We would accomplish many more things if we did not think of them as impossible

- Vince Lombardi

VESTIBULAR FUNCTION & DYSFUNCTION: A CASE STUDY APPROACH

Presented by:

Chris Zalewski
NIH, Audiology zalewski@nidcd.nih.gov

American Academy of Audiology Web Seminar Spring 2011

We would accomplish many more things if we did not think of them as impossible
- Vince Lombardi

HOW DO I PUT THE VESTIBULAR PUZZLETOGETHER FROM THE VARIOUS PIECES ?

Presented by:

Chris Zalewski
NIH, Audiology zalewski@nidcd.nih.gov

American Academy of Audiology Web Seminar Spring 2011

We would accomplish many more things if we did not think of them as impossible
- Vince Lombardi

HOW DO I PUT THE VESTIBULAR PUZZLETOGETHER WHEN VARIOUS PIECES ARE MISSING ?
Presented by:

Chris Zalewski
NIH, Audiology zalewski@nidcd.nih.gov

American Academy of Audiology Web Seminar Spring 2011

Presentation Objectives
1.

Further our comprehensive understanding of vestibular function (and dysfunction) through a case study approach Review the advantages and limitations of various vestibular tests
1. 2. 3. 4.

2.

Computerized dynamic platform posturography Vestibular evoked myogenic potentials Rotational assessment Videonystagmography

3.

Example normal, absent and abnormal responses for each assessment tool Illustrate how the sum of the parts is far better (and often necessary) than any individual measure

4.

Understanding Normal Balance Function

a prerequisite

Balance is a Multi-Sensory Interaction

The maintenance of body equilibrium and posture in everyday life is a complex function involving multi-receptor organs and neural centers. In particular, the visual, somatosensory, and proprioceptive reflexes must be integrated with the vestibular reflexes in order to ensure postural stability. It is the interaction and intimate relationship between these systems that provide balance and postural stability.

Postural Neural Pathways

- Adapted from Canalis & Lambert, 2000

Lateral SCC

eft Labyrinth Le

Saccadic Optokinetic Other System Tracking System Neck Receptors System

Anterior SCC Posterior SCC Utricle Saccule Vestibular Ocular Reflex (VOR) Eye Muscles

Central Vestibular Nuclei

Lateral SCC

Adaptation (cerebellum)

Right Labyrinth

Anterior SCC Posterior SCC Utricle Saccule

Visual Proprioceptive Tactile Other

Vestibular Spinal Reflex (VSR)

Skeletal Muscles

Beyond the Vestibular System

Until recently, clinical vestibular testing was primarily system oriented. An isolation of each system the visual, the somatosensory, and the vestibular was controlled to evaluate each i d independently d tl whereby h b the th overall ll balance b l function f ti of f the th patient was inferred This approach has significant limitations because the visual system (or oculomotor reflexes), the somatosensory system (or vestibulospinal reflexes) AND the vestibular system are complex functions that contribute to a coordinated response where one system can have significant impacts on how another system performs. nevertheless

understanding the primary vestibular role is imperative

The convergence and interaction of sensory information is primary believed to be coordinated through the vestibular system specifically the central l vestibular ib l system (vestibular ( tib l nuclei). l i) The vestibular nuclei can be thought of as the common central processor which coordinates the massive amounts of sensory input in order to formulate the most appropriate sensory output for maintaining posture and balance.

 therefore

A fundamental understanding of the normal vestibular system is imperative to the understanding of balance function and postural control
however, keep in mind that a fundamental understanding of ONLY the vestibular system leaves balance assessment extremely limited and under-investigated

Computerized Dynamic Platform Posturography (CDPP)

Components of CDPP Balance Normal Balance Function using CDPP Case study: A new interpretation of CDPP Case Study: A case of uncompensated vestibular functionor not

Computerized Dynamic Platform Posturography (CDPP)

CDPP is divided into two primary tests:

The Sensory Organization Test (SOT) which manipulates the visual and proprioceptive inputs while determining the effects on equilibrium The Motor Control or Movement Coordination Test (MCT) which evaluates the muscle response to various computer-induced platform perturbations Other specialty Tests

Equipment

Equipment
Sway referenced surround 4 Ant-Post force pressure gauges

1 Shear force pressure gauge

Sway referenced support

Sensory Organization Test (SOT)

Test paradigm consists of 6 subtests which are designed to tease out the overall contributions, as well as the strengths & weaknesses of the y components p of three sensory equilibrium (visual, vestibular & somatosensory)

SOT Subtests 1-3: Fixed Platform

Condition 1

Measures the patients stability while the patient stands on a fixed platform with a fixed visual surround and with eyes open All sensory components are active

Condition 2

Measures the patients stability while the patient stands on a fixed platform with a fixed visual surround and with eyes closed (Romberg Test) Vestibular and somatosensory systems active, absent visual cues Should have little effect on balance since the absence of visual cues have been shown to have minimal effect on equilibrium in the presence of functional vestibular and somatosensory systems

Condition 3

PREF

Measures the patients stability while the patient stands on a fixed platform with eyes open, however, the visual surround is sway referenced to the A-P sway measured by the platform Provides orientationally inaccurate visual cues does the patient rely too heavily upon visual cues (visual preference)? The brain is asked to ignore the inaccurate visual input and rely on the orientationally accurate vestibular and somatosensory (proprioceptive) inputs

SOT Subtests 4-6: Moving Platform

Condition 4

Measures the patients stability while the patient stands on an un-fixed platform with a fixed visual surround and with eyes open As a result, the proprioceptive input to the brain is inaccurate and balance must be maintained by the visual and vestibular system

Condition Co dto 5

Measures the patients stability while the patient stands on an un-fixed platform with eyes closed (visual surround is fixed, but with eyes closed this does not matter) This condition isolates the vestibular system more than any other Since the visual and proprioceptive systems are compromised, balance and equilibrium must be maintained by the vestibular system alone

Condition 6

PREF

Measures the patients stability while the patient stands on an un-fixed platform with eyes open however, the visual surround is sway referenced to the A-P sway measured by the platform This condition also involves the vestibular system but to a lesser degree than condition 5 Both the visual and the proprioceptive systems are compromised, but moreover, the brain must also ignore the inaccurate visual inputs and rely on the orientationally accurate vestibular system (further evaluates visual preference

Understanding the SOT

Center of Gravity (COG)

In normal subjects standing erect, the COG is located in the lower abdominal area and slightly forward of the ankle joints

COG Sway Angle

The degree of sway from the vertical COG using ankle strategy

Understanding the SOT

Limits of Stability (B)

The maximum A-P or lateral sway angle without losing balance (approx. 12.50 off COG) - when the COG sway angle exceeds the limits of stability, stability the patient must step, step stumble, or grasp to regain equilibrium

The Equilibrium Score (A)

Calculated for each condition Represents the angular difference between the patients calculated maximum sway angle and the COG theoretical maximum (12.50) Result is a percentage with 100% indicating perfect stability

SOT Raw Postural Trace Data

The Composite Equilibrium Score (CES)

Calculated percentage score representing the patient's overall equilibrium ability (compared to age, weight and height-matched norms) Examination of the CES provides a global determination of normal versus abnormal

NORMAL TRACINGS

SOT Data
Sensory Analysis Summary

Sensory Analysis

When the composite score falls within the abnormal range, the second interpretation is needed to identify the sensory dysfunction and/or abnormal sensory preference contributing to the overall sensory organization abnormality When the composite score is significantly below the normal limit and the equilibrium scores are abnormal for most conditions, a specific sensory abnormality or pattern may not be discernible. This patient may have a multi-sensory dysfunction (more to come)
SENSORY ANALYSIS

Ratio SOM VIS VEST PREF

Formula
Condition 2 Condition 1 Condition 4 Condition 1 Condition 5 Condition 1 Condition 3+6 Condition 2+5

Questions and Significance if ABNORMAL

Q: Does sway increase when VIS cues are removed ? A: Patient makes poor use of SOM references Q: Does sway increase when SOM cues are inaccurate ? A: Patient makes poor use of VIS references Q: Does sway increase when VIS cues are removed & SOM cues are inaccurate ? A: Patient makes poor use of VEST references Q: Do inaccurate VIS cues result in increased sway compared to no VIS cues ? A: Patient relies on VIS cues even when inaccurate

Case Study: y A Different Approach to Interpreting Dynamic Posturography

History

38 y/o male presents with a previous diagnosis of USHER Syndrome type I Diagnosed at 2 years of age was facilitated by two older siblings with the same diagnosis

Usher Syndrome Types

Type USH1 Visual Onset of RP by ~ 10 years old Onset of RP in late teensearly 20s Onset of RP in late teensearly 20s Auditory Congenital deafness Congenital moderate to severe SNHL Progressive SNHL, may be near normal at birth, deaf when older Vestibular Congenital absence of function Normal Progressive balance dysfunction

USH2

USH3

Hearing History

Believed to be congenitally deaf with most recent audiometric evaluation in middle school that revealed little if any measurable hearing bilaterally Hearing aid history is significant for use of a body aid during elementary school and middle school providing little (if any) success which was limited to sound awareness Hearing aid use discontinued in high school. After, attended Gallaudet University. ASL user.

Balance History

Self reported balance problems, describing himself as clumsy, uncoordinated and overall not great (often walking like a drunk) Attempted school sponsored sport activities such as tennis, basketball and baseball with limited proficiency Unsure of exact age when he started walking, but did report delayed ability

Visual History

Retinitis pigmentosa diagnosed at the age of 2 years Visual ability remained fairly good and consistent through high school During college, RP reported to begin impacting visual acuity and peripheral vision Current visual function: Right eye totally blinded Left visual field is confined to an approximate 5-10 degree range that is left of center field - acuity within FOV is reported to be 20/30 No night vision

Miscellaneous History

Sustained head injury at 6 years of age after running into a tree at night

Resulted in LOC without skull fracture

Utilizes ASL for communication as well as is fluent in tactile ASL

Audiometric Results

No measurable hearing to speech or pure tones bilaterally Normal N lt tympanometry t bilaterally Absent middle ear reflexes bilaterally Absent DPOAEs bilaterally

Videonystagmography Results

Oculomotor assessment was WNL - despite the limited visual field (left eye tested only) Positional i i l testing i was WNL

10

Caloric Testing

Caloric testing revealed absent labyrinthine reactivity to both cool and d warm air i stimuli ti li Ice water irrigations further resulted in no measurable reactivity bilaterally

Rotational & VEMP Testing

Rotational Vestibular Testing revealed absent VOR gain for the frequency range of 0.01-0.64 Hz Absent VEMPs bilaterally

0 ms

0 ms

Conclusions thus far

Profound bilateral SNHL bilaterally Absence of any peripheral vestibular reactivity evidenced by:

Absent VOR response by air and ice water caloric irrigations Absent VOR gain on RVT Absent saccular activity by VEMP testing

What can functional assessment via dynamic platform posturography reveal ?

11

but first,
What is expected on posturography with an USHER type I patient ? absence of vestibular function, right?

what to expect

Conditions 5 & 6 on platform SOT testing isolates and determines overall contributions of the vestibular system to postural stability

Typical USHER type I SOT Pattern

Note the absence of vestibular contribution to over postural stability (condition 5/6 SOT pattern) concomitant to significantly reduced use of visual input

12

Posturography Results for our USHER patient

Computerized Dynamic Platform Posturography Sensory Organization Test Results (SOT)

What Does SOT Reveal ?

Is there vestibular function ? Is there any vestibular contribution when maintaining posture ? What can functionally be said of this patients performance ?

What Does SOT Reveal ?

VNG, RVT and VEMP test results support an absence of vestibular activity in this patient However, this is a functional measure The fundamental question is NOT how much the vestibular system is contributing to postural stability in this patient, but rather

13

What Does SOT Reveal ?

How does this patient perform or function in an environment that f demands a specific sensory input ? In other words, how does this patient function in a vestibular dependant environment ?

CDPP Case Study

A case of uncompensated vestibular function or not?

Case Study 2: History

48 y/o male presents with a previous diagnosis of von Hippel-Lindau (VHL) disease VHL is an autosomal dominant disease causing a mutation on 3p25-26 (tumor suppressor gene function) VHL is characterized by a predisposition to bilateral retinal angiomas, CNS (cerebellar) and spinal cord hemangioblastomas, renal cell carcinomas, pheochromocytomas, islet cell tumors of the pancreas, endolymphatic sac tumors, and renal / pancreatic & epididymal cysts

14

Hearing History

Previously documented bilateral moderate, high frequency, notched sensorineural hearing loss Persistent complaints p of difficulty y with speech p understanding g - particularly in adverse listening environments No history of hearing aid use Hearing history is positive for noise exposure with reported consistent use of hearing protection devices Complaints of pruritis for two months

Balance History

Previous vestibular testing in 2000 revealed:

Bilateral failure of fixation suppression, Saccadic tracking during smooth pursuit testing, Robust caloric response to cold (only) irrigations, and Rotational vestibular testing (RVT) revealed reduced VOR gain above 0.16 Hz with concomitant abnormal phase lead times above this frequency

Patient reports a subjective complaint of continued worsening in balance - particularly when maneuvering over slopes and unstable surfaces. Has experienced increasing fainting episodes over the last year.

Balance History

During routine otoneurologic evaluation, patient was noted to have an abnormal Romberg and tandem walking test Patient noted to have significant postural stability problems and often utilized his surroundings for support. Despite this, patient was noted to lose his stability and fall into the exam chair. Patient regularly used the wall during ambulation

15

Miscellaneous History

Multiple VHL related tumors History most significant for cerebellar surgery in 1993 for resection of hemangioblastomas Patient reports monitoring of cerebellar tumors with no immediate plans for resection

Audiometric Results

Bilateral mild-to-moderate / moderately severe SNHL (right slightly greater than left). Normal speech recognition testing testing. Normal tympanometry bilaterally Middle ear reflexes present contra-lateral at appropriate sensation levels DPOAEs not performed

Videonystagmography Results

Previous findings in 2000 revealed:

Bilateral il l failure f il of f fixation suppression Bilateral cog-wheeling on smooth pursuit

16

Caloric Testing Only

Caloric testing revealed (borderline) reduced vestibular response in the right ear (20%) with no evidence of directional preponderance No evidence to support failure of fixation suppression (improvement from 2000)

LEFT

RIGHT

Conclusions thus far

Mild-to-Moderate / Moderately Severe SNHL bilaterally Right peripheral vestibular pathology secondary to right RVR on caloric irrigations Previous VNG results suggesting a central (cerebellar) pathology contributing to his dizziness Significant central lesion(s) suspected on otoneurologic bedside evaluation

Posturography Results - SOT

Multi-sensory dysfunction with unique sway patterns

17

Posturography Results - MCT

Abnormal MCT postural reflexes with backward translations with unique sway patterns

A closer look

Postural sway patterns are not consistent with normal behavior - even with those of pa o pathologic o og c patients pa e s CDP can be a very reliable test to differentiate nonorganic sway from organic sway

A closer look

7 criteria believed to be consistent with aphysiologic response to CDP

1. 2. 3.

4. 5.

Substandard performance on SOT 1 Lower scores on SOT 1 and 2, higher scores on SOT 5 & 6 Repetitive large-amplitude anteroposterior sway without falling Excessive lateral sway without falling Excessive variability on SOTs 1 &2

Goebel, J. et al. (1996)

18

A closer look (cont)

7 criteria believed to be consistent with aphysiologic response to CDP

1. 2.

MCT Results

Exaggerated motor responses to small platform translations Inconsistent motor responses to small and large, forward and backward translations

Goebel, J. et al. (1996)

Normal response

Another objective measure of aphysiologic sway

9 criteria presented by Mallinson et al. (2005) believed to be consistent with aphysiologic response to CDP
1.

2. 3. 4. 5. 6.

Better performance of first trial of SOT 1 and 2 (when unaware of being measured) than on trials 2 & 3 Scores on SOT 5 & 6 relatively better than scores on SOT 1 & 2 SOT 1 & 2 scores all below 75 (markedly below normal) High inter-trial variability seen in scores across all SOT trials Circular sway patterns with falls Repetitive large amplitude suspicious anteroposterior sway without falls

Mallinson (cont)

(Continued)
1. 2 2.

3.

Exaggerated motor responses to small platform translations Inconsistent motor responses to small and large, forward and backward translations. Non repetitive motor responses to all translations Clinical judgment (gut feeling)

Normal response

19

Score analysis for determination of patients aphysiologic sway

Mallison & Longridge aphysiologic Criteria score

DETERMINATION OF APHYSIOLOGIC PERFORMANCE CHARACTERISTICS

MALLINSON CRITERION Comrehensive Report 1. Better Performance on first trial of SOT 1 & 2 (when unaware of being measured) than on 2. Higher inter-trial variability seen in scores across all SOT trials 3. Lower scores on SOT 1 & 2, higher scores in SO 5 & 6 4. SOT 1 & 2 scores all below 75 SOT COG X-Y Plot 5. Circular sway patterns without any falls Excessive Lateral sway without falls (>2.5 deg) Sway, Shear and Alignment Data 6. Repetitive large-amplitude "suspicious" anterior-posterior sway without falls Motor Control Test 7. Exaggerated motor response to small forward & backward platform translations 8. Inconsistent, non-repetitive motor response to all translations and both adaptaions Clinical Impression 9. Clinical judgement ("gut feeling") 1 1 0.5 0.5 0.5 Enter Score 0 0 0 0.5

4 of 9 score indicating probable aphysiologic performance

TOTAL SCORE
0 of 9 1 of 9 2 of 9 3 of 9 Possible suspicion raised (assessment ofen repeated) Probable Aphysiologic Performance ("malingering Definite Aphysiologic Performance ("malingering") No suspicion of aphysiologic ("malingering") behaviour

Cevette et al. aphysiologic criteria

4 of 9 5/9 to 9/9

4 of 9

CEVETTE APHYSIOLOGIC CRITERION Average C1 C2 C4 C6 87.33333333 66.33333333 28 26 Trial 1 87 80 0 0 Trial 2 88 68 55 36 Trial 3 87 51 29 42

Highest score of 117.9 designated to the group aphysiologic

Aphysiologic Normal Vestibular References: Mallinson Al, Longridge NS;

117.99 97.89 90.52333333 "The highest overall value designates the group to which the patient is most likely to belong" with 95.5% certainty (Cevette et al 676)

A New Set of Criteria for Evaluating Malingering in Work-Related Vestibular Injury. Otol Neurotol 26:000-000, 2005 (in press) Cevette MJ,Puetz B,Marion MS,Wertz ML,Muenter MD Aphysiologic performance on dynamic posturography Otolaryngol Head Neck Surg. 112(6):676-88, 1995.

Conclusions ?

An underlying vestibular pathology with an overlaying functional component Evidence to support components of a central and peripheral etiology to patients pathology

Neurotologic Rotational Testing

Components of Rotational Testing Normal Vestibular R Reactivity i i Interpretation of Rotational Testing Case Study: Vestibular loss or sensory integration dysfunction

20

Rotational Vestibular Testing

Test Paradigm

Patients head is tilted 300 forward to place the H-SCC in the plane of rotation Chair is situated in a lightproof enclosure Standard electrode placement and / or video-oculography Chair is turned by a torque motor for several cycles of sinusoidal rotation at each of seven test frequencies (0.01, 0.02, 0.04, 0.08, 0.16, 0.32, & 0.64 Hz) and possibly higher Patient is kept mentally alert similar to the caloric & positional subtests of the VNG Contraindicated medications must also be ceased 48 hours prior to the test

Rotational Vestibular Testing

Test Paradigm

Head rotation is inferred from the chair rotation Th patients The ti t horizontal h i t l eye position is measured through video-oculography, and a nystagmus tracing is generated by the computer program The clinician can then examine the relationship between the patients head and eye movement

Passive Rotational Vestibular Testing

Two primary stimuli employed during passive rotational testing:

1. 2 2.

Velocity Step Testing Sinusoidal Oscillation Testing

Other tests employed during passive rotational testing:

1. 2. 3. 4. 5.

VOR Suppression Oculomotor Assessment Unilateral Centrifugation Visual-Vestibular Enhancement OVAR

21

Velocity Step Testing

Velocity step testing involves a quick angular acceleration of 1000 / second2 lasting for one second (to the left) At the end of the acceleration, the patient is rotating at a constant velocity of 600, 1000, 2400 / second which is maintained through the entire trial

Velocity Step Testing

In response to this stimulus, a burst of (left-beating) horizontal nystagmus is observed Why W y left e t beating? beat g?

Excitation of the left h-SCC causes a rightward slow-phase pull with a leftward fast phase quick saccade to bring the eyes back to primary position (i.e., repeated left-beating nystagmus)

The response gradually dissipates (without visual fixation), and can be followed by a few beats of nystagmus in the opposite direction

Velocity Step Testing

After the completion of the test, the computer eliminates the quick-phases (fast-phases), and calculates the velocity (intensity) for each slow-phase slow phase beat yielding a plot of slow-phase eye velocity This plot evidences a rapid burst of SPV, an exponential decline back to zero, and finally a weak reversal

22

VOR Time Constant

Despite on-going velocity, the cupula of the h-SCC returns to its resting position at about 6-7 seconds. However, the VOR response continues well past this time frame The persistence of VOR is due to velocity storage The point at which the VOR response decays 37% from its peak response is known as the time constant of the VOR and should be greater than 10 seconds.

Normal Velocity Step Test

Abnormal Velocity Step Test

23

Velocity Step Testing Abnormalities

Velocity Step Testing
Abnormality
1.

Possible Interpretation Peripheral UVL if oculomotor testing is normal, likely labyrinth or VIII Nerve At 600 or 1000 / sec, information is from both labyrinths Non-localizing cupular time constants plus velocity storage gains should be >0.3; if not, consider migraine Abnormal study; non-localizing

Rule Out Inattention; ; too much blinking; bilat loss; fixation Inattention; too much blinking Eye closure (eyes must be open when chair starts & stops)

Low time constant (<10 sec) 600 / sec If 3 of 4 time constants are abnormal Consider peak slow phase velocity; >30% difference between CW & CCW directions?

2. 3.

1.

1.

Significant asymmetric results in peak SPV indicate peripheral UVL and side of loss

2400 / sec

Slow Sinusoidal Oscillation Testing

As the chair and patient begin to rotate, a slow, compensatory eye movement is observed in the di ti opposite direction it the th rotation. t ti An indirect measure of vestibular sensitivity to rotational stimuli Assesses the h-SCC, central systems and the vestibular nuclei

Head Movement
Max VOR Operational Range 26 Hz

Calorics 0 004 Hz 0.004

Rotary R t Ch Chair i 0.01 Hz -2.0 Hz

Normal Head Motion 0.5 Hz 5 Hz

VOR linear range 0.1 Hz 10 Hz

24

Maximum Velocities During typical Daily Life Tasks

Walking Horizontal 36 deg/sec Vertical 32 deg/sec Running Horizontal 62 deg/sec Vertical 87 deg/sec Driving at 30 mph 84 deg/sec Competitive sports and high performance 120 deg/sec
Grossman G, Leigh R, et al. (1988, 1990)

Sinusoidal Oscillation Testing

The most widely used rotational test is the slow-harmonic acceleration test Patient undergoes sinusoidal oscillations about a vertical axis at several different frequencies (0.01, 0 02 0.04, 0.02, 0 04 0.08, 0 08 0.16, 0 16 0.32 0 32 & 0.64 0 64 Hz) Constant-changing accelerations (+/-) achieving a peak 600/sec head velocity The saccadic (fast) eye movement returns the eye to its primary central position and is noted to be in the same direction as the rotation

(0.16 Hz)

Right-Beating on rightward rotation Left-Beating on leftward rotation However, the slow phase is what is measured / calculated (fast-phases are removed by the analysis)

Parameters of Sinusoidal Oscillation Testing

The relationship between slow-phase eye velocity and head velocity is described by three parameters:
1. 2. 3. 4.

Spectral Purity: A measure of the quality of the data collected Gain: the ratio of peak eye velocity to head velocity Phase Angle: The reaction time of eye movement in response to head movement Symmetry: The ratio of rightward and leftward slow-phase eye velocities

NORMAL PATTERN

25

Sinusoidal Oscillation Testing

Again, if the VOR function is to produce equal and opposite eye movements to that of head movement than the SPEV plot in d would have been the exact mirror image of head velocity b However, it was not. The gain was only 0.66 (0.4 / 0.6) That is, the eyes did not move quite quick enough during the nystagmus slow-phase to compensate for, or entirely match head/chair movement
d b

(0.16 Hz)

Sinusoidal Oscillation Testing

A note on VOR Gain

Shown here is an absence of VOR gain Phase and Symmetry are calculated from Gain Phase and Symmetry should be analyzed / interpreted with caution when VOR gain is 1015%

Sinusoidal Oscillation Testing

There are two things that are critical to RVT, which, if you do not control for, you may as well not test your patient

Head restraint Tasking

26

Sinusoidal Oscillation Testing

There are two things that are critical to RVT, which, if you do not control for, you may as well not test your patient

Head restraint Tasking

Parameters of Sinusoidal Oscillation Testing

(0.16 Hz)

The relationship between slowphase eye velocity and head velocity is described by three parameters: p

Gain: the ratio of peak eye velocity to head velocity Phase Angle: The reaction time of eye movement in response to head movement Symmetry: The ratio of rightward and leftward slowphase eye velocities

VOR Phase

The temporal relationship between the velocity of the head (chair) and that of the slow-phase component of the rotational-induced nystagmus. Again, as the chair and patient begin to rotate, the slow, compensatory nystagmus is observed to move in the opposite direction of the chair (head) rotation The delay to which the eye moves is the phase when perfect, rotation. perfect it moves at exactly the same time and pace as the chair only in the opposite direction. BUT THIS DOES NOT HAPPEN in fact, eye movement tends to be ahead of (or lead) chair movement the slower the chair/head rotation

27

Phase Lead by Frequency - Normal

0.01 Hz Average SPV 0.16 Hz Average SPV

0.02 Hz Average SPV

0.32 Hz Average SPV

0.04 Hz Average SPV

0.64 Hz Average SPV

0.08 Hz Average SPV

Parameters of Sinusoidal Oscillation Testing

VOR phase approaches zero (exactly 1800 out of phase) around 0.08 or 0.16 Hz

NORMAL PATTERN

Sinusoidal Oscillation Abnormalities

Sinusoidal Harmonic Acceleration Testing
Parameter Abnormal Result Low VOR gain for low Hzs (<0.04-0,08 Hz) GAIN Low VOR gain for all Hzs High VOR gain for all or most
1.

Possible Interpretation With concomitant abnl phase lead at low Hzs & asymmetry uncompensated UVL on side of asymmetry With no phase abnls but abnl asymmetry, possible irritative or stable lesion (side uncertain) No other abnl abnls s & normal spectral purity purity, compensated UVL is likely BVL given eyes open during test & asymmetry and phase uninterpretable Vestibulotoxic medication, ageing, rare degenerative disorders of the brainstem and / or cerebellum (esp if calorics are normal) Cerebellar lesion (associated occulomotor abnormalities) Has been observed in migraine and hydrops Peripheral vestibular end-organ lesion / vestibular nuclei lesion With concomitant asymmetry, uncompensated UVL (on side of asymmetry) Acute vestibular end organ lesion; vestibular hydrops CNS lesion; (associated occulomotor abnormalities)

Rule Out Insufficient alerting

2. 3 3. 1. 2.

Insufficient altering, restricted EOM, fixation Medications; stimulants Compare with Step Tests & calorics Lateral Medullary Syndrome

1. 2.

1.

Low Hz Phase lead

2. 3.

PHASE

High Hz Phase lead Low / High Hz Phase lead

1.

1.

CNS lesion; (associated occulomotor abnormalities); consider lesions involving brainstem or posterior cerebellum; cerebellar nodulus Two or more consecutive abnormal Hzs; similar to DP on calorics (non-local) With low Hz phase lead, uncompensated peripheral lesion on side of asymmetry Unstable lesion with normal phase findings

1.

SYMMETRY

Asymmetric SPV

2.

28

RVT Case Study

Vestibular Loss or Sensory-Integration Dysfunction ?

CaseStudyHistory:

56 y/o male disabled geologist Occasional non-localizing tinnitus since Aug 2008 Occupational noise history of 10+ years from machinery for specimens as a geologist Repeated sensations of dysequilibrium which he characterizes as occurring once per week with each episodes duration being several minutes each time Reports vague R sensations i during d i ambulation b l i and d has h a very difficult diffi l time i recovering i from unexpected disturbances in his environment He further reports a definitive unsteadiness particularly while maintaining a crouching position Reports hyperacusis to sounds such as pots and pans and coffee scooping 1998 Bells Palsy 1999 right pinna edema Aug 2008 single episode of true vertigo with movement x 1day MRI of brain in June 2008 was WNL CN exam II-VII, IX-XII grossly intact

Audiometry

Normal tympanometry with the exception of hyper-mobility in the left ear Present acoustic stapedial reflexes bilaterally

29

Videonystagmography (VNG)
Nooculomotor abnormalities Noevidenceofanypositionalorspontaneous nystagmus Calorictestingrevealedrobustlabyrinthinereactivity tostandardaircaloricstimuli. Noevidenceofanyfailureoffixationsuppression

Cool Left

Cool Right

290 RB Warm Left Warm Right

220 LB

260 LB

240 RB

Incidence of ENG Abnormalities

2584 ENG results reviewed from a variety of settings - private otolaryngology, neurology, and audiology offices and as well as hospital based audiology and neurology departments. 61% (1571 tests) showed no abnormalities
- Stockwell, 2000

Computerized Dynamic Platform Posturography (CDPP)

30

Dynamic Posturography SOT

Across the Board Pattern Rule out:

Anxiety, Non-Organic, & Multi-Sensory

SOT Raw Sway Data

Raw Sway Data

Center of Gravity Raw Data

Non-Organicity Determination
DETERMINATION OF APHYSIOLOGIC PERFORMANCE CHARACTERISTICS
MALLINSON CRITERION Comrehensive Report 1. Better Performance on first trial of SOT 1 & 2 (when unaware of being measured) than on 2. Higher inter-trial variability seen in scores across all SOT trials 3. Lower scores on SOT 1 & 2, higher scores in SO 5 & 6 4. SOT 1 & 2 scores all below 75 SOT COG X-Y Plot 5. Circular sway patterns without any falls Excessive Lateral sway without falls (>2.5 deg) Sway, Shear and Alignment Data 6. Repetitive large-amplitude "suspicious" anterior-posterior sway without falls Motor Control Test 7. Exaggerated motor response to small forward & backward platform translations 8. Inconsistent, non-repetitive motor response to all translations and both adaptaions Clinical Impression 9. Clinical judgement ("gut feeling") 0 0.5 0 0 0 Enter Score 0 0 0 0

Negative for Mallinson & Longridge

TOTAL SCORE
0 of 9 1 of 9 2 of 9 3 of 9 4 of 9 5/9 to 9/9 Possible suspicion raised (assessment ofen repeated) Probable Aphysiologic Performance ("malingering") Definite Aphysiologic Performance ("malingering") No suspicion of aphysiologic ("malingering") behaviour

0.5

CEVETTE APHYSIOLOGIC CRITERION Average C1 C2 C4 C6 92.33333333 84.33333333 51 49.33333333 Trial 1 94 88 73 52 Trial 2 94 81 80 55 Trial 3 89 84 0 41

Aphysiologic Normal Vestibular

175.32 171.2566667 159.73 "The highest overall value designates the group to which the patient is most likely to belong" with 95.5% certainty (Cevette et al 676)

POSITIVE for Cevette (et al) ???

Black et al, (1999) & Longridge & Mallinson, (2005)

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Dynamic Posturography - MCT

Prolonged postural motor reflex latencies to backward translations Essentially normal amplitude scaling

Rotational Vestibular Testing (RVT)

Slow Harmonic Acceleration (0.01-0.64 Hz) 600 Trapezoidal Step Testing VOR Suppression testing (0.16 Hz & 064 Hz)

RVT Slow Harmonic Acceleration Summary

Increased VOR phase lead

Normal VOR gain

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RVT - 600 Step Testing

RVT - 600 Step Testing

Phase & Time-Constant: An Inverse Relationship

T= 1 (2f)tan T = 1 / (20.01)tan580 T=1/( (0.0628)1.6 ) T = 1 / 0.10048 T = 9.95 sec

If no step testing was conducted, a time constant can be calculated from the phase value of your random sinusoidal oscillation testing at 0.01Hz (generally restricted to 0.04 Hz and below) An inverse relationship exists: as phase angle increases, time constant decreases Increased abnormal phase leads, related to a decrease in time constant, suggests a pathology of the peripheral system however, damage to the central vestibular nuclei within the brainstem may also result in abnormally low time constants

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VOR Fixation Suppression 0.16 Hz

Average Cycle

VOR Fixation Suppression 0.64 Hz

Average Cycle 0.4 gain

Clinical Summary

Asymmetrical, high frequency SNHL (L>R) Normal middle ear function bilaterally Normal VNG Abnormal Dynamic Posturography

Across the Board SOT pattern Prolonged backward MCT latencies Abnormal phase lead across the entire frequency range in the presence of normal VOR gain Abnormal Time Constants Failure of fixation suppression Normal Visual-Vestibular Enhancement

Abnormal RVT

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Conclusions

Patient diagnosed with a positive Lyme titer Sensory integration dysfunction likely involving central vestibular nuclei and motor sensory input (long loop motor pathways) evidenced on CDP Central vestibular pattern on RVT suggesting central vestibular velocity storage integration dysfunction Vestibular compensation outlook is questionable given cerebellar dysfunction in regulating vestibular input/output Sensory integration training suggested to reorganize / retrain sensory management

Vestibular Evoked Myogenic Potentials (VEMP)

Components of VEMP Testing Normal VEMP Responses p Case Study: Answering a question of IAC compromise, or Here comes VEMP to save the day

Vestibular Evoked Myogenic Potentials

Short latency myogenic response to loud clicks recorded on the ipsilateral sternocleidomastoid muscle (SCM) Contraction of the SCM is held at a relatively constant throughout the recording VEMP is an inhibitory response of the SCM

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Vestibular Evoked Myogenic Response Testing (VEMP)

Since 1916 (Tullio), it has been known that the vestibular system (of pigeons) was sensitive to sound. It has been also suggested that the VEMP p may y be a consequence q of the response proximity of the saccule to the stapes footplate and eddy currents set up in the endolymph by sudden movement of the stapes. The VEMP is abolished by selective vestibular neurectomy, but may be present despite profound deafness, as long as there is not significant conductive component present to attenuate the stimulus <88 dB nHL (10 dB at 1000 Hz)

Vestibular Evoked Myogenic Response Testing (VEMP)

Medial Vestibular Nuclei Medial Vestibulospinal Tract Motorneuron of the SCM muscle

This response is thought to be mediated by a neural pathway originating in the saccule, then proceeding via the inferior vestibular nerve, medial vestibular nucleus, and medial vestibulospinal tract to the motor neurons of the SCM muscle.

VEMP Testing Paradigm

VEMP, therefore, is a vestibular originated myogenic electrophysiologic response to loud monaural clicks or tonebursts. Electrode Montage

Active (Non-Inverting) Electrode Middle to Upper of the SCM Ground Forehead Reference (Inverting) Electrode Upper Sternum .1 msec square pulse or 500 Hz tone burst 5.1 c/sec (repetition rate) Insert earphones 88-100 db nHL Reclined position with head turned as far as possible toward the side opposite the stimulating ear and held slightly elevated thus tonically contracting the ipsilateral SCM 200-500 sweeps (< 3minutes) are delivered to the ipsilateral ear with a recording window set to 80 msec and gain set to 5k 30k Myogenic SCM response is averaged and analyzed

Stimulus

Patient

Brainstem m Midline

VEMPs can easily (and almost universally in normal subjects) be recorded from the ipsilateral contracted sternocleidomastoid (SCM) muscle by stimulation with loud monaural clicks or tone bursts. bursts

Saccular Afferents Inferior Vestibular Nerve

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VEMP Waveform

The P1-N1 response, while still an evoked potential, is generated by synchronous changes in motor unit activity: it is a myogenic potential An additional consequence of the myogenic nature of this response is that these evoked potentials are of relatively large size compared with most neurogenic evoked potentials.

VEMP Norms

Normative Parameters of the waveform are identified

(Ochi, Ohashi & Nishino, 2001)

Absolute latencies of P1 (11.30 ms 1.50) Absolute Ab l t latencies l t i of f N1 (20.54 (20 54 ms 2.81) Inter-aural amplitude differences for P1 (0.86 ms 0.61) Inter-aural amplitude differences for N1 (1.68 ms 1.31) P1-N1 ratio (%) (13.6 12.1) VEMP threshold (87.78 dB nHL 4.54) Inter-aural threshold difference (1.67 dB nHL 2.43)

EMG Monitoring Techniques

Intelligent Hearing Systems (IHS)

Target EMG activity range EP sweeps are rejected if EMG activity is outside target range

Target Level EMG Monitoring

Bio-feedback of SCM muscle activation

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VEMP Case Study

Answering a Question of IAC Compromise ? Here comes VEMP to save the day...

Case History

12 year old female with Fanconi Anemia Fanconi Anemia is a type of Inherited Bone Marrow Failure Syndrome Most people with Fanconi's anemia have these types of symptoms

Skin pigment change (darkened areas of the skin, cafe-au-lait spots, vitiligo) Short height Upper pp limb p problems ( (missing, g, extra or misshapen p thumbs; ; small or missing g radius bone in the forearm; problems of the hands and the forearm bone in the lower arm) Small testicles, genital changes Abnormal bones (abnormalities of the hip, spine or rib; curved spine (scoliosis); small head) Abnormal eye/eyelid Malformed kidney Abnormal ears/deafness Abnormal hip, leg, and toe Abnormal digestive tract/heart and lungs Hearing loss
Mental retardation Learning disability Low birth weight Failure to thrive

Other possible symptoms

Patient History

Presents with otitis media in the right ear and a recent CT scan that identified a narrowing of the left IAC. History of profound (no measurable) hearing in the left ear with normal hearing in the right ear. Nuerotologic concern regarding constriction of neural tracts (Facial, Auditory & Vestibular) through left IAC No balance / dizziness complaints reported from patient or her parents
Picture is NOT case study presentation but is Fanconi Anemia (www.google/images.com)

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Audiogram History

Initial audiometric evaluation at the NIH (3/28/2006) SRT RE: 20 dB HL; SAT LE: 94 dB HL Normal tympanometry LE; Flat tympanogram RE (normal volume)

Follow-up Audiometric Evaluation

(5/3/2007)

RE

No response LE

Profound SNHL previously documented in the left ear (2006) OME diagnosed in the right ear on this evaluation

CT Axial Imaging

Significant narrow width

Normal width

Left IAC

Right IAC

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Auditory Brainstem Response

Left Ear Right Ear

Summed response

Summed response

No response LE

Cochlear Microphonic
Left Ear Right Ear

No response LE

VEMP Recordings
CHL

No response LE

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Summary
VEMP
LEFT EAR

Cochlear Microphonic

ABR

No response LE

VEMP
RIGHT EAR

Cochlear Microphonic

CHL effect

ABR
No response LE

Conclusions

Although no left-sided facial nerve effects were observed, VEMP was instrumental in providing the only objective / measurable evidence that IAC stenosis was not impacting p g vestibular integrity g y Although the ABR and CM was most likely absent secondary to the degree of peripheral hearing loss, VEMP was possible in spite of this hearing loss Caloric irrigations were not possible with this patient secondary to tolerance problems

Videonystagmography (VNG)

A comprehensive look at VNG? A Comprehensive Case Study An Unfortunate Vestibular Demise

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a look at VNG testing

Normal results are easy (64%?) S are So no response patients ti t (or ( the th straightforward unilateral weakness patients) Its everything else in between that causes the problemsand no two are (ever or seldom) alike

VNG Testing;
The Didactic Approach - Teaching the Puzzle

Oculomotor Assessment Positional Assessment Caloric Assessment

Providing all the VNG puzzle pieces during didactic presentations like you hope p you y can provide p all the critical pieces p (rules ( this is difficult as y and guidelines) so one can still interpret the entire picture independent of the puzzle (patient). This is difficult as no two patients are alike and can often lead to a dangerous path of misinterpretation

Pitfalls and Errors Rules and Guidelines for Interpretation Anatomy and Physiology Often your best tool of interpretation

PATIENCE, PRACTICE AND PRUDENCE

Comprehensive Case Study

An unfortunate vestibular demise

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History

27 year old male presents to the NIH with an unknown diagnosis First symptoms of visual blurriness in October of 2000 First began g noticing g balance difficulties following g a brain biopsy in May of 2006 Onset of bilateral tinnitus approximately 2 years ago History significant for two distinct episodes of vertigo

Each persisted for 1-2 days First in July / August of 2010 Second episode October / November of 2010 Second episode was noted to be in the vertical plane (backwards)

Medical history

Admitting Sxs: Recurrent encephalopathy, headache, and gastrointenstinal disorder

Onset of Sxs of severe headache at age 6-7 complicated by recurrent sinusitis Migraine with visual scintillations (2-3/month) in teens Depression Recurrent kidney stones Encepaholopathy (age 13) - experienced first episode of transient dysarthria and left upper extremity weakness; CT at this time was unrevealing Second episode of focal neurological dysfunction three years later (age 16); CT consistent with abnormality in left basal ganglia Between 2000-2004 imaging (19 cranial MRIs) showed waxing and waning signal changes with a working diagnosis of an indeterminate forebrain inflammatory process Age 22 (2006) extensively evaluated at Mayo Clinic with non-specific myelin damage and inflammatory cell infiltrate; again given Dx of indeterminate inflammatory process April-July of 2010 self-described his condition as much worse with fatigue, headache, severe vertigo ,blurred vision, oscillopsia, and significant ataxia

Medication history

Prednisone (antiinflammatory) Neurontin (control seizures / neuralgia pain relief) Diazepam (anti-anxiety) Xanax (anti-anxiety) Z l f (depression) Zoloft (d i ) Baclofen (spasticity) Colazal (ulcerative colitis) Fosamax (osteoporosis) Immunosupressive therapy
Note: when possible, meds were suspended for vestibular testing

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Vestibular Assessment

VNG

Positionals Caloric Irrigations Oculomotor SHA 600 & 2400 Step Testing VOR Suppression

Rotational

VEMP Deferred CDPP secondary to significant ataxia

Spontaneous

Vision denied - gaze right

Vision denied - gaze left

30 Left-Beating

Positional Testing - Supine

Supine Head LEFT Supine Head RIGHT
6-70 left-beating 50 left-beating

Horizontal / vision denied

Horizontal / vision denied

40 down-beating

Vertical / vision denied

Vertical / vision denied

Horizontal / fixation

Horizontal / fixation

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Positional Testing - Laterals

Lateral LEFT Lateral RIGHT
40 left-beating 70 left-beating

Horizontal / vision denied

Horizontal / vision denied

30 down-beating

Vertical / vision denied

Vertical / vision denied

Horizontal / fixation

Horizontal / fixation

Caloric Position (Positional)

40 Left-Beating

Summary thus far.

First degree spontaneous nystagmus Direction-fixed, oblique positional nystagmus (leftbeating ; up beating) that fails to abate with visual fixation Left-beating nystagmus in the caloric position

40 Left-Beating

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Caloric Testing
LEFT Cool
90 right-beating g g

LEFT Warm
120 left-beating

Caloric Testing
RIGHT Cool
40 Left Left-Beating Beating

40 Left-Beating

RIGHT Warm
40 Left-Beating

Caloric Summary
RIGHT Cool LEFT Cool

40 Left-Beating g

80 Right-Beating

RIGHT Warm

LEFT Warm

40 Left-Beating

11.50Left-Beating

Absent RIGHT ear labyrinthine reactivity, present left ear response

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Oculomotor Testing - Saccades

Normal Saccades

Oculomotor Testing Smooth Pursuit

Oculomotor Testing - Optokinetic

200 OKN Stimulus 400 OKN Stimulus

600 OKN Stimulus

300/sec (50%)

~17%
400/sec (67%)

Asymmetry with increasing stimulus

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SHA Testing 0.01 Hz

SHA Testing 0.04 Hz

SHA Testing 0.16 Hz

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SHA Testing Summary Data

Velocity Step Testing - 600

37% VOR decay time constant

Velocity Step Testing - 2400

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Velocity Step Testing 2400

Per LEFT Per RIGHT
379.03 LEFT 203.06 RIGHT

Post RIGHT

Post LEFT

Stronger Left VOR response (Right DP)

VOR Suppression Testing

0.08 Hz 0.32 Hz

0.16 Hz
Failure of VOR fixation suppression

0.64 Hz

VEMP Testing

108.03 V

96.70 V

NORMAL VEMP Bilaterally

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MRI Scan

Mild-to-moderate volume loss Extensive leukomalacia involving the periventricular white matter and some subcortical white matter (right>left) Probable lacunar infarct involving the tail of the left putamen as well as a possible cyst in the periventricular white matter of the right parietal lobe Excessive deposition of iron (or other metal) in the globus pallidus bilaterally as well as the dentate nuclei Petechial deposition of iron (or other metal) at multiple locations within the brain parenchyma and along the right central sulcus Creatine in the parietal gray matter is mildly elevated Deficit of NAA in the superior cerebellar vermis and the left thalmus Cysts or adhesions within the trigones of the lateral ventricles (right > left) Several inspissated mucus retension cysts in the maxillary sinuses Thornwaldt cyst in the nasopharynx SUMMARY - VERY ABNORMAL BRAIN MRI

Conclusions

Central Indicators

Non-Localizing Indicators

Peripheral Indicators

Saccadic tracking on smooth pursuit Failure of fixation suppression OKN asymmetry (secondary to SN or cerebellar dysfunction?) Positional nystagmus with fixation

Right-VOR asymmetry on SHA Significantly shortened timeconstants Grossly abnormal VOR phase lead across entire Hz range Direction-fixed, oblique positional nystagmus

Absence of labyrinthine reactivity to caloric stimulus Asymmetric labyrinthine reactivity on high-velocity step testing

Conclusions

Collectively, these results identify both central and peripheral sites of lesions Evidence supports a cerebellar SOL with a right ear peripheral SOL Results provide further evidence to support partial compensation for the peripheral insult (normal VOR gain) however this process may be incomplete (persistent VOR asymmetry) Central compensation is primarily modulated through the cerebellum where there is evidence of a concomitant lesion In light of this evidence, prognosis for full central compensation for the peripheral vestibulopathy is questionable and may be expected to be significantly altered or delayed This may help to explain patients continued (uncompensated) vestibular symptoms

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Wrap up.

Vestibular Assessment if a dynamic process of discovery We may not have all the pieces which is difficult when every patient is a different puzzle

In balance function assessment it should be the clinician's end goal to be one-step ahead during the assessment.the hypothesis generating process should continue as each bit of new information is acquired through [qualitative and/or] quantitative testing - Jacobson, et al. (2008)

Wrap up.

The truth of the matter is.that sometimes the pieces fit together nicely, but many times they do not or worse, they seem to fit together but some pieces are red herrings that lead you to the wrong interpretation The information may be correct, but our interpretation, experience, knowledge, or comprehensiveness of testing limits our understanding of how the pieces fit together

Final thoughts.
Ill leave you with this

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My vestibular testing tenets

(for whatever theyre worth)

If no two vestibular puzzles / results are the same, then it only stands to reason that
1. 2. 3. 4.

vestibular assessment and interpretation is just as dynamic and nonconforming (and sometimes downright confusing) experience in vestibular interpretation is the sum of ones mistakes (and successes) if you never have ALL the pieces, youll only see a portion of the final picture despite countless hours of didactic teaching and tutorials on the pitfalls and (correct) procedures of vestibular assessment, interpretation largely hinges upon fundamentals in anatomy and physiology. if one piece of the puzzle changes (or is placed incorrectly), all the other pieces are just as likely to change which could shift your entire interpretation while good vestibular testing comes from patience, practice, and prudence; great vestibular interpretation comes from better vestibular testing (yepits a circular thought but one that thankfully builds upon itself)

5. 6.

Thanks

There is no such thing as failure. There are only results. - Anthony Robbins

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