Ramsay Hunt Syndrome: What Is It, Causes

What Is It, Causes, Treatment, and More

Author: Anna Hernández, MD

Editor: Alyssa Haag

Editor: Emily Miao, PharmD

Editor: Kelsey LaFayette, DNP, ARNP, FNP-C

Illustrator: Jessica Reynolds, MS

Copyeditor: Stacy Johnson, LMSW

Modified: Jul 27, 2023

What is Ramsay Hunt syndrome?

Ramsay Hunt syndrome, also called herpes zoster oticus, is a rare complication of varicella-zoster virus reactivation, characterized by the triad of facial nerve palsy, ear pain, and a vesicular rash in the auricle or the ear canal. Although this syndrome occurs in less than 1% of varicella-zoster virus infections, it is the second most common cause of non-traumatic facial palsy after Bell palsy.

What causes Ramsay Hunt syndrome?

Ramsay Hunt syndrome is a late complication of the varicella-zoster virus (VZV), which causes chickenpox and shingles. After someone has had chickenpox, the virus can remain dormant in cranial nerves and dorsal root ganglia. Later in life, any stressful condition or illness may cause reactivation and replication of the virus, which then travels through the nerve fibers associated with the involved ganglion. With Ramsay Hunt syndrome, the virus affects the geniculate ganglion and causes symptoms along the territory of the facial nerve; however, other cranial nerves may also be affected. Although Ramsay Hunt syndrome may affect individuals of any age, it occurs more often in older and immunocompromised individuals.

What are the signs and symptoms of Ramsay Hunt syndrome?

Ramsay Hunt syndrome typically presents with facial nerve palsy, ear pain, and a painful rash with fluid-filled vesicles around the external ear canal and auricle. Ramsay Hunt syndrome may rarely occur without a skin rash known as zoster sine herpete.

The facial nerve palsy associated with Ramsay Hunt syndrome disrupts the motor fibers of the facial nerve, causing weakness on one side of the face. This can lead to difficulty performing facial movements, such as smiling, frowning, raising the eyebrows, puffing the cheeks, or whistling. Paralysis of the facial muscles may also cause the face to look different at rest on the affected side. For example, there can be a loss of the characteristic nasolabial fold or less wrinkling in the forehead compared to the unaffected side. Because the orbicularis oculi muscle is also affected, individuals may have difficulty closing their eyes, especially during sleep. This leads to an increased risk of exposure to keratopathy when the eye's cornea becomes damaged from prolonged exposure to the outside environment.

The vesicular rash typically affects the external ear canal and auricle; however, fluid-filled vesicles may also appear on the tongue and palate.

Additionally, other symptoms may be caused by the proximity of the facial nerve to other cranial nerves. Involvement of the vestibulocochlear nerve may result in hearing loss, tinnitus, and vertigo; hoarseness or aspiration may occur due to the involvement of the vagus nerve. Other less common symptoms include changes in taste, dry eye, tearing, increased sensitivity to sound, and nasal obstruction.

How is Ramsay Hunt syndrome diagnosed?

Ramsay Hunt syndrome is diagnosed clinically based on the typical clinical presentation of facial nerve palsy, ear pain, and a vesicular rash in the auricle or the ear canal. In individuals with a vesicular rash that does not accompany facial palsy, it can be challenging to distinguish between Ramsay Hunt syndrome and Bell palsy when there’s weakness or paralysis of the muscles on one side of the face due to an unknown cause. In cases with a high clinical suspicion of Ramsay Hunt syndrome, a sample of blister fluid may be taken to test for the presence of varicella-zoster virus via polymerase chain reaction (PCR).

How is Ramsay Hunt syndrome treated?

Ramsay Hunt syndrome treatment aims to decrease the duration of the symptoms and the risk of complications. It can include antiviral medications (e.g., acyclovir or famciclovir) and corticosteroids (e.g., prednisone) to decrease the associated inflammation. While treatment can be started anytime, it is more effective if it is started within 72 hours of the onset of symptoms. Individuals who have recovered from herpes zoster infection are encouraged to receive vaccination against the varicella-zoster virus to prevent recurrences and reduce the risk of complications, like post-herpetic neuralgia.

Additional measures include protecting the eye's cornea using artificial tears during the day and lubricating ointments at night. Individuals with severe facial paralysis may be instructed to tape the eye closed at night or wear an eye patch to protect the cornea from abrasions.

The facial palsy associated with Ramsay Hunt syndrome has a worse prognosis than that seen in Bell palsy, with only around 70% of individuals recovering completely. Some individuals with facial palsy may develop facial synkinesis, and aberrant innervation of the facial nerve leads to unwanted contractions of the facial muscles during attempted movement (e.g., eye closure when trying to smile). This complication can be treated with massage, physical therapy, botulinum toxin injections, or surgical procedures.

What are the most important facts to know about Ramsay Hunt syndrome?

Ramsay Hunt syndrome occurs due to the reactivation of the varicella-zoster virus on the territory of the facial nerve. The classic triad of facial nerve palsy, ear pain, and a vesicular rash in the ear canal or auricle characterizes it. Facial nerve palsy results in weakness on one side of the face, leading to difficulty smiling, raising eyebrows, or closing the eye. Treatment is aimed at decreasing the risk of complications and includes antiviral medications and corticoid therapy.

Coulson, S., Croxson, G. R., Adams, R., & Oey, V. (2011). Prognostic factors in herpes zoster oticus (Ramsay Hunt Syndrome). Otology & Neurotology, 32(6), 1025–1030. https://doi.org/10.1097/MAO.0b013e3182255727

Crouch, A.E., Hohman, M.H., Moody, M.P., et al. Ramsay Hunt Syndrome. [Updated 2022 Oct 13]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557409/

Epidemiology, clinical manifestations, and diagnosis of herpes zoster. (n.d.). Uptodate.com. Retrieved March 17, 2023, from https://www.uptodate.com/contents/epidemiology-clinical-manifestations-and-diagnosis-of-herpes-zoster

Gondivkar, S., Parikh, V., & Parikh, R. (2010). Herpes zoster oticus: A rare clinical entity. Contemporary Clinical Dentistry, 1(2), 127–129. https://doi.org/10.4103/0976-237X.68588

Monsanto, R. da C., Bittencourt, A. G., Bobato Neto, N. J., Beilke, S. C. A., Lorenzetti, F. T. M., & Salomone, R. (2016). Treatment and prognosis of facial palsy on Ramsay Hunt syndrome: Results based on a review of the literature. International Archives of Otorhinolaryngology, 20(4), 394–400. https://doi.org/10.1055/s-0036-1584267

Zainine, R., Sellami, M., Charfeddine, A., Beltaief, N., Sahtout, S., & Besbes, G. (2012). Ramsay Hunt syndrome. European Annals of Otorhinolaryngology, Head and Neck Diseases, 129(1), 22–25. https://doi.org/10.1016/j.anorl.2011.08.003

Ramsay Hunt Syndrome

What Is It, Causes, Treatment, and More

What is Ramsay Hunt syndrome?

What causes Ramsay Hunt syndrome?

What are the signs and symptoms of Ramsay Hunt syndrome?

How is Ramsay Hunt syndrome diagnosed?

How is Ramsay Hunt syndrome treated?

What are the most important facts to know about Ramsay Hunt syndrome?

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