Cochlear Implant Research Team. Sharon L. Cushing. Outline. Moving Targets: Vestibular and Balance Dysfunction in Children

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1 Moving Targets: Vestibular and Balance Dysfunction in Children Sharon L. Cushing Mayo Clinic 28 th Annual Audiology Conference. Rochester Minnesota, February 3, 218 Cochlear Implant Research Team DIRECTORS l Blake Papsin l Karen Gordon l Vicky Papaioannou RESEARCH ASSISTANTS l Stephanie Jewell STUDENTS l Daniel Wong l Claire Salloum l Sho Tanaka l Patrick Yu l Brad Hubbard l Lauren Schofield l Brittany Harrison l Brooke Allemang FELLOWS l Talar Hopyan l Neil Chadha l James Ramsden COLLABORATORS Local - SickKids l Bob Harrison l Tracy Stockley l Susan Blaser l Adrian James l Sharon Cushing l Paolo Campisi l Mark Crawford l Maureen Dennis l Gina Sohn l Naureen Sohail l Laurie MacDonald l Mary Lynn Feness l Pat Di Santos l Nancy Greenwald- Hood l Susan Druker Local - external l Sandra Trehub l Frank Russo International l Robert Cowan l Richard van Hoesel Outline l vestibular & balance dysfunction l common in SNHL l screening assessment l impact of dysfunction l implants and the impaired vestibular system 1

2 Vestibular dysfunction is common in children with SNHL Otol Neurotol. 213 Vestibular dysfunction is common in children with SNHL 7% dysfunction 35-4% complete areflexia Otol Neurotol. 213 Vestibular dysfunction is common in children with SNHL 7% dysfunction 35-4% complete areflexia vestibular impairment is the single most common associated feature of SNHL Otol Neurotol

3 Measuring Balance Bruininks-Oseretsky (BOT2) Balance Subtest Balance subtest Items Max. Score 1. Standing with feet apart on a line Eyes Open 1 sec. Eyes Closed 1 sec. 2. Walking forward on a line 6 steps 3. Standing on one leg on a line Eyes Open 1 sec. Eyes Closed 1 sec. 4. Walking forward heel to toe on a line 6 steps 5. Standing on one leg on a balance beam Eyes Open 1 sec. Eyes Closed 1 sec. 6. Standing heel-to-toe on a balance beam 1 sec. N= 19 children with CI tested Vestibular loss impacts balance * p=.1 BOT-2 Scale Score Bilateral Vestibular Loss (BVL) 2 normal hearing hearing loss BVL 3

4 Vestibular loss impacts balance * p=.1 BOT-2 Scale Score Bilateral Vestibular Loss (BVL) 2 normal hearing hearing loss BVL Vestibular loss impacts balance * p=.1 BOT-2 Scale Score Bilateral Vestibular Loss (BVL) Mean 4.4 years normal hearing hearing loss BVL Vestibular loss impacts balance balance deficits are not always apparent 4

5 Screening vestibular & balance function 4 easy steps 1. motor milestones 2. motor function 3. horizontal canal 4. otoliths Motor milestones upper limits l head control l 4 months l sitting l 9 months l walking l 18 months 5

6 Screening vestibular & balance function 4 easy steps 1. motor milestones 2. motor function 3. horizontal canal 4. otoliths One leg standing Age Duration (sec) 1 foot standing 3 months 1 (briefly) 36 months 2 4 years 5 5 years 1 One leg standing One Leg Standing Eyes Closed One Leg Standing Eyes Open Otol Neurotol

7 One leg standing One Leg Standing Eyes Closed One Leg Standing Eyes Open One Leg Standing Children age 5 and older Eyes Open < 8 seconds Eyes Closed < 4 seconds Sensitivity Specificity 1% 49% 9% 84% Otol Neurotol. 216 Screening vestibular & balance function 4 easy steps 1. motor milestones 2. motor function 3. horizontal canal 4. otoliths Assess Horizontal Canal Function 7

8 Assess horizontal canal function l vhit l objective l goggle based l goggle free video Head impulse testing (vhit) (EyeSeeCam, Interacoustics) video Head impulse testing (vhit) Synapsys 8

9 Infants < 6 months cannot visually suppress the VOR Screening vestibular & balance function 4 easy steps 1. motor milestones 2. motor function 3. horizontal canal 4. otoliths Subjective Visual Vertical (SVV) -2 o +2 o l Positive à tilt to the right l Negative à tilt to the left l Normal range: ± 2 (Brodsky 215, 216) l Bias àinitial direction of linear marker (Pagarkar 28; Toupet 215) 9

10 Subjective Visual Vertical Chronos System, Interacoustics Screening vestibular & balance dysfunction 4 easy steps 1. motor milestones 2. motor function 3. horizontal canal 4. otoliths Vestibular & balance dysfunction risk by etiology Cochleovestibular anomalies Phelps Waardenburg Klippel Feil Pendred Ototoxicity Usher Syndrome Meningitis CMV ANSD Connexin Mitochondrial Autoimmune 1

11 Vestibular & balance dysfunction risk by etiology Cochleovestibular anomalies Phelps Waardenburg Klippel Feil Pendred Ototoxicity Usher Syndrome Meningitis CMV ANSD Connexin Mitochondrial Autoimmune Ototoxicity in Cancer cochleotoxicity vestibulotoxicity 25-9%? platinum based therapy aminoglycosides cranial radiation surgical therapy Ototoxicity Related Hearing Loss Left Ear Right Ear Frequency (Hz) Frequency (Hz) Level (db HL) n=26 Conductive hearing loss Cochlear implant 11

12 Ototoxicity Related Balance Impairment seconds par+cipants n=26 Ototoxicity Related Balance Impairment seconds Eyes Open par+cipants n=26 Ototoxicity Related Balance Impairment seconds Eyes Open <8 sec par+cipants 35% (8/23) abnormal n=26 12

13 Ototoxicity Related Balance Impairment seconds Eyes Open Eyes Closed <8 sec par+cipants 35% (8/23) abnormal n=26 Ototoxicity Related Balance Impairment seconds Eyes Open Eyes Closed <8 sec <4 sec par+cipants 35% (8/23) abnormal 65% 15/23 abnormal n=26 Vestibular Impairment from Ototoxicity Horizontal Canal Dysfunction 5% Dynamic Visual Acuity 52% VOR loss vhit 42% Utricular Dysfunction Subjective Visual Vertical N = 26 13

14 Ototoxicity in Cancer cochleotoxicity vestibulotoxicity 25-9% 5% platinum based therapy aminoglycosides cranial radiation surgical therapy Congenital Cytomegalovirus l poorer motor performance l 5% absent cvemp l 92% vestibular impairment l 33% complete bilateral loss l 5% progression l? association with ccmv related SNHL l? denominator l dual injury Maes, Ear & Hearing. 216 Bernard et al. Pediatrics. 215 Balance is Impaired in Unilateral SNHL BOT-2 Scaled Score P=.4 Unilateral Hearing Unilateral Loss Normal Hearing Normal Otol Neurotol

15 Balance is Impaired in Unilateral SNHL BOT-2 Scaled Score P=.4 hearing loss Unilateral Hearing Unilateral Loss Normal Hearing Normal Otol Neurotol. 216 Balance is Impaired in Unilateral SNHL BOT-2 Scaled Score Unilateral Hearing Unilateral Loss P=.4 Normal Hearing Normal hearing loss bilateral vestibular loss Otol Neurotol. 216 Balance abilities deteriorate from normal as task difficulty increases Time to fall (s) Normal hearing Unilateral hearing loss * * Increasing difficulty 15

16 Balance abilities deteriorate from normal as task difficulty increases Time to fall (s) Normal hearing Unilateral hearing loss * * Increasing difficulty Children with unilateral SNHL depend on vision for postural control Normal Hearing Unilateral hearing loss 1 75 Source p vision.1 vision * group.2 COP rms 5 25 Task eyes 3 Task eyes 6 open closed Vestibular Dysfunction in Children with Unilateral SNHL Horizontal Canal Dysfunction 48% Caloric Reduction 5% VOR loss vhit Otolithic Dysfunction 62.5% Utricular 42% ovemp Saccular 25% cvemp N = 2 16

17 Learning happens in the Classroom Life happens on the Playground Balance and Navigation Spatial Information Balance and Navigation Proprioception Vision Efference Copy Spatial Knowledge Hearing Kinesthetic Input Vestibular Disclosures (cont'd) Everything I have learned about brain development in children I have learned from 2 people Dr. Karen Gordon Xavier Sloan 17

18 Auditory Deprivation Reduced activity Activity no longer reduced Lee, et al., Nature, 21 Sensory Assembly Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation Sensory Assembly Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation 18

19 Sensory Assembly Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation "Ice Cream, yummy!" Sensory Assembly - Cochlear Loss Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation Spatial Information Balance and Navigation Proprioception Vision Efference Copy Spatial Knowledge Hearing Kinesthetic Input Vestibular 19

20 Consequences of Unilateral SNHL Quality of Life Speech & Language Grade Failure Unilateral Hearing Loss School Performance Behaviour IEP & Speech Therapy Lieu et al.. Laryngoscope. 212 Spatial Information Balance and Navigation Proprioception Vision Efference Copy Spatial Knowledge Hearing Kinesthetic Input Vestibular Balance is Impaired in Developmental Visual Anomalies BOT2 Balance Score P = <.1 P = <.1 Control Strabismus Amblyopia Bachar et al in preparation 2

21 Balance is Impaired in Developmental Visual Anomalies BOT2 Balance Score P = <.1 P = <.1 Control Strabismus Amblyopia hearing loss bilateral vestibular loss Bachar et al in preparation Spatial Information Balance and Navigation Proprioception Vision Efference Copy Spatial Knowledge Hearing Kinesthetic Input Vestibular Cortical Vestibular Projections Lopez & Blanke (211) after Sugiuchi et al. (25) 21

22 Sensory Assembly - Vestibular Loss Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation Sensory Assembly Vestibular Loss effort Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation 22

23 Sensory Assembly Vestibular Loss effort Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation Vestibular effects of cochlear implantation (positive) 6 * p=.29 Balance score (BOT-2) CI off CI on

24 Sensory Assembly Restored? effort Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation Hypothesis l Electrical current from a cochlear implant can spread to the vestibular system VEMP Waveforms (same ear) acoustic electric P 1 P 1 N1 N1 Laryngoscope

25 Electrically-Evoked VEMPs are Faster P1 and N1 peak latencies of cervical VEMPs N1 and P1 peak latencies of ocular VEMPs p<.1, paired t-test Vestibular abnormalities cause functional deficits by Stephanie Jewell Electrical Stimulation Shifts the Perception of Vertical Back to Center Leaning right p<.1 Leaning right 25

26 Sensory Assembly Restored? effort Senses Processing Reassembly stimulus high fidelity representation of the environment pattern recognition faithful representation TransCochlear Stabilization of Balance l accelerometer and gyroscopic sensor l conversion to CI stimulation l head referenced l deviation signalled l transferred electrically to implant l 16 patients OFF ON 26

27 Hearing and Implants Help Balance P =.41 9 Mean Scaled BOT Score CI off Head referenced CI stimulation Time to Fall Approaches Normal 12 1 Max time Time to Fall (s) NORMAL HEARING 2 Tandem Eyes One Foot Eyes BB - One Foot BB - Tandem Tandem Eyes One Foot Eyes BB - One Foot open Open Eyes Open Eyes Open closed Closed Eyes Closed Time to Fall Approaches Normal 12 1 Max time Time to Fall (s) CI NORMAL HEARING 2 Tandem Eyes One Foot Eyes BB - One Foot BB - Tandem Tandem Eyes One Foot Eyes BB - One Foot open Open Eyes Open Eyes Open closed Closed Eyes Closed 27

28 Time to Fall Approaches Normal 12 1 Max time Time to Fall (s) Tandem Eyes open One Foot Eyes Open CI BB - One Foot Eyes Open BB - Tandem Eyes Open Tandem Eyes closed NORMAL HEARING One Foot Eyes Closed BB - One Foot Eyes Closed HEAD REFERENCED CI STIMULATION Take home messages l vestibular & balance dysfunction l common in SNHL l 4 easy steps for balance screening l vestibular & balance dysfunction matters l implants can stimulate an impaired vestibular system 28

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