Evaluation, Diagnosis and Management of Vestibular and Balance Dysfunction in Children
Sharon L. Cushing Robert C. O’Reilly
American Academy of Otolaryngology – Head & Neck Surgery Annual Meeting September 21, 2014 http://www.orlped.com
AAOHNS Instructional Course Materials
Introduction
1. comprehensive evaluation
2. screening for vestibular & balance function
3. clinical populations and cases Comprehensive Evaluation of Vestibular and Balance Function Embryology of the Inner Ear
Week Gestation
10 12 20 32 Birth
Bony Vestibule
Membranous labyrinthe Beginning of brain myelination
Vestibular receptors fully responsive (can elicit Moro reflex)
Vestibular nerve myelination Vestibular Responsiveness
Age (months) Age (years)
Reliable Caloric Responses
2 6 9 2 5 teens
VOR Response VOR Response Mature
VOR Suppression Smooth pursuit Infants < 6 months cannot suppress their VOR Absence of VOR by age 10 months is abnormal History
#1. What does it feel like? Is this vertigo?
sense of motion Anxiety / migraine
or lightheadedness/presyncope/syncope Orthostasis in teenage girls
aura Temporal lobe seizure History
#2. How long does it last?
seconds-minutes BPPV
TIA / migraine hours Meniere’s
days-weeks Labyrinthitis / VN History
#3. How many attacks of vertigo have there been?
One prolonged: VN, labyrinthitis, infarct
Several: Meniere’s, TIA, migraine
Many: BPPV History
#5. What sets it off?
position change BPPV
change in pressure/straining PLF / hydrops / SCD
head trauma EVA History
#6. What makes it worse?
moving or keeping still Vertigo always worse with movement
rolling over in bed rolling over = BPPV History #7. What else happens at the same time?
tinnitus,fullness,hearing loss Hydrops
dysarthria,diplopia,paresthesia VBI
cranial nerve weakness Skull base / intracranial lesion
headache, paroxysmal torticollis Migraine / BRVC
sweating,palps,dyspnea,chest tightness Panic / orthostasis History
#8. What is the background history?
otologic disease Labyrinthitis / fistula / BPPV
-SNHL (syndromic / non-syndromic) / ototoxic medications Congenital / Acquired vest. Hypofunction
-vascular disease (congenital Intracranial vascular lesion (hemangioblastoma) cardiopulmonary disease, von Hippel- Lindau)
-FH neoplasms (NF-2, Gorlin’s Cerebellar lesions (NF, medulloblastoma) syndrome, Costello syndrome etc.)
History
#8. What is the background history?
Anxiety / depression Panic attacks
Motion intolerance Migraine
FH balance disorders Periodic ataxia, migraine, hereditary vestibulopathy
Autoimmune disease Autoimmune inner ear disease
Seizure history Temporal lobe seizures
Ophthalmologic disease Oculomotor anomaly, amblyopia, acuity, depth perception Physical Exam l static testing l spontaneous and gaze evoked nystagmus l hyperventilation l valsalva l tragal compression l dynamic testing l Dix-hallpike maneuver l head thrust maneuver l post- headshake nystagmus l dynamic visual acuity l per-rotatory nystagmus l VOR suppression l vestibulospinal testing l pastpointing l fukuda stepping test l romberg l gait/one foot standing Pediatric Physical Exam l Head and Neck Exam l Per/Post Rotatory nystagmus l Tragal Compression (fistula sign) l Dynamic Visual Acuity l CN II-XII l Rhomberg (modified) l Motor Power Upper/Lower Limbs l Unterberger (Fukuda Stepping test) l Dysdiadokinesia (rapid alternating l Static/Dynamic Balance (BOT-2, one movements) foot standing, running) l Dysmetria (pass pointing) l Dix-Hallpike l VOR Suppression l Hyperventilation l EOM (spontaneous/gaze nystagmus) l Saccadic eye movements l Vergence/fixation Audiometric Assessment l Head thrust testing (reflexes, SRS) l Post head shake nystagmus Vestibular end-organ testing Head thrust testing
aka
Halmagyi test
aka
Head impulse test (HIT)
VOR SUPPRESSION FUKUDA STEP TEST
AKA
UNTERBERGER TEST
DYNAMIC VISUAL ACUITY TESTING
PER-ROTATORY NYSTAGMUS AND POST-ROTATORY NYSTAGMUS HEAD SHAKE NYSTAGMUS
Balance Assessment
Expensive & Complicated vs. Cheap & Easy Gait Analysis l Dynamic balance kinetics and kinematics l Position / movement CM walking straight line (60 Hz data collection) l Self selected speed for 9 meters Computerized Dynamic Posturography
l Limits of stability using 8 standard trials l Reaction time, movement velocity, endpoint excursion, max excursion,directional control, measures of sway Balance Assessment Cheap & Easy Subjective
l Rhomberg (tandem Rhomberg)
l 1 foot standing (eyes open/closed)
l run around the clinic
Balance Assessment Cheap & Easy Subjective
l Rhomberg (tandem Rhomberg)
l 1 foot standing (eyes open/closed)
l run around the clinic
Balance Assessment Cheap & Easy More objective lPeabody Motor Subset (1 – 7 years) l Gross Motor Scale l Fine Motor Scale lBruininks-Oseretsky (4 – 21 years) Test of Motor Proficiency
Measuring Balance Bruininks-Oseretsky (BOT2) Balance Subtest
Balance subtest Items Max. Score 1. Standing with feet apart on a line Eyes Open 10 sec. Eyes Closed 10 sec. 2. Walking forward on a line 6 steps 3. Standing on one leg on a line Eyes Open 10 sec. Eyes Closed 10 sec. 4. Walking forward heel to toe on a line 6 steps 5. Standing on one leg on a balance beam Eyes Open 10 sec. Eyes Closed 10 sec. 6. Standing heel-to-toe on a balance beam 10 sec. Balance Assessment The Bare Minimum
One Leg Standing Eyes Closed One Leg Sensitivity Specificity Standing Eyes Open 100% 49% < 8 seconds Eyes Closed 90% 84% < 4 seconds One Leg Standing Eyes Open Vestibular end-organ testing
l lateral canal function
l caloric l rotational chair l low and high frequency (0.5 to 5 Hz) l video head impulse test goggles
l saccular function l cervical vestibular evoked myogenic potentials (VEMP) l utricular function l ocular VEMP
Lateral Canal Function Lateral Canal Function - Caloric
Bithermal Calorics Making it Child-Proof l Low-frequency (0.01 Hz) } VNG vs. ENG } Air vs. Water Calorics l Assess laterality } Adequate alerting } Wax and middle ear fluid } Kids enjoy being dizzy } Appropriate targets VNG / ENG l Other measures l spontaneous, positional, positioning nystagmus l smooth pursuit tracking l saccadic eye movements l optokinetic nystagmus
Lateral Canal Function - Rotation
l Low to high frequency (0.1 to 15.0 Hz) l Higher frequency than calorics (0.01 Hz) l Physiologic (head/body rotation) l Phase, Gain and Symmetry of the VOR l Limited assessment of laterality l Subject to compensation Rotational Chair Testing Making it Child-Proof
l Better tolerated than calorics l Small children/infants sit in parents lap/car seat l Use VNG vs. ENG vs. Scleral Coil Lateral Canal Function vHIT testing l high frequency horizontal canal function l assesses laterality l alternative to rotary testing Lateral Canal Function vHIT testing
Saccular Function Cervical VEMP (cVemp) Saccular Function - cVEMP (Vestibular Evoked Myogenic Potential)
l Myogenic response of Sternocleidomastoid m. l Tests saccule & inferior vestibular nerve
l P1 = 13 ms RIGHT 12.6 l N1 = 23 ms 50 µV LEFT 11.7 18 .7
19.0 Saccular Function - cVEMP (Vestibular Evoked Myogenic Potential)
l 500 Hz tone burst (75-95 dB HL air or 66 dB pip bone) l Tonic SCM contraction = EMG 50-250 microvolts l 80-100 samples averaged cVEMP (Making it Child-Proof) l Latency differences by age
Latency Range (ms) P1 N1 8.3 – 14.4 14.8 – 21.9 l Active response l Video feedback
Utricular Function Ocular VEMP (oVemp) cervical VEMP ocular VEMP
saccule utricle inferior vestibular nerve superior vestibular ipsi nerve sternocleidomastoid contra inferior oblique Utricular Function l Ocular Vemp (oVemp) l Subjective Visual Vertical Easy pediatric vestibular screen
1. motor milestones
2. standing on one foot
3. head thrust testing 1. Motor Milestones Red Flags 2. Dynamic balance : 1 foot standing
Age Duration (sec) 1 foot standing 30 months 1 (briefly) 36 months 2 4 years 5 5 years 10 3. Lateral Canal Function:Head Thrust Testing Easy pediatric vestibular screen
1. motor milestones
2. standing on one foot
3. head thrust testing
Kids who don’t know they are dizzy
Vestibular and Balance Disorders in Deafness
Differential Diagnosis: Dizziness with hearing loss l Acute onset l O s media l Viral neuroni s/labyrinthi s l Labyrinthine concussion l Autoimmune disease with or without fracture l Cogan’s syndrome l Lyme Disease l EVA, other anatomical variants l Whiplash Differential Diagnosis: Dizziness with hearing loss l Subacute/chronic onset l Meniere’s Disease/Endolympha c Hydrops l HIV, Lyme disease l Ototoxicity l Syphilis l Perilympha c fistula l O s media l Superior semicircular canal dehiscence l Usher Syndrome l EVA l Schwannoma Deafness and Vestibular Function l 232 children Meningitis Abnormal 7% Other l representative etiology Cochlea 13% 21%
Connexin 26 17% Unknown 42% Deafness and Vestibular Function l 232 children Meningitis Abnormal 7% Other l representative etiology Cochlea 13% 21%
Connexin 70% have dysfunction 26 20-40% complete areflexia 17% Unknown 42% Deafness and Balance * 16 p= 0.0001
15
14
13
12
11 BOT-2 Scale Score BOT-2 10
9
8 norms implant Deafness and Balance * 16 p= 0.0001
15
14
13
12
11 Mean 4.4 years BOT-2 Scale Score BOT-2 10
9
8 norms implant Impact of Etiology on Balance
25
STANDARDIZED NORMS 20
MEAN 15
10
5 BOT-2 Balance Subset Scale Score
Abnormal 0 All Cx-26 Unknown Cochlea Meningitis Other Usher’s There are Consequences
vestibular & balance risk of CI failure dysfunction α
Bilateral Areflexia
Odds ratio: 7.6
Implant failure
Kids who know they are dizzy
Vestibular and Balance Disorders
Differential Diagnosis: Dizziness without Hearing Loss
l Acute: l Benign Paroxysmal Positional Vertigo (BPPV) l Migraine variants l Postural orthostatic tachycardia syndrome (POTS) l Epileptic vertigo (esp. post-traumatic) l Vestibular neuronitis
Differential Diagnosis: Dizziness without Hearing Loss
l Chronic Dizziness l Chiari Malformation l Multiple sclerosis l Ocular abnormalities l Post-concussive l Familial periodic cerebellar ataxia (types 1-7) l Vertebro-basilar insufficiency
Tips in the Diagnosis of Vestibular Migraine in Children l prevalence l 2-10% school aged children l in children with normal otoscopic findings, vertigo is commonly caused by migraine and migraine equivalents: benign paroxysmal vertigo (BPVC) of childhood
Childhood Migraine
Benign Paroxysmal Vertigo of Childhood (BPVC) (Benign Recurrent Vertigo of Childhood (BRVC))
l ICHD-IIR1: “childhood periodic syndromes that are commonly precursors of migraine” l Presents at age 1-4 yrs. l Most common cause of dizziness at this age l Nystagmus,nausea,emesis,diaphoresis,torticollis l Brief duration l Resolution in 1-2 years l “Classic” migraine later in life BPVC Benign Paroxysmal Vertigo of Childhood
Diagnostic Criteria - Basser Criteria (1964)
l sudden brief attacks of vertigo (sec. to min.)
l before school age
l accompanied by
l nystagmus l pallor l nausea l phonophobia l photophobia
Childhood Migraine l Migraine with Aura l 14-30% have headache with aura (more than adults) l Occurs in older children l Often different than adults: l headache bilateral l late afternoon onset l < 2 hour duration. Results l 39/240 (16%) l BPVC (10) l migraine with aura (29) § Diagnosed by Peds Neurology and Neurotology l mean age at testing: 11.3 yrs (± 4.7)
l 23 Female; 16 Male
l 10 BRVC (mean age 4.3) (1 child < 2 years)
l 29 Migraine with Aura (mean age 13.5)
l MRI: all normal Results l Audiometry l 3 abnormal (1 bilateral profound) in migraine group l Tympanometry l 10% abnormal (40% of BRVC): Type C or PE tubes l DPOAEs l 1 abnormal (migraine pt. with profound SNHL) l VEMP l 28 tested: all present and normal l Rotational Chair Testing l 37 tested: all essentially normal (high gain/single frequency asymetry) Results l VNG l 7/28 migraine: “central” (positional nystagmus or oculomotor abnormality) l 5/10 BRVC: normal l Gross Motor Development l 53 % abnormal (40% BRVC/ 58% migraine) l “below average” strength, agility, coordination l Gait l 67% full assessment / 33% videos l 51% abnormal (40% BRVC/ 55% migraine) l Posturography l 35% abnormal l Slow reaction time / increased postural sway l BMI l 35% overweight or obese l 57% OW/OB at least 1 Gait/Gross Motor/CDP abnormality l 86% NW – at least 1 Gait/Gross Motor/CDP abnormality Summary
1. vestibular/balance deficits common in deafness
2. screening for vestibular dysfunction is feasible
3. the differential is broad Cases Case Presentation 1 l 16 year old boy l 8 month history of visual blurring and instability l Began while an inpatient l Treated medically for crohn’s related bowel perforation l 2 week course of ampicillin/gentamycin/flagyl l Unstable / Unable to play usual sports (curling) l No acute vertiginous episodes l No audiologic complaints Case Presentation 1
Clinical Assessment l saccades noted on head thrust (Halmagyi) testing bilaterally l visual acuity testing l 20/10 static visual acuity testing l 20/100 dynamic visual acuity l no evidence of post rotatory nystagmus l dynamic instability with eyes closed Case Presentation 1
Ancillary Testing lCaloric – no response to ice water lcVEMP – absent lRotary Chair with scleral coil – reduced gain Case Presentation 1
Diagnosis – Bilateral Vestibular Loss – ototoxicity lgentamycin induced vestibulotoxicity l genetic work-up negative l mutations in mitochondrial RNA (MTRNR1/ MTTS) Fishel-Ghodsian et al, 1997;Gardner et al, 1997 lvestibular rehabilitation program (physiotherapy) Case Presentation 2 l 2.5 year old girl l 3 month history of episodic vertigo l sudden onset l ‘the house is shaking’ l child would close eyes and want to be held l 1-2 minutes in duration l loss of appetite, occasional emesis l following acute episode child would want to go to bed l paroxysmal torticollis as an infant Case Presentation 2 l frequency: 3-5 episodes / month l initial episodes in head hanging position l subsequent episodes while sitting and running l child began avoiding head hanging position Case Presentation 2 l Clinical Exam l Head and Neck exam WNL l EOM Normal l Halmagyi Normal l Ø Post head shake Nystagmus l Gait / Rhomberg Normal l Dix Hallpike Normal l Dynamic Visual Acuity Normal (LEA Symbols Chart) l Cranial Nerves / Cerebellar Exam normal Case Presentation 2 l Audiometry l Normal PTA l Normal tympanometry and middle ear pressure l ENG Caloric – Warm water – equal l VEMP Normal bilateral l Rotational Chair (0.5 to 5 Hz) Normal gains bilaterally l MRI - Normal
Case Presentation 2 : BPVC Benign Paroxysmal Vertigo of Childhood
Diagnostic Criteria - Basser Criteria (1964)
l sudden brief attacks of vertigo (sec. to min.)
l before school age
l accompanied by
l nystagmus l pallor l nausea l phonophobia l photophobia
Case Presentation 3 l 15 year old boy suffered concussive injury following football tackle l Episodic vertigo l Lasting seconds l While rolling onto right side or looking up and right l Frequency 3 – 4 times per day Case Presentation 3 l Clinical Exam l Head and Neck exam WNL l EOM Normal l Halmagyi Normal l Ø Post head shake Nystagmus l Dynamic Visual Acuity Normal (Sloan Letters Chart) l Gait / Rhomberg Normal l Dix Hallpike – symptomatic vertigo with classic geotropic rotatory nystagmus - right head hanging position l Cranial Nerves / Cerebellar Exam normal Case Presentation 3 l Audiometry l Profound SNHL - Right l Normal tympanometry and middle ear pressure l ENG Caloric – equal l VEMP Normal bilateral l Rotational Chair (0.5 to 5 Hz) Normal gains bilaterally l CT - Normal
Case Presentation 3
DIAGNOSIS – Post-traumatic Benign Paroxysmal Positional Vertigo (BPPV) l TREATMENT l Multiple attempts at repositioning with ongoing recurrence of symptoms over 18 months l Posterior semicircular canal occlusion l No further symptoms http://www.orlped.com
AAOHNS Instructional Course Materials