Ear,

Nose and Throat Issues in Children with Down Syndrome

Peggy E. Kelley MD FACS FAAP Associate Professor of Otolaryngology University of Colorado, Denver Childrens Hospital Colorado

 Ear, Nose & Throat issues in children with Down syndrome now about their children What do parents need to k
with Down syndrome related to sleep apnea (tesLng guidelines, relaLonships to adenoid and tonsils)? What do parents need to know about their children with Down syndrome and hearing loss (tesLng, tubes, eect on speech)? What ENT issues should parents be aware of regarding adolescence or young adulthood? How can we ensure that individuals with Down syndrome are hearing? What aids are there? How can we help individuals with Down syndrome to breathe bePer? What aids are there?

Goals
Review normal anatomy and typical problems with the Ear, Nose and Throat. Show how children with Down Syndrome have anatomic dierences that inuence their disease processes and treatments. Have an idea how an ENT(Ear, Nose, and Throat doctor or Otolaryngologist) can help care for your child.

Ear
Hearing
3 parts
Outer Middle Inner

ConducLve Sensorineural

Test with:
Tympanometry Oto-AcousLc Emission(OAE) Auditory Brainstem Response (ABR or BAER) Behavioral Audiometry

Balance
3 semicircular canals
Aected by pressure in the middle ear

Ear
Outer ear
Wax
Build up
Treat with weekly oil

InfecLon
Swimmers ear
Treat with vinegar/ alcohol

Foreign Body
Sponge, beads, etc

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

Ear
Outer Ear- Canal Middle Ear
Fluid/InfecLon ConducLve Hearing Loss
Dont hear as well under water

Clear through Eustachian tube

Inner Ear

Child with Down Syndrome

Outer Ear- Canal Middle Ear
Fluid/InfecLon
Immune system issues

ConducLve Hearing Loss

80% of hearing problems

Clear through Eustachian tube

Skull base shape aPer means does not clear as well

Inner Ear

Ear
Outer Ear- Canal Middle Ear Inner Ear
Sensorineural/ Nerve Hearing Loss
CongenLal (born with)
Newborn screen

Acquired (develops later)

RouLne hearing checks

Balance

Child with Down Syndrome

Outer Ear- Canal Middle Ear Inner Ear 80% have some problem with hearing
Sensorineural/ Nerve Hearing Loss
Congenital (born with)
Newborn screen

Acquired (develops later)

RouLne hearing checks

Balance

Hearing and Speech

In order to have good speech you must have good hearing. Fluid with or without infecLon may decrease your childs hearing. Checking and rechecking hearing is necessary and helpful If you pass one hearing test is does not guarantee that you will pass the next year.

Hearing Helps
Remove the uid and aerate the middle ear Tubes (temporary) Hearing aids for conducLve or sensorineural hearing loss are available.
Behind the ear In the canal not easy secondary to small canals BAHA on implant

FM system in class

Nose/Airway
Warm Filter Humidify Breathe
Remove Tonsils and/or adenoids

Tonsils
Size maPers Tongue size and posiLon maPers Can remove tonsils Less easily operate on base of tongue

Child with Down Syndrome

Breathe
Less room

Warm Filter Humidify

ObstrucLve Sleep Apnea

Child without Down Syndrome

Symptoms:
Snoring/Apnea Restless sleep Behavior issues because Lred Bedwecng Diculty 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/coordinaLon issues School performance may be secondary to other delays

ObstrucLve Sleep Apnea

Child without Down Syndrome

Diagnosis:
Sleep study for mismatch of tonsil size and history Oeen do not need to repeat aeer treatment because symptoms gone aeer medical or 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 symptom to follow

ObstrucLve Sleep Apnea

Child without Down Syndrome

Child with Down Syndrome

Treatment
Oxygen therapy PosiLve pressure mask(CPAP) Tonsillectomy and adenoidectomy
Nasal turbinate reducLon +/-

Treatment:
Tonsillectomy and adenoidectomy
Nasal turbinate reducLon +/-

Growth typically enlarges airway with skull base exion

Growth may worsen airway because skull base does not change Oxygen therapy/CPAP
Non-invasive

Sleep endoscopy to nd site of obstrucLon -More extensive surgical opLons

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 oeen
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 not automaLcally xed with a tonsillectomy & adenoidectomy

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

Adolescence
May have sleep issues reappear. May have progressive hearing loss. It is easier to be re-tesLng at this age and making adjustments than tesLng for the rst Lme and needing big steps. (It is easier to test if you know what the test is going to be.)

Conclusion
Children with Down Syndrome have reasons both anatomic (structural) and physiologic (funcLonal) to have:
Hearing loss, Nasal obstrucLon Breathing problems/sleep apnea Speaking and language challenges

Conclusions
Knowing why a person with Down Syndrome has 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. Gecng to know (and Love) your ENT can be a great thing!

References
(ShoP, 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 Syndrome be Tested? Arch Otolaryngol Head Neck Surg. 2006;132:432-436

Oral cavity comparison

Speech
Sound generator
larynx/voice box Lungs for power Cry or laugh

Language intelligibility
Resonance
Skull base shape and Palate shape and movement

ArLculaLon
Lips, tongue, palate Control of movement- apraxia
Voluntary programming, combining, organising, and sequencing

Sleep Endoscopy
Goals Find site of anatomic blocking Find site of dynamic blocking Uses arLcial sleep
Research on best way to replicate real sleep

Sleep Endoscopy

ObstrucLve Sleep Apnea

Child with Down Syndrome Treatment
Tongue base reducLon/ advancement Hyoid advancement Pharyngoplasty