Congenital middle ear cholesteatoma: experience in 48 cases

We studied 48 patients (48 ears) with congenital cholesteatoma who underwent surgery at our department from 1979 to 2000, and investigated symptoms at initial onset, tympanic membrane findings, cholesteatoma configuration and site, type of surgical procedure, and surgical outcome. Patients were from 2 to 62 years old (mean: 16.7 years), with 60.4% aged 15 years or younger. The symptom at initial onset was hearing loss in most (58.2%). Hearing loss was the main symptom in all with open type cholesteatoma, and most of these patients had normal tympanic membrane findings. The cholesteatoma was located mainly in the superior posterior portion of the tympanic cavity in many patients. The site of involvement was the tympanic cavity in 12 (25.0%), mastoid cavity in 2 (4.2%) and the petrous apex in 1 (2.1%). In many of (31 ears, 64.6%), the cholesteatoma was advanced and extended from the tympanic cavity to the mastoid antrum. For 23 of the 48 ears, treatment was completed in one operation. The remaining 25 ears required staged surgery. Loss of the structure of the upper part of the stapes was seen in 58.3% of patients, so most underwent type IV ossiculoplasty, with types III and I next most common.     

耳内镜下儿童中耳胆脂瘤的处理及疗效分析

目的 探讨耳内镜下经耳道径路切除儿童中耳胆脂瘤的手术适应证及临床疗效,为该类疾病的处理提供临床参考.方法 回顾性分析2017年9月-2020年10月上海交通大学医学院耳科学研究所耳内镜下经耳道径路处理的20例中耳胆脂瘤患儿临床资料,患者年龄3～14岁,平均年龄为(7±0.5)岁.根据术前影像学评估,病变范围均在中耳范围...     

Results of conservative surgery for middle ear cholesteatoma

A retrospective study was carried out to analyze treatment results for cholesteatoma at the University of Iowa Hospitals and Clinics. All patients undergoing primary surgical treatment from January 1, 1969 to December 31, 1973 were followed through October 1976. Treatment failures were based on the occurrence of postoperative cholesteatoma. The probability of being disease free for periods up to five years after original surgery was then estimated for each type of operation. Postoperative cholesteatoma occurred after atticotomy in 17%, intact canal wall mastoidectomy in 35%, and modified radical mastoidectomy in 9%. The postoperative cholesteatoma rate in the group having intact canal wall mastoidectomy was more than twice as high for those patients under age nine. The likelihood of being disease free five years after intact canal wall surgery was estimated to be 36% using the life table method. Disease recurrence was significantly higher after intact canal wall surgery compared to other surgical methods. The effectiveness of this method should be evaluated in a randomized, prospective manner to minimize patient selection and treatment bias.     

中耳先天性胆脂瘤1例

<正>中耳先天性胆脂瘤是少见疾病,常因病变隐匿而导致临床上延误诊断和治疗,随着影像学技术的提高和显微镜检查的普及,发现报道的先天性胆脂瘤病例逐渐增多。先天性胆脂瘤早期可无任何表现,逐渐出现传导性聋、耳痛,听力进行性下降,严重者可出现面瘫和神经性聋,甚至出现颅内并发症[1],由于无流脓、鼓膜穿孔等耳部症状,单侧的听力下降容易被忽略,诊断不易做出。现将解放军405医院耳鼻喉科2014年3月收治的先天性胆脂     

Congenital middle ear cholesteatoma: report of 3 cases.

In this report, we presented 3 cases of congenital middle ear cholesteatoma which occurred in a 12-year-old girl, a 4-year-old boy, and a 6-year-old boy. In all 3 cases, there was a whitish mass behind a normal tympanic membrane. Congenital middle ear cholesteatoma is not a rare disease. In the early stage, it is asymptomatic. But when it progresses, this disorder can destroy conductive systems of the middle ear and cause many symptoms. One patient (Case 1) had a complaint of hearing impairment. She underwent mastoidectomy and tympanoplasty; however, the cholesteatoma recurred. The other 2 patients had no symptoms. The abnormal appearance of their tympanic membrane was found by chance at their local otologists. We performed tympanotomies and removed cholesteatomas without aftereffects. When otologists note an abnormal appearance behind a normal tympanic membrane, with or without symptoms, tympanotomy should be done due to the possibility of congenital middle ear cholesteatoma.     

Congenital cholesteatoma of the middle ear in children]

In a series of 16 middle ear cholesteatomas of a congenital type are reported in children; the youngest (18 months) presented with a bilateral case. Whereas a simple tympanoplasty could cure a localized pearl, typically anterosuperior in the mesotympanum, the stapes is fast eroded (7 cases) if progression goes on. Intact canal wall technique in 2 stages was the typical procedure. Good hearing results were generally achieved (except in one case of fixed footplate): 9 cases/14 with an ABG within 20 dB and an AC level within 30 dB.     

硬质耳内镜在胆脂瘤中耳炎手术治疗中的应用

目的探讨耳内镜在胆脂瘤中耳炎手术治疗中的作用。方法2003—2005年的中耳胆脂瘤患者43例43耳,应用硬性耳内镜协助下行乳突根治 Ⅰ期鼓室成形术,并在耳内镜下随访2年以上(平均2.211±0.323年)。结果术后1.5～3月干耳40例,鼓膜色泽正常。2例内陷袋形成,术后1年经耳内镜手术探查,发现为中耳粘连导致上中鼓室通气不畅。1例术后0.5年因患耳流脓再次手术,为修补之鼓膜与鼓窦口上皮粘连,后鼓室有孤立胆固醇肉芽肿,清除粘连及病变组织后2月干耳。随访测听结果:听力提高10～20dB21耳,21～30dB15耳,>30dB4耳,<10dB3耳。结论耳内镜能够弥补耳显微镜的不足,辅助清除中耳病灶以增加手术成功率。     

中耳畸形并发胆脂瘤三例

图 1　颞骨CT轴位片显示鼓室隔板将左鼓室分成内外两个腔隙,内侧腔隙可见听小骨样结构,咽鼓管呈高密度影　　图 2　颞骨CT冠位片显示双侧听小骨畸形,左侧水平段面神经下垂遮盖前庭窗,鼓室盾板消失　　图 3　颞骨CT轴位片显示左鼓室听小骨形态不规则,与鼓室壁分界不清楚　　先天性中耳畸形并发胆脂瘤临床少见。我院收治 3例,报道如下。例 1　女, 21岁。双耳听力下降 20年、间断流脓 10余年, 2002年 3月 13日入院。检查:双耳轮脚前各有一瘘管口,右外耳道正常,左外耳道轻度狭窄,双侧鼓膜大穿孔,左鼓室有上皮样组织。颞骨CT显示:双侧乳突气…     

儿童中耳先天性胆脂瘤的诊断和预后

目的:探讨儿童中耳先天性胆脂瘤的临床症状、位置、影像学特征、治疗及预后.方法:回顾性分析9例中耳先天性胆脂瘤患儿的临床资料.结果:患儿均行手术治疗,5例有听小骨破坏,术后复发4例,复发率为44.4%.平均随访7.4年,术前平均听阈为28 dB,术后平均听阈为26 dB.结论:中耳先天性胆脂瘤临床少见,发病隐匿,单侧传导性聋为主要表现.术前诊断可根据Levenson诊断标准和CT检查结果.其预后和复发率与病变范围、手术方式、是否2次手术有关.     

Congenital cholesteatoma of the middle ear and mastoid. Apropos of 5 cases]

Five cases of congenital cholesteatoma of the middle ear and mastoid as defined by Derlacki's criteria were encountered over a 14 month period. They make up 5% of all cases of cholesteatoma managed over the same period of time. Three were young children and all presented with unilateral hearing loss. One had associated multiple congenital abnormalities of the external and middle ear. Only in one patient was the diagnosis made on initial otoscopic examination and the remainder diagnosed on CTscan, myringotomy and tympanotomy. All were operated on; three by the intact canal wall technique, one by the canal down technique with mastoid cavity obliteration and one by atticotomy with lateral attic wall reconstruction. One patient required a second stage operation for excision of an extension of disease deep to the superior semicircular canal via the middle cranial fossa approach in order to preserve cochlear function. These five cases illustrate the diagnostic pitfalls of this condition in which silent danger lurks behind an intact tympanic membrane.     

10例先天性中耳胆脂瘤临床分析

目的：探讨先天性中耳胆脂瘤的临床特征及手术方法。方法：回顾性分析10例先天性中耳胆脂瘤患者的临床资料。5例经耳内途径行鼓室探查并一期鼓室成形术,3例行闭合式乳突根治术及鼓室成形术,2例行开放式乳突根治术及鼓室成形术。结果：术中见5例胆脂瘤局限于中后鼓室,3例位于中鼓室及上鼓室,2例病变范围广泛,侵及乳突。术后6个月平均纯音听阈为30dBHL,气骨导差在20dB以内,复查颞骨CT均未发现胆脂瘤残留和复发。结论：先天性中耳胆脂瘤病变隐匿,常于鼓室前方或后方,易破坏听骨链,导致传导性聋;影像学检查可为诊断及术式选择提供依据;早期手术治疗可获得较好的听力重建效果。     

Immunological problems in middle ear cholesteatoma

Regardless of the pathogenesis of congenital and acquired cholesteatoma two questions seem to be fundamental: what is the origin of the keratinizing squamous epithelium, and what influences invasive and hyperproliferative behavior of the epidermis. The authors of this paper advance a hypothesis that the similar mechanism of the skin infection could exist in psoriasis and atopic dermatitis as well as in cholesteatoma. The retraction pocket leads to the loss of the defense mechanisms and development of planctonic bacteria. Direct and indirect signalings of bacterial products, such as endotoxin stimulate the cytokine cascade and therefore the hyperproliferation of the epithelium keratynocytes. The mobilization of the immunological system may an important role in the perimatrix development.     

Colony-stimulating factor in middle ear cholesteatoma

Granulocyte-macrophage colony stimulating factor (GM-CSF) was found in human middle ear cholesteatoma tissues by immunohistochemical technique using mouse monoclonal anti-(human) GM-CSF IgG. Immunofluorescent staining showed the presence of GM-CSF in the peribasal area, in some suprabasal cells of the epithelium, and in the inflammatory connective tissue, especially in the monocytes and fibroblasts. These findings were confirmed by the immunoperoxidase method. Staining of the external ear canal epithelium, however, was significantly weaker than that of the cholesteatoma epithelium. The presence of GM-CSF in cholesteatoma appears to be a response to inflammation occurring in the middle ear cavity. Moreover, our in vitro study showed that GM-CSF induced the proliferation and protein synthesis of basal keratinocytes. This study suggests that GM-CSF is involved in the development and destructive effects of middle ear cholesteatoma.