耳鼻咽喉头颈外科学教学2017刘绮明耳聋眩晕.ppt

,QimingLiu刘绮明Dept.ofOtorhinolaryngology2ndAffiliatedHospitalofGuangzhoumedicaluniversity广州医科大学附属第二医院耳鼻咽喉科教研室,www.ent-,HearingLoss耳聋,Vertigo眩晕,Tinnitus耳鸣,hearing,Keepingbalance,耳有什么功能？,人是如何听到声音？,Boneconduction骨传导,Airconduction空气传导,Hearingloss耳聋,目的与要求,【掌握】1.耳聋的分类和分级。2.常见的感音神经性聋。,Definition定义,Thelesionsintheauditoryconductivepathwaycausetotalorpartialinabilitytohearsound.从外耳到大脑皮层听觉通路上任何一个部位及附近的病变导致不同程度听力损害的总称。,Hearinglosssituation概况,听力残疾（hearingdisability）：各种原因导致双耳不同程度的永久性听力障碍（hearinghandicap），听不到或听不清周围环境声或言语声，以至影响日常生活和社会参与。全世界听力障碍1985年4200万；1995年1.2亿；2001年2.5亿；2012年5%人口（3.6亿）2006年我国有听力语言障碍的残疾人2780万人，占6000万残疾人总数约1/3。200多万聋儿（deafness），其中七岁以下80多万，每年新增聋儿3万余名（60%-遗传）。广东听力残疾136多万人。不同程度听力障碍者在人群中的比例，4564岁为14，6575为30，75岁以上者高于50。2015年WHO，12-35岁中4300万有听力障碍；11亿有不安全用耳听力损失风险。,Classification分类,3basictypes:(whichpartsofhearingpathwayisaffected按病变部位分)ConductiveHearingLoss-CHL传导性SensorineuralHearingLoss(sensory,neural,central)-SNHL感音神经性MixHearingLoss-MHL混合性,Degree分级（WHO，1997年）,Mildloss轻度:2640dBHLModerateloss中度:4160dBHLSevereloss重度:6180dBHLProfoundloss极重度:81dBHL,Audiogram听力图,“threshold”听阈isthesoftestintensitythatsoundisperceived.(0.512k4kHz)“dBHL”isdecibels分贝hearinglevel,如何估计听力损失程度？,ConductiveHearingLoss传导性聋,1、炎症2、外伤3、阻塞4、先天畸形5、听骨链病变,SensorineuralHearingLoss感音神经性聋,。,1、Sensoryhearingloss感音性,2、Neuralhearingloss神经性,Haircell毛细胞,Spiralganglion螺旋神经节,Striavascularis血管纹,3、Centralhearingloss中枢性,Mixhearingloss混合性聋,传音和感音机构同时有病变存在，影响声波传导与感受。如急慢性化脓性中耳炎并发迷路炎、耳硬化症晚期、爆震性聋等。,Audiometry听力学检查,传导性聋,感音神经性聋,混合性聋,正常,纯音测听Audiogram听力图,Audiometry听力学检查,Tympanogram鼓室图,Otoacousticemission（OAE）耳声发射,Auditorybrainstemresponse（ABR）听性脑干反应,Tuningfork音叉检查,Imaging影像学检查,常见的感音神经性聋,1、NonhereditaryHearingLoss非遗传性聋SuddenHearingLoss突发性聋Ototoxicityhearingloss中毒性聋AgedrelatedHearingLoss老年性聋Noise-inducedHearingLoss噪音性聋2、HereditaryHearingLoss遗传性聋,SuddenHearingLoss(SHL)突发性聋,Definition定义:Suddenhappengreaterthan20dBhearingreductionatleasttwoconsecutivefrequenciesin72hoursorless.突然发生的原因不明的在三天之内至少连续两个频率听力下降20dB。Causes:Vascularlesion血管病变VirusInfections病毒感染Autoimmune自身免疫Roundorovalwindowmembranerupture窗膜破裂Neurologicalneoplasm神经系统新生物.,SHLClinicalCharacteristics突发性耳聋临床特征,Suddenhappen突发,nofluctuating无波动性,moderatetosevereloss中重度聋Tinnitus耳鸣Vertigo眩晕Noothercranialnerveslesion无其他颅神经损害Unilateral单侧多见Otherpresentation：auralfullness耳堵塞感Tendstorecoverspontaneously自愈倾向,突发性聋诊治指南,2011德国标准2012美国标准2015中国标准分型：1、低频下降型2、高频下降型3、平坦下降型80dBHL4、全聋型81dBHL,SHLDifferentialdiagnosis鉴别诊断,Meniere,sDisease梅尼埃病AcousticNeuroma听神经瘤FunctionalHearingLoss功能性聋,HereditaryHearingLoss遗传性聋,Syndromic综合征性:30%Pendred先天性聋甲状腺肿大Waardenburg感音性聋及色素异常Usher先天性聋视网膜色素变性Alport遗传性基底膜病，肾-耳-眼病变.Nonsyndromic非综合征性:70%AutosomalDominant常染色体显性1524%,AutosomalRecessive常染色体隐性7585%,X-linkedX连锁12%,Mitochondrial线粒体G,2168AG）线粒体12SrRNA(MTRNR1):(1555AG,1494CT)GJB3(538CT),精准医学PrecisionMedicine,胚胎植入前遗传学诊断(preimplantationgeneticdiagnosis,PGD）中国聋病基因组计划遗传咨询和临床指导,中国首列成功阻断重度遗传性耳聋第三代试管婴儿,Diagnosis耳聋的诊断,History病史Physicalfinding全身体格检查ENT耳鼻咽喉检查Audiometry听力检查Imaging影像学检查其他检查Otherrelatedtest（serologic血清学testing,immunologic免疫testing,genetic基因testing),Treatment耳聋治疗,MedicalManagement药物（针对病因）Hyperbaricoxygentherapy高压氧Acupuncture针灸Surgicaltreatment手术疗法Hearingaid助听器验配Implantablehearingaid植入性助听技术（CochleaImplant人工耳蜗、Brainstemimplants脑干植入)Genetherapy基因治疗Stemcelltreatment干细胞治疗,PreventionofSNHL预防感音神经性聋,Antenatalandperinatalcare孕期和围产期保健Newbornhearing(+genetic基因)screening新生儿听力筛查Agedhearingcare老年人口听力保健Hearingcareandfoodnutritionandhygiene听力保健与食品营养卫生学NoiseExposureAvoidance避免噪声（HPD）Ototoxicmedicationsmustbeadministeredcarefully慎用耳毒性药物,Howtoprotectourear?预防比治疗更重要更有效,EarcareDay爱耳日,Since20003thMarch(3.3)2017theme主题：Prevention,treatmentandpreciseserviceforhearingimpairmentanddeafness防聋治聋，精准服务,HearingAid助听器,助听器验配师:中国-四级(初级)三级二级听力师?:听力与言语康复学专业:浙江中医药大学、北京大学协和医院、滨州医学院、昆明医学院、中山大学新华学院.,Audiologist,HearingAid助听器,气导助听器In-the-Ear(ITE)耳内式Behind-the-Ear(BTE)耳背式In-the-canal(ITC)耳道式Completely-in-the-canal(CIC)深耳道式BodyAid体佩式(盒式),HearingAid助听器,骨导助听器:BAHA（boneanchor)骨锚式助听器非植入式：头戴式植入式：,植入式骨导助听器:bonebridge（骨桥）,Middleearhearingaidimplant人工中耳植入,振动声桥,CochleaImplantCandidates电子耳蜗植入条件,双耳极重度感音神经性聋：语前聋12M-6Y，语后聋任何年龄无法借助听器等改善听力无手术禁忌症监护人或植入者正确的认识和合理期望具备听觉言语康复教育的条件医学评估（听力学、影像学、或基因检测等）心理评估,Auditorycenterimpant听觉中枢植入,Questions,Classificationanddegreeofhearingloss？耳聋分类分级？WhatarethecommonSNHL？常见的感音神经性聋？,Tinnitus耳鸣,Definition定义,tinnitus“tinnere”(Latin)拉丁词Itiscommonlydescribedasaperceptionofsoundintheearorinthebrainthatisnotrelatedtoanexternalacousticsourceorelectricalstimulation.主观上感觉耳内或颅内有声响，但外界并无相应声源存在。,Incidence发生率,一般人群：4.415.1%老年人：33%儿童：1.66.5%50%ofpatientswithsuddenhearingloss突发性聋70%withpresbyacusis老年性聋50-90%withnoise-inducedhearingloss噪音性聋,Classification分类,Acute急性：3个月内Chronic慢性:6个月亚急性：3-6个月Subjective主观性:最常见Objective客观性：,Etiology病因,特发性（idiopathic）：原因不明Auditorysystem(peripheralandcentral)外周和中枢听觉系统病变Hormonalchanges激素水平改变Othersystemicdiseases其他系统疾病Somemedicationsorwithdrawalfromthem一些药物或撤药后反应Psychologicalfactors精神心理性因素,Etiology病因,Somatosounds体声(大部分是客观性)Pulsatile搏动性Myogenic肌源性Eustachiantubeabnormalpatulous咽鼓管异常开放.Jointabnormalities:temporomandibularjointdisorder颞颌关节病变.,Pathophysiology病理生理(neurophysiologicmode神经生理学模式),Jastreboff(1990),情绪反应,躯体反应,Diagnosis诊断,定性、定位、定因、定量History病史Examination检查General一般全身检查NeurotologyandENT神经系统检查和耳鼻咽喉科检查Cochlearandvestibularfunction耳蜗及前庭功能评价Tinnitustest耳鸣检查Imaging:CT/MRI/functional-MRIPsychologicalevaluation精神心理学评价Visualanaloguescale（VAS）视觉模拟评分Tinnitushandicapinventory（THI）耳鸣致残量表Statetraitanxietyinventory（STAI）焦虑特质量表Qualityoflifequestionaire生活质量调查表耳鸣简易评分-中国.刘蓬,Treatment治疗,Tinnituscombinedmanagement(TCM)canbemorebeneficalthanasingletherapy/Individualtherapy综合治疗比单一治疗有效,个体化治疗原则Etilogicaltreatment病因治疗Medication(pharmacotherapy)药物治疗Soundtherapy声治疗Tinnitusretrainingtherapy(TRT)耳鸣习服Cognitivebehavioraltherapy行为认知Biofeedback生物反馈Electricalstimulationtherapy电刺激治疗Transcranialmagneticstimulation(TMS)经颅磁刺激Surgery手术部分体声tinnitusjustlivewithit?适应耳鸣,Treatment治疗(medication药物),Notsingle没有单一,effective有效,specific特定,secure安全,andreliable可靠drugshasyettobeidentified.Vasodiator扩张血管(niacin烟酸，nalador前列腺素E2)Improveinnerearenergymetabolism改善内耳能量代谢(ATP三磷酸腺苷,coenzymeA辅酶A)Calciumchannelblockers钙通道阻滞剂(nimodipine尼莫地平,sibelium西比灵)Anticonvulsant抗惊厥剂(carbamazepine卡马西平)Localanesthetics局部麻醉剂(lidocaine利多卡因,procaine普鲁卡因)Musclerelaxant肌肉松弛剂(myonal乙哌立松)Antidepressants抗抑郁(doxipinum多塞平,estazolam艾司唑仑)苯二氮卓类药物（clonazepam氯硝西泮.）谷氨酸受体拮抗剂（acamprosatecalciam阿坎酸）Others其他(ginkgobiloba银杏制剂),2012耳鸣专家共识及解读中华耳鼻咽喉头颈外科学杂志,原因不明统称为特发性（idiopathic）耳鸣，废除“神经性耳鸣”诊断名词；耳鸣可伴有听觉过敏（hyperacousic）；特发性耳鸣需采用综合疗法，重点减轻或消除耳鸣相关的不良心理反应；对伴有听力下降的耳鸣患者，可使用助听器，人工耳蜗植入（重度极重度感音神经性聋）；建立简洁适合国情的耳鸣评估量表。,Tocuresometimes,toreliefoften,tocomfortalways.,Questions问题,Whataretinnitustreatments？耳鸣的治疗方法？,Vertigo眩晕,目的和要求,【掌握】周围性和中枢性眩晕的区别。梅尼埃病的临床特征。,IAPA(InternationalAssociationofphysiciansinaudiology)国际耳内科医师协会Baranysociety巴拉尼协会：耳神经、耳鼻喉头颈外科、康复医学等专家组成。(2009-ICVD,2015-MD/VM/BPPV.),人是如何维持平衡?,Visual视觉系统Proprioceptive本体感觉Vestibule前庭系统,Anatomyandphysiology解剖生理,3semicircularcanals-angular角加速度,2otolith耳石器-linear线加速度+gravity重力,椭圆囊,球囊,Whatisvertigo?,Vertigo眩晕vert(Latin)拉丁词Vertigoisthesensationofselfmotionwhennoself-motionisoccurringorthesensationofdistortedself-motionduringanotherwisenormalheadmovement.（Internalvertigo内在眩晕）是因机体对空间定位障碍而产生的一种运动性或位置性错觉。可分为旋转性或非旋转性（摇摆、倾斜、漂浮、跳动、滑动）。,前庭症状的描述,ICVD前庭疾病国际分类将前庭症状分为4类：眩晕（vertigo）：自发、诱发（位置、头动、视觉、声音、体位、Valsalva等）。头晕（dizziness）:没有运动错觉的空间定向障碍或损害，头晕不包括眩晕，两者可同时存在。前庭-视觉症状（vestibulo-visual）：外在的眩晕、视震荡、视滞后、视倾斜、运动诱发性视模糊。姿势症状（postural）：不稳、方向性倾倒、平衡相关的近乎跌倒、平衡相关跌倒。,Howtodifferentiatingcentralandperipheralvertigo?如何鉴别周围性和中枢性眩晕？,Peripheralvertigo周围性眩晕,Suddenrotatingvertigo突发性旋转性Severe较剧烈Associatedcochlearsymptoms(unilateralhearingloss耳聋,tinnitus耳鸣,fullness耳胀闷感)“harmonic”vestibularsyndrome前庭反应协调Symptomsaggravateaftermovingofthebodyandheadposition头位或体位变动时眩晕加重Lastseveralsecondstohours持续数秒到数小时Withoutunconsciousness无意识障碍Noassociatedneurologicalsigns无中枢神经系统症状Spontaneousnystagmuscanbehorizontal,orrotational自发性眼震水平或旋转性Vestibularrecruitment前庭重振现象,Centralvertigo中枢性眩晕,Vertigoisnotsuddennotwhirling缓慢非旋转性Severityisnotsure程度不定Noassociatedcochlearsymptoms无耳部症状“disharmonic”vestibularsyndrome前庭反应分离Symptomsnotrelatedwithbodyandheadposition与体位或头位无关Symptomslastseveraldaystomonths持续时间长，数天到数月Somepatientswithunconsciousness可有意识丧失Associatedneurologicalsigns:diplopia复视,dysarthria吞咽障碍,dysphagia发音障碍,numbness麻木,paresis偏瘫,ataxia共济失调Spontaneousnystagmusisusuallyvertical自发眼震通常是垂直方向Vestibulardecruitmentorresponsedelamination前庭减振或反应分离,多学科协作（multidisciplinaryteamwork,MDT）,耳鼻咽喉科神经科心血管科骨科儿科心理科康复科.,Meniresdisease梅尼埃病,Definition定义,Idiopathicendolymphatichydropsofinnerearcharacterizedbyspontaneousattacksofvertigo,withassociatedfluctuatingsensorineuralhearingloss,tinnitusandauralfullness.一种特发性膜迷路积水的内耳病，表现为反复发作性眩晕，波动性感音神经性听力损失，耳鸣和（或）耳胀闷感。,History历史,1861ProsperMenieredescribedclassicsymptomsandattributedtolabyrinth法国医生首先报道此病1871KnappintheorizeddilatationofmembranousLabyrinth1938HallpikeandPortmanconfirmedendolymphatichydropsviatemporalbonehistology1972AAOdefinedthediseasecriteria，1985AAO-HNSrevisedthedefinitionandestablishesreportingprotocols，1995AAO-HNSrevisedthedefinitionandreportingprotocolsagain.美国耳鼻咽喉-头颈外科协会制定的梅尼埃病诊断治疗指南.2015-BaranySocietymakethediseasecriteia.巴拉尼协会制定了梅尼埃病的诊治标准。（中国-2006年贵阳标准）,Incidence发病率,15/100,000,490Y,4050YMale/Female1:1-1:1.3Unilateral单侧first,41.5%bilateralinvolvement双侧受累occurs(Kitahara),Etiology病因,Idiopathic自发性(thecauseisunkrown)Congenitalabnormality先天性解剖结构异常（Endolymphaticductobstructionandendolymphmalabsorption内淋巴管阻塞与内淋巴吸收障碍）Immunological-immunecomplexdeposition免疫反应学说Innerearischemia内耳缺血学说Inflammationandtrauma炎症与外伤Endocrineandautonomicnervesdisorder内分泌障碍自主神经功能紊乱Geneticfactors遗传学说,Pathophysiology病理生理,endolymphatichydrops膜迷路积水,Normalmembranouslabyrinth正常的膜迷路,DilatedmembranouslabyrinthinMenieresdisease(Hydrops)肿胀的膜迷路,Pathophysiology病理生理,Endolymphatichydropsleadstodistortionofmembranouslabyrinth内淋巴积水导致膜迷路变形Reisnersmembranecanbeseenbulgingintothescalavestibuliinsomehistologicstudies前庭膜推向前庭阶Microruptures-endolymphandperilymphmixionsimbalancebiochemicaldisorder-mayleadtoepisodicattacks膜迷路破裂导致症状出现,Clinicalpresentation临床表现,typicalsymptoms典型症状(4features特征)Recurrentattacksofspontaneousvertigo(96.2%)反复自发性眩晕发作:severalminutesorhoursFluctuatingandprogressivehearingloss(87.7%)波动性、渐进性听力下降:diplacusis复听Tinnitus(91.1%)耳鸣Auralfullness耳胀闷感,Examination检查,Vestibularfunctiontest前庭功能检查:Nystagmus自发、位置性眼震Calorictest变温试验:normalorloss(canalparasis半规管麻痹）cVEMP/oVEMP（颈性/眼性前庭诱发肌源性电位）Audiometry听力学检查:Sensorineuralhearingloss感音神经性聋Suprathresholdaudiometry阈上功能检查(recruitment重振)Glyceroltest甘油试验：Audiogram/ECochG/OAE(50-60%)ECochG耳蜗电图:-SP/AP0.4(20-70%)Imaging影像学检查:Temporalbone颞骨CTEndolymphatic膜迷路MRI成像,Diagnosiscriteria诊断标准(2006,guiyang贵阳),Definitediagnosiscriteria确诊标准:Vertigo眩晕Spontaneous自发性,lasting20minutestoseveralhours持续20分钟至数小时(20min12h)Recurrent,musthave2,2episodes反复发作2次或2次以上Vegetativedisordersandimbalance,nounconsciousness可有植物神经紊乱和平衡障碍，没有意识丧失Fluctuatingprogressivesensorineuralhearingloss波动性渐进性感音神经性聋Audiometricallydocumentedhearinglossonatleastonce至少1次测听为感音神经性听力下降Intermittentorpersistenttinnitusand/orauralfullness间歇性或持续性耳鸣、耳胀满感Othercasesexcluded排除其他引起眩晕的疾病,Diagnosiscriteria诊断标准(2006,guiyang贵阳),Possiblediagnosiscriteria可疑诊断标准:vertigoepisodesonlyonce,Audiometrydocumentedsensorineuralhearingloss，withtinnitusandfullness仅一次眩晕发作，纯音测听为感音神经性听力损失，伴耳鸣和耳胀闷感。vertigoepisodes2or2lasting20mintoseveralhours,nohearingloss,notinnitusandfullness发作性眩晕2次或2次以上，每次持续20分钟至数小时，听力正常，不伴耳鸣和耳胀闷感。(20min24h)Fluctuatinglowerfrequencysensorineuralhearingloss,withrecruitment,novertigoepisodes波动性低频感音性听力损失，可出现重振，无眩晕发作。haveaboveoneofthem符合以上任何一条(进一步-Glyceroltest甘油试验,ECochG耳蜗电图,OAE耳声发射,Vestibularfunctiontest前庭功能检查),Differentialdiagnosis鉴别诊断,Benignparoxysmalpositionalvertigo良性阵发性位置性眩晕Vestibularneuronitis前庭神炎Drug-relatedvertigo前庭药物中毒Labyrinthitis迷路炎Suddenhearingloss突发性聋Ramsay-Huntsyndrome亨特综合征CogansyndromeCogan综合征Delayedendolymphatichydrops迟发性膜迷路积水Perilymphfistula外淋巴瘘Headtrauma头部外伤Acousticneuroma听神经瘤Superiorsemicircularcanaldehiscence上半规管裂Posteriorcirculationischemia后循环缺血.,Treatment治疗,Principle:individualtherapy个性化综合治疗,regulateautonomicnerve,improveinnerearmirocirculation,relieveendolymphatichydrops调节自主神经功能，改善内耳微循环，减轻膜迷路积水Education:saltfreediet低盐饮食，nicotine,alcohol-withdrawal避免烟酒Medicaltherapy药物治疗Meniettpulsetherapy低压脉冲治疗Hyperbaricoxygen高压氧Surgerytherapy手术治疗Vestibularrehabilitationtherapy前庭康复治疗?(VRT),Medicaltherapy药物治疗,Vestibularsuppression前庭抑制剂:theohydramine苯海拉明,diphenidol地芬尼多Anticholinergic抗胆碱能药:anisodamine山良菪碱,scopolamine东良菪碱VasodilatorsandCalciumchannelblockers血管扩张及钙离子拮抗剂:batahistine倍他司汀,nimodipine尼莫地平,sibelium西比灵Diuretic(uretic)利尿脱水:chlorthalidone氯噻酮,isosorbid硝酸异山梨酯,surgerytherapy外科治疗,Nondestructiveprocedure非破坏性:Intratympanicsteroids经鼓膜注射类固醇Endolymphaticsacsurgery内淋巴囊手术：decompression减压orshunt分流drainage排水removalofsac(controversial有争议)Cervicalsympatheticnerveamputation颈交感神经切断术,Destructiveprocedure破坏性：intratympanicablationperfusion(Gentamycin)经鼓膜破坏性药物灌注vestibularnervesection前庭神经切断Canalpluggingprocedure半规管阻塞Labyrinthectomy迷路切除,（半年）1YmedicaltherapyIntratympanic-surgerytherapy（药物治疗1年以上无效，考虑鼓室灌注和手术治疗,BenignParoxysmalPositionalVertigo(BPPV)良性阵发性位置性眩晕,Definition定义,Adisordercharacterizedbybriefattacksofvertigo,withassociatednystagmus,provokedbycertainchangeinheadpositions.头部运动到某一特定位置时诱发的短暂眩晕伴随特殊的眼震。Benign:notrelatedtoacentralnervoussystemtumor,selflimitedcourse有自限性的周围性前庭病变Paroxysmal:suddenandepisodic突发，阵发性Positional:initiatedbycertainheadpositions特定头位诱发Vertigo:anillusionofmovement眩晕,History病史,Gettinginoroutofbed,rollingoverinbed起床躺下，左右翻身,Briefattacksofvertigo短暂眩晕发作,BasicAnatomy解剖基础,Fivevestibularendorgans:2otolith耳石器(utricle椭圆囊andsacule球囊)and3semicircularcanals半规管,Otoconia耳石:calciumcarbonatecrystals碳酸钙结晶,Fallfromtheurtricleenteroneofthesemicircularcanal从椭圆囊进入半规管,Pathophysiology病理生理,Cupulolithiasis嵴帽结石otoconia耳石intheutricle椭圆囊breaklooseandadhere粘附tothecupula嵴顶ofthesemicircularcanal,Canalithiasis管石症-otoconiaarefreefloating漂浮inthesemicircularcanal;whentheheadmovesintoaprovokingposition,theotoconiasinkintothemostdependentpositioninthecanal,causingendolymph内淋巴tomove,Causes病因,原发/继发Idiopathic自发(thecauseisunknown,5070%)Headtrauma头部外伤(717%)Infection感染(viralneuronitis病毒性神经炎)Degenerationoftheperipheralendorgan前庭终末器官老化Surgicaldamagetothelabyrinth手术损伤迷路Occlusionoftheanteriorvestibularartery前庭前动脉堵塞,Classification分类,Posteriorsemicircularcanal(mostcommon)后半规管来源(最多见)Horizontalsemicircularcanal水平半规管来源（其次