耳聋基因芯片技术在耳聋病因诊断中的应用

目的使用耳聋基因芯片技术对耳科门诊耳聋患者进行病因诊断。方法收集26例明确为感音神经性听力下降的聋病患者,使用基因芯片检测试剂盒进行检测。结果 26例患者中先天性耳聋者10例,检出率为40.00%;成年感音神经性耳聋者13例,检出率为23.08%;突发性耳聋者3例,检出率为66.67%。结论遗传性耳聋基因检测试剂盒对于明确聋病患者致聋原因有一定的帮助,具有临床推广价值。     

有眩晕及无眩晕突聋的纯音听力表现

本文分析病程２周内的特发性突聋１０９例的有眩晕及无眩晕两组病人的听力损失类型及预后。发现；女性合并眩晕多于男性，有眩晕病人平均年龄高于无眩晕病人。１０９例中深度耳聋型占４１例，平坦听力损失型３１例，高频听力损失型２５例，低频听力损失型９例，中应听力损失碟型３例。１０９例中突聋有眩晕者占４５％。     

Pseudohypacusis: the most frequent etiology of sudden hearing loss in children

Sudden hearing loss is a rare pathology in children. Several factors may be responsible for it although the exact etiology remains frequently undiagnosed. Among them, pseudohypacusis has been reported. However, the extent to which this pathology contributes to sudden hearing loss in children is unknown. This study evaluates the incidence of pseudohypacusis in children presented with sudden hearing loss. The medical records of 48 children presented to our department because of sudden hearing loss from 2002 to 2007 were reviewed. Diagnostic process included both subjective and objective audiological tests while organic hearing losses were further subjected to proper evaluation and treatment. 26 cases (54%) of pseudohypacusis and 22 cases (46%) of organic sudden hearing loss were diagnosed. In the pseudohypacustic group, girls outnumbered boys (16:10) and their mean age was 10.5 years. Pseudohypacusis represents the most frequent etiology of sudden hearing loss in children. Its detection is relatively simple using conventional audiological tests though in some cases even experienced clinicians may come to incorrect diagnosis.     

血白细胞及C-反应蛋白对评价突发性聋发病及预后的作用

目的探讨血白细胞(WBC)及C-反应蛋白(C-reaction protein,CRP)与突发性聋发病及预后的关系。方法选取突发性聋患者162例,按照入院血常规检查结果及是否应用抗生素治疗,分为WBC正常组78例(WBC≤10×109/L),给予常规治疗;高WBC常规治疗组42例(WBC>10×109/L),给予常规治疗;高WBC联合治疗组42例(WBC>10×109/L),给予常规治疗同时联合抗生素治疗。分别检测三组患者治疗前后纯音听阈,比较治疗前后WBC及CRP水平的变化。结果高WBC常规治疗组有效率85.7%,高WBC联合治疗组有效率83.3%,均高于白细胞正常组的64.1%,差异有统计学意义(P<0.05);高WBC常规治疗组与高WBC联合治疗组有效率差异无统计学意义(P>0.05)。高WBC常规治疗组和高WBC联合治疗组治疗后WBC和CRP含量较治疗前均显著降低,差异均有统计学意义(P<0.05);但两组间治疗前后WBC和CRP水平相比,差异均无统计学意义(P>0.05)。低频下降型突聋患者中WBC升高者比例较其他听阈曲线类型者高,且治疗总有效率高于高频下降型和全频下降型患者。结论突聋的发病可能与炎症相关,但不是细菌感染引起的炎症反应,可能是机体的应激系统或免疫系统发挥作用,白细胞升高患者的治疗效果好于白细胞正常者,对白细胞升高的突聋患者采用抗生素治疗无临床意义。     

突发性聋临床流行病学分析

目的进行突发性聋（以下简称突聋）的临床流行病学分析。方法回顾性分析我科2000年-2006年收治突聋患者332例的临床资料：对1980年至2006年我院耳鼻喉科突聋住院人数和耳鼻喉科总住院人数进行了统计。结果332例突聋中,男164例,占49.3％;女168例,占50.6％。发病年龄以41-50岁最多（89例）。右耳患病144例,左耳患病157例,双耳间隔50天至5年相继发生突聋者31例。听力下降前后出现耳鸣者295例,占88.9％,以低调、持续性耳鸣为主。有耳闷者111例,占33.4％。按职业分类：白领127例,蓝领101例,退休或无业居家者80例,学生22例,儿童2例。发病诱因：无明显诱因者78.3％,有感冒病史者10.5％,有劳累、压力大、情绪波动因素者9.3％,有其他因素者1.8％。根据突聋是否伴有头晕或眩晕将其分为三类：单纯表现为听力减退者179例（占53.9％）,伴发头晕者59例（占17.8％）,伴有眩晕者94例（占28.3％）。332例中伴发高血压病、冠心病和／或糖尿病者62人（占18.7％）;其中160例检查了血脂,查出血脂异常者92人（占57.5％）,以甘油三酯升高为主。分析我院1980年至2006年突聋患者住院人数占耳鼻喉科总住院人数的构成比发现：80年代构成比为1.69％,90年代为3.31％,2000年后为4.6％,三者有极显著性差异（P〈0.01）。结论332例突聋中男女发病构成和左右耳患病构成基本相同。以中、青年为高发人群。男性患病与职业有明显相关性,即白领比蓝领和居家者更易患突聋;女性患病与职业无明显相关性。精神压力大、过于劳累的人员易患突聋。突聋中单纯表现为听力减退的病人数多于伴有眩晕者,伴有眩晕者又多于伴有头昏者。其最常见的伴发病症为血脂代谢紊乱,以甘油三酯升高为主。近26年来我院突聋住院人数逐渐增多,提示突聋发病有增多趋势。     

镫骨肌反射阈存在的突聋患者预后及影响因素

目的 分析镫骨肌反射阈存在的突聋患者的预后情况,探讨其影响因素。方法 回顾性分析2018年1月至2021年12月收治的282例镫骨肌反射阈存在的突聋患者的临床资料,采用Excel进行数据整理,SPSS 26.0对收集的资料进行描述性分析及统计分析,对性别、年龄、就诊时间、是否伴有耳鸣、眩晕、基础疾病(糖尿病、高血压、冠心病)、听力曲线类型、听力损失程度、镫骨肌反射阈、镫骨肌反射阈与纯音听阈差值(A-T)等因素进行统计学分析。结果 282例镫骨肌反射阈存在的突发性聋患者中,治愈101例,显效8例,有效54例,无效119例,总有效率57.8%。耳聋侧别(P=0.907)、伴随症状(P_眩晕=0.686,P_耳鸣=0.534)、基础疾病(P_高血压=0.338,P_糖尿病=0.262,P_心脏病=0.780)、镫骨肌反射阈(P_{0.5 kHz}=0.152,P_{1.0 kHz}=0.701,P_{2.0 kHz}=0.810)、镫骨肌反射阈与...     

老年突发性耳聋近期疗效临床分析

目的：分析老年突发性耳聋患者药物治疗的近期疗效。方法：将突发性耳聋患者依据年龄分为老年组（年龄≥60岁,70例）和对照组（年龄〈60岁,70例）,7d一疗程,复查纯音测听结果,比较治疗前后听力改善情况。结果：老年组患者治疗前后患耳言语频率平均听阈提高,差异有统计学意义（P〈0．05）;老年组突聋患者发病时间（≤7d）治疗有效率为61．76％,老年组（发病时间〉7d）治疗有效率为38．89％,差异有统计学意义（P〈0．05）,老年性突聋患者总有效率为49．9％;老年突发性耳聋患者在中度耳聋以下（听阈〈55dB）有效率52％,对照组有效率84．62％,差异有统计学意义（P〈0．05）,而在中重度耳聋患者治疗率差异无统计学意义。结论：病程对于老年突发性耳聋患者预后有重要的影响,尽早的改善末梢血管微循环障碍,可以明显的改善预后;老年突聋患者的预后比年轻患者差。     

突发性聋预后影响因素

目的对影响突发性聋的预后因素进行分析讨论。方法回顾分析249例突发性聋患者临床资料,包括年龄、初诊时间,初诊时听力损失程度,听力曲线类型,是否伴有眩晕和耳鸣,进行畸变产物耳声发射(distortion product otoacoustic emissions,DPOAE)检查结果。结果初诊时间为发病后1～23天,患侧耳初诊时250 Hz～4000 Hz平均听力损失40dB以下31例(12.45%),41 dB～70 dB 80例(32.13%);71 dB～90 dB 74例(29.72%),91 dB以上64例(25.70%)。听力曲线上升型72例,下降型81例,平坦型96例。伴有眩晕96例,伴耳鸣174例。治疗药物包括血管扩张剂、皮质类固醇激素、神经营养剂、抗病毒及能量合剂,疗程2～4周。81例进行畸变耳声发射检查,45例在不同频率被引出,经治疗最终被引出DPOAE的频率听力恢复达痊愈水平。结论高龄患者和年龄小的患者预后不良;初诊时间越早听力恢复越好;听力曲线上升型预后好;伴有眩晕者预后不好;能引出DPOAE者听力恢复好。     

Differentiating the cause of acute sensorineural hearing loss between Ménière's disease and sudden deafness

CONCLUSIONS: Most patients with Ménière's disease (MD) reveal abnormal vestibular-evoked myogenic potentials (VEMPs) and the recruitment phenomenon, whereas most sudden deafness patients display normal VEMPs without the recruitment phenomenon. We therefore recommend using both the recruitment phenomenon and VEMP testing as a diagnostic algorithm to differentiate between MD and sudden deafness as the cause of acute hearing loss. OBJECTIVE: To recommend a diagnostic algorithm to differentiate between MD and sudden deafness as the cause of acute hearing loss. MATERIAL AND METHODS: Between January 2002 and December 2003, 14 consecutive patients with "probable" MD who also had acute sensorineural hearing loss were enrolled in the study, together with another 14 age- and sex-matched patients with idiopathic sudden deafness. Each patient underwent a battery of audiovestibular function tests, including pure-tone audiometry, a stapedial reflex test, distortion-product otoacoustic emissions (DPOAEs), electronystagmography and a VEMP test. All patients were followed for at least 12 months after presentation. RESULTS: Significant differences were found between MD and sudden deafness in terms of the recruitment phenomenon (86% and 21% of cases, respectively) and abnormal VEMP responses (71% and 21% of cases, respectively). However, the diseases did not differ significantly in terms of abnormal DPOAEs or caloric test results.     

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     

梅毒性感音神经性聋的临床分析

目的:探讨梅毒性感音神经性聋的临床表现、诊断和治疗.方法:回顾性分析3例梅毒性感音神经性聋患者的临床症状、体征、血清学及治疗.结果:3例梅毒性感音神经性聋患者中,2例RPR和TPPA均阳性;1例RPR阴性,TPPA阳性;2例在外院已确诊为梅毒并行驱梅治疗;2例为突发性聋起病,伴有耳鸣,1例为耳鸣起病伴高频听力下降.均已排除了其他原因所致的:千聋耳鸣,治疗后听力无改善.结论:梅毒性感音神经性聋可以突发性聋或耳鸣发病,易漏诊.对病因不明的听方下降,伴有耳鸣、眼球震颤的患者,应行必要的实验室检查以明确诊断.     

Prevalence of obstructive sleep apnea syndrome (OSA) in patients with sudden hearing loss. A pilot study

BACKGROUND: It is estimated that in patients with sleep-related breathing disorders the probability of a cerebral vascular infarction (CVI) is 3.1 times that in patients without sleep apnea and that 25-50% of all patients who have a stroke suffer from sleep apnea (OSA) and have a respiratory disturbance index (RDI) higher than 10. CVI may be caused by variations in intracranial pressure or in intracranial hemodynamics owing to decreasing pO(2) and increasing pCO(2) during cessation of airflow. It is suspected that the most common causes of sudden deafness are vasospasm, thrombosis, embolism, hypercoagulation and sludging. The present study analyzed the prevalence of sleep apnea in patients with sudden hearing loss. PATIENTS AND METHODS: A 7-channel polygraph was used to test 33 subjects with normal hearing and 27 patients suffering from sudden hearing loss. Statistical analyses were performed with a Chi-square test and the Mann-Whitney test. RESULTS: We found that 29.6% of the patient group and 21.2% of those in the study control group were suffering from OSA and had RDI >10; this difference was not significant ( p=0.554). Sudden hearing loss may also be an indicator of arteriosclerosis secondary to such risk factors as hypertension ( p=0.005), diabetes ( p=0.003), and hyperlipidemia ( p=0.004), which were highly significant for the patient group. CONCLUSION: Patients who develop sudden hearing loss tend to have OSA more frequently than those in the control group owing to the similar risk factors for cerebral infarction and sudden hearing loss.     

Etiological diagnosis of bilateral, sensorineural hearing impairment in a pediatric Greek population

Early diagnosis, evaluation and treatment of childhood deafness are essential for a child's normal growth. Etiological diagnosis of hearing loss makes prevention, family scheduling and more effective therapy feasible goals. Etiological assessment of sensorineural deafness still remains difficult although recently with the progress of genetics it has become more efficient. In this retrospective study, the etiology of bilateral, sensorineural hearing loss with indication for hearing aids has been studied in 153 hearing impaired children. Etiological diagnosis was based on family and patient record, physical, audiological and laboratory examinations. Among the 94 children who completed the diagnostic protocol etiological groups revealed the following distribution: non-hereditary acquired hearing impairment was present in 36 children (38%) and hereditary was present in 44 (47%) children. The etiology remained unknown in 14 (15%) children. Non-syndromic autosomal dominant type accounted for 13 (29% of hereditary hearing loss) children, non-syndromic autosomal recessive type for 21 (48%) children and syndromic deafness for 10 (23%) children. Modern diagnostic methods, such as genetic testing, help diminish the number of cases with hearing impairment of unknown etiology, for the benefit of children who receive early and appropriate medical, audiologic, genetic and educational counseling based on the etiology of their hearing loss.     

Validity of the Western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease

OBJECTIVE: To assess the validity of the Western blot immunoassay for heat shock protein-70 (hsp-70) for diagnosis of autoimmune inner ear disease. STUDY DESIGN: Retrospective study of 53 patients affected by sudden deafness (n = 19), idiopathic progressive sensorineural hearing loss (n = 24), and Meniere's disease (n = 10) who were treated from 1995 to 1999. The clinical course and response to corticosteroid were evaluated. METHODS: A purified hsp-70 antigen from bovine kidney cell line was used for the Western blot immunoassay. RESULTS: Only five patients (9.4%) showed anti--hsp-70 antibodies: Two presented a sudden sensorineural hearing loss (sudden deafness group), two showed an idiopathic progressive sensorineural hearing loss (idiopathic progressive sensorineural hearing loss group), and one was affected by fluctuating hearing loss (Meniere's disease group). A systemic autoimmune condition was observed in 29.1% of patients with idiopathic progressive sensorineural hearing loss. CONCLUSIONS: The low sensitivity of Western blot immunoassay for patients affected by idiopathic progressive sensorineural hearing loss and Meniere's disease may result from either the long time elapsed from the hearing loss and vertigo to the initial examination or from the increased percentage of cases of systemic autoimmune disease present in patients with idiopathic progressive sensorineural hearing loss. More studies to detect the immune-mediated inner ear disease in Western blot immunoassay-negative patients are required.     

Audiological evaluation of twenty patients receiving pentoxifylline and prednisone after sudden deafness: prospective study

Idiopathic sudden deafness is defined as sudden sensorineural hearing loss of undetermined etiology. As a consequence, various treatments have been developed for this disorder. Our study evaluated the effectiveness of pentoxifylline and prednisone in such treatment. We analyzed this treatment's results in our patients through conventional audiograms, and speech audiometry was performed in the acute stage and during the treatment. We diagnosed idiopathic sudden hearing loss in 20 patients (8 female and 12 male). The left ear was involved in 9 patients and the right in 11. All patients had been examined by us within 15 days from the onset of hearing loss. We compared the hearing threshold results in the different periods in this prospective study.     

病程超过3周的突发性聋患者的临床疗效分析

目的 了解对病程超过3周的突发性聋患者以糖皮质激素等药物进行治疗的意义.方法 对北京大学人民医院耳鼻咽喉科2002年11月至2010年7月住院治疗的48例(58耳)病程超过3周的突发性聋患者的临床资料进行同顾性分析.根据本次入院治疗前的听力曲线分为3组,分别采用不同的治疗方案,观察其疗效.以SPSS 17.0统计软件对数据进行分析,患者治疗前后纯音测听阈值采用t检验.结果 低频下降型(6耳)、高频下降型(22耳)、全频下降型(30耳)治疗后有效率分别为83.3%、31.8%、36.7%.病程≥1年的共12耳,其治疗有效率58.3%(7耳).全频下降型中本次治疗前气导听阈均值≥90 dB(听力级)的9耳,7耳(77.8%)治疗有效,其中3耳(33.3%)达到显效标准;本次治疗前气导听阈均值＜90 dB的患者21耳,其中3耳(14.3%)治疗有效.结论 病程超过3周的突发性聋患者仍有积极治疗的意义.其中低频下降型治疗有效率最高,高频下降型有效率最低,全频下降型有效率居中.病程≥1年的突发性聋患者经积极治疗仍有改善听力可能.全频下降型、听功能损失程度较重的患者,通过积极治疗获益较大.

Abstract：

Objective To investigate and analyze the significance of a course of glucocortieosteroids and other drugs for the treatment of patients with sudden deafness present for at least three weeks.Methods A retrospective review was done on 48 patients (58 ears) with sudden deafness present for at least three weeks or more,who were admitted to the Department of Otorhinolaryngology,Peking University People's Hospital from November 2002 to July 2010.The patients were devided into three groups by the type of hearing threshold.The different treatments were used in the three groups.The SPSS 17.0 software was used to analyze the data.Results In patients with a low tone hearing loss (6 ears) ,83.3% improved.For patients with a high tone loss (22 ears) 31.8% improved.For a flat tone hearing loss (30 ears) 36.7% improved.For patients with a hearing loss more than one year (12 ears) there was improvement in 58.3% (7 ears)of the patients.In 9 ears which had a flat tone hearing loss of 90 dB or greater before treatment,77.8% (7 ears) improved with 33.3% (3 ears) having a significant improvement.In 21 ears which had a hearing threshold was under 90 dB,14.3% (3 ears) improved.Conclusions In the treatment of patients with sudden deafness which was longer than 21 days the treatment was significant,especially for those who had a 90 dB or greater flat-tone type hearing threshold before treatment.Even though the hearing loss was more than a year in some patients there was still a benefit from treatment.     

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。     

Post-irradiation sudden deafness

As in radiation-induced cancer, post-irradiation sudden deafness (PISD) is defined as sudden onset deafness in patients post-irradiation. Fifteen nasopharyngeal carcinoma (NPC) patients with PISD were enrolled in this study. The mean interval from the completion of irradiation to the occurrence of PISD was 12 years. Seven had total deafness, seven had profound hearing loss, and one had moderate hearing loss. Nine out of 15 (60 per cent) patients experienced hearing recovery within three months after treatment. In conclusion, PISD is a late complication in NPC patients post-irradiation. The causative mechanism is theorized as vascular insufficiency.     

影响突发性聋疗效的相关因素分析

目的探讨影响突发性聋治疗效果的相关性因素。方法对90例突发性聋患者的临床资料应用Logistic多因素逐步回归分析方法作回顾性分析。结果突聋的疗效与年龄、性别、单、双耳发病、是否伴有眩晕和耳鸣无关,而与听力损失的程度、听力曲线的类型、发病到初治的时间有显著的相关性。但听力损失的程度在单因素分析时,对突聋疗效的影响无统计学意义。结论突聋患者听力损失越轻,且听力曲线为上升型,发病后治疗越早者,其疗效越好;反之,疗效越差。     

1158例突发性聋患者听力情况分析

目的分析大样本突聋患者听力损失情况。方法对1158例患者（1203耳）进行病史询问及听力学检查,按听力损失频率及损失频率平均听阈分类,部分患者行颞骨CT及头颅MRI检查。结果1158例患者中14．3％为低、中频听力下降,6．2％为中、高频听力下降,15．1％为高频听力下降,4．4％为低、高频听力下降,60．0％为全频听力下降。1158例中查出1例听神经瘤,2例大前庭水管综合征。结论突聋患者听力损失情况不一,以全频下降为主,应注意排除相关疾病。