Congenital Giant Middle Ear Cholesteatoma Combined with Middle Ear Malformation: A Case Report

TANG Yu-qi; HE Wei-ping; GUO Hua-min; LIU Jing

doi:10.3969/j.issn.1672-4933.2026.05.010

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Congenital Giant Middle Ear Cholesteatoma Combined with Middle Ear Malformation: A Case Report

Clinical Research | 更新时间：2026-10-08

- Congenital Giant Middle Ear Cholesteatoma Combined with Middle Ear Malformation: A Case Report
- Chinese Scientific Journal of Hearing and Speech Rehabilitation Vol. 24, Issue 5, Pages: 501-506(2026)
- 作者机构：
  
  1.广州中医药大学第一临床医学院广州 510499
  2.广州中医药大学第一附属医院耳鼻喉科广州 510405
- 作者简介：
- 基金信息：
- DOI：10.3969/j.issn.1672-4933.2026.05.010
  CLC： R764.2
- Received：21 May 2026，
  
  Revised：2026-07-09，
  
  Accepted：15 August 2026，
  
  Published：15 September 2026
- 稿件说明：
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唐语祺,何伟平,郭华民等.先天性巨大中耳胆脂瘤合并中耳畸形个案研究[J].中国听力语言康复科学杂志,2026,24(05):501-506.

TANG Yu-qi,HE Wei-ping,GUO Hua-min,et al.Congenital Giant Middle Ear Cholesteatoma Combined with Middle Ear Malformation: A Case Report[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2026,24(05):501-506.
唐语祺,何伟平,郭华民等.先天性巨大中耳胆脂瘤合并中耳畸形个案研究[J].中国听力语言康复科学杂志,2026,24(05):501-506. DOI： 10.3969/j.issn.1672-4933.2026.05.010.

TANG Yu-qi,HE Wei-ping,GUO Hua-min,et al.Congenital Giant Middle Ear Cholesteatoma Combined with Middle Ear Malformation: A Case Report[J].Chinese Scientific Journal of Hearing and Speech Rehabilitation,2026,24(05):501-506. DOI： 10.3969/j.issn.1672-4933.2026.05.010.

摘要

目的

先天性巨大中耳胆脂瘤合并中耳畸形由于病变位置隐匿、早期症状不典型，易因误诊而延误最佳治疗时机。本文通过分析病例临床资料，系统总结该病的临床特点，回顾耳显微镜下乳突改良根治手术的要点，综合国内外文献探讨该疾病的发病机制，旨在提高临床医师对该病诊断、鉴别诊断的认知能力及个体化手术策略制订，强调影像学在术前评估中的价值与选择合适手术方式的重要意义。

方法

报道1例7岁男性患儿因右耳反复流脓2年就诊，曾多次诊断为急性中耳炎，抗感染治疗效果不佳。于广州中医药大学第一附属医院耳鼻喉科完善检查后明确诊断为先天性巨大中耳胆脂瘤合并中耳畸形。术前评估为Potsic Ⅳ期。经术前讨论及家属知情同意，患儿接受耳显微镜下乳突改良根治术，在保护原有解剖结构的情况下彻底清除病变组织。

结果

术后病理证实胆脂瘤诊断，随访患儿无耳鸣、眩晕、面瘫等术后并发症，可正常交流，暂无2次手术计划。

结论

首要表现为耳道流脓而无面痛、面瘫、眩晕的先天性中耳胆脂瘤易误诊为中耳炎，颞骨高分辨率计算机断层扫描、中耳磁共振成像与弥散加权成像等影像学对该病的早期定位、范围评估和治疗方案选择具有指导意义。在明确先天性胆脂瘤诊断后应尽快采取合适的手术方式彻底清除病变，尽可能保留原有结构，减少病变对周围组织及相关功能的不可逆损害，最大程度提高患者预后及远期生活质量。

Abstract

Objective

This study aims to enhance the diagnostic capability of clinicians in diagnosis and differential diagnosis

promote individualized surgical strategy development

and emphasize the value of imaging in preoperative evaluation and the importance of selecting appropriate surgical approaches

by analyzing the clinical data of a case of congenital giant middle ear cholesteatoma with middle ear malformation

which is prone to misdiagnosis due to its concealed location and atypical early symptoms

often resulting in delayed optimal treatment; this study systematically summarizes the clinical features

reviews the key aspects of modified radical mastoidectomy under otomicroscopy

and discusses the pathogenesis with reference to domestic and international literature.

Methods

This report describes a 7-year-old male who presented with recurrent right otorrhea for 2 years and had received repeated diagnoses of acute otitis media with a poor response to anti-infective therapy; however

following comprehensive examination at our institution

the patient was definitively diagnosed with congenital giant middle ear cholesteatoma with middle ear malformation

and preoperative staging was determined as Potsic stage IV. After thorough preoperative discussion and with informed consent obtained from the family

the patient underwent modified radical mastoidectomy under otomicroscopy

achieving complete lesion removal while preserving the original anatomical structures.

Results

Postoperative histopathological examination confirmed the diagnosis of cholesteatoma. During follow-up

the patient had no postoperative complications such as tinnitus

vertigo

or facial palsy

and demonstrated normal communication ability; no second-stage surgical intervention is currently planned.

Conclusion

Congenital middle ear cholesteatoma with otorrhea as the primary manifestation

but without facial pain

facial palsy

or vertigo

is susceptible to misdiagnosis as otitis media. Imaging modalities including high-resolution CT of the temporal bone

middle ear MRI

and diffusion-weighted imaging are indispensable for early localization

extent assessment

and treatment planning. Once the diagnosis has been established

appropriate surgical intervention should be performed promptly to achieve complete removal of the lesion

preserve native structures to the greatest extent

minimize irreversible damage to surrounding tissues and functions

and thus improve the long-term prognosis and quality of life.

关键词

Keywords

references

Luntz M , Barzilai R . Middle ear cholesteatoma [J ] . Harefuah , 2021 , 160 ( 5 ): 316 - 322 .

Derlacki EL , Clemis JD . Congenital cholesteatoma of the Middle Ear and Mastoid [J ] . Annals of Otology , Rhinology & Laryngology, 1965 , 74 ( 3 ): 706 - 727 .

Levenson MJ , Parisier SC , Chute P , et al . A review of twenty congenital cholesteatomas of the middle ear in children [J ] . Otolaryngology-Head and Neck Surgery , 1986 , 94 ( 5 ): 560 - 567 .

Persaud R , Hajioff D , Trinidade A , et al . Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma [J ] . The Journal of Laryngology & Otology , 2007 , 121 ( 11 ): 1013 - 1019 .

Michaels L . Origin of congenital cholesteatoma from a normally occurring epidermoid rest in the developing middle ear [J ] . International Journal of Pediatric Otorhinolaryngology , 1988 , 15 ( 1 ): 51 - 65 .

Zou Y . Congenital ear malformation (CEM) [J ] . Acta Oto-Laryngologica , 2023 , 143 ( sup1 ): S3 - S16 .

申卫东 , 韩维举 , 杨仕明 , 等 . 先天性中耳胆脂瘤 [J ] . 中国听力语言康复科学杂志 , 2007 , 4 ( 3 ): 27 - 29, 78 .

Bennett M , Warren F , Jackson GC , et al . Congenital cholesteatoma: theories, facts, and 53 patients [J ] . Otolaryngologic Clinics of North America , 2006 , 39 ( 6 ): 1081 - 1094 .

Yung M , Tono T , Olszewska E , et al . EAONO/JOS joint consensus statements on the definitions, classification and staging of middle ear cholesteatoma [J ] . The Journal of International Advanced Otology , 2017 , 13 ( 1 ): 1 - 8 .

Chen JM , Schloss MD , Manoukian JJ , et al . Congenital cholesteatoma of the middle ear in children [J ] . The Journal of Otolaryngology , 1989 , 18 ( 1 ): 44 - 48 .

Levenson MJ , Michaels L , Parisier SC . Congenital cholesteatomas of the middle ear in children: origin and management [J ] . Otolaryngologic Clinics of North America , 1989 , 22 ( 5 ): 941 - 954 .

Denoyelle F , Simon F , Chang KW , et al . International Pediatric Otolaryngology Group (IPOG) consensus recommendations: congenital cholesteatoma [J ] . Otology & Neurotology , 2020 , 41 ( 3 ): 345 - 351 .

Simon F , Remangeon F , Loundon N , et al . Pediatric cholesteatoma follow-up: residual and recurrence in 239 cases with over 5-year hindsight [J ] . The Laryngoscope , 2024 , 134 ( 11 ): 4789 - 4798 .

Koltai PJ , Nelson M , Castellon R , et al . The natural history of congenital cholesteatoma [J ] . Archives of Otolaryngology-Head & Neck Surgery , 2002 , 128 ( 7 ): 804 - 809 .

王丽 , 张璞 , 张勋 , 等 . 乳突先天性胆脂瘤一例 [J ] . 中华耳科学杂志 , 2018 , 16 ( 6 ): 932 - 933 .

秦兆冰 , 董明敏 . 中耳畸形并发胆脂瘤三例 [J ] . 中华耳鼻咽喉头颈外科杂志 , 2005 , 40 ( 3 ): 218 - 219 .

Kwok HM , Cheung CHL , Ng TF , et al . Congenital cholesteatoma: what radiologists need to know [J ] . Pediatric Radiology , 2024 , 54 ( 4 ): 620 - 634 .

Richter GT , Lee KH . Contemporary assessment and management of congenital cholesteatoma [J ] . Current Opinion in Otolaryngology & Head and Neck Surgery , 2009 , 17 ( 5 ): 339 - 345 .

侯昭晖 , 李瑞香 , 杨仕明 . 耳内镜技术在中耳胆脂瘤手术中的应用策略 [J ] . 中华耳科学杂志 , 2017 , 15 ( 4 ): 393 - 397 .

El Jerrari A , Stierle JL , Debry C , et al . Le cholestéatome congénital et les malformations ossiculaires associées [J ] . Annales d'Otolaryngologie et de Chirurgie Cervicofaciale , 1995 , 112 ( 5 ): 258 - 261 .

Huang TS , Lee FP . Congenital cholesteatoma: review of twelve cases [J ] . American Journal of Otology , 1994 , 15 ( 3 ): 276 - 281 .

王佳豪 , 刘云福 , 马晓博 , 等 . 影像学在中耳胆脂瘤诊疗中的应用现状及进展 [J ] . 影像科学与光化学 , 2024 , 42 ( 5 ): 521 - 528 .

Sadé J , Fuchs C . Cholesteatoma: ossicular destruction in adults and children [J ] . The Journal of Laryngology & Otology , 1994 , 108 ( 7 ): 541 - 544 .

Ryan PJ , Patel NP . Endoscopic management of pediatric cholesteatoma [J ] . Journal of Otology , 2020 , 15 ( 1 ): 17 - 26 .

Fitzek C , Mewes T , Fitzek S , et al . Diffusion-weighted MRI of cholesteatomas of the petrous bone [J ] . Journal of Magnetic Resonance Imaging , 2002 , 15 ( 6 ): 636 - 641 .

万怡宁 , 张德军 , 傅则名 , 等 . 磁共振弥散加权成像在先天性中耳胆脂瘤精准诊断与JOS分期中的应用探讨:附12例报告 [J ] . 山东大学耳鼻喉眼学报 , 2021 , 35 ( 6 ): 65 - 69 .

Jindal M , Riskalla A , Jiang D , et al . A systematic review of diffusion-weighted magnetic resonance imaging in the assessment of postoperative cholesteatoma [J ] . Otology & Neurotology , 2011 , 32 ( 8 ): 1243 - 1249 .

杨启梅 , 曹亚亚 , 金龙 , 等 . 颞骨高分辨率CT与DW-MRI融合技术在中耳胆脂瘤的诊断价值研究 [J ] . 临床耳鼻咽喉头颈外科杂志 , 2025 , 39 ( 12 ): 1120 - 1125 .

蒋吉美 , 张皓 , 罗禹 , 等 . 中耳胆脂瘤的影像诊断:高分辨率计算机断层成像与磁共振成像的比较研究 [J ] . 现代生物医学进展 , 2015 , 15 ( 9 ): 1700 - 1704 .

Locketz GD , Li PM , Fischbein NJ , et al . Fusion of computed tomography and PROPELLER diffusion-weighted magnetic resonance imaging for the detection and localization of middle ear cholesteatoma [J ] . JAMA Otolaryngology-Head & Neck Surgery , 2016 , 142 ( 10 ): 947 - 953 .

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