Say it ain’t so, Bieber! We all just want you to sing… but I guess that is challenging to do when half of your face does not move. (Ok… full disclosure… I am that Dad that sings songs loudly to embarrass my children… it is my right! But, in exercising this right, I have come to enjoy Bieber’s lyrical verses… oh what an ironic twist of fate.) It is, honestly, kind of creepy to know that a virus is just living in our nerves… waiting… for that most inopportune time to re-emerge and cause distress. We have previously discussed Varicella Zoster Virus (VZV) and Chickenpox as well as Facial Paralysis. Now let us see how the two may be related – Ramsay Hunt Syndrome:

Ramsay Hunt Syndrome: Basics

• Ramsay Hunt Syndrome is characterized by: [Aydogdu, 2015; Derin, 2014]
  • Auricular Skin Rash
  • Peripheral Facial Paralysis
  • Cochleovestibular Symptoms

• Due to reactivation of Varicella Zoster Virus (VZV). [Aydogdu, 2015; Derin, 2014]
  • VZV is latent within the geniculate ganglion.
  • Reactivation affects CN VII and CN VIII.
  • Also known as “Herpes Zoster Oticus.”

• Ramsay Hunt Syndrome is rare in children. [Aydogdu, 2015; Derin, 2014]
  • Incidence estimated at 2.7/100,000 Children.
  • Develops in 1% of patients with HSV infection.
  • ~10 – 15% of Facial Paralysis is due to Ramsay Hunt Syndrome.
  • However, it is still the 2nd most common cause of Facial Paralysis in children (Bell’s Palsy is #1).

• Symptoms and Prognosis are worse than those for Bell’s Palsy. [Aydogdu, 2015; Derin, 2014]

Ramsay Hunt Syndrome: Presentation

• The syndrome is characterized by 1) ear rash, 2) peripheral facial paralysis, and 3) cochleovesicular symptoms… but…
  • Initial presentation can be subtle at first and…
  • Symptoms evolve over time (so initially you may not see all 3 characteristics).

• Nonspecific findings occur initially: [Aydogdu, 2015]
  • Often starts with a “Viral Prodrome” (like all things in pediatrics).
  • Fever and fatigue.
  • Often lasts for 1-3 days.

• Skin Rash develops next: [Aydogdu, 2015; Derin, 2014]
  • May start with Ear Pain (think about the pain felt prior to the rash of zoster being seen).
  • Develop erythematous vesicular lesions.
  • May be on the pinna or in the external auditory canal or on the Tympanic Membrane.
  • May ALSO involve the oral mucosa!
    • Can see vesicles on anterior tonsillar pillars.
    • May also see lesions on anterior 2/3rds of the tongue.

• Cranial Nerve Involvement then develops. [Aydogdu, 2015; Derin, 2014]
  • CN VII
    • Facial Paralysis develops within 1-2 weeks after rash develops.
  • CN VIII
    • May present with:
      • Nausea, Vomiting, Vertigo, Nystagmus
      • Tinnitus, Hearing Loss (occurs in ~24% of children with Ramsay Hunt Syndrome (RHS)).
      • Can be more prominent than the facial paralysis.
    • Less commonly seen in children than adults with RHS.
    • Presence of these findings is associated with worse prognosis.

Ramsay Hunt Syndrome: Management

• Diagnosis is made clinically. [Aydogdu, 2015; Derin, 2014]
  • VZV IgG and IgM antibody titers can be measured to confirm diagnosis.
  • Lab values do not, however, determine management.

• Therapies to consider: [Aydogdu, 2015; Derin, 2014]
  • Antiviral Rx (ex, Acyclovir)
    • Inhibit viral replication, reduce formation of new lesions, and allow for earlier healing.
    • Ideally started within the first 72 hours.
  • High-Dose Steroids (ex, Methylprednisolone)
    • Reduce the inflammation of the peripheral nerves.
    • Combined steroid with antiviral therapy is more effective than steroids alone.
  • Eye Care
    • Due to the facial paralysis, the eye will be at risk for not being protected.
    • Eye lubricants are recommended.
  • Nausea/Vertigo Management
    • Antiemetics
    • Sedatives

Moral of the Morsel

• Once again… Viruses are not benign! Whether from a Pandemic or from your own nervous system, viruses can do a lot of damage!
• Fever and Ear Pain without AOM. Hmmm… maybe this is early RHS. The otalgia may be the only symptom in the first 3 days.
• Bell’s Palsy? Are you certain? Really evaluate for ear pain / rash or other vertigo symptoms.
• Steroids and Acyclovir. Simple and effective. Try to start them early!

References