Psillas George, Arnaoutoglou Marianthi, Gatsios Trifonas, Rizos Dimitris, Koutsouraki Efrosini, Vital Victor
1st Academic ENT Department, American Hellenic Educational Progressive Association Hospital, Aristotle University of Thessaloniki, 1 Stilponos Kyriakidi St., Thessaloniki, Greece.
Auris Nasus Larynx. 2012 Apr;39(2):229-32. doi: 10.1016/j.anl.2011.03.007. Epub 2011 May 14.
To describe the first published case of recurrent facial nerve palsy associated with bilateral sudden sensorineural hearing loss of autoimmune origin.
A 33-year-old male presented with acute facial palsy on the left following a vesicular herpetic eruption in the external ear canal on the same side. Serologic measurements demonstrated an elevation of IgM antibodies against herpes simplex virus but not for varicella-zoster virus, confirming a Ramsay Hunt-like syndrome due to herpes simplex virus. The following four months, the patient exhibited other three episodes of facial palsy, well responded to steroid treatment. During the clinical course, a sudden sensorineural hearing loss was also diagnosed, initially on the left side and then on both sides. The autoimmune markers such as the antinuclear antibody and the anti-gangliosides antibodies (anti-GM1, anti-GQ1b) were found positive. Despite steroid treatment, hearing did not show any improvement, remaining moderate on the right and severe on the left.
Recurrent facial nerve palsy and bilateral sudden sensorineural hearing loss could be the expression of autoimmune disturbances. The initial triggered factor could be the herpes simplex virus infection, such as a Ramsay Hunt-like syndrome.
描述首例已发表的与自身免疫性双侧突发性感音神经性听力损失相关的复发性面神经麻痹病例。
一名33岁男性，在同侧外耳道出现水疱性疱疹后，左侧出现急性面神经麻痹。血清学检测显示抗单纯疱疹病毒IgM抗体升高，但抗水痘-带状疱疹病毒抗体未升高，证实为单纯疱疹病毒引起的类拉姆齐·亨特综合征。在接下来的四个月里，患者又出现了另外三次面神经麻痹发作，对类固醇治疗反应良好。在病程中，还诊断出突发性感音神经性听力损失，最初是左侧，然后两侧均出现。发现自身免疫标志物如抗核抗体和抗神经节苷脂抗体（抗GM1、抗GQ1b）呈阳性。尽管进行了类固醇治疗，但听力没有改善，右侧仍为中度，左侧仍为重度。
复发性面神经麻痹和双侧突发性感音神经性听力损失可能是自身免疫紊乱的表现。最初的触发因素可能是单纯疱疹病毒感染，如类拉姆齐·亨特综合征。
