What is The Ramsey Hunt Syndrome (RHS)?

The Ramsey Hunt Syndrome (RHS) has gained increasing awareness since Justin Bieber’s diagnosis went public in June. As an audiologist, I immediately wondered if it would affect his hearing which could in turn possibly affect his career.

On the popstar’s Instagram, he showed how the syndrome has affected the muscles on one side of his face. He was unable to blink his right eye, wiggle his nose, or smile. Because of his sudden facial paralysis, Bieber chose to cancel his tour.

What is RHS?

Ramsey Hunt Syndrome (RHS) is a rare neurological disorder caused by the reactivation of the varicella-zoster virus, the same virus that causes chickenpox and shingles. The virus forms a painful, blistering rash and causes the facial nerves to swell and paralyze.

With most patients, the syndrome affects only one side of the face. Anyone who has had chickenpox or shingles is at risk of the virus reactivating; however, this syndrome is very rare, affecting only 5 out of every 100,000 people in the United States, according to National Organization for Rare Disorders.

How RHS Affects Hearing

Three symptoms of RHS are mild to moderate hearing loss, vertigo, and tinnitus, all resulting from a swollen vestibular nerve within the ear. As the syndrome affects the facial nerves, the cochlea malfunctions. The resulting hearing loss is usually temporary, but it may become permanent if left untreated.

In my experience, most medical providers tend not to refer for a hearing evaluation once RHS is diagnosed. Treatment for RHS may include antivirals and strong anti-inflammatory drugs. Because of the strong likelihood of cochlear damage resulting from RHS we recommend getting a hearing evaluation.  Be proactive about your hearing health. If you notice a change in your hearing, reach out to Hearing Solutions of North Carolina for the proper referrals and treatment.

Sources:

Kim CH, Choi H, Shin JE. Characteristics of hearing loss in patients with herpes zoster oticus. Medicine (Baltimore). 2016 Nov;95(46):e5438. doi: 10.1097/MD.0000000000005438. PMID: 27861389; PMCID: PMC5120946.

“Ramsay Hunt Syndrome.” Mount Sinai Health System, https://www.mountsinai.org/health-library/diseases-conditions/ramsay-hunt-syndrome. 

Sencen, Lisa. “Ramsay Hunt Syndrome.” NORD (National Organization for Rare Disorders), 22 June 2022, https://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/#:~:text=According%20to%20one%20estimate%2C%205,potentially%20develop%20Ramsay%20Hunt%20syndrome.