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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. Objectives. Review vestibular physiology and pathophysiology Discuss the evaluation of a child’s vestibular status

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step into the void vestibular deficits in children with hearing loss

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
Objectives
  • 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
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
where does our sense of balance come from
Where does our sense of balance come from?
  • Eyes
  • Sensors in joints, muscles, and feet
  • Balance organs in the ears
vestibular system
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
development of the vestibular system
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
slide9

http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.htmlhttp://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.html

slide10

http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.htmlhttp://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.html

prevalence of vestibular dysfunction
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.

what happens in deaf hoh children
What happens in deaf/HOH children?
  • Semicircular canals may be absent
  • Hair cells may be damaged, absent, or reduced
  • Nerve damage
  • Enlarged vestibular aqueduct
  • ???
better or worse
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.

how does it feel
How does it feel?

http://www.theraider.net/films/crusade/making_4_postproduction.php

how does it feel adult perspective
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
Oscillopia

www.dizziness-and-balance.com

signs of poor vestibular function
Signs of poor vestibular function
  • Low muscle tone
  • Delayed loss of primitive reflexes
  • Delayed gross motor milestones
  • Developmental delays
  • Seizures
  • Nystagmus
  • Easy fatiguability
  • Reflux
signs of poor vestibular function20
Signs of poor vestibular function
  • Low muscle tone
    • Delay in holding head up
    • “Snuggly” baby
    • “Floppy baby”
    • Arching of back
signs of poor vestibular function21
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 function22
Signs of poor vestibular function
  • 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
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

weak vor
Weak VOR
  • Challenges with reading
    • Gaze instability causes problems with acuity*

Braswell & Rine 2006.

www.fotosearch.com

no but it s a red flag consider also
No, but it’s a red flag!Consider also:
  • Vision problems
  • Global developmental delay
  • Autistic spectrum disorder
  • Just taking her sweet time!
tests of vestibular function
Tests of vestibular function
  • Eye tracking tests
  • Positional/positioning tests
    • Dix-Hallpike
    • Supine
  • Rotational tests
    • Rotary chair testing is gold standard
causes of poor vestibular function
Causes of poor vestibular function
  • Postnatal acquired cases
    • Meningitis
    • Labyrinthitis
  • Some forms of syndromic deafness
  • Labyrinthine dysplasia
  • Ototoxicity
conditions associated with chl and poor balance
Conditions associated with CHL and poor balance
  • Usher’s Syndrome (Type 1)
  • Waardenburg Syndrome
  • Pendred syndrome
  • ESPN mutation
  • CHARGE Syndrome
  • Brachio-oto-renal syndrome
  • ….and more!
usher s syndrome
Usher’s Syndrome
  • Autosomal recessive syndrome
  • Hearing loss, vision loss, and variable vestibular dysfunction
    • Visual loss is due to retinitis pigmentosa
  • Three types
usher s syndrome31
Usher’s 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
retinitis pigmentosa
Retinitis pigmentosa

                                  <>

                                  <>

retinitis pigmentosa33
Retinitis pigmentosa

http://www.blindness.org/content.asp?id=45

waardenburg syndrome
Waardenburg Syndrome

http://www.werathah.com/deafness/waardenburg.htm

pendred syndrome
Pendred Syndrome

http://www.bmm.charite.de/rueckschau/ribeiro/ribeiro.htm

charge syndrome
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

brachio oto renal syndrome
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
espn mutation
ESPN Mutation
  • Autosomal recessive mutation
  • Mapped to chromosome 1p36.3
environmental causes
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

vestibular effects of cochlear implantation
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.

evaluation
Evaluation
  • CT of temporal bone
  • Vestibular testing (if possible)
  • Physical, occupational, ? cognitive therapies
  • Genetic appointment
    • Strongly consider testing for Usher’s mutations
  • Vision evaluation
    • ?ERG
therapeutic goals
Therapeutic goals
  • Enhance existing vestibular capabilities
  • Strengthen compensatory mechanisms

http://www.tradecards.com/articles/thread/thread11.html

compensatory mechanisms
Compensatory mechanisms
  • Proprioceptive input
    • Walking barefoot or soft soled shoes
  • Visual input
  • Other sensory systems
therapies for children with poor vestibular systems
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

other interventions
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

does treatment make a difference
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.

treatment challenges
Treatment challenges
  • Lack of data
    • Especially true for infants and toddlers
  • Different causes of balance problems
cautions with poor vestibular function
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
implications for future research
Implications for future research
  • Vestibular hypofunction in infancy and early childhood poorly understood
  • Need for research on both function and treatment
implications for families
Implications for families
  • Share vestibular information with parents
  • Encourage physical activity
  • Continue to screen older children
    • Balance
    • Retinitis pigmentosa
helpful resources
Helpful resources
  • 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
slide54

Genevieve DelRosariogdelrosario@kumc.eduDepartment of PediatricsUniversity of Kansas Medical Center3901 Rainbow BlvdKansas City, KS 66160(913) 588-5908

slide55
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.