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1、耳聋及其防治耳聋及其防治 Deafness and its prevention and treatment 内容提要1、概述、概述2、听觉解剖和生理概要、听觉解剖和生理概要3、定义、分级、定义、分级4、分类及常见病因、分类及常见病因5、预防、预防6、治疗原则及方法、治疗原则及方法1、概述概述n n发病率高;n n对社会经济影响大;n n我国防聋治聋任务艰巨;n n政府关怀、重视。2、听觉解剖和生理概要、听觉解剖和生理概要uu外耳外耳外耳外耳t t耳廓收集声波耳廓收集声波耳廓收集声波耳廓收集声波t t外耳道传递声波及共振外耳道传递声波及共振外耳道传递声波及共振外耳道传递声波及共振uu中耳中耳中
2、耳中耳 t t鼓膜和三个听小骨将声音振动传递至内耳鼓膜和三个听小骨将声音振动传递至内耳鼓膜和三个听小骨将声音振动传递至内耳鼓膜和三个听小骨将声音振动传递至内耳t t中耳的放大器效应中耳的放大器效应中耳的放大器效应中耳的放大器效应uu内耳内耳内耳内耳t t将声音的振动信号转变为电信号，经由听神经传送至中将声音的振动信号转变为电信号，经由听神经传送至中将声音的振动信号转变为电信号，经由听神经传送至中将声音的振动信号转变为电信号，经由听神经传送至中枢听觉系统，产生枢听觉系统，产生枢听觉系统，产生枢听觉系统，产生“ “听觉听觉听觉听觉” ”3、定义、分级定义、分级n nDefinitionDefini
3、tion：n nHearing lossHearing loss, , hearing impairmenthearing impairment, , deafnessdeafness: : Children and Adults who are deaf are those Children and Adults who are deaf are those who cannot hear or understand conversational who cannot hear or understand conversational speech under normal circumst
4、ances. speech under normal circumstances. n nDeafDeaf: Deaf is a total lack of hearing, Deafness : Deaf is a total lack of hearing, Deafness has connotations of discriminationhas connotations of discrimination耳聋分级耳聋分级Degrees of hearing lossn n轻度耳聋轻度耳聋2640dBn n中度耳聋中度耳聋4155dBn n中重度聋中重度聋 5670dBn n重度耳聋重
5、度耳聋7190dBn n极重度耳聋极重度耳聋90dB4、分类及常见病因分类及常见病因 耳聋的分类耳聋的分类 按耳聋病发的时间分类按耳聋病发的时间分类: antenatal pre- birth hearing loss antenatal pre- birth hearing loss inheritanceinheritance1.1.先天性耳聋：出生以前的耳聋先天性耳聋：出生以前的耳聋先天性耳聋：出生以前的耳聋先天性耳聋：出生以前的耳聋 2.2.后天性耳聋：出生以后的耳聋后天性耳聋：出生以后的耳聋后天性耳聋：出生以后的耳聋后天性耳聋：出生以后的耳聋 postnatal after birt
6、h hearing losspostnatal after birth hearing loss按耳聋性质分类按耳聋性质分类:n n传导性耳聋（传导性耳聋（传导性耳聋（传导性耳聋（conductive hearing lossconductive hearing loss）n n感音神经性耳聋（感音神经性耳聋（感音神经性耳聋（感音神经性耳聋（sensorineural hearing losssensorineural hearing loss） 感音性（感音性（感音性（感音性（sensory deafnesssensory deafness），或耳蜗性），或耳蜗性），或耳蜗性），或耳蜗性（c
7、ochlearcochlear） 神经性（神经性（神经性（神经性（nervous deafnessnervous deafness），或蜗后性），或蜗后性），或蜗后性），或蜗后性（retrocochlearretrocochlear）n n混合性聋（混合性聋（混合性聋（混合性聋（mixed hearing lossmixed hearing loss）传导性耳聋传导性耳聋 Conductive hearing lossDefinition:Definition:t tany interruption of passageany interruption of passage of acoust
8、ic of acoustic energy betweenenergy between pinna and oval windowpinna and oval window. .t tLoss of hearing that originates in the Loss of hearing that originates in the outer or middle ear.outer or middle ear.n nExternal auditory canalExternal auditory canaln nEardrumEardrumn nMiddle earMiddle earn
9、 nAuricleAuricleuuMicrotia/AnotiaMicrotia/Anotian nExternal auditory canalExternal auditory canaluuAtresia/StenosisAtresia/StenosisuuObstruction:Obstruction:t tCerumenCerument tForeign BodyForeign Bodyt tOsteoma/NeoplasmOsteoma/NeoplasmuuInfections/Otitis ExternaInfections/Otitis Externa耳廓和外耳道疾病耳廓和外
10、耳道疾病鼓膜鼓膜 Tympanic Membrane(Eardrum)n nPerforation (hole)n nRetractionn nTympanosclerosis (stiffness)Middle Earn nAbsence of ossicles (middle ear bones)n nStiffness of ossiclesuuOtosclerosisuuTympanosclerosisn nDislocation of ossicles (trauma)n nCholesteatoman nTumors感音神经性耳聋感音神经性耳聋 Sensorineural hear
11、ing lossSensorineural - any damage to the organ of Corti or VIII nerve pathway or auditory cortex (SNHL).Sensorineural Hearing Lossn nSensorySensoryuuNoiseNoiset tNoise-induced Noise-induced hearing losshearing losst tAcoustic traumaAcoustic traumauuMedicationsMedicationsuuInfectionInfectionuuHeredi
12、tary/GeneticHereditary/Geneticn nNeuralNeuraluuTraumaTraumauuDegenerationDegeneration (Presbycusis)(Presbycusis)uuTumorsTumorsn nPresbycusisPresbycusisn nHeredityHeredityn nInfectionsInfectionsn nNoise InducedNoise Inducedn nOtotoxicityOtotoxicityn nMenieres DiseaseMenieres Diseasen nTraumaTrauman n
13、Sudden idiopathic/viralSudden idiopathic/viraln nMetabolic (diabetes Metabolic (diabetes mellitus)mellitus)n nAutoimmuneAutoimmunen nNeoplasm (acoustic Neoplasm (acoustic neuroma)neuroma)先天性耳聋和遗传性耳聋先天性耳聋和遗传性耳聋Congenital hearing loss and hereditary hearing lossn nCongenital hearing lossCongenital hea
14、ring loss implies including hereditary implies including hereditary hearing loss or hearing loss due to other factors present hearing loss or hearing loss due to other factors present either in utero (prenatal) or at the time of birth. either in utero (prenatal) or at the time of birth. n nGenetic f
15、actors cause more than 50% of all incidents of Genetic factors cause more than 50% of all incidents of congenital hearing loss in children. May be autosomal congenital hearing loss in children. May be autosomal dominant or recessive, or X-linked dominant or recessive, or X-linked n nOther causes Oth
16、er causes uuIntrauterine infectionsIntrauterine infectionsuuPrematurity Prematurity uuMaternal diabetes Maternal diabetes uuToxemia during pregnancy Toxemia during pregnancy uuLack of oxygen (anoxia) Lack of oxygen (anoxia) 老年性聋老年性聋（Presbycusis）hearing of old age, the effects of agingn nAge related
17、decline in auditory function.Age related decline in auditory function.n nSpeech discrimination is affected commonly.Speech discrimination is affected commonly.n nPresbycusis can occur because of changes in Presbycusis can occur because of changes in the inner ear, auditory nerve, middle ear, or the
18、inner ear, auditory nerve, middle ear, or outer ear. outer ear. n nSome of its causes are aging, loud noise, Some of its causes are aging, loud noise, heredity, head injury, infection, illness, heredity, head injury, infection, illness, certain prescription drugs, and circulation certain prescriptio
19、n drugs, and circulation problems such as high blood pressure.problems such as high blood pressure.传染病源性聋传染病源性聋（deafness due to infective disease）n nMost common Most common uuAdult SNHL Adult SNHL viral cochleitis viral cochleitisuuChildren SNHL Children SNHL meningitis meningitis H.influenzaH.influ
20、enza supperativesupperative labyrinthitislabyrinthitisn nViral .Viral .uuMump Mump unilateral SNHL unilateral SNHLuuMeasal Measal bilateral SNHL bilateral SNHL药毒性耳聋药毒性耳聋（Ototoxicity） drug-induced hearing loss; ototoxicityn nOtotoxicity Ototoxicity is is the the tendency tendency of of certain certai
21、n therapeutic therapeutic agents agents and and other other chemical chemical substances substances to to cause cause functional functional impairment impairment of of the the inner inner ear, ear, and and especially especially of of the the end end organs organs and and neurons neurons of of the th
22、e eighth eighth cranial cranial nerve.nerve.n n一一一一些些些些治治治治疗疗疗疗药药药药物物物物或或或或其其其其它它它它化化化化学学学学物物物物质质质质能能能能引引引引起起起起内内内内耳耳耳耳功功功功能能能能损害，特别是内耳终器和听神经神经元。损害，特别是内耳终器和听神经神经元。损害，特别是内耳终器和听神经神经元。损害，特别是内耳终器和听神经神经元。耳毒性聋的药物耳毒性聋的药物n n氨基糖甙类抗生素：链霉素、庆大霉素、卡那霉素、新氨基糖甙类抗生素：链霉素、庆大霉素、卡那霉素、新氨基糖甙类抗生素：链霉素、庆大霉素、卡那霉素、新氨基糖甙类抗生素：链霉素
23、、庆大霉素、卡那霉素、新霉素、妥布霉素等霉素、妥布霉素等霉素、妥布霉素等霉素、妥布霉素等n n多肽类抗生素：万古霉素和多粘菌素多肽类抗生素：万古霉素和多粘菌素多肽类抗生素：万古霉素和多粘菌素多肽类抗生素：万古霉素和多粘菌素n n抗肿瘤药：氮芥、順铂等抗肿瘤药：氮芥、順铂等抗肿瘤药：氮芥、順铂等抗肿瘤药：氮芥、順铂等n n利尿类药物：利尿酸、速尿等利尿类药物：利尿酸、速尿等利尿类药物：利尿酸、速尿等利尿类药物：利尿酸、速尿等n n水杨酸类药物水杨酸类药物水杨酸类药物水杨酸类药物n n抗疟药：奎宁等抗疟药：奎宁等抗疟药：奎宁等抗疟药：奎宁等n n砷剂砷剂砷剂砷剂n n酒精中毒、烟草中毒、酒精中毒、
24、烟草中毒、酒精中毒、烟草中毒、酒精中毒、烟草中毒、COCO中毒，磷、苯、砷、铅等中毒中毒，磷、苯、砷、铅等中毒中毒，磷、苯、砷、铅等中毒中毒，磷、苯、砷、铅等中毒噪声性聋噪声性聋Noise-induced hearing lossuuExpose noise for long timeExpose noise for long timeuuTemporary Threshold ShiftTemporary Threshold ShiftuuPermanentt Threshold ShiftPermanentt Threshold ShiftuuDifficulty Difficulty h
25、earing hearing high high frequency frequency sounds : 3-6-kHzsounds : 3-6-kHzuuPoor speech discriminationPoor speech discriminationuuTinnitusTinnitusuu早期脱离噪声环境听力下降经常是可逆的早期脱离噪声环境听力下降经常是可逆的早期脱离噪声环境听力下降经常是可逆的早期脱离噪声环境听力下降经常是可逆的uu极重度听力下降少见极重度听力下降少见极重度听力下降少见极重度听力下降少见Normal Hair CellsDamaged Hair CellsNorm
26、al Hair Cells创伤性聋创伤性聋 Traumatic hearing lossn nTemporal bone fracture uu20% Transverse fracture uuSNHL (labyrinth tear) , facial nerve pulsyn nBarotrauma uuSudden pressure change scoobauuperilymph fistula特发性突聋特发性突聋（Idiopathic sudden hearing loss）n n3 3天天天天或或或或更更更更短短短短时时时时间间间间内内内内发发发发生生生生损损损损失失失失在在在在
27、30dB30dB或或或或以以以以上上上上的的的的感音神经性聋感音神经性聋感音神经性聋感音神经性聋n n通常为单侧发病通常为单侧发病通常为单侧发病通常为单侧发病 n n伴伴伴伴有有有有听听听听力力力力方方方方面面面面的的的的其其其其他他他他症症症症状：耳鸣等状：耳鸣等状：耳鸣等状：耳鸣等n n可可可可能能能能的的的的病病病病因因因因：病病病病毒毒毒毒感感感感染染染染和内耳血供障碍和内耳血供障碍和内耳血供障碍和内耳血供障碍n nSNHI SNHI least least 30 30 dB dB in in 3 3 frequency in 3 dayfrequency in 3 dayn nNo
28、involvement of cranial No involvement of cranial nerves other than the nerves other than the eighth nerveeighth nerven nKnown and unknown Known and unknown cause cause n nIdiopathic Idiopathic uuViral theoryViral theoryuuVascular theoryVascular theoryuuothersothersn n40-70% recover40-70% recover自身免疫
29、性耳聋Autoimmune hearing lossn nThe The hallmark hallmark of of autoimmune autoimmune sensorineural sensorineural or or inner inner ear ear hearing hearing loss loss is is its its rapid, rapid, and and sometimes sometimes fluctuating, fluctuating, progression progression to to hearing hearing loss loss
30、 in in both both ears ears occurring over a period of weeks to months. occurring over a period of weeks to months. n nNo No specific specific tests. tests. The The lymphocyte lymphocyte transformation transformation test test and and Western Western blot blot are are helpful helpful in in suggesting
31、 suggesting autoimmune hearing loss. autoimmune hearing loss. n nCorticosteroids Corticosteroids 全身性疾病全身性疾病 systemicn nHypothyroid Hypothyroid 甲状腺功能减退症甲状腺功能减退症甲状腺功能减退症甲状腺功能减退症uuEffect developmemt of middle & inner earEffect developmemt of middle & inner earuuStria vascularis atrophy , degenerate spi
32、ral Stria vascularis atrophy , degenerate spiral gg. gg. uu25-50% 25-50% SNHL SNHLn nSystemic disease Systemic disease ischemia vessel ischemia vesseln nAutoimmune hearing loss Autoimmune hearing loss SLE SLE n n肾脏疾病肾脏疾病肾脏疾病肾脏疾病n n糖尿病糖尿病糖尿病糖尿病n n高血压和高血脂症高血压和高血脂症高血压和高血脂症高血压和高血脂症n n血液性疾病血液性疾病血液性疾病血液性疾
33、病混合性耳聋混合性耳聋 Mixed hearing lossn主观测听: 语音检测,音叉试验, 纯音测听, 阈上和声场测听n客观测听: 声导抗测试, 电反应测听（听性脑干反应、耳蜗电图、多频稳态测听、40Hz相关电位）, 耳声发射5. 耳聋的诊断耳聋的诊断 测听技术测听技术（Audiometry）Tuning fork testAudiogram行为测听n n基本概念：利用婴幼儿对声音刺激的反应进行听力检测，属主观测听。n n特点：需要专业测试设备和专门的测听技术人员，耗时长。检查时要根据患儿年龄和配合程度选择不同的测试方法n n主要测试手段：行为观察测听（behavioral observa
34、tion audiometry, BOA），视觉强化测听(visual reinforcement audiometry, VRA) ，游戏测听(play audiometry, PA)。5、预防预防（Prevention）n n药物性药物性n n感染性感染性n n噪声性噪声性n n遗传性遗传性n n老年性老年性n n代谢性代谢性6、治疗治疗（Treatment）n nMedical treatmentn nSurgeryn nHearing aidsn nCochlear implantMedical Treatmentn nDisease-specific treatmentuuInfec
35、tionsuuAutoimmune diseasesn nSteroidsHearing aids助听器SurgeryTreat underlying (serious) causeCholesteatomaTM perforationAcoustic neuromaImprove hearingStapedectomyCochlear implantFully implantable hearing aids图52l lCochlear implants A cochlear implant bypasses damaged hair A cochlear implant bypasses damaged hair cells and stimulates the auditory nerve cells and stimulates the auditory nerve directlydirectly. . 听觉和言语训练听觉和言语训练小结小结 （Summary）n n常见传导性聋病因常见传导性聋病因n n常见感音神经性聋病因常见感音神经性聋病因n n耳聋的治疗与康复耳聋的治疗与康复THANKS!
