儿童流行性腮腺炎并发急性胰腺炎和感音神经性聋1例及文献复习

目的：探讨流行性腮腺炎并发急性胰腺炎和感音神经性聋的临床特征,听力学表现,早期诊断与预后。方法：报告并分析1例流行性腮腺炎并发急性胰腺炎和感音神经性聋患者的临床资料,并复习有关文献。结果：流行性腮腺炎病毒感染可致多器官损害,出现一种或多种并发症,而同时并发急性胰腺炎和感音神经性聋临床少见,多表现为单侧突发的完全性聋,预后差。结论：流行性腮腺炎可同时并发包括感音神经性聋在内的一种或多种并发症,听性脑干反应对流行性腮腺炎并发感音神经性聋的早期诊断有一定的价值。     

多频稳态诱发电位(ASSR)的临床应用研究(36例极重度感音神经性耳聋幼儿及32例成人感音神经性耳聋患者ASSR测试结果分析)

目的旨在探讨ASSR与ABR在极重度感音神经性耳聋幼儿及成人感音神经性耳聋患者测试中的临床应用价值.方法对36例(72耳)小于3岁极重度感音神经性耳聋幼儿分别行ASSR和ABR测试;对32例(64耳)成人感音神经性耳聋患者分别行ASSR和电测听测试.结果①极重度感音神经性耳聋幼儿ABR均未引出V波,而ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的引出率分别为66.67%、86.11%、88.89%、94.44%,ASSR在0.5 kHz、1 kHz、2 kHz、4 kHz的阈值均数、标准差分别为82.56±9.26 dB HL、90.31±6.94 dB HL、88.12±7.93 dB HL、88.62±8.12 dB HL.②对成人感音神经性耳聋患者0.5 kHz、1 kHz、2 kHz、4 kHz ASSR测试阈值与电测听语频听阈(dB HL)进行配对两两比较的t检验,各组P值均大于0.05,无显著性差异.结论ASSR有助于极重度感音神经性耳聋幼儿残余听力的客观评估,尤以高频听阈为佳;ASSR与电测听在感音神经性耳聋诊断上有良好的一致性.     

晚期梅尼埃病人工耳蜗植入研究并文献复习

目的回顾性分析5例晚期梅尼埃病致双耳极重度感音神经性耳聋患者行人工耳蜗植入术的效果。方法 2006～2011年收集5例晚期梅尼埃病致双耳极重度感音神经性耳聋患者行人工耳蜗植入术,分析患者的术前资料、术中及术后听力、耳鸣及眩晕情况,并复习文献对梅尼埃病行人工耳蜗植入术的适应证、可行性、术前及术后处理等情况进行讨论。结果 5例患者术中均无"井喷"现象出现,术后听力均恢复良好,语言交流能力明显提高,可进行电话交流;其中3例耳鸣消失,2例耳鸣明显减轻;2例术后数天内出现一过性眩晕,治疗后无再出现眩晕发作。结论在眩晕得到控制的前提下,晚期梅尼埃病致双耳极重度感音神经性聋的患者行人工耳蜗植入是可行的,但其确切疗效和转归仍需大宗病例及长期追踪随访。     

人工耳蜗植入术35例报告

多导电极人工耳蜗植入己经成为解决重度或极重度耳聋听力障碍问题的唯一成熟手段。我科于2011-06-2012-06期间对35例重度和极重度感音神经性耳聋患者行多导人工耳蜗植入术,效果满意,现报告如下。1资料与方法1.1临床资料35例患者,男21例,女14例;年龄14个月～17岁,平均9.6岁。语前聋33例,语后聋2例。其     

山西太原129例重度非综合征型感音神经性耳聋基因的筛查

目的应用耳聋基因芯片对重度极重度非综合征型感音神经性耳聋患者进行筛查。方法采集本地区聋哑学校和门诊散发的重度极重度非综合征型感音神经性耳聋患者129人的外周血并提取DNA,应用耳聋基因芯片检测GJB2,GJB3,SLC26A4,线粒体DNA(mitochondrial,mtDNA)12SrRNA热点突变位点。结果该耳聋人群中与筛查位点有关的耳聋比例占41.09%,共检出GJB2基因突变26例(20.16%);mtDNA突变8例(6.2%);SLC26A4基因突变21例(16.28%);未检出GJB3基因突变。结论本组耳聋人群中与筛查位点有关的耳聋比例高达41.09%,GJB2突变是该人群遗传性聋的最常见病因,SLC26A4突变为第二常见病因。     

沧州市特教学校极重度感音神经性聋患者基因分析

目的调查沧州地区极重度感音神经性耳聋患者耳聋基因突变分子病因学情况。方法对沧州市特教学校241名学生进行聋病病因学问卷调查、纯音听力测试并应用基因芯片技术行耳聋基因检测。结果 241名受试者中有39人检出基因突变,占总人数的16.2%,其中GJB2突变25人,占总人数的10.4%,GJB2235delc纯合突变12人,299del AT纯合突变1人,单杂合突变6人,均为235delc位点突变,复合杂合突变6人;GJB3突变1人,占0.41%;SLC26A4突变14人,占5.8%,3人为IVS7-2A>G纯合突变,10人为ⅣS7-2A>G单杂合突变,1人为复合杂合突变。结论沧州市特教学校极重度感音神经性聋人群存在较高的遗传性耳聋发生率,通过耳聋基因检查,可明确耳聋病因,从而达到防聋及评估耳聋预后等积极效果。     

极晚期耳硬化症的镫骨手术治疗

极晚期耳硬化症（far-advanced otosclerosis,FAO)经镫骨手术治疗后常常能有效地使用助听器，获得满意的效果。但临床上易被误诊为重度感音神经性耳聋，失去治疗机会。因此极晚期耳硬化症的正确诊断非常重要。     

极晚期耳硬化症的镫骨手术治疗

极晚期耳硬化症(far-advanced otosclerosis, FAO)经镫骨手术治疗后常常能有效地使用助听器，获得满意的效果。但临床上易被误诊为重度感音神经性耳聋，失去治疗机会。因此极晚期耳硬化症的正确诊断非常重要。     

低频感音神经性聋患者的内耳免疫学探讨

目的 测定抗内耳自身抗体,以研究低频感音神经性耳聋患者与内耳免疫的相关性。方法 通过临床详细询问病史、纯音测听、声导抗测试及ABR测试,选择低频感音神经性聋患者30例做为研究对象。以豚鼠内耳石蜡切片作为抗原,用间接免疫荧光法检测患者血清中的抗内耳抗体。结果 30例患者中有26例血清中抗内耳抗体阳性,阳性率为86.67%,P<0.01有极显著性差异;低频感音神经性聋在青少年组(≤25岁)和女性组呈高发,阳性率均为63.33％(19／30),P<0.05有显著性差异;低频感音神经性聋多为双侧耳聋,少数为单侧耳聋,双侧耳聋阳性率为86.67%(26／30),P<0.01有极显著性差异。结论 自身免疫反应参与了低频感音神经性聋的发病过程;低频感音神经聋与年龄和性别有关;低频感音神经性聋发病多为双侧聋;同时检测患者血清ⅠgM和ⅠgG内耳抗体,可协助诊断。     

MRI内耳水成像在人工耳蜗植入电极的应用价值

目的 了解耳蜗的膜迷路结构。为人工耳蜗手术檀入电极做好术前准备。方法 分析11例双耳极重度感音神经性耳聋的MRI内耳水成像圈像。使用GE Signa Infinity TwinSpeed 1．5T扫描仪，采集内耳所在区域内水信号。送AW4．0工作站进行圈像的后处理。结果 双耳极重度感音神经性耳聋10例显示耳蜗的膜迷路正常，1例双侧耳蜗只显示一圈半，且耳蜗直径缩小。诊断为耳蜗骨化。结论 MRI内耳水成像能清晰显示耳蜗的膜迷路，为人工耳蜗植入电极提供保障。     

Paget's disease and sensori-neural deafness: Temporal bone histopathology of paget's disease

Four cases with Paget's disease of the temporal bone are presented to illustrate the pathogenesis of the associated deafness. One case illustrates the combination of severe deafness due to bilateral otosclerosis with probably asymptomatic bilateral Paget's disease. One case with advanced Paget's disease presents features to explain early stages of sensori-neural deafness before actual cellular invasion of the inner ear. One case of profound deafness due to Paget's disease presents a different stage of cellular invasion of the inner ear by the disease on each side. One case illustrates invasion of the internal auditory meatus by Paget's disease with infiltration of the acoustic division of the nerve and profound deafness.     

双耳先后发病突发性聋患者的临床特点分析

目的分析双耳先后发病突发性聋(突聋)患者的临床特点,提出可能的针对性预防措施。方法回顾性分析2015年12月至2018年3月收治的19例双耳先后发病(间隔时间>3 d)突聋患者的临床资料,包括性别、年龄、既往史、患耳侧别、听阈曲线类型、听力损失程度、发病时间、双耳发病的间隔时间及治疗效果,总结其临床特点,并与同期收治的单侧突聋患者比较。结果 19例双耳先后发病的突聋患者中,先发耳听阈曲线为全聋型10例(52.6%)、平坦型8例(42.1%)、高频下降型1例(5.3%),听力下降以重度和极重度聋为主;后发耳听阈曲线为平坦型15例(78.9%)、全聋型3例(15.8%)、低频下降型1例(5.3%),听力下降以中度和重度聋为主。双耳先后发病组平均年龄高于单侧发病组,治疗后有效率低于单侧发病组(P<0.05)。有高血压、糖尿病等慢性病史组出现对侧突聋的比例(56.5%,13/23)高于无慢性病史组(2.8%,6/214)(P<0.05)。结论先发耳听阈曲线为平坦型或全聋型者、听力损失程度为重度或极重度者,且有高脂血症、高血压、糖尿病等影响血流动力学的慢性疾病史的高龄单侧突聋患者,对侧耳再发突聋的可能性较大;降脂、降压、控制血糖等治疗可能有利于预防对侧耳再发突聋。     

Atypical keratosis obturans

Keratosis obturans is a rare condition characterized by the accumulation of desquamated keratin material in the bony portion of the external auditory canal. Classically, it is reported to present with severe otalgia, conductive deafness and global widening of the canal. A case of keratosis obturans is described in which the principal symptom was a metallic taste and the main finding was extensive erosion of the hypotympanum with exposure of the facial nerve and the annulus of the tympanic membrane. This presenting symptom and resorption pattern are atypical of keratosis obturans and have not been documented previously.     

儿童颞骨骨折临床特点和治疗策略

目的 探讨儿童颞骨骨折的临床特点及治疗策略.方法 回顾性分析2014年7月～2021年7月首都医科大学附属北京儿童医院确诊的477例18岁以下颞骨骨折患儿的临床资料,包括患儿性别、年龄、受伤原因、影像学、面神经及听力学评估、并发症、治疗及预后.结果 477例颞骨骨折患儿中男358例、女119例,男女比例为3∶1,年龄范...     

儿童单侧感音性听力损失的听力学及影像学特征149例分析

目的 探讨儿童单侧感音性听力损失的听力学和影像学表现,分析其病因.方法 回顾性分析149例1月龄～14岁的单侧感音性听力损失患者的就诊途径、听力学和颞骨影像学检查结果.结果 149例患者95.97％为重度、极重度听力损失,即单侧聋.38.26％就诊患儿是由于听力筛查未通过被发现,20.13％患儿自觉单侧听力差就诊发现....     

伴眩晕的突发性聋患者椭圆囊-球囊功能分析

目的通过观察突聋伴眩晕患者主观垂直视觉(subjective visual vertical,SVV)偏斜和前庭诱发肌源性电位(vestibular evoked myogenic potentials,VEMP),了解椭圆囊-球囊功能与症状的关系。方法16例突聋伴眩晕的患者和10例突聋无眩晕的患者分别行SVV及VEMP检查。其中,6例突聋伴眩晕患者行前庭双温试验检查。结果伴晕眩的突聋患者中,VEMP异常者8例,正常8例;SVV异常6例,正常10例,且所有SVV异常者,VEMP均异常。不同程度听力损失之间VEMP正常与异常率无显著性差异(P>0.05),纯音听阈与SVV的关系在统计学上也无相关性(P>0.05)。结论突聋伴眩晕患者可伴有球囊和/或椭圆囊功能障碍,前庭上神经和前庭下神经损伤无特定的规律。全面的前庭功能评价有助于进一步了解伴眩晕的突聋患者的前庭功能状态。     

Labyrinthine window rupture as a cause of acute sensorineural hearing loss

Labyrinthine window rupture (LWR) is one cause of acute sensorineural hearing loss and need for early exploration is clear for good improved hearing. Acute sensorineural hearing loss of 60 dB or more treated from May 2006 to May 2010 were retrospectively analyzed. There were 21 ears of severe deafness, 18 ears of profound deafness, and 10 ears of total deafness. All patients were examined with temporal bone CT. Space-occupying lesions around the labyrinthine windows were suggestive images of LWR. Thirty-five ears were operated for LWR while 14 ears of SHL received conservative treatments. Fifty-seven percent of LWR improved 30 dB or more after sealing of both labyrinthine windows. Of the 15 markedly recovered ears, 14 ears were operated within 2 weeks from the onset. Of the five cured ears, four ears were operated within a week from the onset. As for the hearing prognosis of SHL, 88% of severe and profound deafness improved 30 dB or more but total deafness did not improve more than 30 dB. Exclusion of LWR from SHL and early surgical intervention in LWR will bring about good hearing prognosis to both LWR and SHL.     

The progression of hearing loss in the early stages of sudden deafness

Summary The pathogenesis of sudden deafness is still not known. Therefore, to clarify its pathophysiology, it is important to know whether the progression of hearing loss occurs in the early stage of sudden deafness. The subjects were 11 patients with sudden deafness showing the progression of hearing loss by pure tone audiometry after the onset of the initial attack of hearing loss. The progression of hearing loss was mostly observed within 4–7 days after the onset of the initial attack. Average hearing loss in initial audiometry was severe and the recovery was poor in the majority of cases. An exploratory tympanotomy was performed in five cases and revealed one case of perilymphatic leak from the round window and another case of suspected round-window membrane rupture. As far as the causes of the progression of hearing loss in the early stage of sudden deafness are concerned, the following could be considered (1) aggravation of viral labyrinthitis, (2) aggravation of the vascular lesion of the inner ear, and (3) rupture of the membranous labyrinth of window(s).This paper was presented at the XVIth International Congress of Audiology, May 23–27, 1982, held in Helsinki, Finland     

Epidermoid carcinoma of the external auditory canal

The squamous cell carcinoma of the external auditory canal is extremely rare. The facial paralysis is usual, but pain seems to be the most common symptom. The mean age at diagnosis is 50 to 60 years, with chronic external otitis preceding. The CT-scan is the most important investigation before treatment: surgery and external radiotherapy. Surgical procedure must be large, with or without facial nerve dissection, following by irradiation of the temporal bone and cervical nodes. The generally poor prognosis is explained by the frequency of advanced disease at the time of diagnosis. This paper reports our 4 experiences, with a bilateral carcinoma case.     

Congenital cytomegalovirus infection and sensorineural hearing loss

In a prospective study still in progress, infants with congenital cytomegalovirus (CMV) infection were followed with audiological, ophthalmological, neurological, and psychological tests; 10,328 infants were investigated within a 5-year period (1977-1982) by virus isolation in urine within the first week of life. Fifty (0.5%) had a congenital CMV infection. In this group four children turned out to have total deafness and a fifth possibly a mild hearing disorder. In one case the deafness was associated with severe mental retardation and spastic tetraplegia. The mother of the child had a primary CMV infection in the first trimester. In one of the other cases of severe deafness it could be proven that the mother had had a secondary CMV infection and in further two cases, presumed secondary infections. Prospective serological tests of the mothers would not have revealed more than one of the high risk pregnancies. The value of vaccination against congenital CMV infection is questioned. Screening of newborn infants for congenital CMV infection is recommended in order to reveal infants at high risk for deafness and make an early habilitation possible.