单纯性听骨链畸形的临床诊治探讨

Study on clinical diagnosis and treatment of simple ossicular chain deformity

摘要:

摘要：目的探讨单纯性听骨链畸形的临床诊断与治疗。方法通过对2014年1月~2016年1月收治的6例单纯性听骨链畸形患者的临床资料进行分析，总结探讨其临床诊治。结果6例患者均表现为中度到中重度传导或混合性耳聋。手术探查发现6例患者中，砧骨长脚缺失1例，砧镫关节脱位1例，术中通过部分人工听骨（partial ossicular replacement prosthesis,PORP）进行听力重建；砧骨长脚、镫骨上结构缺失，而镫骨足板活动可1例，术中通过全人工听骨（total ossicular replacement prosthesis,TORP）进行听力重建；镫骨足板固定1例，术中通过镫骨足板开窗，人工镫骨（piston）进行听力重建；砧、镫骨畸形并面神经骑跨前庭窗表面1例，谈话后未行听力重建；砧、镫骨畸形1例，面神经骑跨前庭窗下方，术中行piston听力重建。听骨形态异常患者在术前通过中耳HRCT可初步判断，听骨关节脱位、镫骨固定则需在术中探查以明确诊断。结论准确的听力学检查是单纯性听骨链畸形诊断的第一道门槛，而耳乳突HRCT检查对诊断亦有重要作用，临床中对于难以解释的传导性耳聋必要时行鼓室探查术，避免漏诊。

Abstract:

Abstract:ObjectiveTo investigate the diagnosis and treatment of simple malformation of ossicular chain.MethodsDiagnosis and treatment of simple ossicular chain malformation was summarized by analyzing clinical data of 6 cases suffering from this malformation diagnosed in our department from Jan 2014 to Jan 2016.ResultsAudiological tests showed moderate to moderatesever conductive and mixed hearing loss in all 6 cases. Surgical exploration confirmed absence of incuse long process in one case and dislocation of incudostapedial joint in another, and their malformations were reconstructed by partial ossicular replacement prosthesis (PORP). Absence of incuse long process and superstructure of stapes and without fixation of stapedial footplate was found in one case and the hearing was reconstructed by total ossicular replacement prosthesis (TORP). One patient with fixed stapedial footplate got hearing reconstruction by fenestration of the footplate and implantation of Piston. Malformations of incuse and stapes combined with facial nerve straddling vestibular window existed in one case, but hearing reconstruction was declined by the patient’s family. Hearing was reconstructed by implantation of Piston in one patient with malformations of incuse and stapes and ectopic facial nerve inferior to vestibular window. Morphological abnormalities of ossicular chain could be diagnosed by preoperative highresolution computed tomography (HRCT) of middle ear, while dislocation of ossicular joint and fixation of stapes could not be cleared before exploratory operation.ConclusionAccurate audiological examination is the first threshold for the diagnosis of simple ossicular chain deformity. HRCT examination also plays an important role in the diagnosis. To avoid missed diagnosis of simple ossicular chain deformity, tympanotomy is necessary for difficulttoexplain conductive deafness.