人工耳蜗术前电刺激听觉脑干诱发电位评估残余听力及术后效果分析

目的 探讨最大声刺激无反应患者人工耳蜗术前电刺激听觉脑干诱发电位系统(electrically evoked auditory brainstem responses,EABR)检测听觉通路的方法及分析术后康复效果。方法 选取中国医学科学院北京协和医院2015～2016年最大声刺激无反应患者21例,分析临床资料,人工耳蜗术前采用EABR检测听觉通路,随访术后神经反应遥测(neural response telemetry,NRT)及调机结果,应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 rating,SIR)问卷评价术后听力和言语结果;另选择有残余听力耳聋患者10例作为对照组。结果 实验组21例患者术前EABR的Ⅴ波阈值(178.23±14.18)CL,术后听力言语康复CAP(5.50±1.75)等各指标和对照组统计学分析有显著差别(P<0.05);术后均获得不同程度电听觉,开机测试NRT引出率(33%)低于对照组(90%);两组患者I/O曲线斜率和术后CAP...     

听神经病人工耳蜗植入术中EABR监测及其疗效评估

目的:探讨听神经病的病变部位并观察听神经病人工耳蜗植入术后的疗效。方法:选择在我院行人工耳蜗植入术的8例听神经病患者,同时筛选背景接近的8例非听神经病极重度聋人工耳蜗植入患者作为对照组进行配对。回顾性分析2组患者术前听力学资料;术中耳蜗植入前采用自制电极行电刺激听性脑干诱发反应(EABR)评估听觉通路;植入后检测神经反应遥测(NRT)、EABR;并随访术后调机T、C值,采用听觉行为分级标准(CAP)、言语可懂度分级标准、言语识别率评估术前和开机后1年时听力言语情况。结果:8例听神经病患者术中植入前均可引出EABR波形,但是波形较正常有变异,需要刺激量加大或者调整参数,Ⅴ波潜伏期长短不一;植入耳蜗后NRT反应好,EABR和术中测试波形接近。术后开机均有听性反应,随访开机T、C值与对照组差异无统计学意义;听神经病组患者术前及开机1年CAP分别为6.50±0.94和6.90±0.77,言语识别率(双字词)分别为(85.00±11.66)%和(89.50±9.02)%,与对照组比较差异无统计学意义。结论:术前EABR可以做为评估听神经病(听同步不良患者)听觉通路的有效工具,筛选合适植入者;人工耳蜗可以帮助听神经病患者提高听力及言语能力。     

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。     

人工耳蜗植入前电刺激听神经复合动作电位检测方法的建立和初步应用

目的 建立术中利用探测电极施行电刺激听神经复合动作电位(electrically evoked auditory nerve compound active potentials,ECAP)检测的方法,在植入人工耳蜗装置前评估患者耳蜗听神经功能状况.方法 选择20例人工耳蜗植入患者,其中耳蜗形态发育正常12例,5例双侧前庭导水管扩大,3例双侧耳蜗Mondini畸形.测试完成后全部使用Cochlear人工耳蜗.全麻后常规人工耳蜗手术进路,行标准耳蜗鼓阶开窗,将自制测试用多通道试验电极置入鼓阶,电极连接Cochlear公司体外言语处理器及自制电刺激发生器,连接电脑,采用Custom Sound EP 2.0软件,调整优化刺激参数进行神经反应遥测(neural responsetelemetry,NRT)初步了解听神经功能状态;刺激强度以5 CL为步长递减或递增至反应阈值给予电刺激脉冲,同时自动记录ECAP波形和阈值.植入人工耳蜗后常规进行NRT检测,记录ECAP波形和阈值;术后1个月患者开机后采集T、C值,将两种电极测试所得阈值和开机C值进行相关性研究,并进行数据统计分析.结果 试验电极ECAP引出率为90%,商业电极ECAP引出率为90%,平均阈值分别为(160.50±15.12)CL和(160.00±11.27)CL,两者经统计学检验没有显著性差异(P>0.05);和开机后C值(177.40±10.61)有明显相关性(R2=0.844,r=0.919).结论 成功建立了术中植入人工耳蜗装置前的ECAP检测方法,为内耳和/或听觉通路发育异常及无残余听力患者提供有效的听神经反应信息,对了解听觉系统发育程度及初步预测术后患者康复情况提供客观依据.     

不同内耳畸形患儿人工耳蜗植入后听觉言语能力分析CSCD

目的通过与耳蜗结构正常的人工耳蜗植入(cochlear implant,CI)儿童进行对比研究,探讨不同内耳畸形儿童CI术后的听觉言语发育情况。方法本研究共纳入116例语前聋CI儿童。耳蜗结构正常组50例;内耳畸形组66例,其中前庭导水管扩大(large vestibular aqueduct syndrome,LVAS)组24例,Mondini畸形组14例,共同腔畸形(common cavity deformity,CCD)组12例,内听道狭窄组16例(10例伴蜗神经发育不良)。使用婴幼儿有意义听觉整合量表(infants meaningful auditory integration scale,IT-MAIS)、有意义使用言语量表(meaningful use of speech scale,MUSS)、听觉行为分级问卷(categories of auditoryperformance,CAP)和言语可懂度分级问卷(speech intelligibilityrating,SIR)评估比较各畸形组与耳蜗结构正常组术后0、6、12、24个月听觉言语康复效果。结果经过统计分析得出,听觉感知能力方面,LVAS组及Mondini组与耳蜗结构正常组IT-MAIS和CAP得分在各评估阶段均无显著差异(P>0.05),CCD组及内听道狭窄组与耳蜗结构正常组在开机后的6、12、24个月均有显著差异(P<0.05);言语能力方面,LVAS组与耳蜗结构正常组MUSS和SIR得分在各评估阶段均无统计学差异(P>0.05),Mondini组与耳蜗结构正常组MUSS得分在术后12、24个月有统计学差异(P<0.05),SIR得分在术后各评估阶段均无显著差异,CCD组、内听道狭窄组与耳蜗结构正常组MUSS得分在术后0、6、12、24个月均有差异(P<0.05),SIR得分在术后12、24个月差异有统计学意义(P<0.05)。结论不同内耳畸形患儿CI术后听觉言语发育轨迹与耳蜗结构正常患儿相似,畸形类型不同术后效果差异较大,术前应根据畸形类型选择个性化耳蜗植入方案。     

不同脉宽条件下人工耳蜗植入者术中 电诱发听性脑干反应检测的特点分析

摘要：目的比较不同脉宽条件下人工耳蜗植入者术中电诱发听性脑干反应（electrically evoked auditory brainstem response,EABR）的特点,分析脉宽与EABR波V引出率和阈值之间的关系,总结不同脉宽条件对EABR波V引出率和阈值的影响,选择更优化的EABR脉宽测试参数。方法无残余听力的人工耳蜗植入患者24例作为实验组,其中耳蜗形态正常12例、大前庭导水管综合征（large vestibular aqueduct syndrome,LVAS）4例、Mondini畸形4例、共同腔畸形（common cavity deformation,CCD）2例、内听道狭窄2例;筛选条件相近的24例有残余听力的人工耳蜗植入者配对作为对照组。应用改装的Cochlear Freedom人工耳蜗及自制的铂铱合金球电极对拟行人工耳蜗植入的两组者术中分别给予50、100、200 μs不同脉宽的电刺激,Bio logic Navigator Pro听觉诱发电位仪记录不同脉宽条件下EABR波V引出率和阈值。结果脉宽50、100、200 μs时,EABR波V引出率实验组（均为91.7%）低于对照组（分别为100%、100%、95.8%）,差异无统计学意义（P均>0.05）;而EABR阈值实验组［分别为(183.73±8.96)CL、(151.28±10.05)CL、(120.56±12.82)CL］高于对照组［分别为(175.50±9.14)CL、(142.71±11.45)CL、(110.63±10.24)CL］,差异均具有统计学意义(t值分别为18.87、16.82、17.64,P均<0.05)。结论蜗内单极刺激能诱发出良好的EABR波形,无残余听力的患者EABR阈值要明显高于有残余听力的患者。相对于脉宽200 μs,50、100 μs时EABR波形波V引出率高;脉宽50 μs波形分化更好、动态范围广,内耳畸形严重可能需要适当加大刺激量（如将脉宽改为100 μs）。     

人工耳蜗植入后EABR动态变化的初步研究

目的观察人工耳蜗植入儿童电诱发听性脑干反应(electrically auditory evoked response EABR)的动态变化,了解慢性电刺激后听觉通路反映特性的变化,为听觉系统的可塑性变化研究提供实验依据。方法本研究采用前瞻性设计,对19例平均年龄为3.2±1.0岁的语前聋儿童在接受人工耳蜗植入术中电极植入后进行EABR检测,患者在人工耳蜗植入后5.4±3.2月后再次进行EABR检测,观察EABR阈值、波III、波V潜伏期以及EABR输入输出曲线指标的变化。结果EABR平均阈值从人工耳蜗植入术中的196.9±11.1CL下降到术后5.4±3.2个月的189.2±13.2CL CL,配对t检验显示显著性差异（p=0.006）。平均阈上20CL V波潜伏期从人工耳蜗植入术中的4.72±0.21ms缩短到术后5.4±3.2月的4.60±0.18ms,配对t检验显示显著性差异（p=0.032）。结论耳聋儿童在接受人工耳蜗电刺激后EABR的反应阈值和潜伏期变化在最初的5.4±3.2月已经出现,提示3岁左右年龄段儿童听觉系统具有较好的可塑性,人工耳蜗植入后早期的听觉、言语训练尤为重要。     

人工耳蜗植入术中EABR监测的应用

目的探讨人工耳蜗植入术中进行EABR监测的方法，以了解耳蜗电刺激下听觉传导通路的神经反应情况。方法20例人工耳蜗植入患者，男14例，女6例，平均年龄13．6岁，语前聋患者14例，语后聋患者6例。全麻后安置体表记录电极，将PPS与听觉诱发电位仪触发端口连接，并选定听觉诱发电位仪的外触发模式。人工耳蜗电极植入后，先行常规NRT监测，然后将NRT刺激参数改为EABR模式，采用Basic双极刺激，脉宽50μs，强度由200CL起以10CL为步长递减至反应阈值。结果20例患者均记录到EABR，阈上20CL时Ⅲ波．Ⅴ波的平均潜伏期分别为2．04±0．20ms．3．96±0．41ms。相同刺激条件下的EABR反应平均阈值为148．46±11．63CL，NRT反应平均阈值为160．72±13．56CL。一例脑白质轻度发育异常患儿，术中NRT波形引出良好，EABRⅠ～Ⅳ波分化良好，Ⅴ波波形低钝，Ⅴ波／Ⅲ波振幅比〈1／2，考虑可能存在耳蜗核上性神经发育不良，现正在语言康复训练随访中。结论人工耳蜗植入术中进行EABR监测比NRT能提供更完整的．更接近听觉中枢的神经反应信息，能更进一步了解听觉传导通路的功能状态，以期对患者听力康复的效果提供更准确的预测。     

内耳畸形人工耳蜗植入术后听力言语康复效果分析

目的：使用听觉和言语问卷分级及术后产生听觉的最小电流值（T值）的方法,评估并比较内耳畸形与正常解剖结构语前聋患儿人工耳蜗植入术后的听觉言语康复效果。方法：按术前影像学检查将语前聋人工耳蜗植入患儿分为正常结构组和内耳畸形组,并配对组合,对思儿家长进行问卷形式调查随访,对术后听力及言语康复效果进行评估分析,记录术后1年凋机T值。用秩和检验比较2组听觉行为分级标准（CAP）、言语可懂度分级标准（SIR）结果及T值。结果：人工耳蜗植入患儿家长术前主要担心术后效果不理想及手术并发症的发生,多数认为听力言语康复训练应由医疗机构进行;秩和检验显示：2组CAP及SIR均无显著差异,术后1年调机时产生听觉的T值无显著差异。结论：①内耳畸形患儿人工耳蜗植入术后,经正规康复训练,听力言语康复效果与内耳解剖结构正常植入者相同,人工耳蜗植入术可帮助伴耳蜗畸形的极重度感音神经性聋患者重建听力,重返主流社会;②听力言语康复训练尚有很多方面需要改进。     

以耳部症状首发、合并鼻咽占位的肉芽肿性多血管炎1例并文献复习

目的 探讨1例临床少见的以耳部症状首发、合并鼻咽部占位的肉芽肿性多血管炎(GPA)的临床特征、实验室检查、病理表现及诊疗过程。方法 回顾性分析患者的病例资料,总结病例特点并回顾国内外GPA相关文献。结果 患者以中耳炎、迷路炎症状为首发表现,合并鼻咽部占位,病程中逐渐出现面瘫,三叉神经刺激症状加重。多次留取耳及鼻咽部活检示急慢性炎症细胞浸润。升级抗生素,同时为避免中耳炎侵犯岩骨及颅内行乳突开放术。中耳局部炎症改善后其耳痛、面瘫等仍不缓解,但激素治疗有效,遂进一步完善自免病相关检查并再次行鼻咽部活检,最终确诊为GPA,予激素及免疫抑制剂治疗得以控制症状。术后3个月暂无显著肺部及肾脏受累表现。结论 临床上发现不典型的中耳炎或常规治疗反复不愈,且逐渐进展出现内耳、颅神经侵犯表现如眩晕发作、面神经麻痹等,同时激素治疗有效,且合并鼻咽部占位、鼻窦炎影像学表现,或累及其他器官如肺、肾脏时,均应考虑到GPA的可能。当反复留取病理活检未能取得特异性确诊依据时,动态监测抗中性粒细胞胞浆抗体、红细胞沉降率、尿潜血、胸部CT、血肌酐等也具有重要的提示意义。     

Decreased miRNA-320e correlates with allergy in children with otitis media with effusion

ObjectiveOtitis media with effusion (OME) is a common childhood disease and the main cause of conductive hearing loss in this age group. Many factors predispose to OME but allergy is still widely disputed. The answer may lay in the molecular mechanisms of ear exudate formation and the recent studies showed miRNAs might take part in it. MiRNAs are also potent regulators of allergic response. As miRNAs are present in the middle ear, we hypothesized their expression differs between allergic and non-allergic patients and reflects the difference in pathomechanism of effusion formation between these two groups.Materials and methodsThis study aimed to establish the expression of 5 different miRNAs (miR-223-3p, miR-451a, miR-16-5p, miR-320e, miR-25-3p) in ear exudates in children diagnosed with OME. The allergy group consisted of 18 patients whereas the non-allergic group had 36 patients. MicroRNA was isolated from the middle ear fluid collected during myringotomy and transcribed into cDNA. MiRNA expression was measured with TaqMan™ MicroRNA Assays and analyzed with DataAssist software. The comparative CT method was used for calculating the relative quantification of gene expression based on the endogenous control gene expression (U6 snRNA-001973).ResultsMiR-320e expression was significantly decreased in allergic children with OME. Other studied miRNAs also showed reduced expression in allergic children, but the decrease was not significant.ConclusionsMiRNA expression differs between children with and without allergy in the course of OME, but further studies are needed to explain the exact role of miR-320e and its target genes in OME pathology in allergic patients.     

Experience with vertical partial laryngectomy with special reference to laryngeal reconstruction with cervical fascia

In this paper we report our experience of vertical partial laryngectomy using the superficial cervical fascia; we describe the technique and present the functional and oncological results of this method of treatment. A total of 42 patients with squamous cell carcinoma of the true vocal folds, in stage T(1) (n = 28) or T(2) (n = 14), were treated in our department using vertical partial laryngectomy during the decade 1987-1997. Nine patients had post-operative radiotherapy. The shortest follow-up time was three years. There were six recurrences in all, four in the larynx and two in the neck. All four of the laryngeal recurrences were treated with total laryngectomy and are doing well. Both the patients with neck metastases, who were treated with neck dissection, died. Permanent tracheotomy was necessary in one patient. There were no problems with aspiration. The recurrence rate was 14 per cent, the three-year survival index was 95.2 per cent and the three-year larynx preservation index was 90 per cent. According to our experience, vertical partial laryngectomy, using the method we describe, has a good functional and oncological result for stage T(1) and T(2) tumours.     

Cytokines in children with otitis media with effusion

We assayed 38 middle ear effusions from 23 children aged 4–13 years (mean 7) undergoing tympanostomy tube placements. All fluid was assayed for tumor necrosis factor (TNF) α, interleukin (IL) 1β, IL-8, and IL-10. Cytokine concentrations were measured by means of an enzyme-linked immunosorbent assay. Detectable levels of IL-1β, IL-8, and IL-10 were found in all of the effusions. TNF-α was detected in 18 of the middle ear effusions (47.4%). The mean concentration of TNF-α, IL-1β, IL-8, and IL-10 was, respectively, 0.423 ± 1.39, 30.58 ± 68.7, 7001.9 ± 6743, and 56 ± 58.7 pg/ml. There was a strong, statistically significant correlation between the concentrations of TNF-α and IL-1β (r = 0.87, P = 0.001) and between IL-1β and IL-8 (r = 0.53, P = 0.001). There was no correlation between the concentrations of IL-10 and other cytokines examined or between tympanic membrane pathology and the concentrations of TNF-α, IL-1β, IL-8, or IL-10. The presence of IL-10 in middle ear effusions may be one of the causes of a lack of clinical features of acute inflammation and may lead to a chronic inflammatory state. Received: 25 August 1999 / Accepted: 5 January 2000     

Polymyositis with dysphagia treated with endoscopic balloon dilatation

Polymyositis is characterized by non-specific inflammatory disease associated with an autoimmune disorder involving muscles of the limbs and neck. We report a case of an 80-year-old man who was referred to our clinic with a chief complaint of dysphagia and muscle weakness in all four limbs. The patient was diagnosed with polymyositis based on pathological findings, muscle weakness, electromyogram findings, and an elevated creatine phosphokinase level. The patient was also positive for HLA-DR3. Intravenous predonine administration was initiated, but dysphagia was not improved. We considered a cricopharyngeal myotomy, but this could not be performed because of heart failure. Endoscopic balloon dilation was performed and dysphagia improved on the same day. Therefore, we suggest that this method is a safe and effective approach for polymyositis with dysphagia.     

Otoacoustic emissions in children with otitis media with effusion

OBJECTIVES: Otoacoustic emissions (OAE) are transmitted from the cochlea to the ear canal via the middle ear and the transmission properties of the middle ear directly influence OAE characteristics. The purpose of this study was to establish the mechanisms of changes occurred in middle ear by tympanometric, audiometric and OAE examination. METHODS: Audiometric and tympanometric examination were performed and otoacoustic emissions were recorded from 22 normal ears and 52 ears with middle ear effusions and repeated 3 months later. RESULTS: Results of the air conduction in study group were significantly different from the control group and we found significant recovery in 3 months. When we analysed the DPOAE evaluation results in our study, some of the DPOAE parameters were found to be different between the control and the study group at low frequencies. Changes in the amplitude, especially at low frequencies, were statistically significant after 3 months. CONCLUSION: The results of this study revealed that measurement of otoacoustic emissions, especially distortion product otoacoustic emissions, is helpful in evaluating the condition of middle ear during the treatment.