Ramsay Hunt Syndrome (RHS)

Clinical Treatment of a Patient with Ramsay Hunt Syndrome (RHS)

1. CLINICAL PRESENTATION OF
PATIENT DX WITH RAMSAY HUNT
SYNDROME
By JereHess

2. What is Ramsay Hunt Syndrome (RHS)
a.k.aHerpes Zoster Otticus
Shingles attack C.NVII near one of the ears
Caused by same virus as chickenpox (Variclela-Zoster Virus)
Lies dormant in nerves for years and if the virus reactivates and affects
C.NVII the result is RHS
Classified as a rare disease by the Office of Rare Diseases of
the National Institutes of Health
Fewer than 200,000 affected out of est. 300 million people

3. Signs of RHS
2 primary signs
Painful red colored rash containing fluid-filled blisters on, in,
or around ear
Facial weakness or paralysis
Occurs Ipsilateral to side of affected ear
Other S/S
Hearing loss
Tinnitus
Vertigo
Changes in perception of taste
Difficulty closing one eye
Ear pain

4. Who is Affected and Risk Factors by
RHS
Anyone who has had chickenpox can develop RHS
More commonly
Post menopausal women over 60
Anyone with a weakened immune system
Head traumas

5. Complications
Hearing loss and facial weakness
Can be permanent but more often temporary
Damage to eye (eye pain and blurred vision)
Occurs secondary to facial weakness
Incomplete eye closure causes damage to cornea
Postherpetic neuralgia
Occurs when shingles virus damages nerve fibers causing pain
Can endure after all other S/S of RHShave been eliminated

6. RHSTreatment
Initially
Anti-viral drugs
Corticosteroids
Anti-anxiety meds (vertigo symptoms only)
Pain relievers
Long-term
PT may be prescribed to restore functional capacity

7. DxRHS
First
Medical History and Physical Exam to identify unique s/s
Next
PCR (Polymerase Chain Reaction) test
Take sample of fluid from blisters on the ear
Can also use blood or tear sample (fluid from blister is more accurate)
Misdiagnosis
Why? B/c of rarity of RHS
What? Most commonly misdiagnosed as Bells Palsy
B/c of sudden onset (less than 48 hours) of facial paralysis
Vertigo and otalgia are often disregarded as severe BP or med
side effects
Also misdiagnosed as bacterial ear infection, flu, or inflammation
of sinus

8. PatientHistory
Onset began May 1, 2014
Pt. spontaneously experienced nausea vomiting, dizziness, and left facial
paralysis
Hospitalized for 9 days
Upon discharge from hospital, pt required a walker for ambulation
Secondary to vestibular deficiencies
Pt. stated when symptoms were most severe, unable to move
head in any direction without vomiting
Pt. underwent a variety of Tx before reporting to outpatient
PT

9. Initial Evaluation
Pt. reported to outpatient PT January 8, 2015
Diagnosis:
Left Ramsay Hunt Syndrome
Impaired VOR (Vestibular-occular-reflex)
Impaired balance and gait
Clinical Assessment read…
RHS affected pt’s Left Cranial nerve VII and VIII causing complete left
lateral facial paralysis accompanied by nausea vomiting, dizziness, and
slight left hearing loss. Pt. showed no s/s of acute BPPV (Benign Paroxismal
Positional Vertigo). The patient did present with stable left unilateral
peripheral loss with impaired static and dynamic balance, impaired VOR,
and slightly decreased oculomotor control
Pt. reported
Condition was improving but dizziness still occurred daily during movement

10. Cranial Nerve VII
a.k.aFacial Nerve
Mixed Nerve
(both sensory and motor components)
Sensory portion
Axons in taste buds of anterior tongue
Axons from proprioceptors in mm’s of the face and scalp
Functions includetaste and the innervated mm’s proprioception of touch,
pain, and temperature
Motor portion
Axons of somatic motor neurons that innervate facial, scalp, and neck mm’s,
plus parasympathetic axons that stimulate lacrimal and salivary glands
Functions include facial expressions and secretion of tears and saliva

11. Cranial Nerve VIII
a.k.aVestibularcochlearNerve
Sensory Nerve
2 branches
Vestibular and Cochlear
Cochlear Branch
Axons from the organ of corti
Function is hearing
Vestibular Branch
Axons from the semicircular canals, saccule, and utricle
Function is equilibrium

12. Clinical Assessment and C.N VII and
VIII
Left Lateral Facial Paralysis
Due to infection of C.N VIII
Slight hearing loss
Due to infection of cochlear branch of C.N VIII
Impaired VOR, static, and dynamic balance
Due to infection of vestibular branch of C.N VIII

13. Vestibular System
Equilibrium
Balance
Spacialorientation
Innervated by Cranial Nerve VIII
Primary organs
Semicircular canals, saccule, and utricle

14. Vestibular System(Semicircular Canals)
3 semicircular canals
Anterior, posterior, and horizontal
Should only contain endolymphatic fluid
Detect angular rotation of the head
Collectively the anterior and posterior canals are called the
verticle semicircular canals
Detect flexion and extension of head in saggital plane
Nodding head to say “yes”
Horizontal Canal
Detects rotation of head in transverse plane
Rotating head to say “no”

15. Vestibular System (Saccule and Utricle)
Referred to as Otolithic organs
Detect Horizontal and Verticle displacement
Saccule
Responds to verticle displacement as in jumping rope
Utricle
Responds to horizontal displacement
Contains crystals
Another important note
Left unilateral peripheral loss was stable
Vestibular system has ability to utilize the unaffected side to “make up”
for deficiencies of the affected side
In this case pt. contralateral side of infection (Right) could strengthen to
make up for deficiency of Left side
B/c it was stable the affected side (Left) any strengthening on the
unaffected side (Right) would improve the overall functioning of the
vestibular system

16. Functional Measurements of Initial
Assessment
Romberg firm and foam surface Eyes Close
moderate sway
Tandem firm surface eyes closed
Maintained for 5 seconds before losing balance
Single leg balance Eyes closed
Unable to perform
VOR Test (static and dynamic) using eye chart
Static results 20/20
Verticle results 20/30
Horizontal results 20/50
Other important notes of initial assessment
Pt unable to drive
When ambulating eyes closed pt. would walk in circles.
Loss of Independence (driving, grocery shopping, etc…)

17. Long Term Goals
Romberg firm and foam surface eyes closed with minimum
sway
Tandem firm eyes closed for 15 seconds minimum before losing
balance
Single leg balance eyes closed 7-10 seconds
Horizontal VOR 20/30
Driving and ADL’s goals were set at subsequent reevaluations

18. Reevaluation
February 24, 2015 (47 days after initial assessment)
All LTG’s from Initial Assessment were met except single leg
balance eyes closed and horizontal VOR
Single leg balance eyes closed had improved from unable to
perform to 3-5 seconds. Did not reach goal of 7-10 seconds
Horizontal VOR improved from 20/50 to 20/40. Did not reach
goal of 20/30
All LTG’s that were met were advanced with each subsequent
assessment as they were met and goals involving other
functional assessment tools were added

19. Current Pt. Progress
Romberg firm eyes closed
Initial: Moderate sway. Current: No sway
Romberg foam eyes closed
Initial: Moderate sway. Current: Minimum sway
Tandem firm eyes closed
Initial: 5 seconds. Current: 30 seconds
Single leg balance eyes closed
Initial: Unable to perform. Current: 25 seconds
Can perform single leg balance eyes closed with head turns
Driving
Initial: Unable. Current: 15 mins. of driving (including highway)
Pt. has been able to resume other activities
Grocery shopping, hiking, walking stroller

20. How the Improvements Were Made.
Treatment Plan.
Vestibular strengthening and Neuromuscular Re-ed.
When looking at functional measurements of initial
assessment it is important to note what is being
measured
Maintaining balance involves 3 systems
Proprioception, Visual, Vestibular

21. Visual
Visual is dominant system used for balance
Eyes open allows for use of Visual in balance
Eyes closed eliminates use of Visual in balance
Results in vestibular and proprioception increasing their
function to maintain balance

22. Proprioception
Firm surface and “normal” stance is least
challenging
Treatment challenged proprioception by tandem
stance, single limb support, foam surface
When challenged it places the proprioceptors at a
deficit which makes available systems (vestibular and
visual) increase function to maintain balance

23. Vestibular
Rotation of Head in Transverse Plane
Challenges the horizontal semicircular canal
Flexion Extension of Head in Saggital Plane
Challenges the anterior and posterior semicircular canals
Verticle displacement
Challenges the saccule
Horizontal displacement
Challenges the utricle
Challenging any of these movements places the associated
organ at a deficit which requires the other organs of vestibular
system, visual, or proprioceptors to increase function

24. Examples
Tandem on Foam Eyes Open
Tandem on foam. Challenges the proprioceptors putting them at
a deficit. Eyes open allows full visual use. And no head
movement or displacement allows full use of vestibular system.

25. Examples
Single limb support Eyes closed Horizontal head turns
Single limb support challenges the proprioceptors thus placing them at a deficit. Eyes
closed eliminates use of visual function. Horizontal head turns challenge the horizontal
semicircular canal thus placing it at a deficit. So the primary function for balance of
this movement would come from the remaining organs of vestibular system (Ant. and
post. canals, utricle, and saccule.

26. Other Treatment Strategies
Jumping on trampoline and jumping rope
Causes verticle displacement
Challenges saccule
Single leg ball pick-ups
Single leg balance ball toss

27. Ambulation Training
Walking forward and backward
Tandem (toe to heel)
Braided walking
Walking w/ 180 degree turns
Ladder work multiple patterns
Jogging

28. VOR Treatment
Patient performed VORx1 and VORx2
Eventually progressed to both in combination with forward walking
ambulation
Pt. position is holding object with a single letter with shoulder flexed
to 90 degrees and elbow extended
Object was standard post-it note
VORx1
Pt. keeps gaze fixed on an object while rotating head approximately 45
degrees in transverse plane
VORx2
Pt. keeps gaze fixed on an object while rotating their head in one direction,
as the object is moving simultaneously in the opposite direction of the head

29. VORx1
Head rotated 45 degrees
to the right.
Gaze fixed on object
Starting position. Gaze
fixed on object. Head in
neutral position
Head rotated 45 degrees
to the left. Gaze fixed on
object

30. VORx2
Starting position.
Gaze fixed on object.
Head in neutral
position
Head rotating to right
while object moves
to left. Gaze stays
fixed on object
Head rotating to left
while object moves to
right. Gaze stays
fixed on object