Post on 10-Feb-2016
description
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 historywhich complaints relate to vestibular deficits ?- vestibular tests
low sensitivity - low specificity: new tests?- vestibular diseases
pathophysiological mechanisms: new insights?- therapy
causal versus symptomaticmedication: indication area ? mode of action ?ablationlabyrinthine substitution systems - vestibular implants
which complaints are related to vestibular deficits ?
VERTIGO ?
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)
acute but transient symptoms
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
image stabilisation
balance control
spatial orientation
interpretationlearning
adaptationcompensation
CNSlabyrinths
visiongravitoreceptors blood pressure sensors in large blood vessels
hearing
somatosensorye.g. foot sole pressure
autonomic processes fast blood pressure regulation heart beat frequency nausea / vomiting
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
image stabilisation
balance control
spatial orientation
interpretationlearning
adaptationcompensation
CNSlabyrinths
visiongravitoreceptors blood pressure sensors in large blood vessels
hearing
somatosensorye.g. foot sole pressure
autonomic processes fast blood pressure regulation heart beat frequency nausea / vomiting
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
base of support
Centre Of Mass
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 speedlearning motor activities
→ automatisation
vn
cer
hippocampus basal ganglia
spinal pattern generator
visual cortex
otolith function especially relevant for:
motor learning (retardation in congenital areflexia)maintaining complex posturesstanding or slow walking
on a soft surface (wind-surfing)in darknessin 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
image stabilisation
balance control
spatial orientation
interpretationlearning
adaptationcompensation
CNSlabyrinths
visiongravitoreceptors blood pressure sensors in large blood vessels
hearing
somatosensorye.g. foot sole pressure
autonomic processes fast blood pressure regulation heart beat frequency nausea / vomiting
cmesthal
cortex
pons cer
vn omn
cgl
VOR: 8 msec OKR and Smooth pursuit: >75 msec
head impulse test in unilateral lossstandard video (50 Hz)
loss of gaze stabilisation (towards bad-side)especially for fast head movements
the German experience
VOR 3D: nystagmus 3D
direction = fast phase
magnitude = slow phase
horizontal (left – right)vertical (up – down)torsional (in- and extorsion)
in-torsionex-torsion
nose
left right
up
down
simulation of oscillopsia reduced dynamic visual acuityin case of bilateral vestibular areflexia
Dynamic Visual Acuity (VA) measurement
treadmill: 2, 4 and 6 km/h
decrease of VA during walking
- 0.21 - 0.20 - 0.30normal values
which complaints are related to vestibular deficits ?
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)
acute but transient symptoms
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
clinical relevant knowledge of function and compensation of peripheral lesions
labyrinth canals: rotations statoliths: translations + tilt
sacculus
utriculus
HC
AC
PC
labyrinth
type of movementsrotations canals HC+PC+AC translations + tilt statoliths: utriculus + sacculus
speed of movementsfrequency dependence
frequency dependence semicircular canals ?
0.1 Hz 10 Hzsensitivity
frequency (Hz)
frequency dependence canals: gain
calorics chair head impulses
0.01 Hz 0.1 Hz 10 Hz
sensitivity
frequency (Hz)
ageing (>60) frequency dependence canalspresbyo-vertigo
general population
elderly > 65 yo
sacculus
utriculus
HC
AC
PC
labyrinth
type of movementsrotations canals HC+PC+AC translations + tilt statoliths: utriculus + sacculus
speed of movementsfrequency dependence
labyrinth• rotations: canal system• translations + tilt: statolith systems
statolithsstatoconia
supporting cells haircells nerve
utriculus + sacculusaccelerometers• function based on inertia of statoconia mass• multi-directional symmetrical sensitivity• frequency dependence
Fg
0
velocity
Fg
no discrimination between translation and tilt possible
constant velocity
acceleration deceleration
frequency dependence semicircular statolith systems ?
0.2 Hz 2 Hz 20 Hz
sensitivity
frequency (Hz)
statolith
0.2 Hz 2 Hz 20 Hz
sensitivity
frequency (Hz)
tilt or translation
statolith
0.2 Hz 2 Hz 20 Hz
sensitivity
frequency (Hz)
canals
correct tilt or translation
statolith
0.2 Hz 2 Hz 20 Hz
sensitivity
frequency (Hz)
canalsvision and/orpropriocepsis
correct tilt or translation
statolith
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 !
multi-sensory reference stored in the brain based on learning for fast detection of spatial orientation
detection: no movement
multi-sensory input: no movement
detection of gravity vector no problem
no pathology: motion sickness = natural limitation + neuro-vegetative sensitivity
comparison
stationary reference
fast detection
voluntary self motion
• voluntary control
• feed back• anticipation
detection of gravity vector no problem
self motion
stationary reference
fast detection requires learning
• passive movement• only feed back• impact upon motor control• anticipation ?
detection of gravity vector can be a problem
boat movement
boat = passive motion
inadequate stationary reference
• passive movement• only feed backgravity vector ? learned motor patterns do not apply anymore
boat + self motion
problem with selection of right reference for motor activity: motion sickness
before learning
built up of new reference and motor learning
anticipation
fast identification, more automatic but not easy for everybody
boat + self motion
after learning
wrong reference and adapted to passive motion
off shore again ……
mal de débarquement
no dynamic sensory input
illusion of motion
anti-motion sickness drugs: reduce sensitivity and alertnesslearning versus sedation
canals: orientation in space: constant rotation or stand still ?statoliths: orientation in space: constant translation or stand still ?
orientation relative to gravity: tilt or translation ?
motion sickness !
- almost all subjects are susceptible with correct stimulusunless a low neuro-vegetative sensitivity
- a (partly) working labyrinth is prerequisite for Motion Sickness:
sensitivity
age (years)
motion sickness
central compensation
joint processes set at workto 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
harmonie vestibulairedirected towards lesion side:
- slow phase- falling- finger pointing
sedation impairs central compensationmethyl-prednisolon (100 mg every 2-days ▼) + betahistine 2-3 dd. 48 mg
250 mg/ml endolymphe 50 mg/kg in cats
NOcerebellar shut down
A
B
C
D
E
new neurons and connections
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
central vestibular compensation (e.g. Lacour 1992 - 2010)
effect of high dosage betahistine on central compensation after neurectomy
Cats Tighilet B et al (1995) J Vest ibular Research 5: 53-66
placebo: recovery in 7 weeksbetaserc: recovery in 3 weeks
Patients: Meniere’s disease Redon C et al (2010) J Clin Pharmacol ogy (in press)
placebo: recovery in 3 monthsbetaserc: 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 gravityafter laying down !after turning from one side to another in bedwashing hair under the showerreaching 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
calcium crystals sink into canal and clod together
canalolithiasis hypothesis (Epley)
canalolithiasis: fast position change leads after a latency to vertigo and nystagmus that decreases in time and upon repetition (fatigue)
PC-AD
suppine
utriculus
sit
gravitygravity
clod
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 praynatural remission 100 hrs ?
action !
• Epley
Hallpike is a good begin !but Epley finishes the job
AC-AS
PC-AD
suppine
Epley manoevre PC-ADwait minimal 30 secs after cessation of nystagmus
HC canalolithiasis can occur as a complication after a liberation (e.g. Epley) manoevre
horizontal canalolithiasis AD
geotrope nystagmus(irrespective side of lithiasis)
suppine position
bed bed
suppine position
cupulolithiasis AD ordeep canalolithiasis
apo-geotrope nystagmus(irrespective side of lithiasis)
strong right beating nystagmus very weak right beating nystagmus weak left beating nystagmus
bed bed
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 nystagmusEpley or reversed Epley, Semont
HC: horizontal nystagmus (geo- and apo-geotropic)Roll to healthy side, Vanucchi-Asprella …..
AC: downbeat-rotatory nystagmusDeep 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 manoeuvremay 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
acute nystagmus normal HIT
3rd degree nystagmus abnormal HIT
Cnyrim et al. JNNP, 2008Newman-Tocker et al. Neurology, 2008 Kattah et al. Stroke, 2009
central lesion
peripheral lesion
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 hoursnausea, vomitinghearing loss, tinnitus and/or fullnesswith 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 daysnausea, 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 Vestibulopathycounseling3 dd 48- 120 mg betahistine > 2 months3 dd 25-50 mg cinnerazine25 mg promethazine / 20 mg primperan at an attacktranstympanic dexamethason (5 mg)transtympanic gentamycin (20 mg in buffer)selective neurectomy or labyrinthectomy
Vestibular Migraineprophylaxe: 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
sensor random sensor on
belt with vibrators
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 VestibularImplant
With 3 BranchesStandard 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