Congenital malformation of the middle and outer ear (CMMOE)

  • 文章类型: Review
    没有关于中耳和外耳先天性畸形(CMMOE)的各种听力手术解决方案后效果的综合比较分析的报道。
    分析CMMOE各种听力手术解决方案后纯音平均空气传导阈值(AACT)的改善情况,为选择准确的听力解决方案提供参考。
    对接受各种耳部手术方案的159例(170耳)CMMOE患者进行回顾性分析,包括:(1)外耳道(OEC)的三种情况:①闭锁85耳,②狭窄28耳,③正常57耳。(2)三种常用的听力解决方案:耳膜修复53耳,门44耳和活塞32耳植入。(3)具有不同听力解决方案的三种OEC情况:类型I.OEC的重建(r-OEC),II型。r-OEC和/或不同的鼓室成形术,包括①耳膜修复,②听骨链释放,③口交植入,和④Torp植入,III型。内耳开窗的活塞植入。比较术后短期(0.5年)或长期(0.5-10年)和术前在0.5-4kHz语音频率范围内的AACT以评估疗效。如果样本数≥10,并且不服从正态分布,Kruskal-Wallis多样本秩和检验用于多组的比较,Wilcoxon秩和检验用于两组,P<0.05有统计学意义。如果样本量<10,临床疗效的标准是一个频率改善值≥15dBHL,或10dBHL≤2频率改善<15dBHL在0.125-8KHz。
    AACT的组内比较:(1)OEC的三种情况:闭锁,术后短期内狭窄和正常均P<0.05,而长期仅正常组P<0.05。(2)三种常用的听力解决方案:耳膜修复,Porp和Piston植入在短期和长期均P<0.05,除了耳膜修复P>0.05长期。(3)三种具有不同听力解决方案的OEC情况:1)OEC的闭锁:Porp和活塞植入,r-OEC和听骨链释放短期有效,长期无效,耳膜修复在短期和长期都无效。2)OEC狭窄:耳膜修复,Porp和Piston植入在短期和长期均有效。短期和长期R-OECP>0.05,Torp植入长期无效,3)OEC的正常:Porp,Torp和活塞植入在短期和长期均P<0.05,除了长期Torp>0.05。听骨链的释放是短期和长期临床有效。三组闭锁术后长期AACT值,狭窄,OEC的正常值超过58.7dBHL(除了Porp植入52.5dBHL),51.3dBHL(Porp植入42.5dBHL除外),和37.5dBHL(除了Torp植入是32.6dBHL),分别。
    AACT的组内比较。(1)三组闭锁,OEC的狭窄和正常都是短期有效的,而从长远来看,只有正常组是有效的。(2)三种最常用的耳膜修复手术方案,波特和活塞植入在短期和长期都是有效的,除了长期的耳膜修复。(3)三种不同听力解决方案的OEC情况:某些手术解决方案对CMMOE短期或长期有效,但是根据三种OEC情况的术后长期AACT值,最好选择用于OEC闭锁的听力设备,OEC狭窄的手术或听力设备的综合审查。对于正常的OEC可以考虑手术。
    UNASSIGNED: There are no reports about comprehensive comparative analysis of the effects after various hearing surgery solutions for congenital malformation of the middle and outer ear (CMMOE).
    UNASSIGNED: To analyze the improvement of Average Air-Conduction Threshold (AACT) of pure tone after various hearing surgery solutions for CMMOE and provide a reference for the selection of accurate hearing solutions.
    UNASSIGNED: A retrospective analysis of 159 cases (170 ears) with CMMOE submitted to various ear surgery solutions, including: (1) Three situations of outer ear canal (OEC): ① atresia 85 ears, ② stenosis 28 ears, and ③ normal 57 ears. (2) Three commonly used hearing solutions: eardrum repair 53 ears, Porp 44 ears and Piston 32 ears implantation. (3) Three OEC situations with different hearing solutions: type I. Reconstruction of OEC (r-OEC), type II. r-OEC and/or different tympanoplasty, including ① eardrum repair, ② release of ossicular chain, ③ Porp implantation, and ④ Torp implantation, type III. Piston implantation with fenestration of the inner ear. Compare AACT of postoperative short term (0.5 years) or long term (0.5-10 years) and preoperative in the speech frequency range of 0.5-4 kHz to assess efficacy. If the sample number ≥10, and not subject to normal distribution, the Kruskal-Wallis multi-sample rank sum test is used for the comparison of multiple groups and Wilcoxon\'s rank sum test for two groups, with P < 0.05 being statistically significant. If the sample size <10, the standard of clinical efficacy is one frequency improvement value ≥15 dB HL, or 10 dB HL ≤2 frequency improvements <15 dB HL at 0.125-8 KHz.
    UNASSIGNED: Intra-group comparison of AACT: (1) three situations of OEC: atresia, stenosis and normal all had P < 0.05 postoperatively in short term, while in long term only the normal group had P < 0.05. (2) Three commonly used hearing solutions: eardrum repair, Porp and Piston implantation all had P < 0.05 in short and long terms, except for eardrum repair P >0 .05 in long term. (3) Three OEC situations with different hearing solutions: 1) Atresia of OEC: Porp and Piston implantation, r-OEC and release of ossicular chain were effective in short term and were not effective in long term, and the eardrum repair was not effective in both short and long term. 2) Stenosis of OEC: eardrum repair, Porp and Piston implantation were effective in short and long term. r-OEC P >0 .05 for short and long term, Torp implantation was not effective in long term, 3) Normal of OEC: Porp, Torp and Piston implantation were all P < 0.05 in short and long term except for Torp >0.05 in long term, and release of ossicular chain is both short and long term clinically effective. The AACT values of postoperative in long term for three groups of atresia, stenosis, normal of OEC are over 58.7 dB HL (except Porp implantation 52.5 dB HL), 51.3 dB HL (except Porp implantation 42.5 dB HL), and 37.5 dB HL (except Torp implantation are 32.6 dB HL), respectively.
    UNASSIGNED: Intra-group comparison of AACT. (1) Three groups of the atresia, stenosis and normal of OEC are all effective in short term, while in long term only the normal group is effective. (2) The three most commonly used surgical solutions of eardrum repair, Porp and Piston implantation are effective in short and long terms, except for long term eardrum repair. (3) Three OEC situations with different hearing solutions: some of surgical solutions were effective in short term or long term for CMMOE, but based on the AACT values of postoperative in long term for three OEC situations, it is better to choose a hearing device for atresia of OEC, comprehensive review of surgical or hearing device for stenosis of OEC. Surgery can be considered for normal OEC.
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  • 文章类型: Journal Article
    目前,世界上没有关于先天性耳畸形(CEM)的国际统一标准和报告,这使得信息传递和文献比较变得困难。
    通过对大量CEM样本的统计分析,提出了CEM各方面的统一标准,并提供数据供参考,便于该领域的国际工作和文献比较。
    根据作者30年对CEM的临床和科研工作及我院3231(4714耳)的相关病例,结合文学,进行统计分析。
    本文总结了分类,定义,流行病学,胚胎发育,CEM的致病因素,并对其临床表现进行了阐述,中耳和外耳(CMMOE)的代表性先天性畸形的检查和顺序治疗。我们还介绍了耳廓和内耳的畸形,以便覆盖外部,中耳和内耳。同时,我们介绍我们在这一领域的成就和贡献。
    本研究为CEM的国际统一标准和处理原则提供了参考。
    UNASSIGNED: At present, there are not international unified standards and reports on Congenital Ear Malformation (CEM) in the world, which makes it difficult to transfer information and compare the literature.
    UNASSIGNED: Through the statistical analysis of a large sample of CEM, a unified standard of all aspects of CEM is proposed and the data are provided for reference, which is convenient for the international work and literature comparison in this field.
    UNASSIGNED: Based on the author\'s 30 years of clinical and scientific research work on CEM and the relevant cases of 3231 (4714 ears) in our hospital, and combined with literature, statistical analysis was made.
    UNASSIGNED: This paper summarizes the classification, definition, epidemiology, embryonic development, pathogenic factors of CEM and elaborates on the clinical manifestations, examination and sequence therapy of representative Congenital Malformation of the Middle and Outer Ear (CMMOE). We also introduce malformation of the auricle and inner ear, so as to cover the outer, middle and inner ear. At the same time, we introduce our achievements and contributions in this field.
    UNASSIGNED: This study provides reference to the international unified standard and treatment principle of the CEM.
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  • 文章类型: Journal Article
    在纯音测听法中,当双耳之间的纯音平均空气传导阈值(AACT)的差异超过40dB时,必须在较差的一侧进行HL掩蔽,然而,我们发现,在某些患者中,当双耳AACT差异(AACT-d)小于40dBHL时,掩蔽也有显著性差异.
    评估不同类型耳聋患者纯音测听中掩蔽对不良耳朵的意义,以获得术前准确的听力。
    对163例(163耳)两耳听力差异的病例进行了比较分析,分为三组:G1先天性中外耳畸形(CMMOE)作为传导性耳聋,63耳,G2突发性耳聋作为感音神经性耳聋,65耳,G3中耳炎为传导性或混合性耳聋,35耳AACT-d在以下三种情况下对不良耳罩前后进行了分析:(1)每个频率为0.125-8KHz,(2)平均0.5-4KHz,(3)掩蔽前两耳之间的AACT-d≥40dBHL和<40dBHL的频率。如果样本数据不服从正态分布,Wilcoxon秩和检验用于比较AACT,并且p<0.05被认为具有统计学意义。在1个频率下AACT-d≥15dBHL或在2个频率下10dBHL≤AACT-d在掩蔽前后均有效。
    在三组中,(1)平均每个频率为0.125-8KHz和0.5-4KHz的不良耳罩前后的AACT-d比较,均p<0.05,G1组的AACT-d最大,平均0.5-4KHz为7.5dBHL,前两个分别是0.125KHz和0.25KHz的14.5dBHL和13.8dBHL,分别。(2)AACT-d≥40dBHL和<40dBHL在掩蔽前的两个耳朵之间分布在0.125-8KHz的全频率上,≥40dBHL组的临床有效率为G1(89.3%),G2(45.5%)和G3(5.3%),而<40dBHL组的G1(69.7%),G2(34.4%)和G3(31.3%),分别。
    对于所有三个组,在每个频率0.125-8KHz和平均0.5-4KHz的不良耳罩之前和之后,AACT-d具有统计学意义。在整个0.125-8KHz的整个频率范围内,观察到掩蔽前两耳之间AACT-d≥40dBHL和<40dBHL的分布。耳罩不良前后的AACT-d在所有三组中均显示出临床有效性,G1组有效率最高,AACT-d在0.125KHz和0.25KHz时最高。因此,无论掩蔽前两耳之间的AACT-d是≥40dBHL还是<40dBHL,全频掩蔽应分为三组,特别是对于CMMOE的G1组,特别是在0.125KHz和0.25KHz。
    UNASSIGNED: In pure tone audiometry, when the difference of the Average Air Conduction Threshold of pure tone (AACT) between bilateral ears is more than 40 dB HL masking must be performed on the poor side, However, we found that masking also make significance difference when the binaural AACT difference (AACT-d)was less than 40 dB HL in some patients.
    UNASSIGNED: Assessing the significance of masking for the poor ear in pure tone audiometry in patients with different types of deafness to obtain preoperative accurate hearing.
    UNASSIGNED: A comparative analysis of 163 cases (163 ears) with hearing difference between two ears was conducted, who were divided into three groups: G1 Congenital Malformation of the Middle and Outer Ear (CMMOE)as conductive deafness, 63 ears, G2 sudden deafness as sensorineural deafness, 65 ears, and G3 media otitis as conductive or mixed deafness,35 ears. AACT-d before and after the poor ear masking was analyzed under the following three conditions: (1) 0.125-8 KHz each frequency, (2) 0.5-4 KHz on average, (3) the frequencies of AACT-d ≥ 40 dB HL and <40 dB HL between the two ears before masking. If the sample data did not follow a normal distribution, the Wilcoxon rank sum test was used for comparasion of AACT, and p < 0.05 was considered statistically significant. It is clinically effective for AACT-d ≥ 15 dB HL at 1 frequency or 10 dB HL ≤ AACT-d at 2 frequencies <15 dB HL before and after masking.
    UNASSIGNED: Among the three groups, (1) the comparasion of AACT-d before and after the poor ear masking for each frequency of 0.125-8 KHz and 0.5-4 KHz on average with all p < 0.05, and the AACT-d of the G1 group was the largest, with an average 0.5-4KHz of 7.5 dB HL, and the first two were 14.5 dB HL and 13.8 dB HL at 0.125 KHz and 0.25 KHz, respectively. (2) AACT-d ≥ 40 dB HL and <40 dB HL between the two ears before masking were distributed at the full frequency of 0.125-8KHz, the clinically effective rates of ≥40 dB HL groups were G1 (89.3%), G2 (45.5%) and G3 (5.3%), while those of < 40 dB HL groups were G1 (69.7%), G2 (34.4%) and G3 (31.3%), respectively.
    UNASSIGNED: For all three groups, there was statistically significant in AACT-d before and after the poor ear masking across each frequency of 0.125-8 KHz and on average 0.5-4 KHz. The distribution of AACT-d ≥ 40 dB HL and <40 dB HL between the two ears before masking was observed throughout the full frequency range of 0.125-8 KHz. AACT-d before and after the poor ear masking showed clinical effectiveness in all three groups, with the highest effective rate observed in the G1 group and the highest AACT-d at 0.125 KHz and 0.25 KHz. Therefore, regardless of whether the AACT-d between the two ears before masking is ≥40 dB HL or <40 dB HL, the full frequency masking should be employed in three groups, especially for the G1 group of CMMOE, particularly at 0.125 KHz and 0.25 KHz.
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  • 文章类型: Journal Article
    在先天性中耳和外耳畸形伴感染(CMMOEI)的患者中,结合清创感染病灶和保留带血皮瓣进行耳廓重建是具有挑战性的临床问题。
    创新并引入手术切口,以解决CMMOEI患者合并感染清创的临床难题,同时保留血管化良好的皮瓣进行耳廓重建。
    23例CMMOEI采用耳沟和预制耳垂组合切口,成功地管理了所遇到的感染,并回顾了创新切口的短期和中期结果,以保留血管化良好的皮瓣用于随后的耳廓重建。这23例病例包括10名男性和13名女性,4-14岁(平均8.4岁),7只左耳和16只右耳。14耳狭窄,9耳外耳道闭锁。
    在所有23种情况下,2年随访,成功清除感染,无复发或并发症.局部皮瓣及其血供保存完好,可用于后续耳廓重建。
    新切口可以促进CMOMEI患者感染的清除,并保留耳后组织,以便随后收获血管化良好的皮瓣,以进行随后的耳廓重建。
    UNASSIGNED: It is the challenging clinical issue of combining debridement of infected lesions and retaining the blood skin flap for auricle reconstruction in patients of Congenital Malformation of the Middle and Outer Ear with Infection (CMMOEI).
    UNASSIGNED: To innovate and introduce an surgery incision to solve the challenging clinical issue of a combined debridement of infection yet retaining a well vascularized skin flap for auricle reconstruction in patients with CMMOEI.
    UNASSIGNED: A combined innovated incision of ear sulcus and prefabricated earlobe was used for 23 cases (23 ears) with CMMOEI, The success in the management of the encountered infections, and the short and moderate-term outcome of the innovated incision to preserve a well-vascularized skin flap for subsequent auricle reconstruction were reviewed. The 23 cases include 10 males and 13 females, aged 4-14 years (mean 8.4 years), 7 left ears and 16 right ears. 14 ears stenosis and 9 ears atresia of the outer ear canals.
    UNASSIGNED: In all 23 cases, the infections were successfully cleared without recurrence or complication with 2-year follow-up. The local skin flap and its blood supply were well preserved for subsequent auricle reconstruction.
    UNASSIGNED: The new incision can facilitate clearance of infection in CMOMEI patients, and preserve the retroauricular tissues for subsequent harvesting of a well-vascularized skin flap for subsequent auricle reconstruction.
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  • 文章类型: Journal Article
    背景:先天性中外耳畸形(CMMOE)的术前评估非常重要。目前常用的Jahrsdoerfer评分,基于颞骨的CT扫描图像,通常无法准确评估畸形和听力水平。目的/目标:调查和推广一种简单易懂的评估方法,纯音测听法,用于CMMOE的评估。材料与方法:回顾性分析223例(244耳)CMMOE患者的听力资料。其中,180例(197耳)行探查性鼓室成形术,条件明确:听骨数136例(147耳),形态128例(138耳),前庭窗发育137例(146耳),113例(120耳)颞骨CT扫描。1).分析小骨数量之间的相关性,小骨形态,Jahrsdoerfer评分组及其相应的0.5-4KHz纯音平均空气传导阈值(AACT)。2)分别比较上述各组之间以及前庭窗以0.5-4KHz和0.125-8KHz的频率在发育和未发育组之间的AACT差异。采用Spearman法进行相关分析(计算系数r和p值)。对于服从正态分布的数据,采用单向方差分析(ANOVA)和t检验,否则,使用KruskalWallis多重局部秩符合检验和Wilcoxon秩和检验。p<0.05被认为具有统计学意义。结果:1)各组间听骨数得分的相关系数,小骨形态学评分,Jahrsdoerfer得分及其相应的AACT为r=-0.187(p<0.05),r=-0.073(p>0.05)和r=-0.079(p>0.05),分别。2)各组间基于小骨数或形态学评分和Jahrsdoerfer评分的AACT差异比较,p>0.05,分别。在0.5-4KHz时,已开发和未开发的前庭窗组之间的AACT差异为5.5(63.5/69.0)dBHL(p<.05),在0.125-8KHz频率中,1、2、4KHz为5.7(65.0/70.7)dBHL,8.4(60.7/69.1)dBHL和2(61.5/63.5)dBHL,分别,所有p<0.05,其他频率均p>0.05。结论及意义:1)卵囊数与AACT相关,但不是小骨形态和Jahrsdoerfer评分。2)听骨数量或形态评分与Jahrsdoerfer评分组对应的AACT无显著差异,但是前庭窗不发达的患者听力比前庭窗发达的患者差。因此,AACT可以评估听骨和前庭窗的发育,比Jahrsdoerfer评分更直接反映听力水平。纯音测听法很简单,广泛使用,并且易于访问,使其成为CMMOE的一种新的评估方法。
    Background: The preoperative evaluation of Congenital Malformation of the Middle and Outer Ear (CMMOE) is very important. Jahrsdoerfer score commonly used at present, based on CT scanning images of the temporal bone, is often unable to accurately evaluate deformity and hearing level.Aims/Objectives: To investigate and promote a straightforward and easily accessible assessment method, pure tone audiometry, for the evaluation of CMMOE.Material and Methods: A total of 223 cases (244 ears) CMMOE with hearing data were retrospectively analyzed. Among them, 180 cases (197 ears) underwent exploratory tympanoplasty with clear conditions: ossicle numbers in 136 cases (147 ears) and morphology in 128 cases (138 ears) and vestibular window development in 137 cases (146 ears), and CT scans of temporal bone in 113 cases (120 ears). 1). The correlation was analyzed between ossicle numbers, ossicle morphology, Jahrsdoerfer score groups and their corresponding Average Air-Conduction Threshold of pure tone (AACT) at 0.5-4 KHz. 2) The AACT difference is compared among the above groups respectively and between the developed and undeveloped groups of vestibular window at 0.5-4 KHz and each frequency of 0.125-8 KHz. Spearman method was used for correlation analysis (calculating coefficient r and p values). For the data followed a normal distribution, a one-way analysis of variance (ANOVA) and t-test were employed, otherwise, Kruskal Wallis multiple local rank coincidence test and Wilcoxon rank sum test were used. p <0 .05 was considered statistically significant.Results: 1) The correlation coefficients between the groups of ossicle number scores, ossicle morphology scores, Jahrsdoerfer scores and their corresponding AACT are r = -0.187 (p <0 .05), r = -0.073 (p >0 .05) and r = -0.079 (p > 0.05), respectively. 2) Comparison of AACT difference based on ossicle number or morphological scores and Jahrsdoerfer scores with p > 0.05 among all groups, respectively. The AACT difference between the developed and undeveloped vestibular window groups is 5.5 (63.5/69.0) dB HL(p < .05) at 0.5-4KHz, out of 0.125-8 KHz frequency 1, 2, 4 KHz were 5.7 (65.0/70.7) dB HL, 8.4 (60.7/69.1) dB HL and 2 (61.5/63.5) dB HL, respectively, all p < 0.05, the other frequencies with all p > 0.05.Conclusions and Significance: 1) Ossicle number was correlated with AACT, but not for ossicle morphology and Jahrsdoerfer scores. 2) There was no significant difference in AACT corresponding to ossicle number or morphology scores and Jahrsdoerfer scores groups, but the patients with undeveloped vestibular window had poorer hearing than those with developed ones. Therefore, the AACT can evaluate the development of ossicle and vestibular window, and more directly reflect the hearing level than Jahrsdoerfer score. Pure tone audiometry is simple, widely used, and easily accessible, which making it a new assessment method of CMMOE.
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