Ramsay Hunt Syndrome (herpes zoster oticus) affects the facial nerve near one of ears

Ramsey Hunt

Ramsay Hunt syndrome: symptoms, causes, what is it?

Ramsay Hunt syndrome (herpes zoster oticus) affects the facial nerve near one of your ears, a form of atrial shingles.

Pop star Justin Bieber has announced that he has Ramsay Hunt Syndrome. The varicella zoster virus (VZV) is the cause of this syndrome which leads to auricular shingles. In June 2022, singer Justin Bieber announced via his Instagram account that he suffered from Ramsay Hunt syndrome, which paralyzed his face, forced him to cancel some tour dates and caused him difficulty eating.

Discovered in 1907 by the American neurologist Ramsay Hunt who gave it his name, this syndrome is both a disorder of the immune system and a reactivation of the varicella virus which is part of the herpesviridae (herpes virus). It thus concerns patients who have had a primary chickenpox infection: More than 90% of adolescents in most European countries have already come across the chickenpox virus in childhood. Being in contact with the infectious agent (varicella virus) will stimulate the immune system and if it is deficient, the body creates shingles” adds our expert.

This disease affects the Ramsay Hunt area where the geniculate ganglion (nerve ganglion located in the facial canal of the temporal bone) located at the angle of the mandible (jaw) and the ear is located. The region is traversed by nerves that control the face (facial nerve with branches along the face) and the ear (auditory nerve, the nerve that goes inside the ear).

What causes Ramsay Hunt syndrome?

Primary infection with VZV usually produces varicella, after which the virus becomes latent (it remains at rest) in the neurons of the cranial nerve ganglia” specify the authors of the study published in the Journal of Neurology Neurosurgery and Psychiatry in 2001.

Reactivation of varicella virus causes Ramsay-Hunt syndrome. “This disease particularly affects vulnerable people (immune weakness), which is why it often occurs after the age of 60 because of immunosenescence [progressive deterioration of the immune system linked to age] indicates the infectiologist. The younger we are, the more we are immune to the chickenpox virus, but if our immunity is imperfect, we risk reactivating shingles in the Ramsay Hunt zone.

Read also: Monkeypox | Symptoms, transmission, contagiousness… what you need to know about the disease

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What are the symptoms of Ramsay Hunt syndrome?

Ramsay Hunt syndrome manifests as facial nerve palsy (paralysis of half of the face) and a rash around the ear. Facial paralysis can cause an inability to smile, wrinkle the forehead, difficulty eating, swallowing (fluid “leaks” from the mouth), or closing the eyes on the affected side. “At the level of the ear, we will find tinnitus, vertigo, a decrease in hearing or even deafness.

This disease results from an interaction between a micro-organism (the virus) and an individual. But everyone has a susceptibility, a specific genetic heritage that can give atypical forms of chickenpox. Among the symptoms are:

  • Facial paralysis (resembling Bell’s syndrome paralysis)
  • Ear rashes/vesicles (painful fluid-filled blisters) that scratch, burn
  • Severe ear pain
  • Fever (flu-like condition)
  • Hearing loss (even deafness)
  • Tinnitus (condition when the ears feel ringing)
  • Nausea
  • Vomitings
  • Dizziness (affected inner ear)
  • Nystagmus (involuntary rhythmic oscillation of one or both eyes)
  • Difficulty eating, swallowing

How to diagnose Ramsay Hunt syndrome?

A clinical examination with the patient’s neurological history can put doctors on the trail of Ramsay Hunt syndrome. In order to differentiate it from Bell’s palsy, the doctor may perform PCR for the shingles virus and varicella. Ramsay Hunt syndrome can also be detected by the increased presence of antibodies against VZV.

It is most often the ENT specialists who are consulted first for discomfort in the ear. Rashes around the pinna can be external (visible) or internal (hidden).

Can Ramsay’s syndrome be cured?

This disease is severe since it affects two nerves. It is estimated that the chances of total recovery are between 30% and 70% depending on age, terrain, speed of implementation of treatment etc. At prognostically, the evolution of Ramsay Hunt syndrome is generally favourable.

However, cochleo-vestibular complications including hearing loss have been reported by some authors with an incidence of hearing disorders varying between 19.7% and 85%.

This shows the importance of close collaboration between infectiologist, dermatologist and ENT specialist in order to establish the diagnosis and treat this condition and its complications early.

What is the treatment for Ramsay Hunt syndrome?

The treatment focuses on the disruption of the immune system and on the reactivation of the chickenpox virus: “It will combine a drug that plays on the immune system (corticosteroids) and an antiviral (anti herpetic) like aciclovir with anti-varicella actions “says the infectiologist. The treatment lasts about ten days. It combines positional, auditory and face rehabilitation for at least one month. The effectiveness of the treatment is linked to the precocity of its initiation.

Against viral diseases, treatments are most effective within the first 72 hours of infection after which the virus replicates and escapes the antiviral. On the other hand, the vaccine against shingles is not curative but prevents the disease according to the age of administration (over 50 years old). The studies showed a significant improvement in the condition of patients treated with prednisone and aciclovir within three days of disease onset, report the researchers of the study published in the Journal of Neurology Neurosurgery and Psychiatry in 2001.

Three different neurological syndromes are called Ramsay Hunt syndrome

Their only connection is that they were all first described by the famous neurologist James Ramsay Hunt (1872-1937).

Type 1

Also called cerebellar Ramsay Hunt syndrome, is a rare form of cerebellar degeneration that involves myoclonic epilepsy, progressive ataxia, tremors, and dementia process. Treatment of Ramsay Hunt Syndrome Type 1 is specific to individual symptoms. Myoclonus and seizures may be treated with drugs like valproate. Some have described this condition as difficult to characterize.

Type 2 is the reactivation of shingles in the geniculate ganglion

It is sometimes called herpes zoster oticus and presents variably, including facial nerve lower motor neuron damage, deafness, dizziness, and pain. A triad of ipsilateral facial paralysis, ear pain, and vesicles on the face, on, or in the ear is the typical presentation.

Treatments for Ramsey Hunt Syndrome Type 2 are used to reduce further damage caused by the viral infection. These medications will not reverse any damage that has already occurred at the time that they are prescribed.

Initial treatment with a corticosteroid such as prednisone and an antiviral drug such as acyclovir, valacyclovir or famciclovir for 5 to 7 days is standard; however, some studies have shown later damage to the facial nerve and recommend 21 days of antivirals. Studies indicate that treatment started within 72 hours of the onset of facial paralysis improves the chances of the patient experiencing significant recovery. Chances of recovery appear to decrease when treatment is delayed. Delay of treatment may result in permanent facial nerve paralysis. However, some studies demonstrate that even when steroids are started promptly, only 22% of all patients achieve full recovery of facial paralysis. Treatment apparently has no effect on the recovery of hearing loss.

Meclizine, benzodiazepines such as diazepam, and vestibular therapy are sometimes used to treat the vertigo.

Type 3 is a less commonly referred condition

A work-related neuropathy of the deep palmar branch of the ulnar nerve. It is also called Hunt’s disease or Artisan’s palsy. It is different from Ramsay Hunt syndrome type 1 and Ramsay Hunt syndrome type 2, which are completely unrelated other than having been described by the same researcher, James Ramsay Hunt.

Ramsay-Hunt syndrome, named after the American neurologist James Ramsay Hunt (1874-1937); describes various diseases:

  • Ramsay-Hunt neuralgia: a complication with neuralgia and facial paralysis that often occurs as part of a herpes zoster oticus of the ear nerve.
  • Ramsay Hunt’s ataxia, also known as dyssynergia cerebellaris myoclonica: a rare brain disorder that presents as a lack of coordination of movements, often accompanied by epileptic seizures.
  • Ramsay Hunt’s disease: A work-related neuritis of the ulnar nerve in the hand.
  • Ramsay Hunt’s atrophy of the hand: an obsolete expression for the atrophy of the small muscles of the hand without loss of feeling.
  • Ramsay Hunt’s Juvenile Parkinson’s Syndrome, also known as Hunt’s Paralysis, a Parkinson’s disease -like condition that occurs between the ages of 10 and 30.

Diseases | List of Diseases: dermatological, cardiovascular, respiratory, cancer, eye, genetic, infectious, mental illness, rare

Sources: PinterPandai, BMJ Publishing Group, Mayo Clinic, NORD – National Organization for Rare Disorders, Mount Sinai

Photo credit: Worme M, Chada R, Lavallee L (CC BY 2.0) via Wikimedia Commons

Photo description: representation of the symptoms of Ramsay Hunt syndrome type 2; vesicles on the ear (left) and ipsilateral facial paralysis (right).


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