Step into the void:
Vestibular deficits in
children with hearing loss
Genevieve DelRosario, MHS, PA-C
University of Kansas Medical Center
Kansas City, Kansas
Objectives
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Review vestibular physiology and
pathophysiology
Discuss the evaluation of a child’s
vestibular status
Be able to develop a plan for a child
with vestibular dysfunction
Vestibular system: A “sixth sense”
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Allows us to know where we are in space
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Orient selves with respect to gravity
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Unifying system that allows us to process
information from other senses
Where does our sense of
balance come from?
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Eyes
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Sensors in joints, muscles, and feet
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Balance organs in the ears
Vestibular system

Vestibular ocular system
– Responsible for visual stabilization
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Vestibular spinal system
– Maintains orientation of the body in space
– Contributes to the postural tone
necessary for the acquisition of motor
development milestones
Development of the vestibular system
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Very old in evolutionary terms
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Emerges early in embryonic development
– Prior to vision and hearing
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Peak developmental time is 6-12 months
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Continues development through childhood
http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.html
http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.html
Prevalence of vestibular dysfunction*
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20-70% of children with hearing loss
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Higher in profound HL vs. severe
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Continuum of severity
– Mild loss to vestibular areflexia
* Angeli 2003.
What happens in deaf/HOH children?
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Semicircular canals may be absent
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Hair cells may be damaged, absent, or reduced
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Nerve damage
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Enlarged vestibular aqueduct
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???
Will a deaf child’s
vestibular system get
better?
……Maybe.
Better or worse?
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In general, balance improves as you age
– Vestibular maturation continues through
adolescence
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Vestibular deficits in deaf/HOH may worsen
– Small study showed progressive gross motor
and balance difficulties1
– Contradicted by other studies2
1. Rine et al 2000. 2. Siegel et al 1991.
How does it feel?
http://www.theraider.net/films/crusade/making_4_postproduction.php
How does it feel (adult perspective)?
– Headache
– Feeling of ear fullness
– Imbalance to the point of being unable to walk
– Bouncing and blurring of vision (oscillopsia)
– Inability to tolerate head movement
– Difficulty walking in the dark
– Feel unsteady; actual unsteadiness while moving
– Lightheadedness
– Severe fatigue
In severe cases, symptoms such as oscillopsia and problems
with walking in the dark are not going to go away.
www.vestibular.org
Oscillopia
www.dizziness-and-balance.com
Signs of poor vestibular function
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Low muscle tone
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Delayed loss of primitive reflexes
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Delayed gross motor milestones
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Developmental delays
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Seizures
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Nystagmus
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Easy fatiguability
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Reflux
Signs of poor vestibular function
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Low muscle tone
– Delay in holding head up
– “Snuggly” baby
– “Floppy baby”
– Arching of back
Signs of poor vestibular function
Delayed disappearance of newborn reflexes
– Moro
– ATNR: Asymmetric tonic next response
– Usually disappear by 6-7 months
http://www.frisbee.li/webobtimierte-bilder/abb1.jpg
Signs of poor vestibular function
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Delayed motor milestones
– Average deaf child walks at 14 months
– Average child with Usher’s Type 1 walks at 20 mos
– Delays sitting, crawling, climbing steps, hopping…
– Speech delays
What do older children look like?
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Clumsy
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Unable to walk on a balance beam
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Problems standing with feet together and eyes
closed (Romberg test)
Love spinning,
merry-go-rounds,
water activities
Weak VOR
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Challenges with reading
– Gaze instability causes problems with acuity*
Braswell & Rine 2006.
www.fotosearch.com
My deaf child is a late
walker…does that mean
she has vestibular
problems?
No, but it’s a red flag!
Consider also:
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Vision problems
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Global developmental delay
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Autistic spectrum disorder
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Just taking her sweet time!
Tests of vestibular function
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Eye tracking tests
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Positional/positioning tests
– Dix-Hallpike
– Supine
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Rotational tests
– Rotary chair testing is gold standard
Causes of poor vestibular function
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Postnatal acquired cases
– Meningitis
– Labyrinthitis
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Some forms of syndromic deafness
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Labyrinthine dysplasia
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Ototoxicity
Conditions associated with
CHL and poor balance
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Usher’s Syndrome (Type 1)
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Waardenburg Syndrome
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Pendred syndrome
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ESPN mutation
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CHARGE Syndrome
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Brachio-oto-renal syndrome
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….and more!
Usher’s Syndrome
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Autosomal recessive syndrome
Hearing loss, vision loss, and variable
vestibular dysfunction
– Visual loss is due to retinitis pigmentosa
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Three types
Usher’s syndrome
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Type 1
–
–
–
–
–
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Born profoundly deaf
Vision loss typically noted by age 10
Absent vestibular function
3-6/100,000 individuals
~ 5% of deaf individuals
Type 2
– Moderate to severe hearing loss
– Vision loss typically begins after teen years
– Normal vestibular function
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Type 3
– Born with normal hearing, varying rate of loss
– Night blindness during puberty
– Normal or near-normal vestibular function
Retinitis pigmentosa
<>
Retinitis pigmentosa
http://www.blindness.org/content.asp?id=45
Waardenburg Syndrome
http://www.werathah.com/deafness/waardenburg.htm
Pendred Syndrome
CHARGE Syndrome
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Coloboma of the eye
Heart defects
Atresia of the choanae
Retardation of growth and/or development
Genital and/or urinary abnormalities
Ear abnormalities and deafness
www.charrgesydnrome.org
Brachio-oto-renal syndrome
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Autosomal dominant
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Malformation of ear
– cochlear hypoplasia
– enlargement of the cochlear and vestibular aqueducts
– hypoplasia of the lateral semicircular canal
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Hearing loss
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Malformations of kidney
ESPN Mutation
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Autosomal recessive mutation
Mapped to chromosome 1p36.3
Environmental causes
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Aminoglycoside antibiotics
–
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Gentamicin, streptomycin, kanamycin, tobramycin, neomycin,
amikacin, netilmicin, dihydrostreptomycin, and ribostamycin.
Anti-neoplastics
– Cisplatin, carboplatin
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Environmental chemicals
– Butyl nitrite, mercury, carbon disulfide, styrene, carbon monoxide, tin,
hexane, toluene, lead, trichloroethylene, manganese, xylene, mercury
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Loop diuretics
– Bumetanide, ethacrynic acid, furosemide, and torsemide.
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Aspirin and quinine products
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Infections
www.vestibular.org
Vestibular effects of cochlear implantation
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Rare cause of permanent damage
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Common cause of transient damage
– 20% in one series1
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Anecdotal evidence for improvement
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Hearing with CI does not make a difference2
1: Vilbert et al 2001. 2. Suarez et all 2007.
I think the child I am
treating may have
vestibular problems.
Now what?
Evaluation
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CT of temporal bone
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Vestibular testing (if possible)
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Physical, occupational, ? cognitive therapies
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Genetic appointment
– Strongly consider testing for Usher’s mutations
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Vision evaluation
– ?ERG
Therapeutic goals
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Enhance existing vestibular capabilities
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Strengthen compensatory mechanisms
http://www.tradecards.com/articles/thread/thread11.html
Compensatory mechanisms
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Proprioceptive input
– Walking barefoot or soft soled shoes
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Visual input
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Other sensory systems
Therapies for children with
poor vestibular systems
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Swinging
Rocking
Bouncing/jumping
Dancing
Skipping
Running
Hopping
Jumping rope
Rough and tumble play
http://static.flickr.com/49/1
31593782_00522c7610_m.j
Other interventions
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May wear weighted vests, leg weights, etc
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Consider orthopedic shoes
– OR soft soled shoes
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Offer sensory activities
May need extra time to
process information
www.bright-start.com
Does treatment make a difference?
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Motor development improved post treatment1
– Therapy three times weekly for 12 weeks
– Visual and somatosensory function, balance training
– Significant improvement in motor development
– Insignificant improvement in posturography
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May improve gaze stability2
– Preliminary study of two individuals
1. Rine et al 2004. 2. Braswell and Rine 2006.
Treatment challenges
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Lack of data
– Especially true for infants and toddlers
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Different causes of balance problems
Cautions with poor vestibular function
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Where visual and proprioceptive
information is unreliable
– Eg, swimming in the dark
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Problems with depth perception
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Tunnel vision can cause problems
– Worse in unfamiliar places
Implications for future research
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Vestibular hypofunction in infancy and
early childhood poorly understood
Need for research on both function
and treatment
Implications for families
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Share vestibular information with parents
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Encourage physical activity
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Continue to screen older children
– Balance
– Retinitis pigmentosa
Helpful resources
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What’s going on in there: How the brain and
mind develop in the first five years of life.
Lise Eliot, PhD. 1999
The out of sync child has fun. Carol Stock
Kranowitz & TJ Wylie. 2003.
Vestibular disorders organization
www.vestibular.org
www.boystownhospital.org
Genevieve DelRosario
gdelrosario@kumc.edu
Department of Pediatrics
University of Kansas Medical Center
3901 Rainbow Blvd
Kansas City, KS 66160
(913) 588-5908
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Angeli S. Value of vestibular testing in young children with sensorineural hearing loss.
Arch Otolaryngol Head Neck Surg. 2003;129:478-482.
Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading acuity in
children. Int J Pediatr Otorhinolaryngol. 2006 Nov; 70(11): 1957-1965.
Braswell, J, Rine RM. Preliminary evidence of improved gaze stability following exercise
in two children with vestibular hypofunction. Int J Pediatr Otorhinolaryngol. 2006
Nov;70(11):1967-73. Epub 2006 Oct 4
www.chargesyndrome.org
www.dizziness-and-balance.com
Eliot, L. What’s going on in there: How the brain and mind develop in the first five years
of life. Bantam Books, 1999.
Rine RM, Braswell J, Fisher D, Joyce K, Kalar K, Shaffer M. Improvement of motor
development and postural control following intervention in children with sensorineural
hearing loss and vestibular impairment. Int J Pediatr Otorhinolaryngol. 2004
Sep;68(9):1141-8.
Rine RM, Cornwall G, Gan K, LoCascio C, OHare T, Robinson E, Rice M. Evidence of
progressive delay of motor development in children with sensorineural hearing loss and
concurrent vestibular dysfunction. Perceptual and Motor Skills. 90(3 Pt 2): 11-1-12,
2000 June.
www.sense.org
Siegel JC, Marchetti M, Tecklin JS. Age-related balance changes in hearing-impaired
children. Phys Ther. 1991 Mar;71(3):183-9
Suarez H, Angeli S, Suarez A, Rosales B, Carrera X, Alonso R. Balance sensory
ogranization in children with profound hearing loss and cochlear implants. Int J Pediatr
Otorhinolaryngol. 2007 Feb 1; [Epub ahead of print]
www.vestibular.org
Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with cochlear
implantation. Acta Otolaryngol Suppl. 2001; 545: 29-34.