Ramsay Hunt Syndrome: Causes, Diagnosis and Treatment

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Ramsay Hunt Syndrome [RHS] is a peripheral facial nerve palsy caused by an infection with the virus Varicella Zoster virus. It is generally accompanied by a rash (which can be painful) in the area of the ear and/or the mouth; however, occasionally there will be no rash visible. The auditory nerve may also be involved, leading to hearing and balance effects.

Ramsay Hunt Syndrome

The syndrome is named after Dr J. Ramsey Hunt, the physician who first described the syndrome at a meeting of the American Neurological Association in 1906. He subsequently published his article on the subject in 2007.

When compared with Bell's Palsy, RHS has a higher incidence of incomplete recovery with longstanding sequelae.

Mechanism of Injury / Pathological Process

RHS is caused by reactivation of the varicella-zoster virus (the virus which causes chickenpox) within the sensory ganglion of the Facial Nerve, the 7th Cranial Nerve. As the 8th Cranial Nerve, known as the Auditory Nerve or the Vestibulocochlear Nerve, lies next to the sensory ganglion of the facial nerve within the facial canal, both nerves are usually involved.

Ramsay Hunt Syndrome

What Are the Symptoms of RHS?

  • Decreased movement of one side of the face
  • Painful rash with fluid-filled blisters on the ear skin and ear canal
  • Hearing loss, ringing in the ear
  • Vertigo
  • Dry mouth, dry eyes
  • Change in taste

Diagnostic Procedures

The diagnosis of RHS is a clinical diagnosis, made when the combination of ear pain, a herpetic rash in the ear or mouth and facial palsy occurs; however, virological studies can be used to confirm the diagnosis.

MRI scanning can be used to exclude other causes of facial and auditory nerve dysfunction (such as Acoustic Neuroma).

Differential Diagnosis

The following conditions can present in with similar symptoms:
  • Bell's Palsy
  • Postherpetic Neuralgia
  • Acoustic Neuroma
  • Temporomandibular Disorders
  • Trigeminal Neuralgia

What is the treatment for Ramsay Hunt syndrome?

Full recovery is more likely if antiviral treatment is started within 72 hours of onset of symptoms. Treatment options for Ramsay Hunt syndrome are:

  • Aciclovir 800 mg orally five times daily for 7 days
  • Valaciclovir 1 g three times daily for 7 days
  • Famciclovir 500 mg three times daily for 7 days
  • Combination therapy of antiviral treatment and prednisone (60 mg orally daily for 5 days).
Symptomatic treatment during acute Ramsay Hunt syndrome may include:
  • Systemic corticosteroids
  • Opioid analgesics
  • Anticonvulsants, such as gabapentin and pregabalin
  • Ice packs on the blisters
  • An eye patch if unable to close an eye
  • Artificial tears and lubricating ointments to combat dry eye
  • Transcutaneous electrical nerve stimulation (TENS) and vibration
  • Tepid topical solution of aluminium sulfate.
  • Topical capsaicin can be used for post-herpetic neuralgia but is not well tolerated on facial skin.

What is the outcome for Ramsay Hunt syndrome?

Ramsay Hunt syndrome is associated with a poorer prognosis than Bell palsy; that is, complete recovery is less likely, particularly if antiviral treatment is not started within 72 hours of the onset of symptoms.

The House–Brachmann Scale, which correlates with the degree of facial nerve dysfunction, can be used as a prognostic tool.

Poor prognostic factors include:

  • Diabetes mellitus
  • Advanced age (> 60)
  • Hypertension
  • Vertigo.

Complications may include:

  • Permanent facial paralysis
  • Hearing loss
  • Synkinesis (unwanted facial movements linked to deliberate movements)
  • Polycranial neuropathy
  • Myelitis (inflammation of the spinal cord)
  • Post-herpetic neuralgia.

Physiotherapy Interventions

Physiotherapy management of the facial paralysis or weakness is as detailed in the section on Facial Palsy.

It is also important to provide information on care of the eye in order to prevent the formation of corneal ulcer: see advice page on Dry Eye. Referral to an opthalmologist should be considered.

RHS patients with long term facial palsy may also start to experience dental problems: see advice page on Dental Issues in Facial Palsy.

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