耳鼻咽喉头颈外科学教学 2017刘绮明耳聋眩晕课件

,Qiming Liu刘绮明 Dept. of Otorhinolaryngology 2nd Affiliated Hospital of Guangzhou medical university 广州医科大学附属第二医院耳鼻咽喉科教研室,,Hearing Loss耳聋,Vertigo眩晕,Tinnitus耳鸣,hearing,Keeping balance,耳有什么功能？,人是如何听到声音？,Bone conduction骨传导,Air conduction空气传导,Hearing loss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,The lesions in the auditory conductive pathway cause total or partial inability to hear sound.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。,Hearing loss situation概况,听力残疾（hearing disability）：各种原因导致双耳不同程度的永久性听力障碍（hearing handicap） ，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,3 basic types: (which parts of hearing pathway is affected按病变部位分) Conductive Hearing Loss -CHL传导性 Sensorineural Hearing Loss (sensory,neural,central) - SNHL感音神经性 Mix Hearing Loss - MHL混合性,Degree分级 （WHO，1997年 ）,Mild loss轻度: 2640dBHL Moderate loss中度: 4160dBHL Severe loss重度: 6180dBHL Profound loss极重度: 81dBHL,Audiogram听力图,“threshold”听阈is the softest intensity that sound is perceived. (0.512k4kHz) “dBHL”is decibels 分贝hearing level,如何估计听力损失程度？,Conductive Hearing Loss传导性聋,1、炎症 2、外伤 3、阻塞 4、先天畸形 5、听骨链病变,Sensorineural Hearing Loss感音神经性聋,。,1、Sensory hearing loss感音性,2、Neural hearing loss神经性,Hair cell毛细胞,Spiral ganglion螺旋神经节,Stria vascularis血管纹,3、Central hearing loss中枢性,Mix hearing loss 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,Otoacoustic emission（OAE）耳声发射,Auditory brainstem response （ABR）听性脑干反应,Tuning fork音叉检查,Imaging 影像学检查,常见的感音神经性聋,1、Nonhereditary Hearing Loss非遗传性聋 Sudden Hearing Loss突发性聋 Ototoxicity hearing loss 中毒性聋 Aged related Hearing Loss 老年性聋 Noise-induced Hearing Loss噪音性聋 2、Hereditary Hearing Loss遗传性聋,Sudden Hearing Loss (SHL)突发性聋,Definition定义: Sudden happen greater than 20 dB hearing reduction at least two consecutive frequencies in 72 hours or less.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: Vascular lesion 血管病变 Virus Infections病毒感染 Autoimmune自身免疫 Round or oval window membrane rupture窗膜破裂 Neurological neoplasm 神经系统新生物,SHL Clinical Characteristics突发性耳聋临床特征,Sudden happen突发, no fluctuating无波动性, moderate to severe loss中重度聋 Tinnitus耳鸣 Vertigo眩晕 No other cranial nerves lesion无其他颅神经损害 Unilateral 单侧多见 Other presentation：aural fullness耳堵塞感 Tends to recover spontaneously 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80dBHL 4、全聋型81dBHL,SHL Differential diagnosis 鉴别诊断,Meniere,s Disease梅尼埃病 Acoustic Neuroma听神经瘤 Functional Hearing Loss功能性聋,Hereditary Hearing Loss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% Autosomal Dominant 常染色体显性1524%, Autosomal Recessive常染色体隐性7585%, X-linked X连锁12%, Mitochondrial线粒体 1%,新生儿聋病易感基因筛查,常见的4个基因9个位点 GJB2(35delG,176_191del16,235delC,299_300delAT) SLC26A4基因（IV37-2AG,2168AG） 线粒体 12S rRNA (MTRNR1): (1555AG,1494CT) GJB3(538 CT),精准医学 Precision Medicine,胚胎植入前遗传学诊断(preimplantation genetic diagnosis,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 Physical finding 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Other related test （serologic 血清学testing, immunologic免疫 testing, genetic 基因testing),Treatment 耳聋治疗,Medical Management药物（针对病因） Hyperbaric oxygen therapy 高压氧 Acupuncture针灸 Surgical treatment手术疗法 Hearing aid助听器验配 Implantable hearing aid植入性助听技术（Cochlea Implant人工耳蜗、Brain stem implants 脑干植入) Gene therapy基因治疗 Stem cell treatment 干细胞治疗,Prevention of SNHL预防 感音神经性聋,Antenatal and perinatal care孕期和围产期保健 Newborn hearing (+genetic基因)screening新生儿听力筛查 Aged hearing care老年人口听力保健 Hearing care and food nutrition and hygiene听力保健与食品营养卫生学 Noise Exposure Avoidance避免噪声（HPD） Ototoxic medications must be administered carefully慎用耳毒性药物,How to protect our ear ? 预防比治疗更重要更有效,Ear care Day爱耳日,Since 2000 3th March (3.3) 2017 theme主题：Prevention,treatment and precise service for hearing impairment and deafness防聋治聋，精准服务,Hearing Aid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,Hearing Aid 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 Body Aid 体佩式(盒式),Hearing Aid 助听器,骨导助听器: BAHA（bone anchor)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）,Middle ear hearing aid implant 人工中耳植入,振动声桥,Cochlea Implant Candidates 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,Auditory center impant 听觉中枢植入,Questions,Classification and degree of hearing loss？耳聋分类分级？ What are the common SNHL？常见的感音神经性聋？,Tinnitus耳鸣,Definition定义,tinnitus “tinnere” (Latin)拉丁词 It is commonly described as a perception of sound in the ear or in the brain that is not related to an external acoustic source or electrical stimulation.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% of patients with sudden hearing loss突发性聋 70% with presbyacusis老年性聋 50-90% with noise-induced hearing loss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 Auditory system (peripheral and central)外周和中枢听觉系统病变 Hormonal changes激素水平改变 Other systemic diseases其他系统疾病 Some medications or withdrawal from them一些药物或撤药后反应 Psychological factors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 Eustachian tube abnormal patulous咽鼓管异常开放. Joint abnormalities: temporomandibular joint disorder颞颌关节病变.,Pathophysiology病理生理 (neurophysiologic mode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 Neurotology and ENT神经系统检查和耳鼻咽喉科检查 Cochlear and vestibular function耳蜗及前庭功能评价 Tinnitus test耳鸣检查 Imaging: CT/MRI/functional-MRI Psychological evaluation精神心理学评价 Visual analogue scale （VAS）视觉模拟评分 Tinnitus handicap inventory（THI）耳鸣致残量表 State trait anxiety inventory （STAI）焦虑特质量表 Quality of life questionaire 生活质量调查表 耳鸣简易评分-中国.刘蓬,Treatment治疗,Tinnitus combined management (TCM)can be more benefical than a single therapy/ Individual therapy 综合治疗比单一治疗有效,个体化治疗原则 Etilogical treatment病因治疗 Medication (pharmacotherapy)药物治疗 Sound therapy声治疗 Tinnitus retraining therapy (TRT)耳鸣习服 Cognitive behavioral therapy行为认知 Biofeedback生物反馈 Electrical stimulation therapy电刺激治疗 Transcranial magnetic stimulation (TMS)经颅磁刺激 Surgery手术部分体声 tinnitus just live with it? 适应耳鸣,Treatment治疗 (medication药物),Not single没有单一, effective有效, specific特定, secure安全, and reliable可靠 drugs has yet to be identified. Vasodiator 扩张血管 (niacin烟酸，nalador前列腺素E2) Improve inner ear energy metabolism改善内耳能量代谢 (ATP三磷酸腺苷,coenzyme A辅酶A) Calcium channel blockers 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) Local anesthetics 局部麻醉剂 (lidocaine利多卡因,procaine普鲁卡因) Muscle relaxant 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（acamprosate calciam阿坎酸） Others其他 (ginkgo biloba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。,To cure sometimes, to relief often, to comfort always.,Questions问题,What are tinnitus treatments ？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(International Association of physicians in audiology)国际耳内科医师协会 Barany society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,Anatomy and physiology解剖生理,3 semicircular canals-angular角加速度,2 otolith耳石器-linear线加速度+gravity重力,椭圆囊,球囊,What is vertigo?,Vertigo眩晕 vert (Latin)拉丁词 Vertigo is the sensation of self motion when no self-motion is occurring or the sensation of distorted self-motion during an otherwise normal head movement.（Internal vertigo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。,How to differentiating central and peripheral vertigo? 如何鉴别周围性和中枢性眩晕？,Peripheral vertigo周围性眩晕,Sudden rotating vertigo突发性旋转性 Severe较剧烈 Associated cochlear symptoms (unilateral hearing loss耳聋, tinnitus耳鸣, fullness耳胀闷感) “harmonic” vestibular syndrome前庭反应协调 Symptoms aggravate after moving of the body and head position头位或体位变动时眩晕加重 Last several seconds to hours持续数秒到数小时 Without unconsciousness无意识障碍 No associated neurological signs无中枢神经系统症状 Spontaneous nystagmus can be horizontal, or rotational自发性眼震水平或旋转性 Vestibular recruitment前庭重振现象,Central vertigo中枢性眩晕,Vertigo is not sudden not whirling缓慢非旋转性 Severity is not sure程度不定 No associated cochlear symptoms无耳部症状 “disharmonic”vestibular syndrome前庭反应分离 Symptoms not related with body and head position与体位或头位无关 Symptoms last several days to months持续时间长，数天到数月 Some patients with unconsciousness可有意识丧失 Associated neurological signs : diplopia复视, dysarthria吞咽障碍, dysphagia发音障碍,numbness麻木, paresis偏瘫, ataxia共济失调 Spontaneous nystagmus is usually vertical自发眼震通常是垂直方向 Vestibular decruitment or response delamination前庭减振或反应分离,多学科协作 （multidisciplinary teamwork,MDT）,耳鼻咽喉科 神经科 心血管科 骨科 儿科 心理科 康复科 ,Menires disease 梅尼埃病,Definition定义,Idiopathic endolymphatic hydrops of inner ear characterized by spontaneous attacks of vertigo, with associated fluctuating sensorineural hearing loss, tinnitus and aural fullness. 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,1861 Prosper Meniere described classic symptoms and attributed to labyrinth法国医生首先报道此病 1871 Knappin theorized dilatation of membranous Labyrinth 1938 Hallpike and Portman confirmed endolymphatic hydrops via temporal bone histology 1972 AAO defined the disease criteria，1985 AAO-HNS revised the definition and establishes reporting protocols，1995 AAO-HNS revised the definition and reporting protocols again.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-Barany Society make the disease criteia.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, 4050Y Male/Female 1:1-1:1.3 Unilateral 单侧first, 41.5% bilateral involvement双侧受累 occurs (Kitahara),Etiology病因,Idiopathic自发性(the cause is unkrown) Congenital abnormality 先天性解剖结构异常（Endolymphatic duct obstruction and endolymph malabsorption内淋巴管阻塞与内淋巴吸收障碍） Immunological-immune complex deposition免疫反应学说 Inner ear ischemia 内耳缺血学说 Inflammation and trauma炎症与外伤 Endocrine and autonomic nerves disorder内分泌障碍自主神经功能紊乱 Genetic factors遗传学说,Pathophysiology病理生理,endolymphatic hydrops 膜迷路积水,Normal membranous labyrinth 正常的膜迷路,Dilated membranous labyrinth in Menieres disease (Hydrops) 肿胀的膜迷路,Pathophysiology病理生理,Endolymphatic hydrops leads to distortion of membranous labyrinth内淋巴积水导致膜迷路变形 Reisners membrane can be seen bulging into the scala vestibuli in some histologic studies前庭膜推向前庭阶 Microruptures - endolymph and perilymph mixions imbalancebiochemical disorder-may lead to episodic attacks膜迷路破裂导致症状出现,Clinical presentation临床表现,typical symptoms典型症状(4 features特征) Recurrent attacks of spontaneous vertigo(96.2%)反复自发性眩晕发作 :several minutes or hours Fluctuating and progressive hearing loss(87.7%)波动性、渐进性听力下降: diplacusis复听 Tinnitus(91.1%) 耳鸣 Aural fullness耳胀闷感,Examination检查,Vestibular function test前庭功能检查 : Nystagmus自发、位置性眼震 Caloric test变温试验: normal or loss(canal parasis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 : Sensorineural hearing loss感音神经性聋 Suprathreshold audiometry阈上功能检查( recruitment重振) Glycerol test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: Temporal bone 颞骨CT Endolymphatic膜迷路 MRI 成像 ,Diagnosis criteria诊断标准 (2006,guiyang贵阳),Definite diagnosis criteria确诊标准: Vertigo眩晕 Spontaneous自发性, lasting 20minutes to several hours持续20分钟至数小时(20min12h) Recurrent, must have 2 ,2 episodes反复发作2次或2次以上 Vegetative disorders and imbalance, no unconsciousness可有植物神经紊乱和平衡障碍，没有意识丧失 Fluctuating progressive sensorineural hearing loss波动性渐进性感音神经性聋 Audiometrically documented hearing loss on at least once至少1次测听为感音神经性听力下降 Intermittent or persistent tinnitus and/or aural fullness间歇性或持续性耳鸣、耳胀满感 Other cases excluded排除其他引起眩晕的疾病,Diagnosis criteria诊断标准 (2006,guiyang贵阳),Possible diagnosis criteria可疑诊断标准 : vertigo episodes only once, Audiometry documented sensorineural hearing loss， with tinnitus and fullness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。 vertigo episodes 2 or 2 lasting 20min to several hours,no hearing loss, no tinnitus and fullness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) Fluctuating lower frequency sensorineural hearing loss ,with recruitment ,no vertigo episodes 波动性低频感音性听力损失，可出现重振，无眩晕发作。 have above one of them 符合以上任何一条 (进一步-Glycerol test甘油试验, ECochG耳蜗电图,OAE耳声发射, Vestibular function test 前庭功能检查),Differential diagnosis鉴别诊断,Benign paroxysmal positional vertigo良性阵发性位置性眩晕 Vestibular neuronitis前庭神炎 Drug-related vertigo前庭药物中毒 Labyrinthitis迷路炎 Sudden hearing loss突发性聋 Ramsay-Hunt syndrome 亨特综合征 Cogan syndrome Cogan综合征 Delayed endolymphatic hydrops迟发性膜迷路积水 Perilymph fistula外淋巴瘘 Head trauma头部外伤 Acoustic neuroma听神经瘤 Superior semicircular canal dehiscence上半规管裂 Posterior circulation ischemia后循环缺血 .,Treatment治疗,Principle: individual therapy个性化综合治疗, regulate autonomic nerve, improve inner ear mirocirculation, relieve endolymphatic hydrops 调节自主神经功能，改善内耳微循环，减轻膜迷路积水 Education: salt free diet低盐饮食， nicotine, alcohol-withdrawal避免烟酒 Medical therapy药物治疗 Meniett pulse therapy 低压脉冲治疗 Hyperbaric oxygen高压氧 Surgery therapy手术治疗 Vestibular rehabilitation therapy前庭康复治疗? (VRT),Medical therapy药物治疗,Vestibular suppression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 Vasodilators and Calcium channel blockers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,surgery therapy外科治疗,Nondestructive procedure非破坏性: Intratympanic steroids经鼓膜注射类固醇 Endolymphatic sac surgery内淋巴囊手术： decompression 减压or shunt 分流drainage排水 removal of sac (controversial有争议) Cervical sympathetic nerve amputation颈交感神经切断术,Destructive procedure破坏性： intratympanic ablation perfusion(Gentamycin )经鼓膜破坏性药物灌注 vestibular nerve section前庭神经切断 Canal plugging procedure半规管阻塞 Labyrinthectomy迷路切除,（半年）1Y medical therapy Intratympanic- surgery therapy（药物治疗1年以上无效，考虑鼓室灌注和手术治疗,Benign Paroxysmal Positional Vertigo (BPPV) 良性阵发性位置性眩晕,Definition定义,A disorder characterized by brief attacks of vertigo, with associated nystagmus, provoked by certain change in head positions.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: not related to a central nervous system tumor, self limited course有自限性的周围性前庭病变 Paroxysmal: sudden and episodic突发，阵发性 Positional: initiated by certain head positions特定头位诱发 Vertigo: an illusion of movement眩晕,History病史,Getting in or out of bed, rolling over in bed起床躺下，左右翻身,Brief attacks of vertigo 短暂眩晕发作,Basic Anatomy解剖基础,Five vestibular end organs:2 otolith耳石器(utricle 椭圆囊and sacule球囊) and 3 semicircular canals半规管,Otoconia耳石: calcium carbonate crystals碳酸钙结晶,Fall from the urtricle enter one of the semicircular canal从椭圆囊进入半规管,Pathophysiology病理生理,Cupulolithiasis嵴帽结石otoconia耳石 in the utricle椭圆囊 break loose and adhere粘附 to the cupula嵴顶 of the semicircular canal,Canalithiasis管石症-otoconia are free floating漂浮 in the semicircular canal; when the head moves into a provoking position, the otoconia sink into the most dependent position in the canal, causing endolymph内淋巴 to move,Causes病因,原发/继发 Idiopathic 自发(the cause is unknown ,50 70 %) Head trauma头部外伤(717 %) Infection 感染(viral neuronitis病毒性神经炎) Degeneration of the peripheral end organ前庭终末器官老化 Surgical damage to the labyrinth手术损伤迷路 Occlusion of the anterior vestibular artery前庭前动脉堵塞,Classification分类,Posterior semicircular canal (most common)后半规管来源 (最多见) Horizontal s