Ramsay Hunt syndrome

Overview

Ramsay Hunt syndrome is a rare neurological disorder caused by the reactivation of the varicella-zoster virus (VZV), the same virus responsible for chickenpox and shingles. It primarily affects the facial nerve (cranial nerve VII) near the ear and is characterized by a combination of facial paralysis and a painful, blistering rash in or around the ear, mouth, or face. The syndrome can also involve the vestibulocochlear nerve (cranial nerve VIII), leading to hearing loss and balance problems.

Also known as herpes zoster oticus, Ramsay Hunt syndrome is considered a more severe cause of facial nerve palsy compared to Bell’s palsy and often results in prolonged recovery and a higher risk of residual symptoms. Early diagnosis and treatment are crucial in improving outcomes and minimizing complications.

Causes

Ramsay Hunt syndrome is caused by the reactivation of the varicella-zoster virus (VZV), which remains dormant in the body after an initial chickenpox infection. When reactivated—often triggered by stress, immunosuppression, or aging—the virus travels along the facial nerve and causes inflammation and damage.

The specific involvement of the geniculate ganglion of the facial nerve leads to the hallmark symptoms of Ramsay Hunt syndrome: facial paralysis and vesicular rash. If the infection also affects the vestibulocochlear nerve, it can result in hearing loss and balance disturbances.

Symptoms

The signs and symptoms of Ramsay Hunt syndrome usually appear suddenly and may vary in severity. Common symptoms include:

• Facial paralysis: Sudden weakness or paralysis on one side of the face, causing difficulty with facial expressions, blinking, or closing the eye

• Painful rash: Blistering rash in or around the ear (herpes zoster oticus), sometimes extending to the mouth, tongue, or soft palate

• Severe ear pain: Often precedes the rash and paralysis

• Hearing loss: Sensorineural hearing loss on the affected side

• Vertigo or dizziness: Caused by involvement of the balance portion of the vestibulocochlear nerve

• Tinnitus: Ringing or buzzing in the affected ear

• Dry eye and mouth: Due to impaired gland function controlled by the facial nerve

• Altered taste sensation: Especially affecting the anterior two-thirds of the tongue

In some cases, the rash may be absent (zoster sine herpete), making diagnosis more challenging.

Diagnosis

Diagnosis of Ramsay Hunt syndrome is primarily based on clinical evaluation. The combination of facial paralysis and a vesicular rash in the ear region is usually sufficient for diagnosis. Additional diagnostic steps may include:

• Physical examination: Assessment of facial muscle function and location of the rash

• Medical history: Previous history of chickenpox or shingles, recent stress or illness

• Polymerase chain reaction (PCR): Testing vesicle fluid or cerebrospinal fluid for VZV DNA

• Serological tests: May show elevated VZV antibodies, though not routinely used

• Imaging (MRI): In unclear cases or atypical presentations, MRI may help assess inflammation of cranial nerves

Ramsay Hunt syndrome must be distinguished from other causes of facial paralysis such as Bell’s palsy, Lyme disease, or stroke.

Treatment

Prompt initiation of treatment is crucial in Ramsay Hunt syndrome, ideally within 72 hours of symptom onset, to improve recovery outcomes. Treatment typically includes:

• Antiviral medications: Acyclovir, valacyclovir, or famciclovir to inhibit varicella-zoster virus replication

• Corticosteroids: Prednisone is commonly used to reduce nerve inflammation and swelling

• Pain management: Analgesics or anti-inflammatory drugs for ear pain and neuralgia

• Eye care: Artificial tears, lubricating ointments, or eye patches to protect the cornea if eyelid closure is impaired

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

In some cases, antiviral and steroid therapy may be combined for better effectiveness. Hospitalization is rarely required unless complications arise.

Prognosis

The prognosis of Ramsay Hunt syndrome varies based on the severity of nerve involvement and how quickly treatment is initiated. When therapy begins within 72 hours of symptom onset, the chances of complete recovery are significantly higher. However, full recovery is less common compared to Bell’s palsy.

Potential long-term complications include:

• Persistent facial weakness or asymmetry

• Chronic pain (postherpetic neuralgia)

• Permanent hearing loss or tinnitus

• Facial synkinesis (involuntary movements during voluntary muscle action)

Early diagnosis, aggressive treatment, and rehabilitative support play key roles in improving outcomes and reducing the risk of permanent deficits.