15例先天性中耳畸形临床特点及治疗

Diagnosis and management of congenital ossicular abnormality 15 cases report

目的分析单纯中耳畸形的临床特点,探讨先天性单纯中耳畸形的诊断治疗方法。方法先天性中耳畸形患者15例(15耳)。男11例,女4例。年龄11～37岁,平均年龄(20.4±6.21)岁,单侧发病7例,双侧发病8例。平均气骨导差(50.33±7.73)dB HL,均行颞骨薄层CT扫描。显微镜下行鼓室探查及鼓室成形术,根据不同的畸形情况采用相应的听骨链重建技术。结果 15例中11例颞骨CT显示不同程度的听骨链异常。术中进行镫骨足板开窗或足板全切除4例(4耳)、前庭开窗2例(2耳)、外半规管开窗2例(2耳)、镫骨上结构存在、足板活动正常,行IIIa型鼓室成形术7例(7耳)。结论影像学检查对先天性中耳畸形的诊断十分重要,手术是主要治疗手段,多数病例可通过手术重建听骨链,恢复中耳传音功能,提高听力。但手术涉及内耳比例高,病例选择要慎重同时要做好充分术前准备。

OBJECTIVE To analyze the clinical features and the treatment of congenital ossicular abnormality.METHODS Fifteen cases of congenital ossicular abnormalities（15 ears）were retrospectively studied,including 11 males and 4 females.They were aged 11～37 years,mean age（20.4±6.21）years.There were seven cases of unilateral disease and 8 cases of bilateral disease.The average air-bone gap was（50.33±7.73）dBHL.All cases underwent thin layer CT scan of temporal bone.Exploratory tympanotomy was undergone in all patients.The corresponding ossicular chain reconstruction was chosen depending on the deformity.RESULTS Eleven patients showed different degrees of ossicular chain abnormalities by temporal bone CT.Stapes footplate fenestration or resection of foot plate were made in 4 cases（4 ears）,vestibular window fenestration in 2 cases（2 ears）,lateral semicircular canal fenestration in 2 cases（2 ears）,IIIa type tympanoplasty in 7 patients（7 ears）.CONCLUSION The imaging diagnosis of congenital middle ear malformations is important,surgery is the main treatment to restore middle ear acoustic function and improve hearing.Ossicular reconstruction can be done in most cases.However,a high proportion of surgery involving the inner ear,case selection should be careful and full preparation should be made at the same time before surgery.