上海交通大学医学院附属新华医院耳鼻咽喉-头颈外科 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 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 内淋巴 膜迷路分离此间隔 位于前庭阶和鼓阶 与脑脊液成分类似 高Na+, 低 K+ 位于中阶 与细胞内液成分类似 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 PTA reported 500, 1000, 2000, 3000 kHz If multiple pre and post levels are available, the worst is always used PTA is considered improved / worse if a 10 dB difference is noted SDS is considered improved / worse if a 15% difference is noted Avg spells/month post-treatment (24 mon recommended) x 100 = Control Level Avg spells/month pre-treatment (6 mon recommended)

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

AAO-HNS CHE 1995 Possible Meniere's disease Probable 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 Other cases excluded  See staging chart Certain Meniere's disease Definite Meniere's disease, plus histopathologic confirmation Stage PTA 1 <=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): My dizziness has no effect on my activities at all. 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. 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. 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. 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. 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 kHz If multiple pre and post levels are available, the worst is always used PTA is considered improved / worse if a 10 dB 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 听神经瘤 突发性耳聋 有上感病史、不伴耳蜗症状 眩晕>2周 单侧进行性耳聋耳鸣 眩晕轻 可伴三叉神经症状 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 位置性眩晕 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 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 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) Klockoff (1974) 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) 乙酰唑胺 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 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 1999年 FDA 认证为 II类装置 自我治疗，TID 每次治疗3个1分钟 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 H20 需要鼓膜通气管

Meniett 装置 Gates GA, Green JD. (2002) Densert and Sass (2001) 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 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 Pts A A&B Other Sennaroglu Dex 1mg/ml Qod x 3 mon 24 41% 72% No change in tinnitus or HL Hirvonen Dex 16mg/ml 3 doses in 1 wk 17 76% Barrs Dex 4mg/ml 2x/wk x 1mon 21 52% 3 month data 43% 6 month data Dex 10mg/ml Qwk x 4-6 wks 34 32% 2 year data Arriaga Dex 8mg/ml IT gelfoam x 1 15 No improvement in hearing Silverstein Qd x 3 days 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 现在：庆大霉素 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 暂时性平衡失调，眼球震颤 听力损失 耳鸣 方法 庆大霉素首选：作用位点位于血管纹 副作用: 鼓室内多次给药 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 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

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30dB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%

Overview 急性期治疗 长期稳定者 非破坏性治疗 破坏性治疗 非侵袭性治疗 Alternative options 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