简介:ObjectivesTostudyclinical,imagingfeaturesandtreatmentoutcomesofcongenitalcholesteatomaofmiddleear(CCME).MethodsThisisaretrospectivereviewof10CCMEcasesselectedfrom952cholesteatomacasestreatedbetweenJanuary1995andDecember2005attheDepartmentofOtolaryngology-HeadandNeckSurgery,ChinesePLAGeneralHospital.Themainoutcomemeasureswerethesiteoforigin,clinicalfeatures,surgicalfindings,imagingcharacteristicsandhearingresults.ResultsThemeanageofthe10patientswas16years(rangedfrom10to24years),with6beingolderthan18years.Therewere7malesand3females.Theaveragedelaytodiagnosiswaslongerthan2years.ThemeanpreoperativePTAwas55dBHL,withameanABGof45dB.Typicalcholesteatomaswereseenbehindthetympanicmembraneinthesuperoposteriorquadrantonotoscopyonlyin2patients.HighresolutionCTwascompletedinallpatients.Mostofthepatients(8/10)werediagnosedwithotosclerosisorossicularabnormalitybeforeoperation.Allpatientsunderwentaone-stagetympanoplastyfollowingtransmeatalexplorativetympanotomyandcompletecholesteatomaremoval,exceptone,whounderwentaCWUmastoidectomyduetoextensivecholesteatomainvolvement.Thecholeasteatomalesionwasconfinedtothesuperoposteriormesotympanuminallpatients.ThemeanpostoperativePTAwas20dBHL.Allpatientswerefollowed-upforatleast1.5yearspostoperatively.Revisionprocedureswereperformedin2patientsforhearingdeterioration.Noresidualorrecurrenceofcholesteatomawasfound.ConclusionCCMEisararediseasethatoftengetsdelayeddiagnosis.Residuallesionsandtheprognosismainlydependontheextentofthelesion.
简介:ObjectivesToreportauthors'experiencesinthediagnosisandtreatmentsofcongenitalfirstbranchialfistula(congenitalauriculocervicalfistula).MaterialsandMethodsTwelvecasesofcongenitalfirstbranchialfistulawerereviewed.Ofthese,8underwentfistulectomywithfacialnervedissectionandpartialparotidectomyand4underwentsimplefistulectomy.ResultsTheinneropenings(upperopening)offistulaelayinthefollowingsites:inferioposteriorwallatthejunctionofcartilaginousandbonysegmentsoftheauricularcanalandinferiorwallofcartilaginousauricularcanal.Theouteropenings(loweropening)layalongtheanteriorborderofuppersternocleidomastoidmuscle,atthemastoidtipandposteriortothemandibularangle.Completefistulaeresectionwasachievedinallbutonecase.Elevencaseswerefollowedfor5yearwithnorecurrence.Recurrenceoccurredin1case6monthsaftertheprimarysurgeryandrevisionsurgerywasperformed.ConclusionsPre-operativeradiographyforthelocationandcourseofthefistulaiscrucialforsuccessfulfistularesection,especiallyincaseswithpastinfections.Facialnervedissectionshouldbedoneroutinelyfordeeplylocatedfistulae.
简介:ObjectiveTocomparedifferenttreatmentprotocolsforsuddendeafness(SD),forthepurposeofidentifyinganappropriateapproachtoSD.MethodsAtotalof104patientswithdiagnosisofsuddenhearinglosstreatedfromJan2006toDecember2008wereincludedinthisstudy,ofwhich31receivedthetypicalpharmaceuticaltreatment(groupⅠ),40receivedthetypicalpharmaceuticaltreatmentpluspolarizedliquid(GroupⅡ)and33receivedthehyperbaricoxygeninadditiontothetreatmentincludedinGroupⅡ(GroupⅢ).ResultsThetotalimprovementrate(67.74%,62.50%and75.76%forGroupsⅠ,ⅡandⅢrespectively)wasnotstatisticallydifferentbetweenthethreegroups(P>0.05).ConclusionThethreetreatmentprotocolsaresimilarwhenjudgedbythetreatmentoutcomesinSD,neitherbeingsuperiortotheothers.Thetwoimportantfactorsthatappeartoinfluencetreatmentoutcomesaretheaudiogrampatternanddurationofhearinglossbeforeseekingtreatment.Patientswithupslopingorpeak-typeaudiogramsandtreatedwithin7daysfromtheonsethavebetterprognosisthanothers.
简介:Objective:Toreportascalatympanidrill-outtechniqueformanagingmalformedfacialnervecoveringtheentireovalwindow(OW).Methods:DatafromthreecaseswithOWatresia,malformedstapesandabnormalfacialnervecourseswerereported,inwhichascalatympanidrill-outtechniquewasemployedwithaTORPbetweenthetympanicmembraneandscalatympanifenestrationforhearingreconstruction.Results:Airconductionhearingimprovedintwoofthethreecasesfollowingsurgery.Inthethirdcase,therewasnoimprovementinairconductionhearingfollowingacanalwallupmastoidectomyandtympanoplasty.Therewerenovertigo,tinnitusorsensorineuralhearinglossinthethreecases.Conclusion:Thescalatympanidrill-outtechnique,whichisbasicallyfenestrationattheinitialpartofthebasalturn,providesachoiceinhearingreconstructionwhentheOWiscompletelycoveredbyabarrentlycoursedfacialnerve.
简介:ObjectivesLangerhans'Cellhistiocytosis(LCH)isararedisease,whichremainspoorlyunderstoodandwhosecellularoriginremainsunknown.ToincreaseunderstandingoftemporalboneLCH,itisnecessarytostudyrecentadvancesinthediagnosisandtreatmentofthisdisease.MethodsThelongterm(5to30years)resultsof21temporalboneLCHcasestreatedbetween1973and2003werereviewed.Surgery,radiotherapy,pharmacologictherapyoracombinationofthesetreatmentswereemployedinthesecases.ResultsEighteenpatientswerecured(18/21,85%).Sixpatientsdevelopedresidualdiabetesinsipidus(DI)anddwarfism(28%).Threepatientsdied(14%).ConclusionsTheAlessiclassificationsystemforLCHbasedontheextentofdiseaseaccuratelypredictsprognosisandisausefulguideinselectingtreatmentmethodologies.X-ray,computedtomographyandmagneticresonanceimaginghaveprovedusefulindefiningtheextentofosseousandsofttissuediseases.DiagnosisofLCHisbasedonclinicalpresentations,radiographicfindingsandhistopathologicalresults.Surgeryandradiotherapyarethemaintreatmentmodalities.Pharmacologictherapyshouldbeusedinpatientswithaggressive,disseminate,andrefractorylesions.LCHhasapredilectionforchildrenandprognosisdependsonageandextentofvitalorganinvolvement.
简介:摘要目的MRI、CT与钼靶X线在乳腺疾病诊断中的价值。方法本文选取我院于2013年08月~2014年08月收治的36例乳腺疾病患者,所有患者在手术开始前实施MRI、CT与钼靶X线三种不同方式的诊断检查,进一步和手术后病理结果进行对比分析。结果三种不同检查方法诊断结果和术后病理结果对比后发现采用MRI、CT两种检查方法的诊断结果和术后病理结果之间没有显著性差异,同时钼靶X线诊断结果和MRI、CT以及术后病理结果之间都存在显著性差异(P<0.05),具有统计学意义。结论乳腺疾病患者采用MRI、CT与钼靶X线不同检查方法检查后,其中钼靶X线可以用于早期乳腺疾病诊断,而MRI和CT两种检查方法具有较高的乳腺疾病检出率和诊断特异性,值得在临床中推广应用。
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。
简介:Objective:Basedontheclinicalmanifestationsofahearinglosspatient,thePOU3F4genewastestedfordiagnosisofetiology.Methods:Acomprehensivephysicalexaminationwasperformedontheprobandtoexcludeabnormalitiesofotherorgans,anddetailedaudiologicaltestingandtemporalboneCTscanwerealsoperformed.GenomicDNAwasextractedusingtheproband’speripheralbloodleukocytes.Polymerasechainreactions(PCR)wereperformedinthecodingsequenceofthePOU3F4gene.DirectDNAsequencingwassubsequentlyappliedtoscreentheentirecodingregionofthePOU3F4gene.Results:Theprobandhadseveresensorineuralhearingloss.TemporalCTshowedbilateralcochlearincompletepartition,vestibuledysplasia,internalauditorycanalfundusexpansion,andcochlearinterlinkwiththeinternalauditorycanalfundus.Anovelmutation(c.530C>A(p.S177X))inthePOU3F4genewasfoundinthispatient,creatingannewstopcodonandwaspredictedtoresultinatruncatedproteinlackingnormalPOU3F4transcriptionfactorfunction.Conclusion:ThroughanalysisofthePOU3F4geneandclinicalmanifestationsinthepatient,weconcludethatanovelmutationmayhaveresultedinaprematurestopcodon,contributingtothemutationofPOU3F4gene.