Early identifying vestibular
disorders in children who are deaf
or hard of hearing
WREIC 2017, June 16, 2017 –
Scottsdale Arizona
Christine Yoshinaga-Itano, Ph.D.
Research Professor
Institute of Cognitive Science
University of Colorado, Boulder
Disclosures
• Grant funding: Center for Disease Control,
OENIDRR, RERC
Introduction
•
Individuals with SNHL are at increased risk for
vestibular disorders (Arnvig, 1955; Berrettini et
al, 2005; Tribukait et al, 2004)
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Increased risk of acquired vestibular dysfunction
(Arnvig, 1955; Huygen et al, 1993)
Increased risk with greater degrees of hearing loss
(Antonelli et al, 1999)
Vestibular testing usually not completed
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Reasons given:
Not well tolerated
Lack of normative data
Lack of vestibular complaints
Prevalence of Vestibular Dysfunction
20-70% of children with hearing loss
Depends on etiology & degree of hearing loss, and
comprehensiveness of the testing
(Arnvig, 1955)
Higher in profound vs. severe SNHL
(Sandberg, 1965; Cushing, 2008)
Higher in postnatally acquired (meningitis)
(Arnvig, 1955; Sandberg, 1965)
Higher in some syndromic (Usher, Waardenburg)
(Arnvig, 1955; Huygen, 1993)
Normal vest. funct. in recessive inherited deafness
(Arnvig, 1955)
Wide range of severity
Mild loss to vestibular areflexia
Children with hearing loss and vestibular
disorders
• There are numerous disorders that can result in
congenital audiovestibular impairments.
• These include but are not limited to, Usher
Syndrome, Pendred, Waardenburg, AlbersSchonberg, Albinism, Ushers, Wolfram, and
Alport, BOR, CHARGE syndromes.
• The parents of children that present with these
syndromes can be questioned regarding motor
milestones, any reported dizziness, and whether
they have any concerns about their child’s balance.
Children with Hearing Loss
• Huygen et al (Int’l J of Ped Otorhino 1993)
• –121 children in school for the deaf
• –41% had Vestibular Disorder confirmed by
testing
• •Only canals tested
• •Worse in those with hearing level threshold
more than 90dB, and those with acquired loss
(e.g. rubella, meningitis).
• •Kernicterus (CNS damage 2 to jaundice)
• –associated with vestibular hyper-reactivity
Children with hearing loss
Rine et al, (Pediatric Physical Therapy 1998)
• –80% had abnormal PRNT (Post Rotary Nystagmus
Test), motor development delay and aberrant
responses on dynamic balance test
Tribukait et al. (Acta Otolaryngology. 2004)
• –Tested canals (calorics) and otoliths (SVV and
VEMP)
• •30% canal areflexia (absence of reflexes) bilaterally,
24% asymmetry
• •22% weak or absent VEMPS bilaterally + 19%
unilateral
• •32% positive Subjective Visual Vertical (SVV)
• –70% had at least 2 abnormal tests, only 30%
normal test results
Children with SNHL and cochlear
implantation
• Many with SNHL receive bilateral CI
• •Licameli et al (2009) CI in children
▫ More than 80% reduced or lost otolith function w CI
▫ 60% had reduced gain on VOR (Vestibulo Ocular
Reflex) test
• •Ito (1998) reported 38% of adult patients had
vestibular dysfunction following CI.
• •Limb et al (2005) reported 982 cases (442
children)
▫ 52 (5%) had severe dizziness post op (BPV or
unilateral loss of function)
Children with Cochlear Implants
• Jacot et al (2009)
• •Followed 224 children receiving implants
▫ 50% had normal function prior to surgery; 20%
complete bilateral areflexia, 22.5% unilateral
hypo, 7.5 % bilateral hypo
▫ Post CI: vestibular function changed in 51% of ears
with previous normal function (hyper or hypo)
Vestibular symptoms after
implantation
Fina et al (Otology and Neurology, 2003)
• •75 children participated
• –Recorded symptoms after implantation
▫ •39% experienced dizziness
▫ •25% experienced delayed episodic vertigo (74 days
post)
• •No one has examined motor development or
postural control development in these children
• ** thousands of children receive implants each year
• typically not referred for testing or rehabilitation
• Not possible to diagnose dizziness in a 1 year old or
younger child
Vestibular System
• Sense the position of
the head in space;
movements of the
head and neck
 Stabilizes vision
 Controls posture
 Determines orientation of
body in space.
http://www.bio.davidson.edu/people/midorcas/
animalphysiology/websites/2005/Cowell/index.htm
 Semicircular canals
(SCC): angular motion
 Otoliths: linear
acceleration (gravity)
Motor Development Prerequisites
Movement
Static &
Dynamic
Posture Control
Visual stabilization &
Orientation of body in
space
Somatosensory, visual &
vestibular systems
20 Questions: Dr. Devin McCaslin
• End organs for vestibular function
• There are five organs in the peripheral vestibular
system with each one serving a different
purpose.
• These five organs (three semicircular
canals and two otolith organs (utricle
(horizontal motion) and saccule (vertical
motion)) are connected to the two branches of
the vestibular nerve (i.e. inferior and superior).
• All five end organs are functional at birth.
Is the entire vestibular system fully
developed at birth?
• The labyrinth is morphologically complete at
birth and is the first sensory system to develop.
• The connections between the peripheral vestibular
system and central systems required for balance
continue to develop until the child is
approximately 12 years old (Peterson, Christou, &
Rosengren, 2006).
• It has been reported that vestibular responses peak at
this age (12 years) in order to facilitate the development
of emerging motor skills and postural control.
• Following this critical period, the cerebellum begins to
inhibit the vestibular system centrally.
Central pathways of the vestibular system
• Vestibular afferents project to the cerebellum,
autonomic nervous system, reticular formation,
spinal cord, cortex and visual system.
• One of the key coordinating structures for
balance are the vestibular nuclei (analogous
to the cochlear nuclei). This group of cells
receives information from the labyrinth,
somatosensory system, and visual system and
is a key element in the development of
locomotion and postural control.
Vestibular system and Motor Development
• The action of learning to walk is enormously
complex and has several stages through life.
• The vestibular system, along with vision and
proprioception, all factor into when a child
learns to roll over, crawl, and then walk.
• Vestibular mediated reflexes are present at birth
(e.g., head righting response). It stands to reason
that children with impairments that alter the
vestibular reflexes are slower than their normal
counterparts in reaching key milestones.
Vestibular Reflexes: Vestibulo-ocular
Reflex (VOR)
• One of the primary vestibular reflexes is the
vestibulo-ocular reflex (VOR). When a child
with a normal vestibular system moves
his or her head, the eyes are reflexively
deviated in the opposite direction so that
the image is stabilized on the retina
without blurring. This reflexive eye
movement is called the vestibulo-ocular reflex
(VOR).
Does the vestibular system affect vision?
• Absolutely.
• The VOR is critical for children beginning to
explore their environment.
• By 16 weeks of age, normally developing infants
can move their heads back and forth in the
horizontal plane.
• As infants learn to crawl and begin to reach for
things that interest them, they must be able to
see the objects clearly—even when the head is
moving.
Vestibular system impairment can
degrade a child’s visual acuity
• However, children often do not complain of
visual disturbance with head movement since
they do not have a reference for what is
abnormal.
• Adults with peripheral vestibular impairment
often describe a blurring of the visual field
during head movements and are known to
restrict the speed at which they turn their heads.
This decrease in visual acuity may be readily
observed in children with vestibular
impairments as well.
VOR
• Stimulation of the Ipsilateral Horizontal
Semicircular Canal (hSCC)
• Activates: Ipsilateral: Medial Rectus,
Contralateral: Lateral Rectus
• Inhibits: Ipsilateral: Lateral Rectus,
Contralateral: Medial Rectus
• Generates a horizontal eye movement with slow
phase away from stimulated ear
Vestibulo-spinal reflex (VSR)
• Critically important for the normal development of
posture and gait.
• The VSR is a pathway by which information about head
movement is relayed through the motor neurons in the
anterior horn of the spinal cord to the myotatic reflexes.
• These deep tendon reflexes modulate the tone in the skeletal
muscles of the trunk and extremities.
• The VSR relays information from the cerebellum,
reticular system, and vestibular system to adjust
posture and organize locomotion. In fact, if a
vestibular system is completely impaired on one side,
there is a corresponding reduction in muscle tone on
the same side.
• There are some very good studies showing how the normal
development of posture and locomotion can be disrupted in
the presence vestibular hypofunction (Inoue et al., 2013).
Vestibulo-colic reflex (VCR)
• This reflex activates the musculature of the
neck to stabilize the head.
• The upper limits for a normally developing child
are head control at approximately 4
months and sitting unsupported at 9
months.
• The VCR is a key compensatory response to
keeping the head centered over the body
at this stage of development. Children with
vestibular hypofunction have also been shown to
have delays in controlling their heads (Inoue et
al., 2013).
Signs of poor vestibular function
Low muscle tone
Delay in holding head up, floppy baby
Delayed gross motor milestones
Sitting, crawling, climbing steps, hopping
Speech delays
Below-age level balance abilities.
Clumsy, prblms standing feet together and eyes
closed
Asymmetrical posturing in sitting and
standing
Nystagmus
Visual instability on head movement
Complaints of spinning or dizziness
May be fearful or crave spinning
Adapted from Herdman (2007)
Looking for red flags
• Identify age appropriate motor milestones
• Know which disorders and syndromes are
closely associated with dizziness
• Understand the tools and questionnaires that
are available to screen children for vestibular
impairments
• Know where to send children for quantitative
balance function testing and treatment
Age appropriate motor milestones
• This includes observing activities such as playing, riding
a scooter, climbing, or even simply reading.
• One of the key indicators of a vestibular impairment is
whether a child achieves their traditional milestones at
appropriate times.
• For example, normally developing children will begin to
sit and crawl at approximately 5-6 months.
• That should be contrasted with the 8-18 months
required to reach the same landmark for a child with a
vestibular deficit (Kaga, 1999).
• It can take a child with a vestibular impairment 33
months to learn to walk independently versus only 12
months for an age-matched child with normal function.
• Emerging reports are converging on the fact that
motor milestones are one of the first signs of a
vestibular impairment in children (Rine &
Wiener-Vacher, 2013).
• Furthermore, fine motor skills in children with
vestibular loss have been shown to be delayed.
• These activities include passing an object from
one hand to the other or building a tower with
three cubes.
Benchmark for screening for motor delay
Developmental Milestones: Children
with Vestibular Disorders
Holds head steady
6.8 + 3.1 months
Stable sitting
14.0 + 3.3 months
Standing with support
19.8 + 4.9 months
Standing without support
23.9 + 6.1 months
Walking indoors
29.8 + 8.7 months
Walking outdoors
3.2 + 0.9 years
Running without falls
4.2 + 0.6 years
Disorders associated with vestibular
disorders
• Migraine is the most common cause of dizziness in the
pediatric population, but not the most common cause of
vestibular impairment. The age of onset can be as early as 2
years old.
• Otitis media. There is strong evidence to suggest that
children with chronic otitis media, as well as those that have
suffered a traumatic brain injury, have impaired balance and
more accidents.
• Vestibular neuritis or labyrinthitis, and although rarely,
even Meniere’s disease.
• Benign paroxysmal positional vertigo (BPPV). The
root cause of the BPPV is usually a head trauma or a recent
surgery for cochlear implantation.
• Presence of sensorineural (SNHL) hearing loss is
often associated with vestibular deficits (McCaslin, Jacobson,
& Gruenwald, 2011).
Soft Signs of vestibular dysfunction
•
“Soft” signs of vestibular dysfunction
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Motor delay
Poor posture
Poor balance (Brookhouser et al, 1982;
Admiraal & Huygen, 1997; Brunt & Broadhead,
1982; Butterfield, 1986; Enbron et al, 1991;
Kaga, 1999; Kaga et al, 1981; Rapin, 1974)
Motor Development
• Horn et al (2005)
▫ Deaf children pre- and post-CI
▫ Vineland Adaptive Behavior Scales (VABS)
 Motor, socialization, daily living subscales
▫ Results
 Motor skills less delayed than other subscales
 Acquired HL had lower VABS scores than congenital
 Higher VABS scores correlated with higher language measures
 Motor scores predict post-CI language
 Authors suggest a shared neuro-mechanism between motor and
language systems
Motor Development, cont’d
• Collins-Siegel et al, 1991
▫ Children (4-14 years) with hearing loss greater
than 65 dB
▫ Balance worse than normative data
 4-6 years: 50% worse
 8-14 years: 20% worse
 Balance improves until about 9 years, but does not
reach normal levels
Balance and Motor Deficits:
• de Kegel et al studied balance in children with
moderate to profound, unilateral or bilateral hearing
loss
• All performed equally on balance tests (de Kegel et
al, 2012).
• Though moderate and/or unilateral hearing losses
may not have as great a risk for communication and
language deficits as their profound hearing loss
peers, they have an equal risk for balance and motor
deficits (de Kegel et al, 2012).
Observation Questionnaire
adapted by Debbie Abbott, AuD
student University of Colorado,
Boulder
•
Peabody Development Motor Scales
assess both the vestibule-ocular and vestibulo-spinal systems
birth to 80 months
balance subset
assesses developmental reflexes
balance on the balance beam eyes open and closed
single-leg stance eyes open and closed
hopping
tandem stand and walk
visual tracking
perception
tracing
target activities
Bruininks-Oseretsky Test of Motor
Proficiency (BOT)
assess both the vestibule-ocular and vestibulo-spinal systems
4 to 21 years of age
balance subset
balance on a balance beam in tandem and single-leg stance
single-leg stance eyes open and closed
target and tracing tasks
Romberg test (Ask the patient to stand with their feet together
(touching each other). Then ask the patient to close their eyes. Remain
close at hand in case the patient begins to sway or fall.
finger to nose
bilateral coordination tasks.
balance subset of the BOT appears to be particularly well
suited to identify vestibular dysfunction in children with SNHL
(Crowe & Horak, 1988)
Children 5-12 years of age: Pediatric Dizziness
Handicap Inventory for Patient Caregivers (DHI;
Jacobson & Newman, 1990) (self-report)
McCaslin, Jacobson, Lambert, English, & Kemph, 2015
It should be used to quantify the impact of dizziness on a child’s
everyday life from the perspective of the caregiver.
• Responses are “yes”, “sometimes”, or “no”. The questions
cover the physical, functional, and emotional aspects of a
child’s life that can be affected by impaired balance.
• Interquartile scores have been calculated allowing the
provider to quantify a child’s activity limitation as “none”,
“mild”, “moderate”, or “severe”.
• The pDHI-PC can quickly and reliability provide clinicians
with information regarding a child’s disability/ handicap
imposed by their dizziness. Furthermore, it is useful in
assessing the efficacy of therapeutic or medical intervention.
Pediatric Dizziness Handicap Inventory for Patient
Caregivers (pDHI-PC) Jacobson & Newman, 1990
• Caregivers of children 5-12 years of age (McCaslin, Jacobson,
Lambert, English, & Kemph, 2015).
• It should be used to quantify the impact of dizziness on a
child’s everyday life from the perspective of the caregiver.
• Responses “yes”, “sometimes”, or “no”.
• The questions cover the physical, functional, and emotional
aspects of a child’s life that can be affected by impaired
balance.
• Interquartile scores have been calculated allowing the
provider to quantify a child’s activity limitation as “none”,
“mild”, “moderate”, or “severe”.
• The pDHI-PC can quickly and reliability provide clinicians
with information regarding a child’s disability/ handicap
imposed by their dizziness. Furthermore, it is useful in
assessing the efficacy of therapeutic or medical intervention.
Pediatric Vestibular Symptom
Questionnaire (PVSQ; Pavlou et al.,
2016)
• The motivation was to create a questionnaire that would
quantify the subjective vestibular symptoms that may be
experienced by a child.
• The PVSQ was developed to identify and quantify
subjective vestibular symptoms (dizziness and
imbalance) in children between 6-17 years of age.
• A secondary study aim was to investigate the
relationship between vestibular symptoms and behaviors
indicative of psychological problems in healthy children
and those with a vestibular disorder or concussions.
• The questionnaire was shown to be able to discriminate
between children in a cohort of children presenting with
vestibular symptoms and a normal control group.
Tests of Vestibular End Organ Function
Rine (2009)
Comprehensive testing should include tests of canal and otolith
function
Calorics
Test function of horizontal canal
Reliable in neonates (Donat, 1980)
Reliable in children over 5 (Kenyon, 1988)
Rotational chair
Test function of horizontal canal
Reliable in young children (Angeli, 2003)
Rotary chair testing is gold standard
Vest. evoked myogenic potential (cVEMP) (saccule) (oVEMP) utricle
Test saccular and utricle function
cVEMP Reliable in infants and early childhood (Sheykholesami,
2005)
cVEMP Introduction
• Short latency electromyograms (EMG) recorded
from the tonically contracted
sternocleidomastoid (SCM) muscle in response
to loud acoustic stimuli presented to the saccule
VEMP Pathway
•
Saccule
(Sheykholeslami &
Kaga, 2002)
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•
•
•
Linear accelerometer
Acoustic response
Inferior branch of
CN VIII
Vestibulospinal tract
Sternocleidomastoid
(SCM) muscle
http://www.fitness.ee/lehed/pilt/tekstid/3775.jpg
• The Saccule is the vestibular end organ most
sensitive to sound.
• •Lies under the stapes footplate.
• •Neurons from saccular macula respond to tilts
and click stimuli.
• •Electrical output from the saccule is routed
through the inferior vestibular nerve.
Protocol
• Evoked potential
equipment
• Electrodes
▫ Ground: forehead
▫ Active: sternoclavicular
juncture
▫ Reference: SCM
• Acoustic stimulus
http://www.audiologyonline.com/management/uploads/
articles/ackley_fig2.gif
VEMP Response
Normal Pediatric VEMP Response
•
Normal hearing children, 3-11 years
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Latency
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•
p13: 11.3 msec (8.3-14.4)
n23: 17.6 msec (14.8-21.9)
Amplitude: 122.2 mV (20.9-351.6)
Threshold: 90-95 dB nHL (Kelsch et al, 2006)
Hearing Impaired, 15-17 years
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Severe-profound
42% abnormal VEMP (Tribukait et al, 2004)
cVEMP – Saccule – test of balance
• Vestibular evoked myogenic potentials (VEMP) responses (i.e.
ocular and cervical) have been shown to be useful for testing
children. The tests can be completed relatively quickly, and
have been shown to have excellent clinical utility for assessing
the status of the otoliths (Kelsch, Schaefer, & Esquivel, 2006).
• First, the cervical VEMP (cVEMP) is a response that assesses
the sacculo-collic reflex
• This myogenic response is recorded from an activated
sternocleidomastoid muscle and represents a reflexive
adjustment of the musculature in the neck triggered by
activation of the saccule.
• The pediatric population shows decreased latencies and
increased amplitudes compared to their adult counterparts
(McCaslin, Jacobson, Hatton, Fowler, & DeLong, 2013)
Case 4- EB
•
•
•
•
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24 months
Profound hearing loss
MOTOR CONCERNS?
CI User
Results: Absent response bilaterally
oVEMP - Utricle
• Utricular Branch of the Superior Vestibular Nerve
• The utricle detects linear accelerations and headtilts in the horizontal plane.
• The ocular VEMP (oVEMP) is characterized by an initial
negative peak at ~11 msec that is followed immediately
by a positive peak occurring at approximately ~15 msec.
The negative polarity of the initial deflection suggests
that the oVEMP is an onset response and is most robust
from the contralateral eye. An ipsilateral response also
can be recorded, however, it is inconsistently present.
• Normative data from the pediatric population shows
equivalent latencies and amplitudes compared to their
adult counterparts
• Less commonly done than cVEMPs
ENG/VNG
• Ocular motility – saccades, spontaneous or gazeevoked nystagmus
• Small electrodes placed over the skin around the
eyes during testing
• VNG is the same battery of test but uses goggles
and video cameras to monitor the eyes
• Evaluates movement of the eyes as they follow
movement
VNG: Videonystagmography and
rotational testing- lateral semicircular
canals
• Large screen televisions can now be used for ocular
motor testing which allows for the use of cartoon
characters as targets. The question of how information
obtained from children differs from adults is currently
being studied.
• Dr. Steven Doettl’s laboratory at the University of
Tennessee, Knoxville is studying the errors and quality of
data obtained during the ocular motor testing in children
of different ages (Doettl, Plyler, McCaslin, & Schay,
2015).
• Currently, caloric testing, which evaluates the lateral
semicircular canal, has proven to be a challenge with
children younger than 5 years of developmental
age. There are currently very little data describing the
caloric response in young children.
ENG/VNG
• Test battery that includes measuring spontaneous and gaze nystagmus,
oculomotor testing, positional and positioning testing, and bithermal
caloric irrigation.
• Gaze-evoked nystagmus is recorded via calibrated light bar with gaze 20
degrees to the right, left, upward, and downward.
• Oculomotor testing involves observance of smooth pursuit, optokinetic
nystagmus (OKN), and saccadic eye movements. The light bar may adapt
cartoon characters for the pediatric patient.
• Positioning and positional testing are performed.
• In bithermal caloric irrigation (BCI), which allows independent
assessment of each peripheral vestibular system, the examiner
stimulates the vestibular system with warm or cool air or water. Warm
irrigations result in nystagmic activity beating toward the ipsilateral side
and cool irrigations result in beating toward the opposite side.
Contributions of each ear toward the total eye speed are calculated to
determine presence of unilateral peripheral weakness.
• VNG has proved to be a challenge with children younger than 6 or 7 years of
developmental age
Caloric Tests – dizziness, vertigo,
imbalance
• Not tolerated well by children under 5
• Gold standard
• Changes in temperature within the ear canal to
stimulate part of the vestibular system
• Results in nystagmus
• Identifies peripheral vestibular system
impairment
• Affecting lateral semicircular canals and/or
superior vestibular nerve
Computerized Rotary Chair (CRC)
Testing.
• Cyr (1980) successfully performed CRC on infants as young as 3
months.
• The simple harmonic acceleration subtest involves rotating
the head and body at various test frequencies and time
durations. Pediatric adaptations include seating the child on a
parent’s lap, developing a child-friendly enclosure, testing at only
select frequencies, and tasking with familiar nursery rhymes.
• Primary measures for analysis include:
▫ Gain in (conveying information related to vestibular system
output),
▫ Phase (comparing head/eye movement timing), and
▫ Symmetry (comparing right/left slow component eye velocity).
▫ Because of the nonlinearity of the anatomical system, patients
should be tested at several frequencies (Valente, 2007). Step
velocity testing has been performed successfully with children;
time constant measures are calculated after reaching constant
acceleration and rotational cessation.
COMPUTERIZED DYNAMIC
POSTUROGRAPHY (CDP)
• CDP tests postural stability or the ability to maintain
upright posture in different environmental conditions.
• Maintenance of postural stability depends on sensory information
from: the body’s muscles/joints, eyes, and inner ears.
• This testing investigates relationships among these three sensory
systems and records the balance and posture adjustments made
when different challenges are presented.
• This test may also be used in a rehabilitative setting after a diagnosis
has been determined, and is not performed on all people in the
diagnosis phase.
• CDP tests involve standing still on a platform. The platform may be
still or able to shift, or a visual target may be still or able to move
during testing. Pressure gauges under the platform record shifts in
body weight (body sway) as the person being tested maintains
balance under different conditions. A safety harness is worn as a
precaution, should the patient lose their balance.
Computerized Dynamic Posturography
(CDP).
• This test measures functional balance and relative
contributions of the visual, proprioceptive, and vestibular
systems.
• Examiners have found it to be effective with children as young as
3 years of age. The sensory organization test (SOT) places
the patient on a movable platform containing sensors for measuring
force of the feet in response to movement. The patient faces a visual
surround that may remain stable or be sway-referenced.
• The SOT involves six conditions, with the patient relying on
vestibular cues in the last two. Condition and composite scores are
calculated for:
• Platform and visual surround stable, eyes closed.
• Platform stable and visual surround sway-referenced, eyes open.
• Platform sway-referenced and visual surround stable, eyes open.
• Platform sway-referenced and visual surround stable, eyes closed.
• Platform and visual surround sway-referenced, eyes open.
Computerized Dynamic
Posturography (CDP).
• Motor control testing places the child on a platform that
undergoes unexpected perturbations.
• Forward and backward movements are of varying magnitude,
dependent upon patient height and weight.
• Latency—the time from perturbation initiation to foot force in
maintaining balance—is determined in milliseconds.
Abnormally latent measures may indicate dysfunction.
• Pediatric adaptations include a child-friendly visual surround
and diminishing numbers of trials, if attention span is limited,
but they have not been studied extensively.
• Unexpected platform movements progress toward “toes
upward” and “toes downward” positions. Each test involves
several trials, results of which allow the clinician to note
learning or practice effects as the session progresses. This
information may prove invaluable, as applied toward any
remediation strategies that may be recommended.
Positional/Positioning Testing
• Benign Paroxysmal Positional Vertigo (BPPV)
• Spontaneous and Central Positional Nystagmus
Benign Paroxysmal Positional Vertigo
• Screening for BPPV
▫ •Dix-Hallpike Assesses: Posterior SCC (pSCC)
Up-beating Nystagmus
▫ Anterior SCC (aSCC) Down-beating
Nystagmus
▫ •Roll Tests Assess: Horizontal SCC (hSCC)
Geotropic vs. Ageotropic Nystagmus
BPPV
• Displaced otoconia act as mobile densities
within the canal.
• •Head movement causes mass of otoconia to
shift within the SCC.
• •Endolymphatic fluid becomes displaced,
deflecting the cupula which elicits nystagmus
and vertigo.
3 subtypes BPPV and Nystagmus
• Bilateral Geotropic Nystagmus (Canalolithiasis)
Otoconial debris within posterior arm of hSCC
• •Bilateral Ageotropic Nystagmus Reverts to
Geotropic
• Otoconial debris within anterior arm of hSCC
• •Bilateral Ageotropic Nystagmus
(Cupulolithiasis) Persistent ageotropic
nystagmus
• Otoconial debris located on utricular side of
hSCC
vHIT: video Head Impulse Test
• Evaluates how the eyes and inner ear work together
– all 6 semicircular canals
• A relatively new tool in vestibular assessment has been
showing some promise for evaluating dizzy children is an
adaptation of the bedside Head Impulse Test (bHIT). The
high-tech implementation of the bHIT is the video Head
Impulse Test (vHIT).
• This system employs goggles, an accelerometer, and a high
speed camera, allowing for evaluation all six semicircular
canals.
• There are some challenges with performing the vHIT on
young children (i.e., 5 years old or less). Most notably, it has
been observed that there is more artifact in young children,
the goggles do not always fit snugly on a small head, and the
child must maintain gaze on the target at all times.
Head Thrust Test
• Head-thrust testing (HTT) may also be performed with
young children as part of a medical/physical workup.
• This procedure is based on the VOR, which as
previously mentioned should be observable by the
age of 9 to12 months in typically developing
children.
• The examiner gently rotates the child’s head by
approximately 30° in the yaw plane, while asking the
child to focus on the examiner’s nose.
• One may creatively devise placement of colorful stickers
or cartoon characters to facilitate such testing.
• The examiner incorporates a brief and rapid head thrust
back to center, making certain that visual fixation is
maintained. Any “catch-up” saccade may indicate a
disorder of the ipsilateral semicircular canal.
Balance Screening
• If a provider is interested in grossly screening the balance of a child
36 weeks old and older, there is normative data for standing on one
foot with eyes closed.
• The modified Clinical Test of Sensory Interaction and Balance
(mCTSIB; Shumway-Cook & Horak, 1986) can also be employed.
• This is a test where a child stands both on a hard surface and then
on foam with eyes open and closed. There is published normative
data for this evaluation.
• NeuroCom platform posturography system extensive examination
• The NeuroCom platform has several tests that can evaluate the
child’s reliance on vision, vestibular and somoatosensory
information. Furthermore, this system has additional tests such as
motor control and adaptation that can further evaluate a child's
postural response to unexpected movements.
Research from Sylvette WeinerWacher
Vestibular dysfunction in children
• Symptoms of Vestibular dysfunction are not
specific and not reliable in children
• •Aspecific: vomiting, nausea, instability,
(assimilated to tiredness in infant): «
pseudogastroenteritis »
• •Vertigo is not expressed at all in the youngest
before talking, and very unprecisely described
later
• •Dizziness is well tolerated,
• •Compensation is very rapid in case of partial
vestibular loss
• •Symptoms
Complete absence of vestibular
information (20% of profoundly deaf
children)
• No stabilization of the trunk and head: energy and
attention consumption (Anderson et al) and
difficulties in fine motor control, learning how to
write
• No gaze stabilization during movement: decrease of
dynamic visual acuity (Rine et al) difficulties to
learn to read and write, to lip reading or signs when
moving
• No reference to verticality: disorientation in space
(Brandt et al) and abnormal body representation
• Difficulties to overcome other visua or sensory
acquired impairment
Behavioral consequence of complete
bilateral vestibular loss
• The child cries when s/he is taken into the arms suddenly by
parents and when rocking in their arms. S/he does not like to
be moved and is irritable.
• In a group, s/he withdraws, stays in a corner, stays in his own
world or s/he is aggressive.
• S/he is often and very quickly tired, likes to be lying
down..lacks attention
• During movements, s/he blocks head and trunk and gets stiff
to avoid imbalance, bad motor strategies
• S/he limits him/herself for sensorimotor explorations and
interaction or s/he tumbles frequently, does not recognize
vital risks, gets into trouble, gets unruly, disruptive, aggressive
• Questions of behavioral associated disorder?
• Questions of autism?
Research: Cushing (2008)
40 children, aged 3 to 19 years of age, with severe to profound
SNHL & CIs.
VT calorics, rotary chair and VEMPs
Dynamic balance assessed by balance subset of the (BOT-2)
Results
Calorics – 50% of children had abnormal response
Rotary – 38% of children had abnormal response
VEMPs - 38% of children had abnormal function
BOT2 – children w/ SNHL on average demonstrated poorer
performance on tests of dynamic balance
Incidence of vestibular dysfunction is highly dependent on
etiology
Research: Cushing (2008)
Conclusions: Vestibular and balance dysfunction
occurred in 1/3 of children with SNHL and CIs,
and is highly dependent on etiology, with SNHL
from meningitis associated with worse balance
function than other etiologies.
Rotational chair testing, which assesses higher
frequency motion (0.25–5 Hz) and thus more
“real world” vestibular function, correlated best
with dynamic balance (BOT-2).
Cushing 2009
•
•
•
•
Effects of Cochlear Implantation
•Reduced vestibular function
–Vestibule fibrosis
–Saccular membrane disruption (Tien &
Linthicum 2001)
• •Up to/greater than 58% demonstrate reduced
vestibular function (Cushing et al 2009)
Prevalence of vestibular impairment in
deaf children
• High prevalence (Werner-Vacher) 60% of SNHL
associated with abnormal vestibular responses
• Not correlated to the level of hearing loss
31% (n=28), change in canal VOR
45% (n=40), change in otolith
Types of Vestibular impairments in
profound SNHL implant candidates
population n=224
Normal
7.50%
22.50%
Bilateral areflexia
50%
20%
Asymmetrical
impairment
Partial symmetrical
impairment
Post-Implant Vestibular Impairment
(N=120)
implantation on a functional vestibule
42%
48%
10%
No change
Areflexia
Change but no areflexia
After Cochlear Implantation (WeinerVacher)
• Responses to vestibular testing change after
cochlear implant: 52%
• Canal and otolithic areflexia: 10%
• Hypo-refexia: 18% (canal and/or otolithic)
• Hyper-reflexia: 18% (otolith +/- canal) 6%
(canal)
Post-surgical symptoms suggesting
vestibular impairments are not
reliable
• 27% present symptoms such as vomiting,
instability or vertigo
• Half have post-implant vestibular impairment
• Most complete ipsilateral areflexia post-implant
are symptomatic (vomiting, brief instability)
• But some complete areflexia can have no
symptoms at all!
Two steps bilateral cochlear implant in
young children
• For the Second implant, after 2 to 3 months
interval and vestibular evaluation
• In order to detect a vestibular loss induced by
the first implant that will postpone the second
implant
• If possible, wait for the acquisition of the first
independent steps
• One step bilateral: Cochlear Implant reserved to
bilateral complete vestibular loss assessed with
vestibular testing
Impact on cochlear implantation
protocol in young children
• Weiner-Vacher recommendation:
• No implantation before a minimal vestibular
evaluation: canal tests (caloric, rotary chair,
HIT, and otolith test (VEMP))
• Only unilateral cochlear implantation on the
worst vestibular side
• Bilateral implantion in one step only if there is
an initial bilateral complete vestibular loss (User
and labyrinthitis after meningitis are not always
associated with complete vestibular loss)
Compete vestibular loss should be
diagnosed as early as possible
• For active and early adapted physical therapy
• Multisensory substitution (visual and
proprioceptive) and adaptation of the
environment
• Booklet for parents www.acfos.org
• Email: [email protected]
• Consequences of vestibular loss and what to do
to help the child (collaboration between 5
pediatric physical therapists and 5 pediatric
ENTs from Paris-Ie de France and ACFOS
Vestibular function
• Sadly neglected in the United States
• Because of the risk level of children with hearing
loss, this area of development should be
assessed on all children.
• Relationship between vestibular function and
other aspects of development needs to be
explored.
Ototoxicity
•
•
•
•
•
Medications that destroy inner ear hair cells
–Aminoglycosides
–Loop diuretics
–Chemotherapy agents
•Additional risk factors: renal dysfunction,
concomitant use of vestibulo-/cochleotoxic
medications, prior use, genetic susceptibility
• •May result in severe imbalance, falls, visualmotor problems (eg, oscillopsia)
• •May experience difficulties at school, working
on a computer, learning to drive
17% had subclinical vestibular function
• Otolaryng Head & Neck
Surgery 1998, 119, 695-9
• N=136, 4-9 years
• •31% horizontal spontaneous
gaze nystagmus
• •17% horizontal positional
nystagmus
• •No calorics
• •BOT: 84% significantly below
expected scores
• •Post myringotomy: no
significant ENG anomalies,
improved balance
Otitis Media
Clumsy, delayed walking
•Cause: Middle ear fluid, change in inner ear pressure, toxins
•Significant decrease in motor skill development, balance in children with persistent
otitis media
•
•
•
•
N=30, 5-12 years
•VNG, AC/BC cVEMPs
–No calorics
•No significant findings on
VNG
• Significant findings:
• DHI score
• AC latency P13 N23
Jamie Bogle, Mayo Clinic, Scottsdale, AZ
Genetics and the Vestibular System
• Over 500 genetic mutations known to affect the
auditory / vestibular systems
• –>70 different nonsyndromic loci for genetic
loss
• –Commonly noted in mitochondrial disease
• •30-40% of children with SNHL have vestibular
symptoms
• •Without hearing loss, vestibular
dysfunction often is undiagnosed
Usher Syndrome
Type
I
Ic
Id
If
Ig
II
Iic
Iid
III
Subtype Gene / Protein
Hearing Loss
Vestibular Function
Night Blindness Onset (decade)
Ib
MYO7A Profound Absent 1st
Harmonin
Profound Absent 1st
CHD23 Profound Absent 1st
PCDH15 Profound Absent 1st
SANS
Profound Absent 1st
Iia
Usherin Moderate / progressive
Normal 2nd
VLGR1 Sloping moderate Normal 2nd
Whirlin Sloping moderate Normal 2nd
IIIc
Clarin-1 Progressive
Variable 2nd
Usher Syndrome
• Rare genetic disorder – HL and blindness
• –Retinitis pigmentosa – gradual loss of vision
• Type I
▫
▫
▫
▫
•Absent vestibular function from birth
•Vision loss beginning in childhood
•Reliant on somatosensory information for balance
born profoundly deaf, lose vision in first decade;
balance difficulties & walk late (vestibular loss)
• •Type II
▫ –not born deaf, but hearing loss; no balance problems;
lose vision later (2nd decade)
• •Type III
▫ –gradual loss of hearing & vision; they may or may not
have balance difficulties.
BOR: Branchio-Oto-Renal
• Disrupts development of tissues in the neck, causes
malformations of the ears and kidneys
• •Variable presentation
• •Autosomal dominant; affects 1 in 40,000
• •Vestibular anomalies: LVAS, hypoplasia of the hSCC
• •Hearing loss (>90%)
▫
▫
▫
▫
–Mixed (52%)
–Conductive (33%)
–Sensorineural (29%)
–Progressive (30%)
BOR
NIH; Ceruti et al 2002; Kemperman et al 2002
Major Criteria
Minor Criteria
2nd branchial arch
anomalies
External auditory canal
anomalies
Deafness
Middle ear anomalies
Preauricular pits
Inner ear anomalies
Auricular deformity
Preauricular tags
Renal anomalies
Other: facial asymmetry,
palate abnormalities
Waardenburg Syndrome
• Vestibular symptoms in 20
subjects
• •75% demonstrated reduced
balance function
• •77% demonstrated reduced
VOR
• •29% with hearing loss (5 =
SNHL, 1 = mixed)
• 1/42000-1/50,000
•
•
•
•
•
•
•
•
•
•
Dizziness
Tinnitus
Imbalance
Vertigo
Aural pressure
Headache
Nausea
Visual sensitivity
Noise intolerance
Subjective HL
Waardenburg Syndrome
Major Effects
Minor Effects
SNHL, congenital or progressive
Pigmentation anomalies of the skin:
leukoderma, café au lait spots, vitiligo,
mottling, spotting
Heterochromia irides: eyes of different Synophrys (central fusion of the
colors, segmental color changes within eyebrows)
a single iris, “sapphire blue” irises
White / depigmented hair, usually
manifesting as a white forelock or as
whitening of the eyebrows or lashes
Broad nasal bridge with or without a
high nasal bridge
Dystopia canthorum (lateral
displacement of the medial canthi)
Thin nasal alae
First degree relative with WS
Prematurely gray hair, poliosis
(whiteness of individual head hairs
Pendred Syndrome
• Disorder associated with hearing loss, LVA, and
goiter
• •Autosomal recessive, affects 10% of HL
population
▫ –Most common syndrome
• •Progressive decrease in
▫ –Hearing thresholds
▫ –Vestibular reflexes
▫ –May experience vertigo episodes
• NIH; Reardon & Trembath 1996; Stinckens et al
2001
En/Large Vestibular Aqueduct
Syndrome (LVAS/EVAS)
•
•
•
•
•
5-15% of children with hearing loss
Stepwise, progressive SNHL
Associated with: Pendred syndrome, BOR
Vestibular symptoms
Episodic vertigo: 28.6%
▫ –Motor delay: 9.5%
▫ –Imbalance
• Abnormal vestibular function < 71%
• Symptoms may increase / appear in adulthood
• Grimmer & Hedlund 2007, Berrettini et al 2005
CHARGE
•
•
•
•
•
•
C olomboa
H eart
Choanal Atresia
R etarded growth
G enital anomalies
E ar anomalies
CHARGE Syndrome
CT Imaging
• –Severe SCC anomalies: 94%
• –Abnormal vestibule: 50%
• –Abnormal vestibular aqueduct: 12%
• –Abnormal cochlea: 32%
• •Vestibular dysfunction
• –Abnormal hSCC: 91%
• –Present otolith function: 82%
CHARGE Syndrome
Uwmsk.org/temporalbone/congenital.
html - Abadie et al 2000
Syndromes & Vestibular Involvement
Syndrome
Description
Usher
Type I: congenital, bilateral profound SNHL, retinitis
pigmentosa
Type II: mild-severe progressive high-frequency SNHL
BOR
Preauricular pits / tags, branchial cysts, pseudo-conductive
hearing loss, abnormal kidney development
LVAS
Progressive SNHL, associated with BOR and Pendred
Pendred
Congenital, severe/profound SNHL, abnormal bony labyrinth,
goiter in puberty/adulthood
Waardenburg
Congenital SNHL, pigmentary disturbances of the iris, hair,
skin
CHARGE
Coloboma-heart-atresia-retarded-genital-ear
Vestibular Disorders in Children With
Congenital Cytomegalovirus Infection
Sophie Bernard, MDa, Sylvette Wiener-Vacher, MD, PhDa,b,c,
Thierry Van Den Abbeele, MD, PhDa,b,c, Natacha Teissier, MD,
PhDa,b,c
PEDIATRICS Volume 136, number 4, October 2015
CMV and Vestibular Disorders
• Forty-eight of 52 children (92.3%) had hearing loss and vestibular
disorders.
• Of those, vestibular disorders were
▫ Complete and bilateral in 33.3%,
▫ Partial and bilateral in 43.7%,
▫ Partial and unilateral in 22.9%.
• Serial testing in 14 children showed:
▫ Stable vestibular function in 50%
▫ Deterioration in 50%.
• Congenital CMV infection has a negative impact on postural
development that is correlated with neurologic and vestibular
impairment.
• Vestibular disorders were significantly associated with hearing
disorders, but their respective severities showed no concordance.
• CONCLUSIONS: Vestibular disorders are frequent and severe in
CMV-infected children.
• 0.5-1.0% of infants are infected in the US (Kenneson, 2007)
School-aged children under-performing
• N= 88 children (aged 7-12 years)
• •ENT, hearing and vestibular tests
• –Positional tests, nystagmus, rotary & caloric
tests (no otolith tests)
• –OKN (optokinetic nystagmus)
▫ •49% underperforming
• –68% abnormal vestibular tests(compared to
27% of those not underperforming)
• •Unilateral & bilateral irritative lesions in 68%
Referral and Vestibular Rehabilitation
• If enlarged vestibular aqueduct (EVA) is suspected, a
referral to otolaryngology may be advised.
• If screening and case history point toward a neuritis,
then a visit to otolaryngology may be indicated.
• Once it is determined through quantitative vestibular
testing that a child has a peripheral impairment,
vestibular rehabilitative therapy (VRT) may be an
option.
• This is therapy performed by a specially trained physical
or occupational therapist. Vestibular rehabilitation is
successful in improving postural control, gross motor
skills, dynamic visual acuity, and even reading acuity in
pediatric patients with vestibular deficits (Rine et al.,
2004).
Therapeutic Goals
Enhance existing vestibular capabilities
Strengthen compensatory mechanism
Proprioceptive input
Walk barefoot
Visual input
Other sensory systems
(DelRosario, 2011)
Interventions
Braswell & Rine(2006):
2 children with SNHL and bilateral VH (vestibular
hypofunction)
examined for the effect of visual-vestibular exercises on
dynamic visual acuity (DVA), critical print size (CPS) and
reading acuity (RA)
Conclusion: exercise improves gaze stability that may help
with reading acuity
Interventions
Herdman et al. (2007).
8 patients performed vestibular exercises to enhance
remaining vestibular function
5 patients performed placebo exercises.
Improvement in DVA (Dynamic Visual Acuity) seen in exercise group
No improvement seen in placebo group
Conclusions: Use of vestibular exercises is the main factor
involved in recovery of DVA in patients with BVH (bilateral
vestibular hypofunction).
Interventions
Lewis et al. (1985).
16 children with hearing loss participated in 6 week posture
and body awareness program and control group did not.
Balance in experimental group improved
Control group no improvement in balance.
Conclusions: participation in balance and body awareness
program resulted in significant improvement in balance
skills
Interventions
Medeiros (2005).
16 symptomatic children w/ vestibular disorder and 16
asymptomatic children were treated with VRT (vestibular
rehabilitation therapy).
16 symptomatic children showed improvement
16 asymptomatic children showed no significant changes.
Conclusion: VRT seems to be safe and efficacious
therapeutic option in children with vestibular disorders
Interventions
Rine (2004).
21 children with SNHL and vest. impairment were randomly
assigned to two groups (exercise and placebo) matched for age
and gross motor development level. Therapy 3 times weekly for
12 weeks.
Visual & somatosensory function, balance training.
Significant improvement in motor function
Conclusion: Exercise intervention focused on the enhancement
of sensory integrative postural control abilities is effective for
the arrest of the progressive motor development delay in
children with SNHL and vestibular impairment.
Treatment challenges
Lack of data
Vestibular dysfunction in infancy and early childhood is poorly
understood.
Different causes of balance problems
Need more research on both function and treatment
Why it is so important
Rehabilitation has been shown to be effective in children who
are deaf or hard of hearing
Kathy Jankey, Boys Town National
Institute
• Studies demonstrate that children with
vestibular loss improve:
• –Developmental Milestones (Rine et al., 2004)
• –Progressive delay was halted and performance
moved into the normal range with therapy (Rine
et al., 2004)
• –Critical print size and reading acuity
• (Braswell & Rine, 2006)
• Vestibular loss is treated with gaze
stabilization exercises - ability to stabilize
the visual environment during movement
• –Repeated head and body movements both with
and without visual targets
• –Full range of head movement
• –Varying speed, repetitions and sets to build
endurance
• In children, incorporate imagination in
play
• –Activities with a ball
• –Finger painting
• –TV and video games
• –Again increasing speed, repetitions, and
complexity of environment
• [email protected]