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
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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.

Critical Period for Language Development

A hypothesis that suggests the early
years of a childs life are the sensitive period for language development Following this critical period, language acquisition is more challenging.

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)

The Audiogram

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)

Different Types of Hearing Loss

Sensorineural Hearing Loss
Family History Noise-induced Structural abnormalities

Considered permanent Audiologic management options usually

discussed when identified

Different Types of Hearing Loss

Mixed Hearing Loss Auditory Neuropathy
Risk factors include prematurity, hypoxia (respiratory distress requiring mechanical ventilation), and hyperbilirubinemia

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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

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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

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Incidence of Hearing Loss in Population with DS

May be as high as 78% 34.1% (n=45 infants) in the first year of life,
85% of which is conductive
(Raut, et al., 2011) (Dahle, AJ et al., 1986; Balkany T, 1979)

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

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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)

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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

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What to expect at an appointment

Otoscopy Tympanometry Otoacoustic Emissions Natural sleep or Sedated Auditory
Brainstem Response Behavioral testing

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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
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Behavioral Testing
Visual Reinforcement Audiometry - VRA

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Behavioral Testing
Conditioned Play Audiometry - CPA

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Behavioral Testing
Standard testing

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Results
Normal hearing is adequate to support
speech-language development Abnormal/Hearing Loss medical treatment and/or audiologic intervention likely recommended Inconclusive

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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

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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)

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Treatments
Hearing Aids
Behind-the-Ear (BTE) Bone conduction aid Bone-anchored-hearing-aid (Baha) Cochlear Implant (CI)

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

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

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Questions???
Emily.nightengale@childrenscolorado.org

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