鼓室注射地塞米松治疗突发性感音神经性聋

经外耳道-中耳鼓室途径直接用药到内耳不是新的概念。1990年开展的经鼓室注射庆大霉素治疗梅尼埃病,使鼓室用药治疗内耳疾病技术在近年快速发展。鼓室用药有优于胃肠外或静脉给药的优点：靶向性好、用药量少而局部药物浓度高。除鼓室注射利多卡因治疗耳鸣外,目前鼓室使用的药物主要为庆大霉素和类固醇激素,将来抗氧化剂、耳保护药物（全身用耳毒性药物,如抗生素、抗肿瘤药物联合鼓室用药）、神经生长因子、基因治疗载体（治疗代谢或遗传疾病或后天缺陷）等也有望广泛用于内耳疾病治疗,因此,这种方法具有重要的临床意义。     

地塞米松鼓室内注射治疗难治性突发性感音神经性聋

目的：观察鼓室内地塞米松注射（IDI）治疗难治性突发性感音神经性聋（突聋）的疗效。方法：25例常规方法治疗无效的突聋患者采用IDI,首次剂量为2．5mg,此后,每2天重复注药1次（每次2．5mg）,共4次,总剂量10mg。用药期间密切观察记录听力改变、耳鸣和眩晕等情况。结果：2例失访,23例治疗完成后1个月纯音测听检查：显效3例,0．25～4．00kHz听阈平均提高48．5dB;有效5例,平均提高18．5dB;无效15例;有效率为34．8％（8／23）。结论：IDI治疗突聋,能保证药物高浓度作用于内耳,避免全身用药可能产生的不良反应;治疗操作不复杂,创伤性小,为常规方法治疗无效的突聋患者提供了再次治疗的机会。     

儿童单侧感音神经性聋的病因学分析

通过对４１例（５０耳）单侧感音神经性聋患儿进行纯音听力、声导抗、血糖、血脂、甲状腺功能及ＣＴ检查，对儿童单侧感音神经性聋的病因进行了分析，结果表明：所有患儿血糖、血脂均正常，甲状腺功能也无明显变化；病毒感染、内耳血管畸形可能是致聋的主要原因；对这些患儿应进行定期追踪观察，以排除肿瘤的可能；对小儿原因不明的反应力及学习成绩下降应进行常规听力测定以求早期诊断，提高治疗有效率。     

儿童突发性感音神经性聋的研究进展北大核心CSCD

突发性感音神经性聋(sudden sensorineural hearing loss,SSNHL)(下简称突聋)为耳鼻咽喉头颈外科的常见急症,是指72小时内突然发生的、原因不明的感音神经性听力损失,且至少在相邻的两个频率听力下降≥20 dB[1]。儿童突聋是指年龄在18岁以内的儿童突然发生的、原因不明的感音神经性听力损失[2],该病有自愈的倾向,斯坦福大学的学者Mattox在对88例(年龄最小10岁,最大79岁,平均年龄为46岁)突聋患者进行的前瞻性研究中报道该病的自愈率为65%,大多数自愈发生在2周内[3]。由于本病病因复杂,目前仍未对儿童突聋的最佳治疗方案达成共识,本文主要结合近年来国内外儿童突聋的研究文献,综述儿童突聋的流行病学、病因及其发病机制、治疗、预后影响因素,以期对该病的个体化治疗提供参考。     

圆窗置管微泵灌注地塞米松治疗难治性突发性感音神经性聋

目的：观察圆窗内置管微泵灌注地塞米松治疗难治性突发性感音神经性聋（突聋）的疗效。方法：13例常规方法治疗4．8周无效的突聋患者,作外耳道皮瓣,圆窗置管经微泵灌注地塞米松,每次2．5mg,维持1h,每天2次,连续给药1周。用药期间观察记录听力改变、耳鸣和眩晕等情况。结果：13例患者治疗完成后1个月纯音测听检查：显效2例,0．25～4．00kHz听阈平均提高30．5dB;有效3例,平均提高16．7dB,无效8例,有效率38．5％（5／13）。结论：圆窗置管微泵灌注地塞米松治疗突聋的方法,能保证药物持续泵入和高浓度作用于内耳,优于鼓膜穿刺注药;操作稍复杂,但创伤小,耗费不高,为常规方法治疗无效的突聋患者提供了再次治疗获效的机会。     

重度感音神经性聋儿童的多维嗓音分析

目的 分析不同性别正常儿童及重度感音神经性聋儿童嗓音的声学特点和差异.方法 以130例6～12岁无嗓音相关疾病的正常儿童(男65例,女65例)为正常组;以71例6～12岁先天性重度至极重度感音神经性聋儿童(男34例,女37例)为耳聋组;采用美国Kay公司的计算机多维嗓音分析软件MDVP,比较各组儿童发长元音/a:/的基频(F0)、基频微扰(jitter)、振幅微扰(shimmer)、基频变量(vF0)、峰值振幅变量(yAm)、振幅扰动商(APQ)、噪/谐比(NHR).结果 正常组儿童不同性别间各指标值差异均无统计学意义(均为P＞0.05);耳聋组vF0、vAm分别为2.61％±1.15％和23.57％±8.29％,显著高于正常组(1.77％±1.01％和16.31％±7.75％)(P＜0.01).两组F0、jitter、shimmer、APQ、NHR差异均无统计学意义(均为P＞0.05).结论 6～12岁正常儿童的嗓音客观声学分析无性别差异,重度及以上听力损失患儿在持续平稳发声时不能对代表长时频率变化的基频变量(vF0)和长时振幅变化的峰值振幅变量(vAm)有良好的控制能力,vF0和vAm可作为评估人工耳蜗植入术后其嗓音和言语能力有无提高的参考指标.     

感音神经性聋高频听力易损机制

感音神经性聋是临床常见的疾病,包括噪声性聋、老年性聋、药物性聋、突发性聋等。感音神经性聋多以高频听力损失为主要表现,或由高频听力下降开始（Cole,1988;Mur-phy,1991）。以往人们对高频听力损失的认识比较局限,为何感音神经性聋高频听力比低频更容易受损,并没有明确的结论,本文就感音神经性聋高频听力损失的原因和机理做一综述,以期为采取合适的预防和治疗方案提供依据。     

突发性聋发病因素调查

目的 调查突发性聋(简称突聋)的发病相关因素.方法 对在解放军第306医院耳鼻咽喉头颈外科住院治疗的378例突聋患者采用问卷调查加病历分析形式进行发病相关因素的调查,并统计分析其结果.结果 发病时平均年龄42.0±10.2岁,男女发病为59%和41%;35岁以下年轻人发病所占比例为31%;发病前有过度疲劳史占51.6%;心里压力因素占27.8%;精神刺激因素占18.3%;有上呼吸道感染史占9.5%;有前次发病史者占4.8%;有家族史者占2.4%;脑力劳动和体力劳动者所占比例分别为59.5%和40.5%;无明显季节性因素;患有全身性疾病者占34.1%.结论 突发性聋患者大多有发病的相关因素,过度疲劳、心里压力、精神刺激、全身疾病等是其发病的明显诱发因素,年轻人发病有上升趋势,男性及脑力劳动者发病率较高.     

既往存在感音神经性听力损失突发性聋患者临床特征与疗效分析

目的探讨既往存在感音神经性听力损失突发性聋患者临床特征与疗效。方法对61例(63耳)既往存在感音神经性听力损失突发性聋患者的临床资料进行系统性分析,包括临床表现、疗效评估等,与同期收入院的既往不存在感音神经性听力损失突发性聋患者资料对比,分析发病和影响疗效的高危因素,总结该病发生、发展及预后的特点。结果既往存在和不存在感音神经性听力损失突聋患者的性别、现患耳侧别、病程差异无统计学意义(P值均>0.05),发病年龄差异具有统计学意义(P<0.05)。既往存在和不存在感音神经性听力损失突聋患者的听力曲线类型、听力损失程度差异无统计学意义(P值均>0.05),既往存在感音神经性听力损失突聋患者总有效率低于既往不存在感音神经性听力损失突聋患者总有效率,差异有显著统计学意义(χ²=6.915,P=0.009),既往存在感音神经性听力损失突聋患者痊愈率低于既往不存在感音神经性听力损失突聋患者痊愈率,差异无统计学意义(χ²=0.221,P=0.638)。结论既往存在感音神经性听力损失突聋患者病因复杂,疗效较差,应加大研究制定个性化的诊疗方案。     

儿童突发性感音神经性听力损失的诊治体会

对18例20耳8～14岁突发性感音神经性听力损失患儿的临床资料进行了总结和分析,结果表明儿童突发性感音神经性听力损失发病与首诊之间病程较长,部分患儿有上感、流行性腮腺炎病史,可能和听力损失发生有关.儿童突发性感音神经性听力损失治疗效果类似于成人患者,而全聋者疗效差.东菱克栓酶同样适于治疗儿童突发性感音神经性听力损失且疗效和安全性较好,无明显的出血倾向和其它并发症.     

Sudden sensorineural hearing loss in children and adolescents: Clinical characteristics and age-related prognosis

Objective

Although many studies have investigated sudden sensorineural hearing loss (SSNHL) in adults, there were few studies on SSNHL in the pediatric population; especially research on treatment and prognosis of pediatric SSNHL was limited. The aim of this study was to evaluate clinical characteristics, treatment outcomes and prognostic factors in children and adolescents with SSNHL.

Pediatric sudden sensorineural hearing loss after diving

OBJECTIVE: Sensorineural hearing loss after diving from a low height has been rarely reported especially in children. METHODS: We present and discuss a new case of pediatric sudden sensorineural hearing loss after diving. RESULTS AND CONCLUSION: Medical and surgical approaches (indications and timing of explorative tympanotomy) to this occurrence are still controversial.     

以突聋为首发症状的颅内感染性疾病的临床特征分析

目的探讨以突发性耳聋为首发症状的颅内感染性疾病的临床特点、听力学及影像学表现,以减少误诊。方法对5例以突发性耳聋为首发症状的颅内感染性疾病患者的临床资料、诊断过程以及疾病转归进行分析。结果 5例患者中男4例,女1例,临床表现:听力下降5例,眩晕伴走路不稳3例,头痛伴恶心呕吐4例,发热3例。纯音测听5例均为感音神经性耳聋,双耳3例,单耳2例;声导抗测听鼓室曲线A型5例,声反射不能引出4例;DPOAE检查4例不能引出,前庭功能检查半规管功能低下3例。ABR检查2例为蜗后性改变,影像学检查CT无特殊表现;MRI检查未见明显异常1例,呈现炎症性改变4例。腰穿正常2例,脑脊液压力增高、细胞数高3例。最后确诊疾病:急性小脑炎1例,病毒性脑炎2例,结核性脑膜炎2例。结论临床上在诊治突发性耳聋的患者时应考虑到有颅内病变的可能,尤其对听力损失重度,且为双侧性以及有发热、头痛、眩晕等症状重者需进MRI、ABR、腰穿等检查明确诊断。     

突发性聋与颈静脉球憩室关系初探

为探讨颈静脉憩室（ＪＢＤ）与突发性聋（突聋）的相关性，报告收治突聋１９例中，ＣＴ及磁共振血管造影显示右侧ＪＢＤ３例，共颈静脉孔和乙状窦均明显扩大。其产生内耳功能障碍的机理可能为：①ＪＢＤ压迫与阻塞耳蜗导水管和（或）前庭导水管，甚至内淋巴囊；②ＪＢＤ内的迂回血流对内耳的流体机械力学影响。提示：原因不明的内耳功能障碍病人，应作影像学检查，考虑有无ＪＢＤ的相关影响。     

儿童及成人突发性聋预后的对比分析

目的对比分析儿童及成人突发性聋预后的相关因素,为临床实践及患者预后评估提供依据。方法回顾性分析2008年1月~2016年12月住院治疗的237例（258耳）突发性聋患者的临床资料,其中儿童突发性聋患者（儿童组）26例（29耳）,成人突发性聋患者（成人组）211例（229耳）,采用单因素及多因素分析的方法对比分析两组突发性聋患者的年龄、性别、耳侧、初诊时间、就诊听阈、听力曲线、有否伴有耳鸣、眩晕及耳闷等因素对预后的影响。结果儿童突发性聋患者人数占总人数的11.0%;儿童组就诊听阈平均为（88.7±15.8）dB,高于成人组［（71.8±23.6）dB,(P＜0.05)］;儿童组听力曲线为全聋型的占69.0%,高于成人组（44.5%,P＜0.05）;经治疗后,儿童组的总体有效率为51.7%,成人组总体有效率为45.4%,两者差异无统计学意义（P＞0.05）。对儿童组预后的相关因素分析显示就诊听阈及伴发眩晕对疗效的影响具有统计学意义(P＜0.05);成人组中,初诊时间、就诊听阈及伴发眩晕对疗效的影响具有统计学意义（P<0.05）。结论儿童突发性聋患者就诊时听力损失较成人重,但治疗后两者总体有效率无明显差异（P>0.05）;儿童及成人突发性聋患者中听力损失程度越轻且不伴发眩晕者预后较好;就诊时间越早越有利于成人突发性聋患者的预后;而年龄、性别、耳侧以及是否伴发耳鸣、耳闷对儿童及成人突发性聋的预后无明显影响。     

Radiological quiz. Sudden sensorineural hearing loss due to multiple sclerosis

Objective

A case with sudden sensorineural hearing loss (SSNHL) owing to multiple sclerosis (MS) who had clinical and dramatic radiological improvement just after medical therapy was reported in this article.

Method

Case report and review of related literature.

Results

A 22-year-old female patient with MS related SSNHL was presented in this article. Magnetic resonance imaging (MRI) revealed an MS plaque localized at pons extending from right cochlear nucleus to proximal part of the right cochlear nerve. Most dramatic recovery was present in the 5th day control MRI, where the plaque located on pons disappeared completely. On the 10th day control audiogram hearing recovery was observed and pure tone audiogram levels were almost normal.

Conclusion

Sudden sensorineural hearing loss owing to MS is seen more common than expected. It has good prognosis. Magnetic resonance imaging is also thought to have an important role in diagnosis and treatment efficacy of SSNHL owing to MS.     

儿童突发性聋的临床特征及预后相关因素分析

目的 分析儿童突发性聋的临床特征、疗效及影响预后的相关因素,为临床治疗及预后评估提供依据。 方法 收集2010年1月至2017年10月就诊的67例突发性聋患儿临床资料,对其临床特征及治疗效果进行回顾性分析,同时根据疗效将患者分为总体有效组(36例)及无效组(31例),采用单因素及多因素分析的方法分析患者的性别、年龄、病程、初诊听阈、是否伴发耳鸣、眩晕、病毒感染史、发病季节和听力曲线类型对预后的影响。 结果 儿童突聋患者中64.18%在春冬季发病,其就诊时听阈为(76.62±25.97)dB HL,耳鸣及眩晕伴发率分别为70.15%和61.19%,病毒感染率为19.40%,听力曲线中10.44%为低频下降型、2.99%为高频下降型、34.33%为平坦型及52.24%为全聋型。经治疗后,患者听阈为(60.41±31.52)dB HL,总体有效率为53.73%,其中痊愈率、显效率及有效率分别为20.90%、16.42%和16.42%。多因素分析结果显示,初诊听阈越高及听力曲线为全聋型,预后越差(P均<0.05);伴有病毒感染的非全聋型患者预后较好(P<0.05)。 结论 儿童突发性聋患者病毒感染率较高且大部分在春冬季发病,就诊时听力损失较重并常伴有耳鸣及眩晕,其听力曲线以平坦型及全聋型为主。就诊时听力损伤程度轻、伴有病毒感染的非全聋型患者预后较好。     

Sudden sensorineural hearing loss as a revealing symptom of vestibular schwannoma

Conclusions

Sudden sensorineural hearing loss (SSHL) is a frequent symptom of vestibular schwannoma (VS), often reveals small VSs and does not exhibit specific features. Therefore, every case of SSHL should be evaluated using systematic MRI to rule out VS in order to improve hearing and preservation facial nerve function.

Objective

SSHL leads to the discovery of a VS in a small proportion of cases (2%). However, SSHL appears to be a more frequent occurrence in the history of patients with VS (3–23% in the literature), suggesting a large disparity in the evaluation of SSHL.

Material and methods

A total of 139 consecutive unilateral VSs operated on between 2000 and 2002 were reviewed and analyzed regarding the prevalence, clinical and audiological features of SSHL and their relation to the size of the tumor.

Results

SSHL was observed in 20% of cases at some point in their VS history. The characteristics of SSHL were: (i) lack of a specific audiometric pattern, except that low-tone loss was rare; and (ii) a high rate of hearing recovery (50%). Tumor size was significantly smaller in SSHL-associated VSs compared to other VSs. In the former cases, 96% involved the internal auditory canal.     

突发性感音神经性聋病人的血清TNFα和IL-6检测

为探讨突发性感音神经性聋发病与血清细胞因子的关系，采用夹心法ＥＬＩＳＡ检测了３２例突聋病人、１５例慢性进行性感音神经性聋病人和２０例健康对照者血清肿瘤坏死因子和白细胞介素水平。     

Comparison of Sudden Deafness in Adults and Children

Objectives

Although many studies have assessed sudden deafness in adults, sudden deafness has not been evaluated in children. We therefore evaluated the differences in sudden deafness between children and adults.

Methods

We compared clinical manifestations, including gender, audiogram pattern of initial hearing loss, and recovery rate after treatment in 87 children and 707 adults diagnosed with sudden deafness from September 2003 and August 2012.

Results

There were no differences in sex, side, or audiogram between children and adults (P>0.05 each). Hearing recovery rates in children and adults were 72.4% and 70.6%, respectively (P>0.05). Both children and adults with mild hearing loss showed significantly greater hearing recovery rates than individuals with profound hearing loss (P<0.05 each). The percentage with initially mild and moderate hearing loss was higher in children than in adults, as were the recovery rates of children compared to adults with initially mild, moderate-severe, and profound hearing loss (P<0.05 each). In regard to final hearing outcome after treatment, a low percentage of children showed no improvement whereas a high percentage showed complete recovery; a higher percentage of children than of adults showed complete recovery (P<0.05). Recovery rate from profound hearing loss was significantly higher in children than in adults (60.0% vs. 45.4%, P<0.05).

Conclusion

Degree of hearing loss, gender, side, and recovery rate were similar in children and adults, but the rate of complete recovery was higher in children.