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中华耳科学杂志

中华耳科学杂志

北大核心

CSCD

CSTPCD

Chinese Journal of Otology
本刊主要面向从事耳科学，神经耳科学和耳神经外科学工作的临床、科研及教学人员。办刊方针为;贯彻理论与实践，普及与提高相结合的卫生工作政策。反映我国耳科学临床和科研工作的最新进展，促进国内外学术交流，推动我国耳科学发展。
  • 发文量：2652
  • 被引量：1950
  • H指数：3
  • 开始收录时间：2003(1)
  • CNKI综合因子：1.053
  • 维普期刊影响因子： 1.68
  • 万方期刊影响因子：2.21
  • 创刊时间：2003
  • 国内刊号/CN：11-4882/R
    国际刊号/ISSN：1672-2922
  • 发行周期：季刊
    邮发代号：82-114
  • 主管单位：解放军总医院
    主办单位：解放军总医院耳鼻咽喉科研究所
中华耳科学杂志-联系信息
  • 主编：王秋菊;戴朴（执行）
  • 电话：010-66939502 68286148
  • 邮箱：zhek301@sina.com;zhek301@163.com
  • 地址：北京市海淀区复兴路28号
  • 邮编：100853
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中华耳科学杂志 >2023年第4期

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  • 摘要：Objective To find a more suitable animal model for investigating the noise-induced hearing loss by comparing the effects of 120 dB SPL white noise exposure for three hours on the hearing in bama miniature pigs and guinea pigs. Methods Auditory brainstem response (ABR) in 8 bama miniature pigs and 8 guinea pigs with normal hear-ing was tested before exposure, immediately (P0), 1 day (P1) and 7 days (P7) after exposure. Cochlear specimens were observed using scanning electron microscopy. Results The ABR threshold showed a statistically significant difference between the immediate group and P1 group in guinea pigs, but not in bama miniature pigs. There was a statistically sig-nificant difference between the immediate group and P7 group in bama miniature pigs, but not in guinea pigs. Further-more, the bama miniature pigs showed more severe cochlear hair cell damages than that in guinea pigs. Conclusions The bama miniature pig cochleae are more vulnerable to 120 dB SPL white noise exposure, with more profound hearingloss and more severe pathological changes. The results suggest that bama miniature pig could be a more suitable animal model for noise-induced hearing loss study.%目的 通过120dB SPL宽频带白噪声对巴马香猪和豚鼠各进行单次3小时的暴露,观察比较该种噪声对两种模型动物听力损伤的特点,找到更适用于研究噪声性听力损伤的动物模型.方法选取8只6月龄ABR听阈正常的巴马香猪,和8只5~6周龄ABR听阈正常的豚鼠,设置4个ABR测听记录点:噪声暴露前,噪声暴露后即刻(P0)、1天(P1)、7天(P7).全部测听结束后,取材制作耳蜗标本,进行扫描电镜观察形态.结果通过统计学分析,豚鼠组在120dB SPL宽频带白噪声暴露后即刻的ABR阈值与暴露1天的ABR阈值具有明显差异,具有统计学意义,而巴马香猪组则无明显统计学差异.豚鼠组在噪声暴露前和噪声暴露后7天的ABR阈值无明显统计学差异,而巴马香猪组却有明显的统计学差异.相对于豚鼠,巴马香猪耳蜗标本扫描电镜提示了更多的纤毛异常.结论120dB SPL宽频带白噪声暴露会对巴马香猪和豚鼠造成一定程度的听力损伤.其中巴马香猪较豚鼠表现更为明显的听力损失,且毛细胞出现了更多的病理改变,由此本研究表明在120dB SPL宽频带白噪声单次暴露3小时的条件下,巴马香猪是一个更适于白噪声与听力损伤的研究的动物模型,从而可以更好地为噪声性耳聋提供动物模型.
  • 摘要：Ossiculoplasty is indicated for ossicular chain interruption, while stapedioplasty is for otosclerosis. In ossiculoplasty, House and Fisch had their own criteria in the use of prosthetic ossicles and autologous incus. In sta-pedioplasty, House and Fisch performed differently in anesthesia, the external ear canal incision, the external audito-ry canal flap, exposure of tympanic cavity, stapes footplate window opening, and the footplate window size, etc.%听骨链成形术适用于听骨链中断,镫骨成形术适用于耳硬化症.实施听骨链成形术时House与Fisch在选用人工听小骨与自体砧骨等方面各有标准;实施镫骨成形术时House与Fisch在麻醉方式、是否作外耳道口切口、制作外耳道皮瓣、鼓室暴露范围、镫骨足板开窗步骤顺序、足板开窗大小等均有差异.
  • 摘要：Temporal bone resection is mainly used in the temporal bone malignancy. Subtotal petrosectomy is mainly used for benign temporal bone tumors. There are similarities between these two surgical procedures. However, they differ from each other with different criteria in the selection of subjects, whether or not the parotid gland is re-moved, whether or not the neck dissection is performed, whether or not the mandibular joint capsule is resected, and whether or not the facial nerve is preserved.%颞骨切除术主要应用于颞骨恶性肿瘤,岩骨次全切除术主要应用于颞骨良性肿瘤.两种术式有相似之处,但在选择处理对象、是否切除腮腺、是否实施颈廓清术、是否切除下颌关节囊、是否保留面神经等方面均有不同标准.
  • 摘要：目的 分析研究内耳结构异常患者人工耳蜗植入术的安全性及手术后的疗效情况.方法 选取2011.07-2015.06在我院住院接受人工耳蜗植入的17例内耳结构异常患者的临床资料,其中语前聋14例,语后聋3例,针对内耳结构异常情况采用不同术式.用听觉分级行为标准(Categorise of Auditory Performance,CAP)、言语可懂度分级标准(Speech Intelligibility Rating,SIR)进行术后效果评估.选取24例同期接受人工耳蜗植入的内耳正常的语前聋患者作为对照组,分析并比较两组患者手术并发症及术后疗效.结果:17例患者手术顺利,术后出现1例眩晕,所有患者术后均无脑脊液耳漏、面瘫等并发症.内耳结构异常语前聋患者术后CAP和SIR值分别为5.8±1.4、3.5±1.1;对照组CAP和SIR值分别为6.9±1.3、3.8±0.9,两者差异无统计学意义(P>0.05).结论内耳结构异常非人工耳蜗植入术的绝对禁忌证,根据异常程度制定个性化的手术方案,可以减少并发症的发生.内耳结构异常语前聋患者术后言语康复情况、对声音的自发性觉察能力与耳蜗形态正常患者术后无明显差异.%Objective To study the safety of cochlear implantation in patients with abnormal ear structure and the outcome postoperatively. Methods The clinical data of 17 patients with abnormal ear structure underwent cochlear im-plantation between July 2011 and June 2015 were retrospectively analyzed, which include 14 cases with pre-lingual deafness and 3 cases with post-lingual deafness. Different surgical procedures were used based on the abnormalities of the inner ear. The postoperative evaluation was performed using the auditory classification criteria (CAP) and Speech Intelligibility Rating (SIR). Twenty-four patients with pre-lingual deafness and normal inner ear anatomy were enrolled in this study as control. The complications and postoperative auditory performance were analyzed and compared be-tween the two groups. Results Cochlear implantations were successfully performed in all 17 cases with abnormal ear anatomy. One of the case experienced vertigo postoperatively, however, no other complications including cerebrospinal fluid leakage or facial paralysis occurred. The postoperative CAP and SIR scores were 5.8±1.4 and 3.5±1.1 respectively in patients with abnormal ear anatomy and pre-lingual deafness. In the control group, the CAP and SIR scores were 6.9 ± 1.3 and 3.8 ± 0.9 respectively. There was no significant difference between the two groups (P>0.05). Conclusions Abnormal inner ear anatomy is not an absolute contraindication for cochlear implantation. Selection of a proper surgi-cal procedure according to the type of abnormal inner ear structure could reduce the incidence of complications. There were no significant differences in hearing or speech rehabilitation outcomes between the control and the group with ab-normal inner ear structures.
  • 摘要：患者陈女士，58岁，汉族，河南省人，职员。以“左耳搏动性耳鸣5年”主诉入院。患者于5年前劳累后逐渐出现左侧搏动性耳鸣，持续性，似“火车车轮声”，与心脏跳动一致，仅患者本人可闻及，改变头位对耳鸣无影响，但手指压迫左侧颈部后耳鸣可暂时消失，发病后左耳听力逐渐降低。
  • 摘要：目的分析一个常染色体显性遗传性非综合征耳聋大家系的临床特征,进行候选致病基因的突变筛查.方法对家系成员进行病史采集、全身检查、听力学评估及颞骨CT检查;抽提家系成员外周血基因组DNA;整理分析家系资料绘制系谱图;使用定向捕获联合二代测序技术,对包括所有已知非综合性耳聋的137个耳聋相关基因的外显子及其侧翼内含子序列进行筛查;对可疑基因进行Sanger测序验证.结果该家系共4代,现存家系成员28人,系谱分析符合常染色体显性遗传特征.参与本研究的耳聋患者9人,均为语后聋,均表现为迟发性、渐进性听力下降,发病年龄6~18岁,听力曲线多为平坦型.先证者(Ⅳ-4)检测结果经数据分析滤过后确定2个潜在基因致病突位点:MYH14,c.359C>T,p.S120L;COL11A2,c.4478G>A,p.R1493Q.后续经直接测序验证,在该家系中,只有MYH14,c.359C>T变异和家系的表型共分离,其余1个可疑突变位点在家系中无共分离现象.结论本研究鉴定了一个常染色体显性遗传性非综合征耳聋大家系的致病突变(MYH14,c.359C>T),同时证实高通量基因捕获测序技术是遗传性耳聋的一种行之有效的分子诊断工具.%Objective To investigate the clinical features of a large Chinese family with progressive autosomal dominant hearing loss, and to search for candidate mutational genes. Methods Collections of detailed medical history, physical examinations, audiologic testing, and CT scan of the temporal bones were performed, and genomic DNA was extracted from peripheral blood samples of the family members. The inheritance model of the family was evaluated. 137 deafness genes of the proband were captured and sequenced by targeted next-generation sequencing (NGS), and the re-sults were confirmed by Sanger sequencing.Results This family has 28 members in 4 generations, of whom 9 persons are affected. All affected family members exhibit late-onset, progressive non-syndromic sensorineural hearing loss. The ages of onset were between 6 and 18 year-old. Two heterozygous missenses (MYH14 mutation c.359C>T and COL11A2 c.4478G>A) were identified. Variants were further confirmed by Sanger sequencing. Only the MYH14 muta-tion was co-segregated with autosomal dominant hearing loss phenotype. Conclusions The heterozygous MYH14 muta-tion c.359C>T is responsible for the autosomal dominant sensorineural hearing loss in this Chinese family, and this re-port confirms that targeted NGS technique is a feasible and more cost-effective tool to detect causative mutations in he-reditary hearing loss.
  • 摘要：经迷路径路与经耳囊经路均可应用于切除听神经瘤,House常用前者,Fisch推崇后者.两种手术径路各有特点,一般认为经耳囊径路是在经迷路径路的基础上进行了改进,扩大了手术径路范围,降低了面神经损伤、脑脊液漏等并发症的发生率.%Both the translabyrinthine and transotic approaches are used for acoustic neuroma resection. The trans-labyrinthine approach was frequently used by Dr. House, while Dr. Fisch preferred the transotic approach. Each ap-proach has its own advantages. It is generally believed that the transotic approach is an improved version of the translab-yrinthine approach, which enlarges the scope of the surgical approach and reduces the incidence of complications such as facial nerve injury and cerebrospinal fluid leakage.
  • 摘要：Meniere's disease is an inner ear disorder characterized by recurrent episodes of rotational vertigo, fluc-tuating sensorineural hearing loss, and tinnitus, with or without ear fullness. The pathogenesis of Meniere's disease is unclear. The typical pathological feature of Meniere's disease is endolymphatic hydrops, although the precise mecha-nism is not fully understood. Studies have confirmed that endolymphatic sac plays an important role in endolymph vol-ume regulation, and the cascade of histopathological and electrophysiological changes associated with chronic endolym-phatic hydrops. Using the animal model of endolymphatic hydrops to investigate variable aspects of the disease (e.g., how hydrops occurs, its relationship with pathology and electrophysiology changes, how it affects the inner ear func-tion, and so on) would be helpful to guide the treatment of the disease. The periodic episodes of Meniere's disease sug-gest that controlling the endolymphatic hydrops might be the key to maintain the inner ear homeostasis and to avoid pro-gression of the disease.%梅尼埃病是一种内耳疾病,临床表现以反复发作的旋转性眩晕,波动性感音神经性听力损失,耳鸣和/或耳胀满感为特征.梅尼埃病的病因尚不明确.众所周知,其病理特征为内淋巴积水,但内淋巴积水的具体作用机制尚未完全明确.已有研究证实,内淋巴囊在内淋巴体积调节、与慢性内淋巴积水相关的组织病理及电生理级联变化中具有重要作用.通过内淋巴积水动物模型研究积水产生过程、积水与病理及电生理变化的关系,以及这些变化是如何影响内耳功能的,可以帮助有效治疗梅尼埃病.梅尼埃病的周期性发作提示或许可以通过控制内淋巴积水使内耳维持稳定状态,避免进展性损伤及二次损伤.
  • 摘要：在北京市0-6岁儿童听力保健专家组的指导下，由北京协和医院具体承办了京津冀地区儿童听力诊断中心2016年度第四季度的学术活动。会议由北京协和医院耳鼻咽喉科儿童听力诊断中心商莹莹教授主持。本次活动的主题是:听力损失患者的中枢改变。参加本次学术会议的代表来自多家单位，不仅包括北京市卫计委、北京市妇幼保健院的相关领导，北京市六家儿童听力中心：北京儿童医院、北京大学第三医院、中国聋儿康复中心、北京同仁医院、中国人民解放军总医院、北京协和医院，还包括北京、天津、廊坊妇幼保健院等妇幼保健系统的很多同道，以及北大医院等多家医院的临床工作者。
  • 摘要：Objective To investigate the function of auditory cortex by testing the mismatch negativity (MMN) in patients with cleft lip and/or palate who present with normal pure tone audiometry (PTA) and auditory brainstem re-sponse (ABR). Methods MMN was tested in 39 cases with cleft lip and palate (CLP), 28 cases with cleft lip (CL), 17 cases with cleft palate (CP), and 39 healthy volunteers (normal group) who present with normal PTA and ABR. The dif-ferences in latency and amplitude of MMN among these groups were analyzed. Results The MMN latency of CP group was significantly prolonged compared to normal group and CL patients (P<0.05), and had no significant difference com-pared to CLP patients (P>0.05);the MMN latency in CLP patients was significantly prolonged compared to CL and nor-mal groups (P<0.05);the MMN latency in CL patients showed no significant difference compared to normal group (P>0.05). There is no significant difference in MMN amplitude among all groups (P>0.05). Conclusions Dysfunction of au-ditory cortex could be present in CP and CLP patients, which probably results in speech dysfunction.%目的 对纯音测听(pure tone audiometry,PTA)、听性脑干反应(auditory brain stem response,ABR)正常的唇腭裂患者行听觉失匹配负波(auditory mismatch negativity,AMMN)检查,并与健康正常人对照,判断唇腭裂患者听觉皮层的功能状态,为探讨其言语功能障碍机制提供理论依据.方法唇裂伴有腭裂(Cleft Lip and Palate,CLP)39例,单纯唇裂(Cleft Lip,CL)28例,单纯腭裂(Cleft Palate,CP)17例,健康志愿者(正常组)39例(纯音测听、听性脑干反应均正常)分别行AMMN检查,比较各组间潜伏期及波幅的差异.结果CP组AMMN潜伏期较正常组、CL组显著延长(P<0.05),CP组潜伏期较CLP组无明显差异(P>0.05);CLP组潜伏期较CL组、正常组显著延长(P<0.05);CL组潜伏期与正常组无明显差异(P>0.05).各组间AMMN的波幅无明显差异(P>0.05).结论CP及CLP患者听觉皮层的功能可能存在异常,可能为导致其言语功能障碍的一个原因,但仍然需要结合其言语识别能力、言语功能评定、磁共振(magnetic resonance,MR)、功能性磁共振(functional magnetic resonance imaging,fMRI)加以进一步研究.
  • 摘要：The otology has a long history, in that many schools, masters came forth in large numbers. Dr. Politzer was honored the father of modern otology. Dr. Wulllstein etc. laid the foundation for the development of microsurgery of the ear. Dr. House was the pioneer of modern skull base surgery. Dr. Fisch established the system of lateral skull base surgery. The invention of the binocular surgical microscope (OPMI 1) has changed the history of otology, and the inven-tion of CT and MRI has greatly contributed to the development of skull base microsurgery.%耳科历史源远流长,流派众多,大师辈出.Politzer为现代耳科学创始人,Wulllstein等奠定了显微耳科基石,House创立了现代颅底外科,Fisch建立了侧颅底外科体系.双目手术显微镜(OPMI 1)的发明改变了耳科学的历史,CT、MRI的发明极大地促进了颅底显微外科的发展.
  • 摘要：目的常染色体隐性遗传远端肾小管酸中毒是一种严重的酸碱平衡紊乱性疾病,常常合并有感音神经性聋,ATP6V0A4被证实是其致病的相关基因,目前仍不明确致病机制.我们拟通过研究Atp6v0a4在不同发育阶段小鼠的内耳表达来探究ATP6V0A4在听觉系统形成中的作用.方法选择不同发育时期健康的昆明小鼠(E18,P1,P7,P14,P21),应用免疫荧光标记技术结合激光共聚焦显微镜观察Atp6v0a4蛋白在内耳的定位与表达.West?ern-blot定性定量研究该蛋白在不同时期小鼠内耳蛋白表达变化.结果Atp6v0a4蛋白主要表达部位为耳蜗内毛细胞、外毛细胞、血管纹、内淋巴囊以及螺旋神经节,且在不同发育阶段表达位置稳定.Western-blot结果提示Atp6v0a4在小鼠内耳广泛表达,在不同发育阶段,Atp6v0a4在Corti's器及螺旋神经节及血管纹中表达相对稳定,而在前庭组织中表达下降.结论通过对不同发育阶段的小鼠耳蜗切片进行Atp6v0a4蛋白表达分析研究,明确了Atp6v0a4在小鼠内耳的表达,为深入研究Atp6v0a4在内耳发育过程中的作用和致聋机制奠定了基础.%Objective Autosomal recessive distal renal tubular acidosis (dRTA), a severe disorder of acid–base homeostasis, is often accompanied by sensorineural deafness. The ATP6V0A4 is considered as the causative gene;how-ever, it is unknown how this gene causes the diseases. This study aims to investigate the expression of ATP6V0A4 in the mice inner ear at different stages of development. Methods Kunming mice at different developmental stages (Embryo 18, Postnatal 1, Postnatal 7, Postnatal 14, Postnatal 21) were used. The expression of Atp6v0a4 in the mice inner ear was analyzed using combined techniques of immunohistochemistry and immunofluorescence under confocal laser scan-ning microscopy. In addition, western-blot was used to analyze the expression of Atp6v0a4 quantitatively and qualita-tively in mice inner ear. Results Atp6v0a4 was steadily expressed in the inner and outer hair cells, stria vascularis, spi-ral ganglion, and endolymphatic sac during the inner ear development, as shown by confocal laser scanning microscopy. The western-blot showed that the expression of Atp6v0a4 is relatively stable in the organ of Corti, spiral ganglion, andstria vascularis, while the expression of Atp6v0a4 in vestibule decreased during inner ear development. Conclusions Ex-pression and localization of Atp6v0a4 in the mice inner ear at different developmental stages are demonstrated. This study may pave the way for further investigation in the Atp6v0a4 related deafness mechanisms.
  • 摘要：The middle cranial fossa approach frequently used by Dr. House and the transtemporal supralabyrin-thine approach preferably used by Dr. Fisch can be used for acoustic neuroma resection with hearing preservation. How-ever these two approaches differs in many ways, including the size of the bone window on the squamous portion of the temporal bone, the degree of dura elevation in the middle cranial fossa, whether the middle meningeal artery is used as a landmark, how to locate the internal auditory canal, and the sequence of cutting off the bone in skull base.%House常用的颅中窝径路和Fisch推崇的经颞迷路上径路均可应用于保留听力的听神经瘤切除术.二者在颞鳞部骨窗大小、中颅窝脑膜抬起程度、是否以脑膜中动脉为标志、如何定位内听道、磨除颅底骨质先后顺序等方面均有差异.
  • 摘要：Secretory otitis media is a non-supprative inflammatory disease which is most common in chil-dren. It is characterized by middle ear effusion and hearing loss. If not treated properly, the disease could result in impairment of language development and balance dysfunction. This article reviews recent progress in clinical diag-nosis and treatment of secretory otitis media.%分泌性中耳炎是一种以中耳积液和听力下降为主要特征的中耳非化脓性炎性疾病,以儿童多见,可导致患儿语言发育障碍、平衡功能障碍等,故而了解其病因和发病机制和诊疗方法,对于改善患者症状和生活质量具有重要意义,本文综述了近些年分泌性中耳炎的临床诊治进展.
  • 摘要：一个好医生面对患者会提出四个问题：是什么病？危险吗？病因是什么？如何治疗？如何快速找到病因，作出准确诊断，需要不断学习。很多医生期望学到某种特效疗法，提高手术技术，其实相对于这些技术方法而言，临床思维更重要。现代医学发展，专业逐渐细化，各专业的专家多了，但像我国消化内科的鼻祖，协和医院张孝骞这样的名医大家逐渐少了。
  • 摘要：Objective To investigate the clinical and psychoacoustic characteristics in patients with acute and chronic tinnitus. Methods A total of 343 patients with tinnitus were enrolled in this study. Detailed medical history col-lection, systemic physical examination, audiologic testing, tinnitus evaluation, Tinnitus Handicap Inventory (THI) scale, Self-Rating Anxiety Scale (SAS), and Self-Rating Depression Scale (SDS) were performed. Tinnitus is divided into acute (tinnitus course<6 months) and chronic (tinnitus duration≥6 months), according to the duration of tinnitus. Re-sults The frequency of tinnitus in patients with acute and chronic tinnitus was significantly different (P<0.05). Most pa-tients with acute tinnitus reported ear ringing of low or middle frequency pitch, while more patients with chronic tinni-tus reported high pitch ear ringing. Patients with acute tinnitus were more likely to develop anxious state, while patients with chronic tinnitus were more likely to develop depressive symptoms. There was no significant difference in gender, age, tinnitus loudness, THI, sleeping disturbances between the acute and chronic tinnitus groups (P>0.05). Conclusions The frequency of tinnitus and the association with anxiety and depressive symptoms in patients with acute and chronic tinnitus are instructive in the treatment of tinnitus patients and deserve further investigation.%目的 了解急、慢性耳鸣患者的临床特征,心理声学及伴发症状的情况.方法对343例耳鸣患者进行详细的病史采集,系统的耳部检查、听力学及耳鸣匹配检查,并完成耳鸣残疾量表(THI)及焦虑自评量表(SAS),抑郁自评量表(SDS)的填写.按耳鸣的病程将耳鸣分为急性耳鸣(耳鸣病程<6个月),慢性耳鸣:耳鸣病程≥6个月,最后统计分析急性与慢性耳鸣的临床特征、耳鸣心理声学的特征.结果急、慢性耳鸣患者的耳鸣匹配频率构成差异有统计学意义(P<0.05),急性耳鸣中,低中频所占比例较高,慢性耳鸣中高频所占比例较高;急、慢性耳鸣患者伴发焦虑、抑郁状态差异有统计学意义(P<0.05),急性耳鸣患者较慢性耳鸣患者更倾向伴发焦虑状态,而慢性耳鸣患者更倾向伴发抑郁症状.急、慢性耳鸣患者的性别、年龄分布,耳鸣匹配响度,是否有睡眠障碍、耳鸣严重程度差异无统计学意义(P>0.05).结论急、慢性耳鸣患者的耳鸣频率和伴发焦虑、抑郁症状存在差异,对耳鸣患者的治疗有指导意义,值得进一步研究.
  • 摘要：Canalplasty (external auditory canal plasty) is an integral part of myringoplasty (which is the technique of repairing tympanic membrane). In canalplasty, House and Fisch performed differently in the posterior auricle inci-sion, the posterior auricle periosteal flap, the external auditory canal incision, and management of the skin of the external auditory canal. In myringoplasty, House and Fisch differed in the concepts of underlay and overly, temporal fascia man-agement, tympanic ring management, making external ear canal skin flap, fascia placement, flap placement, and so on.%外耳道成形术是鼓膜成形术的有机组成部分,鼓膜成形术是修补鼓膜的技术.实施外耳道成形术时House与Fisch在耳后切口、耳后骨膜瓣、外耳道内切口、外耳道后壁及前壁皮肤的处理等方面均有差异;实施鼓膜成形术时House与Fisch在内植与外植概念、常用植入筋膜方式、颞肌筋膜处理方式、鼓环的处理、外耳道皮瓣制作、筋膜放置方式、皮瓣复位等方面各有不同.
  • 摘要：Objective Using bioinformatics methods to analyze gene expression microarray of otitis media in order to establish a protein interaction network. Methods The microarray data sets of otitis media from the mouse were down-loaded from the public gene expression database Gene Expression Ombibus (GEO ). The differentially expressed genes were screened by Affymetrix Expression Console software and Transcriptome Analysis Console (TAC) software. Then the differentially expressed genes were introduced into the STRING online database for analysis, and the interaction net-work was established. The interaction data was subsequently introduced into the Cytoscape 3.4.0 software, and the net-work center nodes were screened, and those relatively independent sub-networks associated with the inflammation genes were selected. David online tools were used for functional annotation enrichment analysis of these sub-networks. Results A total of 1166 differentially expressed genes were screened out, including 793 up-regulated and 373 down-reg-ulated. Screened by STRING, interactions of protein products from 459 differentially expressed genes were found, andthe corresponding protein interaction network was obtained. Conclusion In this study, bioinformatics techniques were used to construct the interaction network of the differentially expressed genes for otitis media. The network, especially the center node gene could provide new clues of the molecular mechanism of otitis media, and guide the targeted therapy.%目的利用基因表达数据库(GEO)查找小鼠中耳炎表达谱基因芯片原始数据,建立中耳炎基因互作网络图.方法从公共数据库GEO中下载小鼠中耳炎相关数据集,采用Affymetrix Expression Console软件和Tran?scriptome Analysis Console(TAC)软件筛选差异表达基因,将差异基因导入STRING在线数据库进行分析,绘制差异基因互作网络图,并将互作网络数据导入Cytoscape 3.4.0软件中,筛选网络中心节点,并取其中相对独立、作用较为集中且与炎症基因相关子网络,采用David在线工具对其进行功能注释富集分析.结果共筛选出1166个差异表达基因,其中表达上调793个,表达下调373个,经STRING在线工具筛选后共有459个小鼠中耳炎差异基因编码的蛋白存在相互作用,并得到相应蛋白互作网络,分析其中与炎症相关子网络.结论本研究利用生物信息学技术构建了中耳炎差异基因互作网络,其中的网络中心节点基因,对于中耳炎分子机制研究及靶向治疗具有指导作用.
  • 摘要：The nomenclature, types and surgical resection approaches for glomus jugulare tumors vary. De La Cruz and Fisch created their own typing systems. De La Cruz's typing is simple and practical, and Fisch's typing is more accurate. Infratemporal fossa approach type A is the first choice for those with involvement of internal carotid artery in the temporal bone and/or for those with intracranial jugular involvement.%颈静脉球体瘤的命名、分型与手术方式各异,De La Cruz和Fisch均提出自己的分型系统.De La Cruz的分型简洁实用,Fisch的分型更精确.累及到颈内动脉颞骨段及颅内的颈静脉球体瘤首选颞下窝A型径路.
  • 摘要：耳聋是一种常见的公共健康问题,对社会经济造成极大的损失.X染色体基因突变可引起综合征型遗传性耳聋和非综合征型耳聋,其中非综合征型耳聋相关位点报道甚少.本文针对引起非综合征型耳聋和部分综合征型耳聋的X连锁耳聋基因与位点进行综述,并探讨X连锁耳聋家系的研究策略.%Hearing loss is one of the most common public-health issue that results in enormous cost to the soci-ety. Deafness genes located on the X chromosome can cause syndromic or non-syndromic hearing loss. There are few re-ports about X-linked loci for non-syndromic hearing loss. This article reviews X-linked genes and loci on the X-chro-mosome which lead to syndromic and especially non-syndromic hearing loss.
  • 摘要：听力损失严重影响社交能力.对听障人群早期发现、早期诊断、早期治疗和康复就显得格外重要.目前,新生儿听力筛查技术已经不断完善,对听障儿童的早期发现和早期诊断已经取得显著成就,但是对重度和极重度耳聋患儿的早期治疗即人工耳蜗植入及康复却任重而道远.如何更好地提高人工耳蜗植入患儿的听觉言语康复疗效成为重中之重.本文就人工耳蜗植入患儿听觉言语康复方法、康复疗效评估方法、影响因素及目前存在问题和展望作出如下综述.%Hearing loss has significant impact on patients'social communications. Early detection, diagnosis, treatment and rehabilitation are therefore particularly important. At present, the improved newborn hearing screening technology has led to remarkable progress in early detection and diagnosis of hearing impairment in children. However, there is a long way to go regarding early management of the severely hearing impaired children who underwent cochlear implantation and post-operative rehabilitation. Improvement of hearing and speech rehabilitation outcomes is critical and challenging in this group of patients after cochlear implantation. In this article, the hearing and speech rehabilitation methods for children patients who underwent cochlear implantation, evaluation methods of outcomes, influence factors, problems currently present, and prospects in the field are reviewed.
  • 摘要：Objective To analyze the deafness gene mutations in children with non-syndromic hearing impairment (NSHI), and to investigate the genetic etiology and features of deafness disorders at the molecular level. Methods 94 children with NSHI diagnosed by The Center of Diagnosis and Treatment of Children's Hearing Disorders in Tianjin were screened for 20 hotspot of hearing loss-associated mutations from GJB2, GJB3, SLC26A4 and mitochondrial 12S rRNA (MTRNR1) using the matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS). The data of genetic screening was analyzed with correlation to audiologic testing results. Results In 94 cases with moderate to profound sensorineural hearing loss, 73 of which presented with bilateral sensorineural hearing loss, and 21 with unilateral hearing loss. Deafness gene mutations were found in 31 subjects (32.98%, 31/94), 13 of which with sin-gle gene homozygous mutations, 9 of which with single gene and double-site heterozygous mutations, and 9 with sin-gle-gene and single-site mutations. The positive detective rate of deafness gene mutations in bilateral hearing loss group was 42.47%but no deafness gene was detected in unilateral hearing loss group. The positive detective rate of GJB2 and SLC26A4 in 94 subjects were 17.02%and 15.96%respectively, and no mutations found on GJB3 and MTRNR1. Sixteen subjects showed GJB2 mutations, 8 of which with homozygous mutations, 5 with double-site heterozygous mutations, and 3 with single-site heterozygous mutations. Fifteen subjects with bilateral hearing loss were SLC26A4 (PDS)-positive,5 of which were homozygous (at IVS7-2A>G), 4 subjects with double-site heterozygous mutations, and 6 subjects with single-site heterozygous mutations. The positive detective rates for GJB2 and SLC26A4 genes in the bilateral hearing loss group are 21.92% and 20.55% respectively, however these genes were not detected in the unilateral hearing loss group. Conclusions High proportion of etiologies in patients with NSHI were due to genetic factors. The heritability of bilateral deafness was higher than that of unilateral hearing loss. Regularly audiology follow-up for bilateral deafness and deafness gene-positive children was of great significance. The union screening could provide theoretical and practi-cal basis for the three level preventive measures to reduce birth defects.%目的分析非综合征性听力障碍儿童耳聋基因突变情况,从分子水平探究该人群聋病的遗传病因和特点,为早期诊断、治疗及预防先天性和遗传性耳聋提供科学依据.方法采用基质辅助激光解吸电离飞行时间质谱(MALDI-TOF MS)技术,对天津市儿童听力障碍诊治中心诊断的94例非综合征性听力障碍儿童进行常见耳聋基因(GJB2、GJB3、SLC26A4、线粒体12SrRNA)共20个突变位点的检测,并对检测结果及听力学资料进行统计学分析.结果94例研究对象皆为中度、重度及极重度感音神经性耳聋,其中双侧听力下降组73例,单侧听力下降组21例.94例患儿中31例(32.98%,31/94)检出携带耳聋易感基因突变,单基因纯合突变13例,单基因复合杂合突变9例,单杂合突变9例,其中双侧听力下降组耳聋基因阳性率(42.47%)明显高于单侧听力下降组(0.00%)(χ2=13.31,P<0.01).GJB2基因、SLC26A4基因、GJB3基因及12SrRNA的突变检出率分为17.02%、15.96%、0.00%和0.00%.GJB2阳性例数16例,皆位于双侧听力下降组,其中纯合突变8例,复合杂合突变5例,杂合突变3例.SLC26A4(PDS)基因阳性例数15例,皆位于双侧听力下降组,其中纯合突变者5例(皆位于IVS7-2A>G位点),复合杂合突变者4例,杂合突变者6例.双耳听力下降组的GJB2基因阳性检出率(21.92%)高于单耳听力下降组(0.00%)(P<0.05);SLC26A4基因阳性检出率两组间比较无明显差别(20.55%,0.00%)(P>0.05).结论遗传因素在非综合征性耳聋的致聋病因中所占比例较高,双侧聋遗传性高于单侧聋,对于双侧耳聋及耳聋基因阳性患儿定期进行听力学随访意义重大,耳聋基因筛查是对常规听力学筛查的有效补充,可为降低出生缺陷的三级预防措施提供理论和实践依据.
  • 摘要：Objective To explore acoustic characteristics of Tone4+Tone4 change for preschool children with hear-ing impairment. Methods Tone acoustics parameters were measured between 60 normal children and children with hear-ing impairment from age 3 to 5 years old. Results (1)Both hearing impaired and normal children showed F0max in monosyl-labic tone4 was significantly greater than that in former tone4 and latter tone4 (P<0.05, P<0.01),and F0max in former tone4 was significantly greater than that in latter tone4(P<0.01). (2) F0min of hearing impaired children in monosyllabic tone4 and latter tone4 were significantly greater than normal children (P<0.01).There was no significant difference in F0min in former tone4 for both groups of children. (3) F0(max-min) of hearing impaired children was significantly small-er than normal children (P<0.01).F0(max-min) in monosyllabic tone4 was significantly greater than that in former tone4 and latter tone4(P<0.01, P<0.01), and F0(max-min) of tone4 in latter tone4 was significantly greater than that in former tone4(P<0.01).(4) F0k in former tone4 of deaf children was significantly greater than normal chindren(P<0.01). There was no significant difference in F0k in monosyllabic tone4 and latter tone4 for both hearing impaired and normal chil-dren. Conclusions Both hearing impaired children and normal children will change their tone when pronouncing tone4-tone4 words, and exhibited adult-like pitch pattern. However, the tone4-tone4 pronunciation capabilityfor hearing impaired children were far behind from normal children and showed lower F0minin in latter tone4, smaller F0 (max-min) and uneconomic co-articulation.%目的探讨听障儿童普通话双字调"去+去"连读变调的声学特点.方法比较60名听障和60名健听儿童普通话双字调"去+去"连读时基频最大值,最小值,调域,拟合斜率等声学参数,并进行统计分析.结果(1)两类儿童基频最大值F0max单发位置显著大于前去(P<0.05)和后去(P<0.01),且前去的F0max显著大于后去(P<0.01);(2)听障儿童基频最小值F0min在单发和后去位置均显著大于健听儿童(P<0.01),前去位置两类儿童的F0min无显著性差异;(3)听障儿童的调域F0(max-min)显著小于健听儿童(P<0.01),两类儿童调域F0(max-min)单发位置显著大于后去(P<0.01)和前去(P<0.01),且后去的F0(max-min)显著大于前去(P<0.01);(4)听障儿童拟合斜率F0K前去位置显著大于健听儿童(P<0.01),单发和后去位置两类儿童的F0K无显著性差异.结论听障儿童和正常儿童一样,"去+去"连读时会产生连续变调现象,表现出接近于成人去去变调发音的走势.但听障儿童去去连读的能力显著落后于正常儿童,表现出后去终点基频较高、调域偏小和协同发音不经济等现象.
  • 摘要：乳突根治术分为完璧式乳突根治术和开放式乳突根治术.实施完璧式乳突根治术时House与Fisch在骨性外耳道后壁的处理、面神经定位标志的选择、开放面隐窝的方式、鼓室内铺放塑料片等方面各有特点;实施开放式乳突根治术时House与Fisch在术腔大小、面神经定位标志的选择、如何缩小术腔等方面有所不同.%The mastoidectomy is divided into two types of procedures:the canal wall up or complete wall mastoid-ectomy (closed procedure), and canal wall down mastoidectomy (open procedure). In the canal wall up or intact canal wall mastoidectomy, Dr. House's and Dr. Fisch's techniques are different in management of the posterior wall of bony ex-ternal ear canal, selection of facial nerve landmarks, the way of opening the facial recess, placement of a plastic sheet in the tympanic cavity, and so on. In the"open procedure", Dr. House's and Dr. Fisch's techniques are different in the surgi-cal cavity size, selection of facial nerve landmarks, and how to reduce the surgery surgical cavity.
  • 摘要：目的 探讨电刺激听觉脑干诱发电位(electrical evoked auditory brainstem responses,EABR)辅助Mondini畸形患者人工耳蜗植入手术的方法及术后效果分析.方法 我院自2001年开始为Mondini畸形患者植入人工耳蜗,收集2012年至2015年在我院行人工耳蜗手术的Mondini畸形患者31例,回顾性分析Mondini畸形患者术前听力学特征,术前EABR特点,术中手术特点、术后NRT测试结果及开机调试结果,随访术后听觉言语康复能力CAP(categories of auditory performance)和SIR(speech intelligibility rating)评分.另选择相似耳聋年龄的耳蜗结构正常耳蜗植入患者20例作为正常对照组,测试结果进行对比分析.结果31例Mondini畸形患者术前听力均为极重度感音神经性聋,其中14例无残余听力,对照组7例无残余听力,术前行EABR测试,引出率100%,有反应者植入不同类型电极的人工耳蜗;术中电极全部植入耳蜗内,其中4例出现脑脊液井喷,颞肌封堵,术后无面瘫、脑脊液漏、脑膜炎等手术并发症;所有Mondini畸形患者术后均获得听觉,开机测试神经反射遥测(neural response telemetry,NRT),引出率41.94%低于对照组95%;开机6月平均T值Mondini组171.37±13.14CL;对照组145.34±17.43CL;动态范围Mondini组33.24±10.73CL,对照组43.55±15.36CL,经统计学分析P<0.05,有显著差异;随访6月~4年,Mondini组CAP平均得分6.13±1.15,SIR平均得分3.05±0.62;对照组CAP平均得分7.12±0.80,SIR平均得分4.03±0.91,经两样本比较t检验,P<0.05有显著性差异.结论 Mondini畸形的双侧极重度感音神经性聋患者可以根据残余听力情况在EABR辅助下行人工耳蜗植入,术后听力言语康复较正常耳蜗结构患者效果稍差,但明显好于术前听觉和言语能力.%Objective To investigate the hearing and speech rehabilitation outcomes in the cases with Mondi-ni malformation underwent cochlear implantation with preoperative evaluation using electrically evoked auditory brainstem responses (EABR). Methods Thirty-one cases with Mondini malformation underwent cochlear implanta-tion in our hospital from the year 2012 to 2015. The audiologic testing, preoperative EABR, surgical technique, and postoperative neural response telemetry (NRT) results in these cases were analyzed retrospectively. The hearing and speech rehabilitation ability were evaluated using categories of auditory performance (CAP) and speech intelligibili-ty rating (SIR) scores. Another 20 cases of cochlear implantation with normal cochlea served as the normal control group. Results Thirty-one cases with Mondini malformation presented with profound sensorineural hearing loss, 14 cases of which showing no residual hearing. Seven cases in control group showed no residual hearing. All cases demonstrated presence of EABR response preoperatively. The patients with no residual hearing were implanted with different types of electrodes. Four cases had cerebrospinal fluid (CSF) gusher during procedure, which was controlled by plugging with temporaries muscle, and no postoperative complications occurred. All Mondini malfor-mation patients gained hearing postoperatively. The inducible rate of NRT on the boot day was 41.94%which was lower than that in the control group (which was 95%). About 6 month postoperatively the average T value was 171.37 ± 13.14CL in Mondini group versus 145.34 ± 17.43CL in control group (P<0.05); the dynamic range was 33.24 ± 10.73CL in Mondini group versus 43.55 ± 15.36CL in control group (P<0.05). Postoperative follow-up from 6 months to 4 years show that the Mondini group has the average CAP scores of 6.13±1.15 (7.12±0.80 in con-trol) and SIR scores of 3.05±0.62 (4.03±0.91 in control),with significant difference (P<0.05) compared to the con-trol group. Conclusions Cochlea implantation can be performed in patients with bilateral profound hearing loss in Mondini malformation, and even for those without residual hearing, if preoperative EABR is present. Significantly improved hearing and speech were achieved, though the postoperative hearing and speech rehabilitation outcomes were poorer in Mondini malformation than that with normal cochlear structure.
  • 摘要：2015年WHO国际疾病分类ICD-11 beta草案已将持续性姿势-知觉性头晕(persistent postural-percep?tion dizziness PPPD)列入.它是一种临床常见的头晕形式,伴有持续非旋转性头晕或主观不稳感,且在直立姿态、个人运动、环境中物体运动以及复杂视觉刺激的条件下加重.PPPD的发病机制目前尚未完全阐明,可能的假说有:①经典和操作性条件反射建立假说②再适应失败假说.治疗包括药物治疗、前庭平衡康复训练和认知行为治疗.了解PPPD有助于头晕的诊断和鉴别.本文对PPPD的研究进展进行综述.%Persistent Postural-perception Dizziness (PPPD) has been included by the WHO 11th beta draft of Inter-national Classification of Diseases (ICD) in 2015. It is a clinically common form of dizziness, accompanied by persis-tent non-rotational dizziness or subjective sense of instability lasting 3 months or more. It could be exacerbated in the upright posture, movement of the person, movement of the environmental objects, and complex visual stimulation condi-tions. The pathogenesis of PPPD is not yet fully elucidated. The possible hypotheses are:①Classic and operational con-ditioned reflex hypothesis;②Failure of Re-adaptation. Treatment includes medications,vestibular rehabilitation therapy and cognitive behavioral therapy. This article reviewed the progress in recent research on PPPD.
  • 摘要：Objective To explore the effect of early rehabilitation of cognitive function after cochlear implantation in prelingually older children and teenagers. Methods The categories of auditory performance (CAP), speech intelligibil-ity rating (SIR) and the electronic cognitive assessment system (E-CAS) were used to evaluate twenty prelingually heavy-profound hearing loss patients'auditory, speech performances and cognitive function. The rehabilitation of cogni-tive function was analyzed after cochlear implantation. Results The scores of CAP、SIR and E-CAS in different ages groups were all increased with time after cochlear implantation. Significance differences in the older childrens'simulta-neous processing capacity (P=0.02), attention capacity (P=0.03) and in the teenagers'attention capacity (P=0.00) were observed at the end of the 6th month after cochlear implantation. Significance differences in planning processing capaci-ty of two groups at the end of 1 year (P<0.001). But normal people's score is still higher than patients with cochlear im-plantation (P<0.001). Conclusion Although auditory,language ability and cognitive function are found to be enhanced after cochlear implantation,but the overall cognitive level is still lower than normal children. The restore of cognitive funtion needs a longer time. E-CAS is a useful instrument to evaluate the effect of rehabilitation of cognitive functionin children after CI.%目的 探讨低频型突聋伴耳闷胀感患者耳闷胀感的临床特征、预后及与突聋的关系.方法 收集单侧低频型突聋伴耳闷胀感患者35例,不伴耳闷胀感患者32例,进行听力学及影像学检查,对伴耳闷胀感患者进行耳闷胀感的VAS量表评分及主观分级.比较两组患者耳声发射、听性脑干诱发电位改变;观察耳闷胀感预后,分析耳闷胀感与突聋的关系.结果 两组患者间耳声发射及脑干诱发电位结果 无显著差异(χ2=1.486,1.029;P>0.05).伴耳闷胀感患者耳闷胀感主观分级与听力下降程度呈正相关(r=0.344,P<0.05).伴耳闷胀感组经治疗后听力恢复总有效率32/35(91.4%)略高于不伴耳闷胀感组总有效率27/32(84.4%),差异无统计学意义(χ2=0.791,P>0.05).耳闷胀感恢复总有效率为31/35(88.6%),耳闷改善情况与听力下降改善情况存在相关性(χ2=9.891,P<0.05),pearson列联相关系数r=0.469,P<0.05.结论 低频型突聋伴耳闷胀感患者耳闷程度与听力下降程度相关,听力损失越严重,耳闷胀感的主观分级越重;耳闷胀感预后较好,与听力恢复情况相关;伴耳闷胀感患者听力恢复效果可能略好于不伴耳闷胀感患者,有待进一步观察.
  • 摘要：The stria vascularis and spiral ligament are located in the lateral wall of the cochlea. The blood-laby-rinth barrier (BLB) of the stria vascularis is a well-differentiated capillary network that regulates exchanges between the blood and interstitial fluid in the cochlea. This barrier protects the inner ear from toxic substances that are produced by blood and selectively allows fluids, ions and nutrients into the cochlea. The BLB plays an important role in maintaining cochlear homeostasis. The BLB includes vascular endothelial cells (ECs), basement membrane (BM), elaborated tight and adherens junctions, pericytes (PCs), and perivascular resident macrophage-like melanocytes (PVM/Ms). Interac-tions between ECs, PVM/Ms and PCs, similar to intercellular signaling, play a vital role in controlling vascular permea-bility and providing a suitable environment for hearing function. Breakdown of normal interactions between compo-nents of the BLB participates in genetic defects, inflammation, acoustic injury and aging.%血管纹和螺旋韧带位于耳蜗中阶外侧壁,其中血管纹的血-迷路屏障(blood-labyrinth barrier,BLB)是高度分化的毛细血管网,用于调控耳蜗血液和细胞间液的物质交换.此屏障保护内耳不接触来自血液的有毒物质,并且选择性的透过离子、液体及营养物质至耳蜗.血-迷路屏障对维持耳蜗内稳态有重要的作用.血迷路屏障结构上包括血管纹微血管内皮细胞(endothelial cells,ECs)、周细胞(pericytes,PCs)、血管周围巨噬细胞样黑色素细胞(perivascular resident macrophage-like melanocytes,PVM/Ms)、基膜(basement membrance,BM)、复杂的紧密连接和黏合连接.ECs、PCs和PVM/Ms间的作用,类似于细胞间信号传导,对控制血管渗透性及为听功能提供适宜的环境起着至关重要的作用.在遗传缺陷、炎症、声损伤以及衰老的病理条件下,血-迷路屏障各组份间正常的相互作用遭到破坏,进而导致其通透性增加,引发听力障碍.
  • 摘要：目的 探讨中药耳净散治疗慢性化脓性中耳炎的给药方式、给药时长和临床疗效.方法 选取2014年3月~2016年1月期间我院治疗的180例(192耳)慢性化脓性中耳炎患者,随机分为治疗组和对照组,每组90例患者.治疗组采用复方耳净散喷敷治疗,对照组使用氧氟沙星滴耳液滴耳治疗,比较两组患者治疗不同时长的治愈率和有效率.结果 耳净散治疗组和氧氟沙星滴耳液治疗组的单次治疗(2周)流脓症状控制率分别为87.6%和58.9%,两组比较差异有统计学意义(P<0.05).结论 耳内镜引导下中药耳净散治疗慢性化脓性中耳炎活动期的效果显著,干性治疗比湿性治疗在治疗时长上有明显优势,且安全性好.%Objective To report therapeutic effects of a Chinese herbal preparation–Er Jing San, in relation to ad-ministration modes and duration, in treating active chronic suppurative otitis media. Methods A sample of 180 cases (192 ears) of chronic suppurative otitis media treated at Beijing Chinese Medicine Hospital from March 2014 to January 2015 were randomly divided into a treatment and a control group, with 90 cases in each group. Cases in the treatment group were treated with Er Jing San, while those in the control group were treated with Ofloxacin ear drops. The rate of complete disease resolution and improvement at different treatment time points were compared between the two groups. Results The overall rate of effectiveness was 87.6% in the treatment group and 58.9% in the control group (P<0.05). Conclusions Er Jing San appears to yield treatment outcomes superior over Ofloxacin ear drops in treating active chron-ic suppurative otitis media. Treatment using dry powder preparation seems to hold obvious advantages with improved safety as compared to treatment using fluid ear drops.

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