儿童先天性感音神经性聋中内耳畸形的影像学分析

目的分析先天性感音神经性聋(SNHL)患儿中内耳畸形的颞骨影像学表现。方法对行听力学检查后确诊的先天性SNHL儿童进行颞骨CT和MRI检查,其中188例资料完整,总结影像学所见,按Sennaroglu分类法进行分析讨论。结果CT和MRI显示其中35例(54耳)存在内耳发育畸形,3例(5耳)同时合并中耳及外耳畸形。35例中共同腔畸形2例(4耳);耳蜗发育不全1例(1耳);耳蜗不完全分隔Ⅱ型(IP-Ⅱ)即Mondini畸形13例(26耳);前庭扩大4例(7耳);半规管畸形13例(19耳);内听道狭窄7例(10耳);内听道扩大1例(2耳);前庭导水管扩大19例(34耳)。结论对先天性SNHL患儿行CT及MRI检查非常重要,可发现其中存在的内耳发育畸形。CT及MRI对诊断内耳畸形优势互补,为临床治疗,尤其是人工耳蜗植入适应证的选择提供了重要依据。

Analysis of imaging of inner ear malformations in children with congenital sensorineural hearing loss

Objective To study the imaging of inner ear malformations in children with congenital sensorineural hearing loss(SNHL).Methods CT and MRI examinations were performed on children with SNHL diagnosed by audiological test.One hundred and eighty-eight patients with complete imaging information were obtained.The imaging of inner ear malformations was analyzed according to Sennaroglu's classification. Results Thirty-five patients(54 ears) were found with inner ear malformations by CT and MRI,3 of whom(5 ears) were accompanied by outer and middle ear malformations.Among the 35 patients,2(4 ears) were found to be common cavity deformity,1(1 ear) cochlear hypoplasia,13(26 ears) incomplete partition II(Mondini deformity),4(7 ears) vestibule dilation,13(19 ears) semicircular canal deformity and 19(34 ears) large vestibular aqueduct.Internal auditory canal was found narrow in 7 patients(10 ears) and wide in 1(2 ears) with classic Mondini deformity. Conclusion CT and MRI examinations are of great importance to the diagnosis and treatment of inner ear malformations in children with congenital SNHL,especially for the candidates of cochlear implantation.