Pediatric Vestibular Assessment: Thinking Outside the VNG

1. 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)

2. Financial Disclosure No financial relationships to disclose

3. 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

4. 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)

5. 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

6. 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)

7. Development of Motor Skills (Gans, 2012)

8. 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

9. 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)

10. 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)

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

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

13. 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)

14. 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

15. Concussions: Signs and Symptoms (CDC.gov)

16. 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

17. Concussion

18. 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)

19. 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

20. 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 therapy • The 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)

21. 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

22. 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)

23. 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

24. 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

25. 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)

26. 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 straining • Hand coordination and fine motor skills may be affected (Grahovac, Pundy, & Tomita, 2018; Piper et al., 2019)

27. 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)

28. 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)

29. 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 nerve • Cervical VEMPs (cVEMPs): Assesses the function of the saccule and inferior portion of the vestibular nerve • Video Head Impulse Testing (vHIT): Capability to assess all six semicircular canals

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

31. 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)

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

33. 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)

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

35. 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?

36. 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!

37. 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 cVEMPs • Rotational Chair • vHIT • Calorics only or VNG • Dix Hallpike • High Frequency Headshake

38. 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

39. 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

40. 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

41. 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

42. 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

43. Vestibular Screening

44. 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)

45. 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

46. Case Studies

47. 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

48. 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

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

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