脑白质异常的语前聋患儿人工耳蜗植入术后听觉言语康复效果评估

目的：评价脑白质异常的极重度感音神经性聋患儿人工耳蜗植入术后的听觉言语康复效果。方法：采用听觉行为分级标准（CAP）和言语可懂度分级标准（SIR）对伴脑白质异常的语前聋患儿（研究组）进行术后效果调查,并与无综合征及明显病因的人工耳蜗植入患儿（对照组）进行比较。采用两配对样本的t检验比较两组不同阶段的CAP和SIR的结果。结果：经统计学分析,研究组人工耳蜗植入术后6个月CAP和SIR得分与对照组比较差异有统计学意义（P〈O．05）,术后12个月、24个月差异无统计学意义（P〉O．05）。结论：从短期来看,轻度脑白质异常的语前聋患儿人工耳蜗植入术后,经过正规康复训练,其听力言语康复效果与无综合征及明显病因的语前聋患儿无显著差别。     

单侧人工耳蜗植入学龄前耳聋儿童听觉言语康复效果影响因素分析

目的：探讨学龄前耳聋儿童单侧人工耳蜗植入（cochlear implantation ,CI）后早期康复效果的影响因素。方法对行单侧人工耳蜗植入术且在听觉言语康复机构按照中国残联聋康中心标准进行听觉言语康复1年以上的165例双耳极重度感音神经性聋患儿进行听觉行为分级（categories of auditory performance ,CAP）、言语可懂度分级（speech intelligibility rating ,SIR）评估,分析性别、植入年龄、基因突变类型、助听器佩戴经验以及内耳畸形与康复效果的关系。结果经过多因素回归分析,植入年龄、基因突变与CI儿童的CAP、SIR得分增长相关（P＜0．05）,而性别、助听器佩戴史及内耳畸形（大前庭水管综合症）与CAP、SIR得分增长无关（P＜0．05）。1～3岁组CAP提高速度最快,2、3、4岁组SIR增长速度最快;GJB2基因突变致聋的CI儿童康复12个月后的CAP、SIR得分均高于非G IB2突变者。结论植入年龄为1～3岁者早期听觉康复速度较快,植入年龄为2～4岁者早期言语能力发育速度较快;G JB2突变致聋的儿童人工耳蜗植入后听觉言语康复效果更优;大前庭水管综合症患儿人工耳蜗植入效果与非大前庭水管患儿相同。     

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。     

人工耳蜗植入术后听力及言语康复效果相关因素的初步研究

目的 通过对55例人工耳蜗植入患儿的听力及言语能力进行评估,分析各种可能因素在人工耳蜗植入术后听力及言语康复过程中的作用。方法 通过问卷调查的方式对人工耳蜗植入患者的各项可能影响因素进行统计,并根据听觉行为分级标准（CAP）和言语可懂度分级标准（SIR）对患儿的听力及言语能力进行评估。使用卡方检验对影响术后康复效果的相关因素进行单因素分析;最后将从单因素分析中筛选出来的听觉及言语康复效果的影响因素进行logistic回归分析。结果 logistic回归结果显示植入后时间及家庭经济状况与CAP显著相关,植入后时间、居住地、术后康复模式与SIR显著相关。结论 人工耳蜗植入术后时间、经济因素是影响人工耳蜗植入术后患儿康复效果的重要因素。     

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。     

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.     

伴脑瘫语前聋儿童的人工耳蜗植入

目的探讨伴脑瘫语前聋儿童人工耳蜗植入的疗效。方法回顾性分析1例5年前行人工耳蜗植入术的脑瘫患儿的临床资料及术后5年来的肢体运动状况,并以听觉行为分级标准(categories of auditory performance,CAP)和言语可懂度分级标准(speech intelligibility rating,SIR)评估并比较其手术前后听力言语能力。结果术后该患儿成功重建了听力,经过肢体康复训练,躯体运动障碍完全康复。术后5年声场测听为轻度听力下降,语言能力较听觉能力提高缓慢。CAP为5级,SIR为2级。普通话声调识别率约为43%,声母、韵母识别率分别约为50%、55%。结论对于伴有脑瘫的语前聋儿童经过术前全面评估和审慎观察后可以实施人工耳蜗植入手术,术后需要制定特殊的康复计划方能让患者从人工耳蜗植入术中受益,人工耳蜗植入可以作为脑瘫聋儿的康复手段之一。     

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。     

内耳畸形语前聋患儿人工耳蜗术后康复效果分析

目的　评估内耳畸形语前聋患儿人工耳蜗植入术后康复结果。方法　回顾性分析术前影像学检查为内耳畸形语前聋人工耳蜗21例,并筛选背景接近的21例正常耳蜗结构语前聋人工耳蜗植入患儿配对组合。对患者术前、术后6个月及术后1年3个阶段听力言语康复效果进行评估。结果　9例前庭导水管扩大综合征和6例Mondini 畸形患者与耳蜗结构正常者相比,听觉言语结果无明显差异。畸形比较严重的1例Mondini和5例共同腔患者与耳蜗正常者相比,听觉言语结果有明显的差异。结论　前庭导水管扩大综合征患者人工耳蜗术后效果与耳蜗发育正常者相似,但对严重的Mondini 畸形和共同腔等内耳畸形者行人工耳蜗植入时术前应准确评估畸形的程度及伴发的畸形,以助于术后制定康复方案。     

内耳结构异常患者人工耳蜗植入术的疗效观察

目的分析研究内耳结构异常患者人工耳蜗植入术的安全性及手术后的疗效情况。方法选取2011.07-2015.06在我院住院接受人工耳蜗植入的17例内耳结构异常患者的临床资料,其中语前聋14例,语后聋3例,针对内耳结构异常情况采用不同术式。用听觉分级行为标准(Categorise of Auditory Performance,CAP)、言语可懂度分级标准(Speech Intelligibility Rating,SIR)进行术后效果评估。选取24例同期接受人工耳蜗植入的内耳正常的语前聋患者作为对照组,分析并比较两组患者手术并发症及术后疗效。结果:17例患者手术顺利,术后出现1例眩晕,所有患者术后均无脑脊液耳漏、面瘫等并发症。内耳结构异常语前聋患者术后CAP和SIR值分别为5.8±1.4、3.5±1.1;对照组CAP和SIR值分别为6.9±1.3、3.8±0.9,两者差异无统计学意义(P>0.05)。结论内耳结构异常非人工耳蜗植入术的绝对禁忌证,根据异常程度制定个性化的手术方案,可以减少并发症的发生。内耳结构异常语前聋患者术后言语康复情况、对声音的自发性觉察能力与耳蜗形态正常患者术后无明显差异。     

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.     

Cochlear implantation in patients with auditory neuropathy of varied etiologies

OBJECTIVES/HYPOTHESIS: Auditory neuropathy is a relatively recently described pattern of hearing loss characterized by preservation of outer hair cell function despite absent brainstem auditory evoked responses. Intact outer hair cell function is demonstrated by the presence of otoacoustic emissions and/or a measurable cochlear microphonic on electrocochleography, whereas no synchronous neural activity (absent action potentials) is seen on acoustically evoked brainstem auditory evoked response testing. The study reviews the authors' experience with six patients diagnosed with auditory neuropathy, four of whom have undergone cochlear implantation. MATERIALS AND METHODS: A retrospective review of all medical and audiological charts at the University of Virginia Hospitals (Charlottesville, VA) was performed to identify patients who have undergone cochlear implantation or have been diagnosed with auditory neuropathy, or both. RESULTS: Six patients with hearing loss attributable to auditory neuropathy were identified, four of whom have undergone cochlear implantation. Causes varied, including congenital, infectious, and idiopathic origins. Adults demonstrated subjective auditory perception on promontory stimulation, whereas no repeatable brainstem auditory evoked response waveforms could be demonstrated on pediatric promontory stimulation testing. Patients with implants demonstrated implant-evoked brainstem auditory evoked responses and improved audiological performance. CONCLUSIONS: The six cases presented in the study represent varied causes and, probably, varied sites of lesions of auditory neuropathy. Promontory stimulation has been valuable, particularly in adults. Cochlear implantation allows the opportunity to provide a supraphysiological electrical stimulation to the auditory nerve, with the hope of reintroducing synchronous neural activity. Greater confidence and enthusiasm for cochlear implantation in appropriately selected patients with auditory neuropathy are gained through experience with such diverse cases.     

不同内耳畸形人工耳蜗植入效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with bilateral inner ear malformations. METHODS: Among 410 patients who received multi-channel cochlear implantations from 1996 to 2004 in Beijing Tongren Hospital, 82 patients were diagnosed with inner ear malformations and implanted. A retrospective analysis was performed about the surgical characteristics and mapping characteristics after implantation. RESULTS: (1) All patients had auditory sensations. (2) Gusher was more common than the normal cochlear implantation. (3) The electrodes were inserted in the "cochleostomy" in full length of 80 Patients, but 2 pairs of electrodes remained outside of "cochleostomy" in 2 patients. (4) No serious complications occurred after implantation. (5) The impedance of the electrodes, the T level and C level were similar with the normal cochlear implantation. The results had no significant difference in compare with normal cochlear group (P > 0. 05). (6) The abilities of speech discrimination and spoken language were improved through rehabilitation. CONCLUSIONS: The cochlear implantation can be performed safely in inner ear malformations. The outcome of hearing rehabilitation for patients with inner ear malformations are similar to those children with normal cochlear structure followed the multi-channel cochlear implantation.     

Age at implantation and auditory memory in cochlear implanted children

Abstract

Early cochlear implantation, before the age of 3 years, provides the best outcome regarding listening, speech, cognition an memory due to maximal central nervous system plasticity. Intensive postoperative training improves not only auditory performance and language, but affects auditory memory as well. The aim of this study was to discover if the age at implantation affects auditory memory function in cochlear implanted children. A total of 50 cochlear implanted children aged 4 to 8 years were enrolled in this study: early implanted (1-3y) n = 27 and late implanted (4-6y) n = 23. Two types of memory tests were used: Immediate Verbal Memory Test and Forward and Backward Digit Span Test. Early implanted children performed better on both verbal and numeric tasks of auditory memory. The difference was statistically significant, especially on the complex tasks. Early cochlear implantation, before the age of 3 years, significantly improve auditory memory and contribute to better cognitive and education outcomes.     

内耳共同腔畸形电刺激听觉脑干诱发电位辅助人工耳蜗植入术及其疗效评估

目的:探讨内耳共同腔畸形儿童人工耳蜗植入的诊断、手术后调机及听力言语康复效果.方法:回顾性分析术前影像学证实common cavity内耳畸形的语前聋人工耳蜗植入儿童19例,术中采用电刺激听觉脑干诱发电位(EABR)辅助评估听神经通路;并筛选临床资料相近的19例正常耳蜗结构的语前聋儿童人工耳蜗植入者配对,分析术前听力、...     

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.     

Mondini畸形多道人工耳蜗植入的效果分析

OBJECTIVE: To describe clinical experiences with multi-channel cochlear implantation in patients with Mondini malformation. METHODS: Among 300 patients who received multi-channel cochlear implants from 1996 to 2002 in Beijing Tongren Hospital, 15 patients were diagnosed with Mondini malformation. A retrospective analysis was performed dealing with the surgical techniques, mapping and rehabilitations characteristics after surgery. 15 patients with normal cochlear structure are consider as control group. RESULTS: Gusher is found more common than the normal cochlear implantation, most of them are serious. The electrodes are inserted in the "cochleostomy" in full length of 13 Patients, 2 pairs of electrodes remains outside of "cochleostomy" in 2 patients. No serious complications occurred after implantation. All patients have auditory sensations. The impedance of the electrodes, the T level, C level and the hearing threshold are similar with the normal cochlear implantation group. The results have no significant difference in compare with normal cochlear group(P > 0.05). CONCLUSION: Multi-channel cochlear implantation could be performed safely in patients with Mondini malformation. The primary outcome for patients with Mondini malformation are similar to those with normal cochlear structure following the multi-channel cochlear implantation.     

不同月龄婴幼儿人工耳蜗植入术后听觉能力发育规律及特点

目的：总结不同月龄婴幼儿人工耳蜗植入术后听觉能力的发育规律及特点,为婴幼儿患儿人工耳蜗植入年龄的选择及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139例极重度语前聋婴幼儿患儿参加本次研究。按手术时年龄将患儿分为A组（≤12个月）、B组（13～18个月）、C组（19～24个月）、D组（25～30个月）、E组（31～36个月）。使用婴幼儿有意义听觉整合量表（IT-MAIS）并由经过培训的听力学专业人员对患儿术后听觉能力进行评估。评估采用面对面询问的方式,由患儿家长或监护人回答。评估人员将家长或监护人对每一问题的回答进行详细记录并根据患儿听觉行为出现的频率对其进行评分。分别在术前和术后开机后1、3、6、12个月时对各组患儿进行评估。结果：经两组独立样本T检验,不同性别患儿在术前和术后不同时期听觉能力的差异均无统计学意义（均P〉0．05）。经单因素方差分析,在术前和开机后1、3、6、12个月各年龄组患儿听觉能力的差异均无统计学意义（均P〉0．05）。各年龄组患儿听觉能力得分随开机时间的增加而增加,其差异有统计学意义（P〈0．05）。结论：不同性别婴幼儿患儿术后听觉能力的发育遵循相同的规律。婴幼儿患儿手术时年龄对术后听觉能力的发育影响不大。大部分患儿的听觉能力在术后6个月之内提高较快,其后进入较缓慢的发展期。     

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.     

1例耳蜗前庭神经畸形患者耳蜗植入术前的功能性磁共振评估并文献复习

目的:探讨功能性磁共振(fMRI)评价耳蜗前庭神经畸形患者是否有人工耳蜗植入指征的可能性。方法:对1例15岁双侧耳聋患者,在人工耳蜗植入前进行听力学、影像学检查及fMRI综合评估。结果:纯音测听和听觉脑干反应显示患者为双侧极重度感音神经性聋,瞬态诱发性耳声发射双侧均未引出。颞骨薄层CT显示内耳畸形(不全分隔Ⅰ型),MRI斜矢状位重建显示耳蜗前庭神经畸形。fMRI显示左耳给声,1000Hz100dBHL,右侧听皮层被激活;右耳给声,双侧听皮层均未见激活。左侧人工耳蜗植入术后1个月开机,患者对声音有反应。结论:结合听力学和影像学检查,fMRI可评估人工耳蜗植入候选者,尤其是在影像学检查显示患者耳蜗前庭神经发育畸形时,更是一种有效的评估方法。