What is Ramsay Hunt Syndrome?
Ramsay Hunt syndrome (herpes zoster oticus, and also known as geniculate neuralgia or nervus intermedius neuralgia) is a type of shingles outbreak that affects a facial nerve. In addition to the characteristic shingles rash, Ramsay Hunt syndrome can cause facial paralysis and one-sided hearing loss.
Ramsay Hunt syndrome is not the same as Bell’s palsy, another condition that can cause facial paralysis.
What are Symptoms of Ramsay Hunt Syndrome?
Symptoms of Ramsay Hunt syndrome include:
- Blistering rash in and around the ear on the affected side
- Pain deep in the ear
- Rash that usually appears before the facial paralysis
- Facial paralysis
- Usually most severe about 1 week following onset of symptoms
- Spinning sensation (vertigo)
- Hearing loss
- Ringing in the ears (tinnitus)
- Problems speaking
- Unsteady gait
- Swollen lymph nodes in the neck
- Secondary infection of blisters of the skin of the ear canal, outer ear, or both, resulting in cellulitis
What Causes Ramsay Hunt Syndrome?
Ramsay Hunt syndrome is caused by infection of the geniculate ganglion by herpesvirus 3 (varicella-zoster virus [VZV]). This is the same virus that causes chickenpox. Once a person has recovered from chickenpox, the virus remains in the body and can reactivate years later, causing shingles.
How is Ramsay Hunt Syndrome Diagnosed?
Ramsay Hunt syndrome is usually diagnosed by clinician observation of the key characteristics of the disease. Lab tests that may be indicated include:
- Isolation of varicella zoster virus (VZV) from vesicle fluid
- White blood cell (WBC) count
- Erythrocyte sedimentation rate (ESR)
- Serum electrolytes
- Spinal fluid analysis
- CNS imaging studies
Imaging studies that may be used include:
- Computerized tomography (CT) scan
- Magnetic resonance imaging (MRI)
- Magnetic resonance angiography (MRA)
- Transcranial magnetic stimulation (TMS)
Other tests to help diagnose Ramsay Hunt syndrome include:
- Audiometry for hearing loss in high frequency ranges
- Electronystagmography (ENG)
- Facial motor nerve conductions studies (electroneurography)
- Electromyography of facial innervated muscles, the blink reflex, and nerve excitability testing
What is the Treatment for Ramsay Hunt Syndrome?
Ramsay Hunt syndrome treatment is aimed at minimizing disability and relieving symptoms. In most cases, the sooner treatment is initiated, the better the outcome.
Medications used to treat Ramsay Hunt syndrome include:
- Oral corticosteroids
- Oral acyclovir
- Intravenous high-dose methylprednisolone (not commonly used but may provide some benefit)
- Controlled-release oxycodone for acute pain
- Vestibular suppressants if dizziness or vertigo is severe
- Local anesthetic or topical cocaine for ear pain
- Carbamazepine for nerve pain
Certain specialists may need to be consulted depending on symptoms:
- Infectious disease specialist
- Neurosurgeon or otolaryngologist if structural lesions are found
- Ophthalmologist for eye care
What are Complications of Ramsay Hunt Syndrome?
Complications of Ramsay Hunt syndrome are uncommon and may include:
- Permanent hearing loss
- Permanent facial paralysis
- Brainstem encephalitis
- Cranial nerve XII involvement
- Damage to the cornea of the eye
- Nerve pain from shingles (postherpetic neuralgia)
Factors that contribute to a poor prognosis include:
- Age older than 50 years
- Complete facial paralysis
- Lack of CN VII nerve excitability
How do you Prevent Ramsay Hunt Syndrome?
There is a vaccine for chickenpox that can reduce the risk of being infected with the herpes zoster virus that causes it. People who are 50 years and older are also advised to receive a shingles vaccine.