听神经病及蜗神经发育不良儿童人工耳蜗植入的有效性

摘要

Objective The aim of this study is to assess of cochlear implantation in children with auditory neuropathy and cochlear nerve aplasia by using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR).Methods Twenty one children with cochlear implants participated in this study.They all received cochlear implant surgery at our hospital from January 2004 to October 2010.All children had hearing aid trial and hearing and speech rehabilitation before surgery at least three months.Nine children (7 male,2 female) were diagnosed with auditory neuropathy,twelve (7 male,5 female) with cochlear nerve aplasia.Twenty children (10 male,10 female) with sensorineural hearing loss served as a control group.All the children received cochlear implant for more than six months.Foxty two children with normal hearing served as another control group which were divided into three subgroups according to their age.Group A included 18 children aged under two yrs,group B consisted of 16 children aged from two to four yrs and group C comprised eight children aged above four yrs.CAP and SIR were used to evaluate among all the children and the scores were compared.Results The CAP scores of children with auditory neuropathy,cochlear nerve aplasia,sensorial neural hearing loss and the three subgroups children with normal hearing were 4.44 ± 1.50,4.83 ± 1.69,4.55 ± 1.66,5.22 ± 1.11,6.75 ± 0.45 and 7.00 ± 0.00 respectively,and SIR scores were 2.66 ± 1.11、2.33 ± 1.15,2.40 ± 0.75,2.56 ± 1.04,4.12 ± 0.81 and 5.00 ±0.00 respectively.There were significant differences among the six groups for CAP scores (x2 =35.481,P ＜ 0.001) and SIR scores (x2 =40.549,P ＜ 0.001).No significant differences for CAP and SIR scores were observed between children with auditory neuropathy/cochlear nerve aplasia and sensorial neural hearing loss as well as group A (P ＞ 0.05 for each),and there were significant differences were shown between children with auditory neuropathy/cochlear nerve aplasia and group B as well as group C(P ＜ 0.01 for each aplasia).Conclusions The auditory and speech capabilities of children with auditory neuropathy and cochlear nerve deficiencycan can get benefits from cochlear implants as children with sensorineural hearing loss,however not achieve the level of those with normal hearing after cochlear implantation.The long term effects still need follow-up and evaluation.%目的 通过问卷量表评估,探讨听神经病及蜗神经发育不良儿童的人工耳蜗植入效果.方法 实验组为2004年1月至2010年10月期间接受人工耳蜗植入的符合听神经病或蜗神经发育不良诊断标准、且术前至少佩戴3个月助听器并接受语言训练的患儿,共21例,其中听神经病9例,蜗神经发育不良12例.植入对照组为20例同期植入人工耳蜗的感音神经性聋患儿.所有人工耳蜗植入患儿均开机使用6个月以上.正常听力对照组为42例听力正常的儿童,按年龄分为正常听力A组(年龄在24个月以下)18例、正常听力B组(年龄24～ 48个月)16例和正常听力C组(年龄在48个月以上)8例.采用听觉行为分级(Categories of Auditory Performance,CAP)和言语可懂度分级(Speech Intelligibility Rating,SIR)两个量表进行评分,使用SPSS16.0软件对实验组得分与植入对照组和正常听力对照组进行比较.结果 听神经病组、蜗神经发育不良组、感音神经性聋组以及三组正常听力儿童的CAP得分(x±s,下同)分别为(4.44±1.50)分、(4.83±1.69)分、(4.55±1.66)分、(5.22±1.11)分、(6.75±0.45)分和(7.00±0.00)分,SIR得分分别为(2.66±1.11)分、(2.33±1.15)分,(2.40±0.75)分,(2.56±1.04)分、(4.12±0.81)分和(5.00±0.00)分.CAP和SIR得分在六组受试者间差异均具有统计学意义(x2=35.481,P＜0.001;x2 =40.549,P＜0.001);组内两两比较,听神经病组/蜗神经发育不良组与感音神经性聋组及正常听力A组得分比较,差异无统计学意义(P值均＞0.05),而与正常听力B组及C组相比,差异具有统计学意义(P值均＜0.01).结论 听神经病和蜗神经发育不良儿童接受人工耳蜗植入后,同感音神经性聋患儿一样,能够获得听觉及言语能力的进步,但落后于听力正常的同龄儿童.人工耳蜗提高此类患儿听觉及言语能力的远期效果还有待进一步随访和评估.