Ramsay Hunt syndrome is a condition that arises as a complication of shingles specifically in the facial nerves.^1-3 Shingles is caused by the varicella-zoster virus (VZV), the same virus responsible for chickenpox. The virus remains in the body of people who have had chicken pox but may be inactive (dormant) for a long period of time. When the virus becomes active again (reactivates), it causes shingles and can lead to Ramsay Hunt syndrome if the reactivation affects facial nerves. This causes a rash and paralysis on one side of the face, as well as other symptoms. If Ramsay Hunt syndrome is not treated quickly, it can lead to long-term complications.

Ramsay Hunt syndrome itself is not contagious, but people with this condition can spread the varicella-zoster virus, which can cause chickenpox in people who have not had it or have not been vaccinated.^2,3 If non-immune women catch the virus during pregnancy, this can cause a different rare condition, Congenital Varicella Syndrome (CVS), in their unborn child. People with Ramsay Hunt syndrome can spread the virus and are considered infectious until there is scabbing over the blistered rash.

There are vaccines available to protect against the varicella-zoster virus, with separate vaccines to reduce the risk of chicken pox and shingles which include related complications such as Ramsay Hunt syndrome.^4,5

Synonyms: Ramsay Hunt syndrome type 2, Ramsay-Hunt disease, herpes zoster oticus, geniculate ganglion herpes zoster, facial nerve palsy due to VZV, facial nerve palsy due to herpes zoster infection, facial nerve paralysis due to VZV.

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:3020 Ramsay Hunt syndrome 

ICD-11: 8B88.Y Ramsay Hunt syndrome

Many people with Ramsay Hunt syndrome experience general flu-like symptoms such as fever, aches, headaches, vomiting and tiredness as well as painful earaches for a few days before the other symptoms begin.^4,6 At the same time they may feel pain, itching, or tingling on and around the face before a rash emerges there. Hallmarks of Ramsay Hunt symptoms include:^3,4,6,7

• Facial palsy: weakness, sagging, or paralysis, usually only in one side of the face. This may affect the ability to close the eye or smile on the affected side
• Otalgia: hearing loss, earache, or ringing in one ear, which can be slight or intense
• Rash on the face, in or around an ear and/or mouth, or on one side of the face. The rash is usually blister-like and may be painful
• Vertigo: dizziness or the feeling of moving when holding still
• Dysgeusia: altered taste on one side of the tongue

Ramsay Hunt syndrome can also lead to altered taste, loss of vision as well as facial spasms and permanent facial weakness.^2,3 There may also be permanent damage in the affected nerves, resulting in chronic burning pain and sensitivity for long periods of time even after the rash has cleared (postherpetic neuralgia), which can be debilitating.

Please speak to your medical team to learn more about the symptoms and complications of Ramsay Hunt Syndrome.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

Ramsay Hunt syndrome is caused by reactivation of the varicella-zoster virus (VZV) in the facial nerves. Individuals that are infected with VZV will first develop chickenpox but even after they recover from chickenpox, the virus will remain inactive in their spinal cord.^1-3 The virus can reactivate, usually later in life, resulting in shingles. In cases where the reactivation affects facial nerves it can cause Ramsay Hunt syndrome.

Reactivation of varicella-zoster virus can be triggered by decreased immune system function. Immune function can decrease because of immunosuppressive medications, other diseases, stress and aging. Reactivation of the virus can occur more than once and individuals can get shingles multiple times.

Diagnosis of Ramsay Hunt syndrome is usually made based on medical history and presence of characteristic symptoms such as facial palsy and rashes or blisters in the ear, mouth or other parts of the face and hearing loss. Diagnosis can sometimes be delayed as the symptoms do not always occur at the same time and may appear similar to other conditions.^{1-4, 8}

A sample may be taken from a blistering rash to test for the presence of the varicella zoster virus to confirm the diagnosis. In the absence of a rash, blood or saliva can be tested; however, these tests may not be sensitive enough to detect the virus and are thus not necessary to make a diagnosis.²

MRI, electromyography and other nerve function tests may be conducted to assess the extent of nerve damage or to rule out other causes of facial weakness.^1,2 A differential diagnosis can rule out other conditions that may have similar symptoms, such as Bell’s palsy, acoustic neuroma or trigeminal neuralgia.^1,3,7

Please speak to your medical team to learn more about the available diagnostic pathways for Ramsay Hunt syndrome.

Treatment of Ramsay Hunt syndrome includes antiviral medications to fight the varicella-zoster virus and corticosteroids to reduce inflammation.⁹ Whilst Ramsay Hunt syndrome may resolve by itself over time, prompt treatment with antivirals can reduce the severity of the disease and reduce the risk of permanent complications and disability.² The outcomes of treatment vary between individuals and are greatly influenced by how soon treatment is started – it has been reported that the treatment is most effective if received within three days of symptoms starting.^1-3

There are also management strategies to cope with the symptoms of Ramsay Hunt syndrome.^3,7,9 This may include pain medication, medication for vertigo, eye lubrication to protect the eye from drying out and becoming damaged if an individual is unable to blink due to facial weakness, physiotherapy to regain control of facial muscles as well as other treatment to help with facial spasms and paralysis.

Vaccination against the varicella-zoster virus (also known as herpes-zoster virus) can help protect individuals from shingles and Ramsay Hunt syndrome. Immunisations against this virus are available in Australia, but the cost of immunisation may only be subsidised (funded under the National Immunisation Program (NIP)) for certain high-risk groups. For more information, please see Australian Immunisation Handbook: Zoster (Shingles).

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of Ramsay Hunt syndrome may include general practitioners (GP), otorhinolaryngologist/ENT specialists, neurologists, physiotherapists, and ophthalmologists.^7,8 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

The following guidelines are available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context and may not be up to date. These guidelines are for all forms of herpes zoster (shingles) presentation and complications, including Ramsay Hunt syndrome.

• S2k guidelines for the diagnosis and treatment of herpes zoster and postherpetic neuralgia were developed by an interdisciplinary group of German medical specialists (dermatologists, virologists, ophthalmologists, ENT physicians, neurologists, paediatricians and anaesthesiologists/pain specialists) using a formal consensus process (S2k); published in 2020. S2k guidelines are high quality guidelines which use formal methodological techniques.
• European consensus-based (S2k) Guideline on the Management of Herpes Zoster – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV), Part 2; published in 2016
• European consensus-based (S2k) Guideline on the Management of Herpes Zoster – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV), Part 1: Diagnosis; published in 2016

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

Timely treatment of Ramsay Hunt syndrome may reduce the possibility of long-term complications.^1-3

The Royal Victorian Eye and Ear Hospital Emergency Department: Clinical Practice Guideline on Seventh Cranial Nerve Palsy – this guideline was written for use specifically in The Royal Victorian Eye and Ear Hospital Emergency Department in Melbourne to guide the assessment and treatment of people presenting with seventh cranial nerve palsy, a hallmark of Ramsay-Hunt syndrome, under the guidance of an ophthalmology or ENT registrar.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

There are currently no known organisations in Australia specifically for this rare disease. If you know of any rare disease organisation/s supporting people living with Ramsay Hunt syndrome, please let us know via the Contribute page.

International Organisations:

Ramsay Hunt Syndrome Foundation (United States of America)
Website: https://www.ramsayhuntfoundation.org/

Facial Palsy UK (United Kingdom)
Website: https://www.facialpalsy.org.uk/causesanddiagnoses/ramsay-hunt-syndrome/

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Ramsay Hunt syndrome varies between individuals, and each person’s experience is unique.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Shingles, of which Ramsay Hunt syndrome is a complication of, is a notifiable disease in most states and territories in Australia. State and territory public health authorities should be informed or consulted about cases of varicella-zoster virus infection.^{4, 10}

Information about varicella-zoster vaccination, including other contraindications, can be found at:

• The Australian Immunisation Handbook: Varicella (chickenpox) vaccine information
• The Australian Immunisation Handbook: Zoster (herpes zoster) vaccine information

Further information about Ramsay Hunt syndrome can be found at:

• healthdirect: Shingles
• Genetic and Rare Diseases (GARD) Information Center:Ramsay Hunt Syndrome
• National Organization for Rare Disorders (NORD): Ramsay Hunt Syndrome
• Facial Palsy UK: Ramsay Hunt Syndrome

• Australian Family Physician: Acute unilateral facial nerve palsy
• Orphanet: Ramsay Hunt Syndrome
• Facial Palsy UK: Consensus Document – Recommendations for Supporting the Psychological Well-being of Children and Adults with Facial Palsy – consensus document developed by a panel of international psychology experts, including experts with lived experience of facial palsy, published 2021

This page has been developed by Rare Voices Australia (RVA)’s RARE Portal team.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.