Objective: To report a scala tympani drill-out technique for managing malformed facial nerve covering the entire oval window(OW).Methods: Data from three cases with OW atresia, malformed stapes and abnormal facial ner...Objective: To report a scala tympani drill-out technique for managing malformed facial nerve covering the entire oval window(OW).Methods: Data from three cases with OW atresia, malformed stapes and abnormal facial nerve courses were reported, in which a scala tympani drill-out technique was employed with a TORP between the tympanic membrane and scala tympani fenestration for hearing reconstruction.Results: Air conduction hearing improved in two of the three cases following surgery. In the third case, there was no improvement in air conduction hearing following a canal wall up mastoidectomy and tympanoplasty. There were no vertigo, tinnitus or sensorineural hearing loss in the three cases.Conclusion: The scala tympani drill-out technique, which is basically fenestration at the initial part of the basal turn, provides a choice in hearing reconstruction when the OW is completely covered by abarrently coursed facial nerve.展开更多
Objectives: Facial nerve aberration is the most troublesome situation in congenital malformations of middle ear.The aim of our study is to investigate its imaging and clinical features as well as relevant choice of su...Objectives: Facial nerve aberration is the most troublesome situation in congenital malformations of middle ear.The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement.Methods: A retrospective study involving review of clinical data of 227 patients(256 ears) with congenital middle ear anomaly was undertaken, including preoperative computed tomography(CT) data, surgical records and videos.Results: Aberration involving intratemporal facial nerve was found in 82/256 ears(32.03%) with congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window(50/82 ears, 60.98%), bifurcation(3/82 ears, 3.66%) and transverse over the promontory(3/82 ears, 3.66%), counting for 68.29%(56/82) of the cases with facial nerve aberration. Concomitant stapes malformation was found in 76/82 ears(92.68%) and atresia or stenosis of the oval window in 27/82 ears(32.93%). In 9/82 ears(10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis(TORP) implantation and Vibrant Soundbridge(VSB) implantation.Conclusion: The majority of facial nerve aberration in congenital malformation of middle ear involves displacement of facial nerve, in addition to concomitant malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option.展开更多
目的分析先天性前庭窗闭锁(congenital atresia of the oval window,CAOW)患者的临床特征。方法回顾分析2018年7月至2024年7月合肥医科大学附属医院收治的经手术证实为CAOW的7例(8耳)患者,收集所有患者的临床资料,分析听力学及颞骨高分...目的分析先天性前庭窗闭锁(congenital atresia of the oval window,CAOW)患者的临床特征。方法回顾分析2018年7月至2024年7月合肥医科大学附属医院收治的经手术证实为CAOW的7例(8耳)患者,收集所有患者的临床资料,分析听力学及颞骨高分辨率CT的特点。结果7例患者确诊年龄8~19(13.2±6.9)岁;所有患者均无明显耳廓畸形;表现为鼓膜完整的传导性聋或以传导性聋为主的混合性聋;经耳内镜鼓室探查确诊为CAOW,表现为鼓室内侧壁前庭窗区域形成凹陷,被完整的骨板封闭。高分辨率CT结合鼓室探查发现8耳均合并其他听骨链畸形:其中镫骨缺如8耳,砧骨长脚部分缺如3耳,砧骨长脚与锥隆起之间有畸形骨质连接4耳,锤骨柄短小1耳,锤骨体积偏小1耳。此外,6耳合并面神经畸形:其中4耳表现为面神经分叉,2耳表现为面神经遮窗。纯音测听5耳术前气导听阈≥60 dB HL,8耳气导听阈为(69.0±11.8)dB HL,气骨导差为(52.0±7.0)dB。低频段(125~1000 Hz)气导听阈高于高频段(2000~8000 Hz),气骨导差也大于高频段(P值均<0.05)。结论CAOW患者自幼可出现不伴明显耳廓畸形且鼓膜完整的非渐进性听力下降(气导≥60 dB HL,气骨导差≥50 dB),颞骨高分辨率CT有助于CAOW的诊断,鼓室探查可确诊。展开更多
文摘Objective: To report a scala tympani drill-out technique for managing malformed facial nerve covering the entire oval window(OW).Methods: Data from three cases with OW atresia, malformed stapes and abnormal facial nerve courses were reported, in which a scala tympani drill-out technique was employed with a TORP between the tympanic membrane and scala tympani fenestration for hearing reconstruction.Results: Air conduction hearing improved in two of the three cases following surgery. In the third case, there was no improvement in air conduction hearing following a canal wall up mastoidectomy and tympanoplasty. There were no vertigo, tinnitus or sensorineural hearing loss in the three cases.Conclusion: The scala tympani drill-out technique, which is basically fenestration at the initial part of the basal turn, provides a choice in hearing reconstruction when the OW is completely covered by abarrently coursed facial nerve.
基金supported by Beijing Municipal Science&Technology Commission(No.2171100001017079)for Prof. Shouqin Zhao
文摘Objectives: Facial nerve aberration is the most troublesome situation in congenital malformations of middle ear.The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement.Methods: A retrospective study involving review of clinical data of 227 patients(256 ears) with congenital middle ear anomaly was undertaken, including preoperative computed tomography(CT) data, surgical records and videos.Results: Aberration involving intratemporal facial nerve was found in 82/256 ears(32.03%) with congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window(50/82 ears, 60.98%), bifurcation(3/82 ears, 3.66%) and transverse over the promontory(3/82 ears, 3.66%), counting for 68.29%(56/82) of the cases with facial nerve aberration. Concomitant stapes malformation was found in 76/82 ears(92.68%) and atresia or stenosis of the oval window in 27/82 ears(32.93%). In 9/82 ears(10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis(TORP) implantation and Vibrant Soundbridge(VSB) implantation.Conclusion: The majority of facial nerve aberration in congenital malformation of middle ear involves displacement of facial nerve, in addition to concomitant malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option.
文摘目的分析先天性前庭窗闭锁(congenital atresia of the oval window,CAOW)患者的临床特征。方法回顾分析2018年7月至2024年7月合肥医科大学附属医院收治的经手术证实为CAOW的7例(8耳)患者,收集所有患者的临床资料,分析听力学及颞骨高分辨率CT的特点。结果7例患者确诊年龄8~19(13.2±6.9)岁;所有患者均无明显耳廓畸形;表现为鼓膜完整的传导性聋或以传导性聋为主的混合性聋;经耳内镜鼓室探查确诊为CAOW,表现为鼓室内侧壁前庭窗区域形成凹陷,被完整的骨板封闭。高分辨率CT结合鼓室探查发现8耳均合并其他听骨链畸形:其中镫骨缺如8耳,砧骨长脚部分缺如3耳,砧骨长脚与锥隆起之间有畸形骨质连接4耳,锤骨柄短小1耳,锤骨体积偏小1耳。此外,6耳合并面神经畸形:其中4耳表现为面神经分叉,2耳表现为面神经遮窗。纯音测听5耳术前气导听阈≥60 dB HL,8耳气导听阈为(69.0±11.8)dB HL,气骨导差为(52.0±7.0)dB。低频段(125~1000 Hz)气导听阈高于高频段(2000~8000 Hz),气骨导差也大于高频段(P值均<0.05)。结论CAOW患者自幼可出现不伴明显耳廓畸形且鼓膜完整的非渐进性听力下降(气导≥60 dB HL,气骨导差≥50 dB),颞骨高分辨率CT有助于CAOW的诊断,鼓室探查可确诊。
