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Ramsay Hunt syndrome

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The Ramsay Hunt Syndrome is characterised by sensorineural deafness, vertigo and facial paralysis in association with Herpes Zoster infection. The patient is often elderly.

Herpes zoster infects the geniculate ganglion, more rarely the IXth and Xth nerves and, very occasionally, nerves V, VI or XII.

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 (1)

  • 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
  • some patients develop peripheral facial paralysis without ear or mouth rash, associated with either a fourfold rise in antibody to VZV (varicella zoster virus) or the presence of VZV DNA in auricular skin, blood mononuclear cells, middle ear fluid, or saliva
    • indicates that a proportion of patients with "Bell's palsy" have Ramsay Hunt syndrome zoster sine herpete
    • in a prospective study of patients with Ramsay Hunt syndrome, 14% developed vesicles after the onset of facial weakness
      • therefore, Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy - a high index of suspicion for possible Ramsay Hunt syndrome in patients with Bell's palsy is therefore necessary to ensure anti-virals are not delayed

Incidence of Ramsay Hunt syndrome is 5/100,000 (5).

Recovery of facial nerve function is less likely than in Bell's palsy

  • compared with Bell's palsy (facial paralysis without rash), patients with Ramsay Hunt syndrome often have more severe paralysis at onset and are less likely to recover completely (1)

The prognosis can be improved by treatment with acyclovir.

  • standard first-line treatment for herpes zoster infections at sites in the body other than the ear is the antiviral agent acyclovir, which is given either intravenously or orally. Other antiviral agents that may be prescribed include valacyclovir, famcyclovir or brivudin (2)

A systematic review considered the use of corticosteroids with acyclovir (3):

  • did not identify any randomised controlled trials that assess the effects of using corticosteroids as an adjuvant to antiviral treatment in Ramsay Hunt syndrome. .. If they were entirely harmless and free of adverse effects it might be possible to argue in favour of their use in patients with Ramsay Hunt syndrome, even in the absence of evidence of effectiveness

A more recent review (4) states:

  • early treatment with a combination of acyclovir and prednisone is reported to be effective to treat Ramsay Hunt syndrome (4). However it is noteworthy that the two studies referenced for justification of this treatment strategy preceded the publication date of the Cochrane review (3)

Treatment principles:

  • seek expert advice
  • main goals of treatment are to decrease the incidence of late complications, including spastic facial paralysis and postherpetic neuralgia
  • evidence shows a decrease in long-term complications with the use of oral antivirals and steroids (4)
  • symptomatic management is also critical, especially for two aspects of Ramsay Hunt syndrome:
    • pain
      • analgesia in the short-term such as paracetamol
      • tricyclic antidepressants and gabapentin are useful for the treatment of neuropathic pain and postherpetic neuralgia (5)
    • corneal exposure
      • inabilty to close the eye on the affected side, can lead to irritation and corneal ulceration
        • artificial tears throughout the day and ocular lubricant ointment at night are helpful for the prevention of exposure keratopathy (5)
          • if frank lagophthalmos
            • instruct patients how to stretch the eyelid and how to tape the eye closed at night in such a way as to avoid scratching the cornea in the process
        • requires urgent review by an ophthalmologist
        • eye care advice from Facial Palsy UK states:
          • 1. Frequent instillation of artificial tear drops in the day time (at least every 2 hours) and lubricant ointment (e.g. Lacrilube) at night time.
          • 2. Ointment can be used in the day time also, but this can cause blurring of vision.
          • 3. If drops are needed more than 4 times a day then they should be PRESERVATIVE-FREE drops. Preservatives used in large quantities or over a prolonged period of time may damage the delicate cells on the surface of the eye or cause inflammation.
          • 4. Taping the eye closed at night, ensuring that the eye is fully closed, refer patient to the self-help videos on the Facial Palsy UK website. https://www.facialpalsy.org.uk/support/self-help-videos/
          • 5. General advice is to attempt voluntary eyelid closure several times an hour usually by pushing up the lower lid when blinking. Also to wear sunglasses with visors or wraparounds out of doors; to avoid bright sunlight; to avoid/minimise exposure to dry conditions such as air conditioning/central heating/car fan heaters/demisters.
          • 6. Corneal exposure with a dry eye problem may be overlooked where excessive watering is a symptom. Patients should understand that with this condition the eye may water excessively as a reflex because it is too dry and this will need careful management to avoid permanent loss of vision.
          • 7. A patient with a facial palsy who has a poor Bell's phenomenon is at an increased risk for the development of a corneal ulcer. A patient with a loss of corneal sensation is at an even greater risk.

  • surgical management in the acute setting is controversial (5)

  • in the long term, the management of synkinesis can be accomplished via both conservative and surgical approaches (5)
    • conservative approaches include:
      • massage and physical therapy
      • chemodenervation with botulinum toxin
    • surgical management of synkinesis
      • selective neurectomy and/or myomectomy, or even nerve or functional free muscle transfer to improve smile symmetry

  • psychosocial aspects of living with facial palsy should not be underestimated; ensure the patient knows how to access further support, for example from Facial Palsy UK (6)

Reference:

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