两种常见基因突变对早期人工耳蜗植入儿童听觉言语发展的影响北大核心CSCD

目的探讨GJB2或SLC26A4基因突变对早期人工耳蜗植入(cochlear implantation,CI)儿童听觉言语发展的影响。方法选择于我科接受单侧CI的双耳重度极重度感音神经性聋患儿211例为研究对象,植入年龄为0.67-3.5岁,根据基因检测结果分为GJB2基因突变组共65例、SLC26A4基因突变组共42例,以及未检出基因突变者104例为对照组。分别于术前、开机后6个月、12个月、18个月、24个月及36个月时评估CI儿童的听觉言语能力。评估工具包括:婴幼儿有意义听觉整合量表(Infant-Toddler Meaningful Auditory Integration Scale,ITMAIS)或有意义听觉整合量表(Meaningful Auditory Integration Scale,MAIS),听觉行为分级(Categories of Auditory Performance,CAP),汉语沟通发展量表(Putonghua Communicative Development Inventory,PCDI)以及有意义言语使用量表(Mean⁃ingful Use of Speech Scale,MUSS)。结果随着康复时间的延长,三组CI儿童的听觉言语能力逐步提升。GJB2组在术前及术后各随访时间点的听觉言语能力得分和对照组相比差异无统计学意义(P>0.05)。SLC26A4组的ITMAIS/MAIS得分在术前、开机6个月、12个月及18个月时优于对照组(P<0.05),CAP得分在术前及开机6个月时优于对照组(P<0.05),PCDI得分在术前、开机6个月及12个月时优于对照组(P<0.05)。在其余随访时间点,SLC26A4组CI儿童的听觉言语能力得分和对照组CI儿童相比差异无统计学意义(P>0.05)。结论GJB2或SLC26A4基因突变预示良好的植入后听觉言语康复效果。SLC26A4基因突变对植入后早期的听觉言语发展有积极作用。基因检测有助于预估CI儿童的康复效果。     

婴幼儿有意义听觉整合量表评估极重度聋幼儿助听后早期语前听能发育的价值

目的 探讨婴幼儿有意义听觉整合量表(infant-toddler meaningful auditory integration scale, ITMAIS)评估双耳极重度感音神经性听力损失(sensorineural hearing loss, SNHL)幼儿助听(hearing aid, HA)后6个月内的早期语前听能发育(early prelingual auditory development, EPLAD)规律及价值.方法 以配戴助听器时年龄为1~2岁的51例双耳极重度SNHL幼儿为研究对象,分为1岁组(28例)和2岁组(23例),比较助听器配戴时及配戴后3个月、6个月的ITMAIS得分,并与同龄未助听的不同程度SNHL幼儿比较,以此评估其EPLAD情况.结果 1岁组、2岁组幼儿在配戴助听器后3、6个月时的ITMAIS得分(3个月时分别为30.6%±17.0%、41.4%±23.5%,6个月时分别为41.42%±24.2%、50.1%±27.6%)较配戴前(分别为9.7%±5.2%、17.9%±10.1%)均显著提高(P<0.05);两组幼儿的ITMAIS平均得分在配戴助听器后6个月时接近或达到同龄未助听的重度SNHL幼儿平均水平.结论 助听器有助于极重度SNHL幼儿在配戴助听器早期(6个月)提升其EPLAD水平;ITMAIS测试是一项简单、有效、可用于评估助听器配戴幼儿初期康复效果的方法.     

语前聋患儿人工耳蜗植入术后听觉言语功能发育的观察

目的研究语前聋患儿植入人工耳蜗后听觉言语能力的发育情况及相关因素。方法34例接受人工耳蜗植入的极重度语前聋儿童手术时年龄为1岁2个月～5岁10个月,平均3岁2．15个月,按照植入手术时年龄分为幼龄组（A组,＜3岁,17例）、大龄组（B组,≥3岁,17例）,在植入手术前和开机后3、6、12个月四个时间点采用有意义听觉整合量表（meaningful auditory integration scale ,MAIS）和婴幼儿有意义听觉整合量表（the infant-toddler meaningful auditory integration scale ,IT -MAIS）对患儿进行听觉能力评估。结果全体患儿植入人工耳蜗后量表总分及三组问题的得分均明显高于植入前,并且随着开机时间的延长其得分逐步上升（P＜0．05）,其中评价患儿对声音感知能力的Q3～6在开机后的3个月内进展最为迅速。B组植入术前与听觉辅具有关的问题Q1～2和开机后3个月Q3～6得分高于A组（P＜0．05）,其余各项得分两组间差异无统计学意义。总体上男性和女性患儿各项得分无差异（P＞0．05）,A组女性患者术前Q1～2得分高于男性（P＜0．05）,其余各时间点男女间得分差异无统计学意义（P＞0．05）。结论随着使用人工耳蜗时间的延长,人工耳蜗植入患儿总体听觉能力呈逐步上升的趋势,进步最快的时间为开机后的3个月内,这个阶段较大龄的患儿对声音的察觉能力优于婴幼儿患者,但随着植入时间的延长两组听觉能力的发育无差异;不同性别患儿植入人工耳蜗后的听觉发育无差异。     

语前聋患儿国产人工耳蜗植入效果分析

目的 观察语前聋患儿植入诺尔康-晨星人工耳蜗(Nurotron-Venus cochlear implant,Nurotron-Venus CI)后的听觉言语发育情况及其安全性和稳定性.方法 回顾性分析郑州大学第一附属医院行Nurotron-Venus CI植入的78例语前聋患儿的临床资料,患儿植入年龄为13~96月,按植入年龄将患儿分为 A组(13~24月,16 例)、B 组(25~36月,13 例)、C组(37~48月,16 例)、D 组(49~72月,18 例)、 E 组(73~96月,15 例);在患儿术前及开机后1、3、6、12个月采用有意义听觉整合量表(meaningful auditory integration scale,MAIS)(C、D、E组)、婴幼儿有意义听觉整合量表(infant-toddler meaningful auditory integration scale,IT-MAIS )(A、B组)进行听觉能力评估,开机后1、3、6、12个月使用有意义言语使用量表(meaningful use of speech scale,MUSS)进行言语能力评估.术后行X线耳蜗平片检查明确电极位置及形态,定期随访了解术后相关并发症及佩戴人工耳蜗时间和使用情况.结果 所有患儿术前及开机后1、3、6、12月IT-MAIS或MAIS得分分别为1.67±1.19、6.60±5.12、11.86±5.44、17.41±5.04、22.87±5.46分,术后1、3、6、12月MUSS得分分别为5.01±3.26、8.38±3.58、11.88±3.88、16.58±4.95分,随着植入时间延长得分不断上升(均P<0.05);不同年龄组IT-MAIS或MAIS得分比较:术前A组低于C、D、E组,开机后1、3月A、B组低于E组,开机后6月A组低于C、D、E组,B组低于E组(均为P<0.05);不同年龄组MUSS得分比较:开机后1月B组低于E组,开机后3月A、B、C组低于E组,开机后6月A组低于C、D、E组,B组低于E组,开机后1年A组低于C、D、E组(均为P<0.05).所有患儿在开机后1、3、6、12月MUSS与IT-MAIS或MAIS得分相关系数分别为0.918、0.808、0.881、0.756(均为P<0.05),两者呈正相关.78例患儿术后耳蜗位X线平片均显示电极位置正常,形态良好;2例患儿出现处理器故障,其余CI整体工作状态良好.结论 语前聋患儿国产人工耳蜗植入后1年内,所有患儿的听觉言语能力随时间延长不断提高;小龄组患儿听觉言语得分在术后早期低于大龄患儿;听觉能力在开机后12月时已无差异,但小龄组患儿的言语能力仍低于大龄患儿;听觉整合能力越强,言语使用能力越强;Nurotron-Venus CI安全、效果可靠.     

低龄儿童人工耳蜗植入听觉和言语发声行为影响因素研究

目的研究人工耳蜗(Cochlear implant CI)使用时间、术前残余听力和听觉言语康复训练对低龄CI植入儿童听觉行为和言语发声行为的影响作用。方法研究对象为43例小于3岁CI儿童,植入月龄6~32个月(平均植入月龄17个月)。听觉行为评估素材为婴幼儿日常听觉综合能力量表(the infant-tod-dler meaningful auditory integration scale,IT-MAIS),言语发声行为评估素材为有意义使用言语量表(themeaningful use of speech scale,MUSS)。评估分别在术后开机时和开机后1、2、3、6、9、12个月时进行。结果(1)全体受试儿童IT-MAIS、MUSS总分随着CI使用时间的增长逐步增长(ANOVA,p<0.05);(2)术前残余听力水平对前6个月IT-MAIS得分影响显著(ANOVA,p<0.01),对MUSS得分未见影响作用(p>0.05)。(3)听觉言语康复训练显著影响术后IT-MAIS、MUSS得分。开机后3个月训练组IT-MAIS得分高于非训练组得分(p<0.05)。9、12个月时训练组MUSS得分与非训练组MUSS得分差异有显著意义(p<0.01)。结论(1)在听觉行为方面,随着CI使用时间的延长,受试儿童总体听觉能力呈逐步上升趋势。对声音的辨别和理解能力在3-9个月时有显著增长;在发声行为方面,受试儿童在CI植入6个月以后开始用言语方式表达。(2)术前残余听力在受试儿童CI使用前6个月时对听觉行为有影响作用,9个月以后影响作用逐渐减小;平均听力损失程度对受试儿童的言语发声行为未见显著影响。(3)听觉言语康复训练对听觉行为和言语发声行为体现出重要作用。     

人工耳蜗植入儿童听觉康复效果观察

目的观察语前聋患儿在人工耳蜗开机后一年内听觉能力的发展状况,为康复教学工作提供参考。方法对52例在南京大学医学院附属鼓楼医院进行人工耳蜗植入手术并开机和定期调机的患儿,于开机后半年和一年时分别进行声场评估,得到每位患儿的术后平均听闽,同时由康复教师对患儿进行韵母、声母、声调、单音节词、双音节词、三音节词、短句和选择性听取等8项评估。使用SPSS10．0统计学软件对半年和一年时各项评估结果进行组内纵向统计学比较,并分别就两次言语识别能力评估中音节要素、词语方面各自所含测试项进行组间横向统计学比较。结果开机后半年与一年患几的平均听阈无显著性差异（P〉0．05）。各单项识别率开机后半年与一年纵向比较,差异均有统计学意义（P〈0．001）。组间横向比较,开机半年及一年时,韵母识别能力始终优于声母、声调识别能力,差异具有统计学意义（P〈0．001）,单音节词识别能力相对弱于双音节词、三音节词识别能力,差异亦具有统计学意义（P〈0．001）;两次评估中,选择性听取能力始终落后于其他各项。结论听觉康复过程符合从声音察觉、感知再到理解的规律,对语言的理解识别也符合先简单后复杂的规律,即从音节要素到词语、再到短句。康复过程需有侧重,人工耳蜗开机后前半年需注重听觉感知能力的提高,而后半年需注重对语言理解能力的培养。     

听神经发育不良儿童人工耳蜗植入术后听觉言语能力发展规律及特点

目的 探讨听神经发育不良(cochlear nerve deficiency, CND)儿童人工耳蜗植入(cochlear implant, CI)术后听觉能力、言语能力的变化特点及发展规律，为此类儿童的诊治和康复提供参考。方法 使用婴幼儿有意义听觉整合量表(infant toddler-meaningful auditory integration scale, IT-MAIS)、有意义使用言语量表(meaningful use of speech scale, MUSS)、听觉能力分级(categories of auditory performance, CAP)和言语可懂度分级(speech intelligibility rating, SIR)四个问卷量表，对42例内耳结构正常伴CND的CI儿童(A组)和189例内耳结构正常且听神经发育正常的CI儿童(B组)分别在术前、开机后1、3、6、9、12、24和36个月进行听觉言语能力评估。结果 两组患儿4个问卷得分均随着开机时间的延长而逐渐提高，且B组比A组增长趋势明显。IT-MAIS评估结果显示，在开机后6、9个月时两组得分差异...     

植入年龄对婴幼儿人工耳蜗患者早期听力发育的影响

目的:评估使用人工耳蜗的婴幼儿患者的早期听觉能力,总结其发育规律,探讨植入年龄对患儿人工耳蜗植入术后1年内听觉能力发育的影响,为其有效康复提供临床资料。方法:将272例因极重度语前聋行人工耳蜗植入的婴幼儿根据植入年龄分为3组:A组,植入年龄<18个月(107例);B组,植入年龄18～24个月(74例);C组,植入年龄>24～36个月(91例)。使用听觉行为分级(CAP)问卷对患儿的听觉能力分别在术前及术后开机1、3、6、9、12个月进行评估。结果:患儿CAP得分在术前及术后开机1、3、6、9、12个月之间均差异有统计学意义(均P<0.05)。A、B、C 3组患儿的CAP得分在术后开机1、3个月间差异有统计学意义(P<0.05),而术前和术后开机6、9、12个月之间均差异无统计学意义(均P>0.05)。结论:极重度语前聋患儿人工耳蜗植入后的听觉能力在术后1年内有显著提高,植入年龄对患儿术后早期听觉能力的发育无显著影响,CAP问卷适合在中国临床工作中使用。     

人工耳蜗植入幼儿康复个案研究

目的研究人工耳蜗植入幼儿在不同阶段的听觉康复效果和语言康复效果．为人工耳蜗植入幼儿术后康复训练提供经验。方法采用评估和观察的方法,对一名人工耳蜗植入幼儿术后康复一年的情况进行阶段性评估。结果经过一年的康复训练,该幼儿的听觉康复级别和语言康复级别均达到一级水平,可以和健听人进行简单的交流,并能主动和陌生人说话。结论人工耳蜗植入为重度听力损失的幼儿提供了听觉重建的机会：家园配合的术后康复训练可以使幼儿的听觉言语能力在一年内取得较大的进步。     

语前聋听神经病多道人工耳蜗植入效果初步观察

目的 探讨听神经病患者人工耳蜗植入后的电生理结果 变化并分析其听力言语康复效果.方法 对行人工耳蜗植入术的2例听神经病患儿进行术前听力学评估,术中、术后听觉诱发电位反应监测及开机后1年的随访,获得其开机后6个月与12个月时的听力言语康复效果,并与人工耳蜗使用时间相近的非听神经病耳蜗植入患儿的康复效果进行对比.结果 例1术中神经反应遥测henral response telemetry,NRT)及电刺激听性脑干诱发反应(electric auditory brainstem response,EABR)可引出波形,但重复性不好,EABR V波潜伏期延长.开机12个月时复查,2项电生理检查均引出可重复波形,EABR V波潜伏期在正常范围;听力言语康复效果显著提高,开机1年后有意义听觉整合量表得分优于对照组儿童;例2术中NRT未引出有意义波形,EABR可引出波形,但重复性不好;开机12个月复查,2项电生理检查均引出可重复波形,EABR V波潜伏期在正常范围;术后听力言语康复效果亦有所提高.结论 2例听神经病患者人工耳蜗植入后,术前听觉通路电活动的去同步化均得到一定程度的恢复,听力言语能力也有不同程度提高,说明人工耳蜗植入可以作为听神经病患者实现听力重建,重返主流社会的治疗和康复手段.     

人工耳蜗植入后聋儿听力培建效果的评价

目的：对语前聋儿音植入人工耳蜗后的听力培建历程作出评价。方法：应用（婴幼儿）听觉意义整合量表（MAIS或IT－MAIS）对22名植入Nucleus24型人工耳蜗的语前聋儿童的听力培建效果进行问卷调查，并对得分与使用时间作曲线拟合。结果：患儿蝗MAIS得分在术后3－6个月即可达到较高水平，大龄聋儿畸快于低龄聋儿；问卷涉及的“对耳蜗装置的接纳、对声音的察知、声音意义的建立”这三个导面，得分依次递减。结论：人工耳蜗是（极）重芳语前聋儿童培建听力的可靠手段，培建的过程遵循听觉发展的一般规律，大龄儿童快于低龄儿童。MAIS问卷对于评价听力培建效果、指导家庭康复训练都有一定的实用意义。     

Evaluation of auditory development in infants and toddlers who received cochlear implants under the age of 24 months with the LittlEARS Auditory Questionnaire

Background and Aims

Newborn hearing screening and early intervention for congenital hearing loss have created a need for tools assessing the hearing development of very young children. A multidisciplinary evaluation of children's development is now becoming standard in clinical practice, though not many reliable diagnostic instruments exist. For this reason, the LittlEARS^® Auditory Questionnaire (LEAQ) was created to assess the auditory skills of a growing population of infants and toddlers who receive hearing instruments. The LEAQ relies on parent report, which has been shown to be a reliable way of assessing child development. Results with this tool in a group of children who received very early cochlear implantation are presented.

Methods

The LEAQ is the first module of the LittlEARS^® comprehensive test battery for children under the age of two who have normal hearing (NH), cochlear implants (CIs) or hearing aids (HAs). The LEAQ is a parent questionnaire comprised of 35 “yes/no” questions which can be completed by parents in less than 10 min. Sixty-three children who received unilateral CIs at a young age were assessed longitudinally and their performance was compared to that of a NH group.

Results

All CI children reached the maximum possible score on the LEAQ on average by 22 months of hearing age, i.e. 38 months of chronological age. In comparison, the NH group reached the maximum score by 24 months of age demonstrating that auditory skills of CI children often develop quicker than those of NH children. In the two comparison groups of children aged (a) younger and older than 12 months, and (b) between 6-9 and 21-24 months at first fitting, the early implanted children reached the highest scores faster than the later implanted children. Furthermore, three children with additional needs were tested. They showed slower growth over time but also received benefits from early implantation.

Conclusions

The LEAQ is a quick and effective tool for assessing auditory skills of very young children with or without hearing loss. In our study, the auditory skills of children with CI progressed very quickly after implantation and were comparable with those of NH peers.     

(Re)habilitation nach Versorgung mit einem Kochleaimplantat

Over the last years, indications for cochlear implants (CIs) have changed dramatically. The benefits depend on the preconditions of the individual patient as well as on the subsequent (re)habilitation. Therefore, many variables influencing the hearing and speech perception of a CI user must be kept in mind. As an example, the special situation of children having Turkish as their mother tongue is described. The most convincing argument concerning (re)habilitation is its benefit. Indeed, this benefit represents the final standard of quality and serves as the yardstick for standard assessments of (re)habilitation quality. CI (re)habilitation includes medical, pedagogical, audiological, hearing and speech, and psychological therapeutic aspects.     

Trends in educational placement and cost-benefit considerations in children with cochlear implants.

OBJECTIVES: To study the effect of cochlear implantation on the use of educational resources by profoundly hearing-impaired children and to determine trends in educational cost vs benefit. DESIGN: Retrospective study and cost-benefit analysis. SETTING: Outpatient pediatric cochlear implant program in an academic institution (The Listening Center at Johns Hopkins University School of Medicine, Baltimore, Md), in collaboration with public schools in Maryland and surrounding states. PATIENTS OR OTHER PARTICIPANTS: School-aged children with profound prelingual hearing impairment without other clearly defined disabilities. Thirty-five children with multiple-channel cochlear prostheses and a comparison group of 10 children without implants from 'total communication' programs in the Maryland public school system. INTERVENTIONS: Multiple-channel cochlear implantation and at least 1 year of a systematic auditory skill development program at the Listening Center, compared with standard educational management of children with conventional amplification. MAIN OUTCOME MEASURES: Classroom placement and number of hours of special educational support used. RESULTS: A correlation was observed between the length of cochlear implant experience and the rate of full-time placement in mainstream classrooms (r = 0.10; P= .04). There was also a negative correlation between the length of implant experience and the number of hours of special educational support used by fully mainstreamed children (Pearson product moment correlation = -0.10; P = .03). Children with greater than 2 years of implant experience were mainstreamed at twice the rate or more of age-matched children with profound hearing loss who did not have implants. They were also placed less frequently in self-contained classrooms and used fewer hours of special education support. A cost-benefit analysis based on conservative estimates of educational expenses from kindergarten to 12th grade shows a cost savings of cochlear implantation and appropriate auditory (re)habilitation that ranges from $30000 to $200000. CONCLUSIONS: Cochlear implantation accompanied by aural (re)habilitation increases access to acoustic information of spoken language, leading to higher rates of mainstream placement in schools and lower dependence on special education support services. The cost savings that results from a decrease in the use of support services indicates an educational cost benefit of cochlear implant (re)habilitation for many children.     

听障幼儿人工耳蜗植入术后追赶发育实施效果

目的探讨运用健听小儿听能言语发育进程规律,加速听障幼儿人工耳蜗术后的感知觉发育进程。方法选择1.5～3岁人工耳蜗植入幼儿33例。进行为期5～6个月的听觉言语训练后,考察其追赶发育实施效果。结果听觉感受能力、语言表达能力均有显著改善。结论运用小儿生长发育规律,对听障幼儿实施追赶发育,可缩短开机后听能感知时间,使其尽快在大脑中形成听觉记忆。     

婴幼儿人工耳蜗植入术后听觉能力的发育

目的:观察行人工耳蜗植入术的婴幼儿听觉能力的发展规律,试图为患儿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86例患儿参加本次调查,手术年龄为1～3岁,均为语前聋患儿。评估使用婴幼儿有意义听觉整合量表(ITMAIS),由经过培训的听力学专业人员进行评估。评估人员逐一询问量表中的10个问题,主要调查患儿的发声情况、对声音的觉察能力和对声音的理解能力等三方面的内容,由家长对患儿在日常生活中听力方面的自发性反应做出详细的描述。分别在术前和术后开机后1、3、6、12个月时对患儿的上述情况进行评估。评估人员根据患儿听觉行为出现的频率进行评分。结果:经单因素方差分析,问题1的得分在术前与开机后1、3、6、12个月之间差异均有统计学意义(均P<0.01);问题2的得分在术前与开机后6、12个月之间差异均有统计学意义(均P<0.01);问题3的得分在术前与开机后3、6、12个月之间差异均有统计学意义(均P<0.01);问题4的得分在术前与开机后3、6、12个月之间差异均有统计学意义(均P<0.01);问题5的得分在术前与开机后1、3、6、12个月之间差异均有统计学意义(分别为P<0.05,P<0.01和P<0.01,P<0.01);问题6的得分在术前与开机后3、6、12个月之间差异均有统计学意义(分别为P<0.05,P<0.01,P<0.01);问题7的得分在术前与开机后3、6、12个月之间差异均有统计学意义(均P<0.01);问题8的得分在术前与开机后6、12个月之间差异均有统计学意义(均P<0.01);问题9的得分在术前与开机后3、6、12个月之间差异均有统计学意义(分别为P<0.05,P<0.01,P<0.01);问题10的得分在术前与开机后12个月之间的差异有统计学意义(P<0.05)。结论:患儿术前对日常生活中的声音不能作出准确的反应,而术后的发声情况、对声音的觉察能力和对声音的理解能力均有显著改善。患儿术后对声音理解能力的发育较发声行为和对声音的觉察能力的发育慢。     

植入年龄对婴幼儿人工耳蜗植入患者早期言语可懂度的影响

目的本研究评估使用人工耳蜗的婴幼儿患者早期言语可懂度发育并探讨植入年龄对言语可懂度的影响,为患儿的听力言语康复提供指导。方法共有272例极重度语前聋人工耳蜗植入婴幼儿参加本次研究。手术年龄9～36个月,平均（21±7）个月。根据植入年龄将患儿分为3个组：A组小于18个月,B组为19～24个月,C组为25～36个月。使用言语可懂度分级（speech intelligibility rating,SIR）问卷在术前及术后开机后1、3、6、9个月和1年时对患儿进行评估。结果患儿SIR平均得分在术前、术后开机后1、3、6、9个月和1年之间的差异有显著性。3个组患儿SIR平均得分在术后开机后3个月、6个月之间的差异有显著性,在术前、术后开机后1个月、9个月、1年之间的差异无显著性。结论极重度语前聋人工耳蜗植入婴幼儿患者的言语可懂度在术后1年内有显著提高。植入年龄对婴幼儿人工耳蜗患者术后早期言语可懂度发育没有显著影响。     

Beneficial auditory and cognitive effects of auditory brainstem implantation in children

CONCLUSION: This preliminary study demonstrates the development of hearing ability and shows that there is a significant improvement in some cognitive parameters related to selective visual/spatial attention and to fluid or multisensory reasoning, in children fitted with auditory brainstem implantation (ABI). The improvement in cognitive paramenters is due to several factors, among which there is certainly, as demonstrated in the literature on a cochlear implants (CIs), the activation of the auditory sensory canal, which was previously absent. The findings of the present study indicate that children with cochlear or cochlear nerve abnormalities with associated cognitive deficits should not be excluded from ABI implantation. OBJECTIVES: The indications for ABI have been extended over the last 10 years to adults with non-tumoral (NT) cochlear or cochlear nerve abnormalities that cannot benefit from CI. We demonstrated that the ABI with surface electrodes may provide sufficient stimulation of the central auditory system in adults for open set speech recognition. These favourable results motivated us to extend ABI indications to children with profound hearing loss who were not candidates for a CI. This study investigated the performances of young deaf children undergoing ABI, in terms of their auditory perceptual development and their non-verbal cognitive abilities. PATIENTS AND METHODS: In our department from 2000 to 2006, 24 children aged 14 months to 16 years received an ABI for different tumour and non-tumour diseases. Two children had NF2 tumours. Eighteen children had bilateral cochlear nerve aplasia. In this group, nine children had associated cochlear malformations, two had unilateral facial nerve agenesia and two had combined microtia, aural atresia and middle ear malformations. Four of these children had previously been fitted elsewhere with a CI with no auditory results. One child had bilateral incomplete cochlear partition (type II); one child, who had previously been fitted unsuccessfully elsewhere with a CI, had auditory neuropathy; one child showed total cochlear ossification bilaterally due to meningitis; and one child had profound hearing loss with cochlear fractures after a head injury. Twelve of these children had multiple associated psychomotor handicaps. The retrosigmoid approach was used in all children. Intraoperative electrical auditory brainstem responses (EABRs) and postoperative EABRs and electrical middle latency responses (EMLRs) were performed. Perceptual auditory abilities were evaluated with the Evaluation of Auditory Responses to Speech (EARS) battery - the Listening Progress Profile (LIP), the Meaningful Auditory Integration Scale (MAIS), the Meaningful Use of Speech Scale (MUSS) - and the Category of Auditory Performance (CAP). Cognitive evaluation was performed on seven children using the Leiter International Performance Scale - Revised (LIPS-R) test with the following subtests: Figure ground, Form completion, Sequential order and Repeated pattern. RESULTS: No postoperative complications were observed. All children consistently used their devices for >75% of waking hours and had environmental sound awareness and utterance of words and simple sentences. Their CAP scores ranged from 1 to 7 (average =4); with MAIS they scored 2-97.5% (average =38%); MUSS scores ranged from 5 to 100% (average =49%) and LIP scores from 5 to 100% (average =45%). Owing to associated disabilities, 12 children were given other therapies (e.g. physical therapy and counselling) in addition to speech and aural rehabilitation therapy. Scores for two of the four subtests of LIPS-R in this study increased significantly during the first year of auditory brainstem implant use in all seven children selected for cognitive evaluation.