Ramsay Hunt Syndrome
Introduction to Ramsay Hunt Syndrome
Ramsay Hunt syndrome (RHS), also known as herpes zoster oticus, is a rare neurological disorder characterized by facial paralysis and a rash affecting the ear or mouth. It is caused by the reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve.
Key points:
- Etiology: Reactivation of latent VZV infection
- Incidence: 5 per 100,000 people annually
- Age group: More common in adults over 60, but can occur in children
- Risk factors: Immunocompromised status, stress, fatigue
Clinical Features of Ramsay Hunt Syndrome
The clinical presentation of RHS typically includes a triad of symptoms, but not all may be present in every case:
- Facial nerve palsy:
- Unilateral facial weakness or paralysis
- May affect eye closure, smiling, and other facial movements
- Ear involvement:
- Vesicular rash on the external ear (pinna), external auditory canal, or tympanic membrane
- Otalgia (severe ear pain)
- Hearing loss (sensorineural or conductive)
- Tinnitus
- Vertigo
- Oropharyngeal symptoms:
- Vesicular rash on the anterior two-thirds of the tongue, hard palate, or soft palate
- Taste disturbances (dysgeusia or ageusia) in the anterior two-thirds of the tongue
- Other possible symptoms:
- Nystagmus
- Hyperacusis
- Facial numbness or tingling
- Dry eye or mouth on the affected side
Diagnosis of Ramsay Hunt Syndrome
Diagnosis of RHS is primarily clinical, but additional tests may be performed to confirm the diagnosis and rule out other conditions:
- Clinical diagnosis:
- Detailed history and physical examination
- Evaluation of facial nerve function (House-Brackmann scale)
- Otoscopic examination
- Laboratory tests:
- PCR detection of VZV DNA from vesicle fluid or saliva
- Serum antibody titers for VZV (less reliable)
- Imaging studies:
- MRI with contrast to evaluate the facial nerve and rule out other causes of facial palsy
- CT scan if temporal bone involvement is suspected
- Audiological and vestibular testing:
- Pure tone audiometry
- Electronystagmography (ENG) or videonystagmography (VNG)
- Differential diagnosis:
- Bell's palsy
- Acute otitis media
- Cholesteatoma
- Acoustic neuroma
- Lyme disease
Management of Ramsay Hunt Syndrome
Treatment of RHS aims to manage symptoms, prevent complications, and promote recovery of facial nerve function:
- Antiviral therapy:
- Acyclovir (800 mg 5 times daily for 7-10 days) or Valacyclovir (1000 mg 3 times daily for 7 days)
- Should be started within 72 hours of symptom onset for best results
- Corticosteroids:
- Prednisone (60-80 mg daily, tapered over 10-14 days)
- Used in combination with antivirals to reduce inflammation and improve outcomes
- Pain management:
- Analgesics (acetaminophen, NSAIDs)
- Opioids for severe pain if necessary
- Gabapentin or pregabalin for neuropathic pain
- Eye care:
- Artificial tears and eye lubricants
- Taping the eye shut at night if unable to close completely
- Physiotherapy:
- Facial exercises to prevent muscle atrophy and promote nerve regeneration
- Electrical stimulation (controversial, evidence limited)
- Management of associated symptoms:
- Vestibular rehabilitation for vertigo
- Hearing aids if hearing loss persists
Prognosis of Ramsay Hunt Syndrome
The prognosis of RHS varies and depends on several factors:
- Timing of treatment: Early initiation of antivirals and steroids (within 72 hours) improves outcomes
- Severity of initial symptoms: Complete paralysis at onset indicates a poorer prognosis
- Age: Younger patients generally have better outcomes
- Comorbidities: Immunocompromised patients may have a more prolonged course
Recovery rates:
- Complete recovery of facial function: 70-75% with early treatment
- Partial recovery: Most patients show some improvement within 3-6 months
- Persistent complications: 20-30% may have residual facial weakness, synkinesis, or other sequelae
Long-term follow-up is important to monitor for complications and provide ongoing support.
