Ramsay Hunt syndrome（亨特综合征）

The strict definition of the Ramsay Hunt syndrome is peripheral facial nerve palsy accompanied by an erythematous vesicular rash on the ear (zoster oticus) or in the mouth. J Ramsay Hunt, who described various clinical presentations of facial paralysis and rash, also recognised other frequent symptoms and signs such as tinnitus, hearing loss, nausea, vomiting, vertigo, and nystagmus. He explained these eighth nerve features by the close proximity of the geniculate ganglion to the vestibulocochlear nerve within the bony facial canal.（Ramsay Hunt综合征的严格定义是周围性面神经麻痹伴有耳朵（带状疱疹）或嘴中出现红斑性水疱疹。描述面部麻痹和皮疹的各种临床表现的J Ramsay Hunt也认识到其他常见的症状和体征，如耳鸣，听力下降，恶心，呕吐，眩晕和眼球震颤。他通过膝状神经节与骨性面管内的前庭脉神经的近距离解释了这些第八条神经特征。）

临床上常将Ramsay Hunt 综合征 分为三型：（1）耳廓带状疱疹；（2）耳带状疱疹合并面瘫；（3）耳带状疱疹合并耳蜗及前庭症状。

Hunt将其分为四种类型：（1）面神经感觉支受累产生耳廓皮肤疱疹和疼痛；（2）面神经感觉支和运动支同时受累，产生耳疼和面瘫；（3）面神经感觉支和运动支受累，并听力损失；（4）面神经感觉支和运动支受累，并听力损失和眩晕。同时，由于舌咽神经、迷走神经和面神经在发育上来自同一腮弓，均可能受到膝状神经节病损的影响。

临床表现变异主要表现在：（1）耳部疱疹与面瘫出现的时间可先后不一，如先出现面瘫，后出现耳部疱疹；（2）疱疹出现的位置发生变异即异位疱疹，如疱疹出现于鼓膜、扁桃体、软腭；（3）患者可先出现其他颅神经侵犯症状，最终表现典型的Ramsay Hunt 综合征体征。

附1：

40 year old lady presented with painful vesicular lesions on her right ear of two days duration. One day prior to admission she had developed inability to close her right eye.facial asymmetry and drooling of liquids from right angle of mouth . She also gave history of painful swallowing of solids of one day duration. There was, in addition. History suggestive of tinnitus and decreased hearing in the right ear. 40岁的女士在持续两天的右耳上出现了水疱性病变。入院前一天，她已经无法闭上右眼。面部不对称和口角直流口水。她还给提供了吞咽固体时疼痛病史1天。此外，还有右耳耳鸣及听力下降的病史。

病例学习：亨特综合征

病例（二十五）：典型的亨特综合征-现实中你见过么？

• Ramsay Hunt syndrome.pdf(305.95k)


• Ramsay Hunt Syndrome Treated with Oral Acyclovir.pdf(787.69k)

亨特综合征，学习了，谢谢楼主！