Step into the void: Step into the void: Vestibular deficits in Vestibular deficits in children with hearing children with hearing loss loss Genevieve DelRosario, MHS, Genevieve DelRosario, MHS, PA-C PA-C University of Kansas Medical University of Kansas Medical Center Center Kansas City, Kansas Kansas City, Kansas
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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,
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Step into the void: Step into the void: Vestibular deficits in Vestibular deficits in children with hearing children with hearing lossloss
Will a deaf child’s Will a deaf child’s vestibular system get vestibular system get
better?better?
…………Maybe.Maybe.
Better or worse?Better or worse?
In general, balance improves as you ageIn general, balance improves as you age– Vestibular maturation continues through Vestibular maturation continues through
adolescenceadolescence
Vestibular deficits in deaf/HOH may Vestibular deficits in deaf/HOH may worsenworsen– Small study showed progressive gross Small study showed progressive gross
motor and balance difficultiesmotor and balance difficulties11
– Contradicted by other studiesContradicted by other studies22
How does it feel (adult How does it feel (adult perspective)?perspective)?
– HeadacheHeadache
– Feeling of ear fullness Feeling of ear fullness
– Imbalance to the point of being unable to walk Imbalance to the point of being unable to walk
– Bouncing and blurring of vision (oscillopsia) Bouncing and blurring of vision (oscillopsia)
– Inability to tolerate head movement Inability to tolerate head movement
– Difficulty walking in the dark Difficulty walking in the dark
– Feel unsteady; actual unsteadiness while movingFeel unsteady; actual unsteadiness while moving
– LightheadednessLightheadedness
– Severe fatigueSevere fatigue
In severe cases, symptoms such as oscillopsia and problems In severe cases, symptoms such as oscillopsia and problems with walking in the dark are not going to go away.with walking in the dark are not going to go away.
www.vestibular.org
OscillopiaOscillopia
www.dizziness-and-balance.com
Signs of poor vestibular Signs of poor vestibular functionfunction
Low muscle toneLow muscle tone
Delayed loss of primitive reflexesDelayed loss of primitive reflexes
Delayed gross motor milestonesDelayed gross motor milestones
Developmental delaysDevelopmental delays
SeizuresSeizures
NystagmusNystagmus
Easy fatiguabilityEasy fatiguability
RefluxReflux
Signs of poor vestibular Signs of poor vestibular functionfunction
Low muscle toneLow muscle tone
– Delay in holding head upDelay in holding head up
– ““Snuggly” babySnuggly” baby
– ““Floppy baby”Floppy baby”
– Arching of backArching of back
Signs of poor vestibular Signs of poor vestibular functionfunction
Delayed disappearance of newborn Delayed disappearance of newborn reflexesreflexes– MoroMoro– ATNR: Asymmetric tonic next responseATNR: Asymmetric tonic next response– Usually disappear by 6-7 monthsUsually disappear by 6-7 months
Hearing loss, vision loss, and Hearing loss, vision loss, and variable vestibular dysfunctionvariable vestibular dysfunction– Visual loss is due to retinitis Visual loss is due to retinitis
pigmentosapigmentosa
Three typesThree types
Usher’s syndromeUsher’s syndrome Type 1Type 1
– Born profoundly deafBorn profoundly deaf– Vision loss typically noted by age 10Vision loss typically noted by age 10– Absent vestibular functionAbsent vestibular function– 3-6/100,000 individuals3-6/100,000 individuals– ~ 5% of deaf individuals~ 5% of deaf individuals
Type 2Type 2– Moderate to severe hearing lossModerate to severe hearing loss– Vision loss typically begins after teen yearsVision loss typically begins after teen years– Normal vestibular functionNormal vestibular function
Type 3Type 3– Born with normal hearing, varying rate of lossBorn with normal hearing, varying rate of loss– Night blindness during pubertyNight blindness during puberty– Normal or near-normal vestibular functionNormal or near-normal vestibular function
CColoboma of the eyeoloboma of the eye HHeart defects eart defects AAtresia of the choanae tresia of the choanae RRetardation of growth and/or etardation of growth and/or
development development GGenital and/or urinary abnormalities enital and/or urinary abnormalities EEar abnormalities and deafness ar abnormalities and deafness
Malformation of earMalformation of ear– cochlear hypoplasiacochlear hypoplasia– enlargement of the cochlear and vestibular enlargement of the cochlear and vestibular
aqueductsaqueducts– hypoplasia of the lateral semicircular canalhypoplasia of the lateral semicircular canal
Hearing lossHearing loss
Malformations of kidneyMalformations of kidney
ESPN MutationESPN Mutation
Autosomal recessive mutationAutosomal recessive mutation Mapped to chromosome 1p36.3Mapped to chromosome 1p36.3
Proprioceptive inputProprioceptive input– Walking barefoot or soft soled shoesWalking barefoot or soft soled shoes
Visual inputVisual input
Other sensory systemsOther sensory systems
Therapies for children Therapies for children with poor vestibular with poor vestibular systemssystems SwingingSwinging RockingRocking Bouncing/jumping Bouncing/jumping DancingDancing SkippingSkipping RunningRunning HoppingHopping Jumping rope Jumping rope Rough and tumble play Rough and tumble play
May need extra time toMay need extra time to process informationprocess information
www.bright-start.com
Does treatment make a Does treatment make a difference?difference?
Motor development improved post treatmentMotor development improved post treatment11
– Therapy three times weekly for 12 weeksTherapy three times weekly for 12 weeks
– Visual and somatosensory function, balance Visual and somatosensory function, balance trainingtraining
– Significant improvement in motor developmentSignificant improvement in motor development
– Insignificant improvement in posturographyInsignificant improvement in posturography
May improve gaze stabilityMay improve gaze stability22
– Preliminary study of two individualsPreliminary study of two individuals
1. Rine et al 2004. 2. Braswell and Rine 2006.
Treatment challengesTreatment challenges
Lack of dataLack of data– Especially true for infants and Especially true for infants and
toddlerstoddlers
Different causes of balance Different causes of balance problemsproblems
Cautions with poor vestibular Cautions with poor vestibular functionfunction
Where visual and proprioceptive Where visual and proprioceptive information is unreliableinformation is unreliable– Eg, swimming in the darkEg, swimming in the dark
Problems with depth perceptionProblems with depth perception
Tunnel vision can cause problems Tunnel vision can cause problems – Worse in unfamiliar placesWorse in unfamiliar places
Implications for future Implications for future researchresearch
Vestibular hypofunction in infancy Vestibular hypofunction in infancy and early childhood poorly and early childhood poorly understoodunderstood
Need for research on both Need for research on both function and treatmentfunction and treatment
Implications for Implications for familiesfamilies
Share vestibular information with Share vestibular information with parentsparents
Continue to screen older childrenContinue to screen older children– BalanceBalance– Retinitis pigmentosaRetinitis pigmentosa
Helpful resourcesHelpful resources
What’s going on in there: How the What’s going on in there: How the brain and mind develop in the first five brain and mind develop in the first five years of life. Lise Eliot, PhD. 1999years of life. Lise Eliot, PhD. 1999
The out of sync child has fun. Carol The out of sync child has fun. Carol Stock Kranowitz & TJ Wylie. 2003.Stock Kranowitz & TJ Wylie. 2003.
Department of PediatricsDepartment of PediatricsUniversity of Kansas Medical University of Kansas Medical
CenterCenter3901 Rainbow Blvd3901 Rainbow Blvd
Kansas City, KS 66160Kansas City, KS 66160(913) 588-5908(913) 588-5908
Angeli S. Value of vestibular testing in young children with sensorineural Angeli S. Value of vestibular testing in young children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 2003;129:478-482.hearing loss. Arch Otolaryngol Head Neck Surg. 2003;129:478-482.
Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading acuity in children. Int J Pediatr Otorhinolaryngol. 2006 Nov; 70(11): 1957-acuity in children. Int J Pediatr Otorhinolaryngol. 2006 Nov; 70(11): 1957-1965.1965.
Braswell, J, Rine RM. Preliminary evidence of improved gaze stability Braswell, J, Rine RM. Preliminary evidence of improved gaze stability following exercise in two children with vestibular hypofunction. Int J Pediatr following exercise in two children with vestibular hypofunction. Int J Pediatr Otorhinolaryngol. 2006 Nov;70(11):1967-73. Epub 2006 Oct 4 Otorhinolaryngol. 2006 Nov;70(11):1967-73. Epub 2006 Oct 4
www.chargesyndrome.orgwww.chargesyndrome.org www.dizziness-and-balance.comwww.dizziness-and-balance.com Eliot, L. What’s going on in there: How the brain and mind develop in the first Eliot, L. What’s going on in there: How the brain and mind develop in the first
five years of life. Bantam Books, 1999.five years of life. Bantam Books, 1999. Rine RM, Braswell J, Fisher D, Joyce K, Kalar K, Shaffer M. Improvement of Rine RM, Braswell J, Fisher D, Joyce K, Kalar K, Shaffer M. Improvement of
motor development and postural control following intervention in children motor development and postural control following intervention in children with sensorineural hearing loss and vestibular impairment. Int J Pediatr with sensorineural hearing loss and vestibular impairment. Int J Pediatr Otorhinolaryngol. 2004 Sep;68(9):1141-8.Otorhinolaryngol. 2004 Sep;68(9):1141-8.
Rine RM, Cornwall G, Gan K, LoCascio C, OHare T, Robinson E, Rice M. Rine RM, Cornwall G, Gan K, LoCascio C, OHare T, Robinson E, Rice M. Evidence of progressive delay of motor development in children with Evidence of progressive delay of motor development in children with sensorineural hearing loss and concurrent vestibular dysfunction. Perceptual sensorineural hearing loss and concurrent vestibular dysfunction. Perceptual and Motor Skills. 90(3 Pt 2): 11-1-12, 2000 June.and Motor Skills. 90(3 Pt 2): 11-1-12, 2000 June.
www.sense.orgwww.sense.org Siegel JC, Marchetti M, Tecklin JS. Age-related balance changes in hearing-Siegel JC, Marchetti M, Tecklin JS. Age-related balance changes in hearing-
impaired children. impaired children. Phys Ther. 1991 Mar;71(3):183-9 Phys Ther. 1991 Mar;71(3):183-9 Suarez H, Angeli S, Suarez A, Rosales B, Carrera X, Alonso R. Balance sensory 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 ogranization in children with profound hearing loss and cochlear implants. Int J Pediatr Otorhinolaryngol. 2007 Feb 1; [Epub ahead of print]J Pediatr Otorhinolaryngol. 2007 Feb 1; [Epub ahead of print]
www.vestibular.orgwww.vestibular.org Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with