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Early Hearing Detection and Intervention The Role of the Primary Care Physician AAP CME Teleconferen


Types of Congenital/Early Onset Hearing Loss and Why It Is Important to Know the ... Degree: amount of hearing loss in relationship to normal auditory function ... – PowerPoint PPT presentation

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Title: Early Hearing Detection and Intervention The Role of the Primary Care Physician AAP CME Teleconferen

Early Hearing Detection and Intervention The
Role of the Primary Care PhysicianAAP CME
Teleconference, Part IOctober 15, 2003
Types of Congenital/Early Onset Hearing Loss and
Why It Is Important to Know the Difference
  • Gravel

Hearing Loss
  • Characterized broadly by degree, configuration
    and type,
  • Degree amount of hearing loss in relationship
    to normal auditory function
  • Configuration overall shape or pattern of the
    hearing loss as displayed on the conventional
    audiogram in dB HL as a function of frequency
  • Type site (location) of the auditory disorder

Degree Categories of HL
  • Normal
  • Borderline (Minimal)
  • Mild
  • Moderate
  • Moderate-severe
  • Severe
  • Profound
  • -10 to 15 dB HL
  • 16 to 25 dB HL
  • 26 to 40 dB HL
  • 41 to 55 dB HL
  • 56 to 70 dB HL
  • 71 to 90 dB HL
  • gt 90 dB HL

New York State Department of Health
Hearing Loss
  • Characterized broadly by degree, configuration
    and type,
  • Degree amount of hearing loss in relationship
    to normal auditory function
  • Configuration overall shape or pattern of the
    hearing loss as displayed on the conventional
    audiogram in dB HL as a function of frequency
  • Type site (location) of the auditory disorder

Configuration SlopingHigh frequency
thresholds gt20 dB poorer than low frequency
Frequency in Hz
Hearing Level (HL) in dB
Stach, 1998
Hearing Loss
  • Characterized broadly by degree, configuration
    and type,
  • Degree amount of hearing loss in relationship
    to normal auditory function
  • Configuration overall shape or pattern of the
    hearing loss as displayed on the conventional
    audiogram in dB HL as a function of frequency
  • Type site (location) of the auditory disorder

Types of Hearing LossConductive
  • Reduction of air-conductive sound delivered to
    the normal cochlea during transmission through a
    disordered outer ear and/or middle ear
  • Sound reaching cochlea attenuated to some degree
    (in OME or debris 25 dB HL in complete atresia,
    maximally 60 dB HL)
  • Excellent speech perception when incoming
    acoustic signal is sufficiently intense

Type of Hearing Loss Sensory
  • Damage to outer or outer and inner hair cells of
    the cochlea
  • Differing impact on speech perception depending
    on degree and configuration of hearing loss
  • Multiple audiometric configurations
  • Any degree of hearing loss

Types of Hearing LossMixed
  • Both sensory component and overlying conductive
  • Example child with sensory loss who experiences

Types of Auditory Disorders
  • Neural
  • Outer ear, middle ear and cochlea (OHCs) intact
  • Deficit in neural transmission (auditory
  • Central
  • Conductive, sensory neural pathway intact
  • Processing deficit at higher levels of the
    central nervous system

Hearing Loss Characterized by Ear(s) Affected
  • Bilateral
  • Unilateral (in one ear only)

What are the major genetic and environmental
causes of congenital hearing loss ?Vohr
Characteristics of Children with Hearing Loss
Site Rate Well Baby Nursery 1 per 1000 NICU 10
per 1000 Total population 2-3 per 1000
infants ident annually US 8,000-16,000 Average
career pediatrician 12 patients
JCIH Risk Factors for Infants birth to 28 days
  • Any illness requiring admission to the NICU for gt
    48 hours.
  • Stigmata associated with a syndrome known to
    include SNHL or conductive HL
  • Craniofacial anomalies including the pinna and
    ear canal
  • In utero infections including CMV, herpes,
    toxoplasmosis and rubella
  • Family history of permanent HL

Most Common Specific Environmental Risk Causes
CMV gt 1 risk factor Meningitis Perinatal
Asphyxia Prematurity lt 1500 g Ototoxic
Some Infants pass their hearing screen and have
late onset hearing loss
  • Some of these infants have a risk factor and some
    have no risk factor known to the family or

Risk indicators from 29 d to 2 years of age
  • Stigmata or Syndrome associated with HL
  • In-utero infections
  • Postnatal infections
  • Neonatal indicators such as ECMO
  • Neurodegenerative disorders
  • Head trauma
  • Recurrent or persistent OM

Risk Indicators obtained from the family
  • Parent or caregiver concern regarding hearing,
    speech, language, or developmental delay (parent
    concern has been shown to be a good predictor).
  • Family history of permanent HL in first or second
    degree relatives with onset by 30y or age.

Causes of Permanent Hearing Loss in 100 Infants
50 Environmental 50 50 Genetic 30 syndromes
(gt300) 30 20 gt75 genes ident 20 ½ are GJB2
Connexin 26
Genetic Causes
Single gene Connexin 26 Gene environment Mitocho
ndrial ototoxic Gene gene Gene other gene
The Connexin 26 Gene
  • It is estimated that 50-80 of all autosomal
    recessive congenital deafness may to due to
    mutations in th3e Cx-26 gene on chromosome
  • The Cx gene produces a gap junction protein
    expressed between the outer hair cells and
    supporting cells and is involved with auditory
  • The estimated carrier frequency in the general
    population is 1 in 31. (Estivill et al, 1998)

Genetic Testing
  • Obtaining an adequate sample for DNA testing is
    now quite easy
  • Bilateral buccal smears with a Q tip provide
    adequate genetic material for testing.
  • The follow-up genetic counseling is key to the
    success of genetic testing.

New technologies used in hearing screening
  • Gravel

Otoacoustic Emissions (OAE)
  • By-product of the active processing of healthy
  • Recording of an OAE
  • Indicates healthy OHCs (cochlea)
  • Presence highly correlated with normal hearing
    sensitivity or no greater than a mild hearing
  • Sensorineural hearing losses of greater than
    about 30 dB HL generally result in absent OAE.
  • Since recording OAE requires normal forward and
    backward transmission of energy to and from the
    cochlea, conductive hearing loss associated with
    middle or outer ear abnormality can result in
    absent OAE

Types of OAE
  • Spontaneous OAEs
  • Evoked OAEs
  • Transient OAE (TEOAE, TOAE, or click-OAE COAE)
  • Distortion Product (DPOAE)

  • Elicited by transients or brief stimuli
  • Clicks rapid onset, broadband stimulus
    containing energy from low through high
    frequencies (i.e., across speech frequency range)
  • Tone bursts more frequency-specific

Distortion-Product OAE (DPOAE)
  • Occur as a result of nonlinear processes of the
  • When 2 tones are presented to the cochlea,
    distortion occurs in the form of other tones
    (harmonics) that are not present in the 2
    eliciting tones

Screening Technologies - NeonatesEvoked
Otoacoustic Emissions
  • EOAE Advantages
  • Quick
  • Inexpensive
  • Frequency-specific
  • Identifies cochlear and conductive losses
  • Pass-refer screening devices available
  • EOAE Disadvantages
  • Sensitive to ear canal and middle ear conditions
  • Sensitive to noise (internal external)
  • Cannot identify neural disorders including
    auditory neuropathy
  • High fail rates in some programs.

Device Options for OAE Screening
  • Types
  • Handheld
  • Portable screening devices
  • PC-based hybrids
  • PC-based clinical systems

Auditory Brainstem Response (ABR)
  • Recording (through surface electrodes) of the
    micro-volt electrical activity generated by the
    cochlea and transmitted by the auditory nerve and
    brainstem pathways in response to brief clicks.
  • Clicks produce a synchronized response from
    neural fibers a tracing of the response is a
    series of waves

Screening Technologies NeonatesScreening
(Automated) Auditory Brainstem Response (SABR or
  • ABR Advantages
  • Identifies cochlear, conductive and neural
    losses ? mild-mod.
  • Pass-refer screening devices. Some test both ears
  • Relatively insensitive to transient ear canal,
    middle ear external noise.
  • ? Lower fail rate than OAE
  • ABR Disadvantages
  • Test time
  • Disposable costs
  • Infant state/myogenic artifact
  • Requires electrode prep, placement removal
  • Click can miss unusual configurations of HL

Examples of screening ABR technology
In-Hospital ScreeningTwo Technology Protocol
  • OAE ABR Advantages
  • Low fail rate
  • Depending on test order, identifies cochlear,
    neural and conductive losses
  • Reduced effects of noise
  • Pass-refer screening devices available for both
  • OAE ABR Disadvantages
  • Time
  • Equipment and disposable costs

Why is diagnostic confirmation by an audiologist
skilled in evaluating infants and young children
  • A failed hearing screen may be a false positive
    or an actual fail. These 2 findings need totally
    different management.
  • Therefore, an accurate diagnosis of normal
    hearing, SNHL, auditory neuropathy or conductive
    loss is important as soon as possible to
    minimize parental stress and to decrease the time
    interval between screen fail and starting
  • Parents of late identified children have feelings
    of guilt and frustration.

Early Identification of Hearing Loss is Important
  • Delayed identification, even of mild HL results
  • language delays
  • developmental skill delays, and
  • behavior problems.
  • Subsequent delays in literacy, and academic

Reading Comprehension Scores of Hearing and Deaf
Grade Equivalents
Age in Years
Deaf Children in America 1986
Unilateral or Mild Loss
  • 50 of children either repeat a grade or need
    resource support in school
  • Increased behavioral and linguistic problems
    compared to hearing controls.
  • Bess F,
    Pediatrics 1984

Early Early Intervention for Hearing Loss is
Important because
  • There are dramatic benefits associated with early
    identification and intervention for hearing loss
    before 6 months of age.
  • Children identified and receiving services lt 6 m
    have larger vocabularies, better comprehension
    and better expressive language than children
    identified gt 6 m.

Are Interventions Available to Improve Outcome?
White - language scores of sev to profound

(14 vs 26 m) Apizzo - better language scores at
4 if ident lt2 m Moeller - 100 D/HH children with
early ident - better
outcomes Yoshinago-Itano -
better scores at 36 m if ident lt6 m Early Early
Intervention is better !!!!
AAP JCIH Recommendations Components of EHDI
Programs in the US
  • Universal Newborn Hearing Screeninglt 1 m
  • Effective Tracking and Follow-up as a part of the
    Public Health System
  • Appropriate and Timely Diagnosis of the HL lt 3m
  • Prompt Enrollment in Appropriate EI lt 6m
  • All infants will have a medical home

Importance of Diagnostic Audiologic Confirmation
of Hearing Loss
  • Gravel

Gravel, 2000 Gravel Hood, 1998
Audiologists should have experience with the
assessment of infants children with HL and the
knowledge and equipment necessary for use with
current pediatric assessment methods.
Facilities that lack the expertise or
equipment for assessing infants children
should establish consortial arrangements with
those that do.
Pediatric Working Group, 1996
What are the components of the medical home
work-up for children with congenital hearing
EHDI and the Medical Home
Hospital Screening
Parent Groups Mental Health
Primary Provider
3rd Party Payors
Deaf Community Interpreters
EI Therapists
Deaf Services
The Medical Workup
  • Complete prenatal perinatal hx
  • Family Hx of onset of HL lt age 30
  • Physical for stigmata, ear tabs, cleft palate,
    cardiac, skeletal, microcephaly
  • Refer to ENT / CT of temporal bones
  • Refer to Genetics and Opthalmology
  • Other CMV, EKG

What are some of the questions to ask ?
  • Antenatal history- maternal illness during the
    pregnancy or delivery
  • Neonatal complications, prematurity, jaundice,
    asphyxia, assisted ventilation, ECMO

Examination for Causes
  • Evaluate for dysmorphic features, minor and major
    stigmata and syndromes
  • Other anomalies visual, facial, endocrine,
    cardiac, kidney, hair, and skin
  • Particular attention to the head and neck. HL may
    be associated with abn. pinna, atresia or
    stenosis of the ear canal, ear tags, and bony
    growths in the ear canal.
  • Cleft lip and palate may have middle ear fluid

What to ask about family history ?
  • Is there a family hx of onset of permanent HL lt
    30 years of age ( over 3 generations)
  • Are there other family members with syndromes or
    anomalies ?

Which families may benefit from a Genetic
Referral ?
  • All families with a child with congenital or late
    onset hearing loss
  • Families of a child with stigmata or a syndrome
    will benefit from the information.
  • Families with a child with non-syndromic HL want
    to know the cause ?
  • Some parents who are culturally deaf wish
    information on the risk or cause of HL.

When to refer to Ophthalmology
  • First- Follow periodicity schedule for all
  • Some syndromes with permanent HL have specific
    eye findings such as heterochromia in
  • In Ushers the child is at risk of late onset
    vision loss secondary to retinitis pigmentosa.
    (If sign is the primary communication mode they
    will obviously have a problem.

When to get an EKG ?
  • This is ordered to rule out long QT ( Jervell and
    Lange-Nielsen )syndrome.
  • This syndrome may manifest itself with apnea,
    passing out episodes, or a history of sudden
    death in a relative.

Should there be additional workup ?
  • This needs to be individualized.
  • For example, A NICU infant with IUGR should have
    an MRI and TORCH titers to rule out CMV,
    toxoplasmosis etc.

Children with Cochlear Implants and Meningitis
  • The incidence of meningitis is higher among
    children with cochlear implants.
  • The incidence of Streptococcus pneumoniae
    meningitis was 30 times the incidence in the
    general population. NEJM, July 2003

Recommendations for children with Cochlear
  • Children lt age 2 years should get pneumococcal
    conjugate vaccine (Prevnar) according to the
    routine schedules
  • There are guidelines for older children (CDC)
  • Children with cochlear transplants should be
    monitored and treated promptly for any bacterial

Amplification Choices for Families including
Hearing Aids, FM Systems and Cochlear Implantation
  • Gravel

What is the Goal of Hearing Aid Fitting
  • Ensure children receive full-time use and
    consistent audibility of the speech signal at
    safe and comfortable listening levels as soon as
    hearing loss is confirmed.
  • PWG, 1996

When are Hearing Aids Fit?
For newborns identified by UNHS, fit hearing
aids within one month of confirmation of hearing
loss, preferably before 4 months and no later
than 6 months of age. JCIH, 2000
Which infants are candidates for amplification?
  • Significant, permanent bilateral peripheral
    hearing loss.
  • Mild hearing loss in some cases
  • Some children with unilateral hearing loss, and
    minimal HL
  • Need based on audiogram plus additional
  • Family choice
  • other disabilities/on-going medical issues
  • performance

PWG, 1996
Pre-Selection - Childrens Hearing Aids Should
  • Binaural fitting unless clear contraindication
  • Behind-the-ear style of choice

PWG, 1996
Pre-Selection - Childrens Hearing Aids Should
  • Flexible response characteristics
  • Compression to limit overall output sound
    pressure level of the hearing aid (safety and

PWG, 1996
Directional Microphone Technology
  • Improves directional hearing abilities
  • Hear parent speaking from front noise from
    shopping mall at back of child reduced.

Multiple Memory Hearing Instruments
  • Allows storage of more than one electroacoustic
    response setting
  • Allows switching between memories for various
    listening situations

Digital Signal Processing (DSP) Technology
  • Newer hearing aids that use digital processing of
  • No studies, to date, demonstrate better
    performance of digital instruments over
    conventional hearing aids in either adults or
  • Considerably more expensive

What Other Features Should Childrens Hearing
Aids Include?
  • Safety-related features tamper resistant
  • battery compartment
  • volume control
  • Physical fit (size) and color
  • Earmolds made of soft material

PWG, 1996
How Do We Fit Hearing Aids to Infants Young
  • Use computerized prescriptive fitting procedure
  • Requires only minimal threshold data to begin, so
    fitting can begin early.

From Seewald, 2003
Prescriptive Fitting Procedure
  • Incorporate simple probe microphone measurements
  • Allow audiologist to
  • Pre-select the response characteristics of the
    hearing aids
  • Refine or individualize the hearing aid for the
    unique acoustic characteristics of each infants
  • Verify that the prescriptive frequency-gain and
    output targets have been achieved

From www.babyhearing.org
Goals of FM Fitting
  • Child hears primary talker at level that is
    consistently audible above the background noise
  • Child able to monitor his/her own voice
  • Child hears voices of others who are not wearing
    the FM microphone

Lewis et al., 1998 (Phonak AG)
Why Does the Acoustic Climate (of the Home,
Daycare Setting, etc.) Need to be Considered?
  • Infants and young children with congenital/early
    onset hearing loss
  • Are learning language for the first time
  • Have greater difficulty understanding speech in
    background noise than adults
  • Require a more audible (intense) signal than
    adults to understand speech

Fail Neonatal Screening
1m 2m 3m 4m 5m 6m 7m 8m 9m 10m 11m 12m
Fail Outpatient screening FS-ABR,EOAE AC BC,
tymps Repeat FS-ABR, EOAE,RECD with insert, for
HA selection, tymps Behavioral tymps (with
mold to insert coupling)
Counseling medical/ENT referral Begin processes
for HA procurement
Mold impressions, EI Program
HA Fitting
Observe auditory behaviors tymps
HA Check (molds) Review habilitation, language
RECD, HA modification, (molds) Review
habilitation, language milestones
Behavioral tymps (with mold-to-insert coupling)
Behavioral tymps (with mold-to-insert coupling)
RECD, HA modification, (molds) Review
habilitation, language milestones
Behavioral tymps (with mold-to-insert coupling)
RECD, HA Check, (molds) Validation measures,
language milestones Set habilitation goals for
year 2
Gravel, 2000
Qualifications of Audiologists and Facilities
  • No facility should fit hearing aids to children
    if it lacks the equipment for behavioral,
    electrophysiologic, electroacoustic, and
    probe-microphone/real-ear evaluation.
  • Facilities that lack the expertise or equipment
    should establish consortial
  • arrangements with centers that do.

PWG, 1996
Considerations in Determining the Appropriateness
of Cochlear Implantation including Risks,
Benefits Timing
  • Gravel

What are the Candidacy Criteria for a Cochlear
From www.babyhearing.org
  • Limited benefit from conventional amplification
    following a minimum of 3-6 months use
  • May be sooner following deafness from meningitis
  • Profound hearing loss
  • 12-18 months
  • Severe-to-Profound hearing loss
  • gt18 months
  • Motivated, involved family with child enrolled in
    an intervention program emphasizing spoken
    (oral-aural) language development

Cochlear Implants
  • Acoustic signal picked up by microphone located
    in headset worn at ear level
  • Cord carries sound from microphone to a speech
  • Speech processor digitizes sound into coded
  • Coded signals sent up to the transmitting coil
  • Coil sends coded signals as FM radio signals to
    CI under the skin
  • CI delivers electrical energy to the electrode
    array inserted into the cochlea
  • Electrodes along the array stimulate remaining
    nerve fibers in cochlea

Cochlear Corp. 2002
Selecting a Cochlear Implant Center
  • Experienced cochlear implant team
  • Audiologist, speech-language pathologist, surgeon
  • Others educator of the deaf, psychologist,
    social worker
  • Comprehensive program covering eligibility,
    surgery, device activation, and long-term
  • Multi-disciplinary, family centered approach.
  • Knowledgeable regarding deafness, child
    development, and speech, language and auditory
  • Offers intervention program and continued
  • Follow-up for changes in cochlear implant mapping

  • Similar to early amplification provision,
    children implanted at early ages with more
    experience tend to do better than older children
    who receive implants after greater period of

Listening to Parental Concern About Delayed
Language Development
  • Gravel Vohr

Listening to parent concern about language
  • Parent concerns about hearing, speech, language,
    or developmental delays are strong predictors of
    an actual problem.
  • Providers must avoid statements like Babies
    develop at different rates. Lets take another
    look in about 6 months

Clinical signs of Hearing Loss
  • Delayed early language milestones
  • Unintelligible speech
  • Uncharacteristic voice patterns
  • Child turns TV volume very loud
  • Child only responds to loud sounds/words or in a
    very quiet environment

Myths about hearing and early speech language
  • We dont have to worry because
  • His older brothers and sisters talk for him
  • Boys develop speech much later than girls
  • Twins always have language delays
  • Grandma says that her Dad did not speak until
    he was 3 years old.
  • She has great motor milestones
  • I know he hears because he gets upset every
    time I turn on the vacuum.

Assessment of language delay
  • Administer a speech language screen
  • Check middle ear status for MEE Rx
  • MEE for gt 3 m refer to otolaryngology
  • If receptive /expressive delay refer back to
    audiology for repeat diagnostic
  • Refer to early intervention for speech language
  • Assess for other possible dx PDD, autism
  • Continue to follow-up on speech/language

It is important to respond to concerns about
language immediately !
  • Most children with delays of speech and language
    respond to appropriate medical, audiologic, and
    educational interventions.
  • A successful early screening, identification,
    and intervention program will ultimately permit
    every child with HL to develop to his/her

  • All in-hospital screening failures should receive
  • Failure rate at discharge, once high has
    decreased to 2 or less.
  • Probability 1 in 5 that infant who fails NHS has
    hearing loss
  • Regardless of screening outcome, if parent is
    concerned regarding hearing or speech-language
    development, child should be referred for
    audiologic evaluation

Early Hearing Detection and Intervention CME
Teleconference Series, Part IINovember 12,
20031200 100 pm, Central Standard Time
  • Topics
  • Implementing newly developed AAP guidelines
    important referrals the role of early
    intervention primary cares role in coordination
    of services parenting issues and reactions
    knowledge of and support for communication
    choices cost and reimbursement issues and
    national resources.
  • Faculty Al Mehl and Mary Pat Moeller
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