简介：Cisplatindamagescochlearhaircellsandspiralganglionneuronsthroughcelldeathsignalingpathwaysthatarenotfullyunderstood.Weusedfocusedapoptosisgenemicroarraystostudyearlychangesingeneexpres-sionincochlearculturesfromP3neonatalratstreatedwithcisplatin(0.2mM).After12hoursofcisplatintreat-ment,morethan50%ofthe96genesonthearrayshowedasignificantdecreaseinexpression,consistentwithwidespreadcelldeath.However,after3hoursofcisplatintreatment,10genesshowedsignificantincreaseinex-pressionintotalcochleartissue.Inexperimentswithsubsetsofcochleartissues,at3h,cisplatininducedincreasedexpressionof12genesinthecochlearsensoryepithelium(basilarmembrane)and11genesinthespiralganglion(tissueofRosenthal'scanal,containingthespiralganglion).Theseincludedpro-andanti-apoptoticgenesin-volvedinthep53signalingpathway,TNFreceptorfamily,NF-kappaBpathway,deathdomainfamily,deatheffec-tordomainfamily,Bcl-2family,CARDfamily,TRAFfamily,andGTPsignaltransduction.Althoughthechangesingeneexpressionshowedanoverlapbetweenbasilarmembraneandspiralganglion,otherchanges,whichmayreflecttheuniqueresponseofeachtissue,werealsoobserved.Pifithrin-αblockedcisplatin-inducedup-regulationofgenesinthep53signalingpathwaywhenassayedbybothsuperarrayandrealtimePCR.Thedataaddtoourunderstandingoftheinvolvementofp53incisplatin-inducedototoxicityandotoprotection,conferredbythep53inhibitorPifithrin-α.

简介：NuclearfactorkappaB(NF-κB)isoneofthebest-characterizedtranscriptionfactorsplayingimportantrolesinmanycellularresponsestoalargevarietyofstimuli,includinginflammatorycytokines,phorbolesters,growthfactors,andbacterialandviralproducts.TheaimofthisstudyistodemonstrateNF-κBexpressioninthemousecochleaanditsenhancementinresponsetolipopolysaccharides(LPS)andkanamycin(KA)treatment.MethodsKAtreatmentconsistedofsubcutaneousKAinjectionsat700mg/kgtwiceadaywithaneight-hourintervalbetweenthetwoinjectionsfor3or7days.ForanimalsintheLPStreatmentgroup,asingledoseof0.3mgLPSdissolvedin0.2mlsterilesalinewereinjectedintobothbullaethroughthetympanicmembraneandkepttherefor3hours.Animalsinthecontrolgroupreceivedsubcutaneoussalineinjectionfor7days.Followingimmmunohistochemichalprocessingwithrabbitpolyclonalanti-NF-κBp65antibodies,cryosectionsofthecochleawereexaminedforexpressionofNF-κBp65invariousstructuresinthecochlea.ResultsNF-κBp65expression,identifiedbypresenceofbrownreactionproductscharacteristicofDABimmunohistochemistry,wasvisibleinthespiralligament,spiralprominence,tectorialmembrane(TM),spiralganglionandnervefibers.RelativelyweakNF-κBp65expressionwasalsovisualizedintheorganofCorti.WithintheorganofCorti,theinnerhaircells(IHC),outerhaircells(OHC),innerpillarcells(IP),outerpillarcells(OP),Deiter'scells(DC),andBoettcher'scellsexhibitedstrongerstainingthantheinnersulcuscells,Hensen'scells(HC)andClaudius'cells.NoNF-κBp65expressionwasseeninthenucleusoftheIHCandOHC.NF-κBp65expressionwasincreasedinanimalsexposedtoLPSorKA,demonstratingsignificantdifferencesinthestainingbetweencontrolanimalsandLPS/KA-treatedanimals.NF-κBp65expressionwasnotsignificantlydifferentbetweenLPStreatedandKAtreatedanimalsorbetween3and7daysinKA-treatedanimals.Conclusio

简介：遗传性耳聋是危害人类健康的重大疾病之一，根据是否合并其他系统器官疾病，分为综合征性耳聋和非综合征性耳聋，而非综合征性耳聋具有很高的遗传异质性。迄今为止，常染色体显性遗传非综合征性耳聋（DFNA）已成功定位了64个位点，24个基因（HereditaryHearingLossHomepage：http：／／webhost．ua．8C．be／hhh／）。第五个常染色体显性遗传非综合征性耳聋基因DFNA5（OMIM600994）于1995年在一个高频进展性听力下降的荷兰家系中首先定位在7p15，

简介：患者陈女士，58岁，汉族，河南省人，职员。以“左耳搏动性耳鸣5年”主诉入院。患者于5年前劳累后逐渐出现左侧搏动性耳鸣，持续性，似“火车车轮声”，与心脏跳动一致，仅患者本人可闻及，改变头位对耳鸣无影响，但手指压迫左侧颈部后耳鸣可暂时消失，发病后左耳听力逐渐降低。

简介：ObjectiveAlongwithchangesintheecologysystemandundertheinfluenceofvariousenvironmentalfactors,theincidenceoftumorhasbeenincreasingyearafteryear.Thereisatrendincancertherapytomovetocombinedtherapiesinvolvingsurgery,radiationchemotherapyandgenetherapy.Cancergenetherapyinrecentyearshasbroughtnewopportunitiesfortreatmentoftumor.Itsadvantagesincludelowrateoftolerance,insensitivitytocellcycles,highspecificityandcoverageforbothprimaryandmetastatictumors1,2.However,thisisanewfieldofclinicalresearch.RegardingthecorrelationamongtheSTAT3,CyclinD1andP21genesandtumors,researchhasfocusedontheirexpressionandregulation.Thisarticleprovidesasummaryofrelatedresearch.

简介：6年前感冒后再次出现左耳流脓,并伴有耳痛、头痛、听力下降,自服“消炎药”后症状缓解。患者李女士,75岁,汉族,山东人,以“左侧中耳炎、胆脂瘤多次手术后耳痛、流脓5月余”之主诉入院。患者自幼左耳反复流脓,滴药后可缓解,不伴明显听力下降、耳痛等。

简介：Background:Currently,thereisasignificantlackofdataconcerninglong-termoutcomesfollowingpaediatriccochlearimplantationintermsofqualityoflife.Thereisaneedforalong-term,prospectivestudyinthisregard.Thisstudyaimsathighlightingthepreliminaryresults,oneyearpostsurgeryofafiveyearprospectivestudy.Methods:TheCochlearTMPaediatricImplantedRecipientObservationalStudy（P-IROS）isaprospective,patientoutcomesregistryforroutinelyimplantedchildren.Thestudycollectsdatausingquestionnairespost-surgeryandatregularintervalsuptofiveyears.Results:AtourCentre,159cochlearimplantsurgeryprocedureswerecarriedoutbetweenJanuary2014andDecember2014.CategoryofAuditoryPerformanceIIscoreincreasedfrom‘0’to‘3’atsixmonthsandto‘5’at12monthsforchildrenaged0—3years,althoughthiswasnotstatisticallysignificant.However,thesametrendwasstatisticallysignificantfortheage3—6yearandage6—10yearbrackets.Thequalityoflifeofthechildimprovedsignificantly.Analysisofcommunicationmoderevealedastatisticallysignificantoverallshifttotheauditory-oralmodefromtotalcommunication.Conclusion:Cochlearimplantationisalife-changingintervention.Theevidenceinsupportofwhatitcanachievesafelyisclear.However,thecostsassociatedwithitraisethequestionifitwillremainaneffectiveoptionforlifeinallchildren.TheCochlearP-IROSisanattempttoanswerthesameoverafiveyearperiod.OurstudyinNewDelhi,sofarconcludesthatcochlearimplantationinapopulationwithlimitedaccesstofundsisveryeffective,oneyearaftersurgery.

简介：目的考察3～5岁听障儿童的气质特点及其影响因素，为康复训练提供参考依据。方法选取111名3～5岁听障儿童，采用3～7岁儿童气质问卷施测。结果3～5岁听障儿童在气质各维度上的表现倾向不同（P〈0．05），适应度维度得分最高，其次为趋避性、心境、注意分散度和规律性，注意力持久性和反应阂得分最低。儿童性别、主要照顾人及其受教育水平、职业、家庭结构对听障儿童气质的多个维度具有显著影响（P〈0．05），主要表现在反应强度、注意力持久性、规律性、注意分散度、活动量、心境和适应度维度；助听设备和康复时间对听障儿童气质特点影响不明显（P〉0．05）。结论3～5岁听障儿童的气质受家庭、社会环境等多方面因素影响，但在具体特征上有其独特性。

简介：目的研究10号染色体缺失张力蛋白磷酸酶（phosphataseandtensinhomologuedeletedonchromosometen，PTFEN）、磷酸化Akt（P—Akt）及核转录因子-KB（NF—KB）在中耳胆脂瘤上皮中的表达，探讨P13K（phos—phatidylinositol-3-kinase，磷脂酰肌醇-3激酶）-Akt信号通路在中耳胆脂瘤上皮细胞过度增殖机制中的可能作用。方法采用免疫组织化学SP法（辣根酶标记链霉卵白素连接法，streptavidin—peroxidaseconjugatedmethod）检测30例中耳胆脂瘤组织标本与15例正常外耳道皮肤标本中PTEN、P—Akt及NF—KB蛋白的表达。结果PTEN蛋白阳性表达主要定位于上皮细胞核，其在中耳胆脂瘤上皮中阳性表达率为36．7％，明显低于正常外耳道皮肤组的9313％（P〈0．01）；P—Akt蛋白阳性表达主要定位于上皮细胞胞质，其在中耳胆脂瘤上皮中阳性表达率为70．0％．明显高于正常外耳道皮肤组的26．7％（P〈0．01）；NF—KB蛋白阳性表达定位于上皮细胞核．其在中耳胆脂瘤上皮中阳性表达率为63-3％，明显高于正常外耳道皮肤组的20．0％（P〈0．01）。在30例中耳胆脂瘤上皮组织中，PTEN分别与P—Akt、NF—KB蛋白的表达之间呈显著负相关（P〈0．01），而P—Akt和NF—KB蛋白的表达呈显著正相关（P〈0．01）。结论PTEN、P-Akt和NF—KB在中耳胆脂瘤上皮的异常表达可能在胆脂瘤的发生、发展过程中起重要作用。胆脂瘤上皮中P13K—Akt信号通路的激活可能参与了胆脂瘤上皮细胞过度增殖机制。

简介：ObjectiveChronictinnitusisahighlyprevalentconditionandhasbeenhypothesizedtoresultfromaninnatedisturbanceincentralnervousserotonergictransmission.Giventhefrequentcomorbiditywithmajordepressionandanxiety,wearguethatcandidategenesforthesedisordersarelikelytooverlap.Thepresentstudyaddressesthegeneencodingforthe5-HT1Areceptorasaputativeriskfactorfortinnitus.MethodsIn88subjectswithadiagnosisofchronicsubjectivetinnituswhounderwentadetailedneurootologicalexamination,theentire5-HT1AgenewasamplifiedusingoverlappingPCRproducts.Ampliconswerecustomsequencedbidirectionallyandwerescreenedforvariantsinmultiplealignmentsagainstthehumangenomereference.ResultsWeidentifiedasynonymousC>Texchangeatresidue184(Pro)in7/88subjects,butdetectednomissensevariantsinthepopulationunderstudy.Specifically,thefollowingresidueswerefullyconserved:16(Pro),22(Gly),28(Ile),98(Val),220(Arg),267(Val),273(Gly),and418(Asn).DiscussionThepresentdatacountagainstthecausationofchronictinnitusbyachangeinthe5-HT1Areceptor'saminoacidsequence.However,theallelefrequencyforthe184Prominorallele(0.04)reachedtwicethefrequencyreportedincontrolcohortsfromthesameethnicity.Additionalinvestigationsareinvitedtoclarifytheroleofthe5-HT1Apolymorphisminlargersamples,andtocontrolforcomorbidaffectivedisorders.

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简介：目的：探讨中国人X连锁Alport综合征患者的听力表型与皮肤组织Ⅳ型胶原α5链表达的关系。方法收集2008年8月至2013年8月期间确诊为X连锁Alport综合征的31例患者临床资料，采用纯音听阈测试或ABR＋40Hz相关电位检查＋声导抗＋耳声发射的方式进行听力评估，并采用免疫组化染色检测皮肤组织基膜Ⅳ型胶原α5链的表达，采用Pearson相关分析方法分析二者的关系。结果31例X连锁Alport综合征中听力下降患者均表现为轻、中度感音神经性聋。听觉损害共28例（90.3%），其中22例男性中中度12例，轻度5例，轻微损害5例；女性6例，轻度3例，轻微损害3例。按皮肤Ⅳ胶原α5链染色分级,阴性17例均为男性，其中听力正常4例（3例OAE异常），听力下降13例（轻度4例，中度9例）；大部阴性与可疑阳性各1例，均为男性，分别为中度和轻度；连续＆#177;2例（男性），正常和中度各1例；间断阳性3例均为女性，均为正常或轻度；连续＋＋7例（男3,女4），其中听力正常4例，听力下降3例（2例轻度，1例中度）。染色阴性的11例听力下降患者听阈与年龄呈正相关（P=0.043，r=0.616）。结论X连锁Alport综合征患者皮肤组织Ⅳ胶原α5链的表达男性低于女性，听力表现与皮肤组织Ⅳ胶原α5链的表达有一定的关系，其中Ⅳ胶原α5链表达阴性的患者听力下降程度与年龄存在相关性，但也存在听力正常的患者，说明还有其他因素影响患者的听力。皮肤组织中Ⅳ胶原α5链表达在一定程度上能反映耳蜗基底膜中Ⅳ胶原α5链的表达和功能。