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

1. Central Vestibular
Disorders
Mohamed Shabana MD
Prof of Audiology

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. The vestibular labyrinth answers two
questions basic to the human
condition
• Where am I going?
• Which way is up?

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. The vestibular organ
Horizontal canal
Anterior vertical canal
Posterior vertical canal
Vestibular Nerve
Facial Nerve
Vestibulocochlear
(VIII) Nerve
Cochlea
Cochlear NerveCochlear Nerve
UtricleSaccule

6. The vestibular organ lies in the
temporal bone
Foramen
Magnum

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. depolarization
hyperpolarization
Deformation of the stereocilia
towards the kinocilium causes hyper-
polarization

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. 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. 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. 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. 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. When to suspect
Central ?peripheral
• Neurologic symptoms
• New severe headache
• Type of nystagmus
• Risk factors
• No improvement within 48 hours

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.

17. 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

18. Purely vertical or Purely torsional
nystagmus?????
-Yaw : Horizontal plan
-Pitch: Sagittal plane
-Roll: frontal plane

20. 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

21. Pitch (Saggital)
• It is common
• Related to area affection of bilateral
paramedian medulla, pontomedullary,
pontomesencephalic , or cerebellar flocculus.
• Down beats or up beats syndromes

23. 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

25. SVV

26. Absence of a head thrust sign

27. 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.

28. 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)

29. Central Vestibular Disorders
• 􀂄 Vascular
• 􀂄 Inflammatory
• 􀂄 Neoplastic
• 􀂄 Craniocervical junction disorders
• 􀂄 Inherited ataxias
• 􀂄 Metabolic
• 􀂄 Others

30. 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

31. Vascular
• Ischemic stroke/TIA
• 􀂄 Brainstem
• 􀂄 Cerebellar
• 􀂄 Labyrinthine
• Hemorrhage
• 􀂄 Brainstem
• 􀂄 Cerebellar
• Migraine
• 􀂄 Vertigo
• 􀂄 Dysequilibrium
• 􀂄 Benign paroxysmal vertigo
• 􀂄 Paroxysmal torticollis

32. Inflammatory
• 􀂄 Cerebellitis
• 􀂄 Multiple sclerosis
• 􀂄 Susac syndrome
• 􀂄 Behçet's syndrome
• 􀂄 Systemic lupus erythematosus
• 􀂄 Sarcoidosis
• 􀂄 Infectious
Intracranial complications CSOM

33. Neoplastic
• 􀂄 Brainstem
• 􀂄 Cerebellar
• 􀂄 Fourth ventricle
• 􀂄 Paraneoplastic
• Paraneoplastic cerebellar degeneration
• Opsoclonus/myoclonus

34. Craniocervical junction disorders
• 􀂄 Chiari malformation
• 􀂄 Basilar impression
• 􀂄 Syringobulbia

35. Inherited ataxias
• 􀂄 Autosomal recessive
• 􀂄 Friedreich ataxia
• 􀂄 Ataxia-telangiectasia
• 􀂄 Vitamin E deficiency
• 􀂄 Refsum disease
• 􀂄 Autosomal dominant
• 􀂄 Spinocerebellar ataxias
• 􀂄 Episodic ataxias

36. Metabolic
• 􀂄 Wernicke's encephalopathy
• 􀂄 Diabetes Hypoglycemia
• 􀂄 Vitamin B12 deficiency
• 􀂄 Hypothyroidism
• 􀂄 Hyperventilation

37. Others
• 􀂄 Toxic
• 􀂄 Medications
• 􀂄 Alcohol
• 􀂄 Degenerative
• 􀂄 Parkinson's disease
• 􀂄 Progressive supranuclear palsy
• 􀂄 Multiple systems atrophy
• 􀂄 Normal pressure hydrocephalus

38. 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

39. 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

41. 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

42. 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

43. 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

44. 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

45. 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

46. 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

47. 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

48. 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

49. 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

50. 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)

51. 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

52. Migraine headache
• Diagnosis of Exclusion
• No tests for Migraines
• Suspect the Dx from the History
Migraines are

54. Thank you