Ramsay Hunt Syndrome

Ramsay Hunt Syndrome, or Herpes Zoster Oticus, occurs when the shingles virus affects the facial nerve near one of the ears. Herpes zoster is a painful skin rash in a limited area of the body. The initial infection is varicella, or the chickenpox, and often occurs during childhood. After the infection is cleared, the virus lays dormant in nerve tissue near the brain and spinal cord. The virus can be reactivated and can cause a painful rash in the dermatome area of skin where the virus laid dormant.

The facial nerve is the 7th cranial nerve and controls muscles for facial expression, facial sensation, provides taste sensation, and increases saliva flow to salivary glands. The nerve emerges from the brainstem and reaches the temporal bone where it travels through the internal auditory canal, labyrinthine segment, intratympanic segment, and descending segment. The descending branch branches include the chorda tympani (taste nerve) and nerve for stapedius function (provides innervation for the stapedius muscle in the middle ear). The facial nerve passes through the parotid gland and divides into 5 different branches which include the temporal (innervates the muscle to raise the eyebrows), zygomatic (innervates the orbicularis oculi muscle for eye closure), the buccal (innervates the buccinators and orbicularis oris for mouth closure and cheek muscle activity), the mandibular (innervates the platysma), and the cervical branch.

Symptoms:

Shingles can erupt on any part of the body, but if it reactivates at the facial nerve it may cause Ramsay Hunt Syndrome, which presents with facial weakness, ear pain, and lesions of the external ear and skin of the ear canal. Some individuals also develop hearing loss, vertigo, and tinnitus (ringing of the ear). Of utmost importance is that the eye is cared for because with facial nerve weakness many people have difficulty closing the affected eye and thus are at risk of damage to the eye from the elements.

Ramsay Hunt syndrome affects about 10-15% of patients with acute facial weakness. Hearing loss and vertigo may occur and indicate that the infection has extended to the vestibulocochlear (hearing and balance) nerves.
Potential complications:

• Permanent hearing loss and facial weakness
• Eye damage- some patients are unable to completely close eye on the affected side and can lead to corneal irritation. 
• Postherpetic neuralgia- This occurs when the virus damages nerve at the dermatomic area of the infection and causes significant pain that can last long after the infection.

Work Up:

Physical exam and medical history lead the provider the correct diagnosis and there is usually no need for blood work or spinal taps. The provider can confirm the virus by collecting fluid from a blister with a viral swab. Blood and cerebrospinal fluid studies are rarely indicated; however, lyme titers are occasionally warranted to assess for paraneoplastic syndrome.

Imaging studies are sometimes recommended if symptoms do not completely recover after 90 days, recurrence, or if other cranial nerves are involved.

Treatment:

Early treatment will yield the best outcomes. It is important to start treatment within 48 hours of onset of symptoms to decrease pain and long term complications. Medications include:

Corticosteroids: Steroids provide anti-inflammatory effects and may give patients a higher rate of recovery. High dose steroids are typically prescribed to give the best chance of nerve recovery.

Antiviral therapy: Acyclovir or other antiviral medication is indicated to treat Ramsay Hunt Syndrome.

Pain Control medications: The pain associated with this infection is usually adequately controlled with Tylenol or Ibuprofen. At times, the neuralgia is treated with medications such as Neurontin or Lyrica.