My Journey with Ramsay-Hunt Syndrome

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 is for a better prognosis. Shingles don't always present with blisters. Initiating antiviral treatment within three days of nerve pain onset can reduce nerve damage and improve prognosis. Evidence suggests that IV steroids can enhance prognosis even two years later. An infectious disease specialist has indicated that IV acyclovir and IV steroids are quicker and more efficacious than their oral counterparts. It is recommended to continue antivirals and steroids for two months. Increased swelling correlates with more damage; steroids minimize swelling, while antivirals prevent further damage from the shingles virus, which destroys the nerve's myelin sheath. It is advisable to discuss a two-month medication regimen with your doctor and to seek information or a consultation with an infectious disease specialist or neurologist. Self-advocacy is crucial, especially since many doctors may not have encountered RHS due to its rarity. IV steroids are reported to be beneficial even beyond the initial three days of symptoms. 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 R.H.S. 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 two 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 three 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 works sooner. If your symptoms are because of swelling and not damage, you can fully recover. Getting IV steroids can improve your chances of recovery, even if it's not in the beginning.

RHS impacts both the ear and vestibular nerves, so either an ear, nose, and throat doctor or a neurologist may be equipped to diagnose and treat RHS. Specialists for RHS are rare. RHS affects the ears, vision, and nerves, necessitating three types of specialists: an ENT, a neurologist, and a neuro-ophthalmologist. An ENT may refer you to a specialist for vestibular testing. Physical therapists manage vestibular damage, while neuro-ophthalmologists address eye issues, preventing damage if an eye does not blink, is dry, or has vision problems due to RHS.

Dizziness and trouble with balance are the most common symptoms of vestibular damage. You can also 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 doctor can prescribe physical therapy. It is important to seek a vestibular physical therapist for vestibular rehabilitation, which is essential in teaching the unaffected side to compensate and balance for the loss on the affected side.

Scheduling an appointment with a neurologist is advisable. They can refer you to a specialist for an EMG to determine the extent of paralysis. An EMG, or electromyography, is a diagnostic test that evaluates the health of muscles and the nerve cells that control them. It can identify issues such as nerve dysfunction, muscle dysfunction, or problems with nerve-to-muscle signal transmission.

I purchased a moisture chamber for my eye when it wouldn't close. It's akin to a protective bubble worn over the eye until blinking is possible. If you have an eye condition, consult a neuro-ophthalmologist familiar with Ramsay-Hunt Syndrome. My neuro-ophthalmologist suggested using Optase MGD Advanced Dry Eye Drops and Hylo Night Eye Ointment. It was several months before my eye started blinking again.

Gabapentin is commonly prescribed to treat nerve pain associated with RHS, TN, and other neurological disorders. Though initially developed for epilepsy, its efficacy in nerve pain comes from its nerve-calming effects. My neurologist began my treatment with 300 mg, but due to excessive sleepiness, it was reduced to 100 mg. Gradually, I increased the dosage to 300 mg, then 600 mg, and eventually to 900 mg, taken three times daily. Prior to switching to Lyrica, my regimen included 1800 mg of Gabapentin three times a day. I introduced Pregabalin, the generic form of Lyrica, which is specifically formulated for nerve pain, and it significantly alleviated my pain.

I am currently taking 300 mg of pregabalin three times a day, 20 mg of baclofen three times a day, 30 mg of duloxetine, and 10 mg of hydrocodone as needed for severe facial and eye pain. My medication is prescribed by a palliative care doctor, whom I highly recommend to anyone suffering from nerve pain. For several years, I have been taking amitriptyline, which aids in nerve pain and sleep. Nerve pain is notoriously challenging to manage. Palliative care physicians often prescribe medications that other doctors may hesitate to prescribe. The nerve pain associated with Ramsay-Hunt syndrome is severe. Despite all my medications, I continue to experience intense pain. The lower eyelid of the affected eye and my face endure extreme pain, as if someone is yanking my face upward. A constant burning sensation plagues my face and eye, and any movement of my eye causes pain. It perpetually feels as though there is something in my eye. The medication reduces my pain but does not completely remove it. I would be unable to function without it. I suffer from intense eye pain and vision issues. My nystagmus makes everything appear as if it's spinning like a merry-go-round. Tracking is challenging, and my vision is blurry. The eyelid of the affected eye causes significant pain, making it hard to keep the eye open without substantial 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. 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. During my first dental visit following RHS, I discovered six cavities on the paralyzed side of my mouth. To address this, I purchased a water pick to remove excess food that I couldn't clear by myself. Additionally, I acquired an electric toothbrush and now use both devices after every meal to prevent tooth loss, a frequent complication of RHS.

Reoccurring shingles

The virus responsible for shingles originates from chickenpox and cannot be eliminated from the body. Research indicates that stress can trigger shingles. If it manifests near the ear or face, it may lead to a subsequent episode of Ramsay-Hunt syndrome. The shingles virus becomes active when our immune system is compromised. Stress induces adrenaline, triggering our fight-or-flight response, which in turn suppresses our immune system to allocate resources for immediate action. Shingles has the potential to cause nerve damage in any part of the body where it appears.

I've experienced three episodes of RHS: the first on my left side in July 2013, the second on my right in 2021, and the third on my left side during Christmas 2023. Due to recurrent shingles, I consult an infectious disease specialist and take 1 gram of Valtrex daily, a regimen I'll continue indefinitely. Initially, my doctor advised against the vaccine until a non-live virus option was available. Shingrix, which is not a live virus vaccine and is over 91% effective, seemed a viable choice, reducing the risk of contracting shingles to 9% after two doses. Despite the $200 cost with insurance, I proceeded with the vaccination on my doctor's recommendation. My recurring shingles likely stem from a compromised immune system, evidenced by my IgA immune deficiency and a separate white blood cell disorder that produces abnormal cells. For these conditions, I'm under the care of an oncologist and an immunologist, respectively.

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. Both yoga and tai chi are excellent for reducing stress.

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 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 I 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 steroids 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. I am currently in vision therapy which is helping not just my vision but it is helping with my balance. I wish I had done it sooner. Research your area for vision therapy if your vision or balance was affected by Ramsay-Hunt Syndrome.

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.

My third episode started with 2 shingles in my left ear. Soon after, the left side of my face became more paralyzed and I have a more difficult time talking and I talk out of only the right side of my mouth.

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.