内耳结构异常的语前聋人工耳蜗植入儿童听觉语言康复效果评价

目的评价不同类型内耳结构异常的语前聋儿童人工耳蜗植入术后听觉语言康复效果。方法选取人工耳蜗植入儿童10例(12耳),其中内耳结构发育正常的3例,前庭导水管扩大3例,Mondini畸形1例,蜗孔狭窄1例,内听道狭窄听神经纤细2例,分别采用电刺激诱发听神经复合动作电位(electrically evoked compound action potential,eCAP)、助听听阈、听障儿童听觉能力、语言能力评估标准及方法,从听神经客观电生理水平、听察觉阈、听觉辨识与理解能力以及语言能力4方面评价患儿的康复效果。结果①客观电生理:单纯前庭导水管扩大患者听神经对电刺激敏感度与内耳发育正常者相似,Mondini畸形患者比内耳发育正常者略高,蜗孔狭窄及听神经纤细患者听神经敏感度较差;②听察觉阈:与内耳结构正常及轻度畸形(Mondini畸形)患者相比,蜗孔狭窄及听神经纤细患者听察觉阈偏高,高频比低频阈值低;③听觉辨识与理解能力:在安静环境近距离交谈情境下,单纯前庭导水管扩大、Mondini畸形及蜗孔狭窄患者对熟悉语词和短句的辨识与理解能力与内耳发育正常者无差别,听神经纤细患者得分较低;④语言能力:单纯前庭导水管扩大、Mondini畸形患者与内耳发育正常者无差别,蜗孔狭窄患者的语言交往与表达能力落后于内耳发育正常者,听神经纤细患者在语法、理解、交往、表达4个维度均落后于内耳发育正常者。结论语前聋儿童人工耳蜗植入术后听觉语言康复效果需要使用多种方法综合评价。Mondini畸形患者给予足够电刺激量后,其听觉语言能力可达到内耳发育正常者水平。蜗孔狭窄、听神经纤细等严重内耳畸形患者,人工耳蜗对其听觉语言能力发展有一定帮助,家长需要建立合理期望值并坚持长期康复。

The Rehabilitation Effect after Cochlear Implantation for Prelingual Hearing-Impaird Children with Different Types of Structural of the Inner Ear

Objective Comprehensive assessment methods were used to evaluate the effect of hearing and language rehabilitation after cochlear implantation for children with prelingual deafness.Methods 10 cases(12 ears)with cochlear implants were included.Among them,3 cases had normal inner ear development,3 cases of vestibular aqueduct enlargement,1 case of Mondini malformation,1 case of cochlear foraminal stenosis,and 2 cases of internal auditory canal stenosis.Electrically evoked compound action potential(eCAP),hearing threshold,hearing ability and language ability assessment standards and methods of hearing-impaired children were used respectively.The rehabilitation effect of cases was evaluated from four aspects:objective electrophysiological level of auditory nerve,threshold of auditory perception,auditory recognition and understanding ability,and language ability.Results(1)Objective electrophysiological level:The sensitivity of the auditory nerve to electrical stimulation in patients with EVA was similar to that in patients with normal inner ear development;the sensitivity of the auditory nerve in patients with Mondini malformation was slightly higher than that in patients with normal inner ear development;The sensitivity of the auditory nerve in patients with cochlear stenosis and CND was poor.(2)Threshold of auditory perception:the threshold of auditory perception in patients with cochlear foraminal stenosis and CND was higher,and the threshold of high-frequency was lower than that of low-frequency.(3)Hearing recognition and understanding:In the quiet environment,the patients with EVA,Mondini malformation and cochlear foraminal stenosis had no difference in the ability to recognize and understand familiar words and short sentences compared with the patients with normal inner ear development,and the scores of the patients with CND were lower.(4)Language ability:There was no difference between patients with EVA and Mondini malformation and those with normal inner ear development;then language communication and expression ability of patients with cochlear foraminal stenosis lagged behind those with normal inner ear development;the grammar,understanding,communication and expression of patients with CND lagged behind those with normal inner ear development in four dimensions.Conclusion It is necessary to use comprehensive methods to evaluate the effect of hearing and language rehabilitation after cochlear implantation for children with prelingual deafness.Patients with Mondini deformity should be given enough electrical stimulation to achieve normal inner ear development.In patients with severe inner ear malformation,such as stenosis of the cochlear foraminal and CND,cochlear implant can help them develop their hearing and language ability to some extent,but at the same time,parents need to establish reasonable expectations and insist on long-term rehabilitation.