Middle ear adenoma with neuroendocrine differentiation (MEA-ND) in the pediatric population

Middle ear adenoma with neuroendocrine differentiation is an uncommon tumor of the tympanic cavity. The terminology of this entity has suffered due to a poor understanding of the differentiation of these neoplasms, and has included both “adenoma” as well as “carcinoid tumor.” Immunohistochemical techniques have helped clarify that these tumors all share elements of neuroendocrine differentiation. Occurrences of this tumor in the pediatric population are extremely rare. In the world literature, only three pediatric cases of middle ear adenoma with neuroendocrine differentiation have been described. We report the youngest case to date of middle ear adenoma with neuroendocrine differentiation, in a 13-year-old boy. We discuss the differential diagnosis, surgical management, and pathology of this entity.     

一例中耳腺瘤的诊疗体会并文献复习

正中耳腺瘤是一种临床少见的具有外分泌和神经内分泌分化的中耳原发性肿瘤。1976年由Hyams和Michaels首先报道~([1]),至今国外报道的病例仅54例,国内中耳腺瘤~([2-3])的报道不足10例。因其发病率低(约占耳部肿瘤的4%),病例数少,临床工作者对该病的认识不足。现报告2015年12月我院收治的一例中耳腺瘤,其临床特点与目前已有的文献报道相比有所不同,我们对其进行了系统的回顾性分析并结合文献复习,拟为临床诊治中耳腺瘤提供借鉴。     

罕见中耳巨大胆脂瘤1例

中耳胆脂瘤是一种常见的慢性中耳疾病,特征是中耳腔、乳突腔及岩尖存在高度增殖的角化鳞状上皮和邻近骨质的破坏吸收,上皮细胞脱落、上皮碎屑堆积而形成胆脂瘤[1].现将我科收治的1例罕见巨大胆脂瘤病历资料报告如下.     

Middle ear hemangiona: a case report

A middle ear hemangioma was detected in a 51-year-old man who suffered from fullness and hearing loss in the left ear. This case is presented with a review of the relevant literature. The mass was surgically resected for diagnostic and therapeutic reasons, and pathologic examination revealed a benign hemangioma. The CT, MRI, angiography, and light microscopy findings of the tumor are documented and compared with those a glomus tumor. The significance of this diagnosis is also discussed.     

中耳胆固醇肉芽肿

目的 探讨中耳胆固醇肉芽肿的病因、发病机制以及治疗方法。方法 回顾性总结分析我院1988年3月～2003年3月经手术和病理证实的70例(耳)中耳胆固醇肉芽肿病人的临床资料。70例(耳)中，术前诊断为胆脂瘤型中耳炎37例，骨疡型中耳炎13例，此二者之中考虑胆固醇肉芽肿者13例；中耳乳突术后感染5例，分泌性中耳炎7例，特发性血鼓室8例，其中考虑可能为胆固醇肉芽肿者2例。所有病人均有不同程度听力下降、耳闭塞感或伴耳鸣，亦可有耳溢液、头痛、头昏及其他症状。70例(耳)均接受手术治疗，依照术前检查，根据病变程度不同而选择不同术式。结果 65例(耳)干耳，随访1年以上无复发。3例(耳)行改良乳突根治加鼓室成形术，术后半年流脓，后改行乳突根治术，术后均干耳；2例(耳)行鼓室探查加鼓窦开放术，术后半年及1年又流脓伴听力下降，后改行改良乳突根治加鼓室成形术，术后干耳。结论 中耳胆固醇肉芽肿的病因主要有：含气腔通气受阻、引流障碍及含气腔出血。中耳胆固醇肉芽肿与特发性血鼓室、分泌性中耳炎及胆脂瘤关系密切。术式的选择应根据病变范围和程度来决定，其原则是彻底清除病变，保持通畅引流。     

钛环植入豚鼠中耳腔的生物相容性研究

为给临床上利用钛质听骨行鼓室成形术提供观察依据，将金属钛环按不同时间组分别植入２３０只豚鼠的中耳腔嵌入锤骨头上，对中、内耳组织结构进行光镜和扫描电镜观察，并检测动物的血液生化指标、鼠毛中钛元素的含量和听阀的改变。结果表明，钛环植入前后运物的肝肾功能及鼠毛中钛元素的含量均无明显的变化。１０ｄ和１个月组中耳粘膜有血迹和少量的炎性细胞浸润；扫描电镜观察纤毛结构紊乱，随时间推移逐渐恢复正常。植入前后相比，     

Standardized measurements of the sound transmission of middle ear implants using a mechanical middle ear model

Several ways to evaluate the sound transmission properties of middle ear implants are now established. Besides computer-based simulations using acoustic and electrical analog circuits or finite element analysis, measurements can be performed with temporal bone preparations. Experiments with these preparations consider various anatomical properties, but a large number of parameters influence the outcome of measurements. To facilitate standardized measurements, a mechanical middle ear model was developed that allows comparison of the transfer function of middle ear implants on defined conditions. The model approximates the impedances of the tympanic membrane and inner ear with the aid of thin, flexible membranes. The implants are fit between the membranes, and displacement at an artificial stapes footplate is measured with an optical probe. Fundamental influences on the sound transmission properties of nine different middle ear implants (total ossicular replacement prostheses) were examined. Although the material and shape were different, some of the prostheses revealed very similar transfer functions. The mass of the implant showed the largest influence on sound conduction. With a higher mass, the frequency area above approximately 1 kHz was found to be significantly deteriorated. The lightest implant used was 4 mg and showed the best overall results. These findings show that middle ear prostheses should be as light as possible for optimum high-frequency transmission. Received: 24 May 1998 / Accepted: 9 July 1998     

Primary mucosal malignant melanoma of middle ear — A case report

Primary mucosal malignant melanoma reported in this case is a rare clinical entity. Very few cases of primary mucosal malignant melanoma of middle ear have been reported in the literature till date. The etiopathology, clinical features and management of this disease has been described here.     

中耳内窥镜解剖

目的 :探讨经不同途径的中耳内窥镜解剖所见。方法 :采用 0°,30°,90°的硬质内窥镜 ,对 17个颞骨标本进行了观察 (其中干标本 6个 ,湿标本 11个 ) ,内窥镜插入途径有 :外耳道 ;耳后切口 (包括乳突腔 鼓窦和乳突腔 后鼓室两条亚途径 ) ;中颅窝。结果 :外耳道途径 :采用 0°,30° ,90°的硬质内窥镜 ,所有鼓室结构都可看见 ,包括鼓室窦、面隐窝、上鼓室前隐窝等隐蔽处 ;乳突腔 鼓窦途径 :可以看见乳突腔、鼓窦、上中鼓室等结构 ;乳突腔 后鼓室途径 :可观察到乳突及中下鼓室结构 ;中颅窝途径 :可以观察到上鼓室、鼓窦、乳突腔及鼓室外侧壁的结构 ,中鼓室的大部分结构亦可观察到。结论 :经不同的途径行内窥镜术能清楚地观察到中耳各个结构 ,因此能减少胆脂瘤的复发率及评价术后中耳腔的状态 ,其中 30°的内窥镜提供的视野较好 ;但如经外耳道途径观察后上鼓室及经乳突腔 鼓窦途径观察后鼓室 ,则 90°内窥镜较适合     

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。     

Accessory tragus in the middle ear: A rare congenital anomaly

Objective

We present a very interesting and extremely rare case of an accessory tragus in the middle ear.

Method

Case report and review of literature.

Results

Accessory tragus is not an uncommon congenital malformation of the external ear. The exact prevalence of accessory tragus as an isolated physical finding is unknown but it has been estimated to be 1.7:1000. There are many case reports on accessory tragus but there is no report of accessory tragus being found in the middle ear in English literature. We report the presentation and discuss the radiology and histopathology findings of this interesting case.

Conclusion

To our knowledge, this is the first one to be reported in the English literature.     

单纯中耳畸形的临床分析

目的 :分析单纯中耳畸形的临床和听力学特征 ,手术探查明确畸形形式 ,避免和减少对该病的误诊、误治。方法 :9例 11耳单纯性中耳畸形患者均行鼓室探查术 ,部分行听骨链重建术 ,其中 ,行镫骨切除加钢丝结绨组织栓人工听骨重建 4耳 ,前庭开窗加人工听骨重建 2耳 ,砧镫关节松解术 1耳 ,高分子材料TORP重建听骨1例 ,蜗窗不明确的 3耳 ,仅做鼓室探查。结果 :术中发现砧骨畸形最多 (10 / 11) ,豆状突细小或缺失 ,砧骨游离 ,长突缺失、变细或变粗 ;镫骨缺如 (2 / 11) ;其他 (7/ 11)包括镫骨不完整或游离、固定、前后脚融合 ;砧镫关节粘连(1/ 11) ;面神经和前庭窗无法辨认 (4 / 11) ;鼓岬表面凹凸不平 ,无法辨认圆窗 (3/ 11)。 3例并发其他全身异常。听骨重建后患者听力满意 ,纯音测听示低频比高频提高明显 ,言语频率平均提高 2 4dB。无并发症发生。结论 :单纯先天中耳畸形最终确诊靠鼓室探查术 ,一定条件下镫骨切除或前庭开窗加钢丝结绨组织栓人工听骨重建术可满意提高听力     

Descriptive study of goat external and middle ear through computed tomography and endoscopic evaluation,compared with the human ear

ObjectiveTo describe the external and middle ear of goats, using computed tomography and endoscopic dissection, comparing them with the anatomy of the human ear, aiming to evaluate the viability of this animal model for training in otological endoscopic surgery.MethodsImages of the external and middle ear of goats were produced by computed tomography in the coronal and axial planes and analyzed. The radiological anatomy of the external and middle goat and human ear are described. Some measures of surgical interest were described, such as the length of the external auditory canal, external and internal cross-sectional area of the external auditory canal, middle ear depth, vertical angle of the external auditory canal, length of malleus and incus. After the tomographic study, we analyzed the importance of evaluations through the endoscopic dissection of the goat ear.ResultsThe bony canal is extremely tortuous and shows a bony prominence with significant elevation near the lower edge of the tympanic membrane. The middle ear has a large tympanic bulla in the hypotympanum region. The malleus is shaped similarly to that of the human, but its handle is located anterior to its head. The mastoid is not pneumatized. Compared to the human mastoid, there was a statistically significant difference in the external and internal cross-sectional area, at the depth of the middle ear, in the vertical angle of the canal and in the length of the incus.ConclusionGoat morphometric anatomy showed that this model is suitable for surgical simulation in the initial training for endoscopic otological surgery. It is an important contribution to the development of skills to accelerate the subsequent endoscopic otological surgical training in humans, despite some differences found.     

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     

Multiple osteomas in the middle ear

Osteomas in the middle ear are extremely rare and are generally solitary and small. We present a case of a 28-year-old female patient with multiple middle ear osteomas which arose from the promontory, epitympanum, and lateral semicircular canal. The osteomas were successfully removed by tympanomastoidectomy. This is the first reported case of a patient with middle ear osteomas arising from multiple sites.     

Adenoma of the middle ear mucosa

Benign adenomas of the middle ear are rare tumors. To date, approximately 100 cases of adenomatous neoplasms of the middle ear space have been collected in the United States Armed Forces Institute of Pathology, Washington D.C. This report describes two cases of primary adenomatous neoplasms confined to the middle ear cleft seen at the Department of Otorhinolaryngology at Ludwig Maximilians University Hospital, Munich. Although tumors are derived from lining epithelium of the middle ear mucosa, the unique feature of our first case was based on its origin in the epitympanic part of the tympanic membrane. Histopathology was characterized by an adenomatous morphology without atypia or mitoses, proving its benign behavior. No evidence was found for invasion of adjacent tissue. Simple surgical removal afforded excellent prognosis. Clinical behavior, histological appearance, origin of tumors and differential diagnosis are discussed.     

Changes in tympanograms after middle ear inflation

Summary To determine the effects of politzerization or middle ear (ME) inflation by a catheter, we studied the membranes (29 patients). Serial tympanometries were performed prior to the ME inflation and immediately, 10, 20, 30 and 60 min after the procedure. The present study showed that the more negative the ME pressure was before the inflation, the greater the pressure change was after the treatment. The elevated ME pressure induced by the procedure declined rapidly within the first 20 min after the inflation. This was probably caused mainly by spontaneous elimination of ME gas through the eustachian tube and gas absorption from the ME mucosa. During the post-inflation course, some ears demonstrated more negative pressures in the ME cavity than those before the inflation. The present investigation demonstrated that the use of inflation to treat secretory otitis media had short-term benefits which could be improved by minimizing gas absorption.     

Choristoma of the middle ear

Choristoma is a mass of tissue histologically normal for an organ or part of the body other than the site at which it is located. A rare case of ectopic salivary gland choristoma in the middle ear is described in a 4-year-old boy whose only symptom was a 50 dB conductive hearing loss in the presence of a normal tympanic membrane.