Ramsay Hunt syndrome

The strict definition of the Ramsay Hunt syndrome is peripheral facial nerv e palsy accompanied by an erythematous vesicular rash on the ear (zoster ot icus) or in the mouth. J Ramsay Hunt, who described various clinical presen tations of facial paralysis and rash, also recognised other frequent sympto ms 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. Hunt's analysis of clinical variations of the syndrome now be aring his name led to his recognition of the general somatic sensory functi on of the facial nerve and his defining of the geniculate zone of the ear. It is now known that varicella zoster virus (VZV) causes Ramsay Hunt syndro me. Compared with Bell's palsy (facial paralysis without rash), patients with R amsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely. Studies suggest that treatment with prednison e and acyclovir may improve outcome, although a prospective randomised trea tment trial remains to be undertaken. In the only prospective study of pati ents with Ramsay Hunt syndrome, 14% developed vesicles after the onset of f acial weakness. Thus, Ramsay Hunt syndrome may initially be indistinguishab le from Bell's palsy. Further, Bell's palsy is significantly associated wit h herpes simplex virus (HSV) infection. In the light of the known safety an d effectiveness of antiviral drugs against VZV or HSV, consideration should be given to early treatment of all patients with Ramsay Hunt syndrome or B ell's palsy with a 7-10 day course of famciclovir (500 mg, three times dail y) or acyclovir (800 mg, five times daily), as well as oral prednisone (60 mg daily for 3-5 days). Finally, some patients develop peripheral facial paralysis without ear or m outh rash, associated with either a fourfold rise in antibody to VZV or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva. This indicates that a proportion of patients with "Bell' s palsy" have Ramsay Hunt syndrome zoster sine herpete. Treatment of these patients with acyclovir and prednisone within 7 days of onset has been show n to improve the outcome of recovery from facial palsy.