peggy kelley: ear, nose and throat issues in children with down syndrome

7/27/2019 Peggy Kelley: Ear, Nose and Throat Issues in Children with Down Syndrome

1/28

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


2/28

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?


3/28

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.


4/28

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


5/28

Ear

Outer ear Wax

Build up Treat with weekly oil

InfecLon Swimmers ear

Treat with vinegar/alcohol

Foreign Body Sponge, beads, etc


6/28

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


7/28

Ear

Outer Ear- Canal Middle Ear

Fluid/InfecLon ConducLve Hearing oss

Dont hear as well underwater

Clear through

Eustachian tube Inner Ear


8/28

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


9/28

Ear

Outer Ear- Canal Middle Ear Inner Ear

Sensorineural/ NerveHearing oss

CongenLal (born with) Newborn screen

Acquired (develops later) RouLne hearing checks

Balance


10/28

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


11/28

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.


12/28

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


13/28

Nose/Airway

Warm

Filter Humidify Breathe

Remove Tonsils and/oradenoids


14/28

Tonsils

Size ma ers Tongue size and

posiLon ma ers Can remove tonsils ess easily operate on

base of tongue


15/28

Child with Down Syndrome

Breathe ess room

Warm Filter Humidify


16/28

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


17/28


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


18/28

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


19/28

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


20/28

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


21/28

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.)


22/28

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


23/28

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!


24/28

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


25/28

Oral cavity comparison


26/28

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


27/28

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


28/28


Child with Down Syndrome Treatment

Tongue base reducLon/advancement

Hyoid advancement Pharyngoplasty

peggy kelley: ear, nose and throat issues in children with down syndrome

Documents