The Study Of Clinical Characteristics And The Speech Rehabilitation After Cochlear Implantation In Patients With Vestibular Aqueduct Enlargement

Objective:In this study,we collected and analyzed the clinical data of patients with enlarged vestibular aqueduct(EVA),and observed the possible risks and complications of cochlear implantation(CI)in EVA patients.The results of gene detection and rehabilitation after CI were analyzed,and the clinical characteristics,genotype,curative effect after CI and the relationship between the age of implantation and the curative effect after CI in EVA patients.Methods:A total of 90 patients diagnosed as EVA-parallel CI in the second hospital in Lanzhou University in 2012 ? 2016 were selected to be included in the study to collect the pre-operative clinical data,including the incidence,family history,audiological data and intraoperative conditions.after obtaining the informed consent of the patient's guardian or the patient,the peripheral venous blood is extracted to be detected by the common gene of the deafness,and the auditory and speech recognition capability after the CI operation of the EVA patient is regularly followed and evaluated,a standard short sentence in a noise environment with a Chinese standard short sentence and a fixed signal-to-noise ratio of + 10 dB Speech recognition ability The results of 65 dB SPL,were analyzed by SPSS25.0,and analyzed the correlation between EVA,implantation age and the curative effect after CI.Results:1.fluctuating,progressive and asymmetrical hearing loss is the main clinical manifestation of EVA.acouatically short latency negative response(ANSR)is the characteristic auditory manifestation in ABR detection,and the induced rate is 45%.The average latency is 2.87 ±0.23 ms.Among the 90 cases of EVA,67.78%(61/90)showed simple EVA(LVAS),and 32.22%(29/90)with other inner ear malformations(EVA + IP).2.Among the 90 cases(180 ears)of EVA,8 ears(7.34%)had "gush" during operation,and2.53% of them had "gush" in LVAS group,and the rate of "gush" was 2.53% in the group of LVAS,and the rate of "gush" was 2.53% in the group of LVAS.50.63% of the patients showed changes incerebrospinal fluid(CSF)after opening the window.In the EVA IP group,the incidence of "gush" was 18.75%,65.63% of the patients showed fluctuating cerebrospinal fluid in different degrees after windowing.One ear had vertigo with contralateral hearing loss after(EVA +IP).1 ear had meningitis 4 years after(EVA+ IP),and none of the patients developed meningitis after 4 years.Cerebrospinal fluid leakage,incision infection,facial paralysis and other complications.3.SLC26A4 gene mutation was found in 75 of 90 EVA patients with a carrier rate of 83.33%(75 / 90).Among them,c.919 / 2A > G/c.919-2A > G was the most common genotype,accounting for 25.33%(19 / 75),followed by c.919 / 2A > G,accounting for 10.67%(18 / 75),and the hot spot mutation was c.919 / 2A > G.GJB2 gene mutation was detected in 48% of 5patients(5.56%),and the most common mutation was c.235 del.The frequency of allele C was50%(5 / 10).The mutation of GJB3 gene was detected in 1 patient(1.11%),and the mutation of mtDNA gene was not detected.4.The speech rehabilitation of EVA patients:(1)Auditory threshold: there was no significant difference between LVAS group and control group(P > 0 05),and there was no significant difference between the two groups(P > 0 05).The mean hearing threshold of 0.5K Hz and 2K Hz in LVAS group was significantly higher than that in EVA IP group(P < 0.05),but there was no significant difference in other frequencies(P > 0.05).(2)CAP and SIR scores: compared with EVA IP group and control group,CAP and SIR scores in LVAS group were significantly different from those in control group(P < 0.05);(3)Speech recognition ability: Compared with the group of EVA+ IP,the speech recognition rate in the quiet environment was statistically different(P <0.05).The speech recognition rate in the Chinese standard short sentence and the noise environment has statistical difference(P <0.05);in the group,the speech recognition ability under the quiet environment> the speech recognition ability in the noise environment.5.There was no significant difference in the mean hearing threshold,CAP and SIR scores and speech recognition ability after CI among the three groups of EVA patients with different implantation ages(0-3,3-6,6-9years).Conclusion:1.The audiological characteristics of EVA are as follows: high-frequency hearing loss curve in early stage,low-frequency can exist gas-bone conduction difference and ABR-induced ANSR,HRCT combined with MRI image examination,can improve the accuracy of EVA diagnosis.2.Intraoperative "gush" is the most common complications in EVA patients,such as other internal ear malformations(such as IP type),will increase the risk of "gush" during the operation,and postoperative vertigo and meningitis will also increase the risk of postoperative vertigo and meningitis.3.SLC26A4 gene mutation is the most common molecular cause of EVA and is closely related to the occurrence of EVA.4.The auditory ability of EVA patients after CI is similar to that of NSHL patients with normal inner ear anatomy.The ability of speech recognition and understanding is even better than that of NSHL patients.If accompanied by other inner ear malformations,the curative effect of CI in EVA patients will be affected.5.The age of implantation is not the main factor affecting the curative effect of ci in eva patients,nor is it the key factor to determine whether to accept ci.In the face of the choice of surgical timing of eva patients,it should be considered comprehensively.