When singer Justin Bieber announced recently that he has Ramsay Hunt Syndrome, a rare condition that has paralyzed half his face, the news raised interest and questions across the nation about this disorder linked to the varicella virus, which causes chicken pox and shingles.
Adding to the mystery behind Ramsay Hunt is its symptomatic similarity to Bell’s Palsy, a more common cause of facial paralysis. In both cases, most patients start recovering within days or weeks with appropriate treatment.
Ramsay Hunt Syndrome (RHS) is a rare condition where the varicella virus, the virus that causes chicken pox and shingles, infects the inner ear, resulting in the spread of infection to the nearby nerves. This occurs due to varicella reactivation in those that have had chickenpox or shingles in the past, and after the virus sits quietly in the body for some time.
Svetlana Faktorovich, M.D., a neurologist with Marcus Neuroscience Institute, established at Boca Raton Regional Hospital , part of Baptist Health, said she rarely sees cases of RHS, which accounts for 10 percent to 12 percent of all facial nerve disorders.
Bell’s Palsy is a much more common cause of facial paralysis, accounting for the majority of cases with spontaneous facial paralysis.
“The cause of Bell’s Palsy is unknown, but thought to be due to a viral infection or an inflammatory reaction to a viral infection that affects the 7th cranial nerve,” explains Dr. Faktorovich. “There are many viruses that are associated with Bell’s Palsy, including Herpes Simplex Virus (which causes cold sores), Epstein Barr Virus, COVID- 19, and others. Usually, it comes on as rapid onset, one-sided facial weakness or paralysis — and can be associated with pain around the ear on the same side. Risk factors include pregnancy, having an upper respiratory infection, high blood pressure.”
What are the primary differences between Ramsay Hunt and Bell’s Palsy?
“Unlike Ramsay Hunt Syndrome, other cranial nerves will not be affected in Bell’s Palsy and there is no rash inside the ear,” said Dr. Faktorovich. “In addition, Bell’s palsy tends to cause milder weakness of the face and improves faster. Unlike Ramsay Hunt syndrome, the vast majority of patients with Bell’s palsy will have a very good recovery.”
More on Ramsay Hunt Syndrome
Most often, RHS infects cranial nerve 7, also known as the facial nerve, which innervates the muscles of the face and partially controls taste perception. It can also affect cranial nerve 8, which is responsible for hearing and vestibular function, or your ability to tell where your body is in space, according to Dr. Faktorovich.
“When cranial nerve 8 is involved, that can make a person experience hearing loss, ringing of the ear, vertigo and nausea,” said Dr. Faktorovich. “The most common symptoms seen in RHS include facial paralysis on one side (including difficult closing the eye, raising the eyebrow and smiling), ear pain and rash inside the ear and/or tongue which can be seen by the clinician examining you. However other nearby nerves can be involved as well, in which case additional symptoms may be present.”
Risk factors for RHS include being over the age of 60 and having a compromised immune system. However, anyone with a history of chickenpox or shingles can develop Ramsay Hunt Syndrome.
How is Ramsay Hunt treated?
“Ramsay Hunt syndrome is treated with antiviral medications and steroids as soon as it diagnosed,” explains Dr. Faktorovich. “The anti-viral therapy is meant to treat the infection and the steroids to reduce the inflammation of the affected nerves. Early diagnosis and treatment are important to maximize the chance of recovery.”
What is the prognosis for Ramsay Hunt?
“Although severity and degree of recovery is notably worse than in Bell’s Palsy, many people with Ramsay Hunt syndrome do have significant symptom improvement and potential complete recovery,” said Dr. Faktorovich. “The earlier the diagnosis and treatment initiation, the more complete the recovery.
“In one study of 21 people with Ramsay Hunt, 75 percent showed complete recovery when treatment was started within three days. Meanwhile. 30 percent showed complete recovery when treatment was started after seven days. Of course, most studies on this condition are small in size which is a limitation.”