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What is Ramsay Hunt Syndrome
What is Ramsay Hunt Syndrome?

Ramsay Hunt Syndrome, also known as herpes zoster oticus, is a leading cause of facial paralysis caused by reactivation of the varicella zoster virus (aka the chicken pox virus) affecting the facial nerve. The classic presentation of Ramsay Hunt Syndrome includes sudden onset (within 24-72 hours) facial paralysis, a blistering rash of the ear, and hearing loss and/or dizziness. Some patients will experience facial paralysis and hearing loss or dizziness without ever getting a rash (aka zoster sine herpete).

The main distinguishing features between Bell’s palsy and Ramsay Hunt Syndrome include:

  1. A painful blistering rash of the ear is consistent with Ramsay Hunt Syndrome

  2. “Room spinning” dizziness is consistent with Ramsay Hunt Syndrome

  3. While Bell’s palsy patients often have discomfort around the ear, this pain is not triggered by touch or manipulation of the ear like the pain of Ramsay Hunt Syndrome. The pain of Ramsay Hunt Syndrome is more severe than that of Bell’s palsy. 

  4. While patients with Bell’s palsy and RHS frequently have sensitivity to loud noises, hearing loss is consistent with Ramsay Hunt Syndrome

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How does Ramsay Hunt Syndrome happen?
How does Ramsay Hunt Syndrome happen?

Ramsay Hunt syndrome is thought to be caused by a reactivation of a virus, varicella zoster virus, in the facial nerve. The infection is thought to spread to nearby nerves - hearing and balance.

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How many people get Ramsay Hunt Syndrome?
How many people get Ramsay Hunt Syndrome?

Approximately 5 per 100,000 people per year are affected by RHS. However, many people are misdiagnosed as having Bell’s palsy and so the true incidence is likely higher. Roughly 10% of patients with facial paralysis have it because of RHS. While RHS affects patients of all ages, it is more common in people older than 50 years-old and is rare in children. RHS impacts patients of all races and cultures without preference. 

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What are recommended treatments for patients when they first get Ramsay Hunt Syndrome?
What are recommended treatments for patients when they first get Ramsay Hunt Syndrome?

Patients with suspected Ramsay Hunt Syndrome should be evaluated by a healthcare provider immediately. The provider will perform a complete neurologic exam and may perform blood tests and imaging studies to rule out other causes of facial paralysis. If the diagnosis is confirmed to be Ramsay Hunt Syndrome, the following treatments will typically be initiated:

  1. High dose steroids (e.g., Prednisone), typically 7-10 days of 60mg Prednisone daily within 72 hours of symptom onset. This treatment has the strongest evidence for improving outcomes. 

  2. Antiviral therapy, typically 1000mg Valtrex three times daily for 1 week. This treatment has good evidence for improving outcomes.

  3. Eye protection including drops, gel drops, and ointment to keep the eye moist since patients frequently are unable to fully close their affected eye. Patients may need to tape their eye closed at nighttime when sleeping. 

  4. Referral to an otolaryngologist, an ENT (aka Ear, Nose, and Throat) specialist who can evaluate hearing, balance, and facial paralysis, and ensure appropriate long-term follow up.

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What are the chances I will recover facial movement?
What are the chances I will recover facial movement?

Compared to Bell’s palsy, fewer people make a complete recovery from Ramsay Hunt Syndrome, although many people do fully recover. However, recovery can take longer than recovery from Bell’s palsy. Some patients will continue to see changes in their facial movement a year after RHS onset. Patients almost always recover some facial tone (i.e., symmetry when face is at resting) and facial movement. However, many times aberrant facial nerve regeneration occurs where the facial nerve makes abnormal connections with facial muscles. This “miswiring” leads to the same symptoms seen in chronic Bell’s palsy:

  • Facial asymmetry

  • Smile asymmetry

  • Facial synkinesis: involuntary facial movements such as eye closing with smile

  • Narrowing of the eye

  • Facial tightness

  • Neck tightness

  • Deep nasolabial fold (aka smile lines)

  • Chin dimpling

  • Platysma banding

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Why do I experience hearing Loss? Why do I experience dizziness?
Why do I experience hearing Loss? Why do I experience dizziness?

The nerves for hearing and balance run directly alongside the nerve for facial movement, often causing patients to develop issues with all three systems. Many patients with RHS experience a nerve related (sensorineural) hearing loss, although this is often an under-reported and/or under-detected problem. Hearing loss is most often noticed in the high frequencies (high pitched noises), but can affect both high and low frequencies. Many patients will also experience dizziness and imbalance. Those who have vertigo (room spinning dizziness) are often noted to have greater hearing loss.

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Will my hearing improve? Will my dizziness and balance improve?
Will my hearing improve? Will my dizziness and balance improve?

Recovery is unpredictable. While typically dizziness and balance improve, hearing outcomes vary. Research on hearing and balance outcomes in RHS is sparse. Those individuals who are older, male, have vertigo or have more severe hearing loss are thought to have poorer hearing recovery. Hearing loss will need to be closely monitored over the course of treatment. Hearing loss treatment may need to include hearing aids and/or cochlear implants pending the severity of the hearing loss.

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