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.     

岩骨胆脂瘤的诊断与外科治疗

目的　探讨岩骨胆脂瘤的病因和临床表现特点以及手术方式。方法　对 1986年 12月～ 2 0 0 3年 4月收治的 12例岩骨胆脂瘤患者 (继发 9例 ,原发 3例 )进行回顾性分析。结果　原发岩骨胆脂瘤首发症状为面瘫及听力下降 ,鼓膜正常。继发岩骨胆脂瘤主要表现为耳流脓史 ,听力下降及面瘫 ,鼓膜通常有穿孔或不正常。慢性中耳炎病史及耳科手术史与继发性岩骨胆脂瘤的发生密切相关。颞骨CT可明确病变范围及与面神经的关系 ,能为确定手术方式提供直接的参考。继发及原发岩骨胆脂瘤的治疗原则相同 :彻底清除胆脂瘤上皮。手术入路有 4种 :经迷路、中颅窝、迷路中颅窝联合入路、颅颈联合入路 (迷路下 )。 1例继发胆脂瘤因反复复发而行 4次手术外 ,其余 11例随访 4个月～ 15年无复发。吻合的 3例面神经中 ,2例由HouseBrackmann分级Ⅴ恢复到Ⅳ ;减压及神经连续性完整的 3例中 2例由Ⅳ恢复到Ⅲ ,1例无恢复。结论　继发及原发胆脂瘤病因不相同 ,临床表现各具特点。手术进路的选择取决于病变部位、范围及听力状况 ,经迷路、中颅窝是主要入路。单纯中颅窝入路应采用术腔相对封闭的术式 ;其他入路应采取开放术腔式手术。     

岩骨胆脂瘤的诊断与治疗

目的　探讨岩骨胆脂瘤的诊断与治疗。方法　总结 5例 ( 5耳 ,其中 2耳先天性 ,3耳后天性 )岩骨胆脂瘤的临床表现 ,影像学结果及手术经验。结果　 5耳均有重度聋或全聋 ,3耳面瘫 ,3耳脑膜炎 ;颞骨CT扫描呈低密度边缘光滑的膨胀性缺损 ,磁共振成像 (magneticresonanceimaging ,MRI)中T1加权呈中等均一信号 ,T2加权呈高信号。增强扫描无强化。 4( 1耳先天性 ,3耳后天性 )岩骨胆脂瘤耳采用迷路进路 ,1耳先天性岩骨胆脂瘤采用颅中窝 迷路联合进路手术。术后随访 0 5～ 4年 ,无脑脊液漏 ,未见胆脂瘤复发 ,均获干耳。 3耳面瘫患者 ,1例完全恢复 ,1例Ⅱ级恢复 ,1例Ⅲ级恢复。结论　颞骨CT扫描对临床诊断具有重要作用 ,MRI具有鉴别诊断价值。对于无实用听力患者 ,迷路进路是最实用和理想的手术进路     

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。     

岩骨胆脂瘤的诊断与治疗

目的 探讨岩骨胆脂瘤的诊断与治疗。方法 总结5例(5耳，其中2耳先天性，3耳后天性)岩骨胆脂瘤的临床表现，影像学结果及手术经验。结果 5耳均有重度聋或全聋，3耳面瘫，3耳脑膜炎；颞骨CT扫描呈低密度边缘光滑的膨胀性缺损，磁共振成像(magnetic resonance imaging，MRI)中Tl加权呈中等均一信号，T2加权呈高信号。增强扫描无强化。4(1耳先天性，3耳后天性)岩骨胆脂瘤耳采用迷路进路，1耳先天性岩骨胆脂瘤采用颅中窝-迷路联合进路手术。术后随访0．5～4年，无脑脊液漏，未见胆脂瘤复发，均获干耳。3耳面瘫患者，1例完全恢复，1例Ⅱ级恢复，1例Ⅲ级恢复。结论 颞骨CT扫描对临床诊断具有重要作用，MRI具有鉴别诊断价值。对于无实用听力患者，迷路进路是最实用和理想的手术进路。     

岩骨胆脂瘤诊断治疗初探

目的总结岩骨胆脂瘤诊断和治疗的经验。方法结合文献对2例岩骨胆脂瘤患者术中术后的情况进行分析。结果1耳经迷路上径路切除病变,术中行面神经减压,采取开放术腔术式,术后面瘫由Ⅵ级恢复至Ⅴ级,听力无改善;1耳经迷路径路全切病变,术中行面神经移植,采取闭封术腔术式,面瘫无恢复,听力完全丧失。术后至今分别4年、2年,2耳均无复发。结论手术入路应根据病变情况而定,开放术腔和闭封术腔各有优点,也应根据手术情况灵活采用。     

颞骨先天性胆脂瘤的诊断和治疗

目的分析总结颞骨先天性胆脂瘤的病变部位、范围、大小、临床表现、影像学资料、诊断、鉴别诊断及手术经验,包括受损面神经恢复情况。方法回顾性分析1999年1月至2013年4月诊治的10例颞骨先天性胆脂瘤患者的临床资料和诊治规律。结果 10例颞骨先天性胆脂瘤患者中,年龄为1⁵7岁,男7例,女3例,其中患耳为左侧5例,右侧5例。病变位于乳突区6例,其中1例患者外耳道后壁受累破坏;病变累及乳突区、岩尖4例,术前出现面瘫,面神经骨管均已破坏、裸露,其中1例术中出现脑脊液漏,予同期行脑脊液漏修补术。本组均采取手术治疗的方式,9例行改良开放式乳突根治术,其中1例术中同期行面神经减压+鼓室成形术;1例为人工耳蜗植入患者同期行闭合式乳突凿开鼓室成形术,同期植入人工耳蜗电极,术后语训恢复良好。所有病例术后1年复查CT等没有发现胆脂瘤残留或复发。结论对先天性胆脂瘤通过病史、听力学、影像学资料等进行鉴别诊断,可以做到早期诊断,并选择合适的手术入路。特别是对于岩部先天性胆脂瘤,在出现面瘫等并发症,及时通过CT、MRI等影像学资料,做到早期诊断、早期治疗,可能会避免误诊和漏诊。     

20例先天性中耳胆脂瘤的临床和影像学特征分析

目的：探讨先天性中耳胆脂瘤的位置、分期、临床特征、影像学特征及手术治疗。方法：回顾分析20例先天性中耳胆脂瘤患者的资料。结果：20例先天性中耳胆脂瘤患者Potsic分期I期2例,Ⅱ期0例,Ⅲ期13例,Ⅳ期5例。传导性聋为最常见的临床症状,术前平均纯音听阈为54．1dB,气骨导差为41．7dB。其中1例患者行完壁式乳突根治二期行听骨链重建;2例因病变范围广泛行乳突根治术未行听力重建;5例行完壁式乳突根治一期鼓室成形术,其中1例为先天性听骨链畸形（镫骨上结构未发育）;12例均行一期鼓室成形术。术中探查胆脂瘤主要位于中后鼓室和后上鼓室,主要是砧镫关节处。所有患者术后随访至少1年,对16例患者进行听力学检查,术后平均听阈为35．3dB,气骨导差20．2dB。2例患者复发,3例伴有先天性听骨链畸形。结论：先天性中耳胆脂瘤临床少见,病变隐匿常被延误诊断,其预后与病变临床分期密切相关。     

颞骨先天性胆脂瘤11例分析

目的 探讨颞骨先天性胆脂瘤的病变部位、范围、临床症状、诊断及手术方式.方法 回顾性分析1998年1月～2009年1月诊治的11例颞骨先天性胆脂瘤患者的临床资料.结果 11例先天性中耳胆脂瘤患者的年龄为9～38岁,中位数为15岁,男6例,女5例,术前平均听阈为39 dB HL,气-骨导差为20～55 dB.病变仅位于鼓室...     

颞骨先天性胆脂瘤并发周围性面瘫的诊治

目的探讨合并面瘫的颞骨先天性胆脂瘤的诊断及治疗。方法对2001年4月至2014年4月间在我科手术治疗的7例患者的临床资料进行回顾分析。结果所有病例术后恢复良好,无并发症,随访2月-14年胆脂瘤无复发。面瘫完全恢复者3例,好转者2例,无改善2例。术后患耳全聋者5例,3例为就诊时已确诊全聋,另2例为因胆脂瘤侵入内耳而行迷路部分切除术;1例听力提高15dB;1例无变化。结论早期诊断是降低先天性胆脂瘤面瘫发生率及获得良好术后效果的重要保障。     

螺旋CT在胆脂瘤型中耳炎诊断中的应用

目的：探讨颞骨螺旋CT在胆脂瘤型中耳炎诊断中的作用。方法：回顾性分析经手术及病理证实的104例胆脂瘤型中耳炎患者的CT资料,将手术结果与CT表现进行对比分析。结果：螺旋CT能清楚地显示起源部位不同和大小不等的胆脂瘤的软组织影充填征象及引起的中耳系统细微的骨质破坏征象。术前CT诊断骨壁受侵101耳,而术中发现为90耳,平均符合率为89．1％;术前CT显示胆脂瘤在中耳系统充填部位为108耳,与手术结果平均符合率为94．4％;术前CT诊断听骨链受侵为234耳,而术中证实为209耳,平均符合率为89．3％。结论：螺旋CT对胆脂瘤型中耳炎的诊断,尤其对判定骨质破坏的范围和软组织充填的部位,制定手术方案和评估治疗效果,具有重要的指导意义。     

Predictive validity of MRI in detecting and following cholesteatoma

High recurrence rate of the middle ear cholesteatoma requires regular postoperative follow-up. This study evaluated data from the patients investigated with DW MRI to ascertain (1) the strength of the technique in detecting primary, and residual recurrent cholesteatoma, and (2) its accuracy in differentiating cholesteatoma from postoperative tissue changes. The diagnostic accuracy of two different DW imaging (EPI and non-EPI) techniques was evaluated. The data have been collected prospectively from 33 consecutive patients with either primary cholesteatoma, or with suspicious symptoms for potential cholesteatoma recurrence. The findings from non-EPI (HASTE) DW MR and EPI DW MR images were blindly compared with those obtained during a primary or secondary surgery. Preoperative non-EPI (HASTE) DWI pointed to a cholesteatoma in 25 out of 33 patients. In this subgroup, cholesteatoma were confirmed also by the surgery. In five cases, the non-EPI (HASTE) DWI did not show a cholesteatoma in the temporal bone, which agreed with the surgical findings. Three misclassifications were made by non-EPI (HASTE) DWI, all in the subgroup of patients indicated for primary surgery. The resulting pooled sensitivity of non-EPI (HASTE) DW imaging for diagnosing cholesteatoma in our study amounted to 96.15% (95% confidence interval (CI) 80.36–99.9), specificity was 71.43% (95% CI 29.04–96.33). Positive predictive value was 92.59% (95% CI 75.71–99.09) and negative predictive value 83.33% (95% CI 35.88–99.58). In conclusion, we recommend the non-EPI (HASTE) DW MRI as a valid method for diagnosing cholesteatoma and follow-up after cholesteatoma surgery.     

先天性中耳胆脂瘤临床诊治及误诊分析

目的　探讨先天性中耳胆脂瘤的诊断、治疗方法以及误诊原因,减少漏诊、误诊。方法　对我院收治的9例先天性中耳胆脂瘤患者临床资料进行回顾性分析。结果  9例均行手术治疗,病理确诊,其中6例行听力重建,随访3个月～2年半,气骨导差为20 dB左右,术后1年者复查颞骨CT均未发现胆脂瘤残留及复发。所有患者均有误诊病史,误诊率100%。结论　先天性中耳胆脂瘤临床少见,起病隐匿,易漏诊、误诊,诊断可根据Levenson诊断标准和影像学检查,早期手术治疗可获得较好听力重建效果。提高对本病的认识,影像学检查和仔细的局部检查可提高诊疗水平,避免误诊、误治,避免严重并发症发生。     

Unusual cases of congenital cholesteatoma of the ear.

Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.     

儿童先天性胆脂瘤临床误诊分析(附3例报告)

目的 探讨儿童先天性胆脂瘤的临床特征及早期诊断。方法 通过复习文献并对3例儿童中耳胆脂瘤的临床表现进行分析。结果 该组病例属先天性胆脂瘤;当儿童出现耳痛、听力减退及其并发症时,虽然检查鼓膜完整,亦应做全面而仔细的耳部检查,颞骨CT扫描可对本病做到早期确诊。结论一经确诊,应早期行乳突根治及鼓室成型术治疗,其原则是彻底清除病变组织,保护面神经,保存或提高现有听力。     

The formation of sinus in congenital stenosis of external auditory canal with cholesteatoma

CONCLUSIONS: In congenital stenosis of the external auditory canal (CSEAC) with cholesteatoma, the bony wall of the external auditory canal (EAC) is most commonly involved. This involvement will lead to bone erosion of the EAC and may subsequently lead to the formation of postaural or cervical sinuses. High-resolution computed tomography (HRCT) of temporal bone can show characteristic signs of soft tissue mass in EAC, with adjacent bone erosion. OBJECTIVE: To investigate the clinical features, differential diagnosis and management of CSEAC with cholesteatoma. PATIENTS AND METHODS: The clinical information for 10 cases of CSEAC with cholesteatoma was retrospectively reviewed. RESULTS: The patients' ages ranged from 4.75 to 22 years (average 12 years). The diameter of EACs was < 2 mm. All 10 ears had a history of postaural fistulae or sinuses. Bone erosion of EAC was distinctly shown in HRCT of all cases, as well as soft tissue masses, which led to enlargement of the bony canals. All patients underwent canaloplasty; eight ears received hearing reconstructions at the same time. Cholesteatoma in EACs was confirmed during the operations, accompanied by compression and destruction of the post-superior and/or inferior bony wall. Postoperative pathologic examinations proved the diagnosis of cholesteatoma, and excluded any tissue of bronchial cleft cyst or fistula. After a follow-up of 1-3 years, no recurrent cholesteatoma was found in any of the 10 cases. All reconstructed EACs were clean and smooth. The hearing levels in the eight ears that received hearing reconstructions increased 20-35 dBHL.     

CT对慢性化脓性中耳炎的诊断意义

目的探讨中耳和乳突不同病变组织术前诊断和鉴别诊断方法。方法回顾分析106例慢性化脓性中耳炎（含胆脂瘤中耳炎）患者颞骨薄层CT显示的病变组织，并进行183次CT值测量，与术中发现、术后病理进行对比分析，得出不同病变组织的CT值范围。从中随机抽取60例，由同一组医师根据不同病变组织CT值及周围组织破坏的情况，重新分析做出诊断，将其诊断符合率与放射科常规诊断报告进行比较。根据临床表现和颞骨薄层CT扫描结果对9例慢性化脓性中耳炎患者行预测性诊断，以验证本研究结果对于中耳、乳突不同病变组织术前诊断与鉴别诊断的价值。结果胆脂瘤、肉芽组织、胆脂瘤并肉芽组织、炎性渗出、硬化或钙化组织、黏膜增厚并息肉样变组织的CT值分别为：（46．6±10、3）Hu、（26．6±7．4）Hu、（42．1±11．4）Hu、（-24．6±9．2）Hu、（223．6±63．7）Hu、（23．8±8．5）Hu。根据不同病变组织CT值大小及周围组织的破坏情况对60例患者重新分析诊断，诊断符合率从68．3％提高至81．7％（Х^2=3．96，P〈0．05）。根据临床表现和颞骨薄层CT扫描所见进行预测性诊断，诊断符合率达90．0％。结论CT值虽不能作为诊断和鉴别诊断中耳、乳突不同病变组织惟一可靠的依据，但不失为一种很有意义的线索；根据临床表现、颞骨薄层CT扫描所见对中耳各种病变进行综合分析，可显著提高中耳、乳突病变诊断和鉴别诊断的准确率。     

颞骨CT和咽鼓管功能检查对胆脂瘤型中耳炎咽鼓管鼓室口病变的诊断意义

目的：探讨颞骨高分辨率CT（HRCT）和咽鼓管功能检查对胆脂瘤型中耳炎患者术前咽鼓管鼓室口病变的诊断价值。方法：回顾性分析38例（41耳）胆脂瘤型中耳炎患者术前颞骨HRCT扫描和咽鼓管功能综合测试仪检查结果，并与术中显微镜下探查咽鼓管鼓室口处病变情况进行对比分析。结果：颞骨HRCT发现咽鼓管鼓室口软组织占位影34耳；术中发现鼓室口病变32耳，其中为肉芽组织22耳和胆脂瘤4耳，脓团堵塞3耳，鼓室口周围黏膜肥厚肿胀2耳，黏膜粘连闭锁1耳。咽鼓管功能障碍37耳，其中为阻塞型32耳，闭锁不全型5耳。结论：对胆脂瘤型中耳炎患者术前进行颞骨HRCT扫描和咽鼓管功能检查，对术中有目的地处理咽鼓管鼓室口的病变，制定手术方案和预估治疗效果具有重要意义。     

Intracranial Extension of Acquired Aural Cholesteatoma

Objective Cholesteatoma of the petrous bone extending into the intracranial region is an unusual occurrence. Most cases have been attributed to secondary extension of a primary epidermal blastomatous malformation of the temporal bone into the middle or posterior fossae. Within the past two and a half decades, intracranial extension of acquired aural cholesteatoma has been recognized as a likely alternative to this mechanism. Recent literature has rejoined this observation by considering both primary and secondary cholesteatoma of the petrous bone as a single group, petrosal cholesteatoma. The present study is presented to analyze the clinical presentation, imaging findings, and surgical treatment of six patients with acquired aural cholesteatoma extending into the intracranial region. Findings in this study are compared with the extant literature on congenital and acquired cholesteatoma of the petrous bone. This study proposes that petrosal cholesteatoma is a valid anatomical construct; however, the pathogenesis of petrosal cholesteatoma is still important in understanding the clinical presentation and management of cholesteatoma that extends beyond the usual confines of the middle ear and mastoid. Study Design Retrospective case review conducted at a tertiary referral center. Methods From 1985 to 1999, 477 patients were surgically treated for acquired aural cholesteatoma. Patients with intracranial extension of cholesteatoma were studied. Clinical presentation, imaging studies, operative findings, surgical treatment, and postoperative results were evaluated. Results Six cases in a series of 477 patients with acquired aural cholesteatoma had intracranial extension of disease. In this series, the most frequent pathway for intracranial extension was supralabyrinthine through the supratubal recess into the middle cranial fossa. A less frequent pathway was via the retrofacial air cells into the posterior cranial fossa. Surgical access for removal of intracranial cholesteatoma was accomplished through several approaches including translabyrinthine, transcochlear, retrolabyrinthine, and middle cranial fossa. In two patients who had reoperation for possible residual disease, one was free of residual disease and one was found to have residual cholesteatoma in the region of the horizontal facial nerve. Conclusion Acquired aural cholesteatoma can extend into either the middle or posterior cranial fossae. In this study, cholesteatoma extended into the middle fossa through the supratubal recess along the labyrinthine facial nerve and into or above the internal auditory canal. A less frequent path is through the retrofacial air cells into the posterior fossa. Intracranial acquired cholesteatoma is generally small and presents with complaints related to underlying otitis media rather than the neurological deficits that are often associated with primary petrous bone cholesteatoma. While computed tomography and magnetic resonance imaging are both required to differentiate congenital petrous cholesteatoma from other lesions of the petrous bone, computed tomography of the temporal bone is usually sufficient to diagnosis and define intracranial extension of acquired aural cholesteatoma. These lesions can be completely excised rather than exteriorized.