The Analysis Of Phonetic Features In Prelingual Deafness Children With Cochlear Implant And The Research About It With Genetic Correlation

The deafness is a common disease critically to influence quality of life.Now about 60%of severe to profound hearing loss was caused by heredity.Basing on measurement of acoustic parameters of vowels,consonants and tone features of mandarin in prelingual deafness children with cochlear implant and to study the change in the law and tedency about these acoustic features.At the same time to screen deafness genes in these children and to do the NRT in operations.And then, combining the results of gene screening and NRT to analyse the correlations between them so to verify the function and integrity of auditory nerve pathway in heredity deafness.And to explain the prognostic effects of genetic factors in auditory and speech rehabilitation.In this studies,progressively to analyse the acoustic parameters of GJB2 hearing loss children so to reserch the contributions of GJB2 gene in speech production and to provide evidences of prognostic effects of gene in hearing and speech rehabilitation in prelingual deafness children with cochlear implant.Objective:1.To analyse features of vowels,consonants and tones in prelingual hearing loss children with cochlear implants by subjective way and software of phonetics.To discuss features and regulations of these phonemes so to establish the foundation of analysing intelligibility of speech in prelingual hearing loss children with cochlear implants and to provide ideas and evidences of newer and more objective methods of evaluating speech rehabilitation.2.To screening genes of correlated hearing loss in prelingual deafness children.In operations,to do the NRT and then to combine the results of two parts to analyse the relation bewteen auditory nerve pathway and genes so to provide the evidences and supports of genetic predictable effects for auditory nerve function and rehabilitation efficacy of speech.3.To do the sequencing analysis of JGB2 in these children at the same time progressively to analyse the variations of intelligibility of speech and the production results in children with cochlear implants.To establish the foundantion of future research.Method:1.(part 1):According to different ages and beginning employment time of cochlear implant to divide these children into subgroups.After 2～3moths and 8～10months to measure subjective and phoetic parameters after first mapping, respectively.Utilizing PRAAT software to analyse acoustic parameters at the same time to compare the results with controls so to learn about the variations of these parameters.Furthermore,to compare the results with subjective methods and learn about the difference between them..2.(part 2):Utilizing the Gene Chip to screen the genes of GJB2,GJB3,SLC26A4 and Mitochondrial Gene(mtDNA) which are correlated with hereditory hearing loss.In implanting operations,to monitor the NRT for these children.And then to analyse the relation between them.3.(part 3):Sequencing the complete sequence of GJB2 in prelingual deaf children. And then divide these children into three groups according to with GJB2 mutation and without it.Further studying the intelligibility of speech features about two groups so to analyse the correlation between them.Results:1.In speech intelligibility,the results were very different between two measurements in lower children and older children whose beginning time was lower one year. And the results of second time of lower age children were better than older ones. To analyse subjectively two groups:the lower age children had different results but older ones not.About tone,the results were different between groups and controls.About analysis of phonetic:the formant of vowels were different between deaf childern and controls.The difference were that the value of formants of deaf children were lower or higher than controls.But about consonants,there were very different in plosive,affricate,firctive and nasal between measured children and controls.The variable tendency of tone can be macroscopic reflected by phonetic analysis of tone.2.The results of screen by Gene Chip in participated children were heterozygous, homozygous,compound heterozygous mutations in GJB2,IVS homozygous mutation in SLC26A4 and compound heterozygous in it.By the results of NRT,the waveforms of NRT with GJB2 were more typical and reproducibility by different intensions and better than non-GJB2 ones.3.The results of sequences were not to find new mutational site execpt for 235delC, 299-300delAY and compound heterozygous mutation.These results were same as other literatures and the second part.There were significient difference about the results of intelligibility of speech in two groups,according to GJB2 mutating if or not.The speech production was correlated by the mutation of GJB2.Conclusion:1.The capability of speech production in lower age children were better than the older ones.Following temporal length of device turn-on,the parameters of speech production were gradually closing to normal control but the progress of tone features were not significance.When the time of utilization over one year the progress were slower this result were more obvious in old age.By using the devices,children can corrcet their manner of articulation.The analysis of phonetic can find the fine difference of vowels and consonants between these children and controls so can provide information for correcting articulation.The methods can reflect the variable tedency of tone more directly and precisely.2.The mutation of GJB2 is the most supreme heredity cause.In this test,the mutation rate was the same as other literatures.The waves of NRT were better in children with the mutation of GJB2 than ones without GJB2 mutation.But due to the children were a few so we couldn't find the correlation result between them.But we believe the genetic factor can be used to prognose the integrity of auditory nerve pathway and the gene-screen before operation can provide the help for prognosis.3.The results of speech production in children with GJB2 mutation were better than the ones with non-GJB2.They were negative correlation.So the gene-screen of GJB2 before operation were significant for prognosing the speech production.