Whole-Exome Sequencing Reveals Pediatric Rare Syndromic Hearing Loss / 罕见病研究

  Objective  To discuss the significance of genetic diagnosis of children with syndromic hearing loss by using whole-exome sequencing.  Methods  The clinical data of 34 children with sensorineural hearing loss were collected and the whole exons of genome of the children and their parents were sequenced and analyzed.  Results  Genetic causative gene and mutations have been identified in 19 children, including 4 genes (HARS2, USH2A, GATA3, MITF) related to rare syndromic hearing loss. Fifteen children were diagnosed with non-syndromic hearing loss related gene, including 8 cases with GJB2 mutation, 5 cases with SLC26A4 mutation and 2 cases with MYO15A mutation. Mutations of c.435_437del(p.K147del) and c.1403G > C (p.G468A) in gene HARS2, c.11389+1del in gene USH2A, c.1327delA(p.M443Wfs*33) in gene GATA3, c.627C > A(p.C209X) in gene MITF and c.8033_8057delinsG(p.N2678_D2686delinsS) in gene MYO15A were first reported.  Conclusions  Whole-exome sequencing helps the accurate diagnosis of causes of hearing loss, especially for the rare syndromic hearing loss with atypical clinical manifestations. Information from genetic testing may highlight further recommended exams of structure and functions of related organs.

Vestibular function features and prognosis of vestibular neuritis in children / 中华耳鼻咽喉头颈外科杂志

Objective@#To investigate the clinical characteristics, prognosis and affected branches of vestibular neuritis in children.@*Methods@#Twenty-five patients with vestibular neuritis in ENT department, Beijing Children′s Hospital, from October 2015 to October 2016, were collected. All patients were 4-14 (mean 9.8) years old including 17 boys and 8 girls. The clinical manifestations history, pure tone audiometry (PTA), vestibular function tests were done for each patient. We also took the blood samples for pathogenic virus in order to analyze the premorbid risk factors.@*Results@#Rotational vertigo were complained by all presents. There were 17 cases (68%, 17/25) with nausea and vomiting and 19 cases (76%, 19/25) with balance dysfunction. There were 12 cases (60%, 12/20) with positive results in 20 blood samples for virology, among which 6 cases of influenza B virus and 4 cases of herpes simplex virus, 1 case of cytomegalovirus and 1 case of coxsackie were identified. The results of PTA were normal. Bithermal caloric test was abnormal in 22 cases (88%, 22/25). The ocular vestibular-evoked myogenic potential (oVEMP) in 12 cases (48%, 12/25) and cervical vestibular-evoked myogenic potential (cVEMP) in 5 cases (20%, 5/25) were abnormal. The bithermal caloric test along with oVEMP and cVEMP in 4 cases (16%, 4/25) were abnormal. The bithermal caloric test and oVEMP in 7 cases (28%, 7/25) were abnormal. The bithermal caloric test in 11 cases (44%, 11/25) were abnormal. The oVEMP in 1 cases (4%, 1/25) was abnormal. The cVEMP in 1 cases (4%, 1/25) was abnormal. All patients recovered well, but the time varied. The symptoms of 21 patients were complete recovery within 1 month. 3 patients were complete recovery within 2 months (aged 8 - 14 years old). One patient was complete recovery within 6 months (aged 13 years old).@*Conclusion@#Rotary vertigo is most commonly in children with vestibular neuritis, accompany with imbalance and vomiting. The vestibular neuritis in children might be related with upper respiratory tract infection. Audiometry test is normal. Because of the bithermal caloric test and oVEMP are obvious abnormality, therefore it is speculated that the superior vestibular nerve may most commonly be affected. The younger patients with vestibular neuritis recovered more quickly than the older children.

A comparative study of the influence of emotional conflict on the emotional attention processing of the elderly and the young / 中华行为医学与脑科学杂志

Objective To explore the impact of emotional conflicts on the emotional attention pro-cessing between the elderly and the young. Methods A total of 37 young and 37 elderly participants were enrolled in the study. 2 (group:old group,young group) × 2 (Emotional valence of face:happy,fear) × 2 ( cue type:consistent and inconsistent) mixed experimental design was used among the emotional stroop task and eye tracking task for all of subjects. Results (1) Behavioral response analysis showed that stroop in-terference effects in young and old groups were not significant under different emotional conditions (F=0. 02, P>0. 05). (2) The internal characteristics of the face for the gaze time were analyzed,and the total gaze time of the elderly group (( 402. 28 ± 15. 88) ms ) was significantly longer than that of the younger group ((340. 52±15. 88) ms) under the happy-inconsistent condition(t=2. 75,P<0. 05). The total gaze time of the elderly group under the happy-inconsistent condition was significantly longer than under the fear-inconsis-tent condition((365. 96±13. 95) ms)(t=4. 32,P<0. 05). The first gaze time for a happy face was signifi-cantly longer than that for the fear face((315. 56±13. 13)ms vs (293. 51±13. 23)ms,t=4. 33,P<0. 05), and also showed a positive effect. Conclusion Emotional conflicts have an impact on the emotional atten-tion processing of the elderly,and the elderly are more likely to process the positive stimulation in the control stage.

Associated factors on sleep disorders in systemic lupus erythematosus patients / 中华风湿病学杂志

Objective To investigate the prevalence of sleep disorders and the relevant determinants in a cohort of systemic lupus erythematosus (SLE) patients.Methods One hundred patients with SLE were included in the study.Sleep quality was assessed using the Pittsburgh sleep quality index (PSQI).Depression,anxiety,quality of life,and fatigue were evaluated by patient health questionnaire (PHQ)-9,generalized anxiety disorder (GAD)-7,short form 36 health survey (SF-36),and visual analogue scale (VAS) respectively.The demographic and clinical data were also recorded.SLE disease activity and damage severity were assessed by systemic lupus erythematosus disease activity index (SLEDAI) and systemic lupus erythematosus damage index (SDI) respectively.Mann-Whitnry U test,t test,Logistic regression were used for statistically analysis.Results The prevalence of sleep disorders in SLE patients was 42％.Compared with patients without sleep disorders,the ratio of males and married patients,age,the score of SDI,PHQ-9,GAD-7,and fatigue were higher in SLE patients with sleep disorders,while the score of SF-36 was lower (r＜0.05).Age,SLEDAI,SDI,PHQ-9,GAD-7,and fatigue correlated positively with sleep disorders (The values of r were 0.215,0.230,0.311,0.529,0.455,0.541,P＜0.05).C3 and the score of SF-36 correlated negatively with sleep disorders (The values of r were-0.204,-0.342,-0.490,-0.464,-0.497,-0.590,-0.428,-0.478,-0.398,-0.412,-0.659respectively,P＜0.05).In multi-ple logistic regression analyses,gender (OR=22.22),anxiety (OR =2.895),body pain (OR =0.964),and energy (OR =0.947) were the independent determinants of sleep disorders (R2=0.494,P＜0.01).Conclusion Poor sleep quality is common in SLE patients.Gender,age,disease activity and severity,anxiety,depressed mood,and quality of life contribute significantly to sleep disorders in SLE.

Effects of noise competition on monosyllabic and disyllabic word perception in children / 中华耳鼻咽喉头颈外科杂志

Objective@#The purpose of the present study was to investigate the effects of noise competition on word perception in normal hearing (NH) children and children with cochlear implantation (CI).@*Methods@#To estimate the contribution of noise competition on speech perception, word perception in speech-shaped noise(SSN)and 4-talker babble noise(BN) with Mandarin Lexical Neighborhood Test were performed in 80 NH children and 89 children with CI. Corrected perception percentages were acquired in each group.@*Results@#Both signal to noise ratio (SNR) and noise type influenced the word perception. In NH group, corrected percentages of disyllabic word perception in SSN were 24.2%, 55.9%, 77.1%, 85.1% and 88.9% at -8, -4, 0, 4 and 8 dB SNR, corresponding corrected percentages of monosyllabic word were 13.9%, 39.5%, 60.1%, 68.8% and 80.1%, respectively. In BN noise, corrected percentages of disyllabic word were 2.4%, 24.3%, 55.6%, 74.3% and 86.2%, corresponding monosyllabic word were 2.3%, 20.8%, 47.2%, 61.1% and 74.8%, respectively. In CI group, corrected percentages of dissyllabic word in SSN and BN at 10 dB SNR were 65.5% and 58.1%, respectively. Corresponding monosyllabic word were 49.0% and 41.0%. For SNR=5 dB, corrected percentages of disyllabic word in SSN and BN were 50.0% and 38.1%, corresponding corrected percentages of monosyllabic word were 40.8% and 25.1%, respectively. Analysis indicated that the masking effect were significantly higher in BN compared with SSN.@*Conclusions@#Noise competition influence word perception performance significantly. In specific, the influence of noise on word perception is bigger in children with CI than in NH children. The masking effect is higher in BN noise when compared with SSN.

The Reliability and Validity Evaluation of Mandarin Version of the Parental Perspective Instrument for Pediatric Cochlear Implantation / 听力学及言语疾病杂志

Objective The aim of the present study is to evaluate the reliability and validity of the Mandarin version of the PP (MPP) .Methods The first step in the establishment was to translate the original English version into mandarin version with the method of cross -culture translation .The reliability was performed with the internal consistency analysis and test -retest reliability .The validity was performed for the content validity and structure va-lidity .The samples were from 80 Chinese CI children ,and 43 parents answered this questionnaire again 1 month lat-er to evaluate the test -retest reliability .The average age at cochlear implantation were 26 ± 14 months ,ranging from 7 months to 68 months ,the average duration of CI use were 10 ± 7 months ,ranging from 0 month to 24 months .Results The reliability analysis indicates that the Cronbach'sαcoefficient was 0 .797 ,except for the well-being and happiness ,education ,whose coefficients are respectively 0 .303 ,and 0 .341 ,all of the other sundomainscoefficient were greater than 0 .5 ,indicating the internal consistency was good .Test -retest reliability of the scale Cronbach'sαwas satisfactory .All subdomains and total score of the scale coefficients were greater than 0 .70(P<0 .01) .The validity analysis indicated that the pearson correlation coefficients among the total scale and the 8 subdo-mains were 0 .395~0 .992 ,the correlation coefficients among each subdomains were 0 .09~0 .654 ,which confirmed with the psychological characteristics ,proving its good structure validity .Conclusion The Chinese version of the PP show s good reliability and validity and can be used to evaluation the quality of life in mandarin CI children.

Cross-sectional study of hearing loss among freshmen in university / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#The purpose of the present study was to investigate the prevalence of hearing loss by studying the current status of hearing loss, risk factors of hearing loss, exposure level of noise, and everyday habit of hearing in a group of university students, so as to provide information for hearing loss prevention in university students.@*METHOD@#According to the purposive sampling method, 642 freshmen students participated in the study. Pure tone audiometry, and exposure level of noise scale were performed in all participants.@*RESULT@#(1) According to the hearing loss criterion of WHO/PDH97.3, high frequency hearing loss, and noise induced threshold shifts, the hearing loss prevalence was 0.36%, 20.91% and 6.73%, respectively. (2) Multivariant Logistic regression analysis of high-frequency hearing loss indicated that traffic noise exposure and ear infection were risk factor of high frequency hearing loss.@*CONCLUSION@#There is a high prevalence of high frequency hearing loss in university students (20.91%), which was high related with traffic noise exposure and ear infection.

Assessment and Monitoring of the LittlEARS? Auditory Questionnaire Used for Young Hearing Aid Users in Auditory Speech Development / 听力学及言语疾病杂志

Objective The objective of the present study was to evaluate the early auditory development in hearing impaired infants by LittlEARS? auditory questionnaire .Methods A total of 162 hearing impaired infants participated in the study .LEAQ consists of 35 items ,which were further divided into receptive auditory behavior , semantic auditory behavior ,and productive auditory behavior .The assessment was performed before hearing aid fit‐ting ,3 ,6 and 12 months after hearing aid fitting ,respectively .Results LEAQ scores improved dramatically in the first 12 months after hearing aid fitting ,total LEAQ scores prior to hearing aid fitting ,3 ,6 and 12 months after hearing aid fitting were 10 .47 ,17 .73 ,20 .64 and 26 .47 ,respectively .The receptive auditory behavior performance improved significantly after 3 months of hearing aid use .The duration of hearing aid use for dramatic improvement of semantic auditory behaviors ,and productive auditory behavior was 6 and 12 months ,respectively .The age of hearing aid fitting ,living environments ,degrees of parent education and degrees of hearing loss influenced LEAQscores significantly .Conclusion Hearing impaired infants exhibited significant auditory improvement in the first 12 months after hearing aids fitting .Early hearing aid fitting ,high level of parent education and well living environ‐ments promote preverbal auditory development .LEAQ is an effective tool for clinical auditory assessment and moni‐toring in infants and little toddlers .

A Pilot Study of Noise Effect on Speech Perception in Young Children with Normal Hearing / 听力学及言语疾病杂志

Objective To study normal hearing children's speech perception in Speech Spectrum -Shaped Noise (SSN) and Babble Noise (BN) using the Mandarin lexical neighborhood test .Methods Thirty -four children with normal hearing were included in this study and randomly assigned to the SSN group and the BN group .Each child was given the 12 lists of Mandarin lexical neighborhood test ,word recognition scores were acquired at different SNRs .Results SNR50 of dissyllablic easy word lists ,dissyllablic hard word lists ,monosyllable easy word lists , monosyllable hard word lists in SSN were -3 dB ,-0 .5 dB ,-1 dB and 3 .5 dB ,respectively ;SNR50 of the four categories lists in BN were -3 dB ,2 dB ,0 .5 dB and 10 dB ,respectively .Lexical effects had a significant influence on spoken word recognition in noise .Especially ,word recognition scores of easy words were higher than those of hard words ,dissyllablic words were better than monosyllable words .Conclusion The masking effects of babble noise on spoken speech perception are stronger than speech spectrum -shaped noise for 3-6 year-old normal hearing chil‐dren .Lexical effects also affet children's speech recognition in noise .

Effects of Pre-implant Hearing Aid Fitting on the Open-set Word Recognition in Children with Cochlear Implants during Infants / 听力学及言语疾病杂志

Objective To explore the impact of pre-implant hearing aid fitting on the early open-set word recognition in children who received a cochlear implant(CI) when they were infants .Methods A total of 289 chil‐dren who received a cochlear implant between 0 .9 and 3 .0 years of age were included in this study .According to pre-implant hearing aid fitting ,participants were divided into hearing aid group and non-hearing aid group .The open-set word identification abilities of the cochlear implant children were evaluated via Mandarin Lexical Neighborhood test(M -LNT) .Results Comparisons of the scores of M -LNT between two groups displayed no significant differ‐ence at the same identification age or duration of CI use .Conclusion The abilities of the early open-set word rec‐ognition between hearing aid group and non-hearing aid group didn’t make significant differences for the CI chil‐dren w ho received a cochlear implant betw een 0 .9 and 3 .0 years old .

Analysis of clinical features, metabolic profiling and gene mutations of patients with ornithine transcarbamylase deficiency / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To analyze the clinical features, metabolic profiling and gene mutations of patients with ornithine transcarbamylase deficiency (OTCD) and explore the molecular pathogenesis of OTCD in order to provide a solution for molecular diagnostics and genetic counseling.</p><p><b>METHODS</b>Clinical data of 3 neonates were analyzed. The amino acids level in blood was analyzed with mass spectrum technology. PCR was used to amplify all the 10 exons of OTC gene. The PCR products were directly sequenced to detect the mutations.</p><p><b>RESULTS</b>All of the 3 cases had neonatal onset and showed poor reaction, feeding difficulty, convulsion and neonatal infection. Citrulline levels were significantly decreased. Case 1 had a missense mutation of Y183C. Case 2 showed a missense mutation of V339G in exon 10. And a missense mutations of W332S in exon 9 was detected in case 3.</p><p><b>CONCLUSION</b>Analysis of OTC gene sequences can be used for the diagnosis of OTCD and screening of asymptomatic carriers. Mutation analysis is important for prenatal diagnosis of individuals with a positive family history and genetic counseling. The V339G and W332S mutations have been discovered for the first time. Patients with such mutations may have onset of the disease during neonatal period.</p>

Establishment of a Template for the Assessment of Hearing Aids Outcomes / 听力学及言语疾病杂志

Objective The objective of the present study was to establish a template for the self assessment of hearing aids outcomes according to the evaluation of a large group of hearing aid users .Methods In total ,1 724 subjects participated in the study .The Chinese version of International Outcome Inventory for Hearing Aids (IOI-HA) was used as the evaluation tool .IOI -HA is a seven -item questionnaire ,each item is designed to assess a specific outcome domain .Each item was designed with a five -point rating scale ,a higher rating indicates better outcome .According to the subjects'self reported hearing difficulty when they were not wearing hearing aids (unaid-ed) ,the subjects were divided into two groups :self reported hearing difficulty to be no ,mild ,moderate group ,and self reported hearing difficulty to be moderately severe or severe group ,respectively .Templates for each group were established according to the analysis of the item ratings .Results One thousand two hundred and forty -seven sub-jects accept the investigation ,with a response rate of 72 .3% ,and 1203 completed responses were included in the fi-nal analysis .The Chinese version of IOI-HA scores showed a skewed distribution ,with a mean score ranging from 3 .52 to 4 .19 .The total IOI-HA scores ranged from 9 to 35 ,and the mean total score was 26 .30 .Results showed that for the self reported hearing difficulty (unaided) to be none ,mild or moderate group ,the mean scores for the i-tem of daily use ,benefit ,residual activity limitation ,satisfaction ,residual participation restrictions ,impact on others ,and quality of life were 4 .14 ,3 .67 ,3 .76 ,3 .58 ,3 .56 ,3 .93 and 3 .69 ,respectively .The corresponding mean scores for the self reported hearing difficulty (unaided) to be moderately severe or severe group were 4 .19 ,3 .63 ,3 .64 ,3 . 65 ,3 .52 ,3 .89 ,and 3 .79 ,respectively .A template for the Chinese IOI -HA was established according to the above data .Conclusion The template for the Chinese version of IOI -HA could be served as an effective tool to measure the general effectiveness of the hearing aid outcomes and hearing aid fitting in China ,which would also facilitate the international outcome comparison cross culture .

Analysis of reliability of the Chinese version of satisfaction with amplification in daily life / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the reliability of the Chinese version of Satisfaction with Amplification in Daily Life (SADL).@*METHOD@#The cross-cultural adaption measures were used to translate the SADL into Chinese version. Thirty hearing aid users no younger than 18 years old were included. Test-retest analysis was administrated to the patients without significant changes in health and social status with an interval of two weeks between test and retest.@*RESULT@#Test-retest reliability of the SADL was proved to be satisfactory. All subscales and global score had coefficients that exceeded 0.70 (P < 0.01). Except for "personal image", the score of other subscales and global score in this study are higher than the U. S. interim norms described by Cox and Alexander (1999) (P < 0.01).@*CONCLUSION@#This study completed the translation and test-retest analysis of Chinese version of SADL. We need further discussion about validation of Chinese version of SADL in order to meet basic psychometric criteria of a standard outcome measurement.

The effects of hearing level on auditory performance in infants with hearing aids / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#The purpose of this study is to investigate the effects of hearing level on auditory performance in infants with hearing aids and provide a clinical database for their hearing and speech habilitation.@*METHOD@#A total of 32 infants with prelingual hearing loss participated in this study, ranging in age at hearing aid fitting from 3 to 34 months with a mean of 16 months. According to their hearing level, they were divided into three groups. Infants in group A were with moderate hearing loss (41-60 dB HL). Infants in group B were with severe hearing loss (61-80 dB HL). Infants in group C were with profound hearing loss (>81 dB HL). The infant-toddler meaningful auditory integration scale (IT-MAIS) was used to evaluate their auditory performance. The evaluation was performed before hearing aid fitting and 1, 3, 6, 9, 12 months after fitting.@*RESULT@#The mean scores of auditory performance showed significant improvements with time of hearing aid use for the three groups of infants (P 0.05). However, significant differences were observed between group A and C at 3, 6, 9, 12 months after fitting (P < 0.05). Significant differences were also shown between group B and C at 1, 3, 6, 9, 12 months after fitting (P < 0.05).@*CONCLUSION@#Auditory performance of infants with prelingual hearing loss developed significantly with the use of hearing aids within the first year after fitting. The degree of hearing loss in infants had significant influence on the development of auditory performance.

The application of vibrant sound bridge in microtia whose reconstructive external auditory canal occurred atresia / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the effect of vibrant sound bridge implantation in microtia whose reconstructive external auditory canal occurred atresia.@*METHOD@#Three cases (2 males and 1 female) of microtia had underwent hearing reconstruction operation (Include the external ear canal reconstructive surgery and tympanoplasty). The age ranged from 15 to 18 years and the average age was 17 years. All the 3 cases suffered from conductive hearing loss with the air-bone gap ranging from 51.6 to 65.0 dB HL and the average value being 56. 3 dB HL. All the 3 cases underwent vibrant sound bridge implantation, including the floating mass transducer implanted in the head of stapes in 2 cases and in the niche of round window in 1 case.@*RESULT@#The postoperative hearing level improved from 21.6 to 52.5 dB HL with an average of 32.2 dB HL. There were no complications such as vertigo, tinnitus and facial paralysis.@*CONCLUSION@#Through vibrant sound bridge implantation, the hearing level of microtia whose reconstructive external auditory canal occurred atresia was improved effectively.

Effect of age at cochlear implantation on auditory performance at the early stage after surgery in infants / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To evaluate the auditory performance of infants with cochlear implants at the early stage after surgery, summarize the development of auditory ability, investigate the effect of age at cochlear implantation on auditory performance within the first year after surgery and provide a reference for their habilitation program.@*METHOD@#A total of 272 infants with prelingually profound hearing loss participated in this study, ranging in age at cochlear implantation from 18 to 36 months. The mean age was 21 months with a standard deviation of 7 months. Infants with cochlear implants were divided into three groups according to their age at implantation. Infants in group A were implanted under 18 months of age. Infants in group B were implanted between 18 and 24 months of age. Infants in group C were implanted between 25 and 36 months of age. Categories of auditory performance (CAP) was used to evaluate the auditory performance, which rates auditory abilities in eight categories for a scale of 0 to 7.@*RESULT@#The mean scores of CAP for all infants at each interval were significantly different after implantation. Significant differences were observed in mean scores of CAP among these three groups in 1 and 3 months after switch-on. However there were no significant differences in pre-operation, 6, 9 and 12 months after switch-on.@*CONCLUSION@#There is a significant improvement in auditory performance of infants with prelingually profound hearing loss within the first year after cochlear implantation. The age at cochlear implantation has no critical influence on the development of auditory capabilities at the early stage after surgery. CAP is a practical tool which can be used in clinic in China.

Clinical study of levothyroxine sodium in the treatment of patients with heart failure and euthyroid sick syndrome / 中国基层医药

Objective To investigate effect on patients with chronic heart failure complicated with euthyroid sick syndrome(ESS) who had given small dose of levothyroxine sodium that based on the routine anti heart failure therapy.Methods 70 patients with chronic cardiac insufficiency and ESS were randomly divided into control group and treatment group,the control group patients were given routine anti heart failure treatment,while the treatment group plus levothyroxine sodium tablets for 1year.Observe and compare the relevant examination and laboratory indexes of 2 groups before and after treatment,follow up the number of vascular events about 2 groups within 1year.Results The 6 minutes walk test [ Respectively:(383.0 ± 48.7 ) m,(339.3 ± 37.8 ) m ],NYHA heart function classification[ Respectively:(2.7 ± 0.4)grade,(3.2 ±0.5) grade],LVEF[ Respectively:(40.87 ± 8.18)％,(35.38 ± 7.35 ) ％ ],BNP [ Respectively:( 197.74 ± 20.58 ) ng/L,( 302.01 ± 10.15 ) ng/L ] water of the treatment group [Respectively:(317.0 ± 36.6)m,(323.4 ± 44.8)m,(3.5 ± 0.5) grade,(3.5 ± 0.3) grade,(33.18 ± 7.29)％,(34.74 ± 9.81 ) ％,( 369.75 ± 21.15 ) ng/L,(345.56 ± 18.65 ) ng/L] and the control group were better than before treatment.The treatment group was more significant,the two groups after treatment had a significant difference ( t=2.974,3.698,9.136,7.476,all P ＜ 0.05 ),the number of cardiovascular events of the treatment group was decreased within 1 year,compared with the control group the difference was statistically significant (x2 = 4.96,P ＜ 0.05 ).Conclusion In the treatment of conventional heart failure therapy combined with a small dose of levothyroxine sodium therapy on chronic heart failure patients can improve the patients' quality of life,reduce medical costs.

The contribution of different cochlear insertion region to Mandarin speech perception in users of cochlear implant / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To study the effect of cochlear electrode coverage and different insertion region on speech recognition, especially tone perception of cochlear implant users whose native language is Mandarin Chinese.@*METHOD@#Setting seven test conditions by fitting software. All conditions were created by switching on/off respective channels in order to simulate different insertion position. Then Mandarin CI users received 4 Speech tests, including Vowel Identification test, Consonant Identification test, Tone Identification test-male speaker, Mandarin HINT test (SRS) in quiet and noise.@*RESULT@#To all test conditions: the average score of vowel identification was significantly different, from 56% to 91% (Rank sum test, P 0.05). However the more channels activated, the higher scores obtained, from 68% to 81%.@*CONCLUSION@#This study shows that there is a correlation between insertion depth and speech recognition. Because all parts of the basement membrane can help CI users to improve their speech recognition ability, it is very important to enhance verbal communication ability and social interaction ability of CI users by increasing insertion depth and actively stimulating the top region of cochlear.

Audiometric configuration distribution of hearing loss in a clinical population / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#Audiometric configuration distribution of hearing loss was analyzed for public policy efforts of hearing loss prevention and rehabilitation.@*METHOD@#According to the archived records of the Clinical Audiology Center of Beijing Tongren Hospital, Capital Medical University, audiometric configuration distribution was analyzed by different type,degree of hearing loss and age factor.@*RESULT@#1. Overall audiometric configuration distribution: the percentages of "sloping", "rising", "flat", "U-shaped", and other type of audiometric configuration distributions are 52.8%, 7.6%, 15.4%, 13.2% and 11.0%, respectively. No difference was found between male and female in the audiometric configuration distribution; 2. Audiometric configuration distribution by type of hearing loss: sloping hearing loss dominants sensorineural and mixed hearing loss; 3. Audiometric configuration distribution by degree of hearing loss: sloping hearing loss dominants mild, moderate and severe hearing loss, U-shaped hearing loss dominants profound hearing loss; 4. Audiometric configuration distribution by age: for age group 5 to 7, the highest prevalence of audiometric configuration is "sloping", followed by "flat", "U-shaped", other type and "rising"; with the age increasing, the percentage of sloping hearing loss increased dramatically.@*CONCLUSION@#Audiometric configuration distribution shows obvious characteristic of on the type, degree of hearing loss and age factor. "sloping" hearing loss dominates the audiometric configuration.

Evaluation of a transient noise reduction strategy on the loudness perception and sound quality / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#A current technology for detecting and controlling transient noise in hearing aids (AntiShock) was evaluated. The objective was to evaluate AntiShock on loudness control and whether results in negative changes in sound quality of speech, transient noise and environmental noise and provide implications for hearing aid fitting.@*METHOD@#Twenty-four subjects with sensorineural hearing loss participated in the study. In a single-blinded paradigm, the subjects were asked to rate loudness of transient noise and distortion of speech, transient noise and environmental noise with the AntiShock in both on and off conditions.@*RESULT@#(1) The percentage of the transient noise rated as soft, comfortable, loud, too loud was 3.0%, 72.7%, 22.9% and 1.4%, respectively. There were significant differences in mean scores of loudness perception among listening conditions and between genders by a Two-Way ANOVA, the P values were 0.009 and 0.001, respectively. (2) The percentage of the speech rated as mild distorted, understandable, clear and very clear was 2.5%, 30.6%, 32.9% and 34.0%, respectively. There were significant differences in mean scores of speech distortion under different listening conditions by an One-Way ANOVA (P 0.05). (4) The percentage of the environmental noise rated as mild distorted, clear but soft, clear and natural was 0.4%, 0.8% and 98.8%, respectively. No significant differences in mean scores of nature of environmental noise was found between different listening conditions by an Independent-Samples T Test (P > 0.05).@*CONCLUSION@#AntiShock showed positive effects on the loudness control of the transient noise. Quality of speech, transient noise and environmental noise were not impacted by AntiShock.