先天性无综合征听骨畸形与听骨链重建

Ossicular deformity without syndrome and ossiculoplasty

目的 探讨常见先天性听小骨畸形的临床类型与听骨链重建的方法。方法 2 5例 ( 39耳 )听骨链畸形 ,其中男 9例 ,女 1 6例 ,年龄 8～ 5 4岁 ,平均 ( 2 4 8± 1 2 3)岁。双侧传导性聋 2 8耳 ,单侧 1 1耳 ,其中左 5耳 ,右 6耳。术前听力检查语言频率气骨导差 39 9dB。 2 8耳 (其中 3例双侧 )行鼓室探查 ,并行听骨链重建术。结果 根据Teunissen( 1 993)分型 ,Ⅰ型 (先天性镫骨固定 ) 3耳 ,Ⅱ型 (先天性镫骨固定 +听骨畸形 ) 5耳 ,Ⅲ型 (先天性听骨链畸形 ,镫骨足板活动 ) 1 2例 ( 1 5耳 ) ,Ⅳ型 (听骨链畸形 +蜗窗或前庭窗发育不全 ) 5例 ( 5耳 )。术中发现畸形类型有镫骨足弓与底板分离、足弓部分缺损、砧骨固定、镫骨上部结构缺损、镫骨上部结构缺损伴前庭窗封闭 ,少见的有砧骨长脚缺损、镫骨上部结构缺损伴镫骨肌与砧骨长脚连接。听骨链重建方法有全听骨 (totalossicularreplacementprosthesis ,TORP)重建术 3耳 ,部分听骨 ( partialossicularreplacementprosthesis,PORP)重建术 1 2耳 ,小窗技术人工镫骨植入 8耳 (其中采用人工镫骨固定于锤骨柄 1耳 ) ,半规管开窗 1耳 ,激光在前庭窗开窗 ( 2耳 )、鼓岬开窗 ( 2耳 )后用筋膜封窗再用TORP植入。术后 2周内听力检查平均气骨导差为2 2 3dB。结论 根?

Objective To study on types of ossicular malformation and the option to surgical procedures Methods Malformations of the ossicules in 25 patients were analyzed on the basis of surgical finding involving 9 males and 16 females The age range were 8～54 years (average 24 8±12 3 years) Results of pre operation auditory test showed that 14 patients had bilateral conductive hearing loss whereas eleven cases had unilateral symptoms (left side in 5 cases and right side in 6 cases) Average air bone gap was 39 91 dB The ossiculoplasty was performed in 28 ears of 25 cases Results According to Teunissen′s classification about congenital middle ear anomalies, 25 cases were divided into four classes Three cases had congenital isolated stapes ankylosis as class Ⅰ Five cases had congenital stapes ankylosis in combination with a congenital anomaly of ossicular chain as class Ⅱ Twelve cases had congenital anomalies of ossicular chain and without stapes footplate ankylosis as class Ⅲ Six cases had aplasia or severe dysplasia of the oval window or round window as class Ⅳ Most malformations of ossicles were observed on crura separating from footplat, incudostapedial joint ankylosis, partial crura of stapes, and absence of suprastructure of footplate with or without dysplasia of the oval window or round window Few malformations were absence of long process of incus or absence of superstructure while stapes muscle attached in long process of incus Three ears were implanted with total ossicular replacement prosthesis(TORP) and with partial ossicular replacement prosthesis(PORP) in 12 ears Eight ears were fenestrated in footplate and implanted pistons that were fixed on long process of incus in 7 cases and on malleus handle in one case The prosthesis was placed in a fenestrated hole in vestibular window or promontory coved with fascial in 4 cases Fenestration was performed in one patient′s semicular canal Result of auditory test (Pure tone average PTA)showed that hearing improvement achieved(PTA>15 dB) in 79% and average air bone gap was 22 3 dB in two weeks post surgery Conclusion Application of ossicular chain reconstruction technique is effect on deceasing air bone gap in congenital ossicular anomaly depending on the types of ossicular malformation