Definition of vertigo and BPPV

  • vertigo is defined as the sensation of spinning when the environment is stationary
  • this can happen due to inadequate signals from peripheral receptors or misinterpretation of these signals by the vestibular center, or a combination of both
  • it is necessary to distinguish vertigo from frequent symptoms of nonvestibular origin (orthostatic hypotension, chronic imbalance due to neuropathy, lacunar state, weakness in hypoglycemia, migraine aura, imbalance due to alcohol or drug abuse, feelings of insecurity when wearing inappropriate glasses, etc.)
  • vertigo, usually in combination with other symptoms, can be a sign of posterior circulation stroke; on the other hand, many vertiginous conditions (imbalance) have a nonvascular origin but cause difficulties in the acute phase as they resemble a stroke (stroke mimics)
  • benign paroxysmal positional vertigo (BPPV) is a common cause of vertigo in adulthood and older age
  • it is characterized by brief recurrent episodes of vertigo and is caused by displaced otoconia (calcium carbonate crystals) within the semicircular canals of the inner ear
  • BPPV is triggered by specific changes in head position relative to gravity (when the affected semicircular canal is involved)
    • most commonly when getting up or lying down, rolling over in bed, tilting the head back, or bending over
  • BPPV may occur after head trauma, viral labyrinthitis, or vascular lesions; no clear cause is identified in up to 50% of cases
  • a careful history and clinical examination are important to differentiate between positional and spontaneous vertigo
    • distinguish sustained vertigo from recurrent attacks
    • in elderly patients, it is important to identify concurrent vertiginous conditions (e.g. combination of BPPV and orthostatic dizziness or chronic imbalance due to neuropathy, etc.)

Pathophysiology

  • BPPV most commonly occurs  when otoconia (calcium carbonate crystals, also known as canaliths) become dislodged from the utricle of the inner ear
    • the utricle is a small, fluid-filled structure within the vestibular system responsible for detecting linear acceleration and head position relative to gravity
  • dislodged otoconia can migrate into the semicircular canals, particularly the posterior or lateral canals, where they can cause mechanical irritation of receptors with characteristic symptoms of BPPV
  • the exact cause of BPPV is not fully understood – hormonal influence, calcium metabolism, or inner ear trauma are considered contributing factors
  • there are several theories regarding why otoconia become dislodged:
    • due to age-related degeneration
    • due to head injuries or trauma, such as falls or accidents
    • due to inner ear disorders, such as Meniere’s disease
    • hormonal influences and calcium metabolism disorders are also discussed
    • in most cases, however, the exact cause of otoconia displacement remains unknown, and BPPV may occur spontaneously without a clear precipitating factor

Vestibular apparatus

  • the vestibular apparatus is a sensory organ that ensures the balance in space (in synergy with the visual and proprioceptive organs)
  • it is located in the inner ear within the petrous temporal bone, which contains a bony labyrinth and a membranous labyrinth
    • the bony labyrinth is filled with a fluid known as “perilymph”, which is comparable to cerebrospinal fluid and drains into the subarachnoid space
    • suspended within the bony labyrinth is the membranous labyrinth that contains a fluid known as endolymph, unique in composition due to its high potassium ion concentration
    • endolymph surrounds the sensory epithelium and interacts with hair cells
  • the vestibular apparatus comprises:
    • otolith organs utricle and saccule
      • sensory neuroepithelium in the utricle and saccule is called the macula
    • superior, posterior, and lateral semicircular ducts (canals)
      • sensory neuroepithelium in the semicircular ducts is called crista ampullaris
      • the semicircular ducts work in pairs to detect head movements (angular acceleration); a turn of the head excites the receptors in one ampulla and inhibits receptors in the ampulla on the other side
  • macula and crista ampullaris contain specialized mechanoreceptors – hair cells
    • neuroepithelium in the ducts respond to angular acceleration
    • sensory cells of the macula are irritated by otoliths, providing perception of body position and gravity
    • hair cells in the vestibular system are slightly different from those in the auditory system, in that vestibular hair cells have one tallest cilium, called the kinocilium. Bending the stereocilia toward the kinocilium depolarizes the cell and increases afferent activity. Bending the stereocilia away from the kinocilium hyperpolarizes the cell and results in a decrease in afferent activity.
  • the vestibular (Scarpa) ganglion contains thousands of bipolar neurons that receive sensory input from hair cells within the macula and crista ampullaris
  • axons from the vestibular ganglion join to become the vestibular nerve, which then joins the cochlear nerve to become cranial nerve VIII (vestibulocochlear nerve)
  • signals carried by the vestibulocochlear nerve are then interpreted by the central vestibular system within the brainstem (Deiters’, Schwalbe’s, Bechterev’s, Roller’s nuclei)
  • the central vestibular system unites the peripheral signals from both ascending pathways to elicit eye, head, and body motor responses for control of balance and orientation

Clinical Presentation

Posterior semicircular canal
  • BPPV involving the posterior semicircular canal (PC-BPPV) is one of the most common variants of BPPV
  • typically provoked by specific head movements, such as lying down, bending over, etc.
  • vertigo occurs with a latency of several seconds, accompanied by rotatory nystagmus (fast component toward the undermost ear) with a vertical component (upward)
  • unless the affected person changes position, the seizure subsides within tens of seconds
  • a short-term dizziness may be present after turning onto the healthy side
  • when the patient is examined in the upright position, the findings are completely normal, including vestibular function
  • positional tests such as the Dix-Hallpike maneuver are used to diagnose this condition; the Epley or  Semont maneuvers aim to move displaced otoconia out of the affected semicircular canal
  • unsteadiness may temporarily persist even after the successful maneuver
Horizontal (lateral) semicircular canal
  • the lateral (horizontal) canal is less commonly affected (about 10% of BPPVs)
  • LC-BPPV occurs when lying on the affected side or is provoked by turning the head sideways while lying on the back
  • turning the head from side to side (head is 30° inclined forward) provokes horizontal nystagmus, which has a geotropic or ageotropic direction, short latency (< 5 seconds), and a duration of 20-60 s (supine roll test)
    • geotropic nystagmus – the fast phase beats toward the ground when the patient is lying on the affected side and away from the ground when lying on the unaffected side
    • ageotropic nystagmus – the fast phase beats away from the ground on the affected side and toward the ground on the unaffected side
      • nystagmus that is “ageotropic” (~ 25%) is thought to be caused by debris that is further around the canal and closer to the ampulla than “geotropic” nystagmus
  • unlike posterior semicircular canal involvement, one-third of patients present with caloric hyporeflexia on the affected side

Which is the bad ear?

  • when LC-BPPV follows a treatment maneuver for PC-BPPV, the “bad” ear is considered to be the same one with the posterior canal BPPV
  • in idiopathic cases with geotropic nystagmus, the “bad” ear is assigned to the side with the stronger nystagmus
  • with apogeotropic nystagmus, the bad ear is assigned to the side with the weaker nystagmus (excitation is stronger than inhibition)
Anterior (superior) semicircular canal
  • the prevalence of AC-BPPV is very low and uncertain (~ 3%)
  • the anterior canal is the highest part of the ear, and getting debris there is not easy (except for during yoga or as a consequence of repositioning maneuvers in other canals)
  • triggered by head movements, such as looking up or extending the neck
  • AC-BPPV is diagnosed by positional down-beating nystagmus in the supine position (with torsional movement beating the downside) and up-beating nystagmus on sitting up (not always present)
  • AC-BPPV is more complex than PC-BPPV and includes far more central nervous system conditions (i.e. brain tumors, strokes, Chiari Malformation) as alternative possibilities
Central positional vertigo
Any differences from the above-described symptoms and signs should raise suspicion of a central positional nystagmus
  • positional nystagmus without vertigo, often with no latency, persisting for a long time
    • often purely vertical or beating to the ear above
    • rather mild intensity of vertigo
  • truncal ataxia with the inability to walk is common
  • positional nystagmus does not correspond to the plane of the stimulated semicircular canal
  • the causes of central positional nystagmus include
    • MS
    • cerebellar and brainstem infarcts, hemorrhages, and tumors

Diagnostic evaluation

  • the distinction between central and peripheral positional vertigo is essential
  • diagnosis is mainly clinical, based on a thorough history and examination of positional tests
    • the sensitivity of BPPV tests can be improved by having the patient wear Frenzel goggles
  • a basic neurological examination is completely normal
  • after performing the positional test, provide postural support until the patient is stable
Peripheral    Central
appearance vertical- or horizontal-rotatory purely vertical
latency a few seconds 0
duration resolves quickly (< 1min) resolves slowly (>1 min)
intensity of vertigo intensive mild
mechanism detritus in the semicircular canal central disorder
otolith-ocular pathway
localization semicircular canal brainstem, cerebellum
symptoms reoccur in the upright position yes no

Posterior semicircular canal

  • the patient is sitting length-wise on the examination table
  • the clinician turns the patient’s head 45° to the affected ear
  • while maintaining the head position, guide the patient in a continuous motion from sitting to lying with the head hanging off the table at approximately 20 degrees
  • hold this position for 30-60 seconds or until nystagmus has subsided
  • guide the patient back into a seated position and allow 30 seconds in the sitting position for the patient to recover
  • then repeat the maneuver with the head turned to the opposite side (a 45-degree angle toward the unaffected side)
  • guide the patient in a continuous motion from sitting to lying with the head hanging off the table at approximately 20 degrees
  • hold this position for 30 to 60 seconds or until nystagmus has subsided.
  • guide the patient back into the baseline sitting position
  • do not perform cervical spine hyperextension in patients with cervical spondylosis!
  • used both as a diagnostic and therapeutic maneuver
  • begin with the patient sitting upright on the examination table, facing the examiner
  • the patient’s head is quickly turned 45º to the unaffected side (the side without vertigo symptoms)
  • the patient is then laid down quickly toward the affected side (the side causing vertigo symptoms), with the head remaining at a 45º angle ((the patient’s nose should be pointing upward)
  • this will induce dizziness and nystagmus; hold this position for about 3 minutes or until any vertigo subsides
  • next, the patient is quickly brought up to the opposite side (the unaffected side) while maintaining the same 45º head position (the patient’s nose should be pointing downward toward the floor)
  • hold this position for another 3 minutes (short-term dizziness and nystagmus may occur)
  • finally, guide the patient back to the initial upright sitting position
  • after performing the maneuver, instability may be felt (most often with a pullback); these symptoms resolve within 30 minutes
  • within 24 hours after the maneuver, it is advised to sleep in a semi-sitting position
  • it is necessary to repeat the maneuver several times over the next few days if BPPV symptoms persist
Semont maneuver
  • the patient is sitting length-wise on the examination table
  • while sitting, turn the patient’s head 45º to the affected side
  • while maintaining the 45º head position, guide the patient in a continuous motion from sitting to lying with the head hanging off the table at approximately 20º (provide cervical support during this  procedure)
  • hold this position for 30-60 seconds
  • maintain the 20º head extension and rotate the patient’s head 90º toward the unaffected side so that the patient’s head is approximately 45º toward the unaffected side
  • hold this position for 30-60 seconds
  • while still maintaining the 45º head position, guide the patient into a side-lying position on the shoulder of the unaffected side (the patient’s nose points toward the floor)
  • hold this position for 30-60 seconds.
  • guide the patient back into a sitting position while ensuring that the patient’s head remains at the 45º angle
Epley maneuver

Lateral (horizontal) semicircular canal

  • the individual is in the supine position with their cervical spine flexed to about 30 degrees with their head in a neutral, centered position (Hwu, 2022)
  • then rotate the individual’s head 90 degrees toward the affected side and hold for 30 seconds after the nystagmus and vertigo symptoms stop (the affected ear is down)
  • the head is then returned  back to a neutral position (30s)
  • then turn the head 90 degrees away from the affected side (30s) (affected ear is up)
  • then rotate the whole body and end up lying on your stomach and then sit up
  • if nystagmus and vertigo symptoms persist, multiple BBQ rolls may be required
Lempert maneuver (the left ear is affected)
  • before starting the procedure, ascertain whether the patient has current or past injuries of the neck or spine!
  • the patient is sitting on the edge of the examination table, facing the examiner
  • with a rapid motion, guide the patient into a side-lying position toward the unaffected side
  • while the patient is lying on his side, turn the patient’s head to a 45º angle (the patient’s nose is pointing toward the table).
  • hold this position for 2-3 minutes.
  • the patient is guided back into a sitting position
  • the patient is sitting length-wise on the examination table
  • guide the patient into a supine position; a slight elevation of the head is helpful (30-degree inclined forward position)
  • have the patient turn their head 90 degrees to either side. If the patient does not have enough cervical flexibility to provide maximum otoconia displacement, have them roll onto their shoulder.
  • observe whether nystagmus is present and make note of the severity and the direction
  • guide the patient back into a neutral, supine position
  • turn the patient’s head 90 degrees to the opposite side (or roll onto the shoulder)
  • observe whether there is nystagmus, and assess the severity and direction
  • guide the patient back into the baseline position

Anterior (superior) semicircular canal

  • begin with the patient sitting on the examination table, facing the examiner, with the patient’s head turned away from the affected side at a 45º angle
  • guide quickly the patient into a side-lying position toward the affected ear (patient’s nose should be pointing upward)
  • hold this position for 2-3 minutes
  • while maintaining the 45º head position, guide the patient in a continuous motion from side-lying on the affected side to side-lying on the unaffected side (the patient’s nose points toward the floor)
  • hold this position for 3-5 minutes
  • guide the patient back into the initial sitting position, facing the examiner
  • one begins with head up, then flips to upside down, comes back up into a push-up position with the head turned laterally (45 deg towards the bad ear), and then back to sitting upright

Differential diagnosis

Therapy

  • repositioning maneuvers (as described earlier) can help to reduce the duration of BPPV attacks and sometimes provide immediate relief
    • maneuvers aim to relocate the displaced otoconia from the semicircular canals back into the utricle, where they no longer cause symptoms
    • positioning maneuvers should be repeated as long as the patient experiences the symptoms
    • after the maneuver, it is necessary to sleep with an elevated head  (~ 20-30°)
    • when maneuvers are repeated, their success rate is high
  • pre-treatment with intravenous antiemetics may be necessary in some cases
  • antivertiginous drugs typically have no effect in treating BPPV

Prognosis

  • ~ 10-20% of patients experience a worsening of symptoms within 1-2 weeks of treatment, ~ 50% of patients have a relapse
  • patients should be educated and instructed on how to perform repositioning maneuvers themselves to treat future episodes
  • in most cases, the symptoms of BPPV will spontaneously improve within a few weeks
  • for severe cases of BPPV that do not respond to repositioning maneuvers, surgical intervention may be recommended

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Benign paroxysmal positional vertigo (BPPV)
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