Overview
Ramsay Hunt syndrome (also known as herpes zoster oticus or Hunt syndrome) is a neurological disorder caused by reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (cranial nerve VII). The virus, which causes chickenpox during initial infection, remains dormant in nerve ganglia and can reactivate later in life, particularly during periods of immunosuppression or stress. The syndrome primarily affects the nervous system and the ear, and is characterized by a triad of symptoms: acute peripheral facial nerve paralysis (facial palsy), a painful vesicular rash (herpetic eruption) in or around the ear, and ear pain (otalgia). Additional symptoms may include hearing loss (sensorineural), tinnitus, vertigo, nausea, and altered taste sensation, reflecting involvement of the vestibulocochlear nerve (cranial nerve VIII) and other nearby cranial nerves. The condition typically presents in adults, with increasing incidence in older age groups and in immunocompromised individuals. Ramsay Hunt syndrome is considered the second most common cause of atraumatic peripheral facial paralysis after Bell's palsy, and it generally carries a worse prognosis for facial nerve recovery compared to Bell's palsy. Diagnosis is primarily clinical, based on the characteristic presentation, though laboratory confirmation through PCR testing or viral culture of vesicular fluid can be performed. Treatment involves a combination of antiviral medications (such as acyclovir, valacyclovir, or famciclovir) and corticosteroids (such as prednisone), ideally initiated within 72 hours of symptom onset to improve outcomes. Analgesics may be used for pain management. Supportive care, including eye protection measures when facial paralysis prevents complete eyelid closure, is important to prevent corneal damage. Despite treatment, some patients may experience incomplete recovery of facial nerve function, persistent hearing loss, or postherpetic neuralgia. Physical therapy and facial rehabilitation exercises may be recommended during recovery.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Ramsay Hunt syndrome.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Ramsay Hunt syndrome.
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Common questions about Ramsay Hunt syndrome
What is Ramsay Hunt syndrome?
Ramsay Hunt syndrome (also known as herpes zoster oticus or Hunt syndrome) is a neurological disorder caused by reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (cranial nerve VII). The virus, which causes chickenpox during initial infection, remains dormant in nerve ganglia and can reactivate later in life, particularly during periods of immunosuppression or stress. The syndrome primarily affects the nervous system and the ear, and is characterized by a triad of symptoms: acute peripheral facial nerve paralysis (facial palsy), a painful vesicular
How is Ramsay Hunt syndrome inherited?
Ramsay Hunt syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Ramsay Hunt syndrome typically begin?
Typical onset of Ramsay Hunt syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Ramsay Hunt syndrome?
2 specialists and care centers treating Ramsay Hunt syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.