3Objectives Review vestibular physiology and pathophysiology Discuss the evaluation of a child’s vestibular statusBe able to develop a plan for a child with vestibular dysfunction
4Vestibular system: A “sixth sense” Allows us to know where we are in spaceOrient selves with respect to gravityUnifying system that allows us to process information from other senses
5Where does our sense of balance come from? EyesSensors in joints, muscles, and feetBalance organs in the ears
6Vestibular system Vestibular ocular system Vestibular spinal system Responsible for visual stabilizationVestibular spinal systemMaintains orientation of the body in spaceContributes to the postural tone necessary for the acquisition of motor development milestones
7Development of the vestibular system Very old in evolutionary termsEmerges early in embryonic developmentPrior to vision and hearingPeak developmental time is 6-12 monthsContinues development through childhood
15Better or worse? In general, balance improves as you age Vestibular maturation continues through adolescenceVestibular deficits in deaf/HOH may worsenSmall study showed progressive gross motor and balance difficulties1Contradicted by other studies21. Rine et al Siegel et al 1991.
17How does it feel (adult perspective)? HeadacheFeeling of ear fullnessImbalance to the point of being unable to walkBouncing and blurring of vision (oscillopsia)Inability to tolerate head movementDifficulty walking in the darkFeel unsteady; actual unsteadiness while movingLightheadednessSevere fatigueIn severe cases, symptoms such as oscillopsia and problems with walking in the dark are not going to go away.
19Signs of poor vestibular function Low muscle toneDelayed loss of primitive reflexesDelayed gross motor milestonesDevelopmental delaysSeizuresNystagmusEasy fatiguabilityReflux
20Signs of poor vestibular function Low muscle toneDelay in holding head up“Snuggly” baby“Floppy baby”Arching of back
21Signs of poor vestibular function Delayed disappearance of newborn reflexesMoroATNR: Asymmetric tonic next responseUsually disappear by 6-7 months
22Signs of poor vestibular function Delayed motor milestonesAverage deaf child walks at 14 monthsAverage child with Usher’s Type 1 walks at 20 mosDelays sitting, crawling, climbing steps, hopping…Speech delays
23What do older children look like? ClumsyUnable to walk on a balance beamProblems standing with feet together and eyes closed (Romberg test)Love spinning,merry-go-rounds,water activities
24Weak VOR Challenges with reading Gaze instability causes problems with acuity*Braswell & Rine 2006.
25My deaf child is a late walker…does that mean she has vestibular problems?
26No, but it’s a red flag! Consider also: Vision problemsGlobal developmental delayAutistic spectrum disorderJust taking her sweet time!
27Tests of vestibular function Eye tracking testsPositional/positioning testsDix-HallpikeSupineRotational testsRotary chair testing is gold standard
28Causes of poor vestibular function Postnatal acquired casesMeningitisLabyrinthitisSome forms of syndromic deafnessLabyrinthine dysplasiaOtotoxicity
29Conditions associated with CHL and poor balance Usher’s Syndrome (Type 1)Waardenburg SyndromePendred syndromeESPN mutationCHARGE SyndromeBrachio-oto-renal syndrome….and more!
30Usher’s Syndrome Autosomal recessive syndrome Hearing loss, vision loss, and variable vestibular dysfunctionVisual loss is due to retinitis pigmentosaThree types
31Usher’s syndrome Type 1 Type 2 Type 3 Born profoundly deaf Vision loss typically noted by age 10Absent vestibular function3-6/100,000 individuals~ 5% of deaf individualsType 2Moderate to severe hearing lossVision loss typically begins after teen yearsNormal vestibular functionType 3Born with normal hearing, varying rate of lossNight blindness during pubertyNormal or near-normal vestibular function
36CHARGE Syndrome Coloboma of the eye Heart defects Atresia of the choanaeRetardation of growth and/or developmentGenital and/or urinary abnormalitiesEar abnormalities and deafness
37Brachio-oto-renal syndrome Autosomal dominantMalformation of earcochlear hypoplasiaenlargement of the cochlear and vestibular aqueductshypoplasia of the lateral semicircular canalHearing lossMalformations of kidney
38ESPN MutationAutosomal recessive mutationMapped to chromosome 1p36.3
40Vestibular effects of cochlear implantation Rare cause of permanent damageCommon cause of transient damage20% in one series1Anecdotal evidence for improvementHearing with CI does not make a difference21: Vilbert et al Suarez et all 2007.
41I think the child I am treating may have vestibular problems. Now what?
42Evaluation CT of temporal bone Vestibular testing (if possible) Physical, occupational, ? cognitive therapiesGenetic appointmentStrongly consider testing for Usher’s mutationsVision evaluation?ERG
44Compensatory mechanisms Proprioceptive inputWalking barefoot or soft soled shoesVisual inputOther sensory systems
45Therapies for children with poor vestibular systems SwingingRockingBouncing/jumpingDancingSkippingRunningHoppingJumping ropeRough and tumble play
46Other interventions May wear weighted vests, leg weights, etc Consider orthopedic shoesOR soft soled shoesOffer sensory activitiesMay need extra time toprocess information
47Does treatment make a difference? Motor development improved post treatment1Therapy three times weekly for 12 weeksVisual and somatosensory function, balance trainingSignificant improvement in motor developmentInsignificant improvement in posturographyMay improve gaze stability2Preliminary study of two individuals1. Rine et al Braswell and Rine 2006.
48Treatment challenges Lack of data Different causes of balance problems Especially true for infants and toddlersDifferent causes of balance problems
49Cautions with poor vestibular function Where visual and proprioceptive information is unreliableEg, swimming in the darkProblems with depth perceptionTunnel vision can cause problemsWorse in unfamiliar places
50Implications for future research Vestibular hypofunction in infancy and early childhood poorly understoodNeed for research on both function and treatment
51Implications for families Share vestibular information with parentsEncourage physical activityContinue to screen older childrenBalanceRetinitis pigmentosa
52Helpful resourcesWhat’s going on in there: How the brain and mind develop in the first five years of life. Lise Eliot, PhD. 1999The out of sync child has fun. Carol Stock Kranowitz & TJ WylieVestibular disorders organization
54Genevieve DelRosario gdelrosario@kumc Genevieve DelRosario Department of Pediatrics University of Kansas Medical Center 3901 Rainbow Blvd Kansas City, KS (913)
55Angeli S. Value of vestibular testing in young children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 2003;129:Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading acuity in children. Int J Pediatr Otorhinolaryngol Nov; 70(11):Braswell, J, Rine RM. Preliminary evidence of improved gaze stability following exercise in two children with vestibular hypofunction. Int J Pediatr Otorhinolaryngol Nov;70(11): Epub 2006 Oct 4Eliot, 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 Sep;68(9):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): , 2000 June.Siegel JC, Marchetti M, Tecklin JS. Age-related balance changes in hearing-impaired children. Phys Ther Mar;71(3):183-9Suarez 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 Feb 1; [Epub ahead of print]Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with cochlear implantation. Acta Otolaryngol Suppl. 2001; 545: