Department of Otolaryngology, Bajcsy-Zsilinszky Hospital, Budapest, Hungary.
Eur Arch Otorhinolaryngol. 2013 Mar;270(3):793-804. doi: 10.1007/s00405-012-2126-0. Epub 2012 Jul 29.
To review our current knowledge of the pathologic bone metabolism in otosclerosis and to discuss the possibilities of non-surgical, pharmacological intervention. Otosclerosis has been suspected to be associated with defective measles virus infection, local inflammation and consecutive bone deterioration in the human otic capsule. In the early stages of otosclerosis, different pharmacological agents may delay the progression or prevent further deterioration of the disease and consecutive hearing loss. Although effective anti-osteoporotic drugs have become available, the use of sodium fluoride and bisphosphonates in otosclerosis has not yet been successful. Bioflavonoids may relieve tinnitus due to otosclerosis, but there is no data available on long-term application and effects on sensorineural hearing loss. In the initial inflammatory phase, corticosteroids or non-steroidal anti-inflammatory drugs may be effective; however, extended systemic application may lead to serious side effects. Vitamin D administration may have effects on the pathological bone loss, as well as on inflammation. No information has been reported on the use of immunosuppressive drugs. Anti-cytokine targeted biological therapy, however, may be feasible. Indeed, one study on the local administration of infliximab has been reported. Potential targets of future therapy may include osteoprotegerin, RANK ligand, cathepsins and also the Wnt-β-catenin pathway. Finally, anti-measles vaccination may delay the progression of the disease and potentially decrease the number of new cases. In conclusion, stapes surgery remains to be widely accepted treatment of conductive hearing loss due to otosclerosis. Due to lack of solid evidence, the place of pharmacological treatment targeting inflammation and bone metabolism needs to be determined by future studies.
回顾耳硬化症中病理骨代谢的现有知识,并讨论非手术、药物干预的可能性。耳硬化症被怀疑与麻疹病毒感染缺陷、局部炎症和人耳壳骨恶化有关。在耳硬化症的早期,不同的药物可能会延迟疾病的进展或防止进一步恶化和随之而来的听力损失。尽管有效的抗骨质疏松药物已经问世,但氟化物钠和双膦酸盐在耳硬化症中的应用尚未成功。类黄酮可能会缓解耳硬化症引起的耳鸣,但尚无关于长期应用和对感音神经性听力损失影响的数据。在初始炎症阶段,皮质类固醇或非甾体抗炎药可能有效;然而,长期全身应用可能会导致严重的副作用。维生素 D 给药可能对病理性骨丢失以及炎症有影响。尚未报告免疫抑制药物的使用情况。然而,针对细胞因子的靶向生物治疗可能是可行的。事实上,已经有一项关于英夫利昔单抗局部给药的研究报告。未来治疗的潜在靶点可能包括护骨素、RANK 配体、组织蛋白酶以及 Wnt-β-连环蛋白通路。最后,抗麻疹疫苗接种可能会延迟疾病的进展,并可能减少新发病例的数量。总之,镫骨手术仍然是治疗耳硬化症引起的传导性听力损失的广泛接受的方法。由于缺乏确凿的证据,针对炎症和骨代谢的药物治疗的地位需要未来的研究来确定。