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management of vestibular disorders
Herman Kingma, department of ORL, Maastricht University Medical Centre
Maastricht Research Institute Mental Health and Neuroscience
Faculty of Biomedical Technology, Technical University Eindhoven
problems in management of dizziness
- complex history
which complaints relate to vestibular deficits ?
- vestibular tests
low sensitivity - low specificity: new tests?
- vestibular diseases
pathophysiological mechanisms: new insights?
- therapy
causal versus symptomatic
medication: indication area ? mode of action ?
ablation
labyrinthine substitution systems - vestibular implants
which complaints are related to vestibular deficits ?
VERTIGO ?
acute but transient symptoms
acute unilateral loss or fluctuating function (neuritis, Ménière…)
- acute severe vertigo, severe nausea, falling and imbalance
(the classical leading symptoms for diagnosis)
acute bilateral loss
-acute severe intolerance to head movements, nausea and
imbalance (no vertigo: so the diagnosis is often missed)
remaining peripheral vestibular function loss
with:
-
reduced automatisation of balance
reduced dynamic visual acuity
reduced perception of self motion
hypersensitivity for optokinetic stimuli
enhanced neuro-vegetative sensitivity
secondary: fear and fatigue
somatosensory
e.g. foot sole pressure
gravitoreceptors
blood pressure sensors in
large blood vessels
labyrinths
autonomic processes
fast blood pressure regulation
heart beat frequency
nausea / vomiting
vision
CNS
interpretation
learning
adaptation
compensation
image stabilisation
hearing
spatial orientation
balance control
complaints related to vestibular dysfunction
acute loss or fluctuating function
transient: vertigo, nausea, falling / imbalance
remaining peripheral vestibular function loss
sustained:
- enhanced neuro-vegetative sensitivity
somatosensory
e.g. foot sole pressure
gravitoreceptors
blood pressure sensors in
large blood vessels
labyrinths
autonomic processes
fast blood pressure regulation
heart beat frequency
nausea / vomiting
vision
CNS
interpretation
learning
adaptation
compensation
image stabilisation
hearing
spatial orientation
balance control
complaints related to vestibular dysfunction
acute loss or fluctuating function
transient: vertigo, nausea, falling / imbalance
remaining peripheral vestibular function loss
sustained:
- enhanced neuro-vegetative sensitivity
- reduced ability to discriminate between
self-motion and environmental motion
vestibular impact
upon postural control
- regulation of muscle tone
relative to gravity
- regulation of COM
relative to base of support
balancing
correction steps
- labyrinths important for
balance at low speed
learning motor activities
→ automatisation
Centre Of Mass
base of support
visual
cortex
cer
hippocampus
basal ganglia
spinal pattern generator
vn
otolith function especially relevant for:
motor learning (retardation in congenital areflexia)
maintaining complex postures
standing or slow walking
on a soft surface (wind-surfing)
in darkness
in presence of misleading visual stimuli
labyrinths less relevant for:
walking at normal speed or running (visual anticipation)
bilateral areflexia leads to degeneration of
“head direction” and head “place” cells in the hippocampus
patient with severe bilateral vestibular hyporeflexia
slow tandem walk
fast tandem walk
complaints related to vestibular dysfunction
acute loss or fluctuating function
transient: vertigo, nausea, falling / imbalance
remaining peripheral vestibular function loss
sustained:
- enhanced neuro-vegetative sensitivity
- reduced ability to discriminate between
self-motion and environmental motion
- reduced automatisation of balance
somatosensory
e.g. foot sole pressure
gravitoreceptors
blood pressure sensors in
large blood vessels
labyrinths
autonomic processes
fast blood pressure regulation
heart beat frequency
nausea / vomiting
vision
CNS
interpretation
learning
adaptation
compensation
image stabilisation
hearing
spatial orientation
balance control
cortex
cgl
thal
mes
pons
cer
omn
vn
VOR: 8 msec
OKR and Smooth pursuit: >75 msec
head impulse test in unilateral loss
standard video (50 Hz)
the German experience
loss of gaze stabilisation (towards bad-side)
especially for fast head movements
VOR 3D: nystagmus 3D
direction
=
magnitude =
fast phase
slow phase
horizontal (left – right)
vertical (up – down)
torsional (in- and extorsion)
ex-torsion
in-torsion
up
down
nose
right
left
simulation of oscillopsia  reduced dynamic visual acuity
in case of bilateral vestibular areflexia
Dynamic Visual Acuity (VA) measurement
treadmill: 2, 4 and 6 km/h
decrease of VA during walking
normal values
- 0.21
- 0.20
- 0.30
which complaints are related to vestibular deficits ?
acute but transient symptoms
acute unilateral loss or fluctuating function (neuritis, Ménière…)
- acute severe vertigo, severe nausea, falling and imbalance
(the classical leading symptoms for diagnosis)
acute bilateral loss
-acute severe intolerance to head movements, nausea and
imbalance (no vertigo: so the diagnosis is often missed)
remaining peripheral vestibular function loss
with:
-
reduced automatisation of balance
reduced dynamic visual acuity
reduced perception of self motion
hypersensitivity for optokinetic stimuli
enhanced neuro-vegetative sensitivity
secondary: fear and fatigue
a vestibular function loss implies
permanent impairment
analogue to hearing and visual losses
examples
- Meniere’s disease when attacks are absent or disappeared
- neuritis vestibularis after central compensation
- bilateral vestibulopathy after central compensation
- vestibular loss schwannoma (also after extirpation)
many vestibular syndromes where vertigo is the leading symptom
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- pseudo vestibular neuritis: vestibular TIA or infarction
- motion sickness / mal de debarquement
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- fistula / superior canal dehiscence syndrome (SCDS)
-- central vestibular vertigo
labyrinth
canals:
rotations
statoliths: translations + tilt
clinical relevant knowledge of function
and compensation of peripheral lesions
AC
PC
HC
sacculus
utriculus
labyrinth
type of movements
rotations
translations + tilt
speed of movements
frequency dependence
canals HC+PC+AC
statoliths: utriculus + sacculus
frequency dependence
semicircular canals ?
frequency dependence canals: gain
0.1 Hz
sensitivity
10 Hz
frequency (Hz)
calorics
chair
head impulses
ageing (>60) frequency dependence canals
presbyo-vertigo
sensitivity
general population
elderly > 65 yo
0.01 Hz
0.1 Hz
10 Hz
frequency (Hz)
AC
PC
HC
sacculus
utriculus
labyrinth
type of movements
rotations
translations + tilt
speed of movements
frequency dependence
canals HC+PC+AC
statoliths: utriculus + sacculus
labyrinth
• rotations: canal system
• translations + tilt: statolith systems
statoliths
supporting cells
haircells
statoconia
nerve
utriculus + sacculus
accelerometers
• function based on inertia of statoconia mass
• multi-directional symmetrical sensitivity
• frequency dependence
velocity
constant velocity
acceleration
deceleration
0
Fg
Fg
no discrimination between translation and tilt possible
frequency dependence
semicircular statolith systems ?
sensitivity
statolith
0.2 Hz
2 Hz
20 Hz
frequency (Hz)
sensitivity
statolith
0.2 Hz
2 Hz
20 Hz
frequency (Hz)
tilt or translation
sensitivity
canals
statolith
0.2 Hz
2 Hz
20 Hz
frequency (Hz)
correct
tilt or translation
sensitivity
vision and/or
propriocepsis
canals
statolith
0.2 Hz
2 Hz
20 Hz
frequency (Hz)
correct
tilt or translation
some nasty facts and findings that need to be explained
- divers under water can’t orient themselves without vision !
submersion in water:
principle of inertia of mass in labyrinth remains
→ normal detection of accelerations should be possible
- no detection of orientation when covered by an avalanche
conclusion:
statolith input needs to be confirmed by
other senses, otherwise it will be neglected
which complaints are related to natural limitations ?
motion sickness !
no pathology: motion sickness
= natural limitation + neuro-vegetative sensitivity
multi-sensory reference
stored in the brain
based on learning
for fast detection of
spatial orientation
multi-sensory input:
no movement
comparison
detection:
no movement
detection of gravity
vector no problem
self motion
stationary
reference
voluntary self motion
• voluntary control
• feed back
• anticipation
fast detection
detection of
gravity vector no
problem
boat = passive motion
stationary
reference
boat movement
fast detection
requires learning
• passive movement
• only feed back
• impact upon motor control
• anticipation ?
detection of gravity vector
can be a problem
inadequate
stationary reference
boat + self motion
before learning
• passive movement
• only feed back
gravity vector ? learned motor
patterns do not apply anymore
problem with selection of right reference for motor activity: motion sickness
built up of new
reference and
motor learning
anticipation
boat + self motion
after learning
fast identification, more automatic but not easy for everybody
off shore again ……
wrong reference
and adapted to
passive motion
no dynamic
sensory input
illusion of motion
mal de débarquement
anti-motion sickness drugs: reduce sensitivity and alertness
learning versus sedation
canals:
statoliths:
orientation in space: constant rotation or stand still ?
orientation in space: constant translation or stand still ?
orientation relative to gravity: tilt or translation ?
motion sickness !
- almost all subjects are susceptible with correct stimulus
unless a low neuro-vegetative sensitivity
- a (partly) working labyrinth is prerequisite for Motion Sickness:
sensitivity
motion sickness
age (years)
central compensation
joint processes set at work
to achieve fast and optimal recovery
central compensation: neuroplasticity
- increase of visual sensitivity
- usage of commisural input
- formation of new neurons stimulated by movement
and high dosage betahistine (Lacour 2004)
- reprogramming connections to balance
spontaneous activity of both vestibular nuclei
A
B
NO
cerebellar shut down
harmonie vestibulaire
directed towards lesion side:
- slow phase
- falling
- finger pointing
C
D
new neurons and connections
E
sedation impairs central compensation
methyl-prednisolon (100 mg every 2-days ▼) + betahistine 2-3 dd. 48 mg
250 mg/ml endolymphe
50 mg/kg in cats
central vestibular compensation (e.g. Lacour 1992 - 2010)
maximum compensation is reached within 3-12 months
- stimulated by movement and fastened by betaserc
- impaired by immobilization and vestibular sedation
- STATIC components of compensation are good (in rest)
- neuroplasticity in VN is powerful
- DYNAMIC components of compensation are poor (during movement)
- automatization of complex gait and balance remains impaired
- image stabilisation remains poor
- hypersensitivity to optokinetic stimuli remains
- impaired spatial orientation remains
effect of high dosage betahistine
on central compensation after neurectomy
Cats Tighilet B et al (1995) J Vest ibular Research 5: 53-66
placebo:
betaserc:
recovery in 7 weeks
recovery in 3 weeks
Patients: Meniere’s disease Redon C et al (2010) J Clin Pharmacol ogy (in press)
placebo:
betaserc:
recovery in 3 months
recovery in 1 month
clinical guidelines
acute loss of peripheral vestibular function
(prophylactic in case of ablative therapies)
- movement, multi-sensory activation
- anti-emetics
- methyl-prednisolon: 100,100, 80, 80, 60, 60, 40, 40, 20, 20 mg
- betahistine 3 dd 24 - 192 mg
- avoid sedatives (e.g. cinnerazine, valium)
localisation of labyrinth dysfunction in detail is now possible,
but requires complex equipment
standard vestibular diagnostics:
- positioning tests for BPPV: Hallpike + supine roll test
- spontaneous versus fixation nystagmus
- smooth pursuit and optokinetic nystagmus
- saccades
- head impuls test
- caloric test
- ocular vestibular evoked myogenic potentials (o-VEMP)
normal test result does not exclude
vestibular function loss
most common vestibular syndromes
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- pseudo vestibular neuritis: vestibular TIA or infarction
- motion sickness / mal de debarquement
- phobic postural vertigo / visual vertigo / anxiety / psychogenic vertigo / conversion
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- bilateral vestibulopathy
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- central vestibular vertigo
- fistula / superior canal dehiscence syndrome (SCDS)
Benign Paroxysmal Positioning Vertigo in general:
- induced by a change of head position relative to gravity
after laying down !
after turning from one side to another in bed
washing hair under the shower
reaching for something placed high
- vertigo is induced after a latency (1 – 60 secs)
- nystagmus follows vertigo within a few seconds
- vertigo and nystagmus last about 5-20 secs
- vertigo and nystagmus are less upon repetition
- nystagmus reversible (when coming up again)
differences between HC, PC and AC canalolithiasis
canalolithiasis hypothesis (Epley)
suppine
clod
calcium crystals sink into canal and clod together
utriculus
sit
gravity
gravity
canalolithiasis:
fast position change leads after a latency to vertigo and nystagmus
that decreases in time and upon repetition (fatigue)
possible causes of canalolithiasis and cupulolithiasis
- disturbance in statolith metabolism
(vascular, ageing, Ca2+ metabolism/osteo-porosis?)
- head trauma (statolith detachment)
- bed rest (clod formation in canals)
- neuritis vestibularis, labyrinthitis
- ear surgery
- idiopathic
PC: rotatory-upbeat nystagmus
HC: horizontal nystagmus
AC: downbeat-rotatory nystagmus
VOG recording is the best way to analyse the nystagmus precisely
(fatigue prevents us to see the same type of eye movement
when we repeat the diagnostic positioning manoeuvre)
due to their orientation of the canals in the head:
mostly in the posterior canal
less in the horizontal canal
seldom in the anterior canal
Dix-Hallpike: diagnostic manoevre for PC-BPPV
Halpike: posterior canalolithiasis AD
right PC-canalolithiasis
PC-AD
left PC-canalolithiasis
PC-AS
Therapy
hope and pray
natural remission 100 hrs ?
action !
• Epley
suppine
AC-AS
PC-AD
Hallpike is a good begin !
but Epley finishes the job
Epley manoevre PC-AD
wait minimal 30 secs after cessation of nystagmus
HC canalolithiasis can occur as a complication
after a liberation (e.g. Epley) manoevre
suppine position
horizontal canalolithiasis AD
bed
geotrope nystagmus
(irrespective side of lithiasis)
bed
suppine position
strong right beating nystagmus
very weak right beating nystagmus
weak left beating nystagmus
cupulolithiasis AD or
deep canalolithiasis
bed
bed
apo-geotrope nystagmus
(irrespective side of lithiasis)
HC canalolithiasis and cupulolithiasis
- geotropic nystagmus points to canalolithiasis
- apogeotropic nystagmus points to deep canalolithiasis
the fast phase of the strongest nystagmus beats to the affected side
HC canalolithiasis can change from
geotropic into apo-geotropic vv.
do something doctor!
• Barbecue / Lempert
liberation manoevre for HC
geotropic canalolithiasis AD
270° roll to the healthy side
PC: rotatory-upbeat nystagmus
Epley or reversed Epley, Semont
HC: horizontal nystagmus (geo- and apo-geotropic)
Roll to healthy side, Vanucchi-Asprella …..
AC: downbeat-rotatory nystagmus
Deep Hallpike
if a liberation manoeuvre does not work…
•
the primary cause of the clod formation is not solved (frequent recidives)
•
clod did not reach utriculus 
repeat or use other manoeuvre
may be other canals are involved
•
exclude malign central positioning nystagmus (e.g. cerebellar lesions)
•
it might be a benign central positioning nystagmus
•
plugging: pro’s and contra’s (complications)
many vestibular syndromes where vertigo is the leading symptom
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- vestibular TIA or infarction
- motion sickness / mal de debarquement
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- central vestibular vertigo
- fistula / superior canal dehiscence syndrome (SCDS)
peripheral versus central
higher sensitivity than MRI including DWI
central lesion
peripheral lesion
acute nystagmus
normal HIT
3rd degree nystagmus
abnormal HIT
Cnyrim et al. JNNP, 2008
Newman-Tocker et al. Neurology, 2008
Kattah et al. Stroke, 2009
most common vestibular syndromes
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- pseudo vestibular neuritis: vestibular TIA or infarction
- motion sickness / mal de debarquement
- phobic postural vertigo / visual vertigo / anxiety / psychogenic vertigo / conversion
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- bilateral vestibulopathy
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- central vestibular vertigo
- fistula / superior canal dehiscence syndrome (SCDS)
phobic postural vertigo
visual vertigo / anxiety
psychogenic vertigo
subjective, fluctuating instability, fear to fall and vegetative symptoms, hours,
induced by crowds, visual stimulation, improves by alcohol and during physical activity,
increases during the day, avoidance behaviour, can be secundary to vestibular deficits
more frequent in women
treatment: paroxetine 20-40 mg dd, 3-6 months
+ cognitive behavioural treatment + physio-therapy
most common vestibular syndromes
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- pseudo vestibular neuritis: vestibular TIA or infarction
- motion sickness / mal de debarquement
- phobic postural vertigo / visual vertigo / anxiety / psychogenic vertigo / conversion
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- bilateral vestibulopathy
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- central vestibular vertigo
- fistula / superior canal dehiscence syndrome (SCDS)
Meniere’s Disease (ethiology ?)
spontaneous vertigo attacks lasting 20 minutes to many hours
nausea, vomiting
hearing loss, tinnitus and/or fullness
with or without dropatacks
Recurrent Vestibulopathy (ethiology ?)
similar as Meniere’s disease but no hearing loss or tinnitus
Vestibular Migraine / BPPV of childhood (ethiology ?)
spontaneous vertigo attacks lasting seconds to days
nausea, vomiting
not obligatory: history or present headaches, and/or aura’s
specific triggers: light, food, fatigue, sleep, hormonal cycli ….
history: migraine or BPPV of childhood
Meniere’s Disease and Recurrent Vestibulopathy
counseling
3 dd 48- 120 mg betahistine > 2 months
3 dd 25-50 mg cinnerazine
25 mg promethazine / 20 mg primperan at an attack
transtympanic dexamethason (5 mg)
transtympanic gentamycin (20 mg in buffer)
selective neurectomy or labyrinthectomy
Vestibular Migraine
prophylaxe:
propanolol 1-3 dd 40 mg or metoprolol 1-2 dd 50-100 mg
valproic acid 1-3 dd 500 mg
amitriptyline 1-3 dd 25-50 mg
topiramaat 1 dd 100 mg
BPPV of childhood
1 dd 12.5 mg metoprolol
most common vestibular syndromes
- Benign Paroxysmal Positioning Vertigo and Nystagmus
- vestibular neuritis or labyrinthitis / peripheral vestibular ischemia
- pseudo vestibular neuritis: vestibular TIA or infarction
- motion sickness / mal de debarquement
- phobic postural vertigo / visual vertigo / anxiety / psychogenic vertigo / conversion
- Meniere’s disease (MD)
- recurrent vestibulopathy (vestibular Meniere? no early stage of MD ?
- vestibular migraine (benign paroxysmal vertigo of childhood?)
- bilateral vestibulopathy
- vestibular paroxysms (neuro-vascular compression vestibular nerve,
analogon trigeminus neuralgia)
- vestibular epilepsia
- central vestibular vertigo
- fistula / superior canal dehiscence syndrome (SCDS)
patients with acquired
bilateral vestibular arereflexia
- gentamycine intoxication
- ageing
- auto-immune, vascular
- are they just fine after optimal compensation
and sensory substitution?
- how can we quantify residual function
- is there any need for a vestibular implant?
miniature 6-DOF detector in the belt
4x30x40 mm (gyroscopes + accelerometers)
vibration belt on the trunk
12 vibrators
battery + processor
with zero-posture reset
belt with vibrators
sensor random
sensor on
placebo effect ?
double blind placebo controlled study
vibrotactile vest and belt: supports body tilt and body rotation perception
• in 64 out of 83 patients the effect is significant more than placebo:
vibrotactile feed back improves stance and gait considerably in many patients
• 1 system is now used by a patient for about 1 year: he reports impressive increase in his quality of life
• the new adjustable system allows easy testing in patients
• 5 patients are now provided with an individually custumized belt for long term evaluation
vest
adjustable belt
individual belt
vestibular implants: state of the art knowledge (Guyot, Merfeld, Kingma, Stokroos)
Maastricht Vestibular
Implant
With 3 Branches
Standard Electrode Array
vestibular implants: state of the art knowledge (Guyot, Merfeld, Kingma, Stokroos)
• partial restorage of VOR with electro stimulation via gyroscopes in animals possible
• good and fast adaptation to chronic sustained stimulation in animals
• Rubinstein: influence Meniere attacks ?
• surgical routes in humans explored for stimulation
of PC, HC and AC
Geneve: nerve stimulation / Maastricht: ampullar stimulation
• per-operative and chronic stimulation studies in humans:
Geneve 2010: per operative and chronic nerve stimulation AC and PC possible: but cross talk
Maastricht since 2 february 2011:
in 3 patients peroperative 3D stimulation of all 3 canals possible: no cross talk anymore
• chronic implantation studies running now (Maastricht)
I hope this was
useful to you and help you
with the management of your patients
thank you
www.hermankingma.com