后出现“井喷”,其中植入右侧6例、左侧2例。畸形类别：双侧耳蜗内听道相通伴Mondini畸形或前庭导水管扩大分别为4例、2例,双侧耳蜗内听道不相通+前庭导水管扩大1例,右侧耳蜗内听道相通+左侧Michel畸形右侧Mondini畸形1例。25例患者术后均获得满意听力。结论 内耳畸形中前庭导水管扩大、Mondini畸形和耳蜗听道相通是人工耳蜗植入术中的常见危险因素,术前需行影像学检查,全面评估“井喷”发生风险,降低其发生率,确保手术效果及人工耳蜗听觉建立。     

蜗神经发育不良患者的人工耳蜗植入

目的:观察蜗神经发育不良的患儿人工耳蜗植入术后的效果,探讨术前HRCT和MRI对蜗神经发育不良的诊断及评估价值。方法:回顾性分析10例蜗神经发育不良患儿的术前影像学资料及人工耳蜗植入术后对声音的反应和语训效果。结果:3例(6耳)HRCT提示双侧内耳道狭窄,耳蜗、前庭和半规管发育正常,MRI提示蜗神经发育不良。7例(14耳)HRCT提示双侧内耳道正常,其中1例IP-Ⅰ型,伴前庭半规管发育不良,6例双侧耳蜗、前庭和半规管发育正常;7例MRI提示蜗神经发育异常。10例患儿均行人工耳蜗植入,经过12～32个月的随访,3例学会简单语言,MAIS量表、CAP分数及SIR分级较前提高;1例虽能讲话,但口齿含糊;4例对声音有反应,仅能说"爸爸"、"妈妈"等单音节词;2例无反应(1例取出)。10例蜗神经发育不良患儿同其他蜗神经发育正常儿童相比,术后康复情况明显落后。结论:对蜗神经缺陷患儿,术前仅做颞骨HRCT检查易漏诊,结合内耳道MRI(含内耳道斜矢状位重建)已成必须。若发现疑似病例,应慎重筛选,告知行人工耳蜗植入效果不佳的巨大可能性,严格掌握手术指征。     

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。     

先天性外耳道骨性闭锁患者人工耳蜗植入的手术分析

目的探讨先天性外耳道骨性闭锁行人工耳蜗植入患者的听力学检查、影像学特征及手术径路的选择。方法收集2015年7月—2019年1月诊治的5例先天性外耳道骨性闭锁行人工耳蜗植入术的患者,回顾性分析其病史、听力学检查、影像学特征及手术径路。结果5例患者听力均表现为重度感音神经性聋。影像学表现为外耳道骨性闭锁,伴听骨链畸形,面神经走形异常,均无内耳畸形。5例患者均采用鼓窦径路,其中2例患者经面神经后下植入人工耳蜗。所有患者均为圆窗膜植入,植入电极过程顺利,术后开机反应佳。结论针对外耳道骨性闭锁畸形需行人工耳蜗植入的患者,应采用外耳道骨性闭锁的鼓窦径路的手术方式,开放部分乳突及鼓窦,取出畸形的听骨链,在此基础上,进一步暴露圆窗龛。对于面神经乳突段前移,完全遮挡圆窗龛的患者,则可转经面神经后下暴露圆窗龛。     

耳蜗性耳硬化症人工耳蜗植入的应用研究

分析耳蜗性耳硬化症患者的临床特点、人工耳蜗植入术中情况及人工耳蜗植入术后听觉言语康复效果,为该疾病的诊治提供参考。方法分析4例耳蜗性耳硬化症患者病史资料、听力学检查结果及影像学结果,观察人工耳蜗植入手术的术中所见,并定期对4例患者进行听力学及影像学随访。结果①所有患者人工耳蜗电极均经圆窗膜径路完全植入鼓阶。其中1例于术中发现镫骨完全固定,导致术中鼓阶开孔时外淋巴波动不明显;1例发现圆窗膜骨化,术中鼓阶开孔定位困难;其余2例患者镫骨活动好,圆窗结构清晰。所有患者术中电极阻抗检测均正常,且引出标准的神经反应遥测波形;②术后随访1~5年无术后并发症出现,声场测听示平均听阈为40.8 dBHL,平均言语识别率为77.3%,言语及交流能力较术前提高;颞骨高分辨率CT提示双侧内耳病变范围无明显进展。结论耳蜗性耳硬化症进展缓慢,严重时可导致重度/极重度感音神经性聋,当使用助听器无效时,人工耳蜗植入能帮助患者获得较满意的听觉康复效果。     

小儿专科医院开展人工耳蜗植人手术的优势

目的通过本组6家小儿专科医院开展人工耳蜗植入手术的临床工作,讨论小儿专科医院开展人工耳蜗植入手术的优势。方法从1998年5月-2009年3月11年间,本组6家小儿专科医院耳鼻咽喉科共开展了103例人工耳蜗植入手术。术前信息：全部为双耳重度和极重度感音神经性聋,年龄8．5个月-10．2岁,平均年龄为3．8岁,全部为语前聋患儿。术前进行听力学评估、发育和智力评估、影像学评估、人工耳蜗植入适应证的评估。术前进行全麻风险的评估。进行与综合性医院开展人工耳蜗植入手术的优势比较,包括：①术前评估水平,②术中麻醉风险,③人工耳蜗植入手术结果,④围手术期护理和并发症处理水平。结果①术前评估：术前视觉强化行为测听检查：103例查出有残留听力49例,平均听力98dB,余54例无残留听力。畸变产物耳声发射检查：103例均未引出耳声发射。听觉脑干诱发电位、40Hz、多频稳态诱发电位检查：引出波形41例,未引出波形62例。术前影像学检查：颞骨CT检查103例中正常颞骨形态84例,内耳畸形19例,其中双耳大前庭导水管畸形ll例、Mondini畸形7例、前庭与外半规管共同腔畸形1例。脑常规MRI检查,正常98例、脑白质轻度异常5例。行为和智力测试：格雷费斯智力测试〉86分99例、〈86分4例。术前检查结果与综合性医院检查结果比较,无显著差异。②术中麻醉风险：全麻插管、拔管中出现心电、氧、二氧化碳监测问题、气管支气管痉挛并发症0例,综合性医院发生率为0．37％,有明显的安全优势。③人工耳蜗植入手术结果：103例手术均成功,与综合性医院比较,无明显差异。④围手术期护理与并发症的处理：103例术前常规静脉采血、术中,术后常规建立静脉通道未出现失败,综合性医院出现静脉采血、建立静脉通道失败率约5%右,?     

多导人工耳蜗植入术后耳蜗X线显像

目的 探索适合于评价多导人工耳蜗植入手术效果的术后影像学检查技术。方法 多导人工耳蜗植入术69例69耳,其中Nucleus直电极53耳,Nucleus弯电极11耳,Nucleus22导5耳。手术采用耳后面神经隐窝入路。建立放射线耳蜗显像技术,并且所有多导电子耳蜗植入病例术后7-10天均行耳蜗位头颅X-线检查。结果 在69例69耳手术中均成功地植入多导人工耳蜗电极。术后1.0-1.5月进行开机调试,听阈和言语分辨率均达到比较满意的水平。耳蜗位头颅X-平片显示上半规管和前庭易于辨认,可以作为影像学解剖的重要标志。耳蜗X线显像可以直接而且清晰地显示一个完整的人工耳蜗植入术后的电极在耳蜗内的图像,并且每一个电极阵列都可以很清楚地被识别。影像学检查可以作为植入手术结果的客观资料,而且如果电极扭曲或术后移位很容易发现。对于内耳畸形人工耳蜗植入术后,耳蜗位X-线检查则更现必要,是判断手术结果的重要依据。结论感音神经性耳聋患者植入多导人工耳蜗效果良好。头颅X-线耳蜗显像可以应用于对多导人工耳蜗植入术后结果的评价,而且简单、低放射量、快速和经济,值得广泛推广。     

fMRI evaluation of cochlear implant candidacy in diffuse cortical cytomegalovirus disease

Congenital cytomegalovirus infection is the most frequent nongenetic cause of pediatric hearing loss in the United States, affecting approximately 8,000 children each year. Due in part to variable cytomegalic involvement of the auditory cortex, cochlear implantation outcomes have varied widely. Functional magnetic resonance imaging (fMRI) has the potential to assist in determining candidacy for cochlear implantation through the detection of intact auditory pathways including the cortex. We report a case of a 21-month-old girl with cytomegalovirus-related deafness and diffuse white matter involvement in which fMRI was a determining factor for cochlear implantation and side selection.     

Functional magnetic resonance imaging of hearing-impaired children under sedation before cochlear implantation

OBJECTIVE: To investigate functional magnetic resonance imaging (fMRI) in pediatric cochlear implantation candidates with residual hearing who are under sedation for evaluation of auditory function. DESIGN: During fMRI, subjects heard a random sequence of tones (250-4000 Hz) presented 10 dB above hearing thresholds. Tones were interleaved with silence in a block-periodic fMRI design with 30-second on-off intervals. Twenty-four axial sections (5 mm thick) covering most of the brain were obtained every 3 seconds for a total acquisition time of 5.5 minutes. SETTING: Single tertiary academic medical institution. PATIENTS: Severely to profoundly hearing-impaired children (n=10; mean age, 49.1 months). During fMRI, subjects were awake (n=2) or sedated with pentobarbital sodium if their weight was 10 kg or greater (n=4) or chloral hydrate if their weight was less than 10 kg (n=4). MAIN OUTCOME MEASURES: Detection of brain activation by fMRI in the primary auditory cortex (A1) in hearing-impaired patients under sedation, and correlation of A1 activation with hearing levels measured after cochlear implantation. RESULTS: In most subjects, fMRI detected significant levels of activation in the A1 region before cochlear implantation. The improvement in hearing threshold after cochlear implantation correlated strongly (linear regression coefficient, R=0.88) with the amount of activation in the A1 region detected by fMRI before cochlear implantation. CONCLUSIONS: Functional MRI can be considered a means of assessing residual function in the A1 region in sedated hearing-impaired toddlers. With improvements in acquisition, processing, and sedation methods, fMRI may be translated into a prognostic indicator for outcome after cochlear implantation in infants.     

Cochlear implantation in patients with auditory neuropathy of varied etiologies

OBJECTIVES/HYPOTHESIS: Auditory neuropathy is a relatively recently described pattern of hearing loss characterized by preservation of outer hair cell function despite absent brainstem auditory evoked responses. Intact outer hair cell function is demonstrated by the presence of otoacoustic emissions and/or a measurable cochlear microphonic on electrocochleography, whereas no synchronous neural activity (absent action potentials) is seen on acoustically evoked brainstem auditory evoked response testing. The study reviews the authors' experience with six patients diagnosed with auditory neuropathy, four of whom have undergone cochlear implantation. MATERIALS AND METHODS: A retrospective review of all medical and audiological charts at the University of Virginia Hospitals (Charlottesville, VA) was performed to identify patients who have undergone cochlear implantation or have been diagnosed with auditory neuropathy, or both. RESULTS: Six patients with hearing loss attributable to auditory neuropathy were identified, four of whom have undergone cochlear implantation. Causes varied, including congenital, infectious, and idiopathic origins. Adults demonstrated subjective auditory perception on promontory stimulation, whereas no repeatable brainstem auditory evoked response waveforms could be demonstrated on pediatric promontory stimulation testing. Patients with implants demonstrated implant-evoked brainstem auditory evoked responses and improved audiological performance. CONCLUSIONS: The six cases presented in the study represent varied causes and, probably, varied sites of lesions of auditory neuropathy. Promontory stimulation has been valuable, particularly in adults. Cochlear implantation allows the opportunity to provide a supraphysiological electrical stimulation to the auditory nerve, with the hope of reintroducing synchronous neural activity. Greater confidence and enthusiasm for cochlear implantation in appropriately selected patients with auditory neuropathy are gained through experience with such diverse cases.     

The case for earlier cochlear implantation in postlingually deaf adults

Abstract

Objective: This paper aimed to estimate the difference in speech perception outcomes that may occur due to timing of cochlear implantation in relation to the progression of hearing loss. Design: Data from a large population-based sample of adults with acquired hearing loss using cochlear implants (CIs) was used to estimate the effects of duration of hearing loss, age, and pre-implant auditory skills on outcomes for a hypothetical standard patient. Study sample: A total of 310 adults with acquired severe/profound bilateral hearing loss who received a CI in Melbourne, Australia between 1994 and 2006 provided the speech perception data and demographic information to derive regression equations for estimating CI outcomes. Results: For a hypothetical CI candidate with progressive sensorineural hearing loss, the estimates of speech perception scores following cochlear implantation are significantly better if implantation occurs relatively soon after onset of severe hearing loss and before the loss of all functional auditory skills. Conclusions: Improved CI outcomes and quality of life benefit may be achieved for adults with progressive severe hearing loss if they are implanted earlier in the progression of the pathology.     

不同月龄婴幼儿人工耳蜗植入术后听觉能力发育规律及特点

目的：总结不同月龄婴幼儿人工耳蜗植入术后听觉能力的发育规律及特点,为婴幼儿患儿人工耳蜗植入年龄的选择及制定合理的术后听力言语康复训练计划提供依据。方法：共有139例极重度语前聋婴幼儿患儿参加本次研究。按手术时年龄将患儿分为A组（≤12个月）、B组（13～18个月）、C组（19～24个月）、D组（25～30个月）、E组（31～36个月）。使用婴幼儿有意义听觉整合量表（IT-MAIS）并由经过培训的听力学专业人员对患儿术后听觉能力进行评估。评估采用面对面询问的方式,由患儿家长或监护人回答。评估人员将家长或监护人对每一问题的回答进行详细记录并根据患儿听觉行为出现的频率对其进行评分。分别在术前和术后开机后1、3、6、12个月时对各组患儿进行评估。结果：经两组独立样本T检验,不同性别患儿在术前和术后不同时期听觉能力的差异均无统计学意义（均P〉0．05）。经单因素方差分析,在术前和开机后1、3、6、12个月各年龄组患儿听觉能力的差异均无统计学意义（均P〉0．05）。各年龄组患儿听觉能力得分随开机时间的增加而增加,其差异有统计学意义（P〈0．05）。结论：不同性别婴幼儿患儿术后听觉能力的发育遵循相同的规律。婴幼儿患儿手术时年龄对术后听觉能力的发育影响不大。大部分患儿的听觉能力在术后6个月之内提高较快,其后进入较缓慢的发展期。     

Cochlear implantation in an adult patient with auditory neuropathy

We present a case report of effective cochlear implantation for an adult patient with auditory neuropathy. A 34-year-old man developed bilateral hearing loss at approximately 10 years of age. His speech discrimination score was very severe despite only moderate sensorineural hearing loss. Absence of auditory brainstem responses (ABR) and preservation of distortion product otoacoustic emissions (DPOAE) were confirmed by our audiological examinations. After cochlear implantation, good responses for electrically evoked compound action potential (EAP) and electrically evoked ABR (EABR) were observed. Postoperatively, his audiological performance was significantly improved. We conclude that cochlear implantation can be a valid option for patients with auditory neuropathy.     

内听道斜矢状位磁共振成像在人工耳蜗置入术前评估中的初步应用

目的:探讨内听道(IAC)斜矢状位MRI在人工耳蜗置入术前评估中的应用价值。方法:应用斜矢状位三维快速自旋回波T2加权MRI技术对15例(30耳)拟行人工耳蜗置入术患者的IAC进行扫描。结果:所有患者顺利完成检查,IAC内神经结构显示清楚。13例蜗神经正常的患者行人工耳蜗置入术并已成功开机,2例双侧蜗神经异常者未行耳蜗置入。结论:IAC斜矢状位MRI能清晰显示IAC内蜗神经的形态、大小及IAC的大小,是人工耳蜗置入术前判断蜗神经发育状况的良好方法。     

Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.     

Effect of age at cochlear implantation on auditory skill development in infants and toddlers

OBJECTIVES: To investigate the effect of age at cochlear implantation on the auditory development of children younger than 3 years and to compare these children's auditory development with that of peers with normal hearing. DESIGN: Using a repeated-measures paradigm, auditory skill development was evaluated before and 3, 6, and 12 months after implantation. Data were compared with previously published data from cohorts with normal hearing. PARTICIPANTS: One hundred seven hearing-impaired children (age range, 12-36 months) who received a cochlear implant during clinical trials in North America. MAIN OUTCOME MEASURE: Auditory skill development was assessed using the Infant-Toddler Meaningful Auditory Integration Scale, a tool that provides a quantitative measure in children as young as newborns. RESULTS: Infants and toddlers who receive implants show rapid improvement in auditory skills during the first year of device use regardless of age at implantation, although younger children achieve higher scores. Children who undergo implantation at a younger age acquire auditory skills nearer to those of their peers with normal hearing at a younger age. The mean rate of acquisition of auditory skills is similar to that of infants and toddlers with normal hearing regardless of age at implantation. CONCLUSION: Performing implantation in children with profound hearing loss at the youngest age possible allows the best opportunity for them to acquire communication skills that approximate those of their peers with normal hearing.     

The effect of cochlear implantation and post-operative rehabilitation on acoustic voice analysis in post-lingual hearing impaired adults

Post-lingual deaf adults can develop some vocal abnormalities similar to those developed in pre-lingual deaf individuals. The aim of this work was to study the effect of cochlear implantation followed by post-operative rehabilitation on voice acoustics in post-lingual hearing impaired adults with different durations of hearing loss. The study included 35 post-lingual hearing impaired adults who underwent cochlear implantation. Patients were divided into two groups according to the duration of their hearing loss. Each group was further divided into two subgroups according to whether they received auditory rehabilitation or not. Using the Multi-Dimensional Voice Program (MDVP) parameters, comparisons were made between each subgroup of patients and the normal MDVP Saudi database, and between subgroups of patients. Most of the patients in the two groups reported significant improvement in their MDVP results post-implantation. Further, significantly deviant MDVP parameters were reported in the group of patients with longer duration of hearing loss. Patients who received rehabilitation significantly improved more than those who did not. In conclusion, it appears that cochlear implantation improves the auditory control of voice production in post-lingual deaf adults. Also, it is obvious that cochlear implantation at an early stage of hearing loss gives better results on voice control, especially if augmented with auditory rehabilitation.