Kinga Harmat

University of Pécs Department of Otorhinolaryngology, Head and Neck Surgery

Holistic expertise of neurootology

Budapest, 2017.11.11.

Vestibular disorders II.

Peripheral vestibular

disorders

VISUAL system

PROPRIOCEPTIVE system (our body’s ability to sense where we are in relationship to our surroundings) = kinaesthetic information from the receptors in the skin, muscles, tendon, and joints

VESTIBULAR system (PERIPHERAL and CENTRAL)

Peripheral vestibular disorders BPPV Vestibular neuritis Ménière’s disease (M.D.) Bilateral vestibulopathy Labyrinthitis Fracture of the temporal bone Vestibular schwannoma Superior semicircular canal dehiscence

(SSCD) Vestibular migrain Vestibular paroxysmia

Neurological diseases Ischaemia/haemorrhage TIA (tranzient ischaemic attacks) Cerebellar tumors Virus infections Multiplex sclerosis Antiepileptic, anxiolytic drugs

Internal medical diseases (50%) Orthostatic hypotension Hypertension, antihypertensive

drugs Metabolic disorders– pl.diabetes

mellitus, thyroid Arrhytmia cordis Heart diseases: 63% has

dizziness, 37%: the only symptom!!!

Atherosclerosis Anaemia Toxins, kidney and liver diseases

Psychogenic (panic, phobia)

Ophtalmic diseases

Trauma: Labyrinth concussion

Fracture of the temporal bone Perilymph-fistula

Infection: Vestibular neuritis Herpes zoster oticus Labyrinthitis

Vascular disorders: Neurovascular compression (VIII. cranial nerve compression by vascular loop)

Tumors: Vestibular schwannoma

Others: Ménière’s disease BPPV (benign paroxysmal positional vertigo) Superior semicircular canal dehiscence (SSCD) Bilateral vestibulopathy Large vestibular aqueduct syndrome

Symptoms:

- disequilibrium, imbalance

- positional vertigo and nystagmus

Diagnosis:

- Microscopic ear examination

- Audiometry: pure tone audiometry, tympanometry, acustic (stapedius) reflex, speech audiometry, ABR (cochlear/retrocochlear lesions)

- Spontaneous vestibular symptoms, positional nystagmus, vHIT

- Neurological examination – CT scan (exclude fracture)

Record the exact vestibular status! - judicial significance

Therapy: symptomatic

Trauma

Labyrinth concussion

Fracture of the temporal (pyramid) bone Cause of vertigo: - labyrinth concussion - labyrinth injury - vestibular nerve injury Symptoms: - Transverse (20%) – deafness (sensorineural HL), vertigo, facial paralysis - Longitudinal – conductive (mixed) hearing loss, hemotympanon Dg: physical signs, CT scan (audiometry, nystagmography, electroneuronography…) Ther: symptomatic / surgery (declining facial nerve function, conductive hearing loss)

Perilymph fistula Cause: Round window / Oval window rupture – due to increased pressure Symptoms: Episodic vertigo /positional nystagmus Intensifying, usually mixed hearing loss, tinnitus Head tilt Vertigo - increased pressure Tullio phenomen (vertigo - loud noise) Dg: CT scan, fistula test… Therapy: surgery (fat, muscle, fascia)

Herpes VZV - Primary infection - “chickenpox” Reactivation (ggl geniculi /ggl. spirale / vestibular ggl)

herpes zoster oticus Ramsay-Hunt syndrome: facial nerve, cochlear nerve, vestibular nerve (V, IX, X, XI, and

XII involvement ) Older age (60y) – immune function decrease Symptoms: - Strong pain - ear - Eruptions in the external ear canal (tounge)– few days later - Hearing loss (retrocochlear), vertigo, facial palsy Follow-up! – days Therapy: acyclovir, prednisolon – 3 days, pain control Secondary complications: - bacterial superinfection, - postherpetic neuralgia (PHN), - chronic neuropathic pain at the site of HZ

Labyrinthitis

Haematogenous / direct (trauma /cholest.) / descending / ascending

- Circumscript - cholesteatoma – fistula sign - Diffuse purulent labyrinthitis – suppurative otitis – cochlear and vestibular function

loss - Serous (toxins, viruses: CMV, mumps, herpes ) – arousal symptoms / function loss -

usually function remains - Meningogen (meningococcus) labyrinthitis – complete loss of function (Cochlear

implantation) History: otitis / trauma / meningitis – vestibular symptoms and hearing loss Arousal symptoms: hyperacusis, diplacusis disharmonica (distorsion)

Therapy: - otogen – surgery + antibiotics - haematogenous / descending: antibiotics or antiviral drugs - balance excercises, hearing rehabilitation

Sudden loss of peripheral vestibular function - one side Frequent Cause: Viral infection (HSV) of the vestibular nerve is believed to be the most

common cause / Acute localised ischeamia? Patient history: Sudden onset - begins in minutes or in a few hours, ( viral infection and mild

vertigo attack can be before ) Severe attack of vertigo with nausea and vomiting Lasts more than 24 hours (2-3 days), slow improvement, inbalance can remain

for months. Vertigo even without movement!!! - motion increases their complaints Status: Harmonic peripheral vestibular syndrome

Peripheral HR (horizonto-rotatory) nystagmus toward the healthy side Patient tends to fall toward the affected side HIT is positive on the affected side!!! Video HIT, Caloric test, VEMP

Therapy: Hydration if vomiting persists, antiemetic drugs (dimenhidrinate – Daedalon) Early mobilization!!! Vestibular training– helps the central compensation

(eliminates the symptoms). Corticosteroids, vitamin B, antiviral drugs ? Psychological support! Vestibular suppressants - no longer than 3 days - make recovery more difficult.

Affects hearing and balance! Endolymph hydrops

ATTACKS (20 minutes - 12 hours):

- Vertigo with nystagmus - Nausea / vomiting - Hearing loss on the affected side (sensorineural, fluctuating) – first the low

frequencies, than progressive - Tinnitus on the affected side (low tone) or ear fullness - No neurological signs (like double vision, headache…) - Can’t be explained better with other cause… At least 2 attacks and a documented hearing loss for the dg!!! Can be bilateral (after 30 years, 50% have bilateral disease (Stahle et al, 1991)) Drop attacks (Tumarkin otolith crisis) – collapse (no loss of consciousness) Variants: - Lermoyez sy: intensifying hearing loss, than vertigo (hearing can improve) - Sudden hearing loss, vertigo - years after it – SEH (secondary endolymphatic hydrops)

- Genetic factors

- Extrinsic factors (trauma, otosclerosis, chronic suppurative otitis)

- ADH (vasopressin)

- Allergy

- Viral infections (CMV)

- Autoimmune reaction

- Excytotoxicity, apoptosis

Pathophysiology

Dg: tipical attacks, tipical audiogram (fluctuating, low-tone SNHL), ECoG =electocochleography (SP/AP)

Therapy:

Attacks: antiemetic drugs, hydration

Prevention:

Betahistine ?, diuretics ?

Intratympanal gentamicin = chemical labyrinthectomy

Intratympanal steroid

Surgery ? (saccotomy, labyrinthectomy, neurectomy – n. vestibular)

Rehabilitation (hearing – cochlear implantation, tinnitus retraining therapy, vestibular training)

middle-stage late-stage

early-stage of M.D.

ABR

EcoG

„Certain” : hystopathological signs of EL hydrops

„Definite”: 2 or more tipical vertigo attaks, SN HL measured by audiometry, tinnitus, fullness in the affected ear

„Probable” : at least 1 attack, SN HL- audiometry, tinnitus, fullness in the affected ear

„Possible” : 1 tipical vertigo attack, no audiometry

16

Frequent - older age - trauma - osteoporosis - vestibular neuritis - Méniére’s disease, migrain Cause: Canalolithiasis / cupulolithiasis (displaced otoconia) History: Vertigo attacks last for max. 1 minute, provoked by a specified head

movement (after waking up, looking upwards), usually with nystagmus. Right side – more common Posterior canal – most common Bilateral -traumas 50% recurrence

Posterior canal BPPV - frequent Dg: Dix-Hallpike maneuver / Semont maneuver Therapy: Epley-maneuver / Semont (3x3/nap) (60sec)

Horizontal canal BPPV

Dg: Supine roll test - Geotropic: canalolithiasis - Apogeotropic: cupulolithiasis Therapy:

- Canalolithiasis: BBQ roll maneuver (90°) - Cupulolithiasis: head shaking

BPPV type 2 (Büki B.)

Dg: Dix-Hallpike maneuver Therapy: sitting up from Dix-Hallpike position

Anterior canal BPPV Light cupula – persisting symptoms

Postreposition otolith dizziness – 1-2 weeks. Therapy: mobilisation! Vitamin D - low vitamin D level (renal diseases! - calculus)

Bilateral BPPV – usually posttraumatic (treat one side, control, other side) Multiple canal BPPV (+patients who cannot undergo traditional manual treatment) - Multiple axis patient rotators (Epley Omniax Rotator, TRV chair) Positional nystagmus: BPPV, migrain, perilymph fistula, SSCD, central disorders! (nystagmus latency, duration) Follow-up!!!

The maneuvers moves the displaced otoconia and repositions them into areas where they do not cause problems.

Epley-maneuver Lempert (BBQ roll) maneuver

Bilateral loss of peripheral vestibular function = poor quality of life! Symptoms:

Impaired spatial orientation, postural instability Without movement – no symptom!!! Dizziness while walking! No vertigo! Blurred vision - Oscillopsy (can’t read and recognize peolpe during walking) Soft ground and darkness makes it worse Optokinetic sensitivity (supermarkets worsens) Heartbeat can cause oscillopsy History: vestibulotoxic drugs, chemotherapy, meningitis, encephalitis, 2 sided Méniére’s-disease…

Diagnosis: No nystagmus Head Impulse Test (HIT) is positive bilaterally !!! No caloric response

Therapy: Treatment of immun-mediated inner ear disease Vestibular rehabilitation training (VRT) – to improve gaze and postural stability

Walking sticks Future: sensory substitution devices - implantation? Prevention!!!

Benign tumor, slow growing 80% of cerebellpontin angle tumors 2 sided in M. Recklinghausen (II. type neurofibromatosis) Symptoms:

One sided tinnitus Intensifying hearing loss on the same side (/sudden) Dizziness, dysequilibriometry (rare – due to central compensation) Facial nerve involvement (late symptom) Facial pain, numbness Headache

Diagnosis: - ABR (BERA) - retrocochlear laesion (audiotory brainstem response ) - MRI Therapy:

Surgery (facial function, hearing) Gamma knife (stereotaxic irradiation) Wait and see (MRI – half year)

Dehiscence of the bony canal (third window on the labyrinth) (rare)

Symptoms: - Conductive hearing loss (air-bone gap) - Vertigo attacks provoked by pressure / loud noise, lasting for few

minutes (caughing, sneezing, Valsalva) - Positional vertigo - Autophony (eg.: hear the moving of their eyes) - Vertigo in tunnels

Diagnosis: Audiogram (air-bone gap) + VEMP (vestibular evoked myogenic

potencials), HR CT, Tuning fork, Hennebert sign = positive fistula test

Therapy: surgery (?)

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Causes: AICA - artery compresses the VIII. nerve Dg: MRI, patient history, hyperventillation, Carbamazepin Symptoms: Attacks:

- vertigo for seconds or minutes, provoked by head movement (any direction)

- hearing loss (hearing improvement) - tinnitus - at least 10 uniform attacks / day

Therapy: - Carbamazepin (Tegretol – antiepileptic drug) 400mg - surgery? – microvascular decompression (neurosurgeon) - vestibular neurectomy

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HIT can be positive

Spontaneus nystagmus can be presented

Central positional nystagmus

Hearing loss, tinnitus

20%: endolymphatic hydrops (combined with Ménière-disease)

Therapy = migrain therapy (prophylactic, painkiller)

Criteria:

- At least 5 vertigo attack, 5 min.- 72 hours.

- Migrain in the patient’s history (with or without aura)

(International Classification of Headache Disorders – ICHD)

- 1 or 2 migrain feature at more than 50% of vertigo attacks

- Headache with at least 2 feature:

One sided, pulsatile, severe, physical activity makes it worse

- Photophoby/phonophoby

- Visual aura

No other vestibular or ICHD disorder

Chronic subjective dizziness / Phobic postural vertigo History:

Chronic subjective imbalance or periodic complaints No complaints on the morning Physical activity makes it better Fear of supermarkets and crowd, agoraphoby

Physical examination:

No positive findings (MRI neg. - therapeutic)

30-50 year Obsessive-compulsive personality, minor depression, emotional instability Spontaneous / provoking factors and situations: bridge, stairs, supermarkets, restaurants – visual triggers Anxiety can accompany Stress or organic disease initially (vestibular, 20%)

Phsychotherapy! SSRI

What makes them feel dizzy???

Usually patients feel vertigo (moving sensation)

Accompanied by nausea, vomiting, sweating

In an acute attack patients have nystagmus (spontaneous or positional) and harmonic vestibular syndrome

HIT (head impulse test) positive on 1 side or both sides

Peripheral vestibular disorders

attacks or not? between the attacks do they have complaints? duration of the attacks lasts more than 24 hours = acut vestibular syndrome!!! accompanying symptoms provoking factors medical history: infection, head or neck injury, drug intake, meningitis / encephalitis (ototoxic drugs, e.g. :antibiotics, chemotherapy – bilateral vestibulopathy) former neuronitis /M. Ménière/migrain – BPPV more often occurs accompanying diseases

BPPV

Ménière’s-disease (M.M.)

(SSCD) (3.window-sy)

vestibular paroxysmia

vestibular migrain

panic-syndrome

cardiac disease (e.g. arrhytmia)

subclavian steal sy

TIA (tranziens ischaemic attack)

multiple sclerosis

Seconds BPPV vestibular paroxysmia SSCD (TIA)

Minutes-hours

Ménière’s disease (20min-12hours) vestibular migrain (minutes-72h) TIA

Days

vestibular migrain: max. 72 hour First attack, lasts more than 24 h: vestibular neuronitis ( /labyrinthitis ) stroke cerebellar tumors

discharge from the ear, pain– otitis (labyrinthitis)

1 sided hearing loss during attack / fullness in the ear / tinnitus – M.D. (Ménière’s disease)

hearing loss – AICA infarct, labyrinthitis

autophony – SSCD

drop attack – M.D., TIA

palpitation, pain in the chest, dyspnoe – panic attack

headache – central disorder / migrain

neurological signs (deadly D’s: diplopia, dysarthria, dysphonia, dysmetria, dysphagia, dysaesthesia)

Vertigo without any movement (pl. M.D., neuronitis, central) /no vertigo without movement (e.g.. bilateral vestibulopathy, BPPV, SSCD)

Walking, darkness and soft ground makes it worse – bilateral vestibulopathy Specified head movement– (BPPV)

Loud sound (e.g. SSCD, M.D.)

Pressure (caughing, Valsalva) (SSCD)

Large spaces (functional, bilateral vestibulopathy)

Crowd/elevators (functional)

Bedside examinations (if possible, during attacks)

1. Eardrum (usually negative!!!) 2. Spontanous nystagmus (visual denied) - periferal nystagmus 3. Head shaking test 4. Head Impulse Test - usually positive in periferal lesions 5. Skew-deviation – no vertical skew-deviation 6. Vestibulo-spinal reflexes - toward the affected side 7. Cranial nerves 8. Positional examinations 9. Hearing test – (whisper) (if they complain hearing loss) 10. Tuning fork (Weber, Rinné) 2.+ 4.+ 5.= HINTS +10. = HINTS plus 2.+ 6.= harmonic / dysharmonic vestibular sy

Examinations

VNG (Videonystagmography) – spontaneous nystagmus, caloric test, positional nystagmus…

ENG (Electronystagmography)

vHIT (Video head impulse test) - 6 semicircular canals individually

VEMP (Vestibular evoked myogen potencials – o/c) – utricule, saccule

ECoG (Electrocochleography) - (endolymphatic hydrops)

Subjective audiometry – pure tone audiometry, speech tests, tinnitometry

Objective audiometry – ABR, MLR, ASSR