Ramsay Hunt Syndrome

Ramsay Hunt syndrome is a neurologic disorder caused by reactivation of varicella-zoster virus (VZV) in the geniculate ganglion (ganglion geniculi) of the facial nerve (cranial nerve VII). In essence, it is a distinct form of shingles that involves the ear region and the facial nerve.

Classically, Ramsay Hunt syndrome presents with a triad. This consists of acute unilateral peripheral facial paralysis, severe otalgia, and a painful vesicular rash involving the auricle, external auditory canal, or oral mucosa.

Aetiology and Pathophysiology

The causative agent is varicella-zoster virus (VZV), which becomes latent in sensory ganglia after primary infection (chickenpox) and can persist lifelong. When host immunity wanes (due to age, stress, or comorbid conditions), the virus may reactivate.

In Ramsay Hunt syndrome, reactivation occurs in the geniculate ganglion, the sensory ganglion of the facial nerve (cranial nerve VII). The resulting inflammation and edema can impair facial nerve function, leading to weakness or paralysis of the muscles of facial expression. At the same time, the virus travels along sensory fibers to the skin of the auricle and nearby mucosa, producing the characteristic herpetic vesicles. The vestibulocochlear nerve (cranial nerve VIII) is also often involved because of its close proximity to the facial nerve. This explains the associated hearing and vestibular symptoms.

Clinical significance

Ramsay Hunt syndrome is an ENT and neurologic emergency, as the prognosis for facial nerve recovery is closely linked to early treatment.

Clinical presentation:

• Facial nerve palsy: unilateral peripheral weakness or paralysis leading to facial asymmetry, inability to close the eye, reduced forehead wrinkling, and an asymmetric smile on the affected side.
• Ear pain (otalgia): often severe, shooting, or burning; it may precede other symptoms.
• Vesicular rash: clusters of small vesicles on an erythematous base on the auricle, in the external auditory canal, on the tympanic membrane, and sometimes on the palate or tongue.
• Audiovestibular symptoms (cranial nerve VIII involvement): hearing loss (typically sensorineural), tinnitus, vertigo, nausea, and nystagmus.
• Other features: taste disturbance over the anterior two-thirds of the tongue, dry eye, and sound sensitivity (hyperacusis).

Diagnosis is primarily clinical and is based on the characteristic combination of findings. Treatment should be started as early as possible, ideally within 72 hours. It typically includes high-dose antiviral therapy (e.g., acyclovir or valacyclovir) plus systemic corticosteroids to reduce nerve edema, together with adequate analgesia. In addition, careful eye protection and lubrication are essential to prevent exposure keratitis and corneal injury.

Differential Diagnosis

The main differential diagnosis is Bell’s palsy (idiopathic facial nerve palsy). In Ramsay Hunt syndrome, a herpetic vesicular rash helps distinguish it from Bell’s palsy. Facial weakness is also often more severe. However, the rash may be absent (zoster sine herpete) or may appear after facial weakness begins, which can delay diagnosis. Other causes of facial paralysis should also be considered, including cerebellopontine angle tumors, otitis media, Lyme disease, and stroke. Overall, complete recovery of facial nerve function is less likely in Ramsay Hunt syndrome than in Bell’s palsy.