Ramsay Hunt Syndrome

Question 1

What is another name for Ramsay Hunt Syndrome?

Answer 1

Herpes Zoster Oticus

Question 2

What causes Ramsay Hunt syndrome?

Answer 2

Reactivation of the varicella zoster virus (chickenpox) in the geniculate ganglion of the facial nerve

Question 3

What symptoms are associated?

Answer 3

Pain deep within ear that radiates outward into the pinna (may be paroxysmal at first)
Facial nerve palsy
Tinnitus
Vertigo
Ipsilateral hearing loss or hyperacusis
Vesicular rash - on skin of ear canal, auricle or both (may become infected)

Also:
Difficulty closing one eye
Change in taste perception
Dry mouth and eyes

Question 4

The rash /hermetic blisters is in the distribution of the…

Answer 4

Nervus intermedius - contains the sensory and parasympathetic fibres of facial nerve (joins with the motor root of facial nerve at geniculate ganglion)

Question 5

What signs are present?

Answer 5

Rash or herpetic blisters in distribution of nervus intermedius - can include external auditory canal, pinna, anterior 2/3 of one side of tongue, soft palate

Ipsilateral facial drop or weakness
Hyperacusis (paralysis of stapedius and tensor tympani)

Question 6

Will the weakness follow a LMN or UMN pattern

Answer 6

LMN (forehead NOT spared)

Question 7

What are some differentials?

Answer 7

Bell’s palsy - also has unilateral facial weakness (but no pain or rash)
If vertigo present - vestibular neuronitis or posterior stroke
Trigeminal neuralgia
Otitis externa or media
Referred pain - dental abscess

Question 8

Ramsay Hunt syndrome can occur in anyone who has had…

Answer 8

Chickenpox

Question 9

Who does it typically affect?

Answer 9

Older adults - typically over 60

Question 10

What complications can occur?

Answer 10

Permanent hearing loss and facial weakness (for most it is temporary)
Eye abrasions - compromise to eyelid closure
Lesions may acquire bacterial infection
Postherpetic neuralgia

Question 11

How is it managed?

Answer 11

Early diagnosis and initiation of treatment within 72 hours can improve outcome
Antivirals
Corticosteroids - short regime of high dose prednisolone
Lubricants if problem with closing eye

Question 12

How is it diagnosed?

Answer 12

Clinical examination and history

Viral studies can detect VZV in saliva, tears and blood, but not necessary to establish diagnosis

Question 13

Do the distinctive features e.g otalgia, facial paralysis and rash always develop at the same time?

Answer 13

No - makes it more difficult to diagnose

Question 14

What causes VZV to reactivate?

Answer 14

Immune system depression

Stress

Question 15

Is Ramsay Hunt syndrome contagious?

Answer 15

No, but can get chickenpox form open rash or blister (if no immunity to chickenpox)