The Correlation Of Inner Ear Malformation And Cochlear Nerve Deficiency And The Effect Of Their Cochlear Implantation

Objective:To discuss the morbidity, the classification and the correlation of inner ear malformation and cochlear nerve deficiency, and discuss the feasibilities of cochlear implantation in the children who have inner ear malformation or cochlear nerve deficiency.Methods:In the first part, collected the data of sever sensorineural hearing loss children who had taken HRCT and MRI examination in our hospital in the past two years. The cases of inner ear malformations and cochlear nerve deficiency were classified and analyzed respectively by using chart and table. A more detailed analysis about the information of the cases which have both inner ear malformations and cochlear nerve deficiency was made.In the second part, detailed information of surgery and speech rehabilitation effect of5Mondini cases and1CND case which have had cochlear implantation was recorded.10no deformity cases with cochlear implantation randomly selected set as normal control group. By using Sun’s Deaf children’s hearing speech assessment vocabulary and standard evaluation process, the auditory speech recognition ability of the test object were evaluated. The scores respectively after3,6,9and12months’training were recorded and the data of each group was compared.Results:Inner ear malformations and cochlear nerve deficiency were found in84cases (157ears), with incidence of21.21%, single ear disease13%, double ear disease87%. There were labyrinth related malformations27cases, enlarged vestibular aqueduct (EVA) deformity52cases and cochlear nerve deficiency (CND)15cases. EVA was the most common in inner ear malformation, accounted for61.90%. Mondini was the most common case in cochlea malformations, accounted for46.94%.That CND with narrow internal auditory canal53.33%, with inner ear malformations46.67%and with vestibular nerve deficiency50%. The "Gusher" occurred in all of the5Mondini cases during the cochlear implantation. There were no obvious statistical difference between Mondini deformity children and normal control children in the postoperative auditory speech recognition ability evaluation scores. The postoperative auditory speech recognition ability evaluation scores in1case with CND after cochlear implantation was significantly lower than the other children after3months’ and6months’ speech rehabilitation, but the gap was insignificant after12months’ speech rehabilitation.Conclusion:The incidence of inner ear malformation and cochlear nerve deficiency occurred in congenital sensorineural hearing loss children in Hunan area is21.21%. EVA is the most common inner ear malformation. Mondini is the most common cochlea malformation. Cochlear nerve deficiency is closely related to the inner ear malformation and narrow internal auditory canal. The "Gusher" always occur during the cochlear implantation in Mondini cases. Some inner ear malformation such as Mondini and some cochlear nerve deficiency can take cochlear implantation operation.But it should take a detailed auditory and imaging examination before cochlear implantation and a long-time regular rehabilitation after cochlear implantation.