中耳先天性胆脂瘤二例

中耳先天性胆脂瘤二例赵全义，刘晓梅，纳玉萍有关中耳先天性胆脂瘤的文献国内外均不多见。对此病的诊断及治疗方法尚存在较大争议，本文就收治的２例先天性胆脂瘤进行了讨论。１病例例１女，５岁半，右耳后肿胀２０余天就诊，既往无耳流脓史；右耳后肿胀、稍红，鼓膜完整...     

先天性外中耳畸形伴颞骨胆脂瘤一例

患者男，16岁，因右耳偶流脓2个月于2005年2月4日收入广西医科大学第五附属医院。CT提示颞骨巨大胆脂瘤累及中、后颅窝（图1），磁共振成像提示小脑受压。在脑外科协作下经脑室镜辅助下显微镜下完成第1次手术。术中见颞骨胆脂瘤经小窗与中颅窝、后颅窝相通，硬脑膜暴露。[第一段]     

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     

先天性中耳胆脂瘤

目的探讨先天性中耳胆脂瘤的位置、范围、临床症状、影像学特征及手术治疗。方法本文回顾性分析了解放军总医院耳鼻咽喉-头颈外科自1995年1月～2005年10月诊治的952例胆脂瘤患者的治疗结果,对其中10例先天性中耳胆脂瘤的起源、临床特征及手术前后的听力进行了评估。结果10例先天性中耳胆脂瘤患者的平均年龄为16岁(10～24岁),其中7例为男性,3例为女性,出现症状到临床确诊的平均时间为2年。术前平均纯音听阈为55dB,平均气-骨导差为45dB,有2例患者的鼓膜像上可以看到典型的先天性中耳胆脂瘤表现。所有病例均进行了高分辨率颞骨CT扫描。8例术前分别被诊断为耳硬化症或听骨链畸形。所有病例都在外耳道径路鼓室探查清除胆脂瘤后进行了一期鼓室成型术,除了1例因为病变广泛选择了完壁式乳突根治和鼓室成型术。本组所有病例的胆脂瘤均位于中-后鼓室,主要是在砧镫关节处。术后的平均气-骨导差小于20dB。所有病例术后至少随访1年半,2例病人因为术后听力下降进行了二期手术。本组病例中经手术探查或CT复查,没有发现胆脂瘤残留或复发。结论原发性中耳胆脂瘤临床罕见,常被延误诊断,其预后和残留、复发比例主要决定于病变范围。     

先天性中耳和岩尖胆脂瘤一例

我科于2005年收治罕见先天性中耳、岩尖胆脂瘤1例，报道如下。     

中耳先天性胆脂瘤二例

颞骨先天性胆脂瘤是罕见的具有破坏性的耳部疾病．病变隐蔽，早期无症状，出现症状时已有骨质破坏和耳部功能性和器质性障碍，甚至严重颅内外并发症，常发生于颅内，发生于颞骨者少见，我们曾收治2例，报道如下。     

中耳乳突先天性胆脂瘤五例分析

先天性胆脂瘤在临床上并不少见 ,它可原发于颞骨的各个部位 ,包括岩尖、膝状节区域、中耳及乳突区和颞骨鳞部、外耳道。本文报道近 10年来收治的 5例发生在中耳乳突的先天性胆脂瘤。1　临床资料1.1　病例资料　病例 1,9岁 ,右耳后反复红肿、流脓 4月余 ,听力下降不明显。检查 :右耳后皮肤红肿 ,局部破溃 ,范围约1ｃｍ× 1.5ｃｍ ,乳突区压痛 ,外耳道正常 ,鼓膜完整 ,但浑浊、增厚、暗红。病例 2 ,男性 ,9岁 ,右耳后反复红肿、脓疡半年 ,脓液恶臭伴淡血水 ,听力下降。检查 :右耳后皮肤有一 7ｃｍ× 5ｃｍ红肿包块 ,压痛明显 ,中央破溃 ,有脓…     

中耳胆脂瘤研究进展

中耳胆脂瘤是耳鼻咽喉科较为常见的一种慢性中耳疾病,长期外耳道流脓、鼓膜穿孔、听力下降为其主要特点,它对周围组织具有侵袭性,易引起中耳传音结构的破坏和骨质吸收,进而引起面瘫、迷路炎、耳后骨膜下脓肿及各种颅内外并发症。中耳胆脂瘤的病因及发病机制复杂,治疗方法的疗效争议较多。本文主要将近年来关于中耳胆脂瘤的病因、发病机制及治疗进展进行综述。     

先天性中耳胆脂瘤1例

先天性胆脂瘤又称表皮样囊肿或珍珠瘤,是一种源于皮肤外胚层的先天性肿瘤,为胚胎早期神经沟封闭时,皮肤外胚层的剩件残留发展而成.按其部位,可分为颅外型和颅内型.颅外型的可以发生在中耳和乳突,因其发病隐匿,易被误诊,延误治疗.目前国内外有关先天性中耳胆脂瘤的文献不多见,本文就收治的1例先天性中耳胆脂瘤报告如下.     

先天性特大小儿中耳胆脂瘤一例

先天性胆脂瘤的发病率低,多发现于儿童.一般认为先天性胆脂瘤多为外胚层细胞残留而形成.我科成功地治疗了一例先天性特大小儿中耳胆脂瘤患者,报道如下.     

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。     

Clinical study of congenital cholesteatoma of the middle ear

We studied 35 ears of 34 patients with congenital cholesteatoma who were operated between June 1992 and May 2003, focusing on the localization and progression of congenital cholesteatoma. Patients were 2 to 55 years of age. Congenital cholesteatoma was diagnosed based on two intraoperative findings: 1) no continuity between the tympanic membrane and cholesteatoma, 2) no presence of perforation, retraction, or granulation of the tympanic membrane. All patients had closed-type cholesteatoma, and none formed open-type cholesteatoma, which grows as a flat sheet of epidermis. The primary site of congenital cholesteatoma was classified into 3 types; 1) anterior-superior quadrant, 2) posterior-superior quadrant, and 3) epitympanic, and the origin of these three types of congenital cholesteatoma was difficult to explain by a single theory. We operated on 31 ears by canal wall up tympanoplasty and on 4 ears by canal wall down tympanoplasty. On all but 4 ears, we performed planned-staged operation, including second-look operations, in case of recurrence or residual cholesteatoma. Improvement in hearing after surgery was seen in 22 of the 26 ears treated and followed up for more than 6 months after surgery. By drawing all localization of congenital cholesteatoma in 35 ears, we studied the progression of congenital cholesteatoma and speculated on the original primary site. Congenital cholesteatoma in restricted areas consequently implies good results in hearing after surgery, making earlier diagnosis and treatment desirable.     

Clinical evaluation of congenital cholesteatoma of the middle ear

We conducted a retrospective study to identify the clinical features and surgical observations of congenital cholesteatoma. Sixty patients were diagnosed and underwent surgery for congenital cholesteatoma between April 1987 and May 2002. All diagnoses were made on the basis of two operative findings: 1. the tympanic membrane manifested neither retraction, perforation, nor granulation. 2. the tympanic membrane was not continuous with the cholesteatoma. In this series, congenital cholesteatoma accounted for 7% of all cholesteatomas (853 ears). The patient age ranged from 2 to 48 years. The male to female ratio was 4:1. Seventeen patients had multiple cholesteatoma. Fifty-three patients exhibited closed-type cholesteatomas, while the remaining 7 patients had open-type cholesteatomas that had formed as a flat surface of the epidermis. Patients with open-type cholesteatomas presented with a much more pronounced conductive hearing loss and ossicular erosion or malformation. Twenty-two patients with relatively small cholesteatomas were analyzed to estimate the origin of their cholesteatomas. Of the 22 patients, 13 had anterior superior quadrant (ASQ-type) and 9 had posterior superior quadrant (PSQ-type) cholesteatomas. The mean age at the time of detection was older in the PSQ-type group than in the ASQ-type group and the frequency of ossicular erosion or malformation was more prominent in the PSQ-type group than in the ASQ-type group. The primary site of origin was thought to be the portion between the tympanic ostium of the auditory canal and the semicanal for tensor tympani in the ASQ-type group and near the incudostapedial joint in the PSQ-type group. A planned staged procedure was performed in 29 patients, 15 patients (52%) had residual lesions situated mostly on the oval window, the round window, an exposed facial nerve or an exposed lateral semicircular canal. The frequency of residual lesions in patients who presented with extended, multiple cholesteatoma and those with ossicular malformation was comparable to the frequency of patients who did not present with these features.     

不同类型先天性中耳畸形的手术治疗

探讨先天性中耳畸形的临床分型及其手术策略。方法选取2016年6月—2019年12月收治的23例（33耳）先天性中耳畸形患者进行中耳畸形临床分型及不同的手术策略进行回顾分析,观察术后疗效和听力的恢复情况。术前对23例（33耳）中耳畸形患者均行耳内镜检查、听力学检查及颞骨CT并听骨链三维重建检查。结果23例患者中18例为术前明确畸形,5例为术中探查发现畸形。其中15例为锤、砧骨畸形,选择人工听骨赝复物（TORP或PORP）或自体听骨植入修复;6例为镫骨畸形,选择人工镫骨赝复物（Piston）植入;1例为鼓膜穿孔伴有先天性中耳畸形,先行鼓膜成形术,二期行听骨链重建手术;1例畸形程度比较复杂,面神经走形异常,在前庭窗处分叉,遮挡前庭窗,未能行进一步治疗。术后气导阈值提高至正常的有15例（65.2%）;骨气导差（ABG）恢复至30 dB以内的有16例（69.6%）,ABG术后仍超过30 dB的有5例（21.7%）,1例听力未改善（4.3%）,1例无法行听骨重建（4.3%）。结论先天性中耳畸形术前诊断比较困难,CT检查很难精确评估,手术探查为最有说服力的诊断标准,明确中耳畸形临床分型后选择最佳的手术方式,是治愈疾病并提高患者术后听力最佳选择。     

The clinical evaluations of pathophysiology for congenital middle ear cholesteatoma

PURPOSE: Congenital middle ear cholesteatoma (CMEC) is less common than the acquired variety. Many theories have been put forward to explain the pathophysiology of CMEC; however, none of them have been convincingly proven thus far. This clinical study was performed to analyze the case reviews; assess the characteristic features of CMEC, and evaluate the correlation between those features and the pathophysiology of CMEC. METHODS: The medical records of patients who underwent otologic procedures at the hospitals of the Catholic University, Seoul, Korea, from January 1993 to November 1998 have been reviewed. RESULTS: Of the 14 patients, 3 had cystic lesions isolated to the anterosuperior quadrant of the mesotympanum. The lesions were easy to remove and did not affect the patients' hearing. Eleven patients had more extensive disease with posterior mesotympanum involvement; the lesions were large, were often too extensive to indicate a formative site, and caused ossicular damage. CONCLUSION: CMEC manifests in 2 distinctive forms according to the formation site. This distinctive formation site suggests that the pathophysiology of posterior lesions may be different from that of anterior ones. For early diagnosis of CMEC, a screening program should be established for children to prevent more extensive disease.