简介：【简要病史】患者袁XX,女性,10岁,汉族,山东青岛人,小学生,以＂颈部肿痛伴发热7天＂入院。患者7天前感冒后出现咽喉痛、颈部疼痛伴发热（38度）,吞咽时疼痛加重,但不伴呼吸困难、咳嗽等,在我院门诊按＂颈部包块伴感染＂给予＂头孢哌酮钠舒巴坦钠＂抗感染治疗症状无好转,遂急诊入院。

简介：ObjectivesToevaluateperipheralauditorydysfunctioninseniledementiaofAlzheimer'sdisease(AD)anditsrelationshipwithcognitivedysfunction.MethodsPuretonethresholds,wordrecognitionscores(WRS),acousticimmittanceandauditorybrain-stemresponses(ABR)weretestedtoevaluatetheauditoryfunctionin43ADpatientsand50normalsubjects.ThetestreliabilityinthesesubjectswasexaminedbeforethetestresultswereevaluatedfortheircorrelationwiththeMiniMentalStateExamination(MMSE)score.ResultsTherewerenostatisticallysignificantdifferencesinperipheralauditoryfunctionsbetweenthetwoearsinthetestedsubjectsorbetweenthetwogroupswhentheauditometricresultsoftherightearwerecompared(P>0.05).Also,therewerenostatisticallysignificantdifferencesbetweenthetwogroupswhenaudiometrictestreliability,acousticimpedanceandABRresultswerecompared(P>0.05).ConclutionsThepuretoneaudiometricthresholdandWRSinADpatientsaresimilartothoseincomparablenon-ADsenilesubjects.Peripheralauditorydysfunctionisnotrelatedtocognitivedysfunction.

简介：ThisarticlereviewstheeffectivenessofintratympaniccorticosteroidsforvertigocontrolinM_eni_ere'sdiseaseat2-yearsfollow-upaccordingtotheguidelinesexpressedbytheAmericanAcademyofOtolaryngology-Head&NeckSurgery.DespitetheincreaseduseofintratympaniccorticosteroidsforvertigocontrolinM_eni_ere'sdiseasethereisdebateastotheireffectiveness,particularlycomparedtogentamicin.Evenso,afterjustasinglecourseofinjections,corticosteroidscanreliablyprovidecompletevertigocontrol(ClassA)at2-yearsinabout50%ofcasesasindicatedinarecentdouble-blindrandomizedcontrolledclinicaltrial(Pateletal.,2016).Buttheeffectivenessofintratympaniccorticosteroidstrulyincreaseswhentreatmentisprovided'as-needed',wherebycompletevertigocontrolisestablishedinupto91%ofcases.Onthebasisofavailableliterature,thereisgoodevidencetorecommendtheuseofintratympanicsteroidtreatmentforvertigocontrolinM_eni_ere'sdisease,butpatientsmustbemonitoredfornon-response.Therationalefortreatingpatientsas-neededandthepossiblereasonsforcorticosteroidnonresponsearediscussed.

简介：Bell’spalsyisacommonlyseencranialnervediseaseandcanresultincompromisedfacialappearanceandfunctions.Itsetiology,prognosisandtreatmentarestillbeingdebated.Thispaperisareviewofrecentdevelopmentintheunderstandingofetiology,diagnosisandnon-surgicaltreatmentofBell’spalsy.

简介：ObjectiveToreportoutcomesofnonsurgicalandsurgicalmanagementofMenière’sdiseaseatBeijingTiantanHospital.MethodsPatientswithMenière’sdiseasewerecategorizedintogroupsbasedonhearingandqualityoflife.Individualizedmanagementwasprovided,includinglifestylemodification,drugtherapies,endolymphaticsacdecompressionandlabyrinthectomy.Treatmentoutcomeswereevaluatedduringupto24monthsfollowup.ResultsEightysevenpatientsunderwentlifestylemodificationanddrugtherapies.ThevertigocontrolrateofGradeAandBwas76.9%and83.8%respectively.Sixpatientsreceivedsurgicalmanagement,includingendolymphaticsacdecompression(n=5)andlabyrinthectomy(n=1).Forthesepatients,thevertigocontrolrateofGradeAandBwas80%and100%,respectively.ConcluIsionsManagementofMenière’sdiseasedependsonseveralfactors,i.e.severitiesofvertigoandhearingloss,qualityoflife,surgicalcontraindicationsandpatientsubjectivedesire.Thetreatmentisdrugtherapiesforthemajorityofpatients,aswellaslifestylemodification.Surgicalindicationsarerareandtheleastinvasiveproceduresshouldbeconsideredfirst.Theresultsofsurgeryaregenerallysatisfying.

简介：ObjectiveToscreenformutationsinmyosin-7Agene(MYO7A)inpatientswithprelingualnonsyndromichearingimpairment.Methods31sporadicpatientswithcongenitalhearingimpairmentand65patientsfrom34familieswithprelingualhereditaryhearingimpairmentinChinaweretestedinthisstudy,and100hearingnormalindividualswereusedascontrol.GenomicDNAisolatedfromwholebloodofallsubjectswassubjectedtopolymerasechainreaction(PCR)toamplifyselectedexonsofMYO7Agene.ThePCRproductsweresubsequentlyscreenedusingsinglestrandconformationalpolymorphismanalysis(SSCP)anddirectsequencingwhenthefragmentsshowedanabnormalelectrophoreticpattern.ResultsGgAtransitionatposition617inexon7,whichwouldproduceanA206Gaminoacidsubstitution,wasdetectedintwopatientsbutinnoneoftheunaffectedmembersinthefamilies.ThisheterozygousmissensemutationhappenedwithinahighlyconservedheptapeptidesequenceofMYO7Aprotein,andiscloselyrelevanttopreligualnonsyndromicdeafness.ConclusionsTheA206Gsubstitutionispossiblyanewmutationtocausepreligualnonsyndromichearingimpairment.Ourresultsprovideevidencethatexon7ofMYO7Aisamutationalhotspotingenetichearingimpairment.

简介：目的通过本实验证明s/z比值在聋儿言语呼吸中的临床应用价值.材料:在上海市随机选取4-17岁640名健听儿童,从国内外数家康复中心随机选出共29名配戴助听器且采用腹式呼吸的聋儿接受s/z测试,以及30名接受胸式呼吸训练的配戴助听器聋儿.方法采用"实时言语矫治仪"进行测试,令受试者先发/s/音,然后发/z/音,均符合最长声时的发音要求,然后求其比值.结果s/z比值不受年龄、性别的影响,且聋儿组康复训练前后s/z比值有着显著性差异,胸式呼吸的s/z比远大于腹式呼吸组.结论s/z比值能够进一步检测出聋儿言语呼吸不协调的问题,为制定康复方案提供了有价值的参考;同时证明胸式呼吸不仅使得聋儿的言语呼吸支持不足,而且造成呼吸和发音系统的严重不协调.

简介：目的研究北京市听障儿童智力发育的特点，比较听障儿童与健听儿童智力发育的差别，为北京市听障儿童康复工作提供理论依据。方法采用希一内学习能力测验的健听常模对56名听障儿童和56名相同年龄的健听儿童的智力进行测验。结果3～7岁听障儿童的比率智商是94．99±14．27，56名健听儿童的比率智商是96．49±12．98，两者的差别没有统计学意义；4岁之前二者的智力水平发展一致，随着年龄的增加，二者的差距增大，差别无统计学意义。与健听儿童相比，听障儿童的手眼协调、空间知觉、视觉记忆和抽象思维发展较好，而色彩记忆和分析综合能力发展滞后。结论北京市听障儿童智力发育良好，已接近健听儿童的水平，社会和家庭应继续关注听障儿童的早期干预和心理健康，以期达到全面康复的目的。

简介：目的：对70岁以上老人的听力障碍情况进行调查，了解高龄老年人群的听力损失特点。方法对103例70岁以上老人进行老年听力障碍量表筛查版（HHIE-S）问卷调查，并采集基本健康信息。结果在103例受调查者中，34例存在听力障碍（33.0%）。存在听力障碍的老人中，70～79岁5例（15.6%），80～89岁21例（36.8%），90～98岁8例（57.1%）。罹患糖尿病对于总得分、情绪得分、社交场景得分具有显著影响（P〈0.05）；年龄对社交场景得分有显著影响（P〈0.05），80岁以下的老年人社交场景得分低于80岁以上老年人（P〈0.05）；噪声接触史对社交场景得分有显著影响（P〈0.05）。结论HHIE-S可较好反映70岁以上老年人听力障碍情况。

简介：目的对河北涿州、高碑店地区重度耳聋患者进行分子流行病学调查，了解耳聋的常见分子病因。方法对河北涿州、高碑店市特殊教育学校64名耳聋学生进行遗传性耳聋问卷调查、全面的体格检查、耳鼻咽喉专科检查以及听力学评估（包括纯音测听和声导抗）。对64名非综合征型感音神经性耳聋患者分别进行GJB2基因235delC突变、线粒体DNA12SrRNA基因A1555G点突变的限制性内切酶分析。应用直接测序法检测SLC26A4基因IVS7—2A〉G突变。结果7例（10．93％）携带GJB2基因235delC纯合突变；9例（14．06％）携带GJB2基因235delC杂合突变；6例（9．37％）携带SLC26A4基因ⅣS7—2A〉G纯合突变，12例（18．75％）携带SLC26A4基因IVS7—2A〉G杂合突变：未发现携带线粒体DNA12SrRNA基因A1555G点突变者。结论河北涿州、高碑店地区非综合征型耳聋患者存在较高的GJB2基因235delC和SLC26A4基因ⅣS7—2A〉G突变发生率，而线粒体DNA12SrRNA基因A1555G突变发生率低于全国平均水平。聋病分子流行病学调查提示河北涿州、高碑店地区20.3％的非综合征型耳聋患者在分子水平能够明确诊断．另有32．81％的患者有遗传倾向。进行准确的耳聋早期诊断、遗传咨询、及时干预和治疗在这一地区的聋哑人群中非常重要。