【摘要】：目的:先天性中耳膽脂瘤是少見(jiàn)的耳部疾病,因發(fā)病部位隱匿,侵襲性強(qiáng),出現(xiàn)癥狀時(shí)患者往往已有骨質(zhì)破壞和聽(tīng)力障礙,甚至可導(dǎo)致顱內(nèi)外并發(fā)癥,因此早發(fā)現(xiàn)、早診斷是治療先天性中耳膽脂瘤的關(guān)鍵所在。為了探討先天性中耳膽脂瘤的發(fā)病特點(diǎn)、年齡分布、臨床表現(xiàn)、鼓膜特征,減少誤診,我們擬對(duì)先天性中耳膽脂瘤患者進(jìn)行系統(tǒng)性的臨床病例分析總結(jié)。方法:回顧分析2013年1月至2015年9月吉林大學(xué)第二醫(yī)院耳鼻咽喉-頭頸外科收治的資料完整的先天性中耳膽脂瘤患者的臨床資料共6例,所有患者均行手術(shù)治療且術(shù)后經(jīng)病理證實(shí)為膽脂瘤。分析內(nèi)容包括患者的發(fā)病年齡、臨床癥狀、體征及輔助檢查結(jié)果(包括電子耳內(nèi)鏡、純音測(cè)聽(tīng)、聲導(dǎo)抗、顳骨HRCT等),并對(duì)上述特征進(jìn)行分析總結(jié)。結(jié)果:納入研究者6例,其中男性4例,女性2例,年齡17~46歲,平均年齡30.8歲。臨床表現(xiàn)均有患耳聽(tīng)力下降,其中伴耳悶脹感5例,耳鳴5例,眩暈1例。純音測(cè)聽(tīng)結(jié)果符合傳導(dǎo)性聾5例,混合性聾1例;平均氣導(dǎo)聽(tīng)閾為53d BHL,平均氣骨導(dǎo)差為31d B;電子耳內(nèi)鏡檢查見(jiàn)6例患者鼓膜均完整、無(wú)內(nèi)陷袋形成,其中鼓膜內(nèi)側(cè)團(tuán)塊狀影主要位于中后象限者3例,位于全鼓室者3例;顳骨CT示病變側(cè)乳突呈氣化型2例,硬化型1例,板障型3例;所有膽脂瘤在顳骨HRCT上均表現(xiàn)為軟組織密度影,其中氣化型乳突患者膽脂瘤病變局限于鼓室者1例,侵犯鼓竇者1例。硬化型及板障型患者膽脂瘤局限于鼓室者1例,侵犯鼓竇及乳突者3例,伴半規(guī)管破壞者1例,伴面神經(jīng)管破壞者2例;根據(jù)Kikuchi等對(duì)先天性中耳膽脂瘤的分類(lèi),符合閉合型1例,開(kāi)放型5例;根據(jù)Potsic分級(jí)標(biāo)準(zhǔn),符合Ⅱ級(jí)者1例,Ⅲ級(jí)者2例,Ⅳ級(jí)者3例;所有患者均行手術(shù)治療,按照2012年中華醫(yī)學(xué)會(huì)中耳炎手術(shù)分型標(biāo)準(zhǔn),本研究中行開(kāi)放式乳突切開(kāi)+Ⅱ型鼓室成形術(shù)者3例,鼓室探查+Ⅲ型鼓室成形術(shù)者1例,鼓室探查+Ⅱ型鼓室成形術(shù)者1例,鼓室探查+Ⅰ型鼓室成形術(shù)者1例;術(shù)后隨訪(fǎng)6個(gè)月~2年,目前未發(fā)現(xiàn)膽脂瘤復(fù)發(fā)。結(jié)論:1先天中耳膽脂瘤早期臨床癥狀不明顯,或僅單側(cè)傳導(dǎo)性聽(tīng)力下降,易誤診、漏診,臨床確診時(shí)往往延誤多年。2對(duì)于鼓膜完整,其內(nèi)側(cè)有白色或黃色團(tuán)塊狀影,伴或不伴單側(cè)傳導(dǎo)性聽(tīng)力下降的患者,耳鏡檢查至關(guān)重要,應(yīng)高度警覺(jué)先天性中耳膽脂瘤的可能性。3顳骨HRCT是診斷先天中耳膽脂瘤的重要方法,一旦確診為先天性中耳膽脂瘤,應(yīng)盡快手術(shù)治療。
[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，

本文編號(hào)：2354508