Ramsay Hunt Syndrome

Emergency Management

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.

Clinical Care Guidelines

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.

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

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

Personal Stories

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

If you would like to share your personal story with Rare Voices Australia and have it included on the RARE Portal, please visit Rare Voices Australia: Share Your Story.

Symptoms

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.

Cause/Inheritance

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

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 Differential diagnosis (to rule out other condition) includes 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

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 – 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.

The best way to protect against shingles and Ramsay Hunt syndrome is vaccination against the varicella-zoster virus (also known as herpes-zoster virus). 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).

It is best to speak with your medical team to learn more about possible treatments for Ramsay Hunt syndrome and to determine suitability of treatment. Treatment will depend on an individual’s specific symptoms and complications.

Clinical Care

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.

Research and Data

Please visit Australian Clinical Trials to learn about clinical trials for Ramsay Hunt syndrome in Australia; there may not be any clinical trials currently available.

Information regarding clinical trials for Ramsay Hunt syndrome in other countries can be found at ClinicalTrials.gov; there may not be any clinical trials currently available.

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

Rare Disease Organisation(s)

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. If you are interested in starting an Australian organisation for people living with Ramsay Hunt syndrome, please see Engaged, Ethical and Effective: A Guide for Rare Disease Organisation Leaders in Australia.

International organisations
Facial Palsy UK (United Kingdom)
Website: https://www.facialpalsy.org.uk/
Email: [email protected]

Facial Palsy UK provides an essential service to those affected by facial palsy, through the provision of information and support, promotion of best practice; and engagement in high quality research.

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

Support Services/Resources

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.

Mental Health

Please visit the ‘Mental Health’ sections listed on the National and State Services pages.

Other

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

References

1. Facial Palsy UK. Ramsay Hunt Syndrome. Published 2023. Updated 13 March 2023. Accessed: 5 September 2024. https://www.facialpalsy.org.uk/causesanddiagnoses/ramsay-hunt-syndrome/.
2. Andrew E. Crouch MHH, Minhee P. Moody, Claudio Andaloro. Ramsay Hunt Syndrome. StatPearls [Internet]: Treasure Island (FL): StatPearls Publishing 2024; 28 August 2023. https://www.ncbi.nlm.nih.gov/books/NBK557409/
3. National Organization for Rare Disorders. Ramsay Hunt Syndrome. Published 2005, 2011, 2022. Updated 25 July 2024. Accessed 4 September 2024. https://rarediseases.org/rare-diseases/ramsay-hunt-syndrome/.
4. Shingles. Updated July 2023. Accessed 4 September 2024. https://www.healthdirect.gov.au/shingles.
5. Chickenpox (varicella). Healthdirect Australia Limited. Published 2023. Updated July 2023. Accessed: 23 October 24. https://www.healthdirect.gov.au/chickenpox.
6. Johnson RW, Alvarez-Pasquin MJ, Bijl M, et al. Herpes zoster epidemiology, management, and disease and economic burden in Europe: a multidisciplinary perspective. Ther. Adv. Vaccines. 2015;3(4):109-20. https://doi.org/1177/2051013615599151
7. Gross GE, Eisert L, Doerr HW, et al. S2k guidelines for the diagnosis and treatment of herpes zoster and postherpetic neuralgia. J. Dtsch. Dermatol. Ges. 2020;18(1):55-78. https://doi.org/10.1111/ddg.14013
8. Werner RN, Nikkels AF, Marinović B, et al. 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. J. Eur. Acad. Dermatol. Venereol. 2017;31(1):9-19. https://doi.org/10.1111/jdv.13995
9. Werner RN, Nikkels AF, Marinović B, et al. 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: Treatment. J. Eur. Acad. Dermatol. Venereol. 2017;31(1):20-9. https://doi.org/10.1111/jdv.13957
10. Notification of illness and disease. Updated November 2021. Accessed 5 September 2024. https://www.healthdirect.gov.au/notification-of-illness-and-disease.

Page Last Updated

28/10/2024 12:52