My Journey with Ramsay-Hunt Syndrome
Updated: Aug 11
Face pain in your ear, eyes, nose, and mouth pain could be Ramsay-Hunt Syndrome, a rare complication of shingles that damages the trigeminal nerve. Constant burning pain indicates nerve damage from shingles. The swollen feeling is paralysis. Prompt treatment with an antiviral and steroids are indicated for a better prognosis. Shingles don't always present with blisters. If you get on the antiviral within 3 days of the nerve pain, it reduces the amount of damage done to the nerves and improves your prognosis. There is evidence that IV steroids improve prognosis even after 2 years. I learned from an infectious disease doctor that IV acyclovir and IV steroid is faster and more effective than oral medication. Antivirals and steroids should be continued for 2 months. The more swelling, the more damage. The steroid is to keep down swelling, and the antiviral is to stop the shingle virus from doing more damage. The shingles virus destroys the myelin around the nerve, so it no longer functions. Advocate to be on both medications for 2 months with your doctor. Ask them to look it up or consult with an infectious disease doctor or neurologist. Don't be afraid to advocate for yourself. Many of the doctors have never seen a case of RHS because it's so rare. There is evidence that IV steroids improve prognosis even after the 1st 3 days. They started me on IV steroids and antiviral in the hospital and continued after inserting a port so I could have visiting nurses at home teach me how to administer it at home. In my 2nd episode of RHS my paralyzed face resolved within a day on the IV meds whereas on the oral medication, the paralysis only went from being 84% paralyzed to 64% in 18 months and never improved beyond that. According to my prior, very experienced neurologist, most of your progress will occur in the 1st 18 months and you can progress further for 2 years. The amount of progress depends on how much damage the virus does to the nerves before you start on the antiviral and steroids. And whether the symptoms are due to swelling or damage. If you get on the medication before 3 days, you have a 50/50 chance of full recovery. If you received your medication IV instead of oral, your chances of recovery are higher because IV medication gets to you faster and it works sooner. If your symptoms are because of swelling and not damage, you can have a full recovery. Getting IV steroids can improve your chances of recovery, even if it's not in the beginning.
Because RHS affects both ear, vestibular, and nerves either ear, nose, and throat doctor or a neurologist may have training or experience diagnosing and treating RHS. It's too rare to have a specialist that just treats it. RHS affects our ears, vision, and nerves, we need 3 specialists. An ENT, a neurologist, and a neuro-ophthalmologist. An ENT can further refer you to an ENT specialist who tests vestibular damage. Physical therapists treat vestibular damage. Neuro-ophthalmologists treat your eye and prevent damage to the eye if one eye doesn't blink, has less moisture in your eye, or has vision problems from RHS.
Dizziness and trouble with your balance are the most common symptoms of vestibular damage, but you also can have problems with your hearing and vision. I did vestibular physical therapy for my balance. I had nausea and vertigo. Your ENT should further refer you to an ENT specialist who does vestibular testing. Vestibular testing is a series of tests that evaluate the vestibular system, which controls balance and works with the eyes to maintain clear vision when the head is moving. The tests determine damage to the vestibular portion of the inner ear, which causes dizziness, vertigo, or balance problems. I had a physical therapist who specialized in vestibular rehabilitation. Vestibular physical therapy taught me how to balance. My right side had to learn to compensate for the destroyed vestibular functioning on the left side. I took medication for the nausea.
Any of your doctors can order physical therapy. Find a vestibular physical therapist to do vestibular rehabilitation. Vestibular rehabilitation is crucial to teach the unaffected side how to accommodate and balance the loss of the affected side.
Get an appointment with a neurologist. They can then also refer you for an EMG by a specialized neurologist to discover the extent of the paralysis. An EMG is a diagnostic procedure to assess the health of muscles and the nerve cells that control motor neurons. The results can reveal nerve dysfunction, muscle dysfunction, or problems with nerve-to-muscle signal transmission.
I bought a moisture chamber for my eye when it was not closing. This is like a bubble to wear over the eye until I could blink. See a neuro-ophthalmologist that has experience with Ramsay-Hunt Syndrome for your eye. My neuro-ophthalmologist recommends Optase MGD advanced dry eye drops and Opase Hylo Night eye ointment. It took many months for my eye to blink.
Gabapentin is used most often to treat nerve pain from RHS, TN, and other nerve disorders. Although originally designed for seizures it works because it calms the nerves. My neurologist originally started me at 300 mg, but I had to have him lower it to 100 mg because it made me too sleepy, and then I worked my way up to 300 then 600 mg, then 900 mg 3 times a day. Before I tried Lyrica, I was on 1800 mg of Gabapentin 3x a day. I added Pregabalin the generic of Lyrica because it was created specifically to treat nerve pain and after trying it, it took my pain down.
I am now on 300 mg of pregabalin 3x a day, baclofen 20 mg 3x a day, duloxetine 30 mg, gabapentin 600 mg 3x a day, and hydrocodone 10 mg as needed for severe face and eye pain. The palliative care, doctor prescribes my medication. I highly recommend them to anyone suffering from nerve pain. I took amitriptyline which helps with nerve pain and sleeping for several years. Nerve pain is the most difficult to treat. Palliative doctors will prescribe medication that other doctors are uncomfortable with. The nerve pain from Ramsay-Hunt is extreme. I am still in pain even on all my medications. I have extreme pain in the lower eyelid of the affected eye besides the facial pain where it feels like someone is pulling up my face. My face and eye burn constantly, and it hurts when I move my eye. I always feel like something is in my eye. The medication brings my pain down but doesn't eliminate it. I couldn't function without it. I have severe eye pain and vision problems. I have nystagmus so that everything looks like I'm on a merry-go-round. I have difficulty tracking. It's blurry. The eyelid of the affected eye is very painful. It's difficult to keep my eye open without a lot of pain medication. Palliative care, a branch of hospice, is specialized medical care that focuses on providing relief from pain and other symptoms of a serious illness. They can also help you cope with side effects from medical treatments. Their availability does not depend on whether your condition can be cured and it does not have to be life-threatening. Their teams aim to provide comfort and improve the quality of life for people with chronic illness and pain. This form of care is offered alongside other treatments a person may be receiving. Palliative care is provided by a team of healthcare providers, including doctors, nurses, social workers, chaplains, and other trained specialists. The team works with you, your family, and your other providers to add an extra layer of support and relief that complements your ongoing care. To find a palliative care doctor, you just need to be referred by any one of your doctors. At my 1st dentist appointment after RHS, I had six cavities on the side of my mouth that is paralyzed. I bought a water pic to get the excess food out of my mouth that I couldn't do on my own. I bought an electric toothbrush and use both after every meal to prevent me from losing teeth which is a common consequence of RHS.
The virus that causes shingles comes from chicken pox and can not be cleared from your body. Literature suggests that stress triggers shingles. If it occurs near your ear or face, it can cause another episode of Ramsay-Hunt. The shingles virus erupts when our immune systems are down. Stress triggers adrenaline, our flight or fight response and when that happens, our immune systems are turned off to channel our resources to fight or flee. Shingles can cause nerve damage, wherever it occurs in the body.
I had two episodes of RHS. My first RHS episode was on the left side in July 2013 and my second on the right was in 2021. I see an infectious disease doctor because I have reoccurring shingles. I am prescribed 1 gram of Valtrex daily which I will take for the rest of my life. My infectious disease doctor did not want me to have the vaccine until the shingle vaccine was not the live virus. Unlike Zostavax, Shingrix does not contain a live virus. Shingrix is +91% effective. Therefore, you only have a 9% chance of getting shingles after the two-shot Shingrix. My infectious disease doctor was pushing me to have the vaccine, so I did it even though it cost $200. with my insurance. I have not had shingles since I had the vaccine. I think I had recurring shingles because of an underlying compromised immune system. I have an IgA immune deficiency which is the white blood cells that attack invaders in the eyes, nose, mouth, gut, and colon. I also have a white blood cell disorder that makes abnormal white blood cells. I have an oncologist for the blood disorder. I see an immunologist for IgA deficiency.
Learning stress management techniques particularly how to relax and turn off your adrenaline is helpful. Imagine you are in a relaxing place. Take deep breaths and learn deep breathing techniques. Yoga or tai chi are great stress reducers.
My Ramsay-Hunt Syndrome symptoms started with my ear hurting. I went to the urgent care center where they thought I had an ear infection and gave me an antibiotic. A week later, I went back a second time and then on the tenth day, I woke up to find my face paralyzed on the left side. It looked and felt swollen to me. I had severe nausea, vision problems, and difficulty walking. I had a friend drive me back to urgent care and this time they did not know what was wrong so they sent me to an ear, nose, and throat specialist. He diagnosed Ramsay-Hunt Syndrome and prescribed Valtrex and prednisone and gave me a referral to vestibular physical therapy. I did not fully recover. The medication was too late for a positive outcome. My face went from 84% to 64% paralysis. I regained a partial blink. I have severe pain in my eye and face on the left side. I feel and it looks like I'm on a merry-go-round. I learned how to balance and walk independently but still have balance problems. I have nystagmus, vision problems, and 24/7 vertigo. In the 2nd episode, my 1st symptom was complete facial paralysis on the right side, and I started on IV acyclovir and steroid at the hospital after my insistence. My facial paralysis on that side subsided right away but the hospital doctor refused to believe I could get Ramsay-Hunt twice, even after I broke out with the shingles in the hospital, so they wouldn't continue the medication after I left the hospital, 2 days later. A few days later, I developed more severe balance problems, I had to use a walker and my vision and nystagmus got worse. I had to go to vestibular physical therapy again after the 2nd episode. In this episode, I didn't get the medication long enough for a positive outcome. I am a marriage and family therapist, and I was in private practice until I had to file for disability in 2013 after the first Ramsay-Hunt episode. I kept my office for four years hoping to go back to work. I have severe pain on the left side of my face and my eyelid.
A friend helped me apply for disability, by typing all the information, using the application online. I gathered my doctor's notes, and she dropped them off at the disability office. Another friend, an attorney who helps people file disability in a different state told me not to minimize my symptoms with my doctors because their notes consist of what you say. And that's what disability doctors will read when they review your medical records. I called my state representative and asked for help from someone in their office who helps people gain disability. I got accepted the 1st time.
I woke in 2021 with the right side of my face paralyzed and I was having trouble balancing. I went to the emergency room, but the hospital doctor did not believe I could get Ramsay-Hunt twice because it's so rare. They wouldn't give me the antiviral and the steroid but admitted me to rule out a stroke. After my insistence that they called an infectious disease specialist. I was placed on IV acyclovir and steroids for the two days I was hospitalized. The hospital doctors would not continue the medication despite that I broke out in shingles on my face, neck, and back in the hospital. My facial paralysis on the right side and the balance problems mostly subsided. A few days later, I developed more balance problems and had to use a walker. My vision and nystagmus got worse. I had to go to vestibular physical therapy again. In this episode, I didn't get the medication long enough for a positive outcome. I can walk independently but I was left with more severe balance problems. My eyes do not blink together, and my left eye still doesn't blink nearly as often as the right. Both eye corneas are damaged from being so dry for so long. The eye-brain connection was damaged. My eye's images are diagonal rather than side by side so that the images don't come together. My neuro-ophthalmologist recommends prism eyeglasses that I will have to wear all the time to help my vision. My appointment is in June to have the testing done.
My brain was affected by shingles on my neck called cerebella ataxia. Shingles damage part of the cerebellum that affects balance and cognition. I went to cognitive therapy.
I feel like I'm shocked every time I look in the mirror and my face is crooked like my brain did not retain what I looked like now. I am so self-conscious about it that I don't do Facetime at all because I can't tolerate seeing my crooked mouth talking.
I was inspired by my fellow warriors to write a blog. Although I am a facial pain sufferer from this rare complication of shingles called Ramsay-Hunt Syndrome that left one side of my face paralyzed and left my face in severe pain, I am also a therapist, so I want to share some of the knowledge I've accumulated in hopes of helping others. Please see my blog Coping with Illness and Pain.