聋儿听觉能力康复效果影响因素的研究

目的:探究影响聋儿听觉能力康复效果的相关因素,为听觉能力康复工作提供参考。方法:收集78例聋儿人口学资料和诊疗资料,对听觉能力康复效果的影响因素进行统计分析。结果:裸耳听阈、助听器听阈、初次听觉康复训练时间、初次验配助听器时间和单双耳助听情况与聋儿听觉能力康复有关,其中助听器听阈、初次验配助听器时间和单双耳验配助听器对听觉能力康复效果影响较为明显(P0.05)。结论:聋儿听觉能力康复影响因素较多,应对助听器听阈等主要影响因素进行重点管控,提高康复训练效果。     

人工耳蜗技术:过去、现在与未来

人工耳蜗技术可以帮助绝大多数重度-极重度感音神经性聋患者恢复听力,此项技术最早始于1800年意大利物理学家Alessandro Volta发现电刺激正常耳可以产生听觉. 20世纪60年代开始进入实用阶段,技术发展的方向包括单通道和多通道技术, 1979年北京协和医院单通道人工耳蜗研制成功, 1980年完成了国内第一例人工耳蜗植入术, 1995年5月,完成国内第一例多通道人工耳蜗植入.随着技术的进步,逐渐出现声电联合刺激人工耳蜗、双侧人工耳蜗植入、机器人辅助人工耳蜗植入等, 2012年北京协和医院在国内首次植入声电联合刺激人工耳蜗,双侧人工耳蜗植入研究也位于国内前列.随着手术成功经验越来越多,人工耳蜗适应症逐渐放宽, 2008年北京协和医院在国内首次研发成功术前电刺激听觉诱发电位技术,大量疑难复杂病例成功植入人工耳蜗.国内外也积极开展单侧耳聋的人工耳蜗植入、神经性耳鸣的人工耳蜗植入应用研究以及机器人辅助下人工耳蜗植入.上海交通大学医学院附属第九人民医院于2020年报道国内首例机器人辅助下人工耳蜗植入.同时光学人工耳蜗的研究也进入实验阶段.本文综述了人工耳蜗技术的国内外发展史、目前技术拓展以及未来发展趋势,以期为人工耳蜗的技术进步提供参考.     

卡那霉素致聋豚鼠耳蜗电极植入模型的建立及意义

目的:探讨人工耳蜗电极的插入对耳蜗功能的影响,为研究人工耳蜗植入建立相应的动物模型。方法:取听力正常的豚鼠8只,4只注射卡那霉素联合呋塞米致聋,为致聋组;4只仅注射生理盐水,为对照组。对两组动物行听性脑干反应(ABR)及耳声发射(DPOAE)检查后,将耳蜗电极植入左侧耳蜗。结果:致聋组术侧4个频率段ABR阈移随着时间的推移逐渐减小,术后24 h、48 h、72 h时间段比较无显著性差异(P0.05);对照组术侧ABR阈移随着时间的推移逐渐减小,32 kHz频率的三个时间段比较有显著性差异(P0.05),其余3个频率无显著性差异。此外,致聋组与对照组术侧耳ABR阈移比较均无显著性差异(P0.05)。致聋组术前5个频率的DPOAE无法引出,术后DPOAE仍无法引出;对照组术前DPOAE均可引出,术后术侧的DPOAE均无法引出。术后72 h可见电极周围有组织包绕,固定良好,局部未见明显炎症反应。结论:本实验成功建立了卡那霉素致聋的豚鼠耳蜗电极植入模型,可为人工耳蜗植入术后颞骨病理改变的研究提供实验基础。     

大前庭导水管综合征11例报告

目的:分析大前庭导水管综合征的临床特点及其预防和治疗。方法:应用听力学检查包括纯音听力、儿童游戏测听、声阻抗、听觉脑干诱发电位(Auditory brainstem responses)、40Hz听觉相关电位、耳声发射、多频稳态和影像学检查(CT和／或MRI检查),对11例病例进行分析。结果:全部为感音神经性聋,其中中重度聋为18％,重度聋为55％,极重度聋为27％。听力曲线高频下降型63％,平坦型23％,岛状听力14％。5例有进行性听力下降或突发性听力下降,2例行前庭功能检查,显示前庭功能低下。高分辩内耳CT检查,均为单纯前庭导水管扩大。结论:认为该病为胚胎发育性疾病,其发生与胚胎早期内淋巴管发育障碍有关,感染和头部震荡是该病发生的主要诱因。该病目前尚无确切有效的治疗方法,对有残余听力的聋儿应尽早选配助听器进行听觉、语言训练,可取得较为满意的效果,而听力损失极重,助听器无法达到有效补偿者,或渐进性听力下降至极重度聋者,可选择电子耳蜗植入。     

双侧突发性耳聋疗效的影响因素分析

目的:探讨影响双侧突发性耳聋疗效的因素。方法:回顾性分析2011年2月至2013年5月我科收治的24例(48耳)双侧突发性耳聋的病例资料。其中,男性18例(75.0%),女性6例(25.0%)。年龄14-72岁,平均年龄49.3岁。按照听力曲线分型,低中频下降型0耳(0.0%),中高频下降型16耳(33.3%),平坦型18耳(27.5%),全聋型14耳(29.2%)。分析和比较具有不同听力曲线分型、伴发症状、年龄、伴发疾病、听力损失程度、发病至干预时间的双侧突发性耳聋患者总有效率的差异。结果:具有不同听力曲线类型、发病至干预时间、听力损失程度以及是否伴有高血压、糖尿病、高血脂、眩晕症状的双侧突发性耳聋患者的总有效率比较差异均无统计学意义(P0.05)。伴发耳鸣的双侧突发性聋患者治疗总有效率显著高于不伴发耳鸣患者,差异有统计学意义(P0.05)。年龄≤60岁的双侧突发性耳聋患者的治疗总有效率显著高于年龄60岁者(P0.05)。结论:伴发耳鸣、年龄小于60岁的双侧突发性耳聋患者的治疗效果相对较好,而听力曲线类型、听力损失程度、发病至干预时间、是否伴发眩晕症状与其治疗效果均无关。     

人工耳蜗植入聋儿术前基因检测及家系分析

摘要: 国内外研究表明GJB2、SLC26A4(PDS)和线粒体DNA(Mitochondrial DNA, mtDNA)的病理性突变导致了大部分的遗传性聋。 文章收集了2006年4月~2007年9月接受人工耳蜗(Cochlear implant, CI)植入的14 例患儿及其父母的外周血, 应用基因诊断方法进行 GJB2、SLC26A4(PDS)和mtDNA 1555位点突变检测。结果显示, 35.7%的患儿检测到致病突变, 其中28.6%为GJB2基因突变, 类型均为235delC纯和突变, 其父母为携带GJB2 235delC的杂和子; 7.1%为mtDNA A1555G突变, 其母亲亦携带mtDNA A1555G突变。这表明CI 植入聋儿最常见的基因突变是GJB2 235delC突变, 其次是mtDNA A1555G突变, 通过对耳聋家系常见致病基因的检测和家系分析, 可以对优生优育及减少耳聋发病率提供科学准确的遗传信息。     

听力障碍青少年治疗过程心理干预对患儿语言能力恢复和心境状态的影响

摘要 目的：探讨听力障碍青少年治疗过程心理干预对患儿语言能力恢复和心境状态的影响。方法：选取我院2019年6月到2020年6月共收治的80例7~18岁听力障碍青少年作为研究对象,将患儿随机分为观察组与对照组,每组40例。对所有患儿应用人工耳蜗植入术进行治疗,给予对照组常规言语训练以及健康教育等干预。观察组患儿在常规干预基础上增加全治疗过程的心理干预。对比两组患儿的治疗效果,语言能力恢复,心境状态,ABC评分。结果：观察组患儿治疗的总有效率为92.50%,对照组患儿治疗的总有效率为75.00%,观察组高于对照组（P＜0.05）;两组患儿干预前的言语形成、听觉能力以及听觉感知对比无明显差异（P＞0.05）,两组患儿通过治疗与干预之后,言语形成、听觉能力以及听觉感知明显提升,且观察组评分高于对照组（P＜0.05）;两组患儿干预前的精神、控制、能力、忍受消极情感以及接受变化对比无明显差异（P＞0.05）,两组患儿通过治疗与干预之后,精神、控制、能力、忍受消极情感以及接受变化明显降低,且观察组评分低于对照组（P＜0.05）;两组干预前的兴奋、易激怒、刻板行为以及不恰当语言等评分比较无统计学意义（P＞0.05）,两组患儿干预后,各评分均下降,其中观察组小于对照组,差异有统计学意义（P＜0.05）。结论：对听力障碍青少年在常规治疗与干预基础上增加心理干预,能够提升患儿的治疗效果,提升语言能力恢复情况,减少负面情绪,提升患儿的心境状态,值得临床应用推广。     

突发性耳聋患者焦虑抑郁状况及其影响因素分析

目的:探讨突发性耳聋患者焦虑、抑郁现状,并分析导致患者焦虑、抑郁的影响因素。方法:采用焦虑自评量表(SAS)、抑郁自评量表(SDS)评价2015年2月至2017年12月我院耳鼻喉科收治的156例突发性耳聋患者的焦虑、抑郁心理状态,收集患者一般资料、发病以及治疗情况,采用Logistic多元回归分析突发性耳聋患者焦虑、抑郁的影响因素。结果:本组患者中发生焦虑者48例,发生率为30.77%,发生抑郁者41例,发生率为26.28%。单因素分析结果显示,突发性耳聋患者焦虑、抑郁的发生与年龄、性别、经济收入、文化程度、伴随症状种类数、听力损失程度、耳聋耳鸣程度、合并基础疾病种类数、治疗疗效有关(P0.05)。多因素分析结果显示,年龄为30-60岁、重度或极重度耳鸣、听力损失程度≥70 dBHL、合并基础疾病≥3种、治疗无效是突发性耳聋患者发生焦虑的危险因素,女性、重度耳聋、合并基础疾病≥3种、治疗无效是突发性耳聋患者发生抑郁的危险因素。结论:突发性耳聋患者焦虑、抑郁发生率较高,其焦虑、抑郁水平受年龄、性别、耳聋耳鸣程度、合并疾病种类数、治疗疗效影响,医护人员应关注患者心理健康,积极治疗原发疾病,控制基础疾病,并进行心理疏导,以减轻患者焦虑、抑郁水平。     

儿童社区获得性支原体肺炎病原学与影像学特征分析

目的:回顾性分析我院儿童社区获得性肺炎(Community-acquired pneumonia,CAP)患儿病原学检测结果,为本地区CAP患儿的临床治疗提供参考依据。方法:选取2014年5月-2018年5月812例符合中华医学会儿科学分会制定的儿童社区获得性肺炎(2013修订)诊断标准的CAP患儿,分析患儿的临床特征及影像学检查结果,并进行分析。结果:非重症CAP患儿812例,CAP占10.2%;感染病原体例数481例,阳性检出率为59.2%;1月-1岁龄组与1-5岁龄组比较差异无统计学意义(P0.05);5-14岁龄组发热发生率明显高于前两组,差异均有统计学意义(P0.05)。咳嗽、喘息、固定湿啰音发生率集中在1月-1岁龄组与1-5岁龄组,且高于5-14岁龄组发生率(P0.0);肺部影像学改变的患儿共657例,占80.9%。三个年龄组;肺部影像学改变差异有统计学意义(P0.05)。结论:不同年龄段CAP患儿病原体检出率不同;不同年龄和不同病原学感染的儿童CAP的影像学表现各有特点,对于临床医生早发现、早诊断、早治疗具有一点的指导性意义。     

父母及个人行为对学龄前儿童超重和肥胖的影响（英文）

目的:探讨学龄前儿童超重和肥胖的现状及其影响因素。方法:从青岛市幼儿园招募年龄在3-6岁儿童参与本调查,通过健康体格检查和问卷调查两部分进行。其中体格检查包括身高和体重的测量,问卷调查内容包括父母的相关变量以及儿童个人饮食行为等因素。其中1080份为完整有效数据。结果:学龄前儿童超重和肥胖人数分别占总数的18.80%和8.98%。男孩的超重和肥胖率(31.8%)高于女生(23.8%)。父亲和母亲的超重和肥胖均与儿童超重和肥胖存在关联性(P0.05)。较长屏幕时间、快速进食是超重和肥胖的危险因素(P0.05),而增加体力活动时间为保护因素(P0.05)。偏爱肉类也是与超重/肥胖相关的因素(P0.05)。结论:孩子个性习惯和父母均与学龄前儿童超重和肥胖相关,学龄前儿童超重和肥胖问题仍然是一个重要的公共卫生问题。     

Timing of Surgical Intervention with Cochlear Implant in Patients with Large Vestibular Aqueduct Syndrome

Objectives

(1) To report the speech perception and intelligibility results of Mandarin-speaking patients with large vestibular aqueduct syndrome (LVAS) after cochlear implantation (CI); (2) to compare their performance with a group of CI users without LVAS; (3) to understand the effects of age at implantation and duration of implant use on the CI outcomes. The obtained data may be used to guide decisions about CI candidacy and surgical timing.

Methods

Forty-two patients with LVAS participating in this study were divided into two groups: the early group received CI before 5 years of age and the late group after 5. Open-set speech perception tests (on Mandarin tones, words and sentences) were administered one year after implantation and at the most recent follow-up visit. Categories of auditory perception (CAP) and Speech Intelligibility Rating (SIR) scale scores were also obtained.

Results

The patients with LVAS with more than 5 years of implant use (18 cases) achieved a mean score higher than 80% on the most recent speech perception tests and reached the highest level on the CAP/SIR scales. The early group developed speech perception and intelligibility steadily over time, while the late group had a rapid improvement during the first year after implantation. The two groups, regardless of their age at implantation, reached a similar performance level at the most recent follow-up visit.

Conclusion

High levels of speech performance are reached after 5 years of implant use in patients with LVAS. These patients do not necessarily need to wait until their hearing thresholds are higher than 90 dB HL or PB word score lower than 40% to receive CI. They can do it “earlier” when their speech perception and/or speech intelligibility do not reach the performance level suggested in this study.     

A Structural Equation Modeling Approach to Examining Factors Influencing Outcomes with Cochlear Implant in Mandarin-Speaking Children

Objective

To examine the direct and indirect effects of demographical factors on speech perception and vocabulary outcomes of Mandarin-speaking children with cochlear implants (CIs).

Methods

115 participants implanted before the age of 5 and who had used CI before 1 to 3 years were evaluated using a battery of speech perception and vocabulary tests. Structural equation modeling was used to test the hypotheses proposed.

Results

Early implantation significantly contributed to speech perception outcomes while having undergone a hearing aid trial (HAT) before implantation, maternal educational level (MEL), and having undergone universal newborn hearing screening (UNHS) before implantation had indirect effects on speech perception outcomes via their effects on age at implantation. In addition, both age at implantation and MEL had direct and indirect effects on vocabulary skills, while UNHS and HAT had indirect effects on vocabulary outcomes via their effects on age at implantation.

Conclusion

A number of factors had indirect and direct effects on speech perception and vocabulary outcomes in Mandarin-speaking children with CIs and these factors were not necessarily identical to those reported among their English-speaking counterparts.     

Determination of Benefits of Cochlear Implantation in Children with Auditory Neuropathy

BackgroundAuditory neuropathy (AN) is a recently recognized hearing disorder characterized by intact outer hair cell function, disrupted auditory nerve synchronization and poor speech perception and recognition. Cochlear implants (CIs) are currently the most promising intervention for improving hearing and speech in individuals with AN. Although previous studies have shown optimistic results, there was large variability concerning benefits of CIs among individuals with AN. The data indicate that different criteria are needed to evaluate the benefit of CIs in these children compared to those with sensorineural hearing loss. We hypothesized that a hierarchic assessment would be more appropriate to evaluate the benefits of cochlear implantation in AN individuals.MethodsEight prelingual children with AN who received unilateral CIs were included in this study. Hearing sensitivity and speech recognition were evaluated pre- and postoperatively within each subject. The efficacy of cochlear implantation was assessed using a stepwise hierarchic evaluation for achieving: (1) effective audibility, (2) improved speech recognition, (3) effective speech, and (4) effective communication.ResultsThe postoperative hearing and speech performance varied among the subjects. According to the hierarchic assessment, all eight subjects approached the primary level of effective audibility, with an average implanted hearing threshold of 43.8 ± 10.2 dB HL. Five subjects (62.5%) attained the level of improved speech recognition, one (12.5%) reached the level of effective speech, and none of the subjects (0.0%) achieved effective communication.ConclusionCIs benefit prelingual children with AN to varying extents. A hierarchic evaluation provides a more suitable method to determine the benefits that AN individuals will likely receive from cochlear implantation.     

Long-Term Asymmetric Hearing Affects Cochlear Implantation Outcomes Differently in Adults with Pre- and Postlingual Hearing Loss

In many countries, a single cochlear implant is offered as a treatment for a bilateral hearing loss. In cases where there is asymmetry in the amount of sound deprivation between the ears, there is a dilemma in choosing which ear should be implanted. In many clinics, the choice of ear has been guided by an assumption that the reorganisation of the auditory pathways caused by longer duration of deafness in one ear is associated with poorer implantation outcomes for that ear. This assumption, however, is mainly derived from studies of early childhood deafness. This study compared outcomes following implantation of the better or poorer ear in cases of long-term hearing asymmetries. Audiological records of 146 adults with bilateral hearing loss using a single hearing aid were reviewed. The unaided ear had 15 to 72 years of unaided severe to profound hearing loss before unilateral cochlear implantation. 98 received the implant in their long-term sound-deprived ear. A multiple regression analysis was conducted to assess the relative contribution of potential predictors to speech recognition performance after implantation. Duration of bilateral significant hearing loss and the presence of a prelingual hearing loss explained the majority of variance in speech recognition performance following cochlear implantation. For participants with postlingual hearing loss, similar outcomes were obtained by implanting either ear. With prelingual hearing loss, poorer outcomes were obtained when implanting the long-term sound-deprived ear, but the duration of the sound deprivation in the implanted ear did not reliably predict outcomes. Contrary to an apparent clinical consensus, duration of sound deprivation in one ear has limited value in predicting speech recognition outcomes of cochlear implantation in that ear. Outcomes of cochlear implantation are more closely related to the period of time for which the brain is deprived of auditory stimulation from both ears.     

Diffusion Tensor Imaging of the Auditory Neural Pathway for Clinical Outcome of Cochlear Implantation in Pediatric Congenital Sensorineural Hearing Loss Patients

Although conventional structural MRI provides vital information in the evaluation of congenital sensorineural hearing loss (SNHL), it is relatively insensitive to white matter microstructure. Our objective was to evaluate possible changes in microstructure of the auditory pathway in children with congenital sensorineural hearing loss (SNHL), and the possible distinction between good and poor outcome of cochlear implantation (CI) patients by using diffusion tensor imaging (DTI). Twenty-four patients with congenital SNHL and 20 healthy controls underwent conventional MRI and DTI examination using a 1.5T MR scanner. The DTI metrics of fractional anisotropy (FA) and mean diffusivity (MD) of six regions of interest (ROIs) positioned along the auditory pathway—the trapezoid body, superior olivary nucleus, inferior colliculus, medial geniculate body, auditory radiation and white matter of Heschl''s gyrus—was measured in all subjects. Among the 24 patients, 8 patients with a categorie of auditory performance (CAP) score over 6 were classified into the good outcome group, and 16 patients with a CAP score below 6 were classified into the poor outcome group. A significant decrease was observed in FA values while MD values remained unchanged at the six ROIs of SNHL patients compared with healthy controls. Compared to good outcome subjects, poor outcome subjects displayed decreased FA values at all of the ROIs. No changes were observed in MD values. Correlation analyses only revealed strong correlations between FA values and CAP scores, and strong correlations between CAP scores and age at implant were also found. No correlations of FA values with age at implant were observed. Our results show that preoperative DTI can be used to evaluate microstructural alterations in the auditory pathway that are not detectable by conventional MR imaging, and may play an important role in evaluating the outcome of CI. Early cochlear implantation might be more effectively to restore hearing in SNHL patients.     

Long-Term Cochlear Implant Outcomes in Children with GJB2 and SLC26A4 Mutations

Objectives

To investigate speech and language outcomes in children with cochlear implants (CIs) who had mutations in common deafness genes and to compare their performances with those without mutations.

Study Design

Prospective study.

Methods

Patients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study. All patients underwent mutation screening of three common deafness genes: GJB2, SLC26A4 and the mitochondrial 12S rRNA gene. The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale, speech perception tests and language skill tests.

Results

Forty-eight patients were found to have confirmative mutations in GJB2 or SLC26A4, and 123 without detected mutations were ascertained for comparison. Among children who received CIs before 3.5 years of age, patients with GJB2 or SLC26A4 mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p = 0.001 for CAP; p = 0.004 for SIR) and year 5 (p = 0.035 for CAP; p = 0.038 for SIR). By contrast, among children who received CIs after age 3.5, no significant differences were noted in post-implant outcomes between patients with and without mutations (all p > 0.05).

Conclusion

GJB2 and SLC26A4 mutations are associated with good post-implant outcomes. However, their effects on CI outcomes may be modulated by the age at implantation: the association between mutations and CI outcomes is observed in young recipients who received CIs before age 3.5 years but not in older recipients.     

Environmental Sounds Recognition in Children with Cochlear Implants

The aims of this study were (1) to document the recognition performance of environmental sounds (ESs) in Mandarin-speaking children with cochlear implants (CIs) and to analyze the possible associated factors with the ESs recognition; (2) to examine the relationship between perception of ESs and receptive vocabulary level; and (3) to explore the acoustic factors relevant to perceptual outcomes of daily ESs in pediatric CI users. Forty-seven prelingually deafened children between ages 4 to 10 years participated in this study. They were divided into pre-school (group A: age 4–6) and school-age (group B: age 7 to 10) groups. Sound Effects Recognition Test (SERT) and the Chinese version of the revised Peabody Picture Vocabulary Test (PPVT-R) were used to assess the auditory perception ability. The average correct percentage of SERT was 61.2% in the preschool group and 72.3% in the older group. There was no significant difference between the two groups. The ESs recognition performance of children with CIs was poorer than that of their hearing peers (90% in average). No correlation existed between ESs recognition and receptive vocabulary comprehension. Two predictive factors: pre-implantation residual hearing and duration of CI usage were found to be associated with recognition performance of daily-encountered ESs. Acoustically, sounds with distinct temporal patterning were easier to identify for children with CIs. In conclusion, we have demonstrated that ESs recognition is not easy for children with CIs and a low correlation existed between linguistic sounds and ESs recognition in these subjects. Recognition ability of ESs in children with CIs can only be achieved by natural exposure to daily-encountered auditory stimuli if sounds other than speech stimuli were less emphasized in routine verbal/oral habilitation program. Therefore, task-specific measures other than speech materials can be helpful to capture the full profile of auditory perceptual progress after implantation.     

Neural Correlates of Speech Processing in Prelingually Deafened Children and Adolescents with Cochlear Implants

Prelingually deafened children with cochlear implants stand a good chance of developing satisfactory speech performance. Nevertheless, their eventual language performance is highly variable and not fully explainable by the duration of deafness and hearing experience. In this study, two groups of cochlear implant users (CI groups) with very good basic hearing abilities but non-overlapping speech performance (very good or very bad speech performance) were matched according to hearing age and age at implantation. We assessed whether these CI groups differed with regard to their phoneme discrimination ability and auditory sensory memory capacity, as suggested by earlier studies. These functions were measured behaviorally and with the Mismatch Negativity (MMN). Phoneme discrimination ability was comparable in the CI group of good performers and matched healthy controls, which were both better than the bad performers. Source analyses revealed larger MMN activity (155–225 ms) in good than in bad performers, which was generated in the frontal cortex and positively correlated with measures of working memory. For the bad performers, this was followed by an increased activation of left temporal regions from 225 to 250 ms with a focus on the auditory cortex. These results indicate that the two CI groups developed different auditory speech processing strategies and stress the role of phonological functions of auditory sensory memory and the prefrontal cortex in positively developing speech perception and production.