My Journey with Ramsay-Hunt Syndrome

Pain in the ear, eye, mouth, and face can be a sign of Ramsay Hunt Syndrome, a rare complication of shingles that affects the facial nerves. A constant burning sensation suggests nerve damage from shingles, while a swollen feeling can indicate paralysis. Getting prompt treatment with antiviral medication and steroids can lead to a better outcome. Shingles does not always appear with blisters. Starting antiviral treatment within three days of nerve pain onset can help reduce nerve damage and improve recovery. Research shows that IV steroids may boost outcomes even up to two years later. According to an infectious disease specialist, IV acyclovir and IV steroids work faster and more effectively than oral versions. A two-month course of antivirals and steroids was recommended in 2013 when I had my first RHS episode. Since swelling can cause more damage, steroids help reduce it, while antivirals stop further harm from the shingles virus, which attacks the nerve’s myelin sheath. It’s worth discussing a two-month treatment plan with a doctor and possibly consulting an infectious disease specialist or neurologist. Self-advocacy is key, as many doctors may be unfamiliar with RHS due to its rarity. Even after the first three days of symptoms, IV steroids may still offer benefits.

They put me on IV steroids and antivirals in the hospital, then kept me on them after inserting a port so visiting nurses could come by and teach me how to administer them myself at home. During my second episode of R.H.S., my facial paralysis cleared up within two days on the IV medication. On the oral medication, I only improved from 84% to 64% over 18 months.

According to my former, highly experienced neurologist, most recovery happens within the first 18 months, with potential for continued progress up to two years. How much you improve depends on the extent of nerve damage caused by the virus before starting antiviral and steroid treatment, and whether symptoms stem from swelling or actual damage. Starting medication within three days gives about a 50/50 chance of full recovery. Receiving IV medication instead of oral increases those chances because it works faster. If symptoms are due to swelling rather than damage, full recovery is possible. IV steroids can boost recovery odds even if not given immediately.

Ramsay Hunt Syndrome (RHS) affects the ear and the vestibular nerves. As a result, it can often be diagnosed and treated by either an ear, nose, and throat (ENT) doctor or a neurologist. However, specialists for Ramsay Hunt Syndrome are not very common. Due to its effects on the ears, eyes, and nerves, patients need assistance from three specialists: an ENT doctor, a neurologist, and a neuro-ophthalmologist.

An ENT may recommend vestibular testing and refer you to a specialist if necessary. Physical therapists can help manage any vestibular damage. Meanwhile, neuro-ophthalmologists focus on addressing eye issues, which is crucial for preventing complications such as dryness from lack of blinking or vision problems associated with RHS.

Dizziness and balance issues are common symptoms of vestibular damage. You may also experience hearing and vision problems. I underwent vestibular physical therapy to improve my balance because I struggled with nausea and vertigo. An ENT (Ear, Nose, and Throat) specialist may refer you to an ENT specialist who does vestibular testing. This testing involves a series of assessments that evaluate the vestibular system, which controls balance and works with the eyes to maintain clear vision while the head is moving. These tests help determine any damage to the vestibular portion of the inner ear, which can cause dizziness, vertigo, or balance problems. I worked with a physical therapist specializing in vestibular rehabilitation, who taught me how to regain my balance. Since the vestibular function on my left side was compromised, my right side had to compensate for the loss. I also took medication to manage the nausea. Any doctor can prescribe physical therapy, but it’s crucial to seek a vestibular physical therapist for specialized vestibular rehabilitation. This type of therapy is essential for helping the unaffected side adapt and balance for the loss of function on the affected side.

Scheduling an appointment with a neurologist is recommended, as they can refer you to a specialist for an electromyography (EMG) test to evaluate the extent of paralysis. An EMG is a diagnostic tool that assesses the health of muscles and the nerve cells controlling them. It helps identify problems like nerve dysfunction, muscle issues, or signal transmission between nerves and muscles.

I purchased a moisture chamber for my eye when it wouldn't close. This device acts as a protective bubble over the eye until normal blinking returns. If you have an eye condition, it’s advisable to consult a neuro-ophthalmologist who is experienced with Ramsay Hunt Syndrome. My neuro-ophthalmologist recommended using Optase MGD Advanced Dry Eye Drops and Hylo Night Eye Ointment. It took several months for my eye to start 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 started my treatment at 300 mg, but due to excessive sleepiness, the dosage was reduced to 100 mg. Gradually, I increased the dosage to 300 mg. The dosage increased to 600 mg and eventually to 900 mg, taken three times a day. Before 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, 60 mg of duloxetine, and 10 mg of hydrocodone as needed for severe facial and eye pain. My medication was initially prescribed by a palliative care doctor. I now have a pain doctor whom I highly recommend to anyone suffering from nerve pain. For several years, I have been taking amitriptyline, which helps alleviate nerve pain and improve my sleep. Managing nerve pain is notoriously challenging, and pain management and palliative care specialists often prescribe medications that other physicians may be hesitant to use.

The nerve pain associated with Ramsay-Hunt syndrome is severe. Despite all my medications, I still experience intense discomfort. The lower eyelid of the affected eye and my face endure excruciating pain, as if someone is pulling my face upward. A constant burning sensation affects my face and eye, and any movement of my eye triggers pain. It often feels like something is pulling at the affected areas. It constantly feels like something is in my eye.

The medication helps to reduce my pain, but it does not eliminate it. I would be unable to function without it. I experience intense eye pain and vision problems. My nystagmus causes everything to appear as if it is spinning, similar to a merry-go-round. Tracking objects is difficult, and my vision is often blurry. Additionally, the eyelid of the affected eye causes significant discomfort, making it hard to keep the eye open without taking substantial pain medication.

Palliative care, a branch of hospice, is specialized medical care that focuses on providing relief from pain and other symptoms from 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. Palliative care is offered in conjunction with other treatments you may be undergoing. It involves a team of healthcare professionals, including doctors, nurses, social workers, chaplains, and specialized practitioners. This team works collaboratively with you, your family, and your other healthcare providers to provide extra support and relief that complements your ongoing care. To find a palliative care doctor, you need a referral from one of your doctors.

During my first dental visit after experiencing Ramsay Hunt Syndrome (RHS), I discovered that I had six cavities on the paralyzed side of my mouth. To resolve this issue, I bought a Waterpik to help remove food that I couldn’t clear on my own. Additionally, I got an electric toothbrush, and now I use both devices after every meal to prevent tooth loss, which is a common complication of RHS.

Reoccurring shingles

The virus that causes shingles is the same one responsible for chickenpox and remains in the body for life. Research indicates that stress can trigger outbreaks of shingles. If shingles occurs near the ear or face, it can lead to Ramsay Hunt syndrome. The shingles virus becomes active when the immune system is weakened. Stress triggers the release of adrenaline, activating the body's fight-or-flight response, which can suppress the immune system to prioritize immediate action. Additionally, shingles can cause nerve damage in any area 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, a non-live virus vaccine, is over 91% effective and seems 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 both an oncologist and an immunologist.

Learning stress management techniques, particularly how to relax and calm your adrenaline, is beneficial. 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 started with ear pain. I went to urgent care, where they thought it was an ear infection and gave me antibiotics. A week later, I returned, and on the tenth day, I woke up with the left side of my face paralyzed. It looked and felt swollen, which I realized later was the paralysis. I had severe nausea, vision issues, and trouble walking. A friend took me back to urgent care, but they couldn’t figure out the cause, so they sent me to an ear, nose, and throat specialist. I was diagnosed with Ramsay Hunt Syndrome, prescribed Valtrex and prednisone, and referred to vestibular physical therapy. Unfortunately, the medication came too late for a good recovery. My facial paralysis worsened from 84% to 64%, though I regained a partial blink. I have intense pain in my left eye and face, constant dizziness like being on a merry-go-round, and ongoing balance problems. I also have nystagmus, vision issues, and 24/7 vertigo. During my second episode, the first symptom I experienced was complete facial paralysis on the right side. After insisting they consult with an infectious disease specialist, I was started on IV acyclovir and steroids at the hospital, and the paralysis improved over the next two days. Despite this, both the hospital doctor and my primary care doctor refused to believe I could have Ramsay Hunt twice, even after I developed visible shingles while hospitalized. They would not continue the medication two days later, after I was discharged. My balance problems became more severe; I needed a walker, and my vision and nystagmus worsened. I had to return to vestibular physical therapy. In this episode, I didn’t receive the medication long enough to see a positive outcome. About nine days later, a neurologist identified the RHS and prescribed the antiviral and steroid, though it was quite late in the process. I am a marriage and family therapist who 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 on my eyelid.

A friend helped me apply for disability by entering all the information into the online application. I collected my doctor’s notes, and she delivered them to the disability office. Another friend, an attorney who assists people with disability claims in another state, advised me not to downplay my symptoms with my doctors since their notes reflect what you say—and that’s what disability doctors will read when reviewing your records. I contacted my state representative and got help from someone in their office who supports people applying for disability. I was approved on my first attempt.

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, 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, even though 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 receive the medication for a long enough duration to achieve a positive outcome. Although I can walk independently, I am experiencing more severe balance problems. My eyes do not blink in unison; my left eye blinks far less frequently than my right. Additionally, both of my eye corneas are damaged from prolonged dryness. The connection between my eyes and brain is impaired. As a result, the images I see are diagonal rather than side by side, preventing them from merging properly. My neuro-ophthalmologist has advised me to wear prism glasses continually to improve my vision. I underwent vision therapy that improved my vision and balance. I wish I had done it sooner. Research to see if you have vision therapy in your area if your vision or balance was affected by Ramsay-Hunt Syndrome.

Shingles on my neck affected my brain, resulting in cerebellar ataxia. Shingles damages part of the cerebellum, which 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 increased difficulty talking, out of only the right side of my mouth.

I feel shocked every time I look in the mirror and see my face. My face appears crooked, and it feels like my brain doesn't recognize my current appearance. I'm so self-conscious about it that I avoid using FaceTime entirely because I can’t stand seeing my uneven mouth when I talk.

Inspired by my fellow warriors, I decided to start a blog. I live with facial pain caused by a rare complication of shingles called Ramsay Hunt Syndrome, which has left one side of my face paralyzed and in constant pain. Despite this, I work as a therapist and want to share the knowledge I’ve gained in hopes of helping others. Check out my blog, Coping with Illness and Pain.