Ramsay Hunt syndrome

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2Specialists8Treatment centers

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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:

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Ramsay Hunt syndrome.

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No actively recruiting trials found for Ramsay Hunt syndrome at this time.

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Specialists

2 foundView all specialists →
DM
Desmond A Nunez, MD, MBA
Specialist
PI on 1 active trial
SM
Stephen J Haines, MD
TOLEDO, OH
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA 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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Community

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Latest news about Ramsay Hunt syndrome

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Caregiver Resources

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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.