广西自治区听力残疾人抽样调查结果分析

目的根据广西自治区第二次全国残疾人抽样调查的资料,调查分析广西自治区听力残疾人的数量、地区分布、致残原因等。方法采用分层、多阶段、整群、概率比例抽样方法进行残疾人口调查。全自治区共抽查25个县（市、区）、100个乡（镇、街道）,共200个调查小区。每个调查小区常住人口约400人,调查总户数22569户,实际调查量为80712人,调查的抽样比1．73％。应用数字式便携听力计对每位调查对象进行听力检测,问卷调查病史,专科医师体检并作出病因诊断。结果5837名残疾人中,听力残疾1467人,占总残疾人数的25．13％,居所有残疾之首;其中一级听力残疾70人（4．77％,70／1467）;二级听力残疾127人（8．66％,127／1467）;三级听力残疾689人（46．97％,689／1467）;四级听力残疾581人（39．60％,581／1467）。主要致残原因：老年性聋911人（62．10％,911／1467）;原因不明155人（10．57％,155／1467）;耳疾158人（10．77％,158／1467）;全身性疾病111人（7．57％,111／1467）;其他132人（8．99％,132／1467）。男性821人（56％）,女性646人（44％）,性别比为1．27：1。农村居民910人（62％）,城市居民557人（38％）。年龄分布：0～岁3人（O．20％）,7～岁10人（0．68％）,18～岁215人（14．66％）,≥60岁1239人（84．46％）。结论听力残疾在广西所有残疾人口中发生率最高,以中老年性聋和耳疾占大多数。     

北京市居民听力残疾主要原因调查分析

目的 了解北京市居民听力残疾的主要致残原因.方法 2006年4月1日零时～5月底进行北京市第二次全国残疾人抽样调查.此次调查采用分层、多阶段、整群、概率比例抽样方法 对北京市18个区县1 541万常住人口进行抽样,共调查74 795人,其中男性36 753人,女性38 042人;城镇55 773人,农村19 022人.采用分类变量的统计描述方法 对调查数据进行致残原因分析.结果 北京市居民听力残疾(含多重残疾)的前五位致残原因是:老年性聋、原因不明、全身性疾病、噪声和爆震、中耳炎,其致残率分别为1.10%、0.30%、0.30%、0.20%和0.17%.0～14岁听力残疾(含多重残疾)人群的主要致残原因为遗传、原因不明和其他;15～59岁听力残疾(含多重残疾)人群的主要致残原因为原因不明、药物中毒、中耳炎;60岁及以上听力残疾(含多重残疾)人群的主要致残原因为老年性聋、全身性疾病、噪声和爆震及原因不明.男女性以及城乡听力残疾(含多重残疾)人群的首位致残原因均为老年性聋.结论 老年性聋、全身性疾病、噪声和爆震以及中耳炎是北京市居民听力残疾(含多重残疾)的主要致残原因.     

Some Experiences with Hearing Disability/ Handicap and Quality of Life Measures: Algunas experiencias con discapacidad/desventaja auditiva y medidas de calidad de vida

To achieve detailed information on the outcome of hearing rehabilitation in a clinical setting, measurements of hearing disability and hearing handicap have been introduced, and the present study describes the preliminary experiences with these measures in a series of hearing-impaired subjects referred for audiological evaluation. To outline hearing disability/hearing handicap (HD/HH), before continuation or initiation of treatment, the Gothenburg profile and a generic health-related quality of life—the SF-36 questionnaire–were used to evaluate any adverse effects resulting from hearing disorders. The Gothenburg profile and the SF-36 questionnaire were mailed to 634 subjects appointed for audiological examination with a request to complete the questionnaires at home. Three questions were enclosed concerning the capacity to complete the questionnaires, showing that 1.9 per cent were incapable of completing them, 17.5 per cent needed assistance, 43.5 per cent completed the questionnaires, and 37.1 per cent did not respond to them. Thus the response rate was only 55.5 per cent and, in addition, these responses were characterised by missing data. An arbitrary criterion of an average score per question of 3 for the averaged HD and HH items respectively was applied, resulting in 72.7 per cent indicating HD, whereas 30.3 per cent indicated HH according to the items in the profile. Significant differences in hearing levels were found between those experiencing HD and HH having a score 3 and those with a score <3. When analysing the general health status, general perception of health and social functioning, significantly lower scores were found for those experiencing HD/HH, whereas no significant differences were found between the total sample of hearing-impaired subjects and the age-matched population for these parameters. It is concluded that the present procedure cannot be implemented as a routine outcome measure in a clinical setting and alternative outcome measures should be considered.

Para obtener información detallada sobre los resultados de la rehabilitation en un contexto clínico, se introdujeron medidas clínicas sobre discapacidad y desventaja auditiva (hearing disability/handicap) y el presente estudio describe las experiencias preliminares con estas medidas, en una serie de sujetos con hipoacusia que fueron referidos para evaluación audiológica. Para subrayar la discapacidad/desventaja auditiva (HD/ HH), antes del inicio o continuatión del trata-miento y para evaluar cualquier efecto adverso resultante de los trastornos auditivos, se utilizaron el perfil Gothenburg y el cuestionario genérico de calidad de vida relacionada con la salud (SF-36). El perfil Gothenburg y el cuestionario SF-36 fueron enviados por correo a 634 sujetos citados para examen audiológico, con la solicitud de que llenaran los cuestionarios en casa. Se incluyeron tres preguntas relacionadas con la capacidad de completar los cuestionarios, mostrando que el 1.9 por ciento eran incapaces de completarlos, que el 17.5 por ciento necesitaron ayuda, que el 43.5 por ciento completaron el cuestionario y 37.1 por ciento no respondieron del todo. Por tanto, la tasa de respuesta fue sólo del 55 por ciento y, además, estas respuestas estuvieron caracterizadas por información incompleta. Se aplico un criterio arbitrario de 3 sobre el puntaje promedio por pregunta, para los items HD y HH promediados, resultando en un 52.3 por ciento que indieaba HD, mientras que un 30.3 por ciento indieo HH, de acuerdo los elementos en el perfil. Se encon-traron diferencias significativas en los niveles auditivos de aquellos sujetos que experimentaban HD y HH con puntaje de 3, en relation con aquellos que tenian puntaje de <3. Cuando se analizó el estado general de salud, la perception de la salud y el funcionamiento social, se encon-traron punlajes significativamente menores en aquellos que experimentaban HD/HH, mientras que no se encontraron diferencias significativas en la muestra total de sujetos hipoacúsicos y la población ordenada por edad pará estos para-metros. Se concluye que el presente procedimiento no puede ser implementado para medidas de resultados rutìnarios en un contexto clínico y que se deben considerar medidas alternativas de resultados.     

The Hearing Scale Test for hearing screening of school-age children

Objectives

This study aimed to demonstrate a new modified hearing screening method that can rapidly screen hearing and provide stratified test values for each screened ear of children.

Methods

The proposed Hearing Scale Test (HST) and pure-tone screening (PTS) were applied to 384 school-age children. PTS involved applying four test tones to each tested ear: 500 Hz at 25 dB, and 1000, 2000, 4000 Hz at 20 dB; and classifying the results as “pass” (normal hearing in the ear) or “fail” (possible hearing impairment). The HST employs ten stratified hearing scales from S₁ to S₁₀, with each hearing scale containing four test tones and where adjacent scales differ from each other by 5 dB, ranging from 0 dB (S₁) to 45 dB (S₁₀). The four test tones of hearing scale S₅ are the same hearing criteria and the median reference standard of the stimulus level in the HST. Scales S₁-S₅ on the HST are equivalent to a PTS “pass” result, while S₆-S₁₀ and no response (NR) are equivalent to a PTS “fail” result.

Results

The two screening methods produced comparable “pass” and “fail” results. In the HST, the “pass” results were further stratified as S₁ in 4 ears, S₂ in 52 ears, S₃ in 226 ears, S₄ in 272 ears, and S₅ in 169 ears, while the “fail” results were stratified as S₆ in 23 ears, S₇ in 12 ears, S₈ in 1 ear, S₉ in 2 ears, S₁₀ in 5 ears, and no response (NR) in 2 ears. The hearing screening results of the HST are interpreted as follows: scales S₁-S₅ indicate normal hearing, scales S₆ and S₇ indicate possible hearing impairment, and scales S₈-S₁₀ and NR indicate confirmed hearing impairment.

Conclusions

Conventional PTS only gives a pass/fail result for each screened ear, lacks hearing status assessment, and lacks stratified test values to be recorded for follow-up. In contrast, the HST has stratified hearing scales for each screened ear, which reflects the current hearing status and provides test values that can be recorded for follow-up.     

三个月婴儿的听性行为反应

目的 探索利用行为法对三个月龄婴儿进行听力筛查的可行性。方法 对323名北京市区78－104天的婴儿进行了听力检测，测试声为1kHz,90dB SPL和2kHz,90dB SPL两种纯音。给声时观察并用摄像机记录婴儿的听觉行为反应。结果 听性行为反应在1kHz,90dB和2kHz,90dB的引出率分别为：眼部反应70.6%和71.5%，头部反应39.0%和42.4%，肢体反应82.4%和87.9%，其他反应17.0%和17.3%。结论 公凭上述一种听性行为反应不足以作为听力筛查的依据。若同时观察肢体反应和眼部反应，并以其中之一的引出作为听性行为反应引出的阳性指征，则引出率可达到95％左右，可以满足听力筛查的要求。     

The effect of hearing impairment in older people on the spouse: Development and psychometric testing of The Significant Other Scale for Hearing Disability (SOS-HEAR)

The effects of hearing impairment on the person with the impairment and on their significant others are pervasive and affect the quality of life for all involved. The effect of hearing impairment on significant others is known as a third-party disability. This study aimed to develop and psychometrically test a scale to measure the third-party disability experienced by spouses of older people with hearing impairment. The Significant Other Scale for Hearing Disability (SOS-HEAR) was based on results of a previous qualitative study investigating the effect of hearing impairment on a spouse's everyday life. Psychometric testing with 100 spouses was conducted using item analysis, Cronbach's alpha, factor analysis, and test-retest reliability. Principal components analysis identified six key underlying factors. A combined set of 27 items was found to be reliable (alpha = 0.94), with weighted kappa for items ranging from fair to very good. The SOS-HEAR is a brief, easy to administer instrument that has evidence of reliability and validity. The SOS-HEAR could serve as a means of identifying spouses of older people with hearing impairment in need of intervention, directed towards either the couple or the spouse alone.     

贵州省0～14岁儿童听力障碍流行病学调查

目的 了解贵州省0～14岁儿童耳疾和听力障碍现患率、病因和干预需求的情况.方法 根据WHO方案,用容量比例概率抽样(PPS)方法调查贵州省30个点人群的听力和耳疾情况,抽取其中0～14岁儿童2 068人的资料进行统计分析.结果 0～14岁儿童听力减退现患率为3.48%,听力残疾现患率为1.84%,明显高于国内其他省市水平;7～14岁组听力残疾现患率为2.66%,显著高于0～6岁组(0.51%);男性儿童听力减退现患率为4.22%,显著高于女性儿童(2.54%).听力减退的可能原因分别是:耳疾50.00%(其中急慢性中耳炎占37.40%)、非感染因素11.11%、遗传性聋20.83%、病因不明27.77%.3.58%的调查对象需要得到药物或外科手术治疗;2.32%患儿需要佩戴助听器.结论 贵州省0～14岁儿童听力残疾现患率高于国内其他省市水平,中耳炎是引起儿章听力障碍的主要病因,且儿童遗传性聋比例也较高.     

Anxiety of the mothers with referred baby during Universal Newborn Hearing Screening

Objective

To investigate the anxiety among mothers whom their babies have failed test results in the first stage of Universal Neonatal Hearing Screening Program.

Patients and methods

A cross-sectional study was carried out on mothers whom their baby have positive test results in the first stage of Universal Neonatal Hearing Screening Program. Face to face interview was conducted to obtain data on sociodemographic profiles, knowledge about hearing loss and past medical history. Symptoms experienced by the mothers due to positive hearing test results and level of anxiety were measured by using the Malay translation Beck Anxiety Inventory questionnaire. These mothers were then given an appointment to come for the second screening six weeks after the first screening. The same questionnaire was given to them before the start of the second screening. SPSS version 11.5 was used for data entry and analysis. Wilcoxon signed Rank Test was used to compare the level of anxiety between the first and second screening.

Results

From a total of 78 mothers who were participated during the first screening, 50 of them have completed the study at the second screening (response rate = 64%). Fifty-two percent of them knew about the hearing screening before hand. Ninety-six percent of the mothers became alert about their child response towards sounds after they knew that their child had failed the first hearing screening. During the first screening, 74% of the mothers felt mild anxiety which was decreased to 68% before the mothers undergone the second screening. Moderate anxiety was felt by 10% of the mothers during both the first and second screening. There were 8% of the mothers having severe anxiety during the first screening but have reduced to half (4%) before the mothers undergone the second screening. The anxiety level was significantly less before the second screening with the median score of 5 (IQR: 13.0) compared to after the first screening (8, IQR = 14.25); p = 0.001.

Conclusions

There are considerable portion of the mothers of false-positive test result during Universal Neonatal Hearing Screening Program experienced unacceptable anxiety. This group of mothers needs to be identified and given a necessary help.     

基层医院开展新生儿听力筛查的可行性研究

目的探讨基层医院应用耳声发射进行新生儿听力筛查的可行性及影响因素。方法对2721名新生儿(包括141名听损伤高危儿)出院前进行耳声发射初筛,未通过者42天后复筛,仍未通过者,行听性脑干反应检查。结果自2001年11月至2005年3月,本院实际出生新生儿3150名,正常产2979名,重危监护儿171名,实际接受初筛2721名(其中141例为听损伤高危儿),346例未通过(45例为听损伤高危儿),初筛率为86.4%,初筛通过率87.3%。需复筛的346人,实际复筛306人(37例为听损伤高危儿),复筛率88.4%,276例通过复筛,复筛通过率90.2%。结论基层医院应用耳声发射进行新生儿听力筛查,可早期发现有听力损伤的新生儿。未通过耳声发射筛查者,应行听性脑干反应检查,并与上级医院建立网络联系,协助追踪、随访。     

耳别对新生儿听力筛查结果的影响

目的 探讨耳别对新生儿听力筛查结果 的影响.方法 2008年1月～2010年1月在秦皇岛市第一医院出生的1204例新生儿分别接受左耳和右耳听力筛查,对筛查的通过率进行统计分析.本研究排除早产、窒息、高胆红素血症等可能导致听力障碍的高危因素的新生儿.结果 共筛查1204例,右耳听力筛查的通过率高于左耳.结论 耳别对新生儿的听力筛查结果 有影响.     

北京城乡结合部新生儿普遍听力筛查分析

目的分析北京市城乡结合部开展新生儿普遍听力筛查（UNHS）的现状,为制定合适的听力筛查、随访和干预策略提供依据。方法应用瞬态诱发耳声发射对北京市上地医院出生的正常新生儿进行听力筛查,初筛未通过者分别于42天和/或2.5月复查,复筛未通过者使用听性脑干反应（ABR）、40Hz听觉相关电位（40Hz-AERP）和声导抗进行听力损失诊断。结果可接受听力筛查新生儿5809例,实际筛查5737例,初筛率98.76%（5737/5809）,初筛通过率81.38%（4669/5737）;应复筛1068例,实际复筛590例,复筛未通过104例,复筛未通过率17.63%（104/590）,应转诊104例（1.82%,104/5737）,实际转诊率7.1‰（41/5737）,实际接受诊断性检查28例,确诊先天性听力损失19例（28耳）,其中轻度10例（10/5737,1.74‰）、中度2例（2/5737,0.35‰）、重度1例（1/5737,0.17‰）、极重度6例（6/5737,1.05‰）。该筛查群体中先天性听力损失（包括单耳和双耳）的检出率为3.31‰（19/5737）,双耳听力损失的检出率为1.57‰（9/5737）。结论在城乡结合部实施新生儿普遍听力筛查是可行的,但复筛率及转诊率有待提高,因此制定适合流动人群特点的听力筛查模式是非常重要和必要的。     

Hearing,use of hearing protection,and attitudes towards noise among young American adults

The purpose of the present study was to investigate possible associations between college students’ attitudes, risk-taking behaviour related to noisy activities, and hearing problems such as threshold shifts or self-experienced hearing symptoms. The sample included 258 students aged between 17 and 21 enrolled at the University of Pennsylvania, USA. A questionnaire measuring attitudes towards noise, use of hearing protection, and self-reported hearing symptoms was distributed among the students. After completing the questionnaire a hearing screening, including pure-tone audiometry and tympanometry, was conducted. The result revealed that 26% had thresholds poorer than the screening level of 20 dBHL. Attitudes were significantly related to self-experienced hearing symptoms, but not to threshold shifts. Attitudes and noise sensitivity was, significantly related to use of hearing protection. Hearing protection use was found in activities such as using firearms, mowing lawns, and when using noisy tools but was less reported for concerts and discotheques. It can be concluded that the young adults in this study expose themselves to hearing risks, since the use of hearing protection is in general very low.     

Hearing aids reduce overestimation in pre-fitting self-assessment

Objective

When asking about hearing disability, the self-reported answers are not always equivalent to those of their family and neighbors. It is often experienced that family and neighbors indicate more severe hearing disability. Hearing difficulty itself may prevent hearing impaired subjects from understanding the degree of their own hearing disability. If their hearing impairment interferes with self-assessment of unaided hearing ability, it may change after a non-hearing aid user begins using hearing aids.

Methods

Thirty-four adults who had almost no experience with using hearing aids participated in this study. Unaided hearing disability was assessed with visual analog scale (VAS) and ten 5-point category scales. The assessment was performed not only by the subject (pre-fitting self-assessment) but also by his/her family members, presumed to be understanding persons regarding the degree of his/her hearing disability (family-assessment). For evaluating the effect of amplification on self-assessment, re-assessment was performed more than three months later (post-fitting self-assessment). The overestimation in the pre-fitting self-assessment and the effect of amplification were investigated in comparison to the family- and post-fitting self-assessments.

Results

The pre-fitting self-, family- and post-fitting self-VAS values for total hearing abilities were 50.5 ± 15.6, 45.1 ± 14.7 and 34.3 ± 18.8, respectively. The pre-fitting self-VAS value was significantly higher than the family- and post-fitting self-VAS values (p < 0.05 and p < 0.01, respectively). For the 5-point category scale, the pre-fitting self-assessment was better than the family- and post-fitting self-assessments. Significant differences were observed in 1 and 2 situations, as compared with the family- and post-fitting self-assessments, respectively. These findings suggest the involvement of overestimation in pre-fitting self-assessment and its reduction after amplification with hearing aids. Although the pre-fitting self- and the family-VAS values were independent of the results of audiometric tests, the post-fitting self-VAS value was significantly related to the pure tone threshold and maximum speech recognition score (p < 0.05). Regarding the changes in self-VAS values after amplification, the higher the pre-fitting self-VAS value, the larger the decrease in the self-VAS value. Thus, amplification with hearing aids may reduce overestimation and change self-assessment to reflect the audiometric assessments.

Conclusion

The pre-fitting self-assessment of hearing disability involves over estimation which is exacerbated by hearing difficulty. A high pre-fitting VAS value may reflect a large overestimation in hearing ability. This overestimation can be improved by the amplification with hearing aids.     

Auditory status of persons with intellectual disability at the German Special Olympic Games

Among persons with intellectual disability, the prevalence of hearing impairments is high. During the German Special Olympics Summer Games 2004, a hearing screening was conducted on 755 athletes with intellectual disabilities. Obligatory screening included ear inspection and recording of otoacoustic emissions, and optional screening included tympanometry and brief pure-tone audiometry. 38.0% of the athletes failed the screening. 53.0% needed ear wax removal. 56.1% of the fails indicated sensorineural hearing loss and 13.6% indicated mixed hearing loss. 12.5% of the fails were caused by unremovable ear wax, 1.4% by ear canal affections, and 16.4% by middle ear problems. Left ear fails were more frequent than right ear fails. A peripheral hearing disturbance can thus be expected in every third subject. The high failure rate, a considerable percentage of previously undetected profound hearing loss (1.1%), and the frequent need for ear wax removal, suggest that nearly half of persons with intellectual disabilities need regular otological or audiological consultations.

Sumario

La prevalencia de hipoacusia entre personas con discapacidad auditiva, es alta. Durante los juegos olímpicos de verano 2004 en Alemania, se realizó un tamiz auditivo en 755 atletas con discapacidad intelectual. Éste incluyó otoscopia, prueba de emisiones otoacústicas y opcionalmente una timpanometría y una audiometría breve. El 38% de los atletas no pasaron el tamiz y a 53% se les retiró cerumen. El 56.1% de los que no pasaron el tamiz presentaban hipoacusia sensorineural y 13% hipoacusia mixta; a 12.5% no fue posible retirarles el cerumen, 1.4% presentaban alteraciones del conducto auditivo externo y 16.4% tenían algún problema del oído medio. Fueron más frecuentes las alteraciones en el oído izquierdo que en el derecho. Se puede esperar una alteración auditiva periférica en un tercio de los sujetos. El alto índice de falla en el tamiz, un porcentaje considerable de hipoacusia profunda no detectada (1.1%) y la necesidad frecuente de remoción de cerumen, sugiere que casi la mitad de las personas con discapacidad intelectual necesitan consulta audiológica u otológica regular.     

母体乙肝病毒标志物阳性与新生儿听力复筛结果相关性分析

目的：探讨初筛未通过的新生儿42天听力复筛结果是否受母体乙肝病毒感染的影响。方法采用畸变产物耳声发射（DPOAE）测试,对初筛未通过且乙肝病毒标志物阳性产妇所产新生儿进行42天听力复筛。结果本研究331例新生儿中,听力复筛通过289例,复筛通过率87.31%。母血HBeAg阳性组的脐血HBsAg与HBeAg阳性新生儿显著高于母血HBeAg阴性组（P＜0.01）;同时母血HBeAg阳性组以及母血HBV DNA阳性组的复筛通过率低于阴性组（P＜0.05）。结论妊娠母血HBeAg阳性和母血HBV DNA阳性与新生儿宫内感染以及听力复筛不通过存在一定联系,今后工作中应密切关注和定期随访。     

新生儿听力损失在妇幼保健院的监测

目的 早期发现新生儿听力损失，以便及早干预，促进婴幼儿正常的语言发音。方法 应用瞬态诱发耳声发射技术对2000年5月18日至2001年12月30日济南市妇幼保健院住院分娩的新生儿进行听力普遍筛查，于生后3月左右采用听觉诱发电位技术(ABR和40Hz。AERP)进行诊断。结果 8788例住院分娩新生儿中共筛查8262例(94．0％)，其中8150例(92．7％)新生儿住院期间进行了筛查，初筛通过6920例(84．9％)。1230例未通过初筛，返回在门诊进行复筛的为1108例(1108／1230，90．08％)，复筛未通过49例。确诊41例听力损失，另有1例为迟发性听力损失，共发现新生儿听力损失42例。本组资料显示新生儿听力损失的发病率为5．08‰。双侧先天性听力损失的发病率为2．42‰。因双侧听力损失需要佩戴助听器的为1．3‰。初筛的假阳性率为14．54％，两步筛查后的假阳性率为0．01％，整个筛查程序的灵敏度为95．34％。结论 我国济南市局部城区新生儿先天性听力损失的发病率为5．08‰，与发达国家相比是高的；通过筛查，新生儿听力损失可及早发现，并进行及早干预，有效的促进婴幼儿语言发育。     

中国内地新生儿听力筛查情况的回顾性分析

目的回顾性分析中国内地新生儿听力筛查工作开展现状及相关统计指标,探讨存在的主要问题并提出前瞻性建议。方法通过PUBMED、CBMdisc数据库及互联网检索1998～2008年间发表的有关中国内地新生儿听力筛查文献,设定标准筛选文献,使用SPSS13.0软件对相关数据行统计分析,并对结果进行系统评价。结果共纳入符合标准文献129篇,合计筛查新生儿705 394例。明确对象为普通病房新生儿的耳聋合并发病率为0.20%(880/447 604),新生儿重症监护病房(NICU)新生儿的耳聋合并发病率为2.29%(79/3 446);新生儿聋病发病率男女间无差别;出生后72小时初筛通过率高于出生后48小时(P<0.05);存在高危因素新生儿的耳聋发病率高于正常儿(P<0.05);高危因素组间比较显示存在耳聋家族史及颌面畸形的新生儿耳聋发病率最高(P<0.05)。结论新生儿出生后72小时为初筛最佳时间;有耳聋家族史及颌面畸形新生儿耳聋发病率最高。目前国内新生儿听力筛查工作需要统一筛查手段、方法和程序,加强随诊。     

TEOAE听力初筛通过与未通过新生儿1000Hz鼓室导抗图型比较

目的分析未通过瞬态诱发性耳声发射（TEOAE）听力初筛新生儿的1000 Hz声导抗特点。方法采用GSI-70型自动耳声发射听力筛查仪对新疆自治区人民医院及乌鲁木齐市妇幼保健院出生的新生儿进行听力初筛,同时采用GSI TympStar Version Ⅱ中耳分析仪进行中耳功能测试,收集1000 Hz鼓室导抗图及相关指标,包括鼓室图峰压（the pressure of the peak admittance ,Tpp）、峰声导纳值（peak admittance ,Yp）,峰补偿静态声导纳值（Peak compensated static admittance ,Ypc）,鼓室导抗图宽度（tympanometric width ,TW）。对听力初筛未通过的47耳（未通过组）和通过的1300耳（通过组）的鼓室导抗图分型及单峰型声导纳图各指标进行比较。结果1000 Hz鼓室导抗图分型中,未通过组单峰型声导纳图比例（59．57％）显著低于通过组（74．00％）,而未通过组平坦型声导纳图比例（23．40％）显著高于通过组（8．54％）;通过组单峰型、双峰型声导纳图比例明显高于未通过组,平坦型声导纳图的比例低于未通过组（均为 P＜0．05）。两组间单峰型声导纳图的各指标比较差异无统计学意义。结论 TEOAE听力初筛通过的新生儿1000 Hz声导纳图以单峰型为主,而未通过的新生儿单峰型所占比例较低,平坦型比例高。     

河南洛阳地区新生儿听力与耳聋基因同步筛查临床推广工作现状分析

目的分析河南洛阳地区新生儿听力与耳聋基因同步筛查临床推广工作现状,为同步筛查工作的普遍开展提供现实依据。方法对2016年9月~2018年9月间出生的来自洛阳市区及周边县区27900例新生儿采用筛查型耳声发射和或自动听性脑干诱发电位仪进行听力学初筛和复筛;对其中的9068例新生儿用微阵列芯片法进行我国人群常见的4个耳聋责任基因的9个热点变异进行同步基因筛查。对未通过听力复筛新生儿及基因筛查阳性患儿进行电话随访,指导其在3月龄内进行听力学及遗传学诊断与干预。用SPSS21.0统计软件对数据进行统计学分析。结果27900例新生儿中26537例接受了听力筛查,初筛率为95.11%(26537/27900),初筛阳性率为5.58%(1481/26537),最终确诊听障患儿43例,听力障碍患儿检出率为0.15%(43/27900)。9068名新生儿同期接受了耳聋基因筛查,筛查率为32.50%(9068/27900),基因筛查总体阳性携带率为5.00%(453/9068);其中GJB2基因变异229例,阳性携带率为2.53%(229/9068),最常见变异位点为c.235delC杂合变异;SLC26A4基因变异133例,阳性携带率为1.47%(133/9068),最常见变异位点为IVS7-2A>G杂合变异;GJB3538C>T杂合变异45例,阳性携带率为0.50%(45/9068);线粒体DNA 12S r RNA基因变异21例,阳性携带率为0.23%(21/9068),最常见变异类型为m.1555A>G异质性变异;单基因复合杂合变异6例,阳性携带率为0.07%(6/9068)。。通过同步筛查流程最终确诊患儿14例,听力障碍检出率为0.15%(14/9068)。结论洛阳地区耳聋基因最常见的变异类型是GJB2235delC杂合变异及SLC26A4 IVS7-2A>G杂合变异;耳聋基因筛查是听力筛查的有力补充,特别是对药物敏感性及迟发性耳聋患儿起到早期发现与预警作用;但本地区同步筛查后随访及遗传咨询工作落后,导致筛而不查现象普遍存在,是今后工作改进的重点。