有鼓膜特征的6例先天性中耳胆脂瘤临床病例分析

发布时间：2018-11-24 17:51

【摘要】：目的:先天性中耳胆脂瘤是少见的耳部疾病,因发病部位隐匿,侵袭性强,出现症状时患者往往已有骨质破坏和听力障碍,甚至可导致颅内外并发症,因此早发现、早诊断是治疗先天性中耳胆脂瘤的关键所在。为了探讨先天性中耳胆脂瘤的发病特点、年龄分布、临床表现、鼓膜特征,减少误诊,我们拟对先天性中耳胆脂瘤患者进行系统性的临床病例分析总结。方法:回顾分析2013年1月至2015年9月吉林大学第二医院耳鼻咽喉-头颈外科收治的资料完整的先天性中耳胆脂瘤患者的临床资料共6例,所有患者均行手术治疗且术后经病理证实为胆脂瘤。分析内容包括患者的发病年龄、临床症状、体征及辅助检查结果(包括电子耳内镜、纯音测听、声导抗、颞骨HRCT等),并对上述特征进行分析总结。结果:纳入研究者6例,其中男性4例,女性2例,年龄17~46岁,平均年龄30.8岁。临床表现均有患耳听力下降,其中伴耳闷胀感5例,耳鸣5例,眩晕1例。纯音测听结果符合传导性聋5例,混合性聋1例;平均气导听阈为53d BHL,平均气骨导差为31d B;电子耳内镜检查见6例患者鼓膜均完整、无内陷袋形成,其中鼓膜内侧团块状影主要位于中后象限者3例,位于全鼓室者3例;颞骨CT示病变侧乳突呈气化型2例,硬化型1例,板障型3例;所有胆脂瘤在颞骨HRCT上均表现为软组织密度影,其中气化型乳突患者胆脂瘤病变局限于鼓室者1例,侵犯鼓窦者1例。硬化型及板障型患者胆脂瘤局限于鼓室者1例,侵犯鼓窦及乳突者3例,伴半规管破坏者1例,伴面神经管破坏者2例;根据Kikuchi等对先天性中耳胆脂瘤的分类,符合闭合型1例,开放型5例;根据Potsic分级标准,符合Ⅱ级者1例,Ⅲ级者2例,Ⅳ级者3例;所有患者均行手术治疗,按照2012年中华医学会中耳炎手术分型标准,本研究中行开放式乳突切开+Ⅱ型鼓室成形术者3例,鼓室探查+Ⅲ型鼓室成形术者1例,鼓室探查+Ⅱ型鼓室成形术者1例,鼓室探查+Ⅰ型鼓室成形术者1例;术后随访6个月~2年,目前未发现胆脂瘤复发。结论:1先天中耳胆脂瘤早期临床症状不明显,或仅单侧传导性听力下降,易误诊、漏诊,临床确诊时往往延误多年。2对于鼓膜完整,其内侧有白色或黄色团块状影,伴或不伴单侧传导性听力下降的患者,耳镜检查至关重要,应高度警觉先天性中耳胆脂瘤的可能性。3颞骨HRCT是诊断先天中耳胆脂瘤的重要方法,一旦确诊为先天性中耳胆脂瘤,应尽快手术治疗。
[Abstract]:Objective: congenital middle ear cholesteatoma is a rare ear disease. Because of the occult and aggressive location of the disease, the patients often have bone destruction and hearing impairment, and may even lead to complications outside the brain. Early diagnosis is the key to the treatment of congenital middle ear cholesteatoma. In order to investigate the characteristics of congenital middle ear cholesteatoma, age distribution, clinical manifestations, tympanic membrane characteristics and reduce misdiagnosis, we intend to analyze and summarize the clinical cases of congenital middle ear cholesteatoma. Methods: from January 2013 to September 2015, 6 patients with congenital middle ear cholesteatoma treated in the second Hospital of Jilin University were retrospectively analyzed. All patients were treated surgically and pathologically proved cholesteatoma. The analysis included age of onset, clinical symptoms, signs and results of auxiliary examinations (including electronic otoendoscopy, pure tone audiometry, acoustic conductance meter, HRCT of temporal bone, etc.), and the above characteristics were analyzed and summarized. Results: the study included 6 subjects, including 4 males and 2 females, aged 1746 years, with an average age of 30.8 years. There were 5 cases of hearing loss, 5 cases of tinnitus and 1 case of vertigo. The results of pure tone audiometry were consistent with conductive deafness in 5 cases, mixed deafness in 1 case, mean air-bone conductance difference of 31 dB in 53 d BHL,. In 6 cases, the tympanic membrane was intact and no invagination bag was formed. The medial tympanic mass was mainly located in the middle and posterior quadrant in 3 cases, and in the whole tympanic chamber in 3 cases. CT of temporal bone showed that the mastoid process was vaporized in 2 cases, sclerosing in 1 case and plate barrier in 3 cases. All cholesteatoma showed soft tissue density on HRCT of temporal bone. The lesions of cholesteatoma were localized in tympanum in 1 case and in tympanic sinus in 1 case. Cholesteatoma was localized in tympanum in 1 case, invasion of tympanic sinus and mastoid in 3 cases, accompanied by destruction of semicircular canal in 1 case and destruction of facial canal in 2 cases. According to the classification of congenital middle ear cholesteatoma by Kikuchi, there were 1 case of closed type, 5 cases of open type, 1 case of grade 鈪，

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