语前聋儿童人工耳蜗植入后听觉言语能力评估

目的：评估语前聋儿童人工耳蜗植入术后听觉言语能力的发展规律及其影响因素。方法对107例语前聋儿童人工耳蜗植入术后,应用婴幼儿有意义听觉整合量表（infant－toddler meaningful auditory integration scale／meaningful auditory integration scale ,IT －MAIS／MAIS）、听觉行为分级（categories of auditory performance , CAP）和言语可懂度分级（speech intelligibility rating ,SIR）三种问卷评估其听觉言发展水平,分析植入时间、植入年龄、实际年龄、术前助听器使用情况等对其的影响。结果107例语前聋儿童的植入年龄、植入时间对IT －M AIS／MAIS得分率有显著影响,植入时间、实际年龄对CAP、SIR分级有显著影响（P值均＜0．05）,而术前是否佩戴助听器对IT －MAIS／MAIS得分率、CAP、SIR分级均无显著影响（均为 P＞0．05）。结论植入年龄、植入时间、实际年龄对语前聋儿童人工耳蜗植入后的听觉言语能力发展有显著影响,而术前是否佩戴助听器对其听觉言语能力的影响有待后续研究证实。     

影响人工耳蜗植入术后效果的原因分析

目的　探讨儿童人工耳蜗植入术后言语康复效果的影响因素,更科学的指导聋儿言语康复。方法　选择在我院接受诺尔康CS-10A人工耳蜗植入的76例儿童患者,术后1年使用听觉行为分级标准（categories of auditoryper formance,CAP）和言语可懂度分级标准（speech intelligibility rating,SIR）对患儿术后日常生活环境中的听觉感知水平和言语表达能力作出分级评估。结果　统计学分析显示植入年龄、耳蜗发育有无畸形、家长文化程度、家人是否积极参与患儿术后康复、患儿每天佩戴耳蜗的时间 和术前有无助听器佩戴史6个方面均与术后言语康复效果具有很好相关性。结论　植入年龄越小、耳蜗发育正常、家长文化程度高、家长参与、每天佩戴耳蜗时间越长和术前佩戴助听器均利于患儿言语康复。     

语后聋患者人工耳蜗植入后听觉行为及言语可懂度分级的相关因素分析

目的：应用听觉行为分级（CAP）和言语可懂度分级（SIR）评估语后聋患者人工耳蜗植入后言语交流状况,探讨其得分与听力下降时间、植入时间、术前助听器使用情况的关系。方法：回顾分析23例语后聋人工耳蜗植入者的临床资料,收集其听力下降时间、植入时间、术前助听器使用情况等相关资料,应用CAP、SIR分级评估其言语交流状况。运用IBM SPSS Statistics19软件进行统计描述及结果分析。结果：CAP分级平均得分为6．13分,统计分析示CAP分级与听力下降时间、植入时间、术前助听器使用时间无显著相关关系（均P〉0．05）。患者均为语后聋,其SIR分级的平均得分为4．91分。结论：语后聋患者人工耳蜗植入后CAP分级与听力下降时间、植入时间、术前助听器使用情况无相关性。这可能是由于CAP分级的精确性和细节上的欠缺或该研究样本量限制,未显示出其与3个变量之间的关系。SIR不适用于评估语后聋患者人工耳蜗植人后的言语状况。     

贵州省常见致聋基因突变患儿人工耳蜗植入术后康复效果评估

目的对不同致聋基因突变患儿人工耳蜗植入后1年的听力言语康复效果进行评估,为人工耳蜗手术前预估术后康复效果提供参考。方法对常见的遗传性聋基因突变热点进行检测,对检测结果为杂合突变的人工耳蜗植入者进行相关基因全序列分析。根据基因结果,80例患儿分为4组,SLC26A4基因突变组、GJB2基因突变组、线粒体基因突变组,对照组为未发现SLC26A4、GJB2、线粒体基因突变患儿,使用声场扬声器测试人工耳蜗植入后助听听阈,采用人工耳蜗植入术后效果评估体系对患儿术后听觉言语能力进行评估,比较4组患儿CI植入术后1年康复效果的差异。结果 CI植入术后1年所有人工耳蜗患儿听觉言语能力较术前明显提高,各组患儿术前术后的听觉言语得分差异显著。GJB2基因突变组的患儿术后1年IT-MAIS、CAP分级和SIR分级得分高于对照组,其差异有统计学意义,声场助听听阈及言语年龄和SLC26A4基因突变组及对照组相比略有优势,但无统计学意义;SLC26A4基因突变组术后1年统计学分析显示各项评估结果略高于对照组,不及GJB2基因突变组,但差异无统计学意义;线粒体12S rRNA基因突变组术后1年仅2例各项得分接近对照组平均水平。结论不同致聋基因突变的患儿CI植入术后听觉言语能力均有改善,GJB2基因突变组人工耳蜗植入后1年效果较好,SLC26A4基因突变组效果优势有待长期观察进一步证实。     

人工耳蜗植入术295例语言康复临床分析

目的 研究耳蜗植入术后听觉言语康复效果及其影响因素。 方法 采用听觉行为分级(CAP)、言语可懂度分级(SIR)及视频和录音言语清晰度分析法分别对295例人工耳蜗植入术后患者进行听觉言语康复效果评估。 结果 2例蜗神经细小合并内耳畸形患者开机听阈为45 dB,其余听阈为25~40 dB。单因素分析结果显示:术前助听器的佩戴、植入年龄、植入后时间、术前残余听力、内耳畸形程度、脑白质病变对CAP及SIR差异有统计学意义;手术方法、中耳炎、Waardenburg综合征对CAP、SIR差异无统计学意义。多因素Logistic回归分析结果显示:植入后时间、脑白质病变与CAP、SIR显著相关。 结论 大部分患者可从人工耳蜗植入术中获得满意效果,其听觉言语康复程度受多种因素的影响。     

语前聋患儿一侧人工耳蜗对侧助听器效果分析

目的:通过对人工耳蜗植入对侧耳不同听力损失的患儿联合使用助听器与人工耳蜗语前聋患儿的听觉、语言及学习能力进行评估和比较,探索对患儿更为有效的助听方法,帮助患儿获得最大限度的言语交流。方法:将30例3～6岁语前聋患儿按照植入人工耳蜗对侧耳听力损失程度及是否佩戴助听器,分为一侧人工耳蜗+对侧重度听力损失助听器组(CI+SHA组)、一侧人工耳蜗+对侧极重度听力损失助听器组(CI+PHA组)、单耳人工耳蜗组(CI组)。评估各组在康复3、6、9、12、15、18个月时听觉、语言及学习能力,并记录结果。结果:随着术后康复时间的延长,聋儿听觉、语言及学习能力逐渐提高(P<0.05),CI+SHA组听觉能力优于CI+PHA组及CI组(均P<0.05),语言能力及学习能力无明显差异(P>0.05)。结论:语前聋患儿单耳人工耳蜗植入后,若对侧耳尚有残余听力,佩戴助听器后听觉能力效果显著,长期佩戴有助于患儿的康复。     

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。     

语前聋人工耳蜗植入患者听觉和言语康复效果的问卷分级评估

目的 使用听觉和言语问卷分级的方法评估人工耳蜗植入患者的听觉言语康复效果,分析康复效果的相关影响因素。探讨人工耳蜗术后听觉言语康复效果的问卷评估方法。方法 对97例语前聋人工耳蜗植入患者的家长和康复教师进行调查随访。根据听觉行为分级标准(categoriesof auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rating,SIR)对患者的听觉感知能力和言语产生能力进行分级评估。CAP共分1-8级,SIR共分1-5级。用组内单因素秩和检验和多因素Logistic回归分析CAP和SIR分级结果与植入电极类型、植入年龄、病因、病程、术前助听器使用情况、植入深度、植入时间、康复模式、家庭经济状况等9个因素的关系。结果 单因素分析结果显示:植入体型号(P=0.0439)、植入时间长短(P=0.0001)、康复模式(P=0.0460)、家庭经济状况(P=0.0140)与CAP有关;植入时间长短(P=0.0001)、康复模式(P=0.0271)与SIR有关。植入年龄、病因、病程、植入深度以及术前助听器的佩戴与CAP和SIR均无关。多因素Logistic回归分析结果显示:植入时间长短和家庭经济状况与CAP显著相关;植入时间长短与SIR显著相关。结论植入时间越长,人工耳蜗对患者听觉言语发展的效果越明显。康复模式与手段对人工耳蜗植入后患者的言语和康复能     

语前聋人工耳蜗植入患者听觉和言语康复效果的问卷分级评估

目的使用听觉和言语问卷分级的方法评估人工耳蜗植入患者的听觉言语康复效果，分析康复效果的相关影响因素。探讨人工耳蜗术后听觉言语康复效果的问卷评估方法。方法对97例语前聋人工耳蜗植入患者的家长和康复教师进行调查随访。根据听觉行为分级标准(categories of auditory performance，CAP)和言语可懂度分级标准(speech intelligibility rating，SIR)对患者的听觉感知能力和言语产生能力进行分级评估。CAP共分1—8级，SIR共分1—5级。用组内单因素秩和检验和多因素Logistic回归分析CAP和SIR分级结果与植入电极类型、植入年龄、病因、病程、术前助听器使用情况、植入深度、植入时间、康复模式、家庭经济状况等9个因素的关系。结果单因素分析结果显示：植人体型号(P=0．0439)、植入时间长短(P=0．0001)、康复模式(P=0．0460)、家庭经济状况(P=0．0140)与CAP有关；植入时间长短(P=0．0001)、康复模式(P=0．0271)与SIR有关。植入年龄、病因、病程、植入深度以及术前助听器的佩戴与CAP和SIR均无关。多因素Logistic回归分析结果显示：植入时间长短和家庭经济状况与CAP显著相关：植入时间长短与SIR显著相关。结论植入时间越长，人工耳蜗对患者听觉言语发展的效果越明显。康复模式与手段对人工耳蜗植入后患者的言语和康复能力有着决定性的影响。     

语前聋患者人工耳蜗植入术后听觉言语康复相关影响因素的研究

目的探讨语前聋人工耳蜗植入患者的听觉言语康复效果及其相关影响因素。方法对159例人工耳蜗植入患者家长和康复教师进行调查随访。根据听觉行为分级标准(Categories of Auditory Performance,CAP)和言语可懂度分级标准(Speech Intelligibility Rating,SIR),对患者的听觉能力和言语能力进行分级评估。用组内单因素秩和检验和多因素Logistic回归分析CAP和SIR分级结果与性别、惯用手、内耳发育、颅脑影像学情况、术前助听器使用、术前语训、植入耳侧、植入电极类型、植入年龄、植入后时间、术后语言环境、术后康复模式、家庭经济状况等13个因素的关系。结果单因素分析结果显示,不同的术前助听器使用情况、术前语训、植入后时间、家庭经济状况组间CAP分级结果有显著性差异（P值均<0.05）;不同的植入耳侧、植入后时间、家庭经济状况组间SIR分级结果有显著性差异（P值均<0.05）。多因素Logistic回归分析结果显示,植入后时间、家庭经济状况与CAP、SIR分级结果均有显著相关。结论植入后时间、家庭经济状况对人工耳蜗植入术后患者的听觉言语康复效果起决定性的作用。     

GJB2相关非综合征性感音神经性聋人工耳蜗植入后的疗效观察

目的：评价携带GJB2基因突变的儿童人工耳蜗植入后的疗效。方法：对40例携带GJB2基因突变的人工耳蜗植入患儿进行疗效评估,测试内容包括：声场助听听阈测试、（婴幼儿）有意义听觉整合量表、听觉行为分级标准评分、言语可懂度分级标准评分及普通话早期言语感知测试。以80例未检出已知热点突变者为对照组。结果：携带GJB2基因突变的患儿术后声场助听听阈优于对照组,其余测试与对照组比较差异无统计学意义（P〉0．05）。结论：携带GJB2基因突变的非综合征性感音神经性聋患儿适于行人工耳蜗植人手术,术后效果满意。     

Auditory performance and speech intelligibility of Mandarin-speaking children implanted before age 5

Objectives

(1) To report the auditory performance and speech intelligibility of 84 Mandarin-speaking prelingually deaf children after using cochlear implants (CIs) for one, two, three, four, and five years to understand how many years of implant use were needed for them to reach a plateau-level performance; (2) to investigate the relation between subjective rating scales and objective measurements (i.e., speech perception tests); (3) to understand the effect of age at implantation on auditory and speech development.

Methods

Eighty-four children with CIs participated in this study. Their auditory performance and speech intelligibility were rated using the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales, respectively. The evaluations were made before implantation and six months, one, two, three, four, and five years after implantation. At the fifth year after implantation, monosyllabic-word, easy-sentence, and difficult-sentence perception tests were administered.

Results

The median CAP score reached a plateau at category 6 after three years of implant use. The median SIR arrived at the highest level after five years of use. With five years of CI experiences, 86% of the subjects understood conversation without lip-reading, and 58% were fully intelligible to all listeners. The three speech perception tests had a moderate-to-strong correlation with the CAP and SIR scores. The children implanted before the age of three years had significantly better CAP and monosyllabic word perception test scores.

Conclusions

Five years of follow-up are needed for assessing the post-implantation development of communication ability of prelingually deafened children. It is recommended that hearing-impaired children receive cochlear implantation at a younger age to acquire better auditory ability for developing language skills. Constant postoperative aural–verbal rehabilitation and speech and language therapy are most likely required for the patients to reach the highest level on the CAP and SIR scales.     

伴脑白质病语前聋患儿人工耳蜗植入术后听觉言语康复效果分析

目的 探讨伴脑白质病的语前极重度感音神经性聋患儿人工耳蜗植入的可行性及术后听觉言语康复效果.方法 海南省人民医院耳鼻咽喉科2013年9~11月行人工耳蜗植入的14例伴脑白质病语前聋患儿为研究组(年龄1~6岁,平均3.79±1.93岁),同期无中枢神经系统病变的语前聋患儿16例为对照组(年龄1~6岁,平均4.38±1.93岁),术前均行临床听力学、影像学检查及语言能力、智力水平等评估,经乳突后鼓室面神经隐窝入路一期行人工耳蜗植入术,术后均到海南省聋儿康复中心进行言语康复训练;采用听觉行为分级标准(categories of auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rate,SIR)对两组术后康复效果进行评估,比较两组患儿术后不同阶段的CAP和SIR分级.结果 所有患儿人工耳蜗植入术后均获得听觉反应和不同程度的言语交流能力,随着康复时间延长两组患儿CAP及SIR分级均呈上升趋势;研究组术后6、12和24个月CAP平均分级分别为2.571±0.416、3.714±0.496、5.000±0.492级,SIR平均分级分别为1.357±0.133、2.143±0.275、3.071±0.245级,与对照组CAP(分别为2.688±0.313、3.875±0.364、5.000±0.354级)及SIR(分别为1.500±0.129、2.313±0.176、2.875±0.221级)比较,差异均无统计学意义(P>0.05).结论 伴脑白质病的语前极重度感音性聋患儿经过术前充分评估后可以实施人工耳蜗植入,术后2年内听觉言语康复效果与不伴脑白质病的同龄患儿相当.     

Successful cochlear implantation in prelingual profound deafness resulting from the common 233delC mutation of the GJB2 gene in the Japanese

OBJECTIVES: Recently, we identified three novel mutations of the GJB2 gene in Japanese families with autosomal-recessive non-syndromic deafness.1 Seven of 11 mutated chromosomes (63.6%) contained a 233delC allele, suggesting that the 233delC mutation is the most common mutation of the GJB2 gene in the Japanese population. After it was recognized that cochlear implantation (CI) is of benefit to children with prelingual deafness, we have had a number of prelingual pediatric CI patients. Because children carrying the homozygous 233delC mutation show bilateral prelingual profound deafness, they could be enrolled in the CI program at Osaka University Graduate School of Medicine. The purposes of this study were 1) to analyze the occurrence of the GJB2 mutations in our 15 prelingual pediatric CI patients in whom the cause of non-syndromic deafness was unknown, and 2) to evaluate the auditory function and postoperative speech perception with CI of those GJB2-related deaf subjects. STUDY DESIGN: Retrospective analysis. METHODS: Mutation analysis of the GJB2 gene by direct sequencing was performed with genomic DNA from 15 children born profoundly deaf as a result of unknown causes and implanted with CI. Intraoperative electrically evoked auditory brainstem response (EABR) and intra-/postoperative EAP were measured. The speech perception was evaluated with Infants and Toddlers Meaningful Auditory Integration Scale (IT-MAIS). RESULTS AND CONCLUSIONS: We identified 4 CI patients (26.7%) out of 15 children carrying the homozygous 233delC mutation. Intra- and postoperative evaluation of the auditory system revealed almost intact cochlear and retrocochlear auditory function in these 4 patients. Postoperative auditory testing indicates that their speech perception had become significantly higher in comparison with that of other prelingual CI patients. These results suggest that prelingual deaf children carrying the homozygous 233delC mutation of the GJB2 gene can benefit from CI.     

Analysis of the developmental trajectory and influencing factors of auditory and speech functions after cochlear implantation in Mandarin Chinese speaking children

Abstract

Background: The auditory and speech development of the children with cochlear implants (CIs) are influenced by many factors.

Objective: To study the developmental trajectory and influencing factors of auditory and speech functions for the Mandarin Chinese speaking children with CIs.

Material and methods: The children with CIs undergoing rehabilitation in the same institution from June 2016 to June 2019 were followed up. Their closed monosyllables and disyllables recognition rate, closed average language age, categories of auditory performance (CAP) and speech intelligibility rating (SIR) were evaluated at 0, 1, 3, 6, 12 and 24?months of rehabilitation. The results were analyzed by SPSS 23.0.

Results: 49 children were followed up for 1?year, 29 children for 2?years. The evaluated indicators of auditory and speech functions were improved with the prolongation of rehabilitation and influenced by the age of cochlear implantation, the use of hearing aids before surgery, guardian’s educational degree, the relationship between guardian and child.

Conclusions and significance: The auditory and speech functions of the children with CIs were improved significantly with the prolongation of rehabilitation and influenced by many factors, which can help us to predict the effect of CI more accurately and develop an individualized rehabilitation program.     

43例人工耳蜗植入患者基因检测及术后康复效果分析

目的总结川南地区43例人工耳蜗植入患者的基因突变类型,分析其人工耳蜗术后康复效果,了解两者的相关性。方法对43例耳聋患者行GJB2、SLC26A4、mtDNA 12S rRNA基因检测,有基因位点发生致病突变者为耳聋基因检测阳性,归为A组;其余为基因检测阴性,归为B组。两组患者均行单侧人工耳蜗植入术,于术前、术后3、6、12个月行听觉行为分级（categories of auditory performance,CAP）、言语可懂度分级（speech intelligibility rating,SIR）评估。结果43例患者中基因位点发生致病突变者14例（A组）;耳聋基因筛查阴性29例（B组）。A组中9例患者GJB2检测阳性,6例患者SLC26A4检测阳性,其中1例患者GJB2、SLC26A4检测结果均为阳性,两组患者术后1年内不同时间点 CAP 和SIR 评分均较术前明显提高,且评分随着术后时间的延长逐渐提高(P<0.05),但两组患者术前及术后不同时间点CAP和SIR评分比较均无差异(P>0.05)。结论43例患者中常见致病耳聋基因为GJB2、SLC26A4,人工耳蜗植入术能有效提高患者听力及言语能力,但耳聋基因突变与人工耳蜗植入术后康复效果无明显相关性。     

听神经病谱系障碍患者人工耳蜗术后长期听力康复效果分析

目的 探讨听神经病谱系障碍患者人工耳蜗植入术后长期听力康复效果.方法 回顾性分析46例经我院诊断为听神经病谱系障碍的患者并行人工耳蜗植入(Cochlear Implantation,CI)患者的临床资料、听力及颞骨影像检查结果.对其中12例患儿通过听觉行为分级标准量表(CAP)、言语可懂度分级标准量表(SIR)两种问卷...     

Infants versus older children fitted with cochlear implants: performance over 10 years

Objectives

To investigate the efficacy of cochlear implants (CIs) in infants versus children operated at later age in term of spoken language skills and cognitive performances.

Method

The present prospective cohort study focuses on 19 children fitted with CIs between 2 and 11 months (X = 6.4 months; SD = 2.8 months). The results were compared with two groups of children implanted at 12-23 and 24-35 months. Auditory abilities were evaluated up to 10 years of CI use with: Category of Auditory Performance (CAP); Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS); Peabody Picture Vocabulary Test (PPVT-R); Test of Reception of Grammar (TROG) and Speech Intelligibility Rating (SIR). Cognitive evaluation was performed using selected subclasses from the Griffiths Mental Development Scale (GMDS, 0-8 years of age) and Leiter International Performance Scale-Revised (LIPS-R, 8-13 years of age).

Results

The infant group showed significantly better results at the CAP than the older children from 12 months to 36 months after surgery (p < .05). Infants PPVT-R outcomes did not differ significantly from normal hearing children, whereas the older age groups never reached the values of normal hearing peers even after 10 years of CI use. TROG outcomes showed that infants developed significantly better grammar skills at 5 and 10 years of follow up (p < .001). Scores for the more complex subtests of the GMDS and LIPS-R were significantly higher in youngest age group (p < .05).

Conclusion

This study demonstrates improved auditory, speech language and cognitive performances in children implanted below 12 months of age compared to children implanted later.     

The effect of GJB2 and SLC26A4 gene mutations on rehabilitative outcomes in pediatric cochlear implant patients

To analyze the treatment outcomes in pediatric cochlear implant patients with mutations in GJB2 or SLC26A4 and to determine these mutations’ impact on rehabilitative outcomes. The study included 41 children who received unilateral cochlear implantations. Fifteen of these children had GJB2-related deafness, 10 had SLC26A4-related deafness, and 16 had deafness of unknown etiology. Speech perception and language development evaluations, including the Meaningful Auditory Integration Scale (MAIS), categories of auditory performance (CAP), speech intelligibility rating (SIR) and babbling spurt, were conducted before and after the implantation. Better results for the GJB2 group (vs. the control group) were observed regarding MAIS, CAP and SIR at 24 months after implantation (P < 0.05). The performance of GJB2 group was better than SLC26A4 group, expressed by a significant difference in the variance of CAP and SIR at 24 months postoperatively (P < 0.05). A trend towards earlier babbling spurt onset could be observed for the GJB2 group, intergroup comparison did not reveal any significant difference among the three groups (P > 0.05). The SLC26A4 group performed better than the control group at 12 and 24 months postoperatively, although without a statistically significant difference (P > 0.05). The GJB2 gene mutations had a significantly positive impact on the outcome of cochlear implantation. Patients with SLC26A4-related deafness were shown to benefit from cochlear implantation.