What Triggers Ramsay Hunt Syndrome?

Ramsay Hunt syndrome (RHS), also called herpes zoster oticus or geniculate ganglion herpes zoster, is a late complication of varicella zoster virus infection.

• Varicella zoster virus causes chickenpox in children.
• Once the chickenpox is cleared from the system, the virus stays inactive in the nerve tissue and may get reactivated under certain situations.
• The reactivation of the varicella zoster virus leads to typical vesicular lesions called shingles anywhere on the body.
• When they involve the facial nerve, the condition is called RHS.

RHS occurs when the previously dormant or inactive varicella zoster virus gets reactivated and causes inflammation of a particular part of the facial nerve called the geniculate ganglion. Fewer than one percent of varicella zoster virus infections result in RHS. The disorder has been named after the American neurologist Dr. James Ramsay Hunt who described this disorder.

The House-Brackmann facial nerve grading scale is used to grade the severity of facial nerve involvement in RHS:

No, you cannot catch Ramsay Hunt syndrome (RHS) from an affected person. Because the varicella zoster virus causes the syndrome, people not immunized against this virus can catch the infection from a person with RHS through their skin lesions and develop chickenpox.

Vaccination against varicella zoster virus or previous infection by chickenpox thus helps prevent the infection.

People affected with Ramsay Hunt syndrome generally report severe pain felt deep within the ear. The pain usually often radiates outward, affecting the pinna of the ear. The onset of pain is usually followed by a rash after several hours or days. The rash appears as small fluid-filled painful blisters or vesicles, affecting about 80 percent of the people diagnosed with the syndrome. Moreover, people report facial weakness that reaches its maximum severity within a week of the onset of symptoms.

Symptoms generally include:

• Vesicular rash that affects the ear or mouth
• Facial weakness and loss of facial expressions on the affected side
• Hearing loss
• Vertigo
• Tinnitus (ringing in the ear)
• Ear pain
• Headaches
• Speech difficulty
• Difficulty walking
• Fever
• Cervical adenopathy (enlarged neck glands)
• Nausea
• Vomiting
• Loss of taste
• Dry mouth and eyes
• Difficulty eating and drinking
• Intolerance to sounds

Treatment of Ramsay Hunt syndrome is mainly focused on relieving the symptoms and minimizing complications. Studies report that early treatment initiation, preferably within three days of symptom onset, gives a better outcome than starting the treatment later.

Treatment generally includes:

• Oral corticosteroids
• Oral antiviral medication (acyclovir and famciclovir)
• Intravenous corticosteroids
• Antihistamines such as meclizine and dimenhydrinate
• Anticholinergic medications (transdermal scopolamine)
• Carbamazepine
• Local anesthetic ear drops 
• Pain medications such as ibuprofen, acetaminophen, and opioids
• Transcutaneous electrical nerve stimulation

Your doctor may recommend proper rest to aid recovery. Additionally, they may advise facial rehabilitation exercises. Because there is difficulty closing the eye, special care must be taken to avoid corneal injury. The person is educated about taking care of the eyes. An eye patch, lubricating eye drops, or ointments may be prescribed for the same.

People with Ramsay Hunt syndrome (RHS) may not recover completely, especially if treatment is not initiated within three days (72 hours) of symptom onset. Generally, fewer than half of the affected people will have a complete recovery.

Certain conditions are associated with a poorer prognosis, which includes:

• Age older than 60 years
• Diabetes mellitus
• High blood pressure
• Complete facial paralysis 

Complications of RHS may include:

• Synkinesis (unwanted contractions of the facial muscles during attempted movements)
• Permanent facial paralysis
• Postherpetic neuralgia (persistent nerve pain)
• Hearing loss
• Brainstem encephalitis (inflammation of the brainstem)
• Meningitis (inflammation of the coverings over the brain)
• Other cranial nerve involvement (polycranial neuropathy)
• Myelitis (inflammation of the spinal cord)