Title: Early Hearing Detection and Intervention The Role of the Primary Care Physician AAP CME Teleconferen
1Early Hearing Detection and Intervention The
Role of the Primary Care PhysicianAAP CME
Teleconference, Part IOctober 15, 2003
2Types of Congenital/Early Onset Hearing Loss and
Why It Is Important to Know the Difference
3Hearing 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
4Degree 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
5Hearing 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
7Hearing 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
8Types 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
9Type 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
10Types of Hearing LossMixed
- Both sensory component and overlying conductive
component - Example child with sensory loss who experiences
OME
11Types 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
12Hearing Loss Characterized by Ear(s) Affected
- Bilateral
- Unilateral (in one ear only)
13What are the major genetic and environmental
causes of congenital hearing loss ?Vohr
14Characteristics 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
15JCIH 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
16Most Common Specific Environmental Risk Causes
CMV gt 1 risk factor Meningitis Perinatal
Asphyxia Prematurity lt 1500 g Ototoxic
medications
17Some 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.
18 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
19Risk 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.
20Causes 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
21Genetic Causes
Single gene Connexin 26 Gene environment Mitocho
ndrial ototoxic Gene gene Gene other gene
22The 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)
23Genetic 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.
24New technologies used in hearing screening
25Otoacoustic 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
26Types of OAE
- Spontaneous OAEs
- Evoked OAEs
- Transient OAE (TEOAE, TOAE, or click-OAE COAE)
- Distortion Product (DPOAE)
27TEOAE
- 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
28Distortion-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
29Screening 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.
30Device Options for OAE Screening
- Types
- Handheld
- Portable screening devices
- PC-based hybrids
- PC-based clinical systems
31Auditory 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
32Screening 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
33Examples of screening ABR technology
34In-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
35Why 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.
37Early 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
38Reading Comprehension Scores of Hearing and Deaf
Students
Grade Equivalents
Age in Years
Deaf Children in America 1986
39Unilateral 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
40 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.
41Are 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 !!!!
42AAP 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
43Importance of Diagnostic Audiologic Confirmation
of Hearing Loss
44 Gravel, 2000 Gravel Hood, 1998
45Audiologists 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
46What are the components of the medical home
work-up for children with congenital hearing
loss?Vohr
47EHDI 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
48The 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
49What are some of the questions to ask ?
- Antenatal history- maternal illness during the
pregnancy or delivery - Neonatal complications, prematurity, jaundice,
asphyxia, assisted ventilation, ECMO
50Examination 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
51What 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 ?
52Which 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.
53When 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.
54When 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.
55Should 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.
56Children 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
57Recommendations 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.
58Amplification Choices for Families including
Hearing Aids, FM Systems and Cochlear Implantation
59What 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
60Which 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
61Pre-Selection - Childrens Hearing Aids Should
Include
- Binaural fitting unless clear contraindication
- Behind-the-ear style of choice
PWG, 1996
62Pre-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
63Directional Microphone Technology
- Improves directional hearing abilities
- Hear parent speaking from front noise from
shopping mall at back of child reduced.
64Multiple Memory Hearing Instruments
- Allows storage of more than one electroacoustic
response setting - Allows switching between memories for various
listening situations
65Digital 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
66What 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
67How 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
68Prescriptive 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
69Goals 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)
70Why 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
71Fail 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
72Qualifications 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
73Considerations in Determining the Appropriateness
of Cochlear Implantation including Risks,
Benefits Timing
74What 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
75Cochlear 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
76Selecting 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
77Benefits
- 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
78Listening to Parental Concern About Delayed
Language Development
79Listening 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
80Clinical 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
81Myths 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.
82Assessment 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
83It 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.
84- 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
85Question-and-Answer
86Early 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