内耳畸形小儿人工耳蜗植入术后神经反应遥测值的研究

目的　探讨小儿内耳畸形人工耳蜗植入手术后,植入体不同电极神经反应遥测（neural response telemetry,NRT）的变化特点及规律。方法　对36例澳大利 亚Cochlear Nucleus 24型内耳畸形小儿人工耳蜗植入手术患儿,测试手术中及手术后1年NRT值,并分析其特点及变化规律。结果　患儿术中及开机后1年内同一时间高、中、低频段电极NRT值有显著差异（P<0.05）,低频值较低,高频值较高,总的趋势是术中至术后1年NRT值逐渐增高。结论　内耳畸形小儿人工耳蜗植入手术后NRT值随时间逐渐增高,高、中、低频之间差异大,故调机时要注意各频率之间差异,选取高、中、低频代表性电极测试,以使患者语言感知达到最佳状态。     

小儿正常耳蜗与内耳畸形人工耳蜗植入术后电极阻抗及NRT值对比研究

目的：探讨小儿正常耳蜗与内耳畸形人工耳蜗植入手术后,植人体电极阻抗值及神经反应遥测（NRT）值的变化特点及规律。方法：将88例澳大利亚CochlearNucleus24型人工耳蜗植人手术患儿分为正常耳蜗组与内耳畸形耳蜗组,测试手术中及手术后1年阻抗值及NRT值,并分析对比。结果：两组患者阻抗值差异有统计学意义（P〉0．05）,其阻抗变化趋势相同。开机3个月内阻抗值变化明显,其后趋于稳定。两组患者NRT值差异有统计学意义（P〈0．05）,其NRT值变化趋势相同。总的趋势是低频值较低,高频值较高,术中至术后1年NRT值逐渐增高。结论：电极阻抗值及NRT值开机3个月内变化明显,故需多次调机,以使患者语言感知达到最佳状态。     

电诱发听性脑干反应和电诱发镫骨肌反射阈值在内耳畸形儿童人工耳蜗植入术后调机中的运用

目的　探讨内耳畸形小儿人工耳蜗植入手术后,植入体电诱发听性脑干反应（electrically evoked auditory brainstem responses,EABR）、电诱发镫骨肌反射阈值（electrically evoked stapedius reflex threshold,ESRT）的变化特点及规律,以指导术后设备调试。方法　将88例澳大利亚Cochlear Nucleus24型人工耳蜗植入手术患儿分为耳蜗形态正常组与内耳畸形组,测试手术后1年内不同时期EABR和ESRT值,术后1年运用行为测试法检测主观阈值（T值）和最大舒适阈（C值）,分析特点及变化规律。结果  内耳畸形组患儿术后不同时期EABR和ESRT阈值较正常组高（P＜0.05）,两组EABR和ESRT阈值变化趋势相同,总的趋势是低频值较低,高频值较高,术后1年EABR和ESRT阈值逐渐增高;两组EABR与T值显著相关,ESRT与C值显著相关。结论　内耳畸形组人工耳蜗植入手术后EABR和ESRT阈值变化规律及特点与正常组患儿相同,阈值可用于指导内耳畸形人工耳蜗植入者手术后设备的调试。     

不同内耳畸形人工耳蜗植入效果分析

目的 就不同内耳畸形人工耳蜗植入特点及效果进行分析,积累经验。方法 1996年1月～2004年5月北京同仁医院共开展多道人工耳蜗植入术410例(410耳),对其中双侧内耳畸形患者82例(82耳)进行回顾性分析,就不同原因内耳畸形的人工耳蜗植入手术特点及术后开机调试特点与耳蜗形态正常的人工耳蜗植入者进行了比较。结果 ①所有内耳畸形患者人工耳蜗植入术后都有听觉;②内耳畸形人工耳蜗术中容易出现井喷;③82例中除2例患者各遗留2对蜗外电极外,其余电极均完全植入蜗内;④术后无面瘫、脑脊液漏等并发症;⑤术后开机调试数值与结构正常植入者接近,无统计学差异(P>0.05);⑥术后经过语训,1年后听力及言语能力均有不同程度的提高。结论 伴有内耳发育畸形的深度感音性聋患者可以行人工耳蜗植入手术,植入效果与耳蜗发育正常患者的人工耳蜗植入效果基本一致,人工耳蜗植入术可以作为伴有内耳畸形的深度感音性聋患者的治疗和康复手段。     

磁共振内耳三维重建在Ⅱ型Waardenburg综合征患者中的应用

目的探讨磁共振内耳三维重建在Ⅱ型Waardenburg综合征中的应用。方法回顾性分析2008～2011年期间行人工耳蜗植入术的6例Ⅱ型Waardenburg综合征患儿术前磁共振内耳三维重建影像学检查结果,观察内耳的发育情况及人工耳蜗植入效果。结果 2例内耳无明显异常,4例有半规管畸形,3例有耳蜗畸形,术中均顺利植入全部电极,术后均顺利开机,听觉和言语水平明显提高。结论术前磁共振内耳三维重建影像学检查可清晰显示Ⅱ型Waardenburg患者的前庭、半规管及耳蜗情况,为内耳畸形特别是耳蜗畸形患者的人工耳蜗植入手术提供指导。     

Mondini畸形多道人工耳蜗植入的效果分析

目的　探讨Mondini畸形患者人工耳蜗植入手术的可行性效果及手术特点。方法　我院自 1996年开展多道人工耳蜗植入术以来 ,截止到 2 0 0 2年 10月已经开展手术 30 0例 ,其中典型的Mondini畸形患者 15例 (2 8耳 )。本组对这 15例 (15耳 )植入手术患者进行回顾性分析 ,就Mondini畸形人工耳蜗植入手术特点、开机调试结果、言语康复与耳蜗形态正常的人工耳蜗植入者进行了比较。任意抽出 15例 (30耳 )耳蜗结构正常的人工耳蜗植入者作为对照组与Mondini畸形者进行比较。结果　①Mondini畸形术中 11耳出现井喷现象 ,较对照组多见 ,且井喷比较汹涌 ;严重者术中及术后需要使用甘露醇降颅压 ;② 13例 (13耳 )电极全部植入耳蜗内 ,2例 (2耳 )遗留 2对蜗外电极 ;③术后无面瘫、脑脊液漏、脑膜炎等手术并发症 ;④所有Mondini畸形患者人工耳蜗植入术后都有听觉 ;⑤开机调试时电极反应阈值、最大舒适阈值及阻抗值与对照组数值接近 ,经统计学比较差异无显著性 (P>0 0 5 )。术后平均听阈 (35dBHL)与对照组 (30dBHL)无明显区别。结论　人工耳蜗植入可以作为伴有Mondini畸形的双侧深度感音性聋患者的听觉康复手段 ,植入效果与对照组接近     

内耳畸形聋儿人工耳蜗植入术常见类型及手术并发症

目的 探讨内耳畸形聋儿实施人工耳蜗植入术时常见的类型及并发症。方法 回顾性分析电子耳蜗植入术病历资料170例,对其中的32例双侧内耳畸形患者加以畸形类型及手术并发症总结。结果 ①人工耳蜗植入患儿内耳畸形所占比例(32/170,18.8%)明显高于其他文献报道;②32例内耳畸形中,大前庭导水管23例(占全部畸形数71.3%),大前庭导水管伴其他类型畸形者5例(并发Mondini畸形4例,并发外半规管未发育1例),Mondini畸形2例,Mondini畸形并发外半规管未发育前庭腔扩大1例,耳蜗CT影像疑似为“三叉”无法分类1例;③术中发生严重井喷3例(耳蜗CT影像疑似为“三叉”畸形、Mondini畸形并发外半规管未发育前庭腔扩大1例,及大前庭导水管并发Mondini畸形1例);④耳蜗影像疑似为“三叉”患者,术中发生严重井喷,电极植入困难,4个电极不能植入,术后听力未改善,半年后行对侧耳植入成功;⑤Mondini畸形并发外半规管未发育前庭腔扩大患儿术后半年并发脑脊液耳鼻漏、反复脑膜炎发作,术后1年行手术探查,后治愈。结论 ①人工耳蜗植入常见的内耳畸形包括,大前庭导水管综合征及其相伴发或单发的各类内耳畸形;②内耳畸形非人工耳蜗植入术的绝对禁忌证,但术中严重井喷多见,电极植入不完全多见,术后脑脊液耳鼻漏并发脑膜炎也多发生于畸形耳蜗,术前详细的影像学检查可以对各类畸形进行详细分类,并在术前对手术难度有充分的准备,可以减少相关并发症的发生。     

经耳道皮下径路人工耳蜗植入术

目的:探讨经耳道皮下径路爱益声人工耳蜗植入手术的可能性.方法:40例双耳重度或极重度聋患儿采用经耳道皮下径路行爱益声人工耳蜗植入,术中行电极阻抗测试和电诱发听性脑干反应,术后拍耳蜗位X线片,术后4周开机验证电极植入位置及工作情况.结果:所有患儿电极全部顺利植入耳蜗内,人工耳蜗装置工作状态正常,术后随访6个月无电极脱出或...     

经耳道皮下径路人工耳蜗植入术

目的:探讨经耳道皮下径路爱益声人工耳蜗植入手术的可能性.方法:40例双耳重度或极重度聋患儿采用经耳道皮下径路行爱益声人工耳蜗植入,术中行电极阻抗测试和电诱发听性脑干反应,术后拍耳蜗位X线片,术后4周开机验证电极植入位置及工作情况.结果:所有患儿电极全部顺利植入耳蜗内,人工耳蜗装置工作状态正常,术后随访6个月无电极脱出或其它严重并发症发生.结论:爱益声人工耳蜗可以安全地经耳道皮下径路植入.     

Mondini内耳畸形患者的多通道人工耳蜗植入

目的探讨Mondini内耳畸形患者人工耳蜗植入方法及手术前后听力评估和术后言语康复效果。方法回顾性分析经乳突面隐窝进路行人工耳蜗植入的19例Mondini内耳畸形患者的临床资料，并将其中10例患者植入后听力情况与10例耳蜗发育正常的人工耳蜗植入者(对照组)进行比较。结果19例Mondini内耳畸形患者中，32个电极全部植入者12例，28个电极植入者2例，26个电极植入者2例，22个电极植入者2例，20个电极植入者l例；术中7例发生多量脑脊液流出(ozze)，5例发生脑脊液井喷(gusher)，7例无脑脊液外溢。两组患者术后听力接近，均在30～40dB HL左右，无严重并发症发生，17例Mondini内耳畸形患者术后听觉言语康复效果与耳蜗发育正常者接近，2例稍差。长期效果有待进一步观察。结论人工耳蜗植入适用于Mondini内耳畸形患者，但手术前应进行全面的听力学及影像学评估。     

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。     

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.     

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     

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.     

Vestibular rehabilitation with biofeedback in patients with central imbalance

Central Nervous System disorders may cause important functional unbalance in the maintenance of balance and posture. There is no effective rehabilitation for these symptoms until now.ObjectiveThe aim of this paper is to evaluate the use of tongue electrotactile stimulation on patients with central imbalance using BrainPort.Materials and MethodsThis is a prospective case series study. We evaluated 8 patients with central imbalance, 6 men and 2 women, with mean age of 67.75 years. The patients were submitted to Computed Dynamic Posturography (CDP) and then received 18 sessions of electrotactile stimulation by BrainPort® device for 20 minutes, twice a day. Then they were submitted to a new CDP test and to a self-perception scale to assess symptom remission, partial improvement and no improvement at all.Results75% of the patients reported being more stable. There was no improvement in the balance control of the mass center in these patients.ConclusionThe patients were able to use the electrotactile stimulus to improve their balance control.