语前聋儿童人工耳蜗植入后的调机与声场评估

目的 调查语前聋儿童人工耳蜗植入术后声场主观测听阈值和对应T值的变化趋势。方法 28例人工耳蜗植入儿童。年龄3—6岁。无耳蜗畸形，植入后电极阻抗测试完全正常。对开机后3月、6月和1年时间的声场主观测听阈值与T值的变化进行了统计分析。结果 声场测听阈值在开机1年中呈逐渐降低趋势，开机1年时声场测听的听力水平较3个月和6个月有明显提高（P〈0．05）。植入者术后一年声场主观测听（啭音）听阈为15—35dB HL，听力水平较术前有明显提高。28例植入者T值在开机一年后明显低于六个月和三个月的水平（P〈0．05）。结论 开机后一年内T值和声场测听阈值都呈逐渐下降趋势．并且与3个月和6个月的水平差异具有统计学意义。植入者在熟悉适应了电声信号后，可以在刺激电流强度下降的同时获得更好的听力。     

人工耳蜗术后患儿听阈评估

目的分析人工耳蜗对患者的听觉康复作用,比较声场和头戴式耳机两种人工耳蜗术后助听听阈评估方法的优劣。方法重度和极重度感音神经性听力损失患者27例接受人工耳蜗植入,男性18例,女性9例,植入年龄跨度1.7至18.6岁,平均7.6±5.4岁。开机后分别采用声场扬声器和TDH39头戴式耳机评估患者0.25k、0.5k、1.0k、2.0k、4.0kHz的助听听阈,TDH39耳机还可测试8.0kHz的助听听阈。使用配对t检验对两种方法所得结果进行比较。结果使用声场扬声器得到的助听听阈3FA(即0.5k、1.0k、2.0kHz平均听阈)为39.4±7.7dBHL,4FA(即0.5k、1.0k、2.0k、4.0kHz平均听阈)为37.5±7.3dBHL。使用TDH39头戴式耳机测得的助听听阈3FA为39.3±6.1dBHL,4FA为38.4±5.7dBHL。两者所测得的3FA(P=0.8801)和4FA(P=0.2133)均无统计学差异。使用两种方式测得的0.25kHz(P=0.8756)、0.5kHz(P=0.0630)、1.0kHz(P=0.1980)、2.0kHz(P=0.6866)助听听阈均无统计学差异,4.0kHz(P=0.0062)耳机测试听阈低于声场结果。结论人工耳蜗植入可帮助重度和极重度感音神经性听力损失患者实现良好的听敏度。使用头戴式耳机可获得可靠的植入后助听听阈,比声场测试更加简便易行。     

9例人工耳蜗植入儿童术后康复评估结果分析

目的 探讨3～7岁在训聋儿植入人工耳蜗后听觉言语康复进展情况.方法 使用美国GSI-16配置声场的双声道临床听力计,在标准声场下对9例儿童进行人工耳蜗术后声场听阈测试;采用<聋儿听力语言康复评估方法>,在术后3、6、9、12个月分别进行听觉言语康复评估.结果 9例人工耳蜗植入儿童声场听阈测试均值为26～35 dB HL;随着术后时间的增加,9例儿童的听觉言语评估成绩逐步提高,术后12个月时平均言语识别率达89.8%,平均语言年龄为3.0岁.结论 人工耳蜗植入有助于重度、极重度聋儿的听力语言康复.     

多通道人工耳蜗植入患者声场听阈测试结果分析

目的 探讨声场测试对多通道人工耳蜗植入术后听觉评估的意义。方法 对１１例Ｎｕｃｌｅｕｓ多通道人工耳蜗植入患者定期进行声场听阈测试,并对结果进行统计学处理。结果 全部患者声场测试（啭音）听阀为１５￣４０ｄＢ（ｎＨＬ）,语后聋组术后半年声场听阈明显低于同期语前聋组（Ｐ〈０．０１）,语前聋组术后一年的声场听阈比术后半年时显著下降（Ｐ〈０．０１）,接近语后聋组半年时的听觉水平（Ｐ〉０．０５）。结论 多通道     

REZ-Ⅰ型人工耳蜗植入对成人残余听力的影响

目的 评估REZ-Ⅰ型国产人工耳蜗植入对成人残余听力的影响,探讨该人工耳蜗植入的听力学安全性及其损伤的特点.方法 回顾性分析复旦大学附属眼耳鼻喉科医院2009年9月至2009年11月间16例单侧REZ-Ⅰ型(22通道)人工耳蜗植入者在手术前后的纯音测听、听觉稳态反应(auditory steady-state response,ASSR)、畸变产物耳声发射(DPOAE)及听觉脑干反应(ABR)的资料,比较手术前后残余听力的变化情况.结果 纯音测听植入侧术后残余听力保留率为41.67%;术后植人侧250、500、1000、2000及4000 Hz的残余听力较术前下降,差异具有统计学意义,其中500 Hz下降较明显,平均下降15.3 dB HL,P<0.01;通过与非植入侧对比发现500和1000 Hz的听力损失有统计学意义(P值均<0.05).植入侧术后ASSR阈值在250和500 Hz处较术前升高,差异具有统计学意义(P值均<0.05)),经与非植入侧对比发现500 Hz处ASSR阈值升高有统计学意义(P值<0.05).DPOAE及ABR植入前后差异均无统计学意义(P值均>0.05).结论 REZ-Ⅰ型人工耳蜗植入会对植入侧残余听力造成一定程度的损伤.     

普通话单音节测听表在人工耳蜗植入者中的初步应用

目的采用新编普通话单音节测听表,评估人工耳蜗植入者的言语识别能力。方法选取8名语前聋儿童（甲组）及13名语后聋（乙组）人工耳蜗植入者作为研究对象,男12名,女9名。甲组植入年龄为1．6～11．9岁（中位数2．7岁）,使用年限〉3．4年。乙组植入年龄为3．2～30．9岁（中位数12．0岁）,使用年限〉0．7年以上。在声场中测试人工耳蜗日常使用状态下的啭音听阈,并55dB SPL言语强度下的单音节识别率。部分受试者还接受了65、80dB SPL言语强度下单音节识别率的测试。结果甲乙2组受试者的0．5、1、2、4kHz四频率平均（4 Frequency Average,4FA）声场啭音听阈分别为32．5±3．4dB HL、35．9±4．9dB HL,t检验显示P=0．1046。甲乙2组受试者55dB SPL下的单音节识别率分别为56．8±9．0％、31．41±7．1％,t检验显示P=0．001。结论新编普通话单音节测听表,可有效地用来评价人工耳蜗植入者的言语识别能力。使用年限较短的语后聋患者的单音节识别率尚不及使用年限较长的语前聋儿童。人工耳蜗植入者的单音节识别率一言语声强（Performance—Intensity,P—I）函数曲线较正常成人平均右移30dB以上,提示人工耳蜗使用者的言语听力补偿水平较正常人仍有差距。     

两种不同长度的人工耳蜗电极对不同频率残余听力的影响

目的探讨植入两种不同长度的人工耳蜗电极对患者不同频率残余听力的影响。方法以中耳、内耳无明显异常的15例单侧植入Nucleus 24型人工耳蜗(A组,电极长度17mm)患儿与15例单侧植入Medel C40+15型(B组,电极长度29.2mm)人工耳蜗患儿为对象,两组对象于手术前后分别进行听性稳态反应(ASSR)测试,比较不同长度的人工耳蜗电极植入对不同频率听力的影响。结果①人工耳蜗植入后一月开机经耳蜗电刺激后,有51.67%患者植入耳保留残余听力;②手术前0.5、1、2、4kHz ASSR反应阈A组分别为98.93±6.28、107.80±8.16、104.07±8.83及104.73±5.93dB HL,B组分别为99.67±4.20、110.87±8.17、109.93±7.51及106.93±4.35dB HL;手术后一月上述各频率ASSR反应阈A组分别为101.60±4.64、112.60±6.11、106.13±5.19及106.60±6.25dB HL;B组分别为101.40±3.96、116.33±2.58、113.13±3.84及108.46±3.76dB HL;③术后B组0.5、1kHz的残余听力损伤明显,2、4kHz处残余听力损伤程度与A组差异无统计学意义;④A组术后残余听力损失以1kHz最显著,B组术后残余听力损失以500Hz最显著。结论人工耳蜗植入及耳蜗电极电刺激对植入耳的残余听力并非完全破坏,电极长短对不同频率残余听力会造成不同程度的损害,植入电极越长,植入耳蜗的部位越深,对低频残余听力损伤越明显。     

EABR辅助31例Mondini畸形人工耳蜗植入及术后效果分析

目的探讨电刺激听觉脑干诱发电位(electrical evoked auditory brainstem responses,EABR)辅助Mondini畸形患者人工耳蜗植入手术的方法及术后效果分析。方法我院自2001年开始为Mondini畸形患者植入人工耳蜗,收集2012年至2015年在我院行人工耳蜗手术的Mondini畸形患者31例,回顾性分析Mondini畸形患者术前听力学特征,术前EABR特点,术中手术特点、术后NRT测试结果及开机调试结果,随访术后听觉言语康复能力CAP(categories of auditory performance)和SIR(speech intelligibility rating)评分。另选择相似耳聋年龄的耳蜗结构正常耳蜗植入患者20例作为正常对照组,测试结果进行对比分析。结果 31例Mondini畸形患者术前听力均为极重度感音神经性聋,其中14例无残余听力,对照组7例无残余听力,术前行EABR测试,引出率100%,有反应者植入不同类型电极的人工耳蜗;术中电极全部植入耳蜗内,其中4例出现脑脊液井喷,颞肌封堵,术后无面瘫、脑脊液漏、脑膜炎等手术并发症;所有Mondini畸形患者术后均获得听觉,开机测试神经反射遥测(neural response telemetry,NRT),引出率41.94%低于对照组95%;开机6月平均T值Mondini组171.37±13.14CL;对照组145.34±17.43CL;动态范围Mondini组33.24±10.73CL,对照组43.55±15.36CL,经统计学分析P<0.05,有显著差异;随访6月~4年,Mondini组CAP平均得分6.13±1.15,SIR平均得分3.05±0.62;对照组CAP平均得分7.12±0.80,SIR平均得分4.03±0.91,经两样本比较t检验,P<0.05有显著性差异。结论 Mondini畸形的双侧极重度感音神经性聋患者可以根据残余听力情况在EABR辅助下行人工耳蜗植入,术后听力言语康复较正常耳蜗结构患者效果稍差,但明显好于术前听觉和言语能力。     

微创人工耳蜗植入术及常规人工耳蜗植入术临床比较

目的通过微创人工耳蜗植入术与常规人工耳蜗植入术的临床效果比较,探讨微创人工耳蜗植入术的临床优势。方法回顾性分析121例人工耳蜗植入患者,根据人工耳蜗植入术式的不同分为微创人工耳蜗植入术组(微创组)及常规人工耳蜗植入术组(常规组),比较两组患者的手术时间、电极植入情况、手术切口及并发症的不同,分析两种术式之间的特点。结果 121例人工耳蜗电极均全植入鼓阶,成功率100%。微创组与常规组的平均手术总时间分别为89.53±12.42分钟、92.30±14.16分钟,电极植入平均用时分别为112.16±16.01秒、117.05±26.90秒,平均电极植入次数分别为1.07±0.26次、1.22±0.49次。两组的手术总时间无明显差别(P>0.05),电极植入用时亦无明显差别(P>0.05),微创组平均电极植入次数较常规组少(P<0.05)。微创组及常规组的手术切口长度分别为2.53±0.12cm、8.30±0.56cm,微创组的手术切口长度明显小于常规组(P<0.05)。微创组术后未发现皮下血肿及感染,常规组术后出现皮下血肿3例(4.76%),微创组术后皮下血肿率与常规组比较无明显变化(P>0.05)。微创组和常规组的术后眩晕出现率分别为5.88%、21.83%,微创组术后眩晕率低于常规组术后眩晕率(P<0.05)。结论与常规人工耳蜗植入术相比较,微创人工耳蜗植入术在手术时间不增加的前提下,可确保电极位于鼓阶,并具有外形美观,手术切口小,术中创伤小,术后并发症少等优点。     

GJB2基因突变致聋患儿人工耳蜗植入效果分析

目的 比较GJB2基因突变致聋患儿与非GJB2基因突变且内耳结构正常聋儿人工耳蜗植入术后的听觉言语康复效果.方法 对37例经C下及MRI检查排除内耳畸形的聋儿术前行GJB2基因检查,根据结果 分成A组(GJB2基因突变10例)和B组(非GJB2基因突变27例),术后随访0.5～2年,进行术后的听阈、言语识别率及言语能力评估.结果 37例聋儿人工耳蜗植入手术全部成功,均建立了主观听性反应.A组的声场听阈水平平均为34.41±6.12 dB HL.言语识别率平均为76%; B组的声场听阈水平平均为36.23±4.16 dB HL.言语识别率平均为79%,两组均达到平均言语康复级别二级;两组听觉及言语能力测试结果 均无统计学意义(P>0.05).结论 人工耳蜗患者中GJB2基因突变率高,可能是内耳结构正常的人工耳蜗植入人群耳聋的主要致聋原因;GJB2基因突变致聋患儿与非GJB2基因突变且内耳结构正常聋儿人工耳蜗植入术后效果基本一致.人工耳蜗植入可作为GJB2基因突变致聋患儿的有效治疗手段.     

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.     

学龄前不同年龄段语前聋人工耳蜗植入患儿康复效果比较

目的：分析学龄前不同年龄段语前聋患儿人工耳蜗植入后听觉言语康复效果,探讨影响其康复效果的可能因素。方法将55例植入Freedom人工耳蜗的学龄前语前聋患儿按植入年龄分为1～3岁组32例,＞3～5岁组23例,于术后开机1、3、6、12、18、24个月时分别采用听障儿童听觉言语评估词表及标准评估程序、听觉行为分级（categories of auditory performance ,CAP）和言语可懂度分级（speech intelligibility rating ,SIR）评估并比较两组患儿听觉能力、言语能力及学习能力。结果在术后两年内,随着康复时间的延长,两组对象的平均言语识别率、平均语言年龄、CAP分级及SIR分级均逐渐提高,且在术后各时间段1～3岁组的平均言语识别率、平均语言年龄康复效果优于＞3～5岁组（P＜0．05）;在术后开机1、3、12个月时1～3岁组的CAP评级高于＞3～5岁组（P值分别为0．001、0．002和0．002）;在术后开机1、3、12、24个月时1～3岁组的SIR评分高于＞3～5岁组（P值分别为0．00、0．00、0．024和0．024）;两组间各时间段学习能力比较差异无统计学意义（ P＞0．05）。结论对于语前聋患儿,人工耳蜗植入的年龄越小,术后两年内康复训练时间越长,效果越好;人工耳蜗植入时的年龄和术后康复时间是影响语前聋人工耳蜗植入患儿术后康复效果的关键因素。     

Cochlear implant outcomes in the elderly.

OBJECTIVE: This study aimed to review cochlear implantation with respect to surgical and auditory outcomes in subjects aged 70 years and older. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral centers. PATIENTS: Sixty-five patients aged 70 years or older at the time of implantation were compared to a group of patients aged <70 years. INTERVENTION: Patients underwent multichannel cochlear implantation with either the Clarion or Nucleus device. MAIN OUTCOME MEASURE: Presence or absence of surgical complications and auditory performance with open-set word and sentence recognition testing. RESULTS: In patients implanted at age 70 or older, significant improvement in speech understanding was demonstrated in performance scores using Consonant Nucleus Consonant words, Central Institute for the Deaf sentences, and Hearing in Noise Test sentences at 3, 6, and 12 months when compared to preimplantation scores. However, their performance was slightly poorer when compared to a control group of patients <70 years of age in the same measures at 3, 6, and 12 months. CONCLUSIONS: The elderly population showed significant improvement in auditory performance tests following cochlear implantation compared to their preimplantation scores but performed less well than younger patients.     

老年及老年前期人工耳蜗植入的临床初步研究

目的 探索人工耳蜗在双耳重度或极重度聋和全聋的老年患者中的临床应用安全性和效果.方法 2008年11月至2009年11月解放军总医院先后对8例50岁以上患者行人工耳蜗植入术,其中男5例,女3例;年龄52～76岁,中位数58岁.所有患者术前均为双侧极重度感音神经性听力损失,佩戴助听器无效或效果不佳.观察患者手术耐受及并发症情况.开机后3个月进行助听听阈评估和言语评估.结果 8例患者手术顺利,术中神经反应遥测均引出反应.术后康复顺利,无任何并发症.开机3个月后声场内言语频率平均助听听阈啭音(听力级)为35～50 dB,但言语测听结果个体差异较大.部分患者单音节、双音节、安静及噪声环境下的句子可实现较好识别率.结论 老年前期及老年患者可耐受全身麻醉状态下的人工耳蜗植入,只要术前准备充分则为安全的手术,但部分病例为中耳炎(胆脂瘤)或颞骨外伤等因素导致,需要特别注意中耳情况.老年重度以上听力损失患者人工耳蜗植入可明显改善言语识别能力.     

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。     

人工耳蜗植入术中圆窗耳蜗电图评价耳蜗残余听功能

目的应用术中圆窗耳蜗电图评估极重度感音神经性聋患者耳蜗残余听功能。方法20例患者全麻下人工耳蜗植入手术过程中,行术中圆窗耳蜗电图测试,测出的复合动作电位（CAP）阈值与术前纯音测听或其他听力测试之阈值进行比较。结果20例患者术中圆窗耳蜗电图测试测出的复合动作电位（CAP）阈值与术前1、2、4kHz听力测试阈值分别有较好的相关系数（0．20429,0．04076,0．38163）。结论术中圆窗耳蜗电图可以较准确客观地评估极重度感音神经性聋患者耳蜗残余听功能,且是人工耳蜗植入术前听力学评估方法的有意义的补充。     

Cochlear implants in Waardenburg syndrome

OBJECTIVE: Waardenburg syndrome is an autosomal-dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation. METHODS: Pediatric cochlear implant recipients with documented evidence of Waardenburg syndrome underwent retrospective case review. All patients received their cochlear implants at the study institution followed by outpatient auditory habilitation. Charts were reviewed for etiology and duration of deafness, age at time of cochlear implantation, perioperative complications, duration of use, and performance outcomes. Results of standard tests batteries for speech perception and production administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Seven patients with Waardenburg syndrome and cochlear implants were identified. The average age at implantation was 37 months (range, 18-64 months) and the average duration of use was 69 months (range, 12-143 months). All of these patients are active users of their devices and perform very well after implantation. There were no major complications in this small group of patients. CONCLUSIONS: Children with congenital sensorineural hearing loss without other comorbidities (e.g., developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. Patients with Waardenburg syndrome can be expected to have above-average performance after cochlear implantation.     

Assessment of outcomes of hearing and speech rehabilitation in children with cochlear implantation

ObjectivesThis study aimed to assess the effect of hearing and speech rehabilitation in patients with Nurotron^® cochlear implants.DesignNinety-eight paediatric patients with bilateral severe-to-profound sensorineural deafness who received cochlear implantation were divided into three groups according to age: group A (≤3 years), group B (4–7 years), and group C (8–16 years). All patients were followed up for one year for hearing and speech performance after the surgery. The comprehensive Auditory Perception Assessment, MAIS, CAP and SIR hearing and speech assessments and rating materials were used for assessment before the surgery and at 3, 6, and 12 months after implant activation.ResultsThe scores of patients in the open-set speech assessment, Chinese Auditory Perception Assessment, MAIS, CAP and SIR significantly improved after cochlear implantation in all age groups. The younger the age at implantation, the better the results. Moreover, the hearing and speech performance of cochlear implant recipients gradually improved with the extension of rehabilitation time.ConclusionsNurotron^® Venus? cochlear implantation can improve the hearing and speech performance of patients with bilateral severe-to-profound sensorineural deafness.     

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.