Objective To evaluate the hearing effects in hearing impaired children using FM system .Methods The FM listening evaluation for children questionnaire was used to evaluate 27 cases with sensorineural hearing loss who were fitted hearing aids or cochlear implants ,or using FM system .The auditory skills of the patients were evaluated in four kinds of listening environments :quiet ,noise ,auditory only and distance by this questionnaire . Each question required a subjective score based on the child's response .The higher the scores the better the results . The age range of the participants was 3 .48~5 .50 .On an average ,their experience of using hearing instruments was 2 .56 ± 0 .97 years .Results With local microphones only ,the total score was 67 .55% ;in quiet situation ,the score was 85 .33% ;in noise ,the score was 60 .54% ;with visual only ,the score was 69 .58% and in distance ,the score was 69 .5% .With frequency modulated + microphone ,the total score was 84 .35% ,in quiet situation ,the score was 94 .89% ,in noise ,the score was 81 .35% ,with visual only ,the score was 83 .56% ,and in distance ,and the score was 83 .29% .The score of the subjects with hearing instruments only in quiet was significant higher than those of in noise and long distance without eye clue situation(F=49 .53 ,P<0 .05) .The score of the subjects was significant improved after using FM system (P<0 .05) .The score of the subjects with hearing instruments and FM system in quiet was also significant higher than those of in noise ,long distance ,without eye clue situation(F=24 . 80 ,P<0 .05) .Conclusion FM system could improve the listening experience in hearing impaired children in a com‐plex environment .However ,processes and programs for preschool hearing impaired children should be further standardized and improved when fitting FM system ,as findings of variability in questionnaire performance .

Objective The hearing and speech rehabilitation effects by hearing aid use and cochlear implanta‐tion in hearing -impaired children were compared .Methods A total of infants aged from 6 months to 3 years with severe hearing loss of prelingual nature were included .They were divided into 2 groups :group 1 (18 with hearing aids) and group 2 (14 with cochlear implantation) .There was no significant difference in the degree of hearing loss and age between the 2 groups .They received rehabilitation education of the same content for a year .Their unaided and aided thresholds were tested in sound field while the IT -MAIS was used for evaluating hearing ability of two groups before and after 3 ,6 ,9 ,and 12 months of hearing aid use and cochlear implantation .Results In both groups ,hearing were better in aided conditions than those of in unaided ,while those from the cochlear group were significantly improved at more frequencies than those of the hearing aid group .In general ,the longer use and train‐ing ,the better hearing improvement .The mean scores of auditory performance in the cochlear implantation group at 6 months were significantly higher in comparison with those at 3 months (P<0 .05) .There were significant differ‐ences in mean scores between the two groups at 6 ,9 ,12 months after fitting (P<0 .05) .The verbal ability scores ,hearing and speech rehabilitation levels in the cochlear group were higher than those of in the hearing aid group (P<0 .05) .Conclusion For severe hearing loss children ,cochlear implantation could produce more noticeable effects than hearing aids .

Objective To study the effects of hydrogen dioxide (oxygen free radical donator) and vitamin C (oxygen free radical scavenger) on the electric current of large conductance calcium -activated potassium channels (BKCa channels) in isolated outer hair cells in aging guinea pigs .Methods Acute enzyme was used to isolat outer hair cells of aging guinea pigs ,in which of BKCa channel's electric current was observed and recorded by whole-cell recording mode of patch -clamp .After recording the stable and normal electric current of BKCa channels ,added H2 O2 dilution (0 .2 mmol/L) 40 μl in the 2 ml chambers within freshly isolated outer hair cells so that the concen‐tration of H2 O2 in the balneum would be 4 μmol /L .The groups(n=5) received individually vitamin C solution (5 mg/ml) 0 ,10 ,20 ,40μl in the 2 ml chambers within freshly isolated outer hair cells so that the concentration of vi‐tamin C in the balneum would be 0 ,25 ,50 ,100 μg /ml ,observing and recording the effects of different concentration of vitamin C to electric current of BKCa channels .Results ①In the w hole-cell mode of patch -clamp ,the rapid activation and non-deactivation electric current with a string of large amplitude was recorded ,above -40~ -30 mV activation voltage .The electric current increased with the increasing membrane potential .The amplitude in‐creased continuously and performed characteristics of outward rectification .When the concentration of IbTX was 100 nmol /L ,the activity of the channel was completely blocked and confirmed BKCa channel's electric current .②Medication within three minutes ,when VT was +50 mV ,the BKCa channels'the maximum peak current densities of 4 μmol /L H2 O2 group rose from 22 .09 ± 0 .27 PA /PF to 43 .53 ± 1 .09 PA/PF ,amplification was 97 .06% .In H2 O2 4 μmol /L + vitamin C with different concentrations as 25 ,50 ,100 μg/ml groups ,the BKCa channels'elec‐tric current performed about concentration-dependent inhibition ,and electric current's amplitude and peak current density decreased with the increasing concentration of vitamin C ,the I-V curves were reduced .However ,this still could not be recovered to the normal levels .Conclusion The oxygen free radical /BKCa exists in the process .The vitamin C as oxygen free radical scavenger can reverse the process to a large extent .

Objective This study aimed to define research status of age -related hearing loss ,and provide the basis and direction for future research .Methods We have retrieved all relevant literatures on age -related hearing loss from Pubmed ,and conduct an objective analysis of the existing literatures by Bibliometric analytics and co -word analysis method using co -occurrence bibliographic information mining system and SPSS22 .0 software for data analysis .Results There were a large number of articles and journals about presbycusis and age -related hearing loss .Many countries were involved in the research .Literatures and core authors were mainly from developed coun‐tries such as Europe and the United States .The quantity and quality of Chinese literatures were in a leading position in Asia .The researches focused on the common characteristics of patients ,the epidemiology ,characteristics of hear‐ing ,treatment and laboratory studies .There were some new research directions in recent 5 years ,such as factors as‐sociated with the younger ages before developing presbycusis ,standard design and use of questionnaires ,prevention and control .Conclusion Age-related hearing loss will continue to be a hot topic with growing focus on micro and macro development of multi -disciplinary cooperation .The penetration will be the trend for the future research while the prevention will become a new focus of research .

Objective To study the stability of NRT threshold and the correlation between NRT threshold and the value of behavior T ,C in order to instruct mapping in infant cochlear implant ,and to study whether the elic‐itation of NRT waveform can be applied to determine the effects of rehabilitation .Methods There were 159 cases with complete information in data in this study .150 out of the 159 cases coincided with the study requirements of the changes and correlation .We had collected the NRT thresholds of the electrodes of No .1 ,6 ,11 ,16 ,22 in switch-on ,one month after switched -on ,half a year and one year after switched -on .The analysis of variance method was adopted to compare the threshold changes of each electrode in different stages .We had collected the values of behavior T ,C in one year after switched -on .Pearson regression analysis was used to compare the correlation be‐tween NRT threshold in switch -on and this value of behavior T ,C .We applied MAIS assessment scale to make a questionnaire for all 159 patients during the follow -up at one year after switched -on .We had gathered the scores in three aspects ,the average score in questionnaire and the average hearing threshold of every child .All children were divided into four groups according to whether the elicitation of NRT waveform during - operation ,post-op‐eration .Results Regarding the NRT threshold ,there was no significant difference in the pairwise comparison of four time points(P>0 .05) .There was positive correlation between NRT threshold and behavior T ,C(P<0 .01) , but the correlation was wake .The group with no waveform on the during -operation and post -operation had sig‐nificant differences with the other groups in all scores and average hearing threshold (p<0 .01) .And there was no statistical significance among the other three groups (P> 0 .05) .Conclusion The NRT threshold remains steady from switched -on .A positive correlation exists between NRT threshold and the actual T ,C value .The NRT threshold can be used to estimate the T ,C value .But the accuracy of the measured value is less than the behavior method .We still need to use behavior method to measure as soon as possible .The NRT waveform appearance on the post-operation is more significant than that on the during -operation to prognosis of rehabilitation .

Objective The aim of this study is to analyze the relationship between the development of audito‐ry and speech in infants with hearing loss after hearing aid fitting and discuss the development of speech with the a‐bility of auditory in infants with different degree of hearing loss and different stage of evaluation after hearing aid fit‐ting .Methods A total of 32 infants (17 boys and 15 girls) with prelingual hearing loss participated in this study . The age at hearing aid fitting ranged from 3 to 34 months with a mean of 15 .6 months .According to their hearing level ,all infants in this study were divided into three groups :group of profound hearing loss(16) ,group of severe hearing loss(11) ,group of moderate hearing loss(5) .CAP and SIR were used to evaluate their abilities of auditory and speech at pre -fitting ,1 ,3 ,6 ,9 and 12 months after hearing aid fitting respectively .A linear and regression a‐nalysis was done in the statistic procedure .Results There was a significant correlation between the scores of CAP and SIR for the three groups respectively .The correlation from low to high followed by the group of profound hear‐ing loss (R=0 .656 ,P=0 .000<0 .05) ,group of severe hearing loss (R=0 .758 ,P=0 .000<0 .05) ,group of mod‐erate hearing loss (R=0 .849 ,P=0 .000<0 .05) .There was also a significant correlation between the scores of CAP and SIR for the different evaluating stages respectively .The correlation from low to high followed by pre -fitting (R=0 .559 ,P=0 .000<0 .05) ,1 month after hearing aid fitting (R=0 .575 ,P=0 .000<0 .05) ,3 months after hearing aid fitting (R=0 .726 ,P=0 .000<0 .05) ,6 months after hearing aid fitting (R=0 .823 ,P=0 .000<0 .05) , 9 months after hearing aid fitting (R=0 .838 ,P=0 .000<0 .05) ,12 months after hearing aid fitting (R=0 .855 ,P=0 .000<0 .05) .Conclusion The development of auditory ability is closely related to speech ability which increases with the improvement of auditory ability .The less the infants have the hearing loss and the longer the infants use the hearing aids ,the higher the auditory ability is related to speech ability in infants after hearing aid fitting .

Objective To investigate the impact of pair box 3 (PAX3) gene mutations on transcriptional ac‐tivity of target gene microphthalmia -associated transcription factor (MITF) and the role it plays in the pathogene‐sis of Waardenburg syndrome type I .Methods The 293T cells were transient transfected with wild type (WT ) PAX3 and mutant type (M T ) H80D ,H186fsX5 plasmids .We observed and analysed the regulation effects of WT/MT PAX3 on the transcriptional activities of MITF and the influence of the two mutants on WT PAX3 function u‐sing luciferase activity assays ,detect DNA binding capacity of WT/MT PAX3 to MITF gene promoter using a bioti‐nylated double - stranded oligonucleotide probe containing PAX3 binding motif ATTAAT to precipitate PAX3 , H80D and H186fsX5 respectively .Results H80D mutant was partially functional and was able to transactivate the MITF promoter in part ,but it was dramatically reduced as compared with WT PAX 3(P<0 .01) .H186fsX5 mu‐tant was loss -of -function and failed to transactivate the MITF promoter as compared with WT PAX 3 (P<0 .01) . None of them showed dominant -negative effect on WT PAX3(P>0 .05) .WT PAX3 and H80D mutant were able to bind specifically to the ATTAAT motif on the MITF promoter ,whereas H186fsX5 PAX3 lost the DNA -binding ability .Conclusion The mutations H80D and H186fsX5 made down-regulation of MITF transcription and decrease syn‐thesis of melanin ,which resulted in haploinsufficiency of PAX3 protein and caused mild phenotypes of WS1 .

Objective To investigate the morphological alteration and the protein expression of caspase -3 in long -term denervated posterior cricoarytenoid muscles (PCAMs)in order to find the appropriate time point of rein‐neration in long -term denervated PCAMs .Methods A total of 45 patients with vocal paralysis were recruited and devided into 3 groups ,the 3~6 months denervation group ,the 6~12 months denervation group ,the 1~2 year denervation group .12 adults served as control group .The morphological alteration was evaluated using HE staining and the change in expression of caspase -3 ,an apoptosis related factor ,were observed using immunehistochemistry stain and western blot .Results With elongation of denervation time ,there were increased denaturation in the mus‐cle fibers .The nucleus moved inside and some of them concentrated .Caspase-3 showed weak staining in innerva‐ted ,however ,by 3-6 months of muscle denervation there was a significant accumulation of caspase -3 protein in myofibers ,6~12 months and 1~2 years of denervation ,expression of caspase -3 protein in myofibers was de‐creased significantly .In western blot ,the change in protein expression of caspase -3 was observed an 21-fold (P< 0 .01) increase from 3~6 months denervated muscles to innervated muscles ,11-fold (P< 0 .01) increase from 6~12 months denervated muscles to innervated muscles ,3~fold (P< 0 .01) increase from 1~2 year denervated muscles to innervated muscles .Conclusion The morphological alterations and changes in expression of caspase -3 indicated there was a high amplitude of apoptosis in denervated posterior cricoarytenoid muscles within 1 year .

Objective To study the molecular pathogenesis of non -syndromic deafness in a Chinese family . Methods Clinical materials and DNA sample were obtained from the non -syndromic family with autosomal reces‐sive deafness .The exons and the flanking splicing sites of GJB2 and SLC26A4 were tested in all family members by PCR and direct sequencing .Results There were four deafness patients in the family ,and three of them had the same clinical phenotypes ,including prelingual profound sensorineural hearing loss and enlarged vestibular ,while the re‐mained one only presented to be prelingual profound sensorineural hearing loss without malformation of temporal bone .One type of GJB2 mutation and 3 different types of SLC26A4 mutations were identified in the family .The proband(Ⅲ -1) ,her sister(Ⅲ -2) ,her mother(Ⅱ -4) and her father(Ⅱ -3) carried different biallelic mutations which were SLC26A4 c .919 -2A > G/p .H723R ,p .Q413R/c .919 -2A > G ,p .Q413R/p .H723R and GJB2 c . 235delC/c .235delC ,respectively .Conclusion Different from most reported deafness families with the same molecu‐lar etiology in each one ,interestingly ,the pathogenies were different among all affected members in this family . They were caused by different biallelic mutations of SLC26A4 or GJB2 .

Objective To investigate the characteristic changes of auditory brainstem response (ABR) of nor‐mal hearing infants .Methods A total of 212 cases (327 ears) normal hearing infants were enrolled for ABR test . According to age ,167 ears was in 1~3 months age group ,105 ears was in 3~6 months age group ,55 ears was in 6~12 months age group .Each group consisted of ABR date were statistically analyzed .Results For 75 dB nHL click sound stimulus ,the peak latency and interpeak latency of Ⅲ ,Ⅴ ,Ⅰ — Ⅲ ,Ⅲ — Ⅴ ,Ⅰ — Ⅴwaves of normal hearing infants progressively shortened as they grew older except the wave Ⅰ .The ABR thresholds were in significantly difference among the groups .The amplitude ratio of wave V/I increased with the grewing age .Each group of data showed statistically significant differences .When the stimulus intensity reached at thresholds ,the occurrence rate of wave I ,III and V were different as they grew older .Conclusion Infants with auditory nervous system was in con‐stant development and improvement ,all parts of the auditory pathway of neural development was not balanced .