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目的探讨先天性中耳畸形的临床分型,以及不同类型的先天性中耳畸形与耳聋的相关性,以利于术前中耳畸形的诊断和术式的选择。方法回顾性分析解放军总医院1995年3月-2004年5月收治的经手术证实为单纯先天性中耳畸形的病例(64例,82耳)。根据中耳组织胚胎学发育及手术探查中耳畸形情况进行临床分型,统计学检验各型先天性中耳畸形听阈的差异。结果根据中耳结构组织胚胎学的发育,将先天性中耳畸形分为:A1:先天性锤砧骨畸形;A2:先天性砧镫骨畸形;B型:先天性镫骨固定;C型:先天性前庭窗或蜗窗发育不全或闭锁。听力学检查显示A、B、C3组间在语言频率上差异无统计学意义(P=0·1617),而高频(>2KHZ)的听阈B型及C型与A型均具有统计学意义(P<0·05),并且B、C型先天性中耳畸形存在骨导下降及混合聋。结论中耳结构的组织发育来源不同,在临床上不同类型的先天性中耳畸形累及的范围及程度存在很大差异,从而导致不同程度的传导性聋或混合性聋,其听阈不仅决定于听骨链是否完整,还在于畸形的部位和累及的范围。骨导听阈与气导高频听阈有助于鉴别不同类型的先天性中耳畸形。
Objective To investigate the clinical classification of congenital middle ear deformities and the correlation between different types of congenital middle ear deformities and deafness so as to facilitate the diagnosis and surgical choice of preoperative middle ear deformities. Methods A retrospective analysis was performed on 64 patients (82 ears) with simple congenital middle ear deformity confirmed by surgery from March 1995 to May 2004 in PLA General Hospital. The clinical classification of middle ear deformities was made according to the development of embryonic middle ear tissues and surgical exploration. The differences of hearing thresholds of various types of congenital middle ear deformities were statistically tested. Results According to the development of middle ear histology and embryology, congenital middle ear deformities were divided into: A1: congenital hammer anvil deformity; A2: congenital anvil stump deformity; B type: congenital stapes fixation; C type: Congenital vestibular window or dilated or locked. The audiological examination showed no significant difference in speech frequency between A, B and C3 groups (P = 0.1617), while the high-frequency (> 2KHZ) threshold B, C and A were statistically significant (P <0.05), and there was a decrease in bone conduction and mixed deafness in type B and C congenital middle ear deformities. Conclusions There are different sources of tissue development in the middle ear. There are great differences in the scope and extent of different types of congenital middle ear deformities, which leads to different degrees of deaf or mixed deafness. Whether the bone chain is intact, but also lies in the area and the scope of deformity. Bone conduction threshold and airway high-frequency hearing threshold help to identify different types of congenital middle ear deformity.