Vestibular disorders II. Peripheral vestibular disorders
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1 Vestibular disorders II. Peripheral vestibular disorders Kinga Harmat University of Pécs Department of Otorhinolaryngology, Head and Neck Surgery Holistic expertise of neurootology Budapest,
2 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)
3 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
4 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
5 Labyrinth concussion Trauma 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
6 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)
7 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
8 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
9 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
10 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.
11 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)
12 Pathophysiology - Genetic factors - Extrinsic factors (trauma, otosclerosis, chronic suppurative otitis) - ADH (vasopressin) - Allergy - Viral infections (CMV) - Autoimmune reaction - Excytotoxicity, apoptosis
13 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)
14 early-stage of M.D. middle-stage late-stage
15 ABR EcoG
16 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
17 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
18 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
19 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!!!
20 Epley-maneuver Lempert (BBQ roll) maneuver The maneuvers moves the displaced otoconia and repositions them into areas where they do not cause problems.
21 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!!!
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24 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)
25 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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27 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
28 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
29 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) 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
30 What makes them feel dizzy???
31 Peripheral vestibular disorders 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
32 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
33 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
34 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
35 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)
36 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)
37 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) = HINTS +10. = HINTS plus = harmonic / dysharmonic vestibular sy
38 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
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