Corazzi Virginia, Hatzopoulos Stavros, Bianchini Chiara, Skarżyńska Magdalena B, Pelucchi Stefano, Skarżyński Piotr Henryk, Ciorba Andrea
ENT and Audiology Department, University Hospital of Ferrara, Ferrara, Italy.
Institute of Sensory Organs, Kajetany, Poland.
Expert Rev Clin Immunol. 2021 Mar;17(3):233-246. doi: 10.1080/1744666X.2021.1879640. Epub 2021 Mar 22.
Autoimmune Inner Ear Disease (AIED) can be of a primary or secondary type. To date, a clear pathogenesis of the disease is still not available. Focusing on the secondary forms of AIED, the aim of this review is to (i) assess and describe the hearing involvement in patients affected by autoimmune diseases, (ii) describe the possible association between clinical features (among serological/laboratory data and disease activity/duration) and hearing impairment, (iii) show evidence connecting the AIED types with various etiopathogenetic mechanisms.
A PRISMA-compliant systematic review was performed. Medline, Cochrane, Embase, and Cinahl were searched from 1 January 2015 through to 5 August 2020. Overall, 16 studies (involving 1043 participants) were included in the review. The data in the literature suggested that bilateral mild-to-moderate sensorineural hearing loss is a commonly reported clinical symptom of AIED.
Patients with systemic autoimmune disorders present a cochlear injury which might be associated with the humoral and/or cellular immune response against the inner ear. To date, AIED pathogenesis remains an open issue, due to the rarity of these clinical entities and due to the difficulties in investigating the inner ear immunology, considering the inner ear inaccessibility for tissue sampling.
自身免疫性内耳疾病（AIED）可分为原发性或继发性。迄今为止，该病的明确发病机制仍不清楚。本综述聚焦于AIED的继发性形式，目的是：（i）评估和描述自身免疫性疾病患者的听力受累情况；（ii）描述临床特征（血清学/实验室数据以及疾病活动度/病程）与听力障碍之间可能存在的关联；（iii）展示将AIED类型与各种病因发病机制联系起来的证据。
进行了一项符合PRISMA标准的系统综述。检索了2015年1月1日至2020年8月5日期间的Medline、Cochrane、Embase和Cinahl数据库。本综述共纳入16项研究（涉及1043名参与者）。文献数据表明，双侧轻至中度感音神经性听力损失是AIED常见的临床症状。
全身性自身免疫性疾病患者存在耳蜗损伤，这可能与针对内耳的体液和/或细胞免疫反应有关。迄今为止，由于这些临床实体罕见，且考虑到内耳难以进行组织采样，难以对内耳免疫学进行研究，因此AIED的发病机制仍然是一个悬而未决的问题。
