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Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003. Types of Congenital/Early Onset Hearing Loss and Why It Is Important to Know the Difference. Gravel. Hearing Loss.

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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

Types of Congenital/Early Onset Hearing Loss and Why It Is Important to Know the Difference


hearing loss
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

Borderline (Minimal)






-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

> 90 dB HL

Degree: Categories of HL

New York State Department of Health

hearing loss5
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 sloping high frequency thresholds 20 db poorer than low frequency
Configuration:SlopingHigh frequency thresholds >20 dB poorer than low frequency

Frequency in Hz

Hearing Level (HL) in dB

Stach, 1998

hearing loss7
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 loss conductive
Types of Hearing Loss:Conductive
  • 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
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 loss mixed
Types of Hearing Loss:Mixed
  • Bothsensory component and overlying conductive component
  • Example: child with sensory loss who experiences OME
types of auditory disorders
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
hearing loss characterized by ear s affected
Hearing Loss: Characterized by Ear(s) Affected
  • Bilateral
  • Unilateral (in one ear only)
characteristics of children with hearing loss
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
JCIH Risk Factors for Infants birth to 28 days
  • Any illness requiring admission to the NICU for > 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
Most Common Specific Environmental Risk Causes

CMV > 1 risk factor

Meningitis Perinatal Asphyxia

Prematurity < 1500 g Ototoxic medications

some infants pass their hearing screen and have late onset hearing loss

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.

risk indicators from 29 d to 2 years of age
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
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
Causes of Permanent Hearing Loss in 100 Infants

50% Environmental 50

50% Genetic

30% syndromes (>300) 30

20% >75 genes ident 20

½ are GJB2 – Connexin 26

genetic causes
Genetic Causes

Single gene Connexin 26

Gene + environment Mitochondrial + ototoxic

Gene + gene Gene + other gene

the connexin 26 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 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)
genetic testing
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.
otoacoustic emissions oae
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
types of 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
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
screening technologies neonates evoked otoacoustic emissions
EOAE Advantages:




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.

Screening Technologies - NeonatesEvoked Otoacoustic Emissions
device options for oae screening
Device Options for OAE Screening


  • Handheld
  • Portable screening devices
  • PC-based hybrids
  • PC-based clinical systems
auditory brainstem response abr
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 neonates screening 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

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

Examples of screening

ABR technology

in hospital screening two 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:


Equipment and disposable costs

In-Hospital Screening:Two Technology Protocol
Why is diagnostic confirmation by an audiologist skilled in evaluating infants and young children important?Vohr
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.

early identification of hearing loss is important because
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
reading comprehension scores of hearing and deaf students
Reading Comprehension Scores of Hearing and Deaf Students

Grade Equivalents

Age in Years

Deaf Children in America 1986

unilateral or mild loss
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
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 < 6 m have larger vocabularies, better comprehension and better expressive language than children identified > 6 m.
are interventions available to improve outcome
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 <2 m

Moeller - 100 D/HH children with early ident - better outcomes

Yoshinago-Itano - better scores at 36 m if ident <6 m

Early “Early Intervention” is better !!!!

aap jcih recommendations components of ehdi programs in the us
AAP & JCIH Recommendations Components of EHDI Programs in the US
  • Universal Newborn Hearing Screening< 1 m
  • Effective Tracking and Follow-up as a part of the Public Health System
  • Appropriate and Timely Diagnosis of the HL < 3m
  • Prompt Enrollment in Appropriate EI < 6m
  • All infants will have a medical home

“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 loss vohr
What are the components of the medical home work-up for children with congenital hearing loss?Vohr

EHDI and the Medical Home

Hospital Screening


Parent Groups Mental Health

Primary Provider Child/Family


3rd Party Payors

Deaf Community


EI Therapists


Deaf Services

the medical workup
The Medical Workup
  • Complete prenatal & perinatal hx
  • Family Hx of onset of HL < 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
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
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
What to ask about family history ?
  • Is there a family hx of onset of permanent HL < 30 years of age ( over 3 generations)
  • Are there other family members with syndromes or anomalies ?
which families may benefit from a genetic referral
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
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.
when to get an ekg
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
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
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 implants
Recommendations for children with Cochlear Implants
  • Children < 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.
amplification choices for families including hearing aids fm systems and cochlear implantation

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



What is the Goal of

Hearing Aid Fitting

When are Hearing Aids Fit?

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

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 information:

Family choice

other disabilities/on-going medical issues


Which infants are candidates for amplification?

PWG, 1996

pre selection children s hearing aids should include
Pre-Selection - Children’s Hearing Aids Should Include:
  • Binaural fitting unless clear contraindication
  • Behind-the-ear style of choice

PWG, 1996

pre selection children s hearing aids should include62
Pre-Selection - Children’s Hearing Aids Should Include:
  • Flexible response characteristics
  • Compression to limit overall output sound pressure level of the hearing aid (safety and comfort)

PWG, 1996

directional microphone technology
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
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
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
what other features should children s hearing aids include
What Other Features Should Children’s 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 children
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

prescriptive fitting procedure
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 infant’s ear
    • Verify that the prescriptive frequency-gain and output targets have been achieved


goals of fm fitting
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
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

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

Observe auditory behaviors & tymps

HA Fitting

HA Check & (molds)

Review habilitation, language milestones


HA modification, (molds)

Review habilitation, language milestones

Behavioral & tymps

(with mold-to-insert coupling)

Behavioral & tymps

(with mold-to-insert coupling)


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
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


what are the candidacy criteria for a cochlear implant
What are the Candidacy Criteria for a Cochlear Implant?


  • 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
    • >18 months
  • Motivated, involved family with child enrolled in an intervention program emphasizing spoken (oral-aural) language development
cochlear implants
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

selecting a cochlear implant center
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
  • 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
listening to parent concern about language development
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”
clinical signs of hearing loss
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 delay
Myths about hearing and early speech language delay
  • We don’t 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
Assessment of language delay
  • Administer a speech language screen
  • Check middle ear status for MEE: Rx
  • MEE for > 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
it is important to respond to concerns about language immediately
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.

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

Early Hearing Detection and Intervention CME Teleconference Series, Part IINovember 12, 200312:00 – 1:00 pm, Central Standard Time


Implementing newly developed AAP guidelines; important referrals; the role of early intervention; primary care’s 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