梅尼埃病上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科上海交通大学医学院耳科学研究所上海交通大学医学院耳鼻咽喉科学系杨军定

梅尼埃病上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科上海交通大学医学院耳科学研究所上海交通大学医学院耳鼻咽喉科学系杨军

定义 • 以发作性眩晕、波动性耳聋、耳鸣、耳内闷胀感为主要症状的疾病。

历史 1861 – Prosper Meniere叙述了其典型症状，将此病病因归因于迷路 1871 – Knappin膜迷路膨胀理论 1938 – Hallpike 和Portmann通过颞骨组织学证实与内淋巴积液有关 1972 – AAOO 定义此病标准 1985 – AAO-HNS修正定义并建立报告草案 1995 – AAO-HNS再次修正定义和报告草案

生理学外淋巴位于前庭阶和鼓阶与脑脊液成分类似高Na+, 低 K+ 内淋巴位于中阶与细胞内液成分类似低Na+ 高 K+ 由血管纹产生膜迷路分离此间隔无压力差别

病理生理学内淋巴积液导致膜迷路变形在一些组织学研究中Reisner’s 膜突入前庭阶？

病理生理学内淋巴积液背后的理论内淋巴管/囊阻塞内淋巴管/囊发育不全内淋巴吸收功能改变内淋巴生成改变自身免疫损伤血管源性病毒病因学

诊断

AAO-HNS CHE 1985 Meniere’s is diagnosed by Vertigo Spontaneous, lasting minutes to hours Recurrent, must have more than 1 episode Associated with nystagmus Hearing loss Fluctuating sensorineural Low-frequency or flat Tinnitus Vertigo treatment reporting standard 0 = Complete control 1 -40 = Substantial control 41 -80 = Limited control 81 -120 = Insignificant control > 120 = Worse Hearing treatment reporting standard Avg spells/month post-treatment (24 mon recommended) Avg spells/month pre-treatment (6 mon recommended) PTA reported 500, 1000, 2000, 3000 k. Hz If multiple pre and post levels are available, the worst is always used PTA is considered improved / worse if a 10 d. B difference is noted SDS is considered improved / worse if a 15% difference is noted x 100 = Control Level

AAO-HNS CHE 1995 Meniere’s is diagnosed by Vertigo Spontaneous, lasting minutes to hours Recurrent, must have 2 episodes > 20 min. Nystagmus during episodes Hearing loss Avg (250, 500, 1000) 15 d. B < Avg (1000, 2000, 3000) or Avg (500, 1000, 2000, 3000) 20 d. B > than other ear For bilateral disease Avg (500, 1000, 2000, 3000) > 25 d. B in the studied ear Tinnitus No guidelines Aural pressure No guidelines

AAO-HNS CHE 1995 Possible Meniere's disease Episodic vertigo of the Meniere's type without documented hearing loss, or Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium but without definitive episodes Other causes excluded Probable Meniere's disease One definitive episode of vertigo Audiometrically documented hearing loss on at least one occasion Tinnitus or aural fullness in the treated ear Other causes excluded Definite Meniere's disease Two or more definitive spontaneous episodes of vertigo 20 minutes or longer Audiometrically documented hearing loss on at least one occasion Tinnitus or aural fullness in the treated ear Stage Other cases excluded See staging chart 1 1 Certain Meniere's disease PTA Definite Meniere's disease, plus histopathologic confirmation See staging chart <=25 2 26 -40 3 41 -70 4 >70

AAO-HNS CHE 1995 Functional Level Scale Regarding my current state of overall function, not just during attacks (check the ONE that best applies): 1. My dizziness has no effect on my activities at all. 2. When I am dizzy I have to stop what I am doing for a while, but it soon passes and I can resume activities. I continue to work, drive, and engage in any activity I choose without restriction. I have not changed any plans or activities to accommodate my dizziness. 3. When I am dizzy, I have to stop what I am doing for a while, but it does pass and I can resume activities. I continue to work, drive, and engage in most activities I choose, but I have had to change some plans and make some allowance for my dizziness. 4. I am able to work, drive, travel, take care of a family, or engage in most essential activities, but I must exert a great deal of effort to do so. I must constantly make adjustments in my activities and budge my energies. I am barely making it. 5. I am unable to work, drive, or take care of a family. I am unable to do most of the active things that I used to. Even essential activities must be limited. I am disabled. 6. I have been disabled for 1 year or longer and/or I receive compensation (money) because of my dizziness or balance problem.

AAO-HNS CHE 1995 Reporting Results of Treatment: Vertigo treatment reporting standard A=0 B = 1 -40 C = 41 -80 D = 81 -120 E > 120 F = Secondary treatment required due to disabling vertigo Hearing treatment reporting standard PTA reported 500, 1000, 2000, 3000 k. Hz If multiple pre and post levels are available, the worst is always used PTA is considered improved / worse if a 10 d. B difference is noted SDS is considered improved / worse if a 15% difference is noted

鉴别诊断迷路炎有中耳炎病史中耳内耳手术外伤史瘘管试验阳性耳药物中毒有耳毒性药物应用史如链霉素、庆大霉素等

前庭神经元炎有上感病史、不伴耳蜗症状眩晕>2周听神经瘤单侧进行性耳聋耳鸣眩晕轻可伴三叉神经症状突发性耳聋突发严重感音性聋，单耳，伴或不伴眩晕眩晕能恢复，听力恢复慢，或不能完全恢复

椎基底动脉供血不足可和头位、活动有关可伴有其他颅神经症状椎基底动脉MRA异常位置性眩晕头处于某一个特定位置时出现眩晕持续约数十秒不伴耳鸣和耳聋。

药物治疗

血管扩张剂减少内耳局部缺血，改善内淋巴代谢倍他司汀：最常用，可减少眩晕抗组胺药：作用机制存在争议 Meta分析 (2004) – 仅一项Grade B、四项Grade C 研究, 无一项的结果令人信服

利尿药 Klockoff and Lindblom (1967) HCTZ vs. 安慰剂在 30位患者中的研究表明利尿疗法有效 Klockoff (1974) 76%患者氯噻酮治疗（疗程>7年）有效 Shinkawa/Kimura (1986) 内淋巴积液的动物模型上无明显疗效 Ruckenstein (1991) 分析表明疗效无有意义改变安慰剂有效>50%

利尿药渗透性利尿药(尿素, 甘油) 口味不佳部分患者中症状持续减轻，但疗效仅持续数小时耳蜗电图描记：SP: AP 改变乙酰唑胺静脉给药加重积液和听力损失 (Brookes) 口服给药改善积液 (Shinkawa) 副作用：代谢性酸中毒和肾结石 (Brookes)

Meniett 装置经中耳腔的“Micropressure” 治疗 1999年 FDA 认证为 II类装置自我治疗，TID 每次治疗 3个 1分钟间歇应用，压力范围： 0 -20 cm H 20 需要鼓膜通气管

Meniett 装置 Gates GA, Green JD. (2002) 设计：前瞻性研究, 10 例, 3 -10月眩晕 90% 完全控制 10% 减轻一半问题置管, 耳漏, 阻塞, 排出治疗停止后复发 Densert and Sass (2001) 设计：前瞻性, 37例, 2年眩晕控制 51% 改善41% 失败 8%

鼓室内给药治疗

鼓室内地塞米松 Author Med Protocol Sennaroglu Dex 1 mg/ml Hirvonen Dex 3 doses in 1 16 mg/ml wk 17 Barrs Dex 4 mg/ml 21 Qod x 3 mon 2 x/wk x 1 mon Pts 24 A 41% A&B Other No change in tinnitus 72% or HL No change in tinnitus 76% or HL Barrs Arriaga Dex 8 mg/ml Silverstein Dex 8 mg/ml IT gelfoam x 1 Qd x 3 days 34 3 month data 43% Dex 10 mg/ml Qwk x 4 -6 wks 52% 6 month data 32% 2 year data 15 No improvement in hearing 20 No improvement in hearing or tinnitus

糖皮质激素之作用抑制免疫介导的炎性反应增加耳蜗血流增进耳蜗稳态

鼓室内地塞米松优点直接用药内耳内高浓度较少副作用糖尿病、高血压、溃疡患者均可用不会导致听力损失疾病早期疗效显著

化学性迷路切除 Fowler (1948) and Schuknecht (1957) 确定氨基糖甙疗效原来：链霉素所有病人眩晕控制所有病人重度聋现在：庆大霉素理论治疗目标血管纹的暗细胞半规管的半月板大剂量损伤耳蜗毛细胞

鼓室内庆大霉素庆大霉素首选：作用位点位于血管纹副作用: 暂时性平衡失调，眼球震颤听力损失耳鸣方法注射放置明胶海绵 Microwick 鼓室内多次给药小剂量每周一次一日多次连续给药滴定法

手术治疗

内淋巴囊手术 1927年法国Portmann首创 1954年Yamakawa和Naito改进，内淋巴囊和脑脊液之间分流 Willium House普及—手术显微镜和现代耳科技术，切开引流处放置Teflon管 1966年Shea将Teflon引流条置于内淋巴囊和乳突之间 1975年Stahle和1976年Arenberg内耳活瓣—内淋巴囊内硅树脂单向活瓣，延伸到乳突腔

内淋巴囊手术方法内淋巴囊减压术内淋巴囊分流术乳突分流蛛网膜下腔分流

内淋巴囊手术适应症低频听力损失 30 d. B以下，发作型梅尼埃病，保守治疗无效难治性梅尼埃病双耳梅尼埃病

内淋巴囊手术禁忌症患耳为唯一听力耳（慎用）中晚期患者，听力损失严重严重内耳畸形、Mondini畸形、大前庭导水管综合症中耳炎

前庭神经切断术手术径路乙状窦后径路迷路后径路颅中窝径路迷路下径路

前庭神经切断术—手术适应症严重眩晕，发作频繁，患耳听力尚好，保守治疗超过6 个月或内淋巴囊手术无效迷路破坏后仍有眩晕，并有残留迷路功能外伤性、突发性聋、中耳手术所致的持续性眩晕，保守治疗无效复发性前庭神经炎

前庭神经切断术—禁忌症双侧前庭系统外周病变前庭系统中枢病变慢性中耳乳突炎 60岁以上年老体弱者

前庭神经切断术—并发症感音神经性聋—术中损伤半规管、蜗神经、内听动脉 CSF漏—鼓窦入口、内听道口、乳突小气房和术腔内气房未封闭严密面瘫颅内感染乙状窦、岩静脉损伤严重头痛

前庭神经切断术—疗效眩晕控制率90 -95%

迷路切除术—原则完全切除前庭外周感受器、支配感受器的外周神经纤维，阻断前庭神经冲动的传入

迷路切除术—径路外耳道径路迷路切除术乳突径路迷路切除术

迷路切除术—适应症内淋巴囊手术后眩晕持续存在或术后复发，患耳为严重感音神经性聋或全聋，PTA>80，SDS<20%，对侧听力正常中耳炎或镫骨术后、颞骨骨折等致眩晕严重者

迷路切除术—禁忌症患耳有实用听力患侧为唯一听力耳双耳病变

迷路切除术—手术要点乳突轮廓化、辨认三个半规管彻底清除膜迷路，包括椭圆囊斑和球囊斑前庭腔用肌肉或脂肪填塞，防止前庭末梢形成外伤性神经瘤勿穿破前庭内壁的球囊隐窝，防止CSF漏

迷路切除术—并发症 CSF漏—穿破前庭内壁或进入内听道面瘫全聋眩晕—迷路切除不彻底或形成外伤性神经瘤脑膜炎

迷路切除术—疗效眩晕控制率96. 8 -99%

Vestibular Suppressants Overview 急性期治疗长期稳定者非侵袭性治疗 Alternative options Alternative Therapies Meniett Herbal Hypnosis ? Diuretics Salt Restriction Vasodilators ? Water Therapy 非破坏性治疗药物： IT激素手术：乳突分流破坏性治疗药物： IT 庆大霉素手术： Surgical 前庭神经切断迷路切除 Intratympanic Steroid Therapy Mastoid Shunt Intratympanic Gentamicin Therapy Surgical Ablation Nerve Section Labyrinthectomy

梅尼埃病 上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科 上海交通大学医学院耳科学研究所 上海交通大学医学院耳鼻咽喉科学系 杨 军 定

梅尼埃病上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科上海交通大学医学院耳科学研究所上海交通大学医学院耳鼻咽喉科学系杨军定