Ramsay Hunt Syndrome

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Please note that the following is a general guideline only. For a full assessment, exclusion of any other underlying cause for your symptoms and an individualised treatment approach, you will need to be seen by a qualified specialist.

DEFINITION

Herpes zoster oticus refers to a syndrome of acute otalgia accompanied by a herpetic, vesicular rash. When accompanied by facial paralysis, the syndrome is known as Ramsay Hunt syndrome.

EPIDEMIOLOGY

Second most common cause of acute facial paralysis (second to bells)
Mostly over 60
M=F

PATHOPHYSIOLOGY

is induced by the reactivation of the varicella-zoster virus that remains latent in the geniculate ganglion after primary infection with chickenpox.

CLINICAL SYMPTOMS AND SIGNS

 Prodrome
majority have viral prodrome with otalgia up to a week before FN paralysis

Vesicles
present with severe otalgia and vesicular eruption in the distribution of the nervus intermedius (see below)
90% pinna, 21% oral (palate, uvula, buccal mucosa, tonsil and tongue)
occurs before paralysis in 55% and after in 45%

VII palsy
Usually rapid onset (sometimes take longer than 3 weeks)
Dysgeusia-due to N intermedius involvement
Hyperacuisis-Stapedius?

Other Cranial Nerves
VIII
-20-40%) include SNHL and vestibular dysfunction
-Tinnitus/ vertigo
V Altered sensation


severe ocular complications
uveitis, keratoconjunctivitis, optic neuritis, and glaucoma and are almost always associated with involvement of the V1 

How This Differ from Bells

Clinical-Intense otlagia/ vesicles/ involvement of other cranial nerves
Prognosis-Worse

TREATMENT 

intravenous acyclovir (10 mg/kg three times daily)
oral acyclovir (800 mg five times daily)
oral valacyclovir (500 mg three times daily) for 10 days

In combination with a 3 week tapering course of prednisone (60 to 80 mg/kg daily)



Because of the presence of “skip” regions and diffuse neuritis of the facial nerve, surgical decompression of RHS is not recommended.