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[先天性中耳畸形:临床分析与分类探讨]

[Congenital middle ear malformation: clinical analysis and discussion of classification].

作者信息

Liu Y, Zhao D H, Lin Y S

机构信息

Department of Otorhinolaryngology Head and Neck Surgery, the Sixth Medical Center of People's Liberation Army General Hospital, Beijing 100048, China.

出版信息

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2019 Jul 7;54(7):481-488. doi: 10.3760/cma.j.issn.1673-0860.2019.07.001.

Abstract

To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

摘要

分析先天性中耳畸形的临床特征及合适的手术方法,并探讨其分类。所有病例均来自解放军总医院第六医学中心耳鼻咽喉科。所有病例,包括26例男性患者(耳)、10例女性患者(11耳),年龄7至57岁,外耳道、鼓膜正常,均有传导性听力损失,鼓室导抗图为A型,盖莱试验阴性。所有病例均行鼓室成形术。畸形分为三种类型,即I型(镫骨足板活动):Ⅰa,听骨链畸形但镫骨上部结构正常;Ⅰb,听骨链畸形且镫骨上部结构异常;II型(镫骨足板固定):Ⅱa,听骨链正常,Ⅱb,听骨链畸形;III型:前庭窗骨性闭锁或未发育,或伴有圆窗闭锁。II型和III型畸形可能伴有面神经异常。此外,回顾性分析1982年至2017年发表的有关中耳畸形的文献。总结451耳畸形的临床资料。根据我院37耳样本的修订分类标准,20耳为I型。6例Ⅰa型病例采用部分听骨赝复物(PORP)重建听骨链;14例Ⅰb型病例采用全听骨赝复物(TORP)重建听骨链。II型5耳,其中2耳为Ⅱa型,3耳为Ⅱb型。4例II型耳样本植入活塞式听骨假体,1例植入TORP,术中去除骨化足板并保留骨膜。12耳样本为III型,有前庭窗骨性闭锁、面神经畸形和镫骨上部结构畸形。3耳前庭窗植入活塞式听骨假体,面神经部分覆盖前庭窗。5耳手术放弃,4耳因面神经完全覆盖前庭窗,于鼓阶起始处保留骨膜开口植入TORP。30例有完整随访资料的患者无感音神经性听力损失,平均气骨导差下降23.3±10.7 dB(<0.05)。文献报道的451例先天性中耳畸形中,I型234耳。其中113耳为Ⅰa型,基本手术为听骨链松动及人工或自体PORP植入。Ⅰb型121耳,采用自体或人工TORP及PORP。II型125耳,包括Ⅱa型22耳,Ⅱb型60耳,43耳未细分。II型手术与耳硬化症相同。III型前庭窗闭锁92耳,17耳手术放弃,其余行前庭窗、岬或半规管开窗,用活塞、自体或人工TORP重建听力。结合合适的手术材料和方法参考先天性中耳畸形的分类,耳科医生能更好地理解并为中耳畸形选择合适的手术方法。

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