CLINICAL PRESENTATION OF

PATIENT DX WITH RAMSAY HUNT

SYNDROME

By Jere Hess

What is Ramsay Hunt Syndrome (RHS)

a.k.a Herpes Zoster Otticus

Shingles attack C.N VII 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.N VII 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

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

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

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 RHS have been eliminated

RHS Treatment

Initially

Anti-viral drugs

Corticosteroids

Anti-anxiety meds (vertigo symptoms only)

Pain relievers

Long-term

PT may be prescribed to restore functional capacity

Dx RHS

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

Patient History

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

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

Cranial Nerve VII

a.k.a Facial 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 include taste 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

Cranial Nerve VIII

a.k.a Vestibularcochlear Nerve

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

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

Vestibular System

Equilibrium

Balance

Spacial orientation

Innervated by Cranial Nerve VIII

Primary organs

Semicircular canals, saccule, and utricle

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”

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

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…)

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

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

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

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

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

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

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

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.

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.

Other Treatment Strategies

Jumping on trampoline and jumping rope

Causes verticle displacement

Challenges saccule

Single leg ball pick-ups

Single leg balance ball toss

Ambulation Training

Walking forward and backward

Tandem (toe to heel)

Braided walking

Walking w/ 180 degree turns

Ladder work multiple patterns

Jogging

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

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

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