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

Genevieve DelRosario, MHS, PA-CGenevieve DelRosario, MHS, PA-C

University of Kansas Medical University of Kansas Medical CenterCenter

Kansas City, KansasKansas City, Kansas

ObjectivesObjectives

Review vestibular physiology and Review vestibular physiology and pathophysiologypathophysiology

Discuss the evaluation of a child’s Discuss the evaluation of a child’s vestibular statusvestibular status

Be able to develop a plan for a Be able to develop a plan for a child with vestibular dysfunctionchild with vestibular dysfunction

Vestibular system: A “sixth Vestibular system: A “sixth sense”sense”

Allows us to know where we are in Allows us to know where we are in spacespace

Orient selves with respect to gravityOrient selves with respect to gravity

Unifying system that allows us to Unifying system that allows us to process information from other process information from other sensessenses

Where does our sense Where does our sense of balance come from?of balance come from? EyesEyes

Sensors in joints, muscles, and Sensors in joints, muscles, and feetfeet

Balance organs in the earsBalance organs in the ears

Vestibular systemVestibular system

Vestibular ocular systemVestibular ocular system– Responsible for visual stabilizationResponsible for visual stabilization

Vestibular spinal systemVestibular spinal system– Maintains orientation of the body in Maintains orientation of the body in

spacespace– Contributes to the postural tone Contributes to the postural tone

necessary for the acquisition of necessary for the acquisition of motor development milestonesmotor development milestones

Development of the vestibular Development of the vestibular systemsystem

Very old in evolutionary termsVery old in evolutionary terms

Emerges early in embryonic developmentEmerges early in embryonic development– Prior to vision and hearingPrior to vision and hearing

Peak developmental time is 6-12 monthsPeak developmental time is 6-12 months

Continues development through childhoodContinues development through childhood

http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.htmlvestibular3.html

http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/http://www.qmw.ac.uk/~ugha014/vestibular%20stuff/vestibular3.htmlvestibular3.html

Prevalence of vestibular Prevalence of vestibular dysfunction*dysfunction*

20-70% of children with hearing 20-70% of children with hearing lossloss

Higher in profound HL vs. severeHigher in profound HL vs. severe

Continuum of severityContinuum of severity– Mild loss to vestibular areflexiaMild loss to vestibular areflexia

* Angeli 2003.

What happens in deaf/HOH What happens in deaf/HOH children?children?

Semicircular canals may be absentSemicircular canals may be absent

Hair cells may be damaged, absent, or Hair cells may be damaged, absent, or reducedreduced

Nerve damageNerve damage

Enlarged vestibular aqueductEnlarged vestibular aqueduct

??????

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

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 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

http://www.frisbee.li/webobtimierte-bilder/abb1.jpg

Signs of poor vestibular Signs of poor vestibular functionfunction

Delayed motor milestonesDelayed motor milestones– Average deaf child walks at 14 monthsAverage deaf child walks at 14 months

– Average child with Usher’s Type 1 walks at 20 Average child with Usher’s Type 1 walks at 20 mosmos

– Delays sitting, crawling, climbing steps, Delays sitting, crawling, climbing steps, hopping…hopping…

– Speech delaysSpeech delays

What do older children look What do older children look like?like?

ClumsyClumsy

Unable to walk on a balance beamUnable to walk on a balance beam

Problems standing with feet together Problems standing with feet together and eyes closed (Romberg test)and eyes closed (Romberg test)

Love spinning,Love spinning,

merry-go-rounds,merry-go-rounds,

water activitieswater activities

Weak VORWeak VOR

Challenges with readingChallenges with reading

– Gaze instability causes problems with Gaze instability causes problems with acuity*acuity*

Braswell & Rine 2006.www.fotosearch.com

My deaf child is a My deaf child is a late walker…does late walker…does that mean she has that mean she has

vestibular problems?vestibular problems?

No, but it’s a red flag!No, but it’s a red flag!Consider also:Consider also:

Vision problemsVision problems

Global developmental delayGlobal developmental delay

Autistic spectrum disorderAutistic spectrum disorder

Just taking her sweet time!Just taking her sweet time!

Tests of vestibular Tests of vestibular functionfunction

Eye tracking testsEye tracking tests

Positional/positioning tests Positional/positioning tests – Dix-HallpikeDix-Hallpike– SupineSupine

Rotational testsRotational tests– Rotary chair testing is gold standardRotary chair testing is gold standard

Causes of poor vestibular Causes of poor vestibular functionfunction

Postnatal acquired cases Postnatal acquired cases – Meningitis Meningitis – LabyrinthitisLabyrinthitis

Some forms of syndromic deafnessSome forms of syndromic deafness

Labyrinthine dysplasiaLabyrinthine dysplasia

OtotoxicityOtotoxicity

Conditions associated with Conditions associated with CHL and poor balanceCHL and poor balance

Usher’s Syndrome (Type 1)Usher’s Syndrome (Type 1)

Waardenburg SyndromeWaardenburg Syndrome

Pendred syndromePendred syndrome

ESPN mutationESPN mutation

CHARGE SyndromeCHARGE Syndrome

Brachio-oto-renal syndromeBrachio-oto-renal syndrome

…….and more!.and more!

Usher’s SyndromeUsher’s Syndrome

Autosomal recessive syndrome Autosomal recessive syndrome

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

Retinitis pigmentosaRetinitis pigmentosa

                                   <>                                    <>

Retinitis pigmentosaRetinitis pigmentosa

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

Waardenburg Waardenburg SyndromeSyndrome

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

Pendred SyndromePendred Syndrome

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

CHARGE SyndromeCHARGE Syndrome

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

www.charrgesydnrome.org

Brachio-oto-renal Brachio-oto-renal syndromesyndrome

Autosomal dominantAutosomal dominant

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

Environmental causesEnvironmental causes

Aminoglycoside antibioticsAminoglycoside antibiotics– Gentamicin, streptomycin, kanamycin, tobramycin, Gentamicin, streptomycin, kanamycin, tobramycin,

neomycin, amikacin, netilmicin, dihydrostreptomycin, and neomycin, amikacin, netilmicin, dihydrostreptomycin, and ribostamycin. ribostamycin.

Anti-neoplastics Anti-neoplastics – Cisplatin, carboplatin Cisplatin, carboplatin

Environmental chemicalsEnvironmental chemicals– Butyl nitrite, mercury, carbon disulfide, styrene, carbon Butyl nitrite, mercury, carbon disulfide, styrene, carbon

monoxide, tin, hexane, toluene, lead, trichloroethylene, monoxide, tin, hexane, toluene, lead, trichloroethylene, manganese, xylene, mercury manganese, xylene, mercury

Loop diureticsLoop diuretics– Bumetanide, ethacrynic acid, furosemide, and torsemide.Bumetanide, ethacrynic acid, furosemide, and torsemide.

Aspirin and quinine productsAspirin and quinine products

InfectionsInfectionswww.vestibular.org

Vestibular effects of cochlear Vestibular effects of cochlear implantationimplantation

Rare cause of permanent damageRare cause of permanent damage

Common cause of transient damageCommon cause of transient damage– 20% in one series20% in one series11

Anecdotal evidence for improvementAnecdotal evidence for improvement

HearingHearing with CI does not make a with CI does not make a differencedifference22

1: Vilbert et al 2001. 2. Suarez et all 2007.

I think the child I am I think the child I am treating may have treating may have

vestibular problems. vestibular problems. Now what?Now what?

EvaluationEvaluation

CT of temporal boneCT of temporal bone

Vestibular testing (if possible)Vestibular testing (if possible)

Physical, occupational, ? cognitive Physical, occupational, ? cognitive therapiestherapies

Genetic appointmentGenetic appointment– Strongly consider testing for Usher’s mutationsStrongly consider testing for Usher’s mutations

Vision evaluationVision evaluation– ?ERG?ERG

Therapeutic goalsTherapeutic goals

Enhance existing vestibular Enhance existing vestibular capabilitiescapabilities

Strengthen compensatory Strengthen compensatory mechanismsmechanisms

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

Compensatory Compensatory mechanismsmechanisms

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

http://static.flickr.com/49/131593782_00522c7610_m.jpg

Other interventionsOther interventions

May wear weighted vests, leg May wear weighted vests, leg weights, etcweights, etc

Consider orthopedic shoesConsider orthopedic shoes– OR soft soled shoesOR soft soled shoes

Offer sensory activitiesOffer sensory activities

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

Encourage physical activityEncourage physical activity

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.

Vestibular disorders organization Vestibular disorders organization www.vestibular.orgwww.vestibular.org

www.boystownhospital.orgwww.boystownhospital.org

Genevieve DelRosarioGenevieve DelRosariogdelrosario@kumc.edugdelrosario@kumc.edu

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

cochlear implantation. Acta Otolaryngol Suppl. 2001; 545: 29-34.cochlear implantation. Acta Otolaryngol Suppl. 2001; 545: 29-34.