Middle ear disease

中耳疾病
  • 文章类型: Journal Article
    背景:人工智能(AI)的集成,特别是深度学习模型,改变了医疗技术的格局,特别是在使用成像和生理数据的诊断领域。在耳鼻喉科,AI在中耳疾病的图像分类中显示出希望。然而,现有的模型通常缺乏患者特定的数据和临床背景,限制其普遍适用性。GPT-4Vision(GPT-4V)的出现使得多模态诊断方法成为可能,将语言处理与图像分析相结合。
    目的:在本研究中,我们通过整合患者特异性数据和耳镜下鼓膜图像,研究了GPT-4V在诊断中耳疾病中的有效性.
    方法:本研究的设计分为两个阶段:(1)建立具有适当提示的模型和(2)验证最佳提示模型对图像进行分类的能力。总的来说,305个中耳疾病的耳镜图像(急性中耳炎,中耳胆脂瘤,慢性中耳炎,和渗出性中耳炎)来自2010年4月至2023年12月期间访问新州大学或济池医科大学的患者。使用提示和患者数据建立优化的GPT-4V设置,并使用最佳提示创建的模型来验证GPT-4V在190张图像上的诊断准确性。为了比较GPT-4V与医生的诊断准确性,30名临床医生完成了由190张图像组成的基于网络的问卷。
    结果:多模态人工智能方法实现了82.1%的准确率,优于认证儿科医生的70.6%,但落后于耳鼻喉科医生的95%以上。该模型对急性中耳炎的疾病特异性准确率为89.2%,76.5%为慢性中耳炎,79.3%为中耳胆脂瘤,渗出性中耳炎占85.7%,这突出了对疾病特异性优化的需求。与医生的比较显示了有希望的结果,提示GPT-4V增强临床决策的潜力。
    结论:尽管有其优势,必须解决数据隐私和道德考虑等挑战。总的来说,这项研究强调了多模式AI在提高诊断准确性和改善耳鼻喉科患者护理方面的潜力.需要进一步的研究以在不同的临床环境中优化和验证这种方法。
    The integration of artificial intelligence (AI), particularly deep learning models, has transformed the landscape of medical technology, especially in the field of diagnosis using imaging and physiological data. In otolaryngology, AI has shown promise in image classification for middle ear diseases. However, existing models often lack patient-specific data and clinical context, limiting their universal applicability. The emergence of GPT-4 Vision (GPT-4V) has enabled a multimodal diagnostic approach, integrating language processing with image analysis.
    In this study, we investigated the effectiveness of GPT-4V in diagnosing middle ear diseases by integrating patient-specific data with otoscopic images of the tympanic membrane.
    The design of this study was divided into two phases: (1) establishing a model with appropriate prompts and (2) validating the ability of the optimal prompt model to classify images. In total, 305 otoscopic images of 4 middle ear diseases (acute otitis media, middle ear cholesteatoma, chronic otitis media, and otitis media with effusion) were obtained from patients who visited Shinshu University or Jichi Medical University between April 2010 and December 2023. The optimized GPT-4V settings were established using prompts and patients\' data, and the model created with the optimal prompt was used to verify the diagnostic accuracy of GPT-4V on 190 images. To compare the diagnostic accuracy of GPT-4V with that of physicians, 30 clinicians completed a web-based questionnaire consisting of 190 images.
    The multimodal AI approach achieved an accuracy of 82.1%, which is superior to that of certified pediatricians at 70.6%, but trailing behind that of otolaryngologists at more than 95%. The model\'s disease-specific accuracy rates were 89.2% for acute otitis media, 76.5% for chronic otitis media, 79.3% for middle ear cholesteatoma, and 85.7% for otitis media with effusion, which highlights the need for disease-specific optimization. Comparisons with physicians revealed promising results, suggesting the potential of GPT-4V to augment clinical decision-making.
    Despite its advantages, challenges such as data privacy and ethical considerations must be addressed. Overall, this study underscores the potential of multimodal AI for enhancing diagnostic accuracy and improving patient care in otolaryngology. Further research is warranted to optimize and validate this approach in diverse clinical settings.
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  • 文章类型: Journal Article
    主要目的是确定与未消除相同区域相比,在管壁(CWU)胆脂瘤手术期间消除鼓膜区和乳突腔是否会降低胆脂瘤的复发率和残留率。次要目标是比较两种技术之间的术后听力结果。
    在三级转诊中心进行了一项回顾性队列研究。2015年1月至2020年3月在乌得勒支大学医学中心接受了CWU鼓膜乳状癌切除术治疗胆脂瘤伴或不伴骨闭塞的患者(≥18y),共纳入143只耳朵。中位随访时间分别为1.4(IQR1.1-2.2)和2.0年(IQR1.2-3.1)(p=0.013)。
    所有患者均接受CWU鼓膜乳状核切除术治疗胆脂瘤。对于73耳骨尘,Bonalive®或组合用于消除乳突和鼓膜上区域,其余的耳朵(n=70)没有消失。根据荷兰议定书,纳入的患者计划进行MRI扫描和弥散加权成像(DWI),手术后3年和5年,以检测复发或残留的胆脂瘤。
    主要结局指标是通过MRI-DWI和/或显微耳镜检查评估并通过显微耳镜检查和/或翻修手术确认的复发性和残余胆脂瘤。次要结果指标是术后听力。
    在此队列中,该组采用鼓膜切除术后的管壁切除骨性闭塞术(73耳,51.0%)的胆脂瘤复发(4.1%)和残留(6.8%)率显著低于无闭塞组(70耳,25.7%和20.0%,分别为;p<0.001)。两组术后骨传导阈值无显著差异(平均差异2.7dB,p=0.221)以及手术后6周的平均气-骨间隙闭合(非闭塞组为2.3dB,闭塞组为1.5dB,p=0.903)。
    根据我们的结果,管壁鼓膜切除术与骨闭塞是治疗的选择,因为与无闭塞组相比,复发和残留病率较低。骨消失技术似乎不会影响感知或传导的听力结果,因为这两组之间是相似的。
    UNASSIGNED: The primary objective was to determine whether obliteration of the epitympanic area and mastoid cavity during canal wall up (CWU) cholesteatoma surgery reduces the rate of recurrent and residual cholesteatoma compared to not obliterating the same area. The secondary objective was to compare postoperative hearing outcomes between both techniques.
    UNASSIGNED: A retrospective cohort study was conducted in a tertiary referral center. One-hundred-fourty-three ears were included of patients (≥18y) who underwent a CWU tympanomastoidectomy for cholesteatoma with or without bony obliteration between January 2015 and March 2020 in the University Medical Center Utrecht. The median follow-up was respectively 1.4 (IQR 1.1-2.2) vs. 2.0 years (IQR 1.2-3.1) (p = 0.013).
    UNASSIGNED: All patients underwent CWU tympanomastoidectomy for cholesteatoma. For 73 ears bone dust, Bonalive® or a combination was used for obliteration of the mastoid and epitympanic area, the rest of the ears (n = 70) were not obliterated. In accordance with the Dutch protocol, included patients are planned to undergo an MRI scan with diffusion-weighted imaging (DWI) one, three and five years after surgery to detect recurrent or residual cholesteatoma.
    UNASSIGNED: The primary outcome measure was recurrent and residual cholesteatoma as evaluated by MRI-DWI and/or micro-otoscopy and confirmed by micro-otoscopy and/or revision surgery. The secondary outcome measure was the postoperative hearing.
    UNASSIGNED: In this cohort, the group treated with canal wall up tympanomastoidectomy with subsequent bony obliteration (73 ears, 51.0%) had significantly lower recurrent (4.1%) and residual (6.8%) cholesteatoma rates than the group without obliteration (70 ears, 25.7% and 20.0%, respectively; p < 0.001). There was no significant difference between both groups in postoperative bone conduction thresholds (mean difference 2.7 dB, p = 0.221) as well as the mean air-bone gap closure 6 weeks after surgery (2.3 dB in the non-obliteration and 1.5 dB in the obliteration group, p = 0.903).
    UNASSIGNED: Based on our results, a canal wall up tympanomastoidectomy with bony obliteration is the treatment of choice, since the recurrent and residual disease rate is lower compared to the group without obliteration. The bony obliteration technique does not seem to affect the perceptive or conductive hearing results, as these are similar between both groups.
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  • 文章类型: Case Reports
    肺脑被定义为颅内间隙中存在气体或空气,通常是由于神经创伤引起的。临床上,气颅最常表现为无症状,但可能引起头痛,恶心,呕吐,和困惑。乳突炎引起的气颅是一种不可预见的并发症,仅报道了少数病例。我们报告了一例老年男性,在糜烂性乳突炎伴气颅的情况下出现中风样症状。
    Pneumocephalus is defined as the presence of gas or air in the intracranial space and typically arises as a result of neurotrauma. Clinically, pneumocephalus most often presents asymptomatically but may cause headache, nausea, vomiting, and confusion. Pneumocephalus arising from mastoiditis is an unforeseen complication with only a handful of cases reported. We report a case of an elderly male who presented with stroke-like symptoms in the setting of erosive mastoiditis with pneumocephalus.
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  • 文章类型: Case Reports
    我们提供了一例中耳骨瘤的病例报告,该病例报告在一位健康的32岁女士中表现为逐渐的单侧传导性听力损失。治疗的决定受到疾病负担相对较小的影响,以及骨瘤的大小和位置,这使得手术切除的决定非常困难。考虑到患者的意愿和情况,我们决定在密切随访的同时进行骨传导听力植入.
    We present a case report of a middle ear osteoma presenting as gradual unilateral conductive hearing loss in a healthy 32-year-old lady. The decision for treatment was influenced by the relatively small burden of the disease, and the size and location of the osteoma, which made the decision for surgical excision prohibitively difficult. Taking patient wishes and circumstances into account, the decision was made for a bone conduction hearing implant in conjunction with close follow-up.
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  • 文章类型: Journal Article
    咽鼓管功能障碍(ETD)被认为是中耳疾病以及治疗失败的原因。发病机制可能是慢性感染的结果,过敏,咽喉反流,原发性粘膜疾病,扩张机制和解剖学障碍的功能障碍。因此,了解咽鼓管(ET)的结构和解剖变化变得至关重要,特别是随着新的治疗选择,如输卵管成形术的出现,以确保最佳的治疗效果。
    这项横断面研究是为了使用计算机断层扫描对ET和输卵管周围区域进行多参数测量,并制定了用于输卵管成形术前检查的结构化方案。
    这项研究为期20个月,在100名年龄在18至60岁之间的正常受试者中,他们接受了头部和面部区域的计算机断层扫描(CT)研究,除鼻/咽部和鼻窦疾病以外的适应症。
    平均骨骼,男性的软骨和总体ET长度较高。在女性中,与里德平面的平均ET角较高。在男性中观察到较高的ET腔平均颅尾直径。颈动脉管开裂在两侧的患病率相同(5%),没有明显的性别差异。
    咽鼓管成形术等治疗干预措施将受益于基于术前影像学的计划。这种结构化的协议提供了用于输卵管成形术的术前检查的标准化。
    UNASSIGNED: Eustachian tube dysfunction (ETD) is considered a causative factor for middle ear disease as well as treatment failure. The pathogenesis may be a result of chronic infection, allergy, laryngopharyngeal reflux, primary mucosal disease, dysfunction of the dilation mechanism and anatomical obstruction. Hence, it becomes essential to know the structure and anatomical variations of the Eustachian tube(ET), particularly with the advent of novel therapeutic options such as tuboplasty to ensure optimal therapeutic outcome.
    UNASSIGNED: This cross-sectional study is done to perform multiparametric measurements of the ET and peritubal region using computed tomography and develop a structured protocol for pre-tuboplasty workup.
    UNASSIGNED: This study was done for a period of 20 months, in 100 normal subjects aged between 18 and 60 years, who underwent computed tomography (CT) study of the head and face region, for indications other than nasal/ pharyngeal and sinus disease.
    UNASSIGNED: The mean bony, cartilaginous and overall ET lengths were higher in males. In females, the mean ET angles with Reid\'s plane were higher. Higher mean craniocaudal diameters of the ET lumen were observed in males. Carotid canal dehiscence was seen in equal prevalence on both sides (5%), with no significant gender differences.
    UNASSIGNED: Therapeutic interventions such as eustachian tuboplasty will benefit from preoperative imaging based planning. This structured protocol provides standardization of pre-operative workup for tuboplasty.
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  • 文章类型: Journal Article
    背景:耳部和听力护理计划对于中耳炎(或中耳疾病)的早期发现和管理至关重要。中耳炎和相关听力损失不成比例地影响第一民族儿童。这会影响言语和语言的发展,社会和认知发展,反过来,教育和生活结果。这项范围审查旨在更好地了解高收入殖民地定居者国家的原住民儿童的耳朵和听力护理计划如何旨在减轻中耳炎的负担并增加公平获得护理的机会。具体来说,审查旨在制定计划战略,将每个项目的重点映射到护理路径的4个部分(预防,检测,诊断/管理,康复),并确定表明计划长期可持续性和成功的因素。
    方法:2021年3月使用Medline进行了数据库搜索,Embase,全球卫生,APAPsycInfo,CINAHL,WebofScience核心合集,Scopus,和学术搜索总理。如果计划在2010年1月至2021年3月之间的任何时间开发或运行,则符合资格或纳入。搜索术语包括第一民族儿童等术语,耳朵和听力护理,和健康计划,倡议,竞选活动,和服务。
    结果:27篇文章符合纳入审查的标准,共描述了21项耳朵和听力护理计划。方案采用的策略是:(I)将患者与专科服务联系起来,(二)加强服务的文化安全,和(iii)增加获得耳部和听力保健服务的机会。然而,计划评估措施仅限于输出或服务水平结果的评估,而不是以患者为基础的结果。促进计划可持续性的因素包括资金和社区参与,尽管在许多情况下这些因素有限。
    结论:这项研究的结果突出表明,项目主要在护理路径的两个方面运作-检测和诊断/管理,大概是最需要的地方。有针对性的策略被用来解决这些问题,一些在他们的方法中受到限制。许多程序的成功被评估为输出,许多项目依赖资金来源,这可能会限制长期可持续性。最后,第一民族人民和社区的参与通常只发生在实施期间,而不是整个方案的发展。未来的计划应该嵌入到一个互联的护理系统中,并与现有的政策和资金流挂钩,以确保长期的生存能力。方案应由第一民族社区管理和评估,以进一步确保方案是可持续的,旨在满足社区需求。
    BACKGROUND: Ear and hearing care programs are critical to early detection and management of otitis media (or middle ear disease). Otitis media and associated hearing loss disproportionately impacts First Nations children. This affects speech and language development, social and cognitive development and, in turn, education and life outcomes. This scoping review aimed to better understand how ear and hearing care programs for First Nations children in high-income colonial-settler countries aimed to reduce the burden of otitis media and increase equitable access to care. Specifically, the review aimed to chart program strategies, map the focus of each program against 4 parts of a care pathway (prevention, detection, diagnosis/management, rehabilitation), and to identify the factors that indicated the longer-term sustainability and success of programs.
    METHODS: A database search was conducted in March 2021 using Medline, Embase, Global Health, APA PsycInfo, CINAHL, Web of Science Core Collection, Scopus, and Academic Search Premier. Programs were eligible or inclusion if they had either been developed or run at any time between January 2010 to March 2021. Search terms encompassed terms such as First Nations children, ear and hearing care, and health programs, initiatives, campaigns, and services.
    RESULTS: Twenty-seven articles met the criteria to be included in the review and described a total of twenty-one ear and hearing care programs. Programs employed strategies to: (i) connect patients to specialist services, (ii) improve cultural safety of services, and (iii) increase access to ear and hearing care services. However, program evaluation measures were limited to outputs or the evaluation of service-level outcome, rather than patient-based outcomes. Factors which contributed to program sustainability included funding and community involvement although these were limited in many cases.
    CONCLUSIONS: The result of this study highlighted that programs primarily operate at two points along the care pathway-detection and diagnosis/management, presumably where the greatest need lies. Targeted strategies were used to address these, some which were limited in their approach. The success of many programs are evaluated as outputs, and many programs rely on funding sources which can potentially limit longer-term sustainability. Finally, the involvement of First Nations people and communities typically only occurred during implementation rather than across the development of the program. Future programs should be embedded within a connected system of care and tied to existing policies and funding streams to ensure long term viability. Programs should be governed and evaluated by First Nations communities to further ensure programs are sustainable and are designed to meet community needs.
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  • 文章类型: Journal Article
    背景胆脂瘤被描述为中耳间隙内和周围的鳞状上皮和角质形成细胞的积累。关于沙特阿拉伯胆脂瘤的人口统计学和治疗结果的信息很少。对合并症患病率的评估,并发症和关联,进行了Qassim地区的手术治疗和人口统计学。方法这是一项为期六年的回顾性研究,对在私人医疗机构接受治疗的胆脂瘤患者进行了回顾性研究。从2016年8月到2022年7月。年龄数据,性别,国籍,合并症的存在,手术类型,麻醉类型,从电子病历中收集相关并发症,并使用社会科学统计软件包软件进行分析.结果共检索到60份参与者记录。研究人群的平均年龄为([43.2±SD]21.8)岁。男性优势略高(男性51.7%,女性48.3%)。高血压是最常见的合并症(31.7%),其次是糖尿病(25%)。年龄和性别与手术类型或并发症无统计学意义。结论人口统计变量与临床相关因素无显著相关性,然而,用更大的样本量进行进一步的研究,可靠的临床信息,需要长期随访。
    Background Cholesteatoma is described as the accumulation of squamous epithelium and keratinocytes within and around the middle ear cleft. There is a paucity of information regarding demographic and treatment outcomes for cholesteatoma in Saudi Arabia. An evaluation of the prevalence of comorbidities, complications and associations, of surgical treatment and demographics in the Qassim region was conducted. Methods This was a six-year retrospective review of patients treated for cholesteatoma at a private health facility, from August 2016 to July 2022. Data for age, gender, nationality, presence of comorbidities, type of surgery, type of anesthesia, and associated complications were collected from the electronic medical records and analyzed with Statistical Package for Social Sciences software. Results A total of 60 participants records were retrieved. The average age of the study population was ([43.2 ±SD] 21.8) years. There was a slightly higher male preponderance (males 51.7% and females 48.3%). Hypertension was the most commonly reported comorbidity (31.7%), followed by diabetes mellitus (25%). Age and gender were not statistically significantly associated with type of surgery or complications. Conclusion Demographic variables were not significantly associated with clinical correlates, however, further studies with larger sample sizes, robust clinical information, and long-term follow-up are required.
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  • 文章类型: Journal Article
    未经评估:这项研究评估了阿拉斯加农村地区的听力环境和对生活质量的反思(HEAR-QL)问卷,包括通过社区反馈制作的附录,以反映当地情况。目的是评估阿拉斯加原住民人群的HEAR-QL评分是否与听力损失和中耳疾病成反比。
    UNASSIGNED:儿童和青少年的HEAR-QL问卷是2017年至2019年在阿拉斯加农村进行的一项整群随机试验的一部分。入学的学生在同一天完成了听力测量评估和HEAR-QL问卷。对问卷数据进行了横断面评估。
    UNASSIGNED:共有733名儿童(7-12岁)和440名青少年(13岁以上)完成了问卷调查。有听力损失和无听力损失的儿童的平均听力-QL评分相似(Kruskal-Wallis,p=.39);然而,青少年HEAR-QL评分随着听力损失的增加而显著降低(p<.001)。与没有中耳疾病的儿童(p=.02)和青少年(p<.001)相比,中值HEAR-QL评分均显着降低。在儿童和青少年中,附录评分与HEAR-QL总分密切相关(ρSpearman分别为0.72和0.69).
    UNASSIGNED:在青少年中观察到听力损失与HEAR-QL评分之间的预期负相关。然而,有明显的变异性,不能用听力损失来解释,需要进一步调查。在儿童中未观察到预期的负关联。HEAR-QL评分与儿童和青少年的中耳疾病相关,使其在耳部感染患病率高的人群中具有潜在价值。
    UNASSIGNED:Level2Clinicaltrials.gov注册号:NCT03309553。
    UNASSIGNED: This study evaluated the Hearing Environments and Reflection on Quality of Life (HEAR-QL) questionnaire in rural Alaska, including an addendum crafted through community feedback to reflect the local context. The objectives were to assess whether HEAR-QL score was inversely correlated with hearing loss and middle ear disease in an Alaska Native population.
    UNASSIGNED: The HEAR-QL questionnaires for children and adolescents were administered as part of a cluster randomized trial in rural Alaska from 2017 to 2019. Enrolled students completed an audiometric evaluation and HEAR-QL questionnaire on the same day. A cross-sectional evaluation of questionnaire data was utilized.
    UNASSIGNED: A total of 733 children (ages 7-12 years) and 440 adolescents (ages ≥13 years) completed the questionnaire. Median HEAR-QL scores were similar among children with and without hearing loss (Kruskal-Wallis, p = .39); however, adolescent HEAR-QL scores significantly decreased with increasing hearing loss (p < .001). Median HEAR-QL scores were significantly lower in both children (p = .02) and adolescents (p < .001) with middle ear disease compared with those without. In both children and adolescents, the addendum scores were strongly correlated with total HEAR-QL score (ρSpearman = 0.72 and 0.69, respectively).
    UNASSIGNED: The expected negative association between hearing loss and HEAR-QL score was observed in adolescents. However, there was significant variability that could not be explained by hearing loss, and further investigation is warranted. The expected negative association was not observed in children. HEAR-QL scores were associated with middle ear disease in both children and adolescents, making it potentially valuable in populations where the prevalence of ear infections is high.
    UNASSIGNED: Level 2 Clinicaltrials.gov registration numbers: NCT03309553.
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  • 文章类型: Journal Article
    已经引入了在没有编码知识的情况下操作的机器学习平台(Teachablemachine®)。本研究的目的是评估Teachable机器®诊断鼓膜病变的性能。总共使用3024张鼓膜图像来训练和验证网络的诊断性能。鼓膜图像被标记为正常,渗出性中耳炎(OME),慢性中耳炎(COM),和胆脂瘤.根据分类的复杂性,I级指正常与异常鼓膜;II级定义为正常,OME,或COM+胆脂瘤;和III级区分所有四种病理。此外,使用80张代表性测试图像来评估性能。Teachablemachine®自动创建分类网络,并在上传图像时提供诊断性能。用于将鼓膜分类为正常或异常(I级)的Teachable机器®的平均准确度为90.1%。对于二级,平均准确率为89.0%,Ⅲ级为86.2%.80例代表性鼓膜图像分类的总体准确率为78.75%,和正常的命中率,OME,COM,胆脂瘤占95.0%,70.0%,90.0%,和60.0%,分别。Teachable机器®可以成功地生成用于对鼓膜进行分类的诊断网络。
    A machine learning platform operated without coding knowledge (Teachable machine®) has been introduced. The aims of the present study were to assess the performance of the Teachable machine® for diagnosing tympanic membrane lesions. A total of 3024 tympanic membrane images were used to train and validate the diagnostic performance of the network. Tympanic membrane images were labeled as normal, otitis media with effusion (OME), chronic otitis media (COM), and cholesteatoma. According to the complexity of the categorization, Level I refers to normal versus abnormal tympanic membrane; Level II was defined as normal, OME, or COM + cholesteatoma; and Level III distinguishes between all four pathologies. In addition, eighty representative test images were used to assess the performance. Teachable machine® automatically creates a classification network and presents diagnostic performance when images are uploaded. The mean accuracy of the Teachable machine® for classifying tympanic membranes as normal or abnormal (Level I) was 90.1%. For Level II, the mean accuracy was 89.0% and for Level III it was 86.2%. The overall accuracy of the classification of the 80 representative tympanic membrane images was 78.75%, and the hit rates for normal, OME, COM, and cholesteatoma were 95.0%, 70.0%, 90.0%, and 60.0%, respectively. Teachable machine® could successfully generate the diagnostic network for classifying tympanic membrane.
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  • 文章类型: Case Reports
    迄今为止,同卵双胞胎中的先天性胆脂瘤在耳鼻咽喉科文献中仅被描述过一次。本报告描述了一例单卵双胞胎,有复发性急性中耳炎和双侧中耳积液而无鼓膜穿孔的病史。在插入压力平衡管的鼓膜切开术中,发现两者均患有右侧胆脂瘤,其位置和进展模式几乎相同.在先前的病例系列中,显示了家族性聚集性,并报告了这种情况的可能遗传关联,作者提出了对目前对先天性胆脂瘤疾病的理解的重要补充。
    Congenital cholesteatoma in identical twins has only been described once in Otolaryngology literature thus far. This report describes a case of monozygotic twins with a history of recurrent acute otitis media and bilateral middle ear effusions without tympanic membrane perforation. Upon myringotomy with pressure equalization tube insertion, both were found to have right-sided cholesteatoma with nearly identical location and pattern of progression. In the context of previous case series demonstrating familial clustering and reports of possible genetic associations of this condition, the authors present an important addition to the current understanding of congenital cholesteatoma disorder.
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