1. Step into the void: Vestibular deficits in children with hearing loss
Genevieve DelRosario, MHS, PA-C
University of Kansas Medical Center
Kansas City, Kansas

3. Objectives Review vestibular physiology and pathophysiology
Discuss the evaluation of a child’s vestibular status
Be able to develop a plan for a child with vestibular dysfunction

4. Vestibular system: A “sixth sense”
Allows us to know where we are in space
Orient selves with respect to gravity
Unifying system that allows us to process information from other senses

5. Where does our sense of balance come from?
Eyes
Sensors in joints, muscles, and feet
Balance organs in the ears

6. Vestibular system Vestibular ocular system Vestibular spinal system
Responsible for visual stabilization
Vestibular spinal system
Maintains orientation of the body in space
Contributes to the postural tone necessary for the acquisition of motor development milestones

7. Development of the vestibular system
Very old in evolutionary terms
Emerges early in embryonic development
Prior to vision and hearing
Peak developmental time is 6-12 months
Continues development through childhood

11. Prevalence of vestibular dysfunction*
20-70% of children with hearing loss
Higher in profound HL vs. severe
Continuum of severity
Mild loss to vestibular areflexia
* Angeli 2003.

12. What happens in deaf/HOH children?
Semicircular canals may be absent
Hair cells may be damaged, absent, or reduced
Nerve damage
Enlarged vestibular aqueduct
???

13. Will a deaf child’s vestibular system get better?

14. ……Maybe.

15. Better or worse? In general, balance improves as you age
Vestibular maturation continues through adolescence
Vestibular deficits in deaf/HOH may worsen
Small study showed progressive gross motor and balance difficulties1
Contradicted by other studies2
1. Rine et al Siegel et al 1991.

16. How does it feel?

17. How does it feel (adult perspective)?
Headache
Feeling of ear fullness
Imbalance to the point of being unable to walk
Bouncing and blurring of vision (oscillopsia)
Inability to tolerate head movement
Difficulty walking in the dark
Feel unsteady; actual unsteadiness while moving
Lightheadedness
Severe fatigue
In severe cases, symptoms such as oscillopsia and problems with walking in the dark are not going to go away.

18. Oscillopia

19. Signs of poor vestibular function
Low muscle tone
Delayed loss of primitive reflexes
Delayed gross motor milestones
Developmental delays
Seizures
Nystagmus
Easy fatiguability
Reflux

20. Signs of poor vestibular function
Low muscle tone
Delay in holding head up
“Snuggly” baby
“Floppy baby”
Arching of back

21. Signs of poor vestibular function
Delayed disappearance of newborn reflexes
Moro
ATNR: Asymmetric tonic next response
Usually disappear by 6-7 months

22. Signs of poor vestibular function
Delayed motor milestones
Average deaf child walks at 14 months
Average child with Usher’s Type 1 walks at 20 mos
Delays sitting, crawling, climbing steps, hopping…
Speech delays

23. What do older children look like?
Clumsy
Unable to walk on a balance beam
Problems standing with feet together and eyes closed (Romberg test)
Love spinning,
merry-go-rounds,
water activities

24. Weak VOR Challenges with reading
Gaze instability causes problems with acuity*
Braswell & Rine 2006.

25. My deaf child is a late walker…does that mean she has vestibular problems?

26. No, but it’s a red flag! Consider also:
Vision problems
Global developmental delay
Autistic spectrum disorder
Just taking her sweet time!

27. Tests of vestibular function
Eye tracking tests
Positional/positioning tests
Dix-Hallpike
Supine
Rotational tests
Rotary chair testing is gold standard

28. Causes of poor vestibular function
Postnatal acquired cases
Meningitis
Labyrinthitis
Some forms of syndromic deafness
Labyrinthine dysplasia
Ototoxicity

29. Conditions associated with CHL and poor balance
Usher’s Syndrome (Type 1)
Waardenburg Syndrome
Pendred syndrome
ESPN mutation
CHARGE Syndrome
Brachio-oto-renal syndrome
….and more!

30. Usher’s Syndrome Autosomal recessive syndrome
Hearing loss, vision loss, and variable vestibular dysfunction
Visual loss is due to retinitis pigmentosa
Three types

31. Usher’s syndrome Type 1 Type 2 Type 3 Born profoundly deaf
Vision loss typically noted by age 10
Absent vestibular function
3-6/100,000 individuals
~ 5% of deaf individuals
Type 2
Moderate to severe hearing loss
Vision loss typically begins after teen years
Normal vestibular function
Type 3
Born with normal hearing, varying rate of loss
Night blindness during puberty
Normal or near-normal vestibular function

32. Retinitis pigmentosa
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33. Retinitis pigmentosa

34. Waardenburg Syndrome

35. Pendred Syndrome

36. CHARGE Syndrome Coloboma of the eye Heart defects
Atresia of the choanae
Retardation of growth and/or development
Genital and/or urinary abnormalities
Ear abnormalities and deafness

37. Brachio-oto-renal syndrome
Autosomal dominant
Malformation of ear
cochlear hypoplasia
enlargement of the cochlear and vestibular aqueducts
hypoplasia of the lateral semicircular canal
Hearing loss
Malformations of kidney

38. ESPN Mutation
Autosomal recessive mutation
Mapped to chromosome 1p36.3

39. Environmental causes Aminoglycoside antibiotics Anti-neoplastics
Gentamicin, streptomycin, kanamycin, tobramycin, neomycin, amikacin, netilmicin, dihydrostreptomycin, and ribostamycin.
Anti-neoplastics
Cisplatin, carboplatin
Environmental chemicals
Butyl nitrite, mercury, carbon disulfide, styrene, carbon monoxide, tin, hexane, toluene, lead, trichloroethylene, manganese, xylene, mercury
Loop diuretics
Bumetanide, ethacrynic acid, furosemide, and torsemide.
Aspirin and quinine products
Infections

40. Vestibular effects of cochlear implantation
Rare cause of permanent damage
Common cause of transient damage
20% in one series1
Anecdotal evidence for improvement
Hearing with CI does not make a difference2
1: Vilbert et al Suarez et all 2007.

41. I think the child I am treating may have vestibular problems. Now what?

42. Evaluation CT of temporal bone Vestibular testing (if possible)
Physical, occupational, ? cognitive therapies
Genetic appointment
Strongly consider testing for Usher’s mutations
Vision evaluation
?ERG

43. Therapeutic goals Enhance existing vestibular capabilities
Strengthen compensatory mechanisms

44. Compensatory mechanisms
Proprioceptive input
Walking barefoot or soft soled shoes
Visual input
Other sensory systems

45. Therapies for children with poor vestibular systems
Swinging
Rocking
Bouncing/jumping
Dancing
Skipping
Running
Hopping
Jumping rope
Rough and tumble play

46. Other interventions May wear weighted vests, leg weights, etc
Consider orthopedic shoes
OR soft soled shoes
Offer sensory activities
May need extra time to
process information

47. Does treatment make a difference?
Motor development improved post treatment1
Therapy three times weekly for 12 weeks
Visual and somatosensory function, balance training
Significant improvement in motor development
Insignificant improvement in posturography
May improve gaze stability2
Preliminary study of two individuals
1. Rine et al Braswell and Rine 2006.

48. Treatment challenges Lack of data Different causes of balance problems
Especially true for infants and toddlers
Different causes of balance problems

49. Cautions with poor vestibular function
Where visual and proprioceptive information is unreliable
Eg, swimming in the dark
Problems with depth perception
Tunnel vision can cause problems
Worse in unfamiliar places

50. Implications for future research
Vestibular hypofunction in infancy and early childhood poorly understood
Need for research on both function and treatment

51. Implications for families
Share vestibular information with parents
Encourage physical activity
Continue to screen older children
Balance
Retinitis pigmentosa

52. Helpful resources
What’s going on in there: How the brain and mind develop in the first five years of life. Lise Eliot, PhD. 1999
The out of sync child has fun. Carol Stock Kranowitz & TJ Wylie
Vestibular disorders organization

54. Genevieve DelRosario gdelrosario@kumc
Genevieve DelRosario Department of Pediatrics University of Kansas Medical Center 3901 Rainbow Blvd Kansas City, KS (913)

55. Angeli S. Value of vestibular testing in young children with sensorineural hearing loss. Arch Otolaryngol Head Neck Surg. 2003;129:
Braswell J, Rine RM. Evidence that vestibular hypofunction affects reading acuity in children. Int J Pediatr Otorhinolaryngol Nov; 70(11):
Braswell, J, Rine RM. Preliminary evidence of improved gaze stability following exercise in two children with vestibular hypofunction. Int J Pediatr Otorhinolaryngol Nov;70(11): Epub 2006 Oct 4
Eliot, L. What’s going on in there: How the brain and mind develop in the first five years of life. Bantam Books, 1999.
Rine RM, Braswell J, Fisher D, Joyce K, Kalar K, Shaffer M. Improvement of motor development and postural control following intervention in children with sensorineural hearing loss and vestibular impairment. Int J Pediatr Otorhinolaryngol Sep;68(9):
Rine RM, Cornwall G, Gan K, LoCascio C, OHare T, Robinson E, Rice M. Evidence of progressive delay of motor development in children with sensorineural hearing loss and concurrent vestibular dysfunction. Perceptual and Motor Skills. 90(3 Pt 2): , 2000 June.
Siegel JC, Marchetti M, Tecklin JS. Age-related balance changes in hearing-impaired children. Phys Ther Mar;71(3):183-9
Suarez H, Angeli S, Suarez A, Rosales B, Carrera X, Alonso R. Balance sensory ogranization in children with profound hearing loss and cochlear implants. Int J Pediatr Otorhinolaryngol Feb 1; [Epub ahead of print]
Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with cochlear implantation. Acta Otolaryngol Suppl. 2001; 545: