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