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Ramsay-Hunt Syndrome

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Overview

Also known as herpes zoster oticus, Ramsay Hunt syndrome describes the reactivation of the varicella-zoster virus in the geniculate ganglion of the facial nerve leading to palsy. This usually leads to lower motor neurone (LMN) lesions of the facial nerve.

It is essential to differentiate between upper motor neurone (UMN) and LMN lesions, as a UMN can be the presenting feature of a stroke.

Epidemiology

  • More common >60 years of age, but can happen at any age
  • Severity also increases with age
  • More common in people who are immunocompromised
  • If symptoms affect more than one dermatome, suspect HIV or other causes of immunodeficiency

Risk Factors

  • Increasing age
  • Immunodeficiency

Presentation

Auricular pain often precedes facial nerve palsy:

  • The ear pain is usually deep but can radiate into the pinna and be diffuse and dull
  • Unilateral lower motor neurone facial nerve palsy – the forehead is also affected
    • Upper motor neurone lesions spare the forehead and upper space and suggest a stroke
  • Vesicular rash around the ear or anywhere the facial nerve supplies such as:
    • The anterior 2/3 of one side of the tongue
    • The soft palate
    • The external auditory canal
    • The pinna 
  • Vertigo
  • Tinnitus
  • Dry eyes or excessive tearing
  • Altered taste
  • Hyperacusis

If symptoms affect more than one dermatome, suspect HIV or other causes of immunodeficiency

Differential Diagnoses

Bell’s palsy

  • There is no vesicular rash around the ear or in the distribution of the facial nerve
  • There still may be auricular pain
  • There is no vertigo/tinnitus

Stroke

  • There are signs of a UMN – the upper face and forehead are spared 

Investigations

  • Diagnosis is usually clinical
  • If atypical features are seen, referral for an MRI is considered
  • Consider HIV testing if more than one dermatome is affected

Management

  • 1st-line: oral aciclovir + oral corticosteroids + artificial tears/lubricants

Patient Advice and Complications

Patient Advice

  • Patients should keep the affected eye lubricated and keep their eyes closed using microporous tape at night if needed
  • If patients develop eye e.g. irritation/pain/vision changes, they should seek immediate medical advice

Complications

  • Postherpetic neuralgia
  • Tinnitus
  • Secondary bacterial infection 
  • Ectropion
  • Facial pain and paraesthesia
  • Dry mouth
  • Hyperacusis

Prognosis

  • The rash resolves but unlike Bell’s palsy, complete recovery is seen in 75% of people
  • Factors associated with a poorer prognosis are:
    • Vertigo
    • Age
    • Diabetes
    • Hypertension

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