Rheumatology Unit, Anna Meyer Children's Hospital and University of Florence , Florence , Italy .
Autoimmunity. 2013 Dec;46(8):525-30. doi: 10.3109/08916934.2013.822074. Epub 2013 Aug 15.
Immune-mediated pathogenesis has been suggested for idiopathic sensorineural hearing loss. Recent studies have investigated the relationship between idiopathic sensorineural hearing loss and autoantibodies against inner ear antigens. We conducted a prospective, observational study in a series of pediatric patients affected by idiopathic sensorineural hearing loss. Autoantibodies against inner ear (anti-Cogan peptide, anti-connexin 26, anti-DEP1/CD148 and anti-reovirus), previously described in the serum of patients with Cogan's syndrome, were detected in our population. The characteristics of children whose results were positive were also evaluated to verify if clinical data, disease progression and response to treatment could confirm an immune-mediated pathogenesis. Eleven patients were enrolled and 9 of them were positive for inner ear antibodies. Non-organ specific autoantibodies were present in 5 children out of 9. An immune-mediated condition was diagnosed in 2 cases and minor immune manifestations were found in 2 additional patients. In 5 cases hearing loss remained stable without therapy, while 4 children developed progression. Two subjects were treated with corticosteroids and methotrexate, achieving hearing improvement. Another subject showed stabilization on methotrexate. Inner ear autoantibodies can be positive in children with autoimmune sensorineural hearing loss, and in conjunction with clinical data may assist the clinician in identifying a subset amenable for immune modulation therapy. Large prospective studies are needed to investigate usefulness, diagnostic and prognostic role of these autoantibodies.
免疫介导的发病机制已被提出用于特发性感觉神经性听力损失。最近的研究调查了特发性感觉神经性听力损失与内耳自身抗体之间的关系。我们对一系列特发性感觉神经性听力损失的儿科患者进行了一项前瞻性、观察性研究。在内耳中发现了先前在 Cogan 综合征患者血清中描述的自身抗体（抗 Cogan 肽、抗连接蛋白 26、抗 DEP1/CD148 和抗呼肠孤病毒）。还评估了结果为阳性的儿童的特征，以验证临床数据、疾病进展和治疗反应是否可以确认免疫介导的发病机制。共纳入 11 名患者，其中 9 名患者的内耳抗体呈阳性。9 名患儿中有 5 名患儿出现非器官特异性自身抗体。2 例诊断为免疫介导疾病，2 例患儿发现轻微免疫表现。5 例听力损失在未治疗的情况下保持稳定，而 4 例患儿出现进展。2 名患儿接受了皮质类固醇和甲氨蝶呤治疗，听力有所改善。另一名患儿在甲氨蝶呤治疗下病情稳定。内耳自身抗体可在自身免疫性感觉神经性听力损失的儿童中呈阳性，结合临床数据，可帮助临床医生识别适合免疫调节治疗的亚组。需要进行大型前瞻性研究来探讨这些自身抗体的有用性、诊断和预后作用。
