peggy kelley: ear, nose and throat issues in children with down syndrome
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7/27/2019 Peggy Kelley: Ear, Nose and Throat Issues in Children with Down Syndrome
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Ear, Nose and Throat Issues in
Children with Down SyndromePeggy E. Kelley MD FACS FAAP
Associate Professor of OtolaryngologyUniversity of Colorado, Denver
Childrens Hospital Colorado
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Ear, Nose & Throat issues in children withDown syndrome
What do parents need to know about their childrenwith Down syndrome related to sleep apnea (tesLngguidelines, relaLonships to adenoid and tonsils)?
What do parents need to know about their childrenwith Down syndrome and hearing loss (tesLng, tubes,effect on speech)?
What ENT issues should parents be aware of regardingadolescence or young adulthood?
How can we ensure that individuals with Downsyndrome are hearing? What aids are there?
How can we help individuals with Down syndrome tobreathe be er? What aids are there?
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Goals
Review normal anatomy and typical problemswith the Ear, Nose and Throat.
Show how children with Down Syndrome haveanatomic differences that inuence theirdisease processes and treatments.
Have an idea how an ENT(Ear, Nose, andThroat doctor or Otolaryngologist) can helpcare for your child.
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Ear Hearing
3 parts Outer Middle Inner
Test with: Tympanometry Oto-AcousLc Emission(OAE) Auditory Brainstem
Response (ABR or BAER) Behavioral Audiometry
Balance 3 semicircular canals
Affected by pressure in themiddle ear
ConducLve
Sensorineural
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Ear
Outer ear Wax
Build up Treat with weekly oil
InfecLon Swimmers ear
Treat with vinegar/alcohol
Foreign Body Sponge, beads, etc
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Child with Down Syndrome
Outer Ear- Canal Cerumen (wax) buildup
Hourglass shape
Flaky type wax Oil weekly Have cleaned by health
care provider
Middle Ear Inner Ear
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Ear
Outer Ear- Canal Middle Ear
Fluid/InfecLon ConducLve Hearing oss
Dont hear as well underwater
Clear through
Eustachian tube Inner Ear
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Child with Down Syndrome Outer Ear- Canal Middle Ear
Fluid/InfecLon
Immune system issues ConducLve Hearing oss
80% of hearing problems Clear through
Eustachian tube Skull base shape a er
means does not clear aswell
Inner Ear
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Ear
Outer Ear- Canal Middle Ear Inner Ear
Sensorineural/ NerveHearing oss
CongenLal (born with) Newborn screen
Acquired (develops later) RouLne hearing checks
Balance
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Child with Down Syndrome Outer Ear- Canal Middle Ear Inner Ear 80% have
some problem withhearing Sensorineural/ Nerve
Hearing oss Congenital (born with)
Newborn screen Acquired (develops later)
RouLne hearing checks Balance
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Hearing and Speech
In order to have good speech you must havegood hearing.
Fluid with or without infecLon may decreaseyour childs hearing.
Checking and rechecking hearing is necessaryand helpful
If you pass one hearing test is does notguarantee that you will pass the next year.
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Hearing Helps
Remove the uid and aerate the middle ear Tubes (temporary)
Hearing aids for conducLve or sensorineuralhearing loss are available.
Behind the ear In the canal not easy secondary to small canals BAHA on implant
FM system in class
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Nose/Airway
Warm
Filter Humidify Breathe
Remove Tonsils and/oradenoids
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Tonsils
Size ma ers Tongue size and
posiLon ma ers Can remove tonsils ess easily operate on
base of tongue
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Child with Down Syndrome
Breathe ess room
Warm Filter Humidify
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ObstrucLve Sleep Apnea
Child without Down Syndrome Symptoms:
Snoring/Apnea
Restless sleep Behavior issues because Lred Bedwe ng Difficulty eaLng solid food School performance issues
Child with Down Syndrome Symptoms
May have no snoring
May or not be restless Behavior issues may be
independent of rest EaLng issues may be from
large tonsils or other
neurologic/coordinaLonissues
School performance may besecondary to other delays
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ObstrucLve Sleep Apnea
Child without Down Syndrome Diagnosis:
Sleep study for mismatch of
tonsil size and history O en do not need to repeat
a er treatment becausesymptoms gone a er medicalor surgical treatment.Symptoms are reliable.
Child with Down Syndrome Diagnosis:
Screening sleep study even if
not symptomaLc Repeat to be sure treatment
helped no specic symptomto follow
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ObstrucLve Sleep ApneaChild without Down Syndrome
Treatment: Tonsillectomy and
adenoidectomy Nasal turbinate reducLon +/- Growth typically enlarges
airway with skull base exion
Child with Down Syndrome Treatment
Oxygen therapy PosiLve pressure mask(CPAP) Tonsillectomy and
adenoidectomy Nasal turbinate reducLon +/-
Growth may worsen airwaybecause skull base does notchange
Oxygen therapy/CPAP Non-invasive
Sleep endoscopy to nd site ofobstrucLon
-More extensive surgicalopLons
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Sleep apnea guidelines Test for sleep apnea with a sleep study by age 4
years. High alLtude children may need to be tested earlier
Re-test o en Test with change in sleep or surgery for apnea May depend on diagnosis of sleep apnea Perhaps every 2-3 years to pick up silent apnea
Sleep apnea in child with Down syndrome is notautomaLcally xed with a tonsillectomy &adenoidectomy
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Sleep aids
Breath-rite strips Mouth guards to bring jaw forward
Oxygen PosiLve pressure breathing BiPap or Cpap Surgery
Is an aid not a total x Tracheotomy is a total x for obstrucLon
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Adolescence
May have sleep issues reappear. May have progressive hearing loss.
It is easier to be re-tesLng at this age andmaking adjustments than tesLng for the rstLme and needing big steps.
(It is easier to test if you know what the test isgoing to be.)
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Conclusion
Children with Down Syndrome have reasonsboth anatomic (structural) and physiologic
(funcLonal) to have: Hearing loss, Nasal obstrucLon
Breathing problems/sleep apnea Speaking and language challenges
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Conclusions
Knowing why a person with Down Syndromehas ENT issues can help the family understand
why usual treatments may or may not work. Knowing why can help explain why we
someLmes need to do more. Ge ng to know (and ove) your ENT can be a
great thing!
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References (Sho , S.R. (2000). Down syndrome: Common pediatric ear, nose and throat
problems. Down Syndrome Quarterly , 5(2), 1-6.) Balkany, T.J., Mischke, R.E., Downs, M.P. & Jafek, B.W. (1979). Ossicular
abnormaliLes in Down's syndrome. Otolaryngology: Head and Neck Surgery , 87,372-384.
Sho9 S, et al ObstrucLve Sleep Apnea: Should All Children With Down Syndromebe Tested? Arch Otolaryngol Head Neck Surg. 2006;132:432-436
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Oral cavity comparison
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Speech Sound generator
larynx/voice box ungs for power
Cry or laugh anguage intelligibility
Resonance Skull base shape and Palate shape and movement
ArLculaLon ips, tongue, palate Control of movement- apraxia
Voluntary programming, combining, organising, and sequencing
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Sleep Endoscopy
Goals Find site of anatomic
blocking
Find site of dynamicblocking
Uses arLcial sleep Research on best way to
replicate real sleep
Sleep Endoscopy
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ObstrucLve Sleep Apnea
Child with Down Syndrome Treatment
Tongue base reducLon/advancement
Hyoid advancement Pharyngoplasty
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