Study on the correlationship between hearing loss and cognitive decline among older adults in Beijing / 中华健康管理学杂志

Objective:To study the correlation between hearing loss and cognitive decline in community-dwelling older adults and to analyze the influencing factors.Methods:Using Pure-tone audiometry, the Hearing Handicap Inventory for the Elderly (HHIE), Mini-Mental State Examination (MMSE), Patient Health Questionnaire-9 (PHQ-9), and Generalized Anxiety Disorder, a survey was conducted with 492 community-dwelling older adults aged 60 years and above in Beijing. Age, gender, education level, hearing loss, levels of anxiety and depression, and other factors were used as independent variables, and the MMSE scores were used as dependent variables. Analysis of variance and multiple linear regression were performed.Results:Among the 492 subjects, based on the better ears, 418 (85.0%) had hearing loss and 160 (32.5%) had disabled hearing loss. Factors such as age (β=-0.33, P<0.01), gender (β=0.09, P=0.04), education level (β=0.31, P<0.01), hearing loss (β=-0.11, P=0.02) and marital status (β=0.56, P=0.02) were significantly related to cognitive decline. Gender (β=-0.17, P<0.01) and hearing loss (β=0.08, P=0.02) correlated with anxiety indicators; gender (β=-0.84, P=0.02) and hearing loss (β=0.04, P=0.01) also correlated with depression indicators. Conclusion:Results indicated a correlation between hearing loss and cognitive decline, and between hearing loss and anxiety and depression among community-dwelling older adults in Beijing.

Analysis of genotypes and hearing phenotypes of mutation infants with deafness / 临床耳鼻咽喉头颈外科杂志

Objective@#The aim of this study is to explore the genotype and hearing phenotype of deaf infants with mutation of GJB2 gene.@*Method@#Subjects were 121 infants with GJB2 gene mutations who were treated in the Children's Hearing Diagnosis Center of Beijing Tongren hospital. All subjects were accepted to undertake the universal newborns hearing screening（UNHS） and series of objective audiometry, including auditory brainstem response, distortion product otoacoustic emission, auditory steady-state response and other audiological tests. All subjects were screened for nine pathogenic variants in four genes or all exons of the GJB2 gene, and then were diagnosed as infants with GJB2 gene mutations. Initially, analyzing their genotypes and hearing phenotypes generally. Then, the subjects were divided into two groups according to the genotypes: T/T group（truncated/truncated mutations, 89 cases） and T/NT group（truncated/non-truncated mutations, 32 cases）. Chi-square test was used to analyze the results of UNHS, hearing degree, audiogram patterns and symmetry/asymmetry of binaural hearing phenotype. Eventually, analyzing the results of UNHS. @*Result@#The most common truncated mutation was c.235delC（64.88%, 157/242） and the most common non-truncated mutation was c.109G>A（11.16%, 27/242）. The homozygous mutation of c.235delC/c.235delC was the dominant in T/T group（38.84%, 47/121）, and the compound heterozygous mutation of c.235delC/c.109G>A was the dominant in T/NT group（18.18%, 22/121）. 81.82%（99/121） of subjects failed in UNHS, including 74.38%（90/121） with bilateral reference, 7.44%（9/121） with a single pass. The refer rate of UNHS of group T/T and T/NT were 86.52%（77/89） and 68.75%, respectively. There was a statistically significant difference between the two groups（P<0.05）. 85.95%（104/121） of subjects were diagnosed as hearing loss and 14.05%（17/121） of subjects were diagnosed as normal hearing. The degree of hearing loss: profound, severe, moderate and mild were 31.40%（38/121）, 19.01%（23/121）, 24.79%（30/121） and 10.74%（13/121）, respectively. There was no subjects with normal hearing in T/T group and individuals with severe and profound hearing loss accounted for the highest proportion（65.17%, 58/89）, while in T/NT group, normal hearing accounted for 53.13%（17/32） and mild and moderate hearing loss accounted for the highest proportion（37.5%, 12/32）. There was statistically significant difference between the two groups（P<0.05）. Of 104 patients（208 ears） with hearing loss, the audiogram patterns: flat, descending, ascending, residual, Valley and other types were 49.03%（102/208）, 12.02%（25/208）, 8.65%（18/208）, 7.69%（16/204）, 3.36%（7/204） and 19.23%（40/204）, respectively. The two most common types in T/T group were flat（47.19%, 84/178） and other types（20.22%, 36/178）, while in T/NT group were flat（60.00%, 18/30） and ascending（20.00%, 6/30）. There was statistically significant difference between the two groups（P<0.05）. There were 50 cases（48.07%） with symmetrical hearing phenotype and 54 cases（51.93%） with asymmetrical hearing phenotype. Asymmetry was predominant in T/T group（53.93%, 48/89）, and symmetry was predominant in T/NT group（60.00%, 9/15）. There was no statistically significant difference between the two groups（P>0.05）. @*Conclusion@#In this study, c.235delC/c.235delC homozygous mutation was dominant in T/T group and c.235delC/c.109G>A heterozygous mutation was dominant in T/NT Group. The hearing phenotypes in T/T group were mostly bilateral asymmetric severe hearing loss, and those in T/NT Group were bilateral symmetric mild to moderate hearing loss, special attention should be paid to the audiological characteristics of different genotypes.

Analysis of genotypes and hearing phenotypes of mutation infants with deafness / 临床耳鼻咽喉头颈外科杂志

The aim of this study is to explore the genotype and hearing phenotype of deaf infants with mutation of gene. Subjects were 121 infants with gene mutations who were treated in the Children's Hearing Diagnosis Center of Beijing Tongren hospital. All subjects were accepted to undertake the universal newborns hearing screening（UNHS） and series of objective audiometry, including auditory brainstem response, distortion product otoacoustic emission, auditory steady-state response and other audiological tests. All subjects were screened for nine pathogenic variants in four genes or all exons of the gene, and then were diagnosed as infants with gene mutations. Initially, analyzing their genotypes and hearing phenotypes generally. Then, the subjects were divided into two groups according to the genotypes: T/T group（truncated/truncated mutations, 89 cases） and T/NT group（truncated/non-truncated mutations, 32 cases）. Chi-square test was used to analyze the results of UNHS, hearing degree, audiogram patterns and symmetry/asymmetry of binaural hearing phenotype. Eventually, analyzing the results of UNHS. The most common truncated mutation was c.235delC（64.88%, 157/242） and the most common non-truncated mutation was c.109G>A（11.16%, 27/242）. The homozygous mutation of c.235delC/c.235delC was the dominant in T/T group（38.84%, 47/121）, and the compound heterozygous mutation of c.235delC/c.109G>A was the dominant in T/NT group（18.18%, 22/121）. 81.82%（99/121） of subjects failed in UNHS, including 74.38%（90/121） with bilateral reference, 7.44%（9/121） with a single pass. The refer rate of UNHS of group T/T and T/NT were 86.52%（77/89） and 68.75%, respectively. There was a statistically significant difference between the two groups（0.05）. In this study, c.235delC/c.235delC homozygous mutation was dominant in T/T group and c.235delC/c.109G>A heterozygous mutation was dominant in T/NT Group. The hearing phenotypes in T/T group were mostly bilateral asymmetric severe hearing loss, and those in T/NT Group were bilateral symmetric mild to moderate hearing loss, special attention should be paid to the audiological characteristics of different genotypes.

An Analysis of Hearing Screening Results from 616 Preschool Children / 听力学及言语疾病杂志

Objective To explore the feasibility and effectiveness of otoacoustic emission (OAE) in hearing screening for preschool children .Methods A total of 616 preschool children aged 3～6 (mean age 4 .6 years old) were included in this study .All the subjects received transiently evoked otoacoustic emission (TEOAE) test by trained personnel .The children who failed the hearing screening received diagnostic examination in the hearing ex-amination center .Results Of 616 children ,66 (10 .71％ ) failed the hearing screening and 16 children received audi-ological assessment .In these 16 children ,8 had otitis media with effusion (5 with mild conductive hearing loss ) ,5 had impacted cerumen ,3 had normal hearing ,and none had sensorineural hearing loss .Conclusion OAE can be used effectively in the hearing screening for preschool children .But how to improve the referral rate of children who failed the hearing screening needs further discussion .

Research progress on the etiology of delayed-onset hearing loss in children / 中华耳鼻咽喉头颈外科杂志

Newborn hearing screening is an effective method for early detection of hearing loss, however, it is not able to detect delayed-onset hearing loss. By exploring the etiology of delayed-onset hearing loss in children, it can provide a clinical basis for early detection of delayed-onset hearing loss. Mutations in SLC26A4, mitochondrial, GJB2 and other genes, enlarged vestibular aqueduct, congenital cytomegalovirus infection, extracorporeal membrane oxygenation, and auditory neuropathy et al were more commonly reported risk factors. In this paper, the risk factors related to delayed-onset hearing loss, which are divided into 5 categories: genetic mutation, abnormal inner ear malformation, perinatal factors, auditory neuropathy and no identifiable cause, are reviewed and analyzed.

Pathogen profile and risk factors of infected diabetic foot ulcers / 中国感染与化疗杂志

Objective To explore and analyze the pathogen proifle and risk factors of infected diabetic foot ulcers, and propose appropriate prevention and intervention measures for early recovery of patients.Methods The data of 120 patients with diabetic foot ulcers treated in our hospital from February 2012 to May 2014 were retrospectively analyzed. These patients were classified into infection group (38 patients) and non-infection group (82 patients) based on whether their ulcer was infected or not. The pathogens and risk factors of infection in diabetic foot ulcer were analyzed.Results In this study, 43 strains of pathogenic bacteria were isolated from 38 cases of infection, of which gram positive bacteria accounted for 51.2 %, gram negative bacteria 41.9 %, and fungi 7.0 %.Staphylococcus aureus,Pseudomonas aeruginosa and Enterobacter cloacae were the top three pathogens, accounting for 16.3 % each. Univariate analysis showed that diabetic vascular complication, retinopathy, more than 2 foci of ulcer, osteomyelitis, and diabetic nephropathy were closely associated with infection (P<0.05). Multivariate logistic regression analysis showed that HDL-C, albumin, diabetic vascular complication, number of ulcers, osteomyelitis were independent risk factors for infection in patients with diabetic foot ulcer. Conclusions HDL-C, red blood cell, hemoglobin, diabetic complications, white blood cell, triglyceride, albumin, diabetic vascular complication and smoking are the risk factors for infection in patients with diabetic foot ulcer. These factors should be prevented and controlled to reduce the incidence of infection in diabetic foot ulcer.

Clinical Observation of Jinkui Shenqi Pill versus Shengjing Capsule in the Treatment of Oligoasthenozoosper-mia under Behavioral Intervention / 中国药房

OBJECTIVE:To compare the efficacy and safety of Jinkui shenqi pill versus Shengjing capsule in the treatment of oligoasthenozoosperimia under behavioral intervention. METHODS:98 patients with oligoasthenozoosperimia were randomly divid-ed into Shengjing capsule group(49 cases)and Jinkui shenqi pill(49 cases). All patients received intervention treatment(cognitive intervention,psychological intervention,diet intervention and exercise intervention,etc.). Based on it,Shengjing capsule group re-ceived 1.6 g Shengjing capsules,orally,3 times a day;Jinkui shenqi pill group received 6 g Jinkui shenqi pill,orally,twice a day. They were treated for 3 months. Clinical efficacy,semen quality (semen volume,sperm concentration,sperm motility rate, sperm motility)and sex hormone levels [testosterone(T),follicle stimulating hormone(FSH),luteinizing hormone(HLH),pro-gesterone(P),prolactin(PRL)] before and after treatment,and the incidence of adverse reactions in 2 groups were observed. RE-SULTS:The total effective rate in Shengjing capsule group was significantly higher than Jinkui shenqi pill group,with statistical significance(P0.05). After treatment,semen quality in 2 groups was significantly higher than before,semen volume and sperm motility in Shengjing cap-sule group were higher than Jinkui shenqi pill group,with statistical significances(P0.05). And there was no significant difference in the incidence of adverse reactions(P>0.05). CONCLUSIONS:Under behavioral interven-tion,Shengjing capsule has better efficacy than Jinkui shenqi pill in the treatment of oligoasthenozoosperimia,it can significantly improve semen quality,while Jinkui shenqi pill is better in terms of improving sex hormone levels;and both show good safety.

The review of newborn hearing screening program in neonatal intensive care unit / 临床耳鼻咽喉头颈外科杂志

The incidence of hearing impairment in neonatal intensive care unit (NICU) was much higher than that of well-baby nursery. The incidence of the former was 2%-4%, whereas that of the latter was 0.1%-0.3%. Furthermore, the incidence of auditory neuropathy spectrum disorder, progressive and delayed hearing loss was also higher than those of other infants. Therefore, the newborn hearing screening program in NICU has become an important part of pediatric audiology. In this paper, we reviewed the previous studies and suggested the special procedure of hearing screening and following-up which based on the physiological and pathological characteristics of NICU in order to detect hearing impaired as early as possible.

The Audiological Characteristics of Neonate with Severe Hyperbilirubinemia Need to be Exchanged Transfusion / 听力学及言语疾病杂志

Objective To study the change of audiological characteristics of neonate with severe hyperbilirubi‐nemia need to be exchanged transfusion ,and to explore the correlation between the peak concentrations of serum to‐tal bilirubin and hearing loss .Methods A total of 130 cases of neonate diagnosed with severe hyperbilirubinemia need to be exchanged transfusion were included in this study .Hearing tests of auditory brainstem response (ABR) , distortion product otoacoustic emission (DPOAE) and acoustic immittance were administered to these neonates and they were followed up three months old .They were divided into the normal group ,the mild - moderate group and the severe - extreme group according to the hearing tests results of three months .The peak concentrations of ser‐um total bilirubin and hearing condition among the three groups were analyzed .Results There were 85 neonates with normal hearing (65 .38% ,85/130) ,45 with hearing loss (33 .85% ,45/130 .For 88 ears ,there were 2 cases of single ear and 43 cases of both ears) ,including 11 cases of mild - moderate group (22 ears ,24 .44% ,11/45) and 34 cases with severe - extreme group (66 ears ,75 .56% ,34/45) .There were 16 neonates (32 ears) met the crite‐rion of auditory neuropathy (12 .31% ,16/130) .The difference of the peak concentrations of serum total bilirubin a‐mong the three groups was statistically significant (F=16 .525 ,P=16 .525) .And positive correlation was observed between ABR threshold and peak serum total bilirubin concentration (r=0 .584 ,P<0 .001) .Conclusion The ma‐jor feature of the neonate with severe hyperbilirubinemia need to be exchanged transfusion was extremely severe sen‐sorineural hearing loss in both ears ;and the higher probability of serious hearing loss with the higher peak concen‐trations of serum total bilirubin .

The Characteristics of Spontaneous Otoacoustic Emissions in Full -term Newborns / 听力学及言语疾病杂志

Objective To analyze the characteristics of spontaneous otoacoustic emission in full-term newbo‐rns .Methods The Capella OAE equipment (Madsen ,Denmark) was used to test Spontaneous Otoacoustic Emission (SOAE) in 147 cases (236ears) who have passed the newborn hearing screening with TEOAE(Transient Evoked Otoacoustic Emissions) .Results The SOAE incidence was 56 .77% (male 41 .51% ,female 69 .23% ;left ear 49 .14% ,right ear 64 .17% ) .It was significantly higher in females (P<0 .05) and in right ear (P<0 .05) .The av‐erage amplitude was 11 .78 ± 8 .36 dB SPL( 11 .73 ± 8 .25 dB in male ,11 .81 ± 8 .43 dB SPL in female;11 .97 ± 8 .56 dB SPL in the left ear ,11 .65 ± 8 .22 dB SPL in the right ear) .There were significant differences in genders(P<0 .01) .The frequency of SOAE focused on 3 .2~ 3 .7 kHz(2 .9~3 .4 kHz in males ,3 .4~3 .9 kHz in females ;3 .2~3 .7 kHz in the left ears ,3 .2~3 .6 kHz in the right ears) .There were significant differences in genders(P<0 .01) .The average peak of SOAE was 3 .70 ± 2 .75(3 .86 ± 2 .87 in males ,3 .62 ± 2 .70 in females;3 .70 ± 3 .02 in the left ears ,3 .70 ± 2 .55 in the right ears) .There were no significant differences in genders and laterality .Conclusion The characteristics of SOAE in full-term newborns include higher incidences ,multiple peaks and high frequency distribution .

Cost-effectiveness of three-stage newborns hearing screening in Beijing / 中华流行病学杂志

<p><b>OBJECTIVE</b>To evaluate the cost-effectiveness of two-stage and three-stage hearing screenings for newborns.</p><p><b>METHODS</b>Hearing screening was performed for the normal newborns born in 7 hospitals in Beijing from October 2010 to December 2012 by using two stage and three stage strategies as well as hearing diagnostic test, and the cost effectiveness evaluation of two strategies was conducted. The data about the cost of screening and diagnostic test were from the hospitals. The data about car fare and charge for loss of working time of parents were collected through questionnaire survey. The sensitivity was analyzed according to the compliance rate.</p><p><b>RESULTS</b>A total of 62,695 newborns received initial hearing screening, 5,809 newborns failed, the positive rate was 9.30%. A total of 4,933 newborns received rescreening, 972 newborns failed, the positive rate was 19.70%. Among the newborns failed in rescreening, 412 were provided with hearing diagnostic test and 360 received diagnostic test. The diagnostic test indicated that the hearing of 217 newborns were abnormal (60.28%). A total of 276 newborns received the third screening, 163 newborns failed, in which 125 received diagnostic test and 112 had abnormal hearing (45 had moderate and above hearing impairment), the abnormal rate was 89.60%. The average cost for three-stage screening (37,242 yuan RMB per case) was higher than that for two-stage screening (19,985 yuan RMB per case). With the increase of compliance, the cost-effectiveness of three-stage screening increased.</p><p><b>CONCLUSION</b>The cost-effectiveness of three-stage screening was influenced by screening compliance. It is recommended that three-stage screening strategy might be taken in area where the screening compliance rate is >90%.</p>

A Follow-up Analysis of 573 Cases of Hyperbilirubinemia after Treatment / 听力学及言语疾病杂志

Objective To share the analysis results of follow -up after treatment of hyperbilirubinemia hear-ing .Methods 573 cases of neonatal hyperbilirubinemia by jaundice intervention method was divided into exchange transfusion group (67 cases ,bilirubin concentration range of 344 .2~1 107 .2 μmol/L ,457 .9 μmol/L on average) and therapy group (506 cases ,bilirubin concentration in the range of 205 .2~ 572 .6 μmol/L ,average 285 .2 μmol/L) .The auditory brainstem response ,distortion product otoacoustic emission hearing detection and immittance method were used .The two groups of hearing loss were compared with 836 cases of normal controls .Results The observation group showed that hearing disorder morbidity rate was 3 .49% (20/573) ,for the control group ,the hearing loss morbidity rate was 0 .24% (2/836) .There was a statistically significant difference between the two groups (P<0 .05) .For the exchange transfusion group ,hearing losses accounted for 25 .37% of the overall change (17/67) .For the phototherapy group ,the hearing losses accounted for 0 .59% of the overall (3/506) phototherapy , showing statistically significant difference between the two groups (P<0 .05) .There were hearing impairment with bilateral severe (80% ,16/20) and retrocochlear lesions (65% ,13/20) .Conclusion The bilirubin concentration in blood transfusion for the children tended to have hearing impairment ,more in bilateral severe and retrocochlear le-sions ,hearing loss was irreversible .

Audiological Characteristics in 832 Deaf Children with Biallelic Causative Mutations in GJB2,SLC26A4 Gene / 听力学及言语疾病杂志

Objective To determine the audiological characteristics in 832 deaf children with biallelic causative mutations in GJB2 ,SLC26A4 gene .Methods The 832 patients received deafness gene screening ,553 were GJB2 gene biallelic causative mutations ,279 were SLC26A4 gene biallelic causative mutations .Patients were divided into four groups according to ages of hearing loss onset :<1 ,1~3 ,3~6 ,6~12 years old ,and the audiological character-istics and prevalence of GJB2 ,SLC26A4 gene mutations at different ages of onset .Results The prevalence of GJB2 gene mutations at four groups was 37 .97% (210/553) ,38 .34% (212/553) ,16 .27% (90/553) ,7 .41% (41/553) ,re-spectively ;the prevalence of SLC26A4 gene mutations at four groups was 25 .45% (71/279) ,44 .80% (125/279) , 20 .07% (56/279) ,9 .67% (27/279) ,respectively .The difference between GJB2 and SLC26A4 gene was significant(P=0 .001) .The prevalence of profound hearing loss with GJB2 gene mutations at four groups were 66 .67% (140/210) ,61 .32% (130/212) ,47 .78% (43/90) ,41 .46% (17/41) ,respectively .The difference was significant (P=0 .004) ,while the difference in 279 patients with SLC26A4 gene mutations was not statistically significant (P= 0 . 083) .Conclusion The age of hearing loss onset in patients with biallelic causative mutations in GJB 2 or SLC26A4 gene refers to 0~3 years -old ,hearing loss in patients with GJB2 ,SLC26A4 gene mutations gives priority to pro-found .The age of hearing loss onset is smaller ,the ratio of profound hearing loss is higher .Patients with severe and profound hearing impairment should be performed the genetic testing when the age of onset under 12 .

Analysis the relationship between the found ways and first diagnosis age for large vestibular aqueduct children / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To explore the found ways and first diagnosis age of children with large vestibular aqueduct, and their relations with hearing loss.@*METHOD@#Medical histories of 122 cases of children diagnosed with large vestibular aqueduct by HRCT or MRI had been collected from January 2009 to April 2014 in our hospital children's hearing diagnosis center clinic. Found ways comprise of accepting universal newborn hearing screening (UNHS) group and unaccepting UNHS group. Accepting UNHS children were divided into two ears unpassing group, single ear unpassing group and passing group. The patients in unaccepting UNHS group were divided into not sensitive to sounds, speech stunting, sudden hearing loss, and other group. Analysis the relationship between the found ways and first diagnosis age and their relations with hearing loss.@*RESULT@#There are 84 cases (68.85%) accepting UNHS, the average age of first diagnosis was (17.24 ± 17.08) months; 37 cases (31.15%) are not accepting UNHS. The average age of first diagnosis was (30.92 ± 18.21) months. The average first diagnosis age of accepting UNHS group was more earlier than the unaccepting UNHS group. The difference was statistically signif- icant (P 0.05). In unaccepting UNHS group ,the average first diagnosis age of the mild-to-moderate hearing loss group was later than the very severe hearing loss group (P < 0.01).@*CONCLUSION@#Most of large vestibular aqueduct children can be found and receive diagnosis early by UNHS. But part of these patients with late-onset or progressive hearing loss, especially these with mild-to-moderate hearing loss cannot be found early, which should arouse our attention.

Hearing loss associated with GJB2 gene mutation / 临床耳鼻咽喉头颈外科杂志

Deafness refers to different degrees of hearing loss (HL). The factors leading to HL are complex, among which heredity is a major one. Nonsyndromic hearing loss (NSHL) accounts for 80% of hereditary deafness. More than 140 genes have been regarded to be closely related to NSHL. The mutation of GJB2 (gap junction protein, beta 2) gene accounts for 80% of NSHL and more than 50% of children NSHL, playing the most important role in deafness genes. This paper reviewed the studies on the association between GJB2 gene mutation and HL to provide reference for genetic diagnosis and counseling.

Rapid genetic diagnosis of patients with non-syndromic sensorineural hearing loss / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To evaluate the feasibility of a rapid genetic diagnosis technique in molecular genetics study of hearing loss.@*METHOD@#One hundred and ten subjects including 54 with moderate to profound non-syndromic sensorineural hearing loss were enrolled in this study. All with informed consents. Their peripheral blood samples were taken and tested using Hearing Loss Array Kit developed by Capital Bio. Nine hot mutation sites from 4 high risk genes were tested each.@*RESULT@#The overall positive rate in this cohort was 50.9%, while the positive rate among 54 hearing loss cases was 59.3%. DNA sequence analysis showed 100% coincident with gene chip results.@*CONCLUSION@#This Hearing Loss Array Kit (gene chip) has a high positive detection ratio, its characteristics such as rapid high-flux sensitive and high specific make it possible and promising in future screening and diagnosis in patients with sensorineural hearing loss. Molecular genetic diagnosis also has a profound value in deafness prevention.

Clinical findings of a group of children with auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#In order to extend the knowledge of auditory neuropathy spectrum disorder (ANSD), the clinical data of a group of infants and young children with this disorder was reported.@*METHOD@#This retrospective study involved 84 cases (151 ears). The subjects aged from 2 months to 6 years old. Descriptive analysis was carried out on case history, audiological and imaging results and the outcome of cochlear implantation.@*RESULT@#There were a variety of case history, including 11 of hyperbilirubinemia, 2 of family history of deafness, 3 of hypoxia, 1 of prematurity, 1 of nuclei basales pathology, 2 of congenital atelencephalia,and 13 of cochlear nerve deficiency (CND). The audiological results varied too. Click ABR ranged from no response at the maximum output level to Wave V thresholds as low as 70 dB nHL. Behavioral thresholds were mild in 1, moderate in 2 and profound in 20. The inner ear MRI were normal in 16 cases, CND in 12 and CND accompanied by periventricular leukomalacia in 1. 5 cases were implanted in the affected ear. The results in 3 were comparable with that of cochlear loss, 1 were less effective than that of cochlear loss, 1 became none user of the device.@*CONCLUSION@#ANSD is a heterogeneous group with a wide variety on case history, audiological and imaging results and the outcome of cochlear implant. Therefore, comprehensive clinical examinations are necessary so that individualized intervention program can be made.

A Study of the Intervention of Children with Bilateral Severe Hearing Loss in Countryside of China / 听力学及言语疾病杂志

Objective To investigate the effects of the intervention,rehabilitation and speech development of children with severe hearing loss in some rural areas.Methods 61 children,including 35 males and 26 females,were diagnosed as severe hearing loss with ABR and 40 Hz-AERP from June 2004 to July 2008.All the children failed hearing screening or visited the hospital as outpatients.The ages ranged from 2 to 72 months with the average age of 17.59 months.During telephone follow-up,the questionnaire was used to gather the data regarding the usage of hearing aids,hearing and speech rehabilitation,speech development,and communication abilities.Results 33 (54.10%) children were fitted with hearing aids,and 2 (3.28%) received cochlear implants,while 26(42.62 %) neither had hearing aids nor cochlear implants.10 cases with hearing aids also had speech training,whereas 23 children with hearing aids did not receive the training.2 cases with cochlear implants and 2 cases with hearing aids were found to have good speech development and communication ability,while 31 cases with hearing aids had delayed speech development.26 cases without any devices had to rely on sign language for their commumication.Conclusion Children in rural area with severe hearing loss experience greater speech and communication difficulties because many of them have no access to intervention and speech training.The results suggest that it would be very important to increase public awareness and educate parents to have their children wear hearing aids and receive speech training.

Study on multi-area universal newborn hearing screening in countryside of China / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the feasibility of universal newborn hearing screening in countryside in order to provide reliable evidence in launching this program all over the countryside of China. Method:Subjects were 12 638 infants who were born in 9 counties from Jan 2004 to Dec 2005. TEOAE was used for the fast hearing screening. Infants were screened on the 2-7 days after the birth. The re-screening was conducted in 4-6 weeks if failed in the initial screening,and follow-up were provided continually if they also failed in the re-screening. Result; Ten thouand eight hundred and forty-five of 12 638(85. 8%) were screened including 9 963(91. 9%) normal newborns and 882(8. 1%) newborns with high-risk. Seven thouand four hundred and fifty (68. 7%) newborns passed the initial screening, and 3 395 (31. 3%) people failed. One thouand seven hundred and ninty-three (14. 2%) infants were refused to be screened.Only 2 536 (74. 7%) were re-screened on time, and 859(25. 3%) did not receive re-screening. One hundred and twenty were failed in the re-screening or first screening, and 79 (65. 8%)of them received diagnostic assessment. Among the infants received diagnostic assessment, 6(7.6%)ca-ses were found to have profound hearing loss in both ears, 9(11. 4%)cases were found to be severe hearing loss(7 in both ears and 2 in single ear) , 11(13. 9%)cases were found to be moderate hearing loss (5 in both ear and 6 in single ear), 26 (32. 9%) were found to have slight hearing loss (11 in both ear and 15 in single ears), and 27 (34.2%) were normal. Fifty-two infants were diagnosed as hearing loss with a prevalence of congenital hearing loss(in binaural and monaural) of 0. 5%(52/10845)and a prevalence of bilateral hearing loss of 0. 3%(29/10845). A prevalence of congenital hearing loss was 0. 2% (22/9 963) in well infants and 3. 4% (30/882) in high risk infants. Among the 13 cases of children with severe and profound hearing loss in both ears children, 8(61. 5%)cases were fitted with hearing aids and 1 (7. 7%) case was implanted with cochlear implants. Conclusion:It is necessary and feasible to conduct hearing screening program in the rural area. However, the suitable model to perform the program in the countryside needs to be set up as soon as possible in order to get more poor infants to participate into the hearing screening program for free and increase the screening rate.

Study on multi-area universal newborn hearing screening in countryside of China / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the feasibility of universal newborn hearing screening in countryside in order to provide reliable evidence in launching this program all over the countryside of China.@*METHOD@#Subjects were 12,638 infants who were born in 9 counties from Jan 2004 to Dec 2005. TEOAE was used for the fast hearing screening. Infants were screened on the 2-7 days after the birth. The re-screening was conducted in 4-6 weeks if failed in the initial screening, and follow-up were provided continually if they also failed in the re-screening.@*RESULT@#Ten thousand eight hundred and forty-five of 12,638 (85.8%) were screened including 9,963 (91.9%) normal newborns and 882 (8.1%) newborns with high-risk. Seven thousand four hundred and fifty (68.7%) newborns passed the initial screening, and 3,395 (31.3%) people failed. One thousand seven hundred and ninety-three (14.2%) infants were refused to be screened. Only 2,536 (74.7%) were re-screened on time, and 859 (25.3%) did not receive re-screening. One hundred and twenty were failed in the re-screening or first screening, and 79 (65.8%) of them received diagnostic assessment. Among the infants received diagnostic assessment, 6 (7.6%) cases were found to have profound hearing loss in both ears, 9 (11.4%) cases were found to be severe hearing loss (7 in both ears and 2 in single ear), 11 (13.9%) cases were found to be moderate hearing loss (5 in both ear and 6 in single ear), 26 (32.9%) were found to have slight hearing loss (11 in both ear and 15 in single ears), and 27 (34.2%) were normal. Fifty-two infants were diagnosed as hearing loss with a prevalence of congenital hearing loss (in binaural and monaural) of 0.5% (52/10845) and a prevalence of bilateral hearing loss of 0.3% (29/10845). A prevalence of congenital hearing loss was 0.2% (22/9,963) in well infants and 3.4% (30/882) in high risk infants. Among the 13 cases of children with severe and profound hearing loss in both ears children, 8 (61.5%) cases were fitted with hearing aids and 1 (7.7%) case was implanted with cochlear implants.@*CONCLUSION@#It is necessary and feasible to conduct hearing screening program in the rural area. However, the suitable model to perform the program in the countryside needs to be set up as soon as possible in order to get more poor infants to participate into the hearing screening program for free and increase the screening rate.