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What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome (RHS) is the name given to describe a cluster of symptoms caused by a shingles infection affecting the facial nerve.

Causes

Shingles are caused by the same virus responsible for chickenpox called the Varicella Zoster virus.  People who develop RHS will have had chickenpox usually during childhood.  Recovery from chickenpox is complete once the spots heal.  However, the virus continues to live silently within the previously infected nerves.  Shingles is a reactivation of the chickenpox virus which results in the appearance of new symptoms.

Reactivation of the virus commonly happens when the immune systems are depressed and therefore unable to protect the body from infections in the normal way.  Stress can have a negative impact on the immune system and studies have shown that people experiencing long periods of stress are more likely to become vulnerable to a reactivation of the virus.

RHS is not infectious but people who have not had chickenpox can go on to develop chickenpox if they have direct contact with open blisters/rashes of a person infected with RHS.

Symptoms

The following symptoms are associated with a diagnosis of RHS.  In some cases, there is no rash and not all the symptoms described below will be experienced by everyone.  Importantly, they may not all appear at the same time but follow on from each other.

    • Muscle weakness or paralysis on the affected side of the face which causes the face to droop.  This occurs over a period of a few hours and up to 72 hours from the first symptoms occurring.  You should seek medical advice as soon as you notice any symptoms.
    • Loss of facial expressiveness on the affected side.
    • Difficulty closing the eye or blinking on the affected side.
    • Weakness of the lip and cheek muscles can make eating and drinking difficult.
    • Weakness of the lip and cheek may also make it difficult to pronounce some sounds especially ‘p’ and ‘b’.  It may also cause dribbling on the affected side.
    • A rash or blisters in or around the ear, scalp or hairline.  Blisters may also appear inside the mouth.  The rash or blisters are painful and often described as a burning sensation over the affected area.
    • Ear, face and or head pain which may or may not be associated with a rash/blisters.
    • Changes in hearing on the affected side.
    • Dizziness
    • Tinnitus (ringing in the ear).

Diagnosis

A diagnosis of RHS can be difficult due to the fact that symptoms vary from person to person and symptoms of RHS are similar to other causes of facial weakness especially Bell’s palsy.

The list below outlines what should be included in a medical examination to assist in a diagnosis of RHS.

    • An examination of the ear including the skin around the ear and looking inside the ear.  This will allow the clinician to identify the presence of any blisters or rashes.
    • An examination of the mouth to exclude any blisters inside the mouth especially the roof of the mouth, the tongue and cheeks.
    • A thorough history to determine how and when symptoms arose.
    • Assessment of facial nerve function with a focus on eye closure and blinking.
    • Blood tests to measure whether it contains antibodies to the Varicella Zoster virus which is responsible for causing RHS.
    • Hearing tests.
    • Magnetic Resonance Imaging (MRI) to see if there is any inflammation along the facial nerve and to exclude any other causes.

Treatment 

    • Antiviral medication such as Acyclovir ideally started within the first three days from the onset of symptoms.
    • Oral steroids such as Prednisolone ideally started within the first three days from the onset of symptoms.
    • Painkillers

What sort of recovery can be expected?

    • Early intervention with these medications will help maximise the potential for recovery.  However, recovery invariable depends on the degree of facial nerve injury.  The more severe the damage to the facial nerve, the longer it will take to recover.  A slower recovery lowers the chance of making a complete recovery.
    • If antiviral treatment is given within 72 hours of developing symptoms, the majority of people, approximately 70%, will experience a full recovery.
    • If antiviral treatment is not given within this timeframe, the likelihood of recovery is lower, and only approximately 50% of people will experience a full recovery.
    • If the facial paralysis does not resolve after one month, you should be referred to a facial palsy specialist.  This could be an ENT consultant, neurologist or plastic surgeon.  Where possible a referral to a facial palsy therapist is recommended.  This could be either a physiotherapist or a speech and language therapist who is experienced in the management of facial paralysis.
    • Botulinum Toxin injections may be appropriate for those with persistent symptoms beyond four months, as this may help with facial pain, muscle spasm and involuntary facial movement (synkinesis). 

Eye care

The clinician involved in your diagnosis should outline how you should care for your eye whilst it cannot fully close or blink.  If problems with eye closure and blink persist beyond 2 – 3 weeks you should ask your GP to refer you to an eye specialist. Read more about Eye care