Pediatric Vestibular Assessment for Children Who Are Deaf or Hard of Hearing

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1 Kristen Janky, Au.D., Ph.D., CCC-A Vestibular Audiologist Vestibular Services, Clinical Coordinator Pediatric Vestibular Assessment for Children Who Are Deaf or Hard of Hearing Kristen Janky Au.D., Ph.D., CCC-A Coordinator, Vestibular Services Director, Clinical Vestibular Laboratory 1

2 Questions? For any questions during this broadcast, please send an to: Questions will be answered at the end of the presentation. Learner Objectives At the conclusion of this presentation, the participant will be able to: Describe the signs and symptoms associated with vestibular dysfunction List the etiologies of hearing loss that are commonly associated with vestibular dysfunction List commonly used vestibular assessments What Is The Vestibular System? How do we maintain balance? Somatosensation Visual Vestibular System 2

3 What Is The Vestibular System? What Is The Vestibular System? What Is The Vestibular System? Vestibulo-spinal Reflex (VSR): helps to maintain head and postural stability Vestibulo-collic Reflex (VCR): Righting reflex, stabilize the head Vestibulo-ocular Reflex (VOR): stabilize vision during head movement All reflexes originate in the vestibular system and are modulate by the brain 3

4 Vestibulo-Ocular Reflex (VOR) Vestibulo-Ocular Reflex (VOR) Vestibulo-Ocular Reflex (VOR) 4

5 Vestibulo-Ocular Reflex (VOR) The vestibular balance system is primarily responsible for maintaining steady and clear vision during head movement Implications: overall balance as well as reading, writing, and development What Is Oscillopsia? Visual phenomenon in which the visual environment appears to oscillate Oscillopsia only occurs in response to head movement Subsequently, oscillopsia is associated with poor visual acuity Associated with vestibular loss in both ears What Is Oscillopsia? 5

6 Vestibular Loss - Adults Adults Imbalance Gait abnormalities (wide-based, slow) Greater disability when visual and somatosensory cues are not present Greater risk of falling Blurred vision with movement (i.e., walking, head turns, avoidance of driving, etc.) Limit social interactions Vestibular Loss - Adults Treatment: Physical therapy Compensatory eye movements to help improve gaze stability Improve overall balance ability Functional effects are not well understood When vestibular loss occurs congenitally or during early development, it can effect motor development When looked at over time, this delay was found to be progressive (Rine et al., 2000) 6

7 Specific problematic activities: Sitting unsupported Standing Walking Navigating in the dark Clumsiness Riding a bike Hopping Children with sensorineural hearing loss and bilateral vestibular loss found to have significantly worse visual acuity during head movement (Rine & Braswell, 2003) Visual acuity found to have a relationship with reading acuity Children need larger print size for comfortable reading (Braswell & Rine, 2006a & 2006b) 7

8 Specific problematic activities: Reading (skipping words or letters) Schoolwork Develop fine and gross motor control Age of onset: Congenital demonstrate more deficits Degree of impairment: Valente et al report on two children: one with mild and the other with profound vestibular loss, both exhibiting functional deficits Other variables In spite functional consequences, not all facilities perform vestibular testing Awareness of risk factors and symptoms 8

9 The incidence of vestibular dysfunction estimated between 30 91% (Shinjo et al., 2007; Tribukait et al., 2004; Zhou et al., 2009) Incidence higher as severity of hearing loss increases (Angeli et al., 2003) Post-natally acquired cases Meningitis Cytomeglovirus (CMV) Ototoxicity Rubella Anoxia Some syndromic deafness Usher s Waardenburg Pendred Alport s Labyrinthine dysplasia Auditory Neuropathy (some cases) 9

10 Secondary to Cochlear Implantation General anesthesia and/or temporary disturbance of inner ear fluids (Licameli et al., 2009) Secondary to cochlear implantation Range incidence of vestibular dysfunction following implantation Estimate vestibular dysfunction in 40-86% following CI (Licameli et al., 2009; Vibert et al., 2001, Krause et al., 2012, Melvin et al., 2009, Jin et al., 2006) Demonstrate developmental delay 10

11 Signs And Symptoms Signs and symptoms associated with vestibular dysfunction (Cronin & Rine, 2010) Dizziness Nystagmus Signs And Symptoms Signs and symptoms associated with vestibular dysfunction (Cronin & Rine, 2010) Difficult with visual acuity Difficulty with spatial relationships Presence of Hearing loss Motion sickness or sensitivity (avoids or craves) Headaches (family history of migraine headaches) Developmental and reflex delays (slow to learn to ride a bike, swim, hop, stair climb, etc) Difficulty with dancing or certain sports Diagnosing Vestibular Loss Caloric Testing Rotary Chair Testing Vestibular Evoked Myogenic Potential (VEMP) Testing Dynamic Visual Acuity (DVA) testing 11

12 Caloric Testing Right ear calorics Caloric Testing Left ear calorics Rotary Chair Children < 5 years When bilateral vestibular loss suspected Chair oscillates back & forth Electrodes (infrared camera) or goggles Sing Songs 12

13 Rotary Chair Rotary Chair VEMP Vestibular Evoked Myogenic Potential (VEMP) Electromyogram: measuring modulation of muscle activity A good alternative to caloric testing which is not generally tolerated in the pediatric population Ear specific information, as opposed to rotary chair 13

14 Ocular VEMP Ocular VEMP Ocular VEMP Utricle and superior branch of the vestibular nerve 14

15 Cervical VEMP Cervical VEMP Saccule and inferior branch of the vestibular nerve Dynamic Visual Acuity How clear is vision during head movement? Rine & Braswell (2003) developed DVA for ages % sensitivity and specificity for identifying bilateral weakness (Rine & Braswell, 2003) 15

16 Referring For Vestibular Testing When to refer: Hearing Loss High risk population Signs and Symptoms Developmental Delay Pre and Post cochlear implantation Suspect vestibular loss Parent Concern Treatment When vestibular loss is diagnosed, then what? Visual System Assessment Somatosensory System Assessment Vestibular Rehabilitation Vestibular Rehabilitation Preliminary studies demonstrate that children with vestibular loss improve: Developmental Milestones (Rine et al., 2004) Progressive delay was halted and performance moved into the normal range with therapy (Rine et al., 2004) Critical print size and reading acuity (Braswell & Rine, 2006) 16

17 Vestibular Rehabilitation Vestibular loss is treated with gaze stabilization exercises - ability to stabilize the visual environment during movement Repeated head and body movements both with and without visual targets Full range of head movement Varying speed, repetitions and sets to build endurance Vestibular Rehabilitation In children, incorporate imagination in play Activities with a ball Finger painting TV and video games Again increasing speed, repetitions, and complexity of environment Vestibular Rehabilitation Balance Systems Integration Integrating Visual, Vestibular, and Somatosensory Challenging the systems to optimize benefit Setting up home and school environment Compensation 17

18 Other Recommendations Night lights Scuba Diving Swimming Sports Child play: learning to ride a bike, roller/ice skate Summary Primary purpose of the vestibular balance center is to maintain steady vision during head movement If vestibular loss is suspected, vestibular testing can be completed Summary If vestibular loss is diagnosed, appropriate therapy should be initiated Vestibular rehabilitation can improve both developmental milestone delay and visual acuity 18

19 References Braswell J, Rine RM. Preliminary evidence of improved gaze stability following exercise in two children with vestibular hypofunction. Int J Pediatr Otorhinolaryngol 2006;70: Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading acuity in children. Int J Pediatr Otorhinolaryngol 2006;70: Cronin, GW, Rine, RM (2010). Pediatric vestibular disorders: Recognition, evaluation, and treatment. VEDA. Herdman SJ (2007). Vestibular Rehabilitation 2 nd Ed. F.A. Davis Co., Philadelphia. Jin Y, Nakamura M, Shinjo Y et al. Vestibular-evoked myogenic potentials in cochlear implant children. Acta Otolaryngol 2006;126: References Krause E, Louza JP, Wechtenbruch J et al. Influence of cochlear implantation on peripheral vestibular receptor function. Otolaryngol Head Neck Surg 2010;142: Licameli G, Zhou G, Kenna MA. Disturbance of vestibular function attributable to cochlear implantation in children. Laryngoscope 2009;119: Melvin TA, Della Santina CC, Carey JP et al. The effects of cochlear implantation on vestibular function. Otol Neurotol 2009;30: O Reilly, RC, Grindle, C, Zwicky, EF, Morlet, T (2011). Development of the vestibular system and balance function: Differential diagnosis in the pediatric population. Otolaryngol Clin N Amer, 44 (2): References O Reilly, RC, Morlet, T, Nicholas, BD, Josephson, G, Horlbeck, D, Lundy, L, Mercado, A (2010). Prevalence of vestibular and balance disorders in children. Otol Neurotol, 31: Rine, RM (2009). Growing evidence for balance and vestibular problems in children. Audiological Med, 1-5. Rine RM, Braswell J, Fisher D et al. Improvement of motor development and postural control following intervention in children with sensorineural hearing loss and vestibular impairment. Int J Pediatr Otorhinolaryngol 2004;68: Rine RM, Braswell J. A clinical test of dynamic visual acuity for children. Int J Pediatr Otorhinolaryngol 2003;67:

20 References Rine RM, Cornwall G, Gan K et al. Evidence of progressive delay of motor development in children with sensorineural hearing loss and concurrent vestibular dysfunction. Percept Mot Skills 2000;90: Shinjo Y, Jin Y, Kaga K. Assessment of vestibular function of infants and children with congenital and acquired deafness using the icewater caloric test, rotational chair test and vestibular-evoked myogenic potential recording. Acta Otolaryngol 2007;127: Tribukait A, Brantberg K, Bergenius J. Function of semicircular canals, utricles and saccules in deaf children. Acta Otolaryngol 2004;124: References Valente, LM (2011). Assessment techniques for vestibular evaluation in pediatric patients. Otolaryngol Clin N Amer, 44 (2), Valente LM, Goebel JA, Sinks B. Pediatric vestibular evaluation: two children with sensorineural hearing loss. J Am Acad Audiol 2012;23: Vibert D, Hausler R, Kompis M et al. Vestibular function in patients with cochlear implantation. Acta Otolaryngol Suppl 2001;545: Zhou G, Kenna MA, Stevens K et al. Assessment of saccular function in children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg 2009;135: Kristen Janky 555 North 30 th Street Omaha, Nebraska

21 A production of the Lied Learning & Technology Center at

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