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ENT clinic - Ramsay Hunt syndrome

ENT GPSI Dr Raj Singh continues our series with a disease that is often misdiagnosed

 

Case

A 51-year-old woman presented to her GP with a three-day history of gradually increasing left-sided head pain.

She was being treated for hypertension and also used a triptan to control her migraine. But she insisted this head pain was different, especially in the way it radiated out to her ear.

Examination revealed a reddened, swollen right external auditory canal with a normal tympanic membrane. She was diagnosed as having simple otitis externa and prescribed ciprofloxacin drops.

Two days later she presented to A&E with dramatically worsened head pain, left-sided pulsatile tinnitus, vertigo and left-sided facial weakness. The swelling and redness in her left ear had worsened and there were now four small vesicles on the concha. She was seen by an ENT consultant and – after she confirmed she had had varicella infection as a child – diagnosed with Ramsay Hunt syndrome. She was given a dose of intravenous acyclovir and steroids, then discharged with a two-week course of oral acyclovir and steroids. At three weeks' follow-up the symptoms had all resolved.

The problem

  • Ramsay Hunt syndrome is a varicella zoster virus infection of the head and neck involving the facial nerve, usually the seventh cranial nerve.
  • It accounts for 18% of facial palsies in adults and 16% of all causes of unilateral facial palsies in children.1
  • It is thought to be the cause of as many as 20% of clinically diagnosed cases of Bell's palsy.2
  • There is some confusion over nomenclature – there are three syndromes that use the same name: the one described here, also called herpes zoster oticus; Ramsay Hunt cerebellar syndrome, a rare condition involving seizures and cognitive impairment; and Ramsay Hunt syndrome III, a neuropathy of the ulnar nerve.

Features

  • Patients usually present with paroxysmal pain deep within the ear – often radiating out into the pinna – but may have a more constant, diffuse background pain.
  • Up to 80% of cases also have a vesicular rash of the ear or mouth – soft palate and anterior two-thirds of the tongue – usually developing hours or even days after the onset of pain. 
  • Lower motor neuron facial paresis or palsy can develop after the rash and pain and usually reaches maximum severity by a week after onset of symptoms.
  • Other features can include vertigo, hearing loss, tinnitus, headaches, dysarthria, gait ataxia and fever.

Diagnosis

  • The diagnosis is clinical and straightforward when classic features are present: peripheral facial nerve paresis with associated rash or herpetic blisters in ear or mouth.
  • The unilateral facial weakness is very similar to Bell's palsy, but the rash is the key differentiator.
  • Take a careful history – ask about childhood varicella infection – and perform a focused but thorough physical examination.
  • Initiation of treatment within 72 hours of symptom onset improves outcomes, so urgent referral is warranted.3

Management

  • Ramsay Hunt syndrome is a self-limiting disease, not usually associated with mortality.
  • Complete recovery rate is around 50% – 75% if treatment is started within 72 hours – and the primary morbidity is from facial weakness.
  • Poor prognostic factors include age older than 50 years and complete facial paralysis.
  • Oral steroids and acyclovir – oral or IV followed by oral – is the usual treatment.

 

Dr Raj Singh is an ENT GPSI in Manchester

 

References

1 Bhupal H. Ramsay Hunt syndrome presenting in primary care. Practitioner 2010;254:33-53

2 Gilchrist J. Seventh cranial neuropathy. Seminars in Neurology 2009;29:5-13

3 Uscategui T, Doree C, Chamberlain I et al. Corticosteroids as adjuvant to antiviral treatment in Ramsay Hunt syndrome (herpes zoster oticus with facial palsy) in adults. Cochrane Database Syst Rev 2008;3:CD006852

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