The Importance of Hearing and Hearing Loss in Children with

Down Syndrome

Emily Nightengale, AuD, CCC-A Megan Hedman, BA, Audiology Extern

What is Hearing? •  Hearing is one of our five senses •  It is the ability to perceive sound •  Hearing begins with our ears and ends in our brain •  Hearing is necessary for speech-language

development in children

2

Language Development •  There are a variety of theories as to how children

learn language. •  Infants and newborns are born without language,

known as the pre-linguistic phase. •  By 3 months old, babies can discriminate between

different sounds. •  By 6 months old, they can recognize sounds in their

primary language (may start producing them through babbling).

•  By 12 months old, infants have the ability to understand and produce meaningful words.

3

Critical Period for Language Development •  A hypothesis that suggests the early

years of a child’s life are the sensitive period for language development

•  Following this critical period, language acquisition is more challenging.

4

What is Hearing Loss? •  Hearing loss is the inability to hear some or all

sounds within the normal range •  It can be multidimensional because hearing

difficulties can occur at different frequencies (different sounds) and at different levels of intensity (volume)

•  There are different types of hearing difficulties (i.e. what part of the ear is not functioning properly)

5

The Audiogram

6

Different Types of Hearing Loss •  Conductive Hearing Loss

§  Ear canal occlusion from earwax § Otitis media or middle ear effusion §  Eardrum perforation § Ossicular chain dysfunction

•  Can typically be treated medically by physician (ENT)

7

Different Types of Hearing Loss •  Sensorineural Hearing Loss

§  Family History § Noise-induced §  Structural abnormalities

•  Considered permanent •  Audiologic management options usually

discussed when identified

8

Different Types of Hearing Loss •  Mixed Hearing Loss

•  Auditory Neuropathy § Risk factors include prematurity, hypoxia

(respiratory distress requiring mechanical ventilation), and hyperbilirubinemia

9

10

11

12

13

Incidence and Etiology

•  Incidence §  Estimations of 1-6 per 1,000 infants born annually as deaf or

hard-of-hearing (D/HH) in the U.S.

•  2-4 per 100 newborns in the NICU •  Hearing loss incidence increases to 9-10 per 1,000 in school

age population

•  Etiology §  Approximately 50% of childhood hearing loss is due to genetic

factors §  20%-50% is attributable to environmental causes (i.e. CMV,

Meningitis) §  25% to 30% is of unknown etiology

14

Hearing loss is the most frequently occurring birth

abnormality

•  Consistent access to speech and environmental stimuli is critical for speech and oral language development

•  If a child is identified as having a permanent hearing loss, audiologic intervention is needed as soon as possible

•  Newborns confirmed with hearing loss can wear hearing aids within the first few weeks of life

15

Incidence of Hearing Loss in Population with DS •  May be as high as 78% (Dahle, AJ et al., 1986; Balkany T, 1979)

•  34.1% (n=45 infants) in the first year of life, 85% of which is conductive (Raut, et al., 2011)

§  1/3 of infants normalized after treatment §  1/3 of infants remained unaltered §  1/3 did not follow-up

16

DS and Conductive Hearing Loss

•  Otitis Media (“ear infections” and “Eustachian tube dysfunction”) §  93% at age 1, 68% by age 5 (n=87 kids)

(Barr et al., 2011)

§  >43% (n=344 kids) of newborns with DS who passed their NHS developed a conductive hearing loss requiring insertion of ventilation (“pressure equalization” or “PE”) tubes (Park et al., 2012)

17

DS and Inner Ear Issues

•  Inner ear anomalies (Blaser, S. et al., 2006; Intrapiromkul, J. et al., 2012)

§ CT scans detected malformations in inner ear in 74.5% of patients (n= 51)

§ Narrow IAC was seen for 24.5% of patients with Down syndrome and in 57.1% of ears with SNHL

§ Malformations noted in the vestibular organ

§  Early onset presbycusis

18

What to expect at an appointment •  Otoscopy •  Tympanometry •  Otoacoustic Emissions •  Natural sleep or Sedated Auditory

Brainstem Response •  Behavioral testing

19

Behavioral Testing

•  Behavioral Observation – BO §  Presenting a sound and watching for

timely, consistent behavior(s) that may indicate audition • Eye shifts • Start/stop sucking on pacifier or bottle • Body/hand tensing • Crying/startling • Head turn

§ Not assessing true/absolute thresholds

20

Behavioral Testing

•  Visual Reinforcement Audiometry - VRA

21

Behavioral Testing

•  Conditioned Play Audiometry - CPA

22

Behavioral Testing

•  Standard testing

23

Results

•  Normal – hearing is adequate to support speech-language development

•  Abnormal/Hearing Loss – medical treatment and/or audiologic intervention likely recommended

•  Inconclusive

24

“Inconclusive”

•  The limited reliability of the responses obtained is unable to rule-out or confirm a hearing loss §  Further testing is necessary in order to be

certain of the accuracy of the results •  Very common in pediatric audiology that

testing is inconclusive and requires additional visits to determine diagnosis

25

Treatments

•  Ear Nose & Throat referrals §  Surveillance for early intervention to

maximize health and educational achievement (Barr et al., 2011)

• Anatomical differences (ear canal size) • Cerumen (wax) removal • Otitis media (ear infections) • Eustachian tube dysfunction (PE tube

placement)

26

Treatments

•  Hearing Aids §  Behind-the-Ear (BTE)

§  Bone conduction aid

§  Bone-anchored-hearing-aid (Baha)

§ Cochlear Implant (CI)

27

Communication

•  Communication can include: §  Touch §  Vision (facial expressions, eye contact) § Gestures (body language) §  Sound §  Sign Language

28

http://www.cdc.gov/ncbddd/hearingloss/freematerials/Communication_Brochure.pdf

Communication Strategies

•  Keep your face in view •  Speak clearly

§ Do not shout or exaggerate your mouth movements

•  Use effective clarification strategies § Rephrase or add contextual cues

•  Avoid background noise •  Be assertive, patient, and

accommodating

29

Parent Support

•  Hands & Voices §  Supporting families without a bias around

communication modes or methodology • Guide By Your Side • Advocacy Support • Training & Networking

§ www.handsandvoices.org •  Help for Hearing Aid Wearers

30

Questions??? Emily.nightengale@childrenscolorado.org

31