What is Ramsay Hunt syndrome?

What is Ramsay Hunt Syndrome

Ramsay Hunt syndrome is a neurological condition caused by reactivation of varicella-zoster virus (VZV) in the geniculate ganglion, presenting with the classical triad of ipsilateral facial paralysis, otalgia (ear pain), and vesicular rash in the ear or mouth. 1

Pathophysiology

• The syndrome results from VZV reactivation that produces an inflammatory reaction in the seventh cranial nerve geniculate ganglion 2
• The virus infects and inflames the geniculate ganglion, which lies in close proximity to the vestibulocochlear nerve within the bony facial canal, explaining the frequent eighth nerve involvement 3

Clinical Presentation

Classical Triad

• Ipsilateral facial paralysis (peripheral facial nerve palsy) 1
• Otalgia (severe ear pain) 1, 4
• Vesicular rash - erythematous vesicular lesions on the ear (zoster oticus) or in the mouth 1, 3

Additional Common Features

• Vestibulocochlear nerve involvement causing:
  • Sensorineural hearing loss 1, 5
  • Tinnitus 1, 5
  • Vertigo 1, 5
  • Nystagmus 1, 5
  • Nausea and vomiting 5, 3

Critical Diagnostic Pitfall

• 14% of patients develop vesicles AFTER the onset of facial weakness, meaning Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy 3
• Some patients develop peripheral facial paralysis without any rash (zoster sine herpete), diagnosed only by fourfold rise in VZV antibody or presence of VZV DNA in auricular skin, blood, middle ear fluid, or saliva 3

Prognosis and Severity

• Patients with Ramsay Hunt syndrome have more severe paralysis at onset and are less likely to recover completely compared to Bell's palsy 3
• Ramsay Hunt syndrome accounts for approximately 2-10% of all facial nerve palsy cases 2
• The course and outcome of facial nerve palsy is determined by the early clinical picture, while otological symptoms depend on the extent of nerve involvement 2

Complications

• Persistent facial weakness - more common and severe than in Bell's palsy 3
• Permanent hearing impairment - though less common than facial palsy, it represents a significant long-term complication 2
• Brainstem encephalitis - rare but serious complication that can involve the solitary nucleus, spinal trigeminal nucleus and tract, and vestibular nucleus 6

Contagiousness

• Ramsay Hunt syndrome is approximately 20% as contagious as chickenpox 7
• Transmission occurs through direct contact with fluid from vesicular lesions or rarely through airborne routes 7
• The contagious period begins 1-2 days before rash onset and continues until all lesions have dried and crusted, typically 4-7 days after rash onset 7
• Immunocompromised patients may experience prolonged viral shedding (7-14 days or longer) 7