简介：AlportsyndromeisanX-linkedsyndromethatresultsinnephritis,renalfailure,sensorineuralhearingloss,andeyedeficits.Asaresultofsensorineuralhearingloss,theseindividualsarelikelytoexperiencedifficultiesintheareaofspeechandlanguage.Whilestudiesinthepasthaveexaminedthespeechandlanguagecharacteristicsofchildrenwithsyndromicsensorineuralhearingloss,toourknowledgetherearenopreviousstudiestohavedocumentedthespeechandlanguagecharacteristicsofthesechildrenonalong-termbasis.Thecurrentstudyaddressesthislimitationbyreportingspeech,language,hearing,andfunctionoftwinbrotherswithX-linkedAlportsyndromeacrossaseven-yearperiod.Informationwascollectedbyexaminingthemedicalrecordsoftheparticipantsaswellasthroughaverbalinterviewwiththeparticipants'guardian.Resultsrevealedthattheparticipants'hearingabilitiesgraduallydeterioratedovertheseven-yearperiodwhichaffectedtheirspeechandlanguagedevelopmentaswell.Thekidneyfunctiontestsrevealedsignificantpresenceofhematuria(bloodintheurine)aswellasproteinuria(proteinintheurine)suggestingchronickidneydysfunction.Thislongitudinalstudydemonstratesthefunctionalrelationshipbetweenthekidneysandthecochlea,althoughtheyappeartobeindependentofoneanother.AsindividualswithAlportsyndromeexhibitsystemiccomplications,interdisciplinarycollaborationisessentialamonghealthcareprovidersincludingaudiologists,speech-languagepathologists,nephrologists,andophthalmologisttopromoteevidence-basedpractice.

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.