Step into the void: Vestibular deficits in children with hearing loss

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Step into the void Vestibular deficits in
children with hearing loss

• Genevieve DelRosario, MHS, PA-C
• University of Kansas Medical Center
• Kansas City, Kansas

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Objectives

• Review vestibular physiology and pathophysiology
• Discuss the evaluation of a childs vestibular
  status
  
• Be able to develop a plan for a child with
  vestibular dysfunction

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Vestibular system A sixth sense

• Allows us to know where we are in space
• Orient selves with respect to gravity
• Unifying system that allows us to process
  information from other senses
  

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Where does our sense of balance come from?

• Eyes
• Sensors in joints, muscles, and feet
• Balance organs in the ears

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Vestibular system

• Vestibular ocular system
• Responsible for visual stabilization
• Vestibular spinal system
• Maintains orientation of the body in space
• Contributes to the postural tone necessary for
  the acquisition of motor development milestones

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Development of the vestibular system

• Very old in evolutionary terms
• Emerges early in embryonic development
• Prior to vision and hearing
• Peak developmental time is 6-12 months
• Continues development through childhood

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http//www.qmw.ac.uk/ugha014/vestibular20stuff/v
estibular3.html

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http//www.qmw.ac.uk/ugha014/vestibular20stuff/v
estibular3.html

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Prevalence of vestibular dysfunction

• 20-70 of children with hearing loss
• Higher in profound HL vs. severe
• Continuum of severity
• Mild loss to vestibular areflexia

Angeli 2003.
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What happens in deaf/HOH children?

• Semicircular canals may be absent
• Hair cells may be damaged, absent, or reduced
• Nerve damage
• Enlarged vestibular aqueduct
• ???

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Will a deaf childs vestibular system get better?
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Maybe.
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Better or worse?

• In general, balance improves as you age
• Vestibular maturation continues through
  adolescence
  
• 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.
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How does it feel?
http//www.theraider.net/films/crusade/making_4_po
stproduction.php
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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
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Oscillopia
www.dizziness-and-balance.com
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Signs of poor vestibular function

• Low muscle tone
• Delayed loss of primitive reflexes
• Delayed gross motor milestones
• Developmental delays
• Seizures
• Nystagmus
• Easy fatiguability
• Reflux

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Signs of poor vestibular function

• Low muscle tone
• Delay in holding head up
• Snuggly baby
• Floppy baby
• Arching of back

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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.jp
g
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Signs of poor vestibular function

• Delayed motor milestones
• Average deaf child walks at 14 months
• Average child with Ushers Type 1 walks at 20
  mos
  
• Delays sitting, crawling, climbing steps,
  hopping
  
• Speech delays

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What do older children look like?

• Clumsy
• Unable to walk on a balance beam
• Problems standing with feet together and eyes
  closed (Romberg test)
  
• Love spinning,
• merry-go-rounds,
• water activities

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Weak VOR

• Challenges with reading
• Gaze instability causes problems with acuity

Braswell Rine 2006.
www.fotosearch.com
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My deaf child is a late walkerdoes that mean she
has vestibular problems?
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No, but its a red flag!Consider also

• Vision problems
• Global developmental delay
• Autistic spectrum disorder
• Just taking her sweet time!

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Tests of vestibular function

• Eye tracking tests
• Positional/positioning tests
• Dix-Hallpike
• Supine
• Rotational tests
• Rotary chair testing is gold standard

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Causes of poor vestibular function

• Postnatal acquired cases
• Meningitis
• Labyrinthitis
• Some forms of syndromic deafness
• Labyrinthine dysplasia
• Ototoxicity

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Conditions associated with CHL and poor balance

• Ushers Syndrome (Type 1)
• Waardenburg Syndrome
• Pendred syndrome
• ESPN mutation
• CHARGE Syndrome
• Brachio-oto-renal syndrome
• .and more!

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Ushers Syndrome

• Autosomal recessive syndrome
• Hearing loss, vision loss, and variable
  vestibular dysfunction
  
• Visual loss is due to retinitis pigmentosa
• Three types

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Ushers syndrome

• Type 1
• 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
• Type 3
• Born with normal hearing, varying rate of loss
• Night blindness during puberty
• Normal or near-normal vestibular function

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Retinitis pigmentosa
                                   
                                   
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Retinitis pigmentosa
http//www.blindness.org/content.asp?id45
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Waardenburg Syndrome
http//www.werathah.com/deafness/waardenburg.htm
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Pendred Syndrome
http//www.bmm.charite.de/rueckschau/ribeiro/ribei
ro.htm
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CHARGE Syndrome

• 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
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Brachio-oto-renal syndrome

• Autosomal dominant
• Malformation of ear
• cochlear hypoplasia
• enlargement of the cochlear and vestibular
  aqueducts
  
• hypoplasia of the lateral semicircular canal
• Hearing loss
• Malformations of kidney

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ESPN Mutation

• Autosomal recessive mutation
• Mapped to chromosome 1p36.3

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Environmental causes

• Aminoglycoside antibiotics
• Gentamicin, streptomycin, kanamycin, tobramycin,
  neomycin, amikacin, netilmicin,
  dihydrostreptomycin, and ribostamycin.
  
• Anti-neoplastics
• Cisplatin, carboplatin
• Environmental chemicals
• Butyl nitrite, mercury, carbon disulfide,
  styrene, carbon monoxide, tin, hexane, toluene,
  lead, trichloroethylene, manganese, xylene,
  mercury
• Loop diuretics
• Bumetanide, ethacrynic acid, furosemide, and
  torsemide.
  
• Aspirin and quinine products
• Infections

www.vestibular.org
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Vestibular effects of cochlear implantation

• Rare cause of permanent damage
• Common cause of transient damage
• 20 in one series1
• Anecdotal evidence for improvement
• Hearing with CI does not make a difference2

1 Vilbert et al 2001. 2. Suarez et all 2007.
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I think the child I am treating may have
vestibular problems. Now what?
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Evaluation

• CT of temporal bone
• Vestibular testing (if possible)
• Physical, occupational, ? cognitive therapies
• Genetic appointment
• Strongly consider testing for Ushers mutations
• Vision evaluation
• ?ERG

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Therapeutic goals

• Enhance existing vestibular capabilities
• Strengthen compensatory mechanisms

http//www.tradecards.com/articles/thread/thread11
.html
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Compensatory mechanisms

• Proprioceptive input
• Walking barefoot or soft soled shoes
• Visual input
• Other sensory systems

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Therapies for children with poor vestibular
systems

• Swinging
• Rocking
• Bouncing/jumping
• Dancing
• Skipping
• Running
• Hopping
• Jumping rope
• Rough and tumble play

http//static.flickr.com/49/131593782_00522c7610_m
.jpg
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Other interventions

• May wear weighted vests, leg weights, etc
• Consider orthopedic shoes
• OR soft soled shoes
• Offer sensory activities
• May need extra time to
• process information

www.bright-start.com
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Does treatment make a difference?

• 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
• May improve gaze stability2
• Preliminary study of two individuals

1. Rine et al 2004. 2. Braswell and Rine 2006.
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Treatment challenges

• Lack of data
• Especially true for infants and toddlers
• Different causes of balance problems

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Cautions with poor vestibular function

• Where visual and proprioceptive information is
  unreliable
  
• Eg, swimming in the dark
• Problems with depth perception
• Tunnel vision can cause problems
• Worse in unfamiliar places

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Implications for future research

• Vestibular hypofunction in infancy and early
  childhood poorly understood
  
• Need for research on both function and treatment

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Implications for families

• Share vestibular information with parents
• Encourage physical activity
• Continue to screen older children
• Balance
• Retinitis pigmentosa

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Helpful resources

• Whats 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

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Genevieve DelRosariogdelrosario_at_kumc.eduDepartme
nt of PediatricsUniversity of Kansas Medical
Center3901 Rainbow BlvdKansas City, KS
66160(913) 588-5908
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• www.chargesyndrome.org
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• Eliot, L. Whats going on in there How the brain
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• www.sense.org
• Siegel JC, Marchetti M, Tecklin JS. Age-related
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• Suarez H, Angeli S, Suarez A, Rosales B, Carrera
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  Feb 1 Epub ahead of print
• www.vestibular.org
• Vibert D, Hausler R, Kompis M, Visher M.
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