Emily Nightengale and Megan Hedman - The Importance of Hearing Loss in Children With Down Syndrome -...
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Transcript of Emily Nightengale and Megan Hedman - The Importance of Hearing Loss in Children With Down Syndrome -...
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.
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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.
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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)
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The Audiogram
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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)
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Different Types of Hearing Loss • Sensorineural Hearing Loss
§ Family History § Noise-induced § Structural abnormalities
• Considered permanent • Audiologic management options usually
discussed when identified
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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% (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
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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
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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
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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??? [email protected]
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