Abbate A, Viaggio M B, Piras D, López L, Hubscher O, Nogués M
Sección Clínica Médica, Hospital Ramos Mejía, Buenos Aires, Argentina.
Medicina (B Aires). 2001;61(6):860-2.
The audiovestibular system can be affected by an immunologic etiology. The immune-mediated inner ear disease (IMIED) is a syndrome that includes rapidly progressive sensorineural hearing loss, vertigo and tinnitus, which occurs as a primary disorder or complicates certain autoimmune systemic conditions. However, if treated promptly with immunosuppression, the audiological sequel of IMIED may be avoided. We present a 28 year old female patient, who after rhinitis and mioarthralgias developed a vestibular syndrome. A week later she experienced bilateral hearing loss that progressed to deafness in 72 hours. The examination revealed horizontal and torsional nystagmus, a disrupted vestibulo-ocular reflex and vertigo with the positional changes. Laboratory data were normal except for eritrosedimentation rate (75 mm/1 hour). The autoantibodies usually present in rheumatologic autoimmune systemic diseases were negative. The antibodies to the 68-kD antigen found in the inner ear were positive. The chest x-ray and sinus x-ray were normal. The head magnetic resonance imaging with gadolinium and ear computed tomography were normal. Cerebrospinal fluid studies showed normal findings. With the possible diagnosis of IMIED we started early treatment with corticosteroids, with improvement in auditory and vestibular function thereafter. We highlight the early recognition of IMIED as a differential diagnosis in patients with acute bilateral hearing loss, because prompt treatment with immunosuppression might have a positive effect on auditory function recovery.
听觉前庭系统可能受到免疫病因的影响。免疫介导性内耳疾病(IMIED)是一种综合征,包括快速进展的感音神经性听力损失、眩晕和耳鸣,可作为原发性疾病出现,或使某些自身免疫性全身性疾病复杂化。然而,如果及时进行免疫抑制治疗,IMIED的听力后遗症是可以避免的。我们报告一名28岁女性患者,在出现鼻炎和肌关节痛后发展为前庭综合征。一周后,她出现双侧听力损失,并在72小时内进展为耳聋。检查发现水平和扭转性眼球震颤、前庭眼反射中断以及体位改变时出现眩晕。除血沉(75mm/1小时)外,实验室检查数据均正常。通常在风湿性自身免疫性全身性疾病中出现的自身抗体为阴性。在内耳发现的针对68-kD抗原的抗体呈阳性。胸部X光和鼻窦X光检查正常。钆增强头部磁共振成像和耳部计算机断层扫描均正常。脑脊液检查结果正常。鉴于可能的IMIED诊断,我们开始早期使用皮质类固醇治疗,此后听觉和前庭功能有所改善。我们强调早期识别IMIED作为急性双侧听力损失患者的鉴别诊断,因为及时进行免疫抑制治疗可能对听觉功能恢复产生积极影响。