目的：探讨新生儿耳聋基因与听力联合筛查的意义。方法对2014年6～12月出生的546例新生儿，采集足跟血进行基因测序，对常见的4个耳聋基因的20个位点进行筛查；包括 GJB2（35delG、167delT、176＿191de116、235delC、299＿300delAT），GJB3（538C→ T、547G→A），SLC26A4（281C→ T、589G→ A、IVS7－2A→ G、1174A→T、1226G→ A、1229C → T、IVS15＋5G → A、1975G → C、2027T → A、2162C → T、2168A → G ），线粒体DNA12S rRNA（1494C→T、1555A→G），同时利用 TEOAE和AABR进行联合听力筛查。结果546例中，22例（4．03％，22／546）有耳聋基因突变，包括：GJB2基因突变14例，占2．56％（14／546）；SLC26A4基因突变7例，占1．28％（7／546）；线粒体DNA12SrRNA基因突变1例，占0．18％（1／546）。听力初筛未通过25例（4．58％，25／546），25例均进行复筛，复筛未通过12例，确诊9例听力损失，其中双耳极重度听力损失1例，双耳重度听力损失2例，双耳中度听力损失2例，左耳中度听力损失4例，这9例中8例耳聋基因筛查异常。结论单纯进行听力筛查或者单纯进行耳聋基因筛查可能会漏掉部分耳聋患儿，新生儿耳聋基因联合听力筛查，有利于耳聋儿童的早期发现与早期干预。

Objective To study the normal ranges and features for click-auditory brainstem responses of children in 3~5 years old.Methods Normal results of click-ABR from 132 normal children (224 ears)were ana-lyzed.The children were divided into three groups:Group A (74 ears from children of 3 years old),Group B (78 ears from children of 4 years old)and Group C (72 ears from children of 5 years old).The ABR data were statisti-cally analyzed by ANOVA with SPSS.Results At 80 dB nHL,the wave Ⅰ,Ⅲ,and Ⅴ latencies and intervals of wave Ⅰ- Ⅲ,and wave Ⅰ-Ⅴ of ABR for 3 -year old children were 1.24 ±0.09 ms,3.53 ±0.16 ms,5.39 ± 0.23 ms,2.30±0.15ms and 4.15 ±0.22 ms,respectively,and for 4 -year-old children,they were1.23 ±0.10 ms,3.52±0.39 ms,5.30±0.21 ms,2.28±0.39 ms and 4.07 ±0.22 ms,respectively.For 5 - year-old chil-dren,they were1.24±0.10 ms,3.67±0.63 ms and 5.34±0.19 ms,2.42±0.63 ms and 4.09±0.19 ms,respec-tively.The thresholds for 3-year children were 22.57 ±4.40 dB nHL,for 4 - year children,21.15 ±4.83 dB nHL,and for 5 - year children,21.11±3.48 dB nHL.Among three groups,only latency of wave V and interval of wave I-V had statistical differences,and a further analysis showed the statistical differences in Groups A and B. No statistical differences were observed in Groups B and C.The latency of wave I and thresholds of ABR for the children of different ages were not significantly different.Conclusion The resvits of this study suggest the brainstem may mature at 4 years old.

To review the change and development of the model of hearing and deafness gene screening of the newborn in Hubei region in China. In general,it can be divided into three stages:exploring and building of newborn hearing screening model;combination of gene and hearing screening in Newborn;development and research of the hearing diagnosis technology in Newborn. As the newborn hearing screening has been deepened and reformed,the screening technology and content become enriched and improved. The strategy to add the gene screening into the newborn hearing screening can be helpful to find out the late-onset hearing loss and the high risk subjects,which is significant to discover and diagnose the deafness early.

由国际诱发反应测听协作组（International E‐voked Response Audiometry Study Group ， IERASG）主办、韩国听力学协会和韩国耳鼻咽喉－头颈外科协会承办的IERASG组织第24届学术年会于2015年5月10～14日在韩国釜山天堂酒店举行。本次IERASG釜山年会，汇聚了来自世界各地的专家学者五百多人，其中包括国际听力学协会（International Society of Audiology ，ISA）执委会主席Linda Hood教授（美国）、IERASG主席John D 。 Durrant教授（美国）、国际耳内科医师协会（IA PA ）主席Jose Barajas教授（西班牙）以及IERASG执委会委员24名，分别来自世界五大洲，主要委员来自美国和欧洲国家，还有来自南美的古巴和巴西、亚洲的韩国和日本、俄罗斯、澳大利亚等代表其他几大洲的委员；本次IERASG釜山年会也首次迎来了来自中国的六人参会代表团。

目的 评估舌下免疫治疗(sublingual immunotherapy,SLIT)儿童组(4～14岁)和成人组(≥14岁)中-重度变应性鼻炎(allergic rhinitis,AR)的疗效差异.方法 收集坚持用药并定期随访的189例中-重度变应性鼻炎患者,其中儿童组患者103例,成人组患者86例.随访并记录在治疗前、治疗半年、治疗1年患者的症状评分及用药情况.结果 与治疗前相比,成人组及儿童组患者,在治疗半年和1年后症状评分和用药积分均明显降低,差异具有统计学意义(P＜0.01).治疗半年后,儿童组的症状评分及用药积分改善程度明显优于成人组,差异具有统计学意义(P＜0.05)；但治疗1年后,两者的改善程度差异无统计学意义(P＞0.05).结论 SLIT治疗儿童及成人中-重度变应性鼻炎均能取得较好疗效,但治疗早期儿童疗效优于成人.

Objective To explore the prevalence of hearing loss in the infants with congenital malformation in the neonatal surgical ward and to determine whether the congenital abnormality are the risk factors of the hearing loss and which screening mode is suitable to these infants.Methods The study group comprised 574infants with congenital malformation beyond risk factors of hearing loss published by Chinese Ministry of Health in neonatal surgical ward in Wuhan Women and Children Medical Care Center from January 2008to December 2009.The control group included 6 698healthy neonates born in the obstetrical department in the same hospital and in the same period.Before discharged,infants in the study group were employed with AABR and TEOAE for hearing screening.The infants referred to hearing screening would have a hearing test set including high frequency(1 000Hz)tympanometry(HFT),ABR and DPOAE when they were 3months old.The infants in the control group were used TEOA for the first hearing screening.The infants referred to screening were rescreening when they were one month to 42 days old.The same hearing test set were performed in infants who still referred to rescreening.The prevalence of hearing loss in the two groups were compared.Results The infants who failed screening in study group with TEOAE was 7.3%(42/574)and those with AABR was 5.9%(34/574).Fourtyfive infants were performed hearing test set and two of them were diagnosed with hearing loss.The prevalence of hearing loss was 3.48‰(2/574).There was 2.28%(153/6 698)of infants in the control group failed in first screening and 1.57%(105/6 698)of infants failed in rescreening.And 105infants were performed hearing test set and 15of them were diagnosed with hearing loss.The prevalence of hearing loss was 2.24‰.There was no significantly statistical difference in the prevalence of hearing loss between the two groups.The failure rate of TEOAE in the study group was statistically higher than that of in control group.Conclusion The congenital malformation in this study was not a risk factor of hearing loss.The infants with the malformation should be performed the same hearing screening mode as normal neonates do,but the failure rate may be higher,thas a follow-up is necessary.

Objective To study the characteristic and the clinical application of auditory brainstem response(ABR) in infants with middle ear dysfunction. Methods 85 infants(170 ears,group A) with middle ear dysfunction,83 infants(166 ears, group B) of sensorineural deafness with middle ear normal function and 41 normal infants(82 ears,group C) were studied.The peak latencies of Ⅰ,Ⅲ,Ⅴwaves and interval peak latencies of Ⅰ-Ⅲ,Ⅲ-Ⅴ,Ⅰ-Ⅴwaves and the response threshold of ABR were compared in every type. Results In group A,24 ears had normal response threshold of ABR(≤30 dB nHL),response threshold of 73 ears were 40 dB nHL,54 ears were 50 dB nHL,19 ears were 60 dB nHL.In group B,ABR threshold of 83 ears(50%) were 40 dB nHL,52 ears(31.33%) were 50 dB nHL,31 ears(18.67%) were 60 dB nHL.While in group C,ABR threshold in all ears were normal(≤30 dB nHL).At 80 dB nHL,the waves Ⅰ,Ⅲ,Ⅴ latencies of ABR in three groups were compared.In group A,the peak latencies of waves Ⅰ,Ⅲ,Ⅴ were more delayed than those in the other two groups(P<0.01),but the interval peak latencies of Ⅰ-Ⅲ,Ⅰ-Ⅴwaves were shorter than the others(P<0.01).The interval peak latencies of Ⅲ-Ⅴwave were not significantly different in three groups(P>0.05).The peak latencies and the interval peak latencies were not significantly different between the group B and group C(P>0.05).At equal response threshold of ABR,the peak latencies of waves Ⅰ,Ⅲ,Ⅴ were more delayed than the control groups(P<0.01). Conclusion The increased response threshold of ABR were more noticeable for those infants with middle ear dysfunction,and the peak latency of waves Ⅰ was prolonged.The prolonged wave Ⅰ latency of ABR demonstrated clinical values to assess the middle ear function.

Objective To evaluate the onset time of dermatophagoides farinae drops with symptomatic drugs for the treatment of allergic rhinitis.Methods Three hundred patients with allergic rhinitis caused by Dermatophagoides farinae were divided into 4 to 13 years old,14 to 28 years old,29 to 40 years old and more than 40 years old and enrolled in this self-contrast study.Clinical efficacy was evaluated by symptom scores after sublingual immunotherapy and the scores were compared with pre-treatment scores.The changes of VAS scores in the course of treatment were observed and the influence of age on the onset time was analyzed.Results Two hundred and Fourty four cases in 300 cases of patients were with onset time of 1-7 weeks,with an average of 3.09 weeks,and the total effective rate was 81.3%.The symptom scores before treatment and after taking effects were 8.15±1.87 and 5.77±3.4.The symptom scores decreased dramatically(P<0.01).VAS scores of different groups of ages before treatment were 8.4±1.68,8.3±0.86,8.35±0.69,and 8.32±0.83.The changes of VAS scores in the course of treatment had significant differences(P<0.01).The age was younger the onset time was sooner.Conclusion The dermatophagoides farinae drops with symptomatic drugs cans rapidly and quickly treat allergic rhinitis,the average onset time was 3.09 weeks and the age is younger,the onset time is sooner.

OBJECTIVE:The goal was to know more about the characteristic of auditory neuropathy spectrum disorder (ANSD) for high risk infants.METHOD:The newborn hearing screening was performed with automatic auditory brainstem response (AABR) and transient evoked otoacoustic emission(TEOAE) for the infants in the NICUs from August 2007 to January 2011. After subsequent rescreening, children with AABR test referred were perform hearing test set including high frequency (1000 Hz) tympanometry, ABR, DPOAE and/or Cochlear Microphonics (CMs) in 3 months old. Only infants demonstrated severely abnormal ABRs along with preserved DPOAEs and/or CMs were scheduled for re-examination in 6-8 months old and Behavior audiometry in 8-12 months old.RESULT:Eighteen infants (14 cases were bilateral and 4 cases were unilateral) considered as suffering from AN in 3 months old. All of them showed ABR thresholds > or = 80 dB nHL or absent at maximum test intensity. Follow-up examination revealed 9 cases (18 ears) with restoration of ABR to normal or a lower ABR thresholds and a resolution of ANSI) in 11 out of 18 infants retested in 6-8 months old. CMs were present in all ears but DPOAE were not present in 4 ears with middle ear pathology in 3 months test. Behavioral hearing of 10 cases ranged from mild (n = 2), moderate(n = 4) to severe and profound loss (n = 4).CONCLUSION:ANSD in high risk neonates could show the temporary character. It was too difficult to forecast the prognosis, they would be to follow up to at least 3 years old for newborn ANSD.

Objective To study the hearing developmental stages of newborns and infant of 1~36 months in terms of normal values and developmental characteristics. Methods 239 normal hearing newborns and infants were 1~36 months with 359 males and 216 females ears.The criteria for normal hearing thresholds were defined as ABR ≤30 dB nHL.The ABR components consisted of wave latencies and interpeak latencies.ASSR includes thresholds of 0.5 kHz,1 kHz,2 kHz and 4 kHz.The cluster analysis was employed to studying ABR and ASSR responses as a function of age of 1 month interval.t test was used to analyzing genders and ears,respectively in the group. Results Cluster analysis results suggested three stages: 1~4 months,5~24 months and 25~36 months.Stage 1: ABR threshold was 25.62±4.99 nHL,ASSR 500 Hz was 44.43±11.92 nHL,1 000 Hz 37.53±9.92 nHL,2 000 Hz 30.67±13.38 nHL,4 000 Hz 33.71±15.77 nHL.Stage 2: threshold of ABR was 22.92±4.52 nHL,ASSR 500 Hz 44.57±11.68 nHL,1 000 Hz 38.02±10.73 nHL,2 000 Hz 27.65±9.70 nHL,4 000 Hz 29.34±12.45 nHL.Stage 3: threshold of ABR was 23.90±4.79 nHL,ASSR 500 Hz 45.56±12.92 nHL,1 000 Hz 39.63±12.53 nHL,2 000 Hz 29.42±10.15 nHL,4 000 Hz 31.11±13.48 nHL.There was no statistical difference between left and right ears in every stage.And there were statistical differences on latencies of Wave I,III,and interpeak latencies I-III and I-V between male and female in every stage and were smaller in females.ANOVA results indicate that the indices of above decrease linearly with age.Thresholds of ABR and ASSR only decreased between stage 1 and two,the difference had statistical significance. Conclusion 5 months and 25 months are the landmark month in hearing development in 1~36 months.Neural transmission to the cochlea was well developed after birth but neural transmission to the central auditory pathway has not matured at three years old.

目的了解儿童阻塞性睡眠呼吸暂停低通气综合征(OSAHS)对ASSR检测的不良影响。方法对59例OSAHS儿童和45例正常儿童行ASSR检测,比较两组结果。结果 OSAHS组患儿ASSR检查的时间(33.32±4.25分钟)比对照组(17.58±2.18分钟)明显延长(P<0.05),0.5、1、2、4kHz ASSR反应阈明显高于对照组(P<0.05)。结论 OSAHS患儿的ASSR各频率反应阈均比正常儿童偏高,检测时间较正常儿童延长。

目的探讨儿童阻塞性睡眠呼吸暂停低通气综合征(OSAHS)缺氧程度对中耳功能的影响。方法对144例(288耳)OSAHS患儿行多导睡眠监测(PSG)及声导抗检查,分析不同程度最低血氧饱和度(LSaO2)和呼吸暂停低通气指数(AHI)的OSAHS患者声导抗异常率。结果 144例(288耳)OSAHS患儿中,74例轻度LSaO2患者中声导抗异常21例(28.38%),28例中度LSaO2患者中声导抗异常11例(39.29%),42例重度LSaO2患者中声导抗异常16例(38.10%),不同程度LSaO2患者的声导抗异常率差异无统计学意义(P>0.05);60例轻度AHI的患者中声导抗异常21例(35.0%),50例中度AHI的患者中声导抗异常14例(28.0%),34例重度AHI的患者中声导抗异常13例(38.24%),不同程度AHI患儿的声导抗异常率差异无统计学意义(P>0.05)。结论本组不同缺氧程度的OSAHS患儿的中耳功能无显著差异。

OBJECTIVE:To explore the best methods and skill for the removal of difficult and high risk tracheobronchial foreign body under bronchoscope.METHODS:A retrospective review was performed between August 1995 to August 2012. There were 4217 children with tracheobronchial foreign body, among them, 272 were diagnosed as high-risk, highly difficult tracheobronchial foreign bodies confirmed by clinical manifestations, foreign body type and bronchoscopy.RESULTS:In 271 children, the tracheobronchial foreign body was removed under bronchoscope, the success rate was 99.6%; only one child with a pen cap blocking the left lower lobe bronchus was transferred to the department of thoracic surgery, and the foreign body was finally removed by thoracotomy. Eighty-five children (among them, 82 children were under 1 year of age) had II-II degree laryngeal obstruction, the emergency surgery was performed to remove the foreign body and to relieve the laryngeal obstruction. Twenty-six children had lung infection and 27 children had failed foreign body removal surgery before, in all these children, the foreign body was removed after infection control. There were 17 children with the pen cap as the tracheobronchial foreign body, direct removal was successful in 12 children with the history less than two weeks; in 4 children, the foreign body was removed after 0.1% epinephrine saline flush, and 1 case with the homemade bronchial foreign body hook remove. There were 26 children with the whistle as the foreign body, and 32 children had large and sharp foreign bodies. In these cases, the foreign bodies were removed together with the bronchoscope. Forty-two children had multiple or fragile foreign bodies, and 16 children had subsegmental bronchial foreign bodies. In these cases, the foreign bodies were removed with forceps under direct vision and intraoperative bronchial lavage.In This series, 129 children received intraoperative bronchial lavage, among them, 127 children showed normal X-ray changes one week after operation. Two children with a history of more than 1 month complicated with pulmonary consolidation. After bronchial lavage, pneumothorax and subcutaneous emphysema occurred, which recovered after treatment. No glottic edema, asphyxia, and other complications were found, the complication rate of surgery was 0.7%.CONCLUSION:For the removal of highly difficult and high risk tracheobronchial foreign bodies, preoperative analysis and discussion should be sufficient, appropriate surgical skill and surgical instruments may improve the success rate of the surgery and prevent the operation complications.

目的 探讨气、骨导ABR测试在儿童分泌性中耳炎诊疗中的应用价值.方法 回顾性分析151例(246耳)分泌性中耳炎(OME组)和60例(120耳)正常儿童(正常对照组)气、骨导ABR检测结果.结果 ①OME组气导ABR波V反应阈正常59耳(23.98%),轻度异常96耳(39.02%),中度异常91耳(36.99%);ABR波Ⅰ、Ⅲ、Ⅴ潜伏期正常22耳(8.94%),各波潜伏期延长224耳(91.06%),Ⅰ-Ⅴ波间期无明显改变156耳(63.41%),Ⅰ-Ⅴ波间期缩短90耳(36.59%);波Ⅴ反应阈正常和轻度异常组Ⅰ- Ⅴ波间期无明显改变,中度异常组Ⅰ-Ⅴ波间期缩短,差异有统计学意义(P<0.05).②OME组骨导ABR波V反应阈及35 dB nHL刺激强度下各波潜伏期与对照组比较,反应阈正常195耳(79.27%),异常51耳(20.73%),反应阈异常者波Ⅰ、Ⅲ、Ⅴ潜伏期也较正常组及反应阈正常者明显延长(P<0.05).结论 大多数分泌性中耳炎儿童的气导ABR反应阈轻、中度异常但骨导ABR反应阈正常,少数患儿气导ABR反应阈中度异常且骨导ABR反应阈轻度异常.

Objective To study proper uses of 226 Hz and 1 000 Hz tympanograms in infants of 3～7 months old.Methods 226 Hz and 1 000 Hz tympanograms were tested with Oto100 Flex in infants of 3～7 months old(291 cases,529 ears),then ABR were examined.Results of 226 Hz and 1 000 Hz tympanograms were compared with those of ABR Wave I latencies using kappa consistency analysis.Results Kappa values between ABR Wave I latencies and 1 000 Hz tympanogram of 3 months old,4 months old and 5 months old were 0.732,0.78 and 0.718,respectively.And kappa values between ABR Wave I latencies and 226 Hz tympanogram of 3 months old,4 months old and 5 months old were 0.257,0.611 and 0.501,respectively.While values of 1 000 Hz of 6 months old and 7 months old were 0.608 and 0.583,and values of 226 Hz of 6 months old and 7 months old were 0.616 and 0.441.Conclusion Compared with ABR Wave I latencies,1 000 Hz tympanometry is more suitable for infant younger than 5 months(including 5 months old).226 Hz and 1 000 Hz tympanometry are not recommended for infants of 6 months old and 7 months old.

Objective To discuss the safety of sublingual immunotherapy(SLIT)to the patients with allergic rhinitis and/or allergic asthma.Methods One hundred and eighty-two patients with allergic rhinitis and/or allergic asthma were treated with routine medical and Dermatophagoides farinae drops.All the adverse reactions and severity of those were analyzed.Results None serious adverse reaction was reported in this study.8.24% of the patients appeared mild-to-moderate adverse reactions.In addition,incidence of adverse reaction in patients under 14 years old were higher than that in patients over 14 years old.Conclusion Only mild or moderate adverse reactions were reported in patients treated with SLIT.This study appears to provide further evidence that SLIT is a high-safety administration route of immunotherapy.

Objective To study the incidence and lesion site of hearing loss and the correlation between the peak of serum bilirubin levels and hearing thresholds in severe neonatal hyerbilirubinemia.Methods The prospective study was carried out in the neonatal intensive care unit of Wuhan Children's Hospital from January in 2008 to April in 2009.100 neonates with severe hyperbilirubinemia as the only risk factor for hearing loss was determined and 30 normal neonates were recruited.Hearing tests included tympanometry,ABR and DPOAE and followed up at 3 months old and 6 months old.The peak of serum bilirubin levels and corresponding ABR thresholds were analyzed statistically.Results 15 neonates with 22 ears showed abnormal ABR or with no responses.Among them,6 neonates with 9 ears did not pass DPOAE,9 neonates with 13 ears passed DPOAE.3 neonates showed improved hearing thresholds for ABR at 3 months follow-up,1 neonate had poorer hearing thresholds than before.There were no changes in auditory function at 6 months.There was positive correlation between the peak of serum bilirubin levels and ABR threshold.Conclusion The results indicate that hyperbilirubinemia may lead to sensorneural deafness and the auditory disorders as neuropathy.Hearing loss may be undulatory.Some of them were recovery.It suggests that monitoring auditory development is critical.

Objective To study the causes of Otitis Media with effusion(OME)in Children.Methods A retrospective analysis was employed to review 357 cases of OME(698 ears).Results In 357 cases,133 cases(37.3%) had adenoid hypertrophy,89 cases(24.9%) had sinusitis,and 76 cases(21.3%) had nasal allergy,59 cases(16.5%) bacteria infection,viral infection,mycoplasma infection and/or choamydiae infection.Conclusion Clinicians should pay attention to adenoid hypertrophy,sinusitis,nasal allergy,bacteria infection,viral infection,and mycoplasma and choamydiae infection in Children with OME.It's helpful to increase cure rate and decrease recurrent rate of OME in children.

目的探讨皮肤点刺试验在变应性鼻炎患者诊疗中的意义。方法选择前臂屈侧,采用变应原皮试液作皮肤点刺试验。结果 586例变应性鼻炎患者中变应原尘螨的阳性率最高,占50.68%,其次是蟑螂,占7.7%,其他变应原阳性率较低。结论变应原皮肤点刺实验可列为常规检查,作为变应性鼻炎诊断和治疗的参考。

Objective To investigate the application of 1 000 Hz and 226 Hz tympanometry in infants of 36 months younger.Methods The tympanograms were recorded from 648 infants with a MADSEN OTOFLE100 Middle-Ear analyzer.The infants were divided into four groups:Group 1 (0~3 months),Group 2 (3~6 months),Group 3 (6~9 months),and Group4 (9~36 months ).The data for each group were statistically analyzed.Results Using 226 Hz tympanometry ,66.82%(147/220) were abnormal in Group 1,33.49%(33/218) in Group 2,34.76%(73/210) in Group 3 and 1.85%(12/648) in Group 4.Using 1 000 ympanometry,4.09%(9/220) were abnormal in Group 1,11.01%(24/218) in Group 2,32.86%(69/210) in Group 3 and 57.10%(370/648) in Group 4.Conclusion In 0~6 month's infants,1 000 Hz tympanometry was an sensitive diagnostic test to the middle ear dysfunction,while 226 Hz tympanometry failed to be so.The combination of 1 000 Hz and 226 Hz tympanometry will be suitable in the diagnosis of the middle ear function in 6~9 month infants and 226 Hz tympanometry can objectively assess middle ear function in 9~36 month infants.