Ramsay Hunt syndrome

What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome is a rare peripheral facial neuropathy. It is a type of shingles due to reactivation of the varicella zoster virus (VZV) in the geniculate ganglion of cranial nerve VII, which supplies the facial nerve.

• Ramsay Hunt syndrome is characterised by unilateral facial weakness and painful blisters — either in the ear canal on the same side as the facial palsy or inside the mouth.
• Sometimes, the involvement of cranial nerve VIII results in auditory (hearing) and/or vestibular symptoms (dizziness). Infection can also spread to cranial nerves III, IV, V, VI, IX, X, XI and XII.
• Blisters may be absent in some patients, in which case the infection is known as zoster sine herpete.

Ramsay Hunt syndrome is also known as herpes zoster oticus, geniculate ganglionitis, Ramsay Hunt disease and Hunt syndrome. The name was coined by neurologist James Ramsay Hunt in 1907.

Who gets Ramsay Hunt syndrome?

Ramsay Hunt syndrome occurs in individuals with a previous history of varicella (chickenpox).

• Ramsay Hunt syndrome is rare, having an incidence of approximately 5 per 100,000 population.
• It is slightly more common in females than in males.
• Ramsay Hunt syndrome is mainly seen in adults, particularly in people over 60 years of age, but it may rarely be seen in children.
• 12% of peripheral facial nerve palsies are attributable to Ramsay Hunt syndrome.
• Ramsay Hunt syndrome is the second most common cause of non-traumatic peripheral facial paralysis.

What causes Ramsay Hunt syndrome?

After primary infection by VZV (ie, chickenpox), there is a latent period where VZV remains dormant in the neurones of the cranial nerves, the dorsal root ganglion and autonomic ganglia. The reactivation of VZV in the geniculate ganglion of cranial nerve VII is what leads to the syndrome. VZV can also spread down the axons of other cranial nerves that share a blood supply with the geniculate ganglion.

Reactivation of VZV is more likely for:

• Individuals with an immunodeficiency, such as infection with human immunodeficiency virus (HIV) or malignancy
• People on immunosuppressive treatment, corticosteroids, chemotherapy, or radiation therapy
• Individuals experiencing infection or physical or emotional stress.

What are the clinical features of Ramsay Hunt syndrome?

The distinctly characteristic features of Ramsay Hunt syndrome are unilateral facial nerve paralysis and a painful vesicular rash on the ear and the mucous membrane of the oropharynx or palate. About 20% of patients develop facial nerve palsy several days before the blisters appear. About 10% of patients with Ramsay Hunt syndrome do not develop any blisters.

Other symptoms of Ramsay Hunt syndrome can include:

• Flu-like symptoms, such as a fever and headache, for 3–7 days before the rash appears
  • Aching or painful sensations in the ear
  • Macules, papules, and coalescing vesicles in the painful sites, which then rupture with crusting
  • Loss of taste sensation in the anterior two-thirds of the tongue
  • Dry eyes and dry mouth
  • Tinnitus, hearing loss or hyperacusis (increased sensitivity to sound), vertigo, nausea, and vomiting
  • Symptoms due to dysfunction of other cranial nerves.

Herpes zoster 

Facial nerve palsy 

Facial nerve palsy 

How is Ramsay Hunt syndrome diagnosed?

The diagnosis of Ramsay Hunt syndrome is usually made clinically. The diagnosis can be confirmed via testing with a VZV polymerase chain reaction assay (using ear exudate). This test is particularly useful to distinguish the zoster sine herpete variation of Ramsay Hunt syndrome from Bell palsy (idiopathic acute facial nerve palsy).

Magnetic resonance imaging (MRI) may be undertaken if there are polycranial neurological symptoms.

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.