Background: The development of hearing and plasticity of brain after cochlear implantation (CI) for prelingually deaf children with white matter changes (group A) is unclear. Objective: To investigate the development of hearing for children in group A during 1 year after CI activation, compared with non-complicated peers (group B). Materials and methods: Twenty-one and 22 children (average age is about 5 years old) were included in groups A and B, respectively. Questionnaires were used to assess the hearing ability at pre-CI, 1, 6 and 12 months after CI activation (called Mpre, M1, M6, M12 for short). In addition, MMRs to pure tones were investigated at the same three time points after CI. Results: There is no significant difference in scores on questionnaires, MMR incidence, latency or amplitude between children in two groups. Scores on questionnaires showed a significant progressive improvement in two groups during the first year after CI. Furthermore, MMR incidence at M6 was significantly higher than that at M1, and MMR latency at M12 was significantly shorter than that at M1.

为进一步贯彻实施国家“一带一路”战略部署,中国残联积极加强同“一带一路”周边国家在残疾人康复领域的沟通交流与合作.2018年,中国听力语言康复研究中心(以下简称“语康中心”)有幸与柬埔寨普里安东医院建立友好合作关系,同心协力,圆满完成“澜沧江—湄公河合作专项基金项目”框架下的“柬埔寨听力语言康复专业高端人才培训及助听设备免费资助项目”各项工作,按项目执行计划实现了预期目标,取得良好成效.

目的 探索1～6人工耳蜗植入儿童术前及术后18个月内言语感知能力的发展规律.方法 选取31例人工耳蜗植入儿童作为被试,采用标准版普通话言语感知测试(mandarin early speech perception test,MESP)与简易版普通话早期言语感知测试(low-verbal mandarin early speech perception test,LV-MESP),选取7个时间点(术前、开机当天、开机后1、3、6、12和18个月)做纵向追踪,进行普通话言语感知测试.结果 能够完成MESP的人数随时间延长而显著增多,术后12个月能够完成MESP的人数显著高于术后3个月,术后18个月能够完成MESP的人数显著高于术后6个月.不能完成LV-MESP的人数随时间延长而显著减少,术后12个月时不能完成LV-MESP的人数显著低于术后6个月,能够完成的人数无显著变化.结论 LV-MESP是MESP的有益补充,人工耳蜗开机后康复训练1年期是听障儿童听觉言语发育的关键期,也是言语感知能力提高的敏感期,科学有效的人工耳蜗调试和康复训练可以促进听障儿童言语感知能力的逐步提高.

目的 通过对3～6岁普通幼儿园儿童进行纯音听阈测试,探讨纯音测听在该年龄段儿童听力筛查及诊断的必要性及其影响因素和测试技巧.方法 对59名118耳3～6岁普通幼儿园在园儿童进行纯音测听并分析测试结果.结果 ①58例儿童的纯音听阈结果正常,1例儿童左耳的纯音听阈异常、右耳正常,听力异常检出率为1.70％.②3～6岁儿童在250 Hz的听觉阈值显著高于500 Hz及以上频率的阈值,500 Hz阈值显著高于1000 Hz及以上频率的阈值(P＜0.05).有部分儿童在250Hz、500 Hz的纯音听闽阈值显示异常.③在左耳250 Hz频率上,性别的主效应显著,男童左耳在250 Hz频率上的阈值显著低干女童(P＜0.05).结论 定期有效的听力检测可及时发现后天性或迟发性听力损失提.在集中给大量的普通儿童做听力筛查或定期听力检测中,可首选“听声举手”方法进行测试,少数不配合儿童可采用游戏测听法.

Background: Few studies on speech performance of children after cochlear implantation (CI) described isolated large vestibular aqueduct syndrome (LVAS). Objective: To investigate speech developmental trajectories of infants with LVAS after CI, and to compare with those who have structurally normal inner ears. Materials and methods: 1112 infants with congenital severe to profound hearing loss participated in this study. 150 infants in group A were diagnosed with LVAS, 962 infants in group B with structurally normal inner ear. The speech performance was assessed via the Meaningful Use of Speech Scale (MUSS). The evaluations were performed pre-implant, 1, 3, 6, 9, 12, 24, 36, 48 and 60 months after CI. Results: The mean scores of the MUSS improved over a 5-year period after implantation in both groups A and B. The LVAS group presented similar speech developmental trajectory to the non-LVAS group at each assessment interval, except pre-operation. There were significant differences in mean scores between vocalizing behavior and oral communication skills, clarification skills of infants in both two groups. Conclusions and significance: Speech performance of infants with LVAS developed rapidly after CI and was similar to infants with structurally normal inner ear. For infants with isolated LVAS, CI had a significant effect and should be recommended as a therapeutic option.

Objective The study aimed to explore the characteristics of auditory mismatch response (MMR) in hearing-impaired children on the day when the cochlear implant (CI) was started (power-up) and the speech processor was programmed, and to investigate the effects of wearing hearing aids (HAs) before cochlear implantation on the early stage of postoperative auditory cortex plasticity, providing some demonstrative data for the objective evaluation of postoperative early auditory ability in children who underwent cochlear implantation. Methods The participants were 34 children with profound sensorineural hearing loss, who underwent cochlear implantation. The classical passive Oddball paradigm was adopted, using a pair of vowels which only have different lexical tones. The standard stimulus was /a2/ and the devious stimulus was /a4/. Results 1) On the day of CI activation, the auditory MMR has been elicited in 30 children; the MMR incidence was 88%. 2) We observed both positive and negative auditory MMR waveforms. And logistic regression analysis showed that it was influenced by the age at cochlear implantation. 3) The duration with HA before surgery significantly influenced the MMR latency. The children with longer duration of HA use have much earlier latency of MMR. 4) There was a significant positive correlation between the age at HA use initiation and MMR amplitude. Earlier initial HA use was associated with smaller amplitude. Conclusions MMR in response to Mandarin lexical tone can be recorded in most pediatric patients who had experience with HA on the day of CI power up. MMR is closely associated with the age at cochlear implantation, duration of HA use, and the age at HA use initiation. Hearing-impaired children should wear HA as early as possible and ensure consistent usage.

Concurrent hearing and genetic screening of newborns is expected to play important roles not only in early detection and diagnosis of congenital deafness, which triggers intervention, but also in predicting late-onset and progressive hearing loss and identifying individuals who are at risk of drug-induced HL. Concurrent hearing and genetic screening in the whole newborn population in Beijing was launched in January 2012. This study included 180,469 infants born in Beijing between April 2013 and March 2014, with last follow-up on February 24, 2018. Hearing screening was performed using transiently evoked otoacoustic emission (TEOAE) and automated auditory brainstem response (AABR). For genetic testing, dried blood spots were collected and nine variants in four genes, GJB2, SLC26A4, mtDNA 12S rRNA, and GJB3, were screened using a DNA microarray platform. Of the 180,469 infants, 1,915 (1.061%) were referred bilaterally or unilaterally for hearing screening; 8,136 (4.508%) were positive for genetic screening (heterozygote, homozygote, or compound heterozygote and mtDNA homoplasmy or heteroplasmy), among whom 7,896 (4.375%) passed hearing screening. Forty (0.022%) infants carried two variants in GJB2 or SLC26A4 (homozygote or compound heterozygote) and 10 of those infants passed newborn hearing screening. In total, 409 (0.227%) infants carried the mtDNA 12S rRNA variant (m.1555A>G or m.1494C>T), and 405 of them passed newborn hearing screening. In this cohort study, 25% of infants with pathogenic combinations of GJB2 or SLC26A4 variants and 99% of infants with an m.1555A>G or m.1494C>T variant passed routine newborn hearing screening, indicating that concurrent screening provides a more comprehensive approach for management of congenital deafness and prevention of ototoxicity.

2018年3月3日是第19次全国爱耳日和第6次世界听力日.今年爱耳日的主题是“听见未来,从预防开始”.2018年初,中国残疾人联合会、教育部、卫生计生委等15部委联合发出通知[1],号召开展全国性爱耳、护耳活动,大力宣传听力残疾预防的重要性和必要性,贯彻落实《残疾预防和残疾人康复条例》[2],推动建立健全针对不同人群、不同生命周期的听力残疾防控体系.文件中明确提出了“预防为主、关口前移”,“重心下沉、全民动员”的基本原则,并指出要在有效控制出生缺陷和发育障碍致残、着力减少疾病致残、努力减少伤害致残以及完善康复服务体系等方面采取有效行动,有效减少残疾发生.

Objective: This study aims to investigate the prevalence of hearing loss, and to analyse the major demographics and risk factors that influence the prevalence in older adults of China. Design: Cross-sectional investigation. Study sample: Probability proportionate to size sampling (PPS) was adopted for this survey. Among 45,052 individuals, 6984 older adults (>= 60 years) were selected as subjects for this study. Results: The prevalence of hearing loss defined as a speech frequency pure tone average of more than 25 dB HL in the better ear was 58.85%. Age and gender were the factors most strongly associated with hearing loss after multivariate adjustment. Ear disease, diabetes, hypertension, atherosclerosis, noise exposure, and ototoxic drugs were significantly correlated with hearing loss. The largest effects were found for ear disease and noise exposure (OR = 2.83 [95% CI: 2.43-3.29]; OR = 2.59 [95% CI: 1.80-3.72]). Conclusions: Hearing loss is prevalent in nearly two thirds of adults aged 60 years and older in China population. Chronic diseases, ear disease, and noise are important factors in adults aged 60 years and older.

"十三五"时期是全面建成小康社会、打赢脱贫攻坚战的关键阶段,是加快推进听力语言残疾人小康进程的关键时期。3月3日,我们迎来第1 8次"全国爱耳日",为使更多有康复需求的听力残疾人受益,满足听力残疾人个性化需求,中国残联等15个中央、国家部委共同确定"防聋治聋,精准康复"为2017年第18次"全国爱耳日"活动的主题。帮助广大残疾人与全国人民一道共建共享小康社会是"十三五"时期残疾人工作的主要任务。党中央

既往未将双耳非对称性听力损失(asymmetric hearing loss ,AHL )的极端案例 -单侧听力损失(unilateral hearing loss , UHL )或单侧聋(single side deafness ,SSD)纳入人工耳蜗植入的适应症.近年来有人开始尝试为单侧聋伴有严重耳鸣的患者患耳植入人工耳蜗 ,结果发现不但可以有效地减轻甚至消除耳鸣 ,而且还发现适应后可以与健耳听力很好地整合 ,从而达到双耳聆听的效果.

Routing mapping can help most of cochlear implant recipients hear, but special mapping tactics should be applied for some special cases such as recipients suffered from cochlear malformation, ossification, less cooperation with mapping and unusual ex-cochlear electrodes to maximize cochlear implant mapping outcomes.Authors reviewed their very rich mapping experience and summarized in this paper. The purpose is to provide a reference for clinical audiologists who are dedicate in cochlear implant recipients' mapping work.

OBJECTIVE:To investigate the basic factors of the progress amplitude of hearing and speech rehabilitation effect of preschool deaf children with cochlear implants, and provide guidance for the improvement and optimization of rehabilitation strategies.METHOD:Using the standard hearing and language assessment tools, tracked and evaluated 1 422 CI preschool deaf children for a period of one year, and calculated the effect of hearing and speech rehabilitation, carried out the correlation analysis and variance analysis among different grouping variables.RESULT:(1) There was a negative correlation (P<0.01) between the rehabilitation effect and cochlear implantation age, existed the different degree of positive correlation (P<0.01) between the rehabilitation effect and parents cultural level, but no correlation between the rehabilitation effect and parents hearing status.(2) Father's education level, in comparison to mother's education level, had greater impact on the children rehabilitation effect.(3)There was positive correlation(r=0.689, P<0.01) between the progress amplitude of hearing and speech rehabilitation effect. (4) The progress amplitude of auditory and language rehabilitation effect of 2-3 years old group was the highest value(the progress amplitude of hearing and speech recognition rate reached 77.5%, the progress amplitude of language age progress rate reached 2.02 years old), and there were significant differences (P<0.05) between over 3 years old groups.CONCLUSIONS:(1) To expect the better progress amplitude of rehabilitation effect, cochlear implant age should not be more than 3 years old. (2) Father's effect in the process of rehabilitation is more helpful for deaf children's learning enthusiasms.

Objective To investigate the prevalence, etiology, rehabilitation demands and service condition of hearing disorders based on the whole population in Jilin Province, China. Methods Using the probability proportion to size (PPS) sampling, 9246 (93.3%) out of 9909 residents sampled form 36 counties were targeted for investigation from August, 2014 to January, 2015, followed the WHO Ear and Hearing Disorders Survey Protocol. The hearing loss and disability were classified as WHO recommended and Classification and Grading Criteria of Disability (GB/T 26341-2010). Results The standardized prevalence of hearing loss and disability was 16.41%and 4.78%, re-spectively. Age, sex, residence, occupation and marriage status, education level and household income were significantly associated with hearing loss prevalence, while nationality was not. The main etiologies included non-infectious disease (47.33%), ear disease (14.17%), un-known causation (13.89%), and noise (8.59%). Among all people with hearing loss, those who accepted intervention service accounted for 11.02%. Among all people with hearing disability, those who used hearing aids accounted for 5.58%, and 0.67%used artificial cochlea. Con-clusion Demographics and socioeconomic factors are significantly associated with the prevalence of hearing loss. The main etiology con-tains non-infectious disease, ear disease and noise. Both the rate of service utilization among people with hearing loss and the rate of adopt-ing hearing aids among people with hearing disability are low. It is needed to do more in prevention and rehabilitation of hearing impairment.

OBJECTIVE:The purpose of the current study was to evaluate the relationship between the presence or absence of cortical auditory evoked potentials (CAEPs) to speech stimuli and the performance of speech perception in Chinese pediatric recipients of the Nurotron(®) cochlear implant (CI).We also wanted to determine how the CAEPs might be used as an indicator for predicting early speech perception and could provide objective evidence for clinical applications of CAEPs. METHODS:23 pediatric unilateral CI recipients participated in this study. 15 males 8 females, and their ages at implantation ranged from 13 to 68 months, with a mean age of 36 months. CAEPs and Mandarin Early Speech Perception (MESP) tests were used to evaluate the audibility and speech perception of these CI users. The tests were administered at the first, second, third, and fourth year after the CI surgery. RESULTS:All the subjects demonstrated improvements in detection of speech sounds with CI. The percentages of participants who could detect all three stimuli were 26% (6/23) at first year, to 100% (23/23) at the fourth year post-implantation. The percentages of participants who passed the Category 6 of MESP were from 9% (2/23) at first year, to 91% (21/23) at the fourth year post-implantation. Significant correlations (p<0.05) were found between CAEP scores and MESP at the first, second, third year after the CI surgery. The multiple regression equation for prediction of MESP categories from CAEP scores and hearing ages was MESP=1.088+(0.504×CAEP score)+(0.964×hearing ages) (F=72.919, p<0.001, R(2)=0.621). CONCLUSION:The results of this study suggested that aided cortical assessment was a useful tool to evaluate the outcomes of cochlear implantation. Cortical outcomes had a significant positive relationship with the MESP, which predicted the early speech perception of CI recipients.

Objective: To investigate the prevalence, severity of hearing disorders and demographics of people with hearing disorders based on the whole population in Jilin, Guangdong, Shannxi and Gansu provinces in China. Methods: According to " WHO Ear and Hearing Disorders Survey Protocol" , 144 clusters were chosen with probability proportional sampling(PPS) method from the four provinces covering 194, 688, 061 residents. Audiological test, otological examination and questionnaire surveying were conducted for all samples from August, 2014 to September, 2015. The hearing disorders were classified according to WHO criteria and classification. Results: Among 47 511 targeted residents, 45, 052 individuals (94.82% response rate) participated in the survey. The standardized prevalence rates of hearing disorders and disabling hearing disorders were 15.84 % and 5.17 % respectively. Almost 50% of people with hearing disorders had no awareness of it or its starting time. There was significant difference in the prevalence among people of different ages, genders, occupations, provinces, marital status and education levels. The prevalence of hearing disorders increased significantly as age grew. People above 60 years old occupied 55.31% of the total hearing disorders. The prevalence of hearing disorders among male, people of low education and those who lost husband or wife, as well as workers and farmers was relatively higher. Conclusions: The prevalence of hearing disorders is high, and hearing disorders are " invisible" . Demographics and socioeconomic factors significantly influence the prevalence of hearing disorders.

In recent years, hearing and speech rehabilitation in China has achieved a great progress while still facing many challenges, the storage of audiological professional talent is one of the most important problems. As the technology resource center of national hearing and speech rehabilitation, China Rehabilitation Research Center for Deaf Children (CRRCDC)have carried out many programs to cultivate professionals through academic education, certification education, continuing education, etc. But there are still many problems, including supply and demand contradiction, lack of professional talents, low systematic training ability, incomplete support policy for employees, etc. It is urgent to promote education-employment integration construction, cultivate professionals in different levels and better curriculum design.

1前言<br>　　2014年3月3日是第15次全国爱耳日，主题为“爱耳护耳健康听力--预防从初级耳科保健做起”。初级耳科保健的重要任务之一是防止听力障碍的发生。世界卫生组织报道，至少50%的听力障碍是可以预防的[1]，了解和认识导致听力障碍的原因并加以预防及治疗，能有效地降低听力障碍的发生率。

中华医学会耳鼻咽喉头颈外科学分会、中华耳鼻咽喉科杂志编委会2003年在长沙组织讨论并制订了《人工耳蜗植入工作指南（2003年，长沙）》（简称“2003版指南”），对人工耳蜗植入的临床工作起到了积极的指导作用[1]。2007年卫生部颁布了《人工耳蜗临床技术操作规范》[2]。随着人工耳蜗基础研究和临床工作的蓬勃发展以及产品的研发升级，人们对人工耳蜗的认识也在不断提高，原有指南已经不能完全适应临床发展的需要。本刊编委会先后于2006年和2010年组织召开了人工耳蜗植入相关论坛，就当时大家关心的热点问题进行了探讨，初步达成一些共识[3,4]。近年来在国家项目的推动下，人工耳蜗植入数量迅速增长，迫切需要对旧的指南进行修订，出台既能与国际标准接轨，又符合我国国情的新指南。为此，中华耳鼻咽喉头颈外科杂志编委会2012年12月在海南三亚召开了人工耳蜗植入指南修订会，50余位从事人工耳蜗植入相关工作的手术医生、听力学家及康复专家出席了会议。会议在2003版指南的框架基础上，分别就患者选择标准、特殊病例的指导性建议，植入前、后的听力评估及开机调试，言语康复及相关评估等多个议题展开讨论。指南修订以与会专家意见为主体，参照国内近年的相关操作规范以及国外最新的人工耳蜗植入相关指南性文件，同时亦参考大量的国内外相关文献，反复讨论修改，本着求同存异的原则，基本达成共识。