What are the clinical presentation and features of Ramsay Hunt syndrome?

Clinical Presentation of Ramsay Hunt Syndrome

The clinical presentation of Ramsay Hunt syndrome includes the classical triad of ipsilateral facial paralysis, otalgia, and vesicular rash, along with potential vestibulocochlear nerve involvement causing hearing loss, tinnitus, vertigo, and nystagmus. 1, 2

Classical Triad

• Ipsilateral facial paralysis: Presents as a lower motor neuron pattern of weakness affecting both upper and lower face on the affected side 3
• Otalgia (ear pain): Often severe and may precede other symptoms; can be the initial presenting complaint 4, 3
• Vesicular rash: Erythematous vesicular (herpetic) lesions typically found in the external auditory canal, auricle, or oral mucosa 1, 3

Important: The initial presentation of Ramsay Hunt syndrome may mimic Bell's palsy if vesicles have not yet appeared! In a prospective study, 14% of patients developed vesicles after the onset of facial weakness 3

Additional Features of Cranial Nerve VII Involvement

• Sensory disturbances: Impairment of taste in anterior 2/3 of tongue 3
• Ocular features: Lagophthalmos (inability to close eyelid completely) and decreased lacrimation 4, 3

Features of Cranial Nerve VIII Involvement

• Sensorineural hearing loss and tinnitus: Due to proximity of the geniculate ganglion to cranial nerve VIII within the bony facial canal 2, 5
• Vertigo and nystagmus: Result from vestibulocochlear nerve involvement 2, 5
• Associated symptoms: Nausea and vomiting may occur due to vestibular dysfunction 5

Potential Involvement of Other Cranial Nerves

• In rare cases, Ramsay Hunt syndrome can involve additional cranial nerves (III-XII) 6
• Cranial nerves V, X, and possibly XII involvement has been reported, resulting in:
  • Dysphagia (difficulty swallowing) 6
  • Pharyngeal wall and vocal fold paralysis 6
  • Reduced laryngeal elevation 6

Complications

• Ramsay Hunt syndrome can rarely be complicated by VZV encephalitis 7
• Patients with Ramsay Hunt syndrome often have more severe paralysis at onset compared to Bell's palsy 3
• Less likely to recover completely compared to Bell's palsy if not treated promptly 3

Diagnostic Considerations

• Audiometric evaluation should be performed to assess hearing function 5
• Early diagnosis is crucial for prompt treatment and optimal outcomes 8
• Ramsay Hunt syndrome may initially be indistinguishable from Bell's palsy, especially if vesicles have not yet appeared 3

The clinical presentation described in the question is accurate and aligns with the established features of Ramsay Hunt syndrome as documented in medical literature and guidelines.