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

1
Early Hearing Detection and Intervention The
Role of the Primary Care PhysicianAAP CME
Teleconference, Part IOctober 6, 2004
2
Types of Congenital/Early Onset Hearing Loss and
Why It Is Important to Know the Difference

• Gravel

3
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

4
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
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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

6
Configuration SlopingHigh frequency
thresholds gt20 dB poorer than low frequency
Frequency in Hz
Hearing Level (HL) in dB
Stach, 1998
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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

8
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

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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

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Types of Hearing LossMixed

• Both sensory component and overlying conductive
  component
• Example child with sensory loss who experiences
  OME

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Types of Auditory Disorders

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

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Hearing Loss Characterized by Ear(s) Affected

• Bilateral
• Unilateral (in one ear only)

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What are the major genetic and environmental
causes of congenital hearing loss ?Vohr
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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
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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

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Most Common Specific Environmental Risk Causes
CMV gt 1 risk factor Meningitis Perinatal
Asphyxia Prematurity lt 1500 g Ototoxic
medications
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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
  physician.

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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

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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.

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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
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Genetic Causes
Single gene Connexin 26 Gene environment Mitocho
ndrial ototoxic Gene gene Gene other gene
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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
  13q11-q12
• The Cx gene produces a gap junction protein
  expressed between the outer hair cells and
  supporting cells and is involved with auditory
  transduction.
• The estimated carrier frequency in the general
  population is 1 in 31. (Estivill et al, 1998)

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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.

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New technologies used in hearing screening

• Gravel

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Otoacoustic Emissions (OAE)

• By-product of the active processing of healthy
  OHC
• Recording of an OAE
• Indicates healthy OHCs (cochlea)
• Presence highly correlated with normal hearing
  sensitivity or no greater than a mild hearing
  loss
• 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

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Types of OAE

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

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TEOAE

• 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

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Distortion-Product OAE (DPOAE)

• Occur as a result of nonlinear processes of the
  cochlea
• 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

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Screening Technologies - NeonatesEvoked
Otoacoustic Emissions

• EOAE Advantages
• Quick
• Inexpensive
• Frequency-specific
• Identifies cochlear and conductive losses
  mild-mod?
• 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.

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Device Options for OAE Screening

• Types
• Handheld
• Portable screening devices
• PC-based hybrids
• PC-based clinical systems

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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

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Screening Technologies NeonatesScreening
(Automated) Auditory Brainstem Response (SABR or
AABR)

• ABR Advantages
• Identifies cochlear, conductive and neural
  losses ? mild-mod.
• Pass-refer screening devices. Some test both ears
  simultaneously.
• 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

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Examples of screening ABR technology
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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
  technologies

• OAE ABR Disadvantages
• Time
• Equipment and disposable costs

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Why is diagnostic confirmation by an audiologist
skilled in evaluating infants and young children
important?Vohr
36

• 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
  treatment.
• Parents of late identified children have feelings
  of guilt and frustration.

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Early Identification of Hearing Loss is Important
because

• Delayed identification, even of mild HL results
  in
• language delays
• developmental skill delays, and
• behavior problems.
• Subsequent delays in literacy, and academic
  performance

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Reading Comprehension Scores of Hearing and Deaf
Students
Grade Equivalents
Age in Years
Deaf Children in America 1986
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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

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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.

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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 !!!!
42
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

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Importance of Diagnostic Audiologic Confirmation
of Hearing Loss

• Gravel

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Gravel, 2000 Gravel Hood, 1998
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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
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What are the components of the medical home
work-up for children with congenital hearing
loss?Vohr
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EHDI and the Medical Home
Hospital Screening
Audiology
Parent Groups Mental Health
Primary Provider
Child/Family
ENT
3rd Party Payors
Deaf Community Interpreters
EI Therapists
Genetics
Deaf Services
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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

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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

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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

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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 ?

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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.

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When to refer to Ophthalmology

• First- Follow periodicity schedule for all
  patients
• Some syndromes with permanent HL have specific
  eye findings such as heterochromia in
  Wardenburgs.
• 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.

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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.

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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.

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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

57
Recommendations for children with Cochlear
Implants

• 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
  infections.

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

• Gravel

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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
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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
  information
• Family choice
• other disabilities/on-going medical issues
• performance

PWG, 1996
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Pre-Selection - Childrens Hearing Aids Should
Include

• Binaural fitting unless clear contraindication
• Behind-the-ear style of choice

PWG, 1996
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Pre-Selection - Childrens Hearing Aids Should
Include

• Flexible response characteristics
• Compression to limit overall output sound
  pressure level of the hearing aid (safety and
  comfort)

PWG, 1996
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Directional Microphone Technology

• Improves directional hearing abilities
• Hear parent speaking from front noise from
  shopping mall at back of child reduced.

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Multiple Memory Hearing Instruments

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

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Digital Signal Processing (DSP) Technology

• Newer hearing aids that use digital processing of
  incoming
• No studies, to date, demonstrate better
  performance of digital instruments over
  conventional hearing aids in either adults or
  children
• Considerably more expensive

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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
67
How Do We Fit Hearing Aids to Infants Young
Children?

• Use computerized prescriptive fitting procedure
• Requires only minimal threshold data to begin, so
  fitting can begin early.

From Seewald, 2003
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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
  ear
• Verify that the prescriptive frequency-gain and
  output targets have been achieved

From www.babyhearing.org
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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)
70
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

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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
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
milestones
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
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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
73
Considerations in Determining the Appropriateness
of Cochlear Implantation including Risks,
Benefits Timing

• Gravel

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What are the Candidacy Criteria for a Cochlear
Implant?
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

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Cochlear Implants

• Acoustic signal picked up by microphone located
  in headset worn at ear level
• Cord carries sound from microphone to a speech
  processor
• Speech processor digitizes sound into coded
  signals
• 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
76
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
  habilitation.
• Multi-disciplinary, family centered approach.
• Knowledgeable regarding deafness, child
  development, and speech, language and auditory
  development
• Offers intervention program and continued
• Follow-up for changes in cochlear implant mapping

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Benefits

• 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
  deafness

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Listening to Parental Concern About Delayed
Language Development

• Gravel Vohr

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Listening to parent concern about language
development

• 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

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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

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Myths about hearing and early speech language
delay

• 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.

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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
  therapy
• Assess for other possible dx PDD, autism
• Continue to follow-up on speech/language

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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
  potential.

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• All in-hospital screening failures should receive
  follow-up
• 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

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Question-and-Answer
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Early Hearing Detection and Intervention CME
Teleconference Series, Part IINovember 17,
20041200 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