Presentation is loading. Please wait.

Presentation is loading. Please wait.

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

Similar presentations


Presentation on theme: "Step into the void: Vestibular deficits in children with hearing loss Genevieve DelRosario, MHS, PA-C University of Kansas Medical Center Kansas City,"— Presentation transcript:

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

2

3 Objectives Review vestibular physiology and pathophysiology Review vestibular physiology and pathophysiology Discuss the evaluation of a childs vestibular status Discuss the evaluation of a childs vestibular status Be able to develop a plan for a child with vestibular dysfunction 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 Allows us to know where we are in space Orient selves with respect to gravity Orient selves with respect to gravity Unifying system that allows us to process information from other senses Unifying system that allows us to process information from other senses

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

6 Vestibular system Vestibular ocular system Vestibular ocular system –Responsible for visual stabilization Vestibular spinal system 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 Very old in evolutionary terms Emerges early in embryonic development Emerges early in embryonic development –Prior to vision and hearing Peak developmental time is 6-12 months Peak developmental time is 6-12 months Continues development through childhood Continues development through childhood

8

9

10

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

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

13 Will a deaf childs vestibular system get better?

14 ……Maybe.

15 Better or worse? In general, balance improves as you age In general, balance improves as you age –Vestibular maturation continues through adolescence Vestibular deficits in deaf/HOH may worsen Vestibular deficits in deaf/HOH may worsen –Small study showed progressive gross motor and balance difficulties 1 –Contradicted by other studies 2 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 Low muscle tone Delayed loss of primitive reflexes Delayed loss of primitive reflexes Delayed gross motor milestones Delayed gross motor milestones Developmental delays Developmental delays Seizures Seizures Nystagmus Nystagmus Easy fatiguability Easy fatiguability Reflux Reflux

20 Signs of poor vestibular function Low muscle tone 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 Delayed motor milestones –Average deaf child walks at 14 months –Average child with Ushers Type 1 walks at 20 mos –Delays sitting, crawling, climbing steps, hopping… –Speech delays

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

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

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

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

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

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

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

30 Ushers Syndrome Autosomal recessive syndrome Autosomal recessive syndrome Hearing loss, vision loss, and variable vestibular dysfunction Hearing loss, vision loss, and variable vestibular dysfunction –Visual loss is due to retinitis pigmentosa Three types Three types

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

32 Retinitis pigmentosa <> <>

33 Retinitis pigmentosa

34 Waardenburg Syndrome

35 Pendred Syndrome

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

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

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

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

40 Vestibular effects of cochlear implantation Rare cause of permanent damage Rare cause of permanent damage Common cause of transient damage Common cause of transient damage –20% in one series 1 Anecdotal evidence for improvement Anecdotal evidence for improvement Hearing with CI does not make a difference 2 Hearing with CI does not make a difference 2 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 CT of temporal bone Vestibular testing (if possible) Vestibular testing (if possible) Physical, occupational, ? cognitive therapies Physical, occupational, ? cognitive therapies Genetic appointment Genetic appointment –Strongly consider testing for Ushers mutations Vision evaluation Vision evaluation –?ERG

43 Therapeutic goals Enhance existing vestibular capabilities Enhance existing vestibular capabilities Strengthen compensatory mechanisms Strengthen compensatory mechanisms

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

45 Therapies for children with poor vestibular systems Swinging Swinging Rocking Rocking Bouncing/jumping Bouncing/jumping Dancing Dancing Skipping Skipping Running Running Hopping Hopping Jumping rope Jumping rope Rough and tumble play Rough and tumble play _00522c7610_m.j pg

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

47 Does treatment make a difference? Motor development improved post treatment 1 Motor development improved post treatment 1 –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 stability 2 May improve gaze stability 2 –Preliminary study of two individuals 1. Rine et al Braswell and Rine 2006.

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

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

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

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

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

53

54 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: 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. 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 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 Eliot, L. Whats going on in there: How the brain and mind develop in the first five years of life. Bantam Books, Eliot, L. Whats going on in there: How the brain and mind develop in the first five years of life. Bantam Books, 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, 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. 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 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] 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: Vibert D, Hausler R, Kompis M, Visher M. Vestibular function in patients with cochlear implantation. Acta Otolaryngol Suppl. 2001; 545:


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

Similar presentations


Ads by Google