Pediatric Vestibular Assessment: Thinking Outside the VNG - PowerPoint Presentation

Pediatric Vestibular Assessment: Thinking Outside the VNG Patricia Mazzullo, Au.D ., CCC-A, F-AAA Clinical Audiologist Walter Reed National Military Medical Center Bethesda, MD Adjunct Professor Graduate Center of the City University of New York (CUNY)

Financial Disclosure No financial relationships to disclose

Dizziness in Children Dizziness is not a frequent problem among children Generally only accounts for 1% of visits to balance centers ( Rinna et al., 2005) Li et al. (2016) performed a nationally representative study with over 10,000 children to assess the prevalence of dizziness and balance problems in children Approximately 5% (3.3 million) of children have dizziness and balance

Postural Control in Children The peripheral and central nervous system components function together to convert and regularly update head position and movement information for processing by the central nervous system for the coordination and execution of basic motor reflexes and/or complex movements of the eyes, head, limbs and trunk (Day & Fitzpatrick, 2005) Disruption of the vestibular system (peripheral or central) may result in difficulty with gaze stability and balance impairment Postural control relies on redundant sensory information from vestibular, visual, and somatosensory receptors regarding head and body position and movement ( Rine & Wiener- Vacher , 2013)

Vestibular Dysfunction in Children with Hearing Loss A study performed by Jacot et. al (2009) revealed that approximately 50% of children with bilateral sensorineural hearing loss have vestibular deficits It has been suggested that as severity of hearing loss increases, vestibular loss is more likely to occur ( Brookhouser & Worthington, 1991) A higher prevalence of vestibular loss has been associated with meningitis, Pendred syndrome, CHARGE syndrome, other cochleovestibular abnormalities, CMV, and Connexin-26( Brookhouser , Cyr, & Beauchaine , 1982; Cushing et al., 2013; Janky & Givens, 2015; Yan & Liu, 2019; Yoshimura et al., 2016; Zagolski , 2008) Occurring in nearly all cases of CHARGE and meningitis Varying degrees

Vestibular Loss in Children with Cochlear Implants Approximately 50% of children have vestibular loss prior to cochlear implantation Approximately 10% are at risk for additional vestibular loss directly related to the process of implantation Trauma to the saccule Cochlear implant failure may be directly related to vestibular loss ( Licameli et al., 2009; Jacot et al., 2009; Jin et al., 2006)

Development of Motor Skills 3 months 7 months 9 months 12 months 24 months Raises head and chest when lying on stomach Sits with (following by without) support of hands Crawling on hands and knees Sits without assistance Walks alone by 18 monthsStarts to use eye and hand coordinationSupports weight on legsWalking with assistancePulls self up into standing positionBegins to runBegins to support headSupports head when sittingUpper body – turns from sitting to crawling positionCrawls forward on belly,Creeps on hands & knees & supports trunkCan push a wheeled toyPushes down with legs when feet are placed on the floorRolls overStands momentarily without supportMoves eyes in all directionsAbility to visually track objects improvesWalks holding onto furniture ( Gans , 2012)

Gross Motor Delays Regarding gross motor function, typically developing children sit without support between the age of 3.8 and 9.2 months, crawl between 5.2 and 11.4 months, stand independently between 6.9 and 16.9 months, and walk independently between 8.2 and 17.6 months ( World Health Organization [WHO], 2006 ) Children with vestibular loss are delayed in acquiring these milestones; they may acquire independent head control at 5 months or later and walk independently at 18 months or later (Inoue et al., 2013; Janky & Givens, 2015) Be mindful of delays in gross motor skills, and think of what could be going on to delay these milestones

Causes of Pediatric Vertigo/Dizziness Migraines* Most common cause of vertigo in children (may account for approximately 25%) Benign Paroxysmal Vertigo (BPV) of Childhood* May account for approximately 20% of vertigo in children Head trauma Approximately 10% Temporal Bone Fractures May cause BPPV (generally in older children) May cause a leakage of inner ear fluids Concussions Vestibular Neuritis and Labyrinthitis Children account for 5% of the cases of VN(Rine and Wiener-Vacher, 2013; Hain, 2012; Davitt, Delvecchio, and Aronoff, 2017)

Causes of Pediatric Vertigo/Dizziness (continued) Tumors Posterior Fossa Malformation of the inner ear Visual and oculomotor difficulties Vestibulotoxic drugs Bilateral vestibular paresis ( Rine and Wiener- Vacher , 2013; Hain, 2012)

Causes of Pediatric Vertigo/Dizziness (continued) Otitis Media Syphilis Hypotension Psychogenic dizziness Low blood glucose Epilepsy Dehydration Autoimmune disorders

Dizzy Kids: Signs and Symptoms Visible Disorientation Frequent Falls Clinging on objects or parents Pallor Fearfulness Complaining of feeling “funny” Reporting stomache Vomiting

Importance of Identifying Vestibular Dysfunction in Children We know that children generally compensate from vestibular dysfunction quicker than adults HOWEVER, vestibular dysfunction in children relates to delayed gross motor skills, abnormal visual acuity, and difficulty reading in some cases Abnormal visual acuity related to difficulty tracking and difficulty reading Social isolation? Anxiety? The implications of vestibular pathologies in children is still being explored, the full impact vestibular loss has on children is unknown This stresses the importance of identifying vestibular pathologies in children (Janky & Givens, 2015; Braswell & Rine , 2006)

Migraines and Migraine Variants Migraines are the most common cause of dizziness in children Dizziness is accompanied by, preceded by, or followed by a headache Other symptoms may be nausea and vomiting, photophobia, phonophobia , or osmophobia Vestibular migraines generally have an onset ~ puberty Always ask about a family history of migraines

Concussions: Signs and Symptoms ( CDC.gov )

Concussion Treatment Best treatment for cortical concussions is rest Best treatment for labyrinthine concussions is movement and VRT Symptoms may last 1 hour to several weeks What if they have both?? Limited research on this topic

Concussion

Benign Paroxysmal Positional Vertigo (BPPV) After Sports Related Trauma in Children Reimer et al. (2018) performed a study assessing pediatric sports related concussion (SRC) patients 115 children underwent a vestibular assessment 10% (12 participants) were diagnosed with BPPV BPPV was successfully treated in all of these participants Encouraged vestibular assessment in children with SRC Encouraged a comprehensive management of pediatric SRC requiring a multidisciplinary approach to address the heterogeneous pathophysiology of persistent post-concussion symptoms (Reimer et al., 2018)

Paroxysmal Torticollis of Infancy Rare functional disorder characterized by recurrent episodes of torticollic posturing of the head in healthy children Benign Head is tilted to one side SCM muscle becomes shortened and contracted May be migraine variant, or a BPVC variant Onset within first 5 years of life Often within the first 3 months

Paroxysmal Torticollis of Infancy Episodes usually lasts hours to days Episodes may by associated with other symptoms including: Vomiting Pallor Perspiration Apathy or irritability Unsteady gait Upwardly-diverted gaze Abnormal truncal posture ( tortipelvis ) Contraction of the posterior neck muscles ( retrocollis)Some children with paroxysmal torticollis receive VRT and general physical therapyThe condition often resolves by age 3 to 5 years, but it may be replaced by a different migraine variant or other form of migraine(Rothner, 2018)

Benign Paroxysmal Vertigo (BPVC) of Childhood Most common childhood vestibular disorder associated with dizziness It was first described by Basser in 1964 and was thought  to be a variant of vestibular neuritis Commonly seen in children ages 2-12 Considered a central vestibular pathology NOT THE SAME AS BPPV Not associated with the displacement of otoconia

BPVC Occurs spontaneously in healthy children Attacks of true vertigo is the main symptom Duration of attacks is brief During severe attacks children may become very frightened, weak, and may perspire excessively Complete recovery several minutes after the attack These attacks generally spontaneously cease without any residual disability Tinnitus, hearing loss, loss of consciousness, and headaches are generally not present Vertigo is not provoked ( Kostic , 2012)

BPVC Since attacks are generally brief, usually no treatment is given BPV of childhood and paroxysmal torticollis usually resolve without treatment However, children with dizziness or unsteady gait may receive VRT to work on strengthening balance skills and coordination May be a migraine variant 30-50% of these children develop classic migraines around the time of puberty Some are diagnosed with Meniere’s Disease in adulthood Vestibular march

Pediatric Meniere’s Disease Controversial The rarity of “definite” MD in pediatric patients is most likely because small children cannot clearly describe their symptoms such as tinnitus and aural fullness, which are essential for the diagnosis of definite MD Brantberg et al. (2012) suggested that young children with idiopathic recurrent vertiginous attacks more than 20 min, accompanied by fluctuating low frequency HL may have definite MD

Chiari Malformation Structural defects in the cerebellum Congenital Present at birth; however, most do not begin to develop symptoms until midlife Four types of Chiari Malformation Type I most common Generally presents in childhood or early adulthood (Piper et al., 2019)

Chiari Malformation Symptoms Neck pain Dizziness Balance problems Muscle weakness Numbness or other abnormal feelings in the arms or legs Vision problems Difficulty swallowing Tinnitus Hearing loss Vomiting Insomnia Depression Headache made worse by coughing or strainingHand coordination and fine motor skills may be affected(Grahovac, Pundy, & Tomita, 2018; Piper et al., 2019)

Chiari Malformation in Babies Signs and Symptoms: Fussiness when being fed Crying with a lot of arching of the back Inconsolable cry Drooling more than normal Weak cry Trouble gaining weight Arm weakness Developmental delays CM Type II is usually seen in infants/children with a form of spina bifida called myelomeningocele ( Grahovac , Pundy, & Tomita, 2018; Piper et al., 2019)

General Ataxia People with ataxia have problems with coordination because parts of the nervous system that control movement and balance are affected Disorders of the cerebellum can cause ataxia Can cause gait imbalance, incoordination of arms and legs, slurred speech and impaired eye-movement control Some people also experience mood, memory and concentration problems ( www.rch.org.au )

Vestibular Testing Overview Videonystagmography /Electronystagmography (VNG/ENG): Identifies or rules out possible peripheral vestibular dysfunction or central dysfunction Specifically assesses the horizontal SCC Rotational Chair: Identifies or rules out possible peripheral vestibular dysfunction or central dysfunction. Assesses central compensation of peripheral vestibular dysfunction. Specifically the horizontal SCC Platform Posturography : Measures postural control and postural sway Multi-system Vestibular Evoked Myogenic Potentials (VEMPs): Assesses the function of the otoliths, and vestibular nerve, can identify possible semicircular canal dehiscence Ocular VEMPs (oVEMPs): Assesses the function of the utricule and superior portion of the vestibular nerveCervical VEMPs (cVEMPs): Assesses the function of the saccule and inferior portion of the vestibular nerveVideo Head Impulse Testing (vHIT): Capability to assess all six semicircular canals

Fill your boat! AuDs Pediatricians Otologists Ophthalmologists Vestibular Rehab PTs Neurologists Assessing dizziness or imbalance in children should be a team approach

Dynamic Visual Acuity (DVA) Provides functional indirect measure of VOR Change in visual acuity of greater than .2 log MAR (3 lines or greater) is indicative of peripheral vestibular dysfunction ( Rine & Braswell, 2003; Schubert, Herdman , & Tusa , 2001) Can be used for ages 3 and older Reduced DVA in adults have been associated with increased risk of falling, difficulty reading, and decreased quality of life (Hall et al., 2004; Whitney et al., 2009; Guinand et al., 2012)

Eye Charts Snellen Chart for older children who can read and identify letters LEA Chart for younger children who can identify shapes

DVA Static visual acuity (SVA) is performed first Identify the lowest line the child can read all letters with no head movement DVA Tilt the child’s head 30º yaw, oscillate head back and forth at about 2 Hz (2 cycles per second) Identify the lowest line the child can read all letters with head movement A loss of three lines or more is considered clinically significant and suggestive of peripheral vestibular dysfunction (cannot lateralize side of dysfunction)

DVA Inexpensive Does not require expensive equipment when performing DVA-Non-instrumented (DVA-NI) Quick to perform

Case History Ask about a family history of migraines Is the child afraid of the dark? Does he/she have motion sickness? Does he/she avoid play or certain activities? Does he/she seem disoriented after quick movements? Does he/she complain of stomach aches? (May be nausea) Ask about possible precipitating events Was he/she hurt at sports practice recently? Any recent falls? Did he/she have a cold or virus before this started?

Techniques Stickers Bubbles Things that light up Tell parent(s) to bring child’s favorite toy or stuffed animal Ipad or smartphone to play videos Ask parent(s) what the child likes, TV shows, characters, animals, etc. Don’t forget to task if doing a rotary! Sing songs, ask about family and friends, have them tell a story Everything is a game!

Vestibular Assessment Protocol To Consider (and Adjust) 3 to 11 years oVEMPs and cVEMPs Rotational Chair vHIT Dix Hallpike High Frequency Headshake 11 years + oVEMPs and cVEMPsRotational ChairvHITCalorics only or VNGDix HallpikeHigh Frequency Headshake

Test Considerations: VEMPs Consider two audiologists – if possible One to run the equipment One to engage the child and confirm contraction of the appropriate muscles oVEMPs – consider putting a sticker on the ceiling cVEMPs – consider holding a phone, toy, tablet, etc. behind, and below, the patient You must insure an appropriate contraction of the muscles being tested (SCM or extraocular muscles) to make an analysis

Test Considerations: Rotational Chair Consider use of a booster seat when necessary Small children may sit on parent’s lap Pediatric googles or electrodes (if rotary chair is enclosed in a capsule) Don’t forget to task if doing a rotary! Sing songs, ask about family and friends, have them tell a story

Test Considerations: vHIT Use a small sticker instead of the designated target Ask child questions about the sticker to maintain their gaze on the sticker Make a game out of the test What is the child “doing” with his/her eyes? (use your imagination and sticker as reference) Catching bad guys Bringing the princesses back to the castle

Test Considerations: VNG Put a sticker on your nose to perform Dix Hallpike or bedside HIT to maintain the child’s gaze on your nose Some clinics consider use of slightly cooler temperatures for calorics (if performed) Some clinics recommend performing vHIT first, if vHIT is abnormal THEN consider calorics VNG/ENG and Rotational Chair evoke dizziness and require darkness (denied vision)vHIT and VEMPs do not evoke dizziness and do not require darkness

Pediatric Vestibular Rehabilitation Treatment can focus on: Treatment of BPPV Improving balance and safety with mobility   Learning to brace falls Improving tolerance for motions the child tends to avoid   Improving adaptive responses to sensory input   Visual-motor exercises   Can improve DVA which in turn may improve reading ability

Vestibular Screening

Vestibular Screening Utilized by Castiglione and Lavender DVA HIT ( bHIT or vHIT ) Single Leg Stance Standing on Foam Eyes Open and Closed (Castiglione & Lavender, 2019)

Vestibular Screening Continued Janky et al. (2018) suggests consideration for referral for a vestibular evaluation for children with hearing loss greater than 66 dB HL, especially those who sit after 7.25 months or walk after 14.5 months, or if their parents have a concern regarding their general gross motor development

Case Studies

Case #1: Patient “A” 3 year 3 month old female Wide-based gait and frequent falls Occasional visible disorientation following quick movements Strong family history of migraines on both sides, and concern regarding migraines in 6 year old older brother Unremarkable birth and medical histories Had been seen by her pediatrician and a physiatrist prior to vestibular testing

Case #1: ”A”’s Vestibular Assessment Results Dix Hallpike Maneuver: WNL, bilaterally cVEMP : Present at 95 dB HL, bilaterally Rotational Chair: Low gain in the low frequencies recovering to borderline normal gain Lateral vHIT : WNL, left and right ” A” appeared symptomatic on the day of the vestibular evaluation

Case #1: “A”’s Vestibular Assessment Results Continued

Case #1: Diagnosis A was diagnosed with BPVC Underwent an MRI of the brain, which was negative Began vestibular rehabilitation with a pediatric PT

Case #1: Follow Up A returned 6 months later for repeat rotational chair testing Parental report of continued disorientation at times A was able to brace her falls, resulting in less injuries Improvement in her balance and gait Rotational chair was unremarkable – normal gain across all frequencies Central compensation occurring? Potential peripheral manifestation when symptomatic

Case #2: “S” 12 year old male reporting dizziness and brief LOC for several months “I get so dizzy I pass out” 3 trips to the ER Reportedly unremarkable MRI Referred by ENT However, the child reported he has been feeling better within the last few weeks

Case #2: “S” What changed? Is he compensating? In between the ENT visit and vestibular eval , patient was seen by neurology and Dx with childhood epilepsy Began seizure medication

Case # 2: “S” - Audiometric and Vestibular Assessment Results Hearing WNL 250Hz-8kHz, AU cVEMPs and oVEMPs , WNL, bilaterally Appropriate amplitudes and no significant asymmetries noted Unremarkable rotational chair evaluation Negative Dix Hallpike Maneuver, head left and head right

Case #2: “S” - Vestibular Assessment Results

Case #2: “S”’s Diagnosis S’s dizziness was attributed to his new diagnosis of epilepsy His symptoms were controlled by continued use of his seizure medication

Case #3: ”R” 14 year old female reporting episodic vertigo lasting approximately 1 hour in duration for 3 months Provoked and unprovoked Provoked symptoms occurred with bed mobility and bending Vertigo was reported to be occasionally exacerbated by quick movements Nausea and emesis Frequent occipital headaches Falls related to dizziness were denied Subjective hearing loss in the LEFT ear without fluctuation Bilateral tinnitus Missing school due to dizziness Cannot participate in usual activities and sports

Case #3: “R” Audio Despite subjective HL in the left ear, R had a low frequency SNHL in the right ear. Confirmed with the Weber test, lateralizing to the left 250Hz-1kHz

Case #3: “R” Vestibular Assessment Repeat audio revealed stable hearing Electrocochleography : Unremarkable – not suggestive of endolymphatic hydrops, AU cVEMPs and oVEMPs were unremarkable, bilaterally VNG: Bithermal caloric results revealed a bilateral weakness suggestive of a bilateral peripheral vestibular dysfunction or central dysfunction RC SPV: 2º; RW SPV: oº; LC SPV: 0º; LW SPV: 2º DNT ice water calorics at audiologist’s discretion (due to patient’s age)Dix Hallpike was negative head left and head rightSlight downbeating nystagmus noted with vision denied throughout test

Case # 3: “R” - Diagnosis Following an MRI, R was diagnosed with Chiari Malformation R was referred to pediatric neurology for treatment and medical management A watch and wait approach was pursued

Case #4: ”M” 8 year old male with parental and physician concerns regarding a vestibular dysfunction M reported that ”the room turns and turns” Significant motion sickness was reported, especially on the school bus M was very reliant on his glasses, and reported he felt dizzier without them M also had a new-found fear of the dark, and requested that the light in his room stay on at night M’s mother reported occasional falls; however, she was very concerned that he no longer wanted to play outside or with his classmates or siblings because he would get sick

Case #4: “M” - Results Hearing WNL 250Hz-8kHz Recent ophthalmology/optometry visit No recent change in vision, prescription was appropriate No further eye issues were discerned Dix Hallpike was Negative, Head left and Head Right vHIT was WNL for all six semicircular canals oVEMPs were WNL, bilateral cVEMPs revealed a significant asymmetry of 44% with the right response smaller than the leftAbnormal function of the saccule, inferior portion of the vestibular nerve, and/or their afferent pathways could not be ruled out

Case # 4: “M” M was sent to vestibular rehabilitation A nightlight for the bedroom was recommended M and his mother were encouraged to attempt to improve activity level at home M’s mother was counseled regarding motion sickness and coping mechanisms, and to consider limiting reading, tablet use, or other visual stimulation while in moving vehicles

Thank you! Contact: Patricia.m.mazzullo.civ@mail.mil Reference list available upon request