Ramsay Hunt syndrome type 2

Overview

Ramsay Hunt syndrome type 2, also known simply as Ramsay Hunt syndrome, is a neurological disorder caused by the reactivation of the varicella-zoster virus (VZV), the same virus that causes chickenpox and shingles. It is characterized by a painful, vesicular rash around the ear or mouth, facial paralysis on the same side of the face, and often hearing loss or vertigo. The condition results from the virus affecting the geniculate ganglion of the facial nerve (cranial nerve VII).

Ramsay Hunt syndrome type 2 is considered one of the more severe causes of facial nerve paralysis, with a more complicated recovery process compared to Bell's palsy. Prompt recognition and treatment are crucial to improving outcomes and minimizing long-term complications such as permanent facial weakness or auditory issues.

Causes

The primary cause of Ramsay Hunt syndrome type 2 is the reactivation of the dormant varicella-zoster virus in the geniculate ganglion of the facial nerve. After a person recovers from chickenpox, the virus remains dormant in nerve tissue and can reactivate years later, typically during periods of stress, immunosuppression, or aging.

When reactivated, the virus travels along the facial nerve and causes inflammation, leading to paralysis and the characteristic vesicular rash. The condition is not directly contagious, but the varicella-zoster virus itself can be transmitted to individuals who have not had chickenpox or the chickenpox vaccine, causing them to develop chickenpox rather than Ramsay Hunt syndrome.

Symptoms

The symptoms of Ramsay Hunt syndrome type 2 are usually unilateral (affecting one side of the face) and can vary in severity. The hallmark features include:

• Facial paralysis: Sudden weakness or paralysis of facial muscles on one side, leading to drooping, inability to close the eye, and asymmetrical facial expression

• Vesicular rash: Painful blister-like lesions on the ear (herpes zoster oticus), in the ear canal, or around the mouth and tongue

• Ear pain: Severe otalgia often precedes the rash and paralysis

• Hearing loss: Sensorineural hearing loss on the affected side

• Vertigo and dizziness: Due to involvement of the vestibulocochlear nerve (cranial nerve VIII)

• Tinnitus: Ringing in the ears

• Dry mouth and eyes: Due to impaired glandular function controlled by the facial nerve

• Altered taste perception: Especially in the anterior two-thirds of the tongue

In some cases, symptoms may be incomplete or occur in stages, making early diagnosis more challenging.

Diagnosis

Diagnosis of Ramsay Hunt syndrome type 2 is primarily clinical, based on the combination of facial paralysis and vesicular rash. Diagnostic steps may include:

• Clinical examination: Assessment of facial nerve function, rash distribution, and neurological signs

• Patient history: Recent or past history of chickenpox, shingles, or immunosuppression

• Polymerase chain reaction (PCR) testing: Detection of varicella-zoster virus DNA from vesicle fluid or cerebrospinal fluid

• Blood tests: May reveal elevated VZV antibodies, although not routinely needed

• Imaging (MRI): In unclear or severe cases, MRI may help assess inflammation of the facial nerve

Differential diagnosis includes Bell’s palsy, Lyme disease, acoustic neuroma, stroke, and other causes of facial nerve palsy or ear infections.

Treatment

Early and aggressive treatment is crucial for improving outcomes in Ramsay Hunt syndrome type 2. The primary goals are to limit viral replication, reduce nerve inflammation, and manage symptoms. Treatment strategies include:

• Antiviral medications: Acyclovir, valacyclovir, or famciclovir are prescribed to inhibit the virus; most effective when started within 72 hours of symptom onset

• Corticosteroids: Prednisone is commonly used to reduce inflammation of the facial nerve and improve recovery

• Pain management: NSAIDs, acetaminophen, or stronger analgesics for severe ear or facial pain

• Eye care: Artificial tears, eye patches, or lubricating ointments to prevent corneal damage if the eye cannot fully close

• Physical therapy: Facial exercises to maintain muscle tone and promote nerve recovery

Hospitalization may be necessary for severe or complicated cases, especially if the patient has difficulty eating, drinking, or protecting their eye.

Prognosis

The prognosis for Ramsay Hunt syndrome type 2 varies based on the severity of symptoms and how quickly treatment is initiated. With early treatment, especially within 72 hours of onset, many patients recover partially or fully. However, complete recovery rates are lower compared to Bell’s palsy.

Potential long-term complications include:

• Permanent facial weakness or paralysis

• Chronic pain (postherpetic neuralgia)

• Persistent hearing loss or tinnitus

• Facial synkinesis (involuntary movement during voluntary actions)

Early medical intervention and rehabilitation greatly improve the chances of recovery and reduce the risk of residual deficits. However, some patients may continue to experience symptoms for months or even permanently.