update on vestibular disorders disclosure...1 update on vestibular disorders konrad p. weber...

1

Update on Vestibular Disorders

Konrad P. WeberInterdisciplinary Center for Vertigo and Neurological Visual Disorders

University Hospital ZürichEAN Spring School 2018

Stare Splavy, 12 May 2018

Disclosure

The author acts as an unpaid consultant and has received funding for travel from GN Otometrics.

Cerebellum

Labyrinth

Vision

Proprioception

The 6th Sense

Bewegungsempfinden

ErnstMach1838– 19161875

Ferner &Staubesand 1982

2

Push-Pull Cooperation of Horizontal Semicircular Canals

Ewald‘ssecondlaw

ExcitationoftheipsirotationalcanaldrivestheVORbetterthaninhibitionofthecontrarotationalcanal.

SlowHeadImpulse

BothcanalscontributetotheVOR.

FastHeadImpulse

Predominantlyexcitationoftheipsirotationalcanal.

DrDolittle‘sPushmi-Pullyu

Vestibular Haircells

8. Cranial nerve

Left Vestibular Neuritis

Weberetal.Neurology 2008 EyeVelocity

VestibularNeuritis OvertSaccades

Vestibulo-OcularReflex

NormalSubject Vestibulo-OcularReflex

EyeVelocity

Left Vestibular Neuritis

Weberetal.Neurology 2008

3

EyeVelocity

VestibularNeuritis

CovertSaccades

EyeVelocity

VestibularNeuritis

OvertSaccades

What you see is not what you measure!

• Bedside Head Impulse TestAssessment of the catch-upsaccade.

• Video Head Impulse TestVestibulo-ocular reflex andcatch-up saccades.

Modified Vertical Head ImpulsesLARP: left anterior – right posteriorRALP: right anterior – left posterior

Video: free iphone app ‚aVOR‘MacDougall et al. PLoS ONE 2013.

Free iPhone App: aVOR Otolith Organs

I I I I I I I I I I

I I

Clinical Test Healthy Subjects

Superior Vestibular

Neuritis

Inferior Vestibular

Neuritis

Unilateral Vestibular

Loss

Horizontal HIT

Anterior HIT

oVEMP

cVEMP

Posterior HIT

= Normal Response

Horizontal canal ampulla

VestibularDivisionVIII

nerve

Inferior

Superior

CochlearDivision

Anteriorcanal

ampulla

cochlea

Posterior canal

ampulla

Saccular macula

“shank”

“hook”

Utricular macula

= Abnormal Response

Linear Acceleration

4

Why VEMPs?Hexapod Eccentric Rotation

Measuring the linear vestibulo-ocular reflexis notoriously difficult!

CrocodylidsBirdsMammals

Reptiles

Amphibians

(Carey&Amin,2006)

NocochleaUseotolithsfor‚hearing‘Sensitivetomid-frequencies

Inhumans,vestibuleandotolithhaircellsstillhavepropertiesallowingactivationbysound

Non-physiologicalstimuli?

Whyaretheotolithsselectivelyactivated?Evolution

Air-conducted sound Bone-conducted vibration

Curthoys 2010

Sound and Vibration are effective and easy stimuli for the otoliths

Cervical VEMP

SCM

Ocular VEMP

Inferior oblique

Mainly saccule

Mainly utricle

Colebatch and Rothwell, J Physiol, 1993; 2004

• Evoked by air-conducted sound• Recorded in the ipsilateral SCM

Standard cVEMP

Simultaneous surface and singlemotor unit recordings from the SCM

Inhibitory ReflexIpsilateral Projection

P13

N23

ríêáÅäÉ

oVEMPocular Vestibular Evoked Myogenic Potentials

Weber KP, Rosengren, SM, Clinical utility of oVEMPs. Curr Neurol Neurosci Rep, 2015.

Oculomotor nerveOculomotor

nucleus

Vestibularnucleus

Vestibular nerve

Medial longitudinalfasciculus (MLF)

5

Standard oVEMPContralateral projection

Iwasaki S et al. Neurology 2007

SummaryCervical VEMPs

Vestibulo-collic reflex

Ipsilateral projection

Elicited with sound

Otolith test

Mainly saccule

Ocular VEMPs

Vestibulo-ocular reflex

Contralateral projection

Elicited with vibration

Otolith test

Mainly utricle

Rosengren SM, Welgampola MS, Colebatch JG. Vestibular evoked myogenic potentials: past, present and future. Clinical Neurophysiology 2010, 121:636-651.

Complete Testing of the PeripheralVestibular System

I I I I I I I I I I

I

I


Superior Vestibular

Neuritis

Inferior Vestibular

Neuritis


Loss

Horizontal HIT

Anterior HIT

oVEMP

cVEMP

Posterior HIT

= Normal Response



nerve

Inferior

Superior

CochlearDivision

Anteriorcanal

ampulla

cochlea

Posterior canal

ampulla

Saccular macula

“shank”

“hook”

Utricular macula

= Abnormal Response

Courtesy I.Curthoys

Left PosteriorRight Posterior

Left LateralRight Lateral

Left AnteriorRight Anterior

Vestibular Neuritis

Vestibular Test Battery

1P

1N

95 R

1P

1N

95 L

N10

P15

N10

P15

cVEMP (Saccule)oVEMP (Utricle)

Subjektivevisualvertical:19° left

1:50 1:40 1:30 1:20 1:10 1:00 0:50 0:40 0:30 0:20 0:10

R 30°C

R 44°C

1:501:401:301:201:101:000:500:400:300:200:10

30

28

26

24

22

20

18

16

14

12

10

8

6

4

2

0

-2

-4

-6

-8

-10

-12

-14

-16

-18

-20

-22

-24

-26

-28

-30

L 44°C

L 30°C

Canalparesisfactor:94%left

Fundus Calorics


I I I I I I I I I I

I I


Superior Vestibular

Neuritis

Inferior Vestibular

Neuritis


Loss

Horizontal HIT

Anterior HIT

oVEMP

cVEMP

Posterior HIT

= Normal Response



nerve

Inferior

Superior

CochlearDivision

Anteriorcanal

ampulla

cochlea

Posterior canal

ampulla

Saccular macula

“shank”

“hook”

Utricular macula

= Abnormal Response

Courtesy of CurthoysSuperior Vestibular Neuritis

Aw STetal.Neurology 2001TaylorRLetal.Neurology 2016

6

Left PosteriorRight Posterior

Left LateralRight Lateral

Left AnteriorRight Anterior

Patient with Vertigo & Hearing Loss Vestibular Test BatteryAudiogram cVEMP

dB

HL

dB

HL

-10 -10

0 0

10 10

20 20

30 30

40 40

50 50

60 60

70 70

80 80

90 90

100 100

110 110

120 120

130 130

125

125

250

250

500

500

750

750

1k

1k

1.5k

1.5k

2k

2k

3k

3k

4k

4k

6k

6k

8k

8k

12k

12k

Hz

Hz

Right Left

1P1N

95dB Left

1P

1N

95dB Right


I I I I I I I I I I

I I


Superior Vestibular

Neuritis

Inferior Vestibular

Neuritis


Loss

Horizontal HIT

Anterior HIT

oVEMP

cVEMP

Posterior HIT

= Normal Response



nerve

Inferior

Superior

CochlearDivision

Anteriorcanal

ampulla

cochlea

Posterior canal

ampulla

Saccular macula

“shank”

“hook”

Utricular macula

= Abnormal Response

Courtesy of CurthoysVestibulo-Cochlear Lossaka ‚Inferior Vestibular Neuritis‘

‘Isolated’ Posterior Canal Dysfunction

0 200 400 600 800−100

0

100

200

gain=0.841, saccamp=0.282°/trial

−100

0

100

200


−100

0

100

200


−100

0

100

200


−100

0

100

200


−100

0

100

200


LA RA

RHLH

RPLP

1.0

0.5

time (msec)head

and

eye

vel

ocity

(°/s

ec)

0 200 400 600 800

time (msec)

0 200 400 600 800

time (msec)0 200 400 600 800

time (msec)

0 200 400 600 800

time (msec)0 200 400 600 800

time (msec)

head

and

eye

vel

ocity

(°/s

ec)

head

and

eye

vel

ocity

(°/s

ec)

head

and

eye

vel

ocity

(°/s

ec)

head

and

eye

vel

ocity

(°/s

ec)

head

and

eye

vel

ocity

(°/s

ec)

Vestibular Schwannoma

dB

HL

-10

0

10

20

30

40

50

60

70

80

90

100

110

120

130

125 250 500 750 1k 1.5k 2k 3k 4k 6k 8k 12kHz

air conducted

mask.

right

left

without with

bone conducted

without with

Pure-tone audiogram: CPT (ri/le)=5/23%

0 20 40 60 0 20 40 60

13P

23N

13P

23N

bone-conducted cVEMPs: AR=51%right sacculus left sacculus

time (msec) time (msec)peak

-to-p

eak

ampl

itude

(uV

)

peak

-to-p

eak

ampl

itude

(uV

)

bone-conducted oVEMPs: AR=47%right utriculus left utriculus

peak

-to-p

eak

ampl

itude

(uV

)

peak

-to-p

eak

ampl

itude

(uV

)

N10

P15

N10

P15

2:20 2:10 2:00 1:50 1:40 1:30 1:20 1:10 1:00 0:50 0:40 0:30 0:20 0:10

R 30°C

R 44°C

2:202:102:001:501:401:301:201:101:000:500:400:300:200:10

222120191817161514131211109876543210-1-2-3-4-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-22

L 44°C

L 30°C

caloric irrigation: response 44% weaker on left side

‚Isolated‘ Loss ofPosterior Canal Function

2904 patients with 3D vHIT52 patients with • 40 unilateral posterior (77%)• 12 bilateral posterior (23%)

• >80% associated deficits in unilateral posterior loss

• <20% associated deficits in bilateral posterior loss– Calorics– oVEMP– cVEMP– Audiogram

Tarnutzer AAetal.2017

Hxofvestibularneuritis25%

Menière’sdisease23%

Unclearunilateralhypofunction

12%

Unclearbilateralhypofunction

14%

Schwannoma13%

Variouscauses*13%

Distributionofdiagnoses(n=52)

7

0

1

2

3

4

# of

add

ition

al a

udio

-ves

tibul

ar s

enso

rs a

ffece

d

all pa

tients

histor

y of

VN Menièr

e‘sdis

ease

schw

anno

ma

uncle

arhy

pofun

ction

vario

us

*p≤0.006

*p=0.007*p=0.024

20%

25%

25%

50%

40%

40%27%

23%

23%

27%

15%

15%

30%

40%

25%

25%

32%

18%

22%

19%

31%

28%

ipsilesional hearing lossipsilesional caloric hypofunction

ipsilesional cVEMP hypofunction

ipsilesional oVEMP hypofunction

Associated Deficits in ‘Isolated’ Loss of Posterior Canal Function Correlation vHIT – calorics / VEMPs

Expected patterns (based on innervation & vascularization): Posterior SCC + • sacculus (cVEMP): 40%• cochlea (PTA): 60%• sacculus + cochlea: 35%• lateral SCC (calorics): 65%• utriculus (oVEMPs): 50%• lateral SCC + utriculus: 38%• Involvement of any of the three

(calorics, oVEMP, cVEMP): 83%

Modified afterBradshawetal.2009JARO

0.5

0.7

0.8

1.0

RH LH

RP LP

RA LA

left horizontal canalright horizontal canal

gain=0.53, sacc amp=3.26°/trial

left anterior canal


right anterior canal


left posterior canal


right posterior canal


he

ad

an

d e

ye

ve

locity [

de

g/s

]

he

ad

an

d e

ye

ve

locity [

de

g/s

]

he

ad

an

d e

ye

ve

locity [

de

g/s

]

time [msec] time [msec]

he

ad

an

d e

ye

ve

locity [

de

g/s

]

he

ad

an

d e

ye

ve

locity [

de

g/s

]

he

ad

an

d e

ye

ve

locity [

de

g/s

]

time [msec]

time [msec]

time [msec]

time [msec]

50 100 150 200-100

0

100

200

0


50 100 150 200-100

0

100

200

0

50 100 150 200-100

0

100

200

050 100 150 200-100

0

100

200

0

50 100 150 200-100

0

100

200

050 100 150 200-100

0

100

200

0

gain

Anterior Canal Sparingafter Gentamicin Vestibulotoxicity 109 Patients (out of 2123)

with Bilateral Vestibular Loss

Tarnutzer AAetal.Clin Neurophysiol.2016

VOR Gain Saccade Amplitude

0.5

0.7

0.8

1.0

RH LH

RP LP

RA LA

0.5 1.0

2.0

3.0

4.0°/trial

RH LH

RP LP

RA LA

Disease-Specific Anterior Canal Sparing

Relativesparing ofthe anterior SCCs

8

Hierarchical Cluster Analysis101 patients with bilateral vestibular loss

Tarnutzer AAetal.2018

Anterior Canal Sparing

• Anterior canalsless prone to damageor better recovery?

• Accumulation of gentamicinat more caudally locatedcanals (posterior and lateral)?

• Common in

– Gentamicin vestibulotoxicity

– Menière‘s disease

– Unknown etiology


I I I I I I I I I I

I

I


Superior Vestibular

Neuritis

Inferior Vestibular

Neuritis


Loss

Horizontal HIT

Anterior HIT

oVEMP

cVEMP

Posterior HIT

= Normal Response



nerve

Inferior

Superior

CochlearDivision

Anteriorcanal

ampulla

cochlea

Posterior canal

ampulla

Saccular macula

“shank”

“hook”

Utricular macula

= Abnormal Response

Courtesy I.Curthoys

Thank you for your attention!

update on vestibular disorders disclosure...1 update on vestibular disorders konrad p. weber...

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