Narayan Manisha, Sreedharan Suja, Pai Rohit, Shenoy Sajjan
Department of Otorhinolaryngology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, 575001, Karnataka, India.
Department of Neurology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, 575001, Karnataka, India.
Iran J Otorhinolaryngol. 2021 Jan;33(114):45-48. doi: 10.22038/ijorl.2020.47096.2608.
The relationship between autoimmune disease and sensorineural loss is well documented in literature. Immune mediated sudden hearing loss is asymmetric, bilateral and rapidly progressive but responds well to steroid therapy. However association of cranial nerve neuropathies with sudden hearing loss is rare.
A 41 year old female presented with sudden mixed hearing loss and developed multiple cranial nerve palsies within a month. Blood and Cerebrospinal fluid analysis revealed an undiagnosed rheumatoid arthritis. She responded well to definitive therapy with cyclophosphamide and azathioprine.
If sudden hearing loss is associated with cranial neuropathy, an autoimmune work-up is highly recommended.
自身免疫性疾病与感音神经性听力损失之间的关系在文献中有充分记载。免疫介导的突发性听力损失多为不对称、双侧且进展迅速，但对类固醇治疗反应良好。然而，颅神经病变与突发性听力损失的关联较为罕见。
一名41岁女性出现突发性混合性听力损失，并在一个月内出现多处颅神经麻痹。血液和脑脊液分析显示患有未确诊的类风湿性关节炎。她对环磷酰胺和硫唑嘌呤的确定性治疗反应良好。
如果突发性听力损失与颅神经病变相关，强烈建议进行自身免疫性检查。
