Central Vestibular Disorders

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Presented by Professor Mohamed Shabana , Unit of Audiology, Cairo University

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Central Vestibular Disorders

  1. 1. Central Vestibular Disorders Mohamed Shabana MD Prof of Audiology
  2. 2. • The vestibular organs sense head motion: canals sense rotation; otoliths sense linear acceleration (including gravity). • The central vestibular system distributes this signal to oculomotor, head movement, and postural systems for gaze, head, and limb stabilization.. • The visual system complements the vestibular system. • Visuo-vestibular conflict causes acute discomfort. • Peripheral and brainstem vestibular dysfunction causes pathological sense of self-motion and visuo-vestibular conflict.
  3. 3. The vestibular labyrinth answers two questions basic to the human condition • Where am I going? • Which way is up?
  4. 4. The vestibular labyrinth answers the two questions basic to the human condition by sensing • Head angular acceleration (semicircular canals) – Head rotation. • Head linear acceleration (saccule and utricle) – Translational motion. – Gravity (and by extension head tilt).
  5. 5. The vestibular organ Horizontal canal Anterior vertical canal Posterior vertical canal Vestibular Nerve Facial Nerve Vestibulocochlear (VIII) Nerve Cochlea Cochlear NerveCochlear Nerve UtricleSaccule
  6. 6. The vestibular organ lies in the temporal bone Foramen Magnum
  7. 7. Each vestibular organ has a sensor for head acceleration, driven by hair cells similar to those in the cochlea • In the cochlea vibration induced by sound deforms the hair cells. • In the labyrinth acceleration deforms the hair cells. • In the semicircular canals the sensing organ is the ampulla
  8. 8. depolarization hyperpolarization Deformation of the stereocilia towards the kinocilium causes hyper- polarization
  9. 9. Dizziness • Dysequilibrium • Vertigo (a sense of motion of person or the visual surround) • Presyncope (near-faint, light-headedness) • Psychophysiologic dizziness (anxiety and panic) • Overlapping • Motion sickness
  10. 10. Acute vertigo • A result of vestibular imbalance • Asymmetry in tonic activity within the vestibular system • Vegetative symptoms (nausea, vomiting, and diaphoresis) • Get worse with head movement • Ataxia
  11. 11. Rotation reflects imbalance • Semicircular canal • Tendency to fall to the unaffected side (+ve) Upright tilting Imbalance • Otolith • Tendency to fall to the affected side.
  12. 12. Central vestibular disorders • Identifying these is critical • *Common 25% older patients presenting to ER with acute isolated vertigo have a cerebellar infarction • Life-threatening • The earlier the Dx the better the Px • Severe neurologic sequelae *Acta Neurol Scand 91:43–48, 1995
  13. 13. For: Otolaryngologist It is : • Challenging • have To differentiate central from peripheral • May have both peripheral and central components. • Urgency of the workup • Neurologic, medical, or psychiatric
  14. 14. When to suspect Central ?peripheral • Neurologic symptoms • New severe headache • Type of nystagmus • Risk factors • No improvement within 48 hours
  15. 15. Central nystagmus • Multi-directional • -ve Alexander's law • Unaffected by removal of fixation • Purely vertical or Purely torsional nystagmus • Constant and does not wane with time • Absence of a head thrust sign • Multiple Gaze-evoked nystagmus (<30 degrees ) • Occasionally present in only one direction of gaze similar to peripheral nystagmus.
  16. 16. Gaze-paretic nystagmus • Weakness of the extra-ocular muscles OR • their innervations Gaze-evoked nystagmus Gaze-evoked nystagmus is usually due to a defect in the central neural integrators controlling gaze- holding
  17. 17. Purely vertical or Purely torsional nystagmus????? -Yaw : Horizontal plan -Pitch: Sagittal plane -Roll: frontal plane
  18. 18. Yaw (horizontal Plane) • It is rare in Central vertigo • More in BPPV, spontaneous nystagmus (peripheral). • Also in past pointing. • Cane be caused by lesions in the area of entrance of the vestibular nerve in the medulla
  19. 19. Pitch (Saggital) • It is common • Related to area affection of bilateral paramedian medulla, pontomedullary, pontomesencephalic , or cerebellar flocculus. • Down beats or up beats syndromes
  20. 20. Roll (Vertical plane) • It is indicative of unilateral lesion • It is due to lesions from the vertical canals and otoliths to vestibular nuclei, MLF (contra) and integration centers for vertical and torsional eye movements. • Signs in the form of head tilt, vertical convergence, ocular torsion, SVV
  21. 21. SVV
  22. 22. Absence of a head thrust sign
  23. 23. There are 3 major vestibular reflexes • Vestibulo-ocular reflex – keep the eyes still in space when the head moves. • Vestibulo-colic reflex – keeps the head still in space – or on a level plane when you walk. • Vestibular-spinal reflex – adjusts posture for rapid changes in position.
  24. 24. estibular Nuclei Abducens Nucleus Oculomotor Nucleus The horizontal vestibulo-ocular reflex (VOR) Left Medial Rectus Right Lateral Rectus Lateral Medial Nucleus Prepositus Hypoglossi Oculomotor Nerve (III) Abducens Nerve (VI)
  25. 25. Central Vestibular Disorders • 􀂄 Vascular • 􀂄 Inflammatory • 􀂄 Neoplastic • 􀂄 Craniocervical junction disorders • 􀂄 Inherited ataxias • 􀂄 Metabolic • 􀂄 Others
  26. 26. Duration of the Symptoms • Short rotatory or postural vertigo attacks lasting for seconds to minutes, TIA and basilar/vestibular migraine. • Persistent rotatory or postural vertigo hours to days, in infarction, haemorrhage, or MS with corresponding deficit • Days and weeks usually corresponding to permanent damage to brain stem or cerebellum
  27. 27. Vascular • Ischemic stroke/TIA • 􀂄 Brainstem • 􀂄 Cerebellar • 􀂄 Labyrinthine • Hemorrhage • 􀂄 Brainstem • 􀂄 Cerebellar • Migraine • 􀂄 Vertigo • 􀂄 Dysequilibrium • 􀂄 Benign paroxysmal vertigo • 􀂄 Paroxysmal torticollis
  28. 28. Inflammatory • 􀂄 Cerebellitis • 􀂄 Multiple sclerosis • 􀂄 Susac syndrome • 􀂄 Behçet's syndrome • 􀂄 Systemic lupus erythematosus • 􀂄 Sarcoidosis • 􀂄 Infectious Intracranial complications CSOM
  29. 29. Neoplastic • 􀂄 Brainstem • 􀂄 Cerebellar • 􀂄 Fourth ventricle • 􀂄 Paraneoplastic • Paraneoplastic cerebellar degeneration • Opsoclonus/myoclonus
  30. 30. Craniocervical junction disorders • 􀂄 Chiari malformation • 􀂄 Basilar impression • 􀂄 Syringobulbia
  31. 31. Inherited ataxias • 􀂄 Autosomal recessive • 􀂄 Friedreich ataxia • 􀂄 Ataxia-telangiectasia • 􀂄 Vitamin E deficiency • 􀂄 Refsum disease • 􀂄 Autosomal dominant • 􀂄 Spinocerebellar ataxias • 􀂄 Episodic ataxias
  32. 32. Metabolic • 􀂄 Wernicke's encephalopathy • 􀂄 Diabetes Hypoglycemia • 􀂄 Vitamin B12 deficiency • 􀂄 Hypothyroidism • 􀂄 Hyperventilation
  33. 33. Others • 􀂄 Toxic • 􀂄 Medications • 􀂄 Alcohol • 􀂄 Degenerative • 􀂄 Parkinson's disease • 􀂄 Progressive supranuclear palsy • 􀂄 Multiple systems atrophy • 􀂄 Normal pressure hydrocephalus
  34. 34. Others • 􀂄 Epilepsy • 􀂄 Trauma • 􀂄 Brain contusion • 􀂄 Post-concussion syndrome • 􀂄 Physiologic • 􀂄Mal de debarquement syndrome • 􀂄Motion sickness • 􀂄 Psychophysiologic • 􀂄Chronic anxiety • 􀂄 Panic disorder • 􀂄 Phobic postural vertigo • 􀂄 Psychogenic gait disorder
  35. 35. Others • 􀂄 Global cerebral hypoperfusion • 􀂄 Vasovagal presyncope • 􀂄 Reduced cardiac output • 􀂄 Autonomic insufficiency • 􀂄 Hypovolemia • 􀂄 Multisensory disturbance • 􀂄 Peripheral neuropathy • 􀂄Cervical or thoracic myelopathy • 􀂄Visual loss • 􀂄 Superior oblique myokymia • 􀂄Voluntary nystagmus
  36. 36. Migraine Headaches • 20,000 Patients Diagnosed with Migraine Who had HA at least once per year – 17.6% Adult females – 5.7 % Adult males – 4% children • 18% had HA one or more per month • Highest prevalence 35-45 years • Lowest prevalence > 50 years • Of those in the 20,000 deserving Dx of Migraine only – 29% males and 41% females aware Prevalence Study
  37. 37. Migraine Events • Migraines are Neurological events • Most common symptoms is Headache • Events can range from no pain to severe pain with permanent ischemic damage • Most common non-pain form of a migraine is visual, but any aura symptom can occur in the absence of pain, including dizziness
  38. 38. Migraine Events HIS Classification • Migraine without aura • Migraine with aura • Migraine with prolonged aura – one Symptom lasts > 60 min but < 7 days • Basilar migraine • Migraine aura without headache • Childhood periodic syndromes • Migrainous infarction
  39. 39. Migraine Head Ache History Clues • Head pain localizes • May be associated with eyes • Throbbing • Light or sound sensitivity - motion sickness especially in childhood • Scintillating lights - with or without pain • Family members with migraine • Mild to severe - hormonal and food triggers • Headache with caffeine withdrawal
  40. 40. Migraine classification - IHS • Migraine without aura (“Common migraine”) – At least five attacks meeting the criteria below • Duration 4-72 hours • Headache has at least two of the following: – Unilateral location – Pulsating quality – Moderate to severe intensity (inhibits or prohibits daily activities) – Aggravation with physical activity that increases intra-cranial pressure, eg. Walking stairs, straining,, etc • During headache at least one of the following: – Nausea and / or vomiting – Photophobia and / or phonophobia
  41. 41. Migraine classification - IHS • Migraine with aura (“Classic migraine”) – Meets criteria for Migraine without aura with the following addition • Reversible neurological dysfunction • Gradual onset over minutes, lasting < 1 hour • Headache before, during or up to 1 hour after aura • Migraine aura without headache (“acephalgic migraine”, “migraine equivalent”) – Aura as described above - head pain never develops. Rarely can last for hours
  42. 42. Migraine classification - IHS • List of symptoms that constitute an aura – Bilateral visual distortions – Paresthesia – Muscle weakness / coordination loss – Fluctuant hearing, unilateral or bilateral – Tinnitus, unilateral or bilateral – Lightheadedness / imbalance to true vertigo - movement provoked or spontaneous
  43. 43. Basilar Migraine (Basilar Artery Migraine) • Meets criteria of Migraine with aura but has two or more of the following auras – Visual symptoms affecting all fields – Dysarthria – Vertigo – Tinnitus – Hearing loss – Diplopia – Ataxia – Bilateral sensory changes or weakness – Decreased consciousness Migraine classification - IHS
  44. 44. Vestibular Migraine Study (Cutrer & Bahol) 91 patients • Symptoms 70% true vertigo 30% dizziness, imbalance, rocking, motion sensitivity • Relationship to headache 5% consistently preceding or during headache 65% variable 30% completely independent
  45. 45. Duration of Vertigo Spells in Migraine (Cutrer and Baloh, 1992) • Seconds 7% • Minutes to 2 hours 31% • 2 - 6 hours 5% • 6 – 24 hours 8% • > 24 hours 49% (weeks of motion sickness punctuated by vertigo)
  46. 46. Migraine vs Meniere’s • Migraine – Spontaneous Vertigo – Unilateral tinnitus and fluctuant hearing – Permanent progressive hearing loss unlikely – Mild ENG findings including mild asymmetry – Duration of vertigo seconds to days • Meniere’s – Spontaneous Vertigo – Unilateral tinnitus and fluctuant hearing – Permanent progressive hearing loss likely – Mild to significant ENG findings - mild to significant asymmetry – Duration >20 min <24 hours
  47. 47. Migraine headache • Diagnosis of Exclusion • No tests for Migraines • Suspect the Dx from the History Migraines are
  48. 48. Thank you

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