| Ojective: By analyzing the CT and MRI imaging features of inner ear of children and adolescents with sensorineural hearing loss(SNHL),all congenital inner ear malformations(IEMs)in the study subjects were diagnosed and classified,and the incidence of congenital IEMs was counted.The effect of hearing rehabilitation after cochlear implantation(CI)in patients with congenital IEMs was analyzed,which provided guidance for pre-operative and post-operative evaluation of CI.methods: children and adolescents aged between 3 months and 18 years old with SNHL were selected as the study subjects,who visited the otorhinolaryngology diagnosis and treatment center of Xinjiang Uygur Autonomous region people's Hospital between March 2015 and March 2017.The CT and MRI imaging features of the inner ear were analyzed retrospectively.According to the diagnostic classification system of Sennaroglu congenital IEMs,all the congenital IEMs were diagnosed and classified,and the incidence of congenital IEMs was counted.At the same time,the profound prelingual patients with LVAS or Mondini deformity were used as observation group.The results of hearing threshold were collected before CI,1 month,6 months and 12 months after CI.Compared with the control group with normal inner ear structures,the effect of hearing rehabilitation after CI was evaluated by repeated analysis of variance(ANOVA)with spss23.0 software.results: There were 189 cases(350 ears)of congenital IEMs that found in 690 cases of SNHL.330 ears were severe to extremely severe sensorineural deafness.The incidence of congenital IEMs was 27.4%.Unbiaural deformity was 14.8% and biaural deformity was 85.2%.Cochlear malformation accounted for 44.4%.Vestibular malformation accounted for 52.9%.EVA accounted for 52.4%.Semicircular canals malformation accounted for 30.2%.Cochlear nerve malformation accounted for 21.7%.Internal auditory malformation accounted for 21.2%.Cochlear aperture abnormalities accounted for 8.5%.In the cochlear malformation,Michel malformation accounted for 1.2%.Cochlear aplasia accounted for 4.8%.Common cavity deformity accounted for 6.0%.Incomplete partitpion(IP)type? accunted for 8.3%.Modini malformation accounted for 53.6%.IP-? accounted for 1.2%.Cochlear hypoplasia(CH)accounted for 29.8%,and the percentage of type ?,?,III and IV of CH was 4.8%,4.8%,15.5% and 4.8% respectively.The effect of hearing rehabilitation after CI for congenital inner ear malformations was analyzed.There was significant difference in the average hearing threshold of sound field before and after operation in the observation group and in the control group(P<0.05).There was no significant difference in the interaction effect between the time point of the average hearing threshold test and the group(P>0.05).The average hearing threshold of the observation group and the control group was consistent with the change of the test time,and decreased with the prolongation of the test interval after operation,and the hearing was gradually improved.There was no significant difference in the average hearing threshold of sound field between the two groups(P>0.05).conclusion: Most of the congenital IEMs were severe or profound SNHL,and the IEMs were mainly in the form of bilateral compound deformity.The ratio of EVA and vestibular malformation was the highest in congenital IEMs.The ratio of them was similar,followed by cochlear malformation.In cochlear malformation,Mondini malformation accounted for the highest,followed by cochlear dysplasia.Familiar with the imaging features of congenital IEMs has an important guiding role for the selection of cochlear implant patients,choice of ear side,determination of operation and approach,preparation of electrode type,prediction of complications during and after operation,prediction of postoperative effect,etc.The hearing rehabilitation effect of the patients with LVAS or Mondini malformation was good,and there was no obvious difference in the hearing rehabilitation effect after CI between them and the SNHL patients with normal inner ear structure. |
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