Peripheral Vestibular Pathology Flashcards
Regarding Presbystasis, discuss:
1. What is it?
2. Causes? 5 contributors
3. Implications?
4. Treatment
DEFINITION: Disequilibrium of aging
CAUSES:
1. Decreased sensory input (ears, eyes, peripheral sensory, proprioception)
2. Decreased adaptive/compensatory ability
3. Decreased cognitive function (spatial sense, perception, hallucination, CVA effects)
4. Medication side effects increase
5. General medical conditions: weakness, syncope, BP, cardiac
IMPLICATIONS:
1. Falls: 30% over 60yo, 50% over 80yo fall annually
2. 10-15% fractures
3. 50% of those hospitalized for hip fracture never return to pre-fracture function
TREATMENT:
1. Improve general medical conditions
2. Fall risk reduction (glasses, diabetic foot checks, walkers, OT, PT, modify meds, etc.)
3. Vestibular habituation exercises to strengthen adaptive mechanisms
List a differential of vestibular problems based on timing of vertigo
Seconds:
- BPPV
- Vestibular Paroxysmia
Minutes to hours:
- Meniere’s disease (ie. Idiopathic Endolymphatic hydrops)
- Secondary endolymphatic hydrops (e.g. Otic Syphillis, Cogan’s disease, Mumps, Hypothyroid, HSV, Mondini, Delayed endolymphatic hydrops, Recurrent vestibulopathy, Vogt-Koyanagi-Harada)
- Vascular (vertebrobasilar insufficiency, stroke)
- CPA neoplasm
- Otosyphillis
- Vestibular migraine
- Vestibular migraine of childhood & recurrent vertigo of childhood
Days:
- Vestibular neuritis
- Labyrinthitis
- Persistent Postural-Perceptual Dizziness (PPPD)
Fluctuating duration:
- Inner ear fistula
- Superior canal dehiscence syndrome (SCDS) or 3rd window
- Inner ear trauma (ie. Penetrating, Non-penetrating, Barotrauma)
- Familial Vestibulopathy/ Familial ataxias
- Bilateral vestibular deficit (e.g. ototoxicity, bilateral temporal bone trauma, autoimmune, meningitis, sepsis)
- TIA
- Endolymphatic sac tumor
What is the differential for bilateral vestibular hypofunction?
- Ototoxic medications (e.g. chemotherapy, aminoglycosides)
- Neurodegenerative disorders (e.g. ALS)
- Trauma (e.g. bilateral temporal bone fractures)
- Meniere’s disease
- Infection: meningitis
- Autoimmune: Cogan’s
“MOM ANNTS”
Menieres disease
Ototoxic medications
Meningitis
Autoimmune (E.g. Cogan’s)
NF2
Neurodegenerative diseases
Temporal bone trauma (bilateral)
Sepsis
What are 2 prerequisites for central compensation of peripheral vertigo?
- Intact visual system
- Intact proprioceptive system
What are 5 causes for the inability to adapt to vestibular dysfunction?
- Vestibular suppressants/medications
- Inactivity
- Advanced age
- Fluctuating vestibular deficit
- CNS or cerebellar pathology
“FAVIC”
Fluctuating vestibular deficit
Advanced age
Vestibular suppressant medication
Inactivity
Central pathology or tumors
What are the characteristics of horizontal positional nystagmus that suggest a central disturbance? 4
- Sustained, large-amplitude nystagmus that is present during visual fixation
- Nystagmus in more than one head position
- Nystagmus associated with vertical (and especially downbeat) component
- Positional nystagmus associated with other neurologic signs or symptoms
What is the pathophysiology of BPPV, for both canalithiasis and cupulolithiasis?
CANALITHIASIS: Loose otoconia in the posterior non-ampullated arm of the semicircular canal
CUPULOLITHIASIS: Otoconia is attached to the cupula, making ampulla of the cupula responsive to gravity:
How does nystagmus in Cupulolithiasis change or differ from that of canalithiasis? 4
- Nystagmus lasts longer duration (can persist for imnutes or even as long as the patient remains in the provocative position)
- Less (minimal or absent) latency to the onset of nystagmus
- Less fatiguable
- Direction of nystagmus opposite to the canalithiasis
What are 9 causes of BPPV?
- Idiopathic (48%)
- Head trauma (most common secondary cause, 7-17%)
- Vestibular neuritis (15%)
- Meniere’s Disease (5%)
- Migraines (< 5%)
- Inner ear surgery (< 1%)
- Otologic infections
- Vitamin D deficiency (currently investigated)
- Prolonged bed rest
Basically top two most common are idiopathic (almost half) and trauma ~20%, then basically anything ear disease related / inner ear disease could trigger BPPV
Other causes of positional vertigo other than BPPV (4)
Anything that changes specific gravity of endolymph vs cupula
- Alcohol - Positional alcohol nystagmus
- Heavy water intoxication
- Ethylene glycol poisoning
- Glycerol
HEGA
Heavy water
Ethylene glycol
Glycerol
Alcohol
Describe the features of BPPV nystagmus affecting the posterior SCC
- Latency (5-15s)
- Short duration (20-30 seconds, < 1 minute)
- Crescendo-decrendo nystagmus over 20-30 seconds (< 1 minute)
- Upwards and torsional [geotropic] jerk rotatory nystagmus (direction does not change with repeat stimulation)
- Reversibility on sitting up (otoconia fall back in the opposite direction)
- Fatiguability response (on repeat testing, due to dispersion of otoconia)
Any nystagmus OTHER than this should make you suspect other canal involved or central
When is vestibular function testing indicated in BPPV? List 4.
- Atypical nystagmus
- Failed or repeated failed response to Epley
- Suspected additional vestibular pathology
- Frequent recurrences of BPPV (25% with separate recurrences of BPPV are more likely to have associated vestibular pathology)
What are symptoms of posterior SCC BPPV?
- Vertigo with true positional trigger
- Lying down, bending forward, looking up, rolling in bed
- Wakes patient up (suggestive)
- Night time symptoms – 60x RR of having BPPV
- Vertigo short (seconds to minutes)
- Resolves completely – may have leftover symptoms
- Recurrent periods
- Average period length 2 weeks
Describe the features of BPPV nystagmus affecting the lateral/horizontal canal
- Horizontal nystagmus (in the plane of the horizontal SCC) triggered by supine roll test or Bow & Lean test
- Fatiguability
- Latency
- Short duration (20-30 seconds)
What are the symptoms of lateral SCC BPPV?
- Symptoms tend to be more severe, and can happen when turning to either side (unlike posterior canal)
- Symptoms tend to be more prolonged
- Often history is more chronic with longer episodes or chronic dizziness
- Most commonly occurs after Epley, in which case affected side is always same
- Often periods of posterior SCC BPPV, interspersed with some other non-specific episodes
What are the diagnostic criteria for posterior SCC BPPV?
A. Recurrent attacks of postitional vertigo or positional dizziness provoked by lying down or turning over in the supine position
B. Duration of attacks < 1 minute
C. Positional nystagmus elicited after a latency of one or few seconds by the Dix-Hallpike maneuver or side-lying maneuver (Semont diagnostic maneuver). The nystagmus is a combination of torsional nystagmus with the upper pole of the eyes beating toward the lower ear combined with vertical nystagmus beating upward (toward the forehead) typically lasting < 1 minute
D. Not attributable to another disorder
What are the diagnostic criteria for lateral SCC BPPV?
A. Recurrent attacks of positional vertigo or positional dizziness provoked by lying down or turning over in the supine position
B. Duration of attacks < 1 minute
C. Positional nystagmus elicited after a brief latency or no latency by the supine roll test, beating horizontally toward the undermost ear with the head turned to either side (geotropic direction changing nystamus) and lasting < 1 minute
D. Not attributable to another disorder
OR;
A. Recurrent attacks of positional vertigo or positional dizziness provoked by lying down or turning over in the supine position
B. Positional nystagmus elicited after a brief latency or no latency in the supine roll test, beating horizontally toward the uppermost ear with the head turned to either side (apogeotropic direction changing nystagmus), and lasting > 1 minute
C. Not attributable to another disorder
How do you diagnose posterior SCC BPPV?
How do you diagnose lateral SCC BPPV?
Posterior SCC BPPV
- Dix Hallpike Test
Horizontal SCC BPPV
- Supine Roll Test (Pagnini-Lempert)
- Bow and Lean Test
Describe the diagnostic tests for lateral canal BPPV
- Supine Roll Test
- Lay supine like DHP
- Flex neck 30 degrees (brings horizontal SCC perpendicular to ground)
- Turn head towards the left and right - Bow and Lean Test
- Observe direction nystagmus when bowing forward and leaning back
Discuss the treatment for BPPV (all forms)
POSITIONAL MANEUVERS:
- Cawthorne Habituation exercises: intensive, and provokes intense vestibular symptoms
- Posterior Canal BPPV:
- Epley
- Semont (actually a treatment)
- Brandt-Daroff (habituation exercise, not commonly used) - Horizontal Canal BPPV:
- Lempert (BBQ roll)
- Gufoni - Superior Canal BPPV:
- Deep head hang (Yacovino)
SURGICAL OPTIONS:
1. Posterior SCC occlusion
2. Singular neurectomy (supplies posterior SCC ampulla) - Inferior vestibular neurectomy through MCF approach
OTHER:
1. TRV chair (fancy chair that moves in 3D space and performs particle repositioning maneuvers)
Describe the Epley Maneuver
- Patient is placed upright position with the head turned 45 degrees toward the affected ear (ear that was positiion on the DHT)
- Rapidly lay back to the supine head-hang 20 degree position, which is maintained for 20-30 seconds
- Head turned 90 degrees toward the other (unaffected side) and held for 20 seconds
- Head then turned a further 90 degrees (usually necessitating the patient’s body to also move from the supine position to lateral decubitus) so that patient’s head is nearly in the facedown position. Hold for 20-30 seconds
- Patient is then brought into upright sitting position, completing the maneuver
There is no evidence for post-procedural postural restrictions
What are the success rates of Epley according to nystagmus direction when rolled to the contralateral side?
- If same direction: 80%
- Reverse direction: 10% (otolith went back into posterior SCC)
- No nystagmus: 50% (particles divided into some going to vestibule and some back to posterior SCC, cancelling each other)
Recurrence rate - 30% in 1 year, 50% in 5 years
ie. What do you expect the nystagmus to do when you move the head during epley; should move it in the same direction, otherwise Epley likely to not work
Describe the Semont Maneuver
Generally used for patients that have limited neck mobility
- Start with the patient sitting on a table or flat surface with the head turned away from the affected side
- Quickly put the patient into the side-lying position, toward the affected side, with the head turned up. Nystagmus will occur, keep for 20 seconds after all nystagmus has ceased (some recommend up to 1-2 minutes)
- Quickly move patient back up and through the sitting position so that they are on the opposite side-lying position with the head facing down (head did not change position). Keep patient here for 30 seconds (Some say 2-10 minuates)
- At a normal or slow rate, bring the patient back up to the sitting position
Describe the Brandt-Daroff Exercises
- Sit on bed with legs hanging over the edge.
- Look away from the side of pathology.
- Lie down with head on bed toward the pathologic side, keeping head at 45 degree angle
- Stay in the position for 30 seconds
- Sit upright x 30 seconds
- Repeat on the other side
- Do each cycle x 5 to complete the exercise
- Repeat 3 times a day for 14 days
Vancouver Page 246
After doing particle repositioning maneuvers, the patient is still not better. What are 3 possible reasons for this?
- Re-entry of particles into the posterior canal
- Conversion to horizontal canal BPPV
- Canalith jam (may need to reverse epley, or use a vibrator)
What is Canalith Jam?
- Otoconia form a bolus and completely occlude the canal
- Get persistent nystagmus unaffected by position
- Can have reduction in HIT in the affected canal that resolves upon head shaking
Treatment: Vibrator
Describe canalolithiasis and cupulolithiasis. How do you differentiate horizontal SCC canalolithiasis vs. cupulo/short arm lithiasis?
How can you tell which ear is affected?
Ewald’s second law: Ampullopedal flow is stimulatory in the horizontal SCC
LONG ARM LATERAL CANALITHIASIS
- Similar concept to endolymph. Otoconia and endolymph move in same direction.
- Head turn to affected side will cause otoconia in the long arm to move towards the ampulla –> Stimulatory ie. brain things the body is turning to the same affected side –> slow phase correction drifts contralaterally –> correction (nystagmus) beats ipsilateral –> GEOTROPIC NYSTAGMUS (towards the ground)
SHORT ARM LATERAL CANALITHIASIS
- Short arm canal is on the opposite side of the crista/cupula compared to the lateral arm
- Head turn to affected side will cause endolymph to move in opposite direction, but in this case it is moving AWAY from the ampulla
- Ampullofugal = Inhibitory
- Head turn to affected side –> inhibitory –> brain thinks its turning to the opposite side –> slow phase correction drifts ipsilaterally –> correction (nystagmus) beats contralaterally –> APOGEOTROPIC NYSTAGMUS (away from the ground)
CUPULOLITHIASIS
- Concept is that the otoconia is stuck to the cupula, and when endolymph flows it “pulls” the otoconia from the cupula, which causes a “pull away” effect –> ie. pulls away from cupula = ampullofugal = inhibitory
- Same outcome nystagmus as short arm lateral canalithiasis
IN SUMMARY:
1. Geotropic nystagmus = Long arm canalithiasis
2. Apogeotropic nystagmus = Short arm or Cupulolithiasis
3. Geotropic + ear with stronger response is the affected ear (because it is getting “stimulated” more when you turn that side)
4. Apogeotropic + ear with stronger response is the normal ear (because it is inhibited when you turn to that side, so it’s “stimulated” on the other side)
Draw a 4x4 table comparing:
Intensity of nystagmus (stronger on left side vs. stronger on right side)
and
Side of origin and mechanism of BPPV (Apogeotropic vs. Geotropic nystagmus)
What would the type of horizontal canal BPPV be for each of these scenarios?
Apogeotropic + Stronger on left
- Right Cupulolithiasis
Apogeotropic + Stronger on right
- Left Cupulolithiasis
Geotropic + Stronger on left
- Left Canalithiasis
Geotropic + Stronger on right
- Right Canalithiasis
Kevan Page 67 Otology
Darren BPPV lecture
What are two ways to identify the affected ear (left or right) with horizontal SCC BPPV?
A. Determine if Nystagmus is geotropic or apogeotropic
- Geotropic = stronger side is effected
- Apogeotropic = stronger side is normal side
B. If unclear, then do the Bow and Lean test
1. Bowing forward
- In long arm: Ampullopedal flow (since the canal is 30 degrees angled up, bowing forward makes the otoconia “fall” a bit) –> nystagmus towards the affected side
- In short arm/cupulo: Ampullofugal flow –> nystagmus away from affected side
- Leaning Back
- In long arm: Ampullofugal flow –> nystagmus away from affected side
- In short arm/cupulo: Ampullopedal flow –> nystagmus towards the affected side
https://www.vestibular.today/blog/the-bow-and-lean-test
Describe the BBQ Roll / Lampert Maneuver for lateral SCC BPPV
- Do the Supine Roll Test
- Then continue to roll the patient 360 degrees in 90 degree increments
Modified technique:
1. Does a 270 degree rotation as that should anatomically be sufficient to get otoconia out of SCC
Describe the Gufoni maneuver for lateral SCC BPPV
Different maneuvers for canalithiasis vs. short arm/cupulolithiasis
Mnemonic: GGG/AAA
LONG ARM CANALITHIASIS: GGG
1. Geotropic Nystagmus
2. Good ear down
3. Ground (look towards ground)
- The patient is taken from the sitting position to a side lying position with their GOOD (less symptomatic) ear towards the ground
- The patient’s head is quickly turned TOWARDS the ground 45-60 degrees and held in position for 1-2 minutes
- Head angle is maintained until fully upright and then may be straightened
SHORT ARM CANALITHIASIS: AAA
1. Apogeotropic Nystagmus
2. Affected ear down
3. Away from the ground (look away from ground)
- Patient is taken from the sitting position to a side lying position with their AFFECTED (symptomatic) ear down
- Patient’s head is then quickly turned AWAY from the ground 45-60 degrees and held in position for 1-2 minutes
- Head angle is maintained until fully upright and then may be straightened
- This maneuver brings the otoconia into the long arm - AND THEN THE GEOTROPIC MANEUVER MUST BE PERFORMED
Regarding Superior / Anterior Canal BPPV, discuss:
1. How common is it?
2. What is the expected nystagmus?
3. What is the treatment?
Epidemiology:
- Less common than posterior SCC and horizontal SCC
Features of Nystagmus:
1. Vertical (down-beating) and torsional (beats toward the affected side fast phase (activation of ipsilateral superior rectus and contralateral inferior oblique)
Treatment:
1. Epley for the contralateral posterior canal
2. Alternative maneuver: Lie back with head turn to the affected side with head hanging for 30-45s then quickly sit up
- In Yacovino deep head hang there is no moving head side to side
What is “light cupula”?
- BPPV with persistent geotropic direction-changing positional nystagmus without latency or fatiguability
What is Meniere’s disease, and what is the pathophysiology of Meniere’s disease?
MENIERE’S DISEASE
- Believed to be associated with endolymphatic hydrops (accumulation of endolymph within the inner ear)
- Patients with MD had evidence of endolymphatic hydrops on post-mortem pathologic analysis
- However, not all patients with endolymphatic hydrops on pathology had a history of MD
PATHOPHYSIOLOGY
- Controversial overall
- Accumulation of endolymph due to inadequate drainage of endolymph from the endolymphatic sac
- Accumulation within the inner ear causes preceding aural symptoms including low frequency hearing loss, tinnitus, aural fullness
- Rupture of membranous labyrinth then results in vertiginous episode due to leakage of potassium rich endolymph into perilymph
- Over time, this results in permanent injury to the inner ear structures and progressive SNHL
What is the Barany Diagnostic criteria for Meniere’s Disease? (Main one that is used now)
DEFINITE MD:
1. 2 or more episodes of vertigo lasting anywhere from 20 minutes to 12 hours
2. Fluctuating aural symptoms (aural fullness, tinnitus, subjective hearing loss) occuring before, during, or after an episode
3. Audiometrically confirmed low frequency SNHL during an episode
4. Not better accounted for by any other vestibular diagnosis
PROBABLE MD:
1. 2 or more episodes of vertigo lasting anywhere from 20 minutes to 24 hours
2. Fluctuating aural symptoms (aural fullness, tinnitus, subjective hearing loss)
3. Not better accounted for by any other vestibular diagnosis
What is the AAO-HNS Diagnostic Criteria for Meniere’s Disease?
This is now largely replaced by the Barany criteria
CERTAIN MD:
1. All the features of definite MD, plus histopathologic confirmation (ie. on autopsy)
DEFINITE MD:
1. Two or more episodes of vertigo lasting at least 20 minutes
2. Audiometrically confirmed HL on at least 1 occasion
3. Tinnitus or aural fullness in the suspected ear
4. Other causes excluded
PROBABLE MD:
1. One definite episode of vertigo
2. Hearing loss documented by audiogram at least once
3. Tinnitus or aural fullness in the suspected ear
4. Other causes excluded
POSSIBLE MD:
1. Episodic vertigo without hearing loss
2. SNHL, fluctuating or fixed, with disequilibrium but without definitive episodes (no audiogram)
3. Other causes excluded
What is the AAO 1994 staging of Meniere’s disease?
Stage 1 = ≤ 25dB
Stage 2 = 26-40dB
Stage 3 = 41-70dB
Stage 4 = > 70dB
Four tone average of the PTA at 0.5,1,2, and 3 kHz
What is the etiology or theories of Meniere’s disease?
Largely unknown.
Theories:
1. Endolymphatic Hydrops hypothesis
2. Migraine Hypothesis
3. Autoimmunity
4. Viral infection
5. Ischemia of endolymphatic sac or inner ear
6. Congenital anatomic variations
Describe the two main proposed etiologies for Meniere’s disease:
1. Endolymphatic Hydrops Hypothesis
2. Migraine Hypothesis
ENDOLYMPHATIC HYDROPS HYPOTHESIS
- Onset of hydrops is probably multifactorial, including viral infection, autoimmunity, and other disorders as causes
- Since the 1950s, the spells of Meniere’s have been ascribed to sudden silencing of the inner ear due to contamination of the perilymph with endolymph after rupture of the membrane
- Two theories of hydrops development:
1. Overproduction of endolymph from Marginal cells of stria vascularis/dark cells (macula)
2. Insufficient reabsorption of endolymph from endolymphatic sac
MIGRAINE HYPOTHESIS:
- Migraine explains many cases of the disorder, perhaps through mechanism of ischemia due to vasospasm
- Genetic factors or the presence of an ion channelopathy have been invoked to explain the association
What is the differential diagnosis of Meniere’s disease?
- Vestibular migraines (large overlap)
- Autoimmune inner ear disease
- Cerebellopontine angle lesions
- Stroke/TIA
- Otosyphillis
“VASCO”
Vestibular migraine
Autoimmune inner ear disease
Stroke/TIA
CPA lesions
Otosyphillis
