Symptoms of Ramsay Hunt Syndrome

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. Explanations with Dr Benjamin Davido, infectious disease specialist at Garches AP-HP hospital.

[Mise à jour le 14 juin 2022 à 16h23] Known in France as Sicard syndromethe Ramsay Hunt syndrome been talked about since the singer justin bieber revealed to be affected on June 11 on his account instagram. This is a auricular shingles. This rare condition is generally observed in sub-Saharan Africa. She causes a facial paralysis (which resembles Bell’s palsy) and skin rashes the ear. Definition, causessymptoms, healing, Pictures and treatment. What we know.

Definition: what is Ramsay Hunt syndrome?

Ramsay-Hunt syndrome or “herpes zoster oticus” is a form of auricular zona. Discovered in 1907 by American neurologist Ramsay Hunt which gave it its name, this syndrome is both dysregulation of the immune system and reactivation of the varicella virus which is part of the herpesviridae (herpes virus). It thus concerns patients who have presented a primary infection of varicella: “More than 90% of teenagers in most European countries has already come across the chicken pox virus in childhood“recalls Dr. Benjamin Davido, infectious disease specialist at the Garches AP-HP hospital.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 is 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. The region is crossed by nerves that control the face (facial nerve with ramifications along the face) and the ear (auditory nerve, the nerve that goes inside the ear).

Symptoms of Ramsay Hunt Syndrome © Journal of Neurology, Neurosurgery, and Psychiatry

What causes Ramsay Hunt syndrome?

Primary infection with VZV usually produces varicella, after which the virus becomes latent (it remains quiescent) in the neurons of the cranial nerve ganglia“say the authors of the study published in the Journal of Neurology Neurosurgery and Psychiatry in 2001. The reactivation of the 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. due to immunosenescence [altération progressive du système immunitaire lié à l’âge]” indicates the infectiologist. The younger we are, the more we are immunized against the varicella virus but if our immunity is imperfectwe risk reactivate a shingles from the Ramsay Hunt zone.

When the syndrome affects a young patient, a dysfunction of the immune system must be sought.

For instance, in North America, they bet on a wide vaccination against chickenpox but we suspect that this is associated with a higher risk of developing shingles because the immunity of vaccination is weaker than that produced by the contraction of the virus when we are young ” says Dr. Davido. When the syndrome affects a young patient, it is necessary to seek a immune system dysfunction. Shingles occurs particularly in a situation of weakness of the immune system (lack of vitamin D, lack of sun, stress, after organ transplant, chemotherapy, HIV infection etc). “In addition, chickenpox is a spring disease and France is currently in a situation of a chickenpox epidemic.“underlines our interlocutor.

What are the symptoms of Ramsay Hunt syndrome?

Ramsay Hunt syndrome is manifested by facial nerve palsy (paralysis of half the face) and a rash on the ear. Facial paralysis can cause 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, dizziness, loss of 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“continues Dr. Davido. Among the symptoms we find:

Erythema and skin vesicles in the right Ramsay Hunt area
Erythema and skin vesicles in the right Ramsay Hunt area © National Library of Medicine
  • A paralysis facial (resembling Bell’s syndrome paralysis)
  • Of the ear blisters/rashes (painful fluid-filled blisters) that scratch, burn
  • Severe ear pain
  • Of the fever (flu-like symptoms)
  • A hearing loss (even deafness)
  • Of the tinnitus
  • Of the nausea
  • Of the vomiting
  • Of the dizziness (inner ear affected)
  • Of the nystagmus (involuntary rhythmic oscillation of one or both eyes)
  • Difficulty eating, swallowing

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 can carry out a Herpes zoster and varicella virus PCR. Ramsay Hunt syndrome can also be detected by increased presence of antibodies against VZV. These are most often the ENT who are consulted first for ear discomfort. Rashes around the pinna can be external (visible) or internal (hidden)“says Dr. Davido.

Can Ramsay Syndrome be cured?

This disease is severe since it affects two nerves. It is estimated that the chances of full recovery are between 30% and 70% depending on age, terrain, speed of implementation of treatment, etc.“emphasizes Dr. Davido. “At the prognostic level, the evolution of Ramsay Hunt syndrome is in generally favorable. However, cochleo-vestibular complications, in particular 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.“conclude the researchers of the article published in the COSA-CMF review in February 2020.

What is the treatment for Ramsay Hunt syndrome?

The effectiveness of the treatment is linked to the precocity of its initiation

Treatment focuses on the immune system disorder and on the reactivation of varicella virus : “He will combine a drug that plays on the immune system (the corticosteroids) and one antiviral (anti herpetic) like aciclovir with anti varicella actions“says the infectiologist. The treatment lasts about ten days. “We combine a positional, auditory and face rehabilitation for at least one month“adds Dr. Davido. The effectiveness of the treatment is linked to the precocity of its initiation.”Against viral diseases, treatments are at their maximum efficiency within the first 72 hours of infection after which the virus replicates and escapes the antiviral” argues Dr. Davido. On the other hand, the shingles vaccine is not curative but prevents the disease depending on the age of administration (over 50). “Studies have shown a significant improvement in the condition of patients treated with prednisone and aciclovir within three days of illness onset” report the researchers of the study published in the Journal of Neurology Neurosurgery and Psychiatry in 2001.

Thanks to Dr Benjamin Davido, infectious disease specialist at Garches AP-HP hospital.

Sources:

– Ramsay Hunt syndrome: a case observed in a subject infected with the human immunodeficiency virus in Abidjan, Revue du COSA-CMF, February 19, 2020

– Ramsay Hunt Syndrome, The Pan African Medical Journal, October 21, 2015

– Ramsay Hunt Syndrome, The Journal of Neurology, Neurosurgery, and Psychiatry August 1, 2001

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