evaluation, diagnosis and management of...
TRANSCRIPT
American Academy of Otolaryngology – Head & Neck Surgery Annual Meeting September 21, 2014
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
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
Bony Vestibule
20
Beginning of brain myelination
Membranous labyrinthe
Birth
Vestibular nerve myelination
32
Vestibular receptors fully responsive (can elicit Moro reflex)
Vestibular Responsiveness
Age (months)
2 6
VOR Response
teens
Smooth pursuit VOR Suppression
9
Reliable Caloric Responses
Age (years)
5 2
VOR Response Mature
Absence of VOR by age 10 months is abnormal Infants < 6 months cannot suppress their VOR
History
#1. What does it feel like?
sense of motion or
lightheadedness/presyncope/syncope
aura
Is this vertigo?
Anxiety / migraine
Orthostasis in teenage girls
Temporal lobe seizure
#2. How long does it last?
seconds-minutes
hours
days-weeks
BPPV
TIA / migraine Meniere’s
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
change in pressure/straining
head trauma
BPPV
PLF / hydrops / SCD
EVA
History
#6. What makes it worse?
moving or keeping still
rolling over in bed
Vertigo always worse with movement
rolling over = BPPV
History
#7. What else happens at the same time?
tinnitus,fullness,hearing loss
dysarthria,diplopia,paresthesia
cranial nerve weakness
headache, paroxysmal torticollis
sweating,palps,dyspnea,chest tightness
Hydrops
VBI
Skull base / intracranial lesion
Migraine / BRVC
Panic / orthostasis
History
#8. What is the background history?
otologic disease
-SNHL (syndromic / non-syndromic) / ototoxic medications
-vascular disease (congenital
cardiopulmonary disease, von Hippel- Lindau)
-FH neoplasms (NF-2, Gorlin’s
syndrome, Costello syndrome etc.)
Labyrinthitis / fistula / BPPV
Congenital / Acquired vest. Hypofunction
Intracranial vascular lesion (hemangioblastoma)
Cerebellar lesions (NF, medulloblastoma)
History
#8. What is the background history?
Anxiety / depression
Motion intolerance
FH balance disorders
Autoimmune disease
Seizure history
Ophthalmologic disease
Panic attacks
Migraine
Periodic ataxia, migraine, hereditary vestibulopathy
Autoimmune inner ear disease
Temporal lobe seizures
Oculomotor anomaly, amblyopia, acuity, depth perception
History
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 Tragal Compression (fistula sign) l CN II-XII l Motor Power Upper/Lower Limbs l Dysdiadokinesia (rapid alternating
movements) l Dysmetria (pass pointing) l VOR Suppression l EOM (spontaneous/gaze nystagmus) l Saccadic eye movements l Vergence/fixation l Head thrust testing l Post head shake nystagmus
l Per/Post Rotatory nystagmus l Dynamic Visual Acuity l Rhomberg (modified) l Unterberger (Fukuda Stepping test) l Static/Dynamic Balance (BOT-2, one
foot standing, running) l Dix-Hallpike l Hyperventilation
Audiometric Assessment (reflexes, SRS)
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 l Peabody Motor Subset (1 – 7 years)
l Gross Motor Scale l Fine Motor Scale
l Bruininks-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.
One Leg Standing Eyes Closed
One Leg Standing Eyes Open
One Leg Standing
Sensitivity Specificity
Eyes Open < 8 seconds
100% 49%
Eyes Closed < 4 seconds
90% 84%
Balance Assessment The Bare Minimum
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
l Low-frequency (0.01 Hz) l Assess laterality
Making it Child-Proof } VNG vs. ENG
} Air vs. Water Calorics } 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 l N1 = 23 ms
12.6
11.7
18.7
19.0
50 µV
RIGHT
LEFT
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
Saccular Function - cVEMP (Vestibular Evoked Myogenic Potential)
cVEMP (Making it Child-Proof)
l Latency differences by age
l Active response l Video feedback
Latency Range (ms)
P1 N1
8.3 – 14.4 14.8 – 21.9
Utricular Function
Ocular VEMP (oVemp)
cervical VEMP ocular VEMP
saccule inferior vestibular nerve
ipsi sternocleidomastoid
utricle superior vestibular
nerve 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
Vestibular and Balance Disorders in Deafness
Kids who don’t know they are dizzy
Differential Diagnosis: Dizziness with hearing loss
l Acute onset l Viral neuroni/s/labyrinthi/s l Autoimmune disease l Cogan’s syndrome l EVA, other anatomical variants
l O//s media l Labyrinthine concussion
with or without fracture l Lyme Disease l Whiplash
Differential Diagnosis: Dizziness with hearing loss
l Subacute/chronic onset
l HIV, Lyme disease l Syphilis l O//s media l Usher Syndrome l Schwannoma
l Meniere’s Disease/Endolympha/c Hydrops l Ototoxicity l Perilympha/c fistula l Superior semicircular canal dehiscence l EVA
l 232 children
l representative etiology
Unknown 42%
Abnormal Cochlea
21%
Meningitis 7% Other
13%
Connexin 26
17%
Deafness and Vestibular Function
l 232 children
l representative etiology
Unknown 42%
Abnormal Cochlea
21%
Meningitis 7% Other
13%
Connexin 26
17%
Deafness and Vestibular Function
70% have dysfunction
20-40% complete areflexia
* p= 0.0001
8
9
10
11
12
13
14
15
16
norms implant
BO
T-2
Scal
e Sc
ore
Deafness and Balance
* p= 0.0001
8
9
10
11
12
13
14
15
16
norms implant
BO
T-2
Scal
e Sc
ore
Mean 4.4 years
Deafness and Balance
MEAN
STANDARDIZED NORMS
All Unknown Cx-26 Meningitis Abnormal Cochlea Usher’s Other 0
5
10
15
20
25
BOT-‐2 Ba
lance Subset Scale Score
Impact of Etiology on Balance
There are Consequences
vestibular & balance dysfunction risk of CI failure α
Bilateral Areflexia
Odds ratio: 7.6
Implant failure
Vestibular and Balance Disorders
Kids who know they are dizzy
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 l Caloric – no response to ice water l cVEMP – absent l Rotary Chair with scleral coil – reduced gain
Case Presentation 1
Diagnosis – Bilateral Vestibular Loss – ototoxicity
l gentamycin induced vestibulotoxicity
l genetic work-up negative l mutations in mitochondrial RNA (MTRNR1/ MTTS)
Fishel-Ghodsian et al, 1997;Gardner et al, 1997
l vestibular 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
