Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003.

Early Hearing Detection and Intervention – Early Hearing Detection and Intervention – The Role of the Primary Care PhysicianThe Role of the Primary Care Physician

AAP CME Teleconference, Part IAAP CME Teleconference, Part IOctober 15, 2003October 15, 2003

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

Know the DifferenceKnow the Difference

GravelGravel

Hearing LossHearing Loss

Characterized broadly by Characterized broadly by degreedegree, , configuration configuration and and typetype, , – Degree: amount of hearing loss in relationship Degree: amount of hearing loss in relationship

to normal auditory functionto normal auditory function– Configuration: overall ‘shape’ or pattern of the Configuration: overall ‘shape’ or pattern of the

hearing loss as displayed on the conventional hearing loss as displayed on the conventional audiogram in dB HL as a function of frequencyaudiogram in dB HL as a function of frequency

– Type: site (location) of the auditory disorderType: site (location) of the auditory disorder

Degree: Categories of HLDegree: Categories of HL

• NormalNormal• Borderline (Minimal)Borderline (Minimal)• MildMild• ModerateModerate• Moderate-severeModerate-severe• SevereSevere• ProfoundProfound

• -10 to 15 dB HL-10 to 15 dB HL• 16 to 25 dB HL16 to 25 dB HL• 26 to 40 dB HL26 to 40 dB HL• 41 to 55 dB HL41 to 55 dB HL• 56 to 70 dB HL56 to 70 dB HL• 71 to 90 dB HL71 to 90 dB HL• > 90 dB HL> 90 dB HL

New York State Department of Health






-10

0

10

20

30

40

50

60

70

80

90

100

110

.25k .5k 1k 2k 4k 8k

Configuration:Configuration: SlopingSlopingHigh frequency thresholds >20 dB poorer than low frequencyHigh frequency thresholds >20 dB poorer than low frequency

Hea

ring

Leve

l (H

L) in

dB

Frequency in Hz

Stach, 1998






Types of Hearing Loss:Types of Hearing Loss:ConductiveConductive

• Reduction of air-conductive sound delivered to the Reduction of air-conductive sound delivered to the normal cochlea during transmission through a normal cochlea during transmission through a disordered disordered outer earouter ear and/or and/or middle earmiddle ear

• Sound reaching cochlea Sound reaching cochlea attenuated attenuated to some degree to some degree (in OME or debris ~25 dB HL; in complete (in OME or debris ~25 dB HL; in complete atresia, maximally ~60 dB HL)atresia, maximally ~60 dB HL)

• Excellent speech perception when incoming Excellent speech perception when incoming acoustic signal is sufficiently intenseacoustic signal is sufficiently intense

Type of Hearing Loss: Type of Hearing Loss: SensorySensory

• Damage to outer or outer and inner hair cells Damage to outer or outer and inner hair cells of the cochleaof the cochlea

• Differing impact on speech perception Differing impact on speech perception depending on degree and configuration of depending on degree and configuration of hearing losshearing loss

• Multiple audiometric configurations Multiple audiometric configurations

• Any degree of hearing lossAny degree of hearing loss

Types of Hearing Loss:Types of Hearing Loss:MixedMixed

• BothBoth sensorysensory component and overlying component and overlying conductiveconductive component component

• Example: child with sensory loss who Example: child with sensory loss who experiences OMEexperiences OME

• Neural Neural – Outer ear, middle ear and cochlea (OHCs) intactOuter ear, middle ear and cochlea (OHCs) intact– Deficit in neural transmission (auditory Deficit in neural transmission (auditory

neuropathy)neuropathy)

• CentralCentral– Conductive, sensory & neural pathway intact Conductive, sensory & neural pathway intact – Processing deficit at higher levels of the central Processing deficit at higher levels of the central

nervous systemnervous system

Types of Auditory Disorders:Types of Auditory Disorders:

• BilateralBilateral

• Unilateral (in one ear only)Unilateral (in one ear only)

Hearing Loss: Hearing Loss: Characterized by Ear(s) AffectedCharacterized by Ear(s) Affected

What are the major genetic and What are the major genetic and environmental causes of congenital environmental causes of congenital

hearing loss ?hearing loss ?

VohrVohr

Characteristics of Children with Hearing LossCharacteristics 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 daysJCIH Risk Factors for Infants birth to 28 days

• Any illness requiring admission to the NICU for Any illness requiring admission to the NICU for >> 48 hours. 48 hours.

• Stigmata associated with a syndrome known to Stigmata associated with a syndrome known to include SNHL or conductive HLinclude SNHL or conductive HL

• Craniofacial anomalies including the pinna and Craniofacial anomalies including the pinna and ear canalear canal

• In utero infections including CMV, herpes, In utero infections including CMV, herpes, toxoplasmosis and rubellatoxoplasmosis and rubella

• Family history of permanent HLFamily history of permanent HL

Most Common Specific Environmental Risk CausesMost 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 Some Infants pass their hearing screen and have late onset hearing losslate onset hearing loss

Some of these infants have a risk Some of these infants have a risk factor and some have no risk factor factor and some have no risk factor known to the family or physician.known to the family or physician.

Risk indicators from 29 d to 2 years of ageRisk indicators from 29 d to 2 years of age

• Stigmata or Syndrome associated with HLStigmata or Syndrome associated with HL

• In-utero infectionsIn-utero infections

• Postnatal infectionsPostnatal infections

• Neonatal indicators such as ECMONeonatal indicators such as ECMO

• Neurodegenerative disordersNeurodegenerative disorders

• Head traumaHead trauma

• Recurrent or persistent OMRecurrent or persistent OM

Risk Indicators obtained from the familyRisk Indicators obtained from the family

• Parent or caregiver concern regarding hearing, Parent or caregiver concern regarding hearing, speech, language, or developmental delay (parent speech, language, or developmental delay (parent concern has been shown to be a good predictor).concern has been shown to be a good predictor).

• Family history of permanent HL in first or second Family history of permanent HL in first or second degree relatives with onset by 30y or age.degree relatives with onset by 30y or age.

Causes of Permanent Hearing Loss in 100 Causes of Permanent Hearing Loss in 100 Infants Infants

50% Environmental 50

50% Genetic

30% syndromes (>300) 30

20% >75 genes ident 20

½ are GJB2 – Connexin 26

Genetic CausesGenetic Causes

Single gene Connexin 26

Gene + environment Mitochondrial + ototoxic

Gene + gene Gene + other gene

The Connexin 26 GeneThe Connexin 26 Gene

• It is estimated that 50-80 % of all autosomal It is estimated that 50-80 % of all autosomal recessive congenital deafness may to due to recessive congenital deafness may to due to mutations in th3e Cx-26 gene on chromosome mutations in th3e Cx-26 gene on chromosome 13q11-q1213q11-q12

• The Cx gene produces a gap junction protein The Cx gene produces a gap junction protein expressed between the outer hair cells and expressed between the outer hair cells and supporting cells and is involved with auditory supporting cells and is involved with auditory transduction.transduction.

• The estimated carrier frequency in the general The estimated carrier frequency in the general population is 1 in 31. population is 1 in 31. (Estivill et al, 1998)(Estivill et al, 1998)

Genetic TestingGenetic Testing

• Obtaining an adequate sample for DNA Obtaining an adequate sample for DNA testing is now quite easytesting is now quite easy

• Bilateral buccal smears with a Q tip provide Bilateral buccal smears with a Q tip provide adequate genetic material for testing.adequate genetic material for testing.

• The follow-up genetic counseling is key to The follow-up genetic counseling is key to the success of genetic testing.the success of genetic testing.

New technologies used in hearing New technologies used in hearing screeningscreening

GravelGravel

Otoacoustic Emissions (OAE)Otoacoustic Emissions (OAE)• By-product of the active processing of healthy OHCBy-product of the active processing of healthy OHC• Recording of an OAERecording of an OAE

– Indicates healthy OHCs (cochlea)Indicates healthy OHCs (cochlea)– Presence highly correlated with normal hearing sensitivity or Presence highly correlated with normal hearing sensitivity or

no greater than a mild hearing lossno greater than a mild hearing loss

• Sensorineural hearing losses of greater than about 30 dB Sensorineural hearing losses of greater than about 30 dB HL generally result in absent OAE. HL generally result in absent OAE.

• Since recording OAE requires normal forward and Since recording OAE requires normal forward and backward transmission of energy to and from the backward transmission of energy to and from the cochlea, conductive hearing loss associated with middle cochlea, conductive hearing loss associated with middle or outer ear abnormality can result in absent OAEor outer ear abnormality can result in absent OAE

Types of OAETypes of OAE

• Spontaneous OAEsSpontaneous OAEs

• Evoked OAEsEvoked OAEs– Transient OAE (TEOAE, TOAE, or Transient OAE (TEOAE, TOAE, or

click-OAE: COAE)click-OAE: COAE)

– Distortion Product (DPOAE)Distortion Product (DPOAE)

TEOAETEOAE

• Elicited by transients or brief stimuliElicited by transients or brief stimuli– Clicks: rapid onset, broadband stimulus Clicks: rapid onset, broadband stimulus

containing energy from low through high containing energy from low through high frequencies (i.e., across speech frequency frequencies (i.e., across speech frequency range) range)

– Tone bursts: more frequency-specificTone bursts: more frequency-specific

Distortion-Product OAE (DPOAE)Distortion-Product OAE (DPOAE)

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

• When 2 tones are presented to the cochlea, When 2 tones are presented to the cochlea, “distortion” occurs in the form of other “distortion” occurs in the form of other tones (harmonics) that are not present in the tones (harmonics) that are not present in the 2 eliciting tones2 eliciting tones

Screening Technologies - NeonatesScreening Technologies - NeonatesEvoked Otoacoustic EmissionsEvoked Otoacoustic Emissions

EOAE EOAE Advantages:Advantages:• QuickQuick• InexpensiveInexpensive• Frequency-specificFrequency-specific• Identifies cochlear and Identifies cochlear and

conductive losses: mild-conductive losses: mild-mod?mod?

• Pass-refer screening Pass-refer screening devices availabledevices available

EOAE EOAE Disadvantages:Disadvantages:

• Sensitive to ear canal and Sensitive to ear canal and middle ear conditionsmiddle ear conditions

• Sensitive to noise (internal Sensitive to noise (internal & external)& external)

• Cannot identify neural Cannot identify neural disorders including auditory disorders including auditory neuropathyneuropathy

• High fail rates in some High fail rates in some programs.programs.

Device Options for OAE ScreeningDevice Options for OAE Screening

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

Auditory Brainstem Response (ABR)Auditory Brainstem Response (ABR)

• Recording (through surface Recording (through surface electrodes) of the micro-volt electrodes) of the micro-volt electrical activity generated electrical activity generated by the cochlea and by the cochlea and transmitted by the auditory transmitted by the auditory nerve and brainstem nerve and brainstem pathways in response to brief pathways in response to brief clicks.clicks.

• Clicks produce a Clicks produce a synchronized response from synchronized response from neural fibers; a tracing of the neural fibers; a tracing of the response is a series of wavesresponse is a series of waves

Screening Technologies – NeonatesScreening Technologies – NeonatesScreening (Automated) Auditory Brainstem Screening (Automated) Auditory Brainstem

Response (SABR or AABR)Response (SABR or AABR)

ABR ABR Advantages:Advantages:• Identifies cochlear, Identifies cochlear,

conductive and neural conductive and neural losses: ? mild-mod.losses: ? mild-mod.

• Pass-refer screening Pass-refer screening devices. Some test both ears devices. Some test both ears simultaneously.simultaneously.

• Relatively insensitive to Relatively insensitive to transient ear canal, middle transient ear canal, middle ear & external noise.ear & external noise.

• ? Lower fail rate than OAE? Lower fail rate than OAE

ABR ABR Disadvantages:Disadvantages:• Test timeTest time• Disposable costsDisposable costs• Infant state/myogenic Infant state/myogenic

artifactartifact• Requires electrode prep, Requires electrode prep,

placement & removalplacement & removal• Click can miss unusual Click can miss unusual

configurations of HLconfigurations of HL

Examples of screeningABR technology

In-Hospital Screening:In-Hospital Screening:Two Technology ProtocolTwo Technology Protocol

OAE + ABR OAE + ABR AdvantagesAdvantages::

• Low fail rateLow fail rate• Depending on test order, Depending on test order,

identifies cochlear, neural identifies cochlear, neural and conductive lossesand conductive losses

• Reduced effects of noiseReduced effects of noise• Pass-refer screening Pass-refer screening

devices available for both devices available for both technologiestechnologies

OAE + ABR OAE + ABR Disadvantages:Disadvantages:

• TimeTime

• Equipment and disposable Equipment and disposable costscosts

Why is diagnostic confirmationWhy is diagnostic confirmation by an by an audiologist skilled in evaluating infants and audiologist skilled in evaluating infants and

young childrenyoung children important? important?

VohrVohr

A failed hearing screen may be a false positive or an A failed hearing screen may be a false positive or an actual fail. These 2 findings need totally different actual fail. These 2 findings need totally different management.management.

Therefore, an accurate diagnosis of normal hearing, Therefore, an accurate diagnosis of normal hearing, SNHL, auditory neuropathy or conductive loss is SNHL, auditory neuropathy or conductive loss is important as soon as possible to minimize important as soon as possible to minimize parental stress and to decrease the time interval parental stress and to decrease the time interval between screen fail and starting treatment. between screen fail and starting treatment.

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

Early Identification of Hearing Loss is Early Identification of Hearing Loss is Important becauseImportant because

• Delayed identification, even of mild HL Delayed identification, even of mild HL results inresults in

language delayslanguage delays

developmental skill delays, and developmental skill delays, and

behavior problems.behavior problems.

• Subsequent delays in literacy, and Subsequent delays in literacy, and academic performanceacademic performance

Reading Comprehension Scores of Reading Comprehension Scores of Hearing and Deaf StudentsHearing and Deaf Students

1.0

2.0

3.0

4.0

5.0

6.0

7.0

8.0

9.0

10.0

8 9 10 11 12 13 14 15 16 17 18

Deaf

Hearing

Age in Years

Gra

de

Eq

uiv

alen

ts

Deaf Children in America 1986

Unilateral or Mild LossUnilateral or Mild Loss

• 50% of children either repeat a grade or 50% of children either repeat a grade or need resource support in school need resource support in school

• Increased behavioral and linguistic Increased behavioral and linguistic problems compared to hearing controls.problems compared to hearing controls.

Bess F, Pediatrics 1984Bess F, Pediatrics 1984

Early “Early Intervention” for Hearing Early “Early Intervention” for Hearing Loss is Important becauseLoss is Important because

• There are dramatic benefits associated with early There are dramatic benefits associated with early identification and intervention for hearing loss identification and intervention for hearing loss before 6 months of agebefore 6 months of age..

• Children identified and receiving services < 6 m Children identified and receiving services < 6 m have larger vocabularies, better comprehension have larger vocabularies, better comprehension and better expressive language than children and better expressive language than children identified > 6 m.identified > 6 m.

Are Interventions Available to Are Interventions Available to Improve Outcome?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 AAP & JCIH Recommendations Components of EHDI Programs in the USof EHDI Programs in the US

• Universal Newborn Hearing Screening< 1 mUniversal Newborn Hearing Screening< 1 m

• Effective Tracking and Follow-up as a part of the Effective Tracking and Follow-up as a part of the Public Health SystemPublic Health System

• Appropriate and Timely Diagnosis of the HL < 3mAppropriate and Timely Diagnosis of the HL < 3m

• Prompt Enrollment in Appropriate EI < 6mPrompt Enrollment in Appropriate EI < 6m

• All infants will have a medical homeAll infants will have a medical home

Importance of Diagnostic Audiologic Importance of Diagnostic Audiologic Confirmation of Hearing LossConfirmation of Hearing Loss

GravelGravel

O bserva tion of Behaviors to acous tic s igna ls(c ross -check &

auditory func tion)

A BR: F S (A C & BC ) & Neuro

A cous tic Im m ittance

E O A E

Birth - 5 m os

A BR: F S (A C & BC ) & Neuro(c ross -check & docum ent

phys io logic func tion)

V isua l Re inforcem ent A udiom etry (V RA )


E O A E

6 m os - ~ 24 m os

A BR: F S (A C & BC ) & Neuro(c ross -check & docum ent

phys io logic func tion)

Play A udiom etry


E O A E

~ > 24 m os

C om prehens ive A udio logic A ssessm ent(inc ludes case h is tory)

Gravel, 2000; Gravel & Hood, 1998Gravel, 2000; Gravel & Hood, 1998

““Audiologists should have experience with the Audiologists should have experience with the assessment of infants & children with HL and the assessment of infants & children with HL and the knowledge and equipment necessary for use with knowledge and equipment necessary for use with

current pediatric assessment methods”.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 What are the components of the medical home work-up for children with congenital home work-up for children with congenital

hearing loss?hearing loss?

VohrVohr

EHDI and the Medical Home

Parent Groups Mental Health

Hospital Screening

Audiology

Primary Provider

Child/Family

ENT

GeneticsEI Therapists

3rd Party Payors

Deaf Community

Interpreters Deaf Services

The Medical WorkupThe Medical Workup

• Complete prenatal & perinatal hxComplete prenatal & perinatal hx

• Family Hx of onset of HL < age 30Family Hx of onset of HL < age 30

• Physical for stigmata, ear tabs, cleft Physical for stigmata, ear tabs, cleft palate, cardiac, skeletal, microcephalypalate, cardiac, skeletal, microcephaly

• Refer to ENT / CT of temporal bonesRefer to ENT / CT of temporal bones

• Refer to Genetics and OpthalmologyRefer to Genetics and Opthalmology

• Other: CMV, EKG Other: CMV, EKG

What are some of the questions to ask ?What are some of the questions to ask ?

• Antenatal history- maternal illness during Antenatal history- maternal illness during the pregnancy or deliverythe pregnancy or delivery

• Neonatal complications, prematurity, Neonatal complications, prematurity, jaundice, asphyxia, assisted ventilation, jaundice, asphyxia, assisted ventilation, ECMOECMO

Examination for CausesExamination for Causes

• Evaluate for dysmorphic features, minor and Evaluate for dysmorphic features, minor and major stigmata and syndromesmajor stigmata and syndromes

• Other anomalies – visual, facial, endocrine, Other anomalies – visual, facial, endocrine, cardiac, kidney, hair, and skincardiac, kidney, hair, and skin

• Particular attention to the head and neck. HL Particular attention to the head and neck. HL may be associated with abn. pinna, atresia or may be associated with abn. pinna, atresia or stenosis of the ear canal, ear tags, and bony stenosis of the ear canal, ear tags, and bony growths in the ear canal.growths in the ear canal.

• Cleft lip and palate may have middle ear fluidCleft 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 Is there a family hx of onset of permanent HL HL << 30 years of age ( over 3 generations) 30 years of age ( over 3 generations)

• Are there other family members with Are there other family members with syndromes or anomalies ?syndromes or anomalies ?

Which families may benefit from a Genetic Which families may benefit from a Genetic Referral ?Referral ?

• All families with a child with congenital or All families with a child with congenital or late onset hearing losslate onset hearing loss

• Families of a child with stigmata or a Families of a child with stigmata or a syndrome will benefit from the information.syndrome will benefit from the information.

• Families with a child with non-syndromic HL Families with a child with non-syndromic HL want to know the cause ?want to know the cause ?

• Some parents who are culturally deaf wish Some parents who are culturally deaf wish information on the risk or cause of HL.information on the risk or cause of HL.

When to refer to OphthalmologyWhen to refer to Ophthalmology

• First- Follow periodicity schedule for all patientsFirst- Follow periodicity schedule for all patients• Some syndromes with permanent HL have Some syndromes with permanent HL have

specific eye findings such as heterochromia in specific eye findings such as heterochromia in Wardenburgs.Wardenburgs.

• In Ushers the child is at risk of late onset vision In Ushers the child is at risk of late onset vision loss secondary to retinitis pigmentosa. (If sign is loss secondary to retinitis pigmentosa. (If sign is the primary communication mode they will the primary communication mode they will obviously have a problem.obviously have a problem.

When to get an EKG ?When to get an EKG ?

• This is ordered to rule out long QT ( Jervell This is ordered to rule out long QT ( Jervell and Lange-Nielsen )syndrome.and Lange-Nielsen )syndrome.

• This syndrome may manifest itself with This syndrome may manifest itself with apnea, passing out episodes, or a history of apnea, passing out episodes, or a history of sudden death in a relative.sudden death in a relative.

Should there be additional workup ?Should there be additional workup ?

• This needs to be individualized. This needs to be individualized.

• For example, A NICU infant with IUGR For example, A NICU infant with IUGR should have an MRI and TORCH titers to should have an MRI and TORCH titers to rule out CMV, toxoplasmosis etc.rule out CMV, toxoplasmosis etc.

Children with Cochlear Implants and MeningitisChildren with Cochlear Implants and Meningitis

• The incidence of meningitis is higher among The incidence of meningitis is higher among children with cochlear implants.children with cochlear implants.

• The incidence of Streptococcus pneumoniae The incidence of Streptococcus pneumoniae meningitis was 30 times the incidence in the meningitis was 30 times the incidence in the general population. general population. NEJM, July 2003NEJM, July 2003

Recommendations for children with Cochlear Recommendations for children with Cochlear ImplantsImplants

• Children < age 2 years should get Children < age 2 years should get pneumococcal conjugate vaccine (Prevnar) pneumococcal conjugate vaccine (Prevnar) according to the routine schedulesaccording to the routine schedules

• There are guidelines for older children (CDC)There are guidelines for older children (CDC)

• Children with cochlear transplants should be Children with cochlear transplants should be monitored and treated promptly for any monitored and treated promptly for any bacterial infections.bacterial infections.

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

Cochlear ImplantationCochlear Implantation

GravelGravel

Ensure children receive full-time use and consistent Ensure children receive full-time use and consistent audibility of the speech signal at safe and comfortable audibility of the speech signal at safe and comfortable listening levels as soon as hearing loss is confirmed.listening levels as soon as hearing loss is confirmed.

PWG, 1996PWG, 1996

What is the Goal of Hearing Aid Fitting

For newborns identified by UNHS, For newborns identified by UNHS, fit hearing aids within one month offit hearing aids within one month of

confirmation of hearing loss, preferably beforeconfirmation of hearing loss, preferably before4 months and no later than 6 months of age.4 months and no later than 6 months of age.

JCIH, 2000JCIH, 2000

When are Hearing Aids Fit?

Which infants are candidates for amplification?Which infants are candidates for amplification?

• Significant, permanent Significant, permanent bilateral peripheral bilateral peripheral hearing loss.hearing loss.

• Mild hearing loss in Mild hearing loss in some casessome cases

• Some children with Some children with unilateral hearing loss, unilateral hearing loss, and minimal HLand minimal HL

• Need based on Need based on audiogram audiogram plusplus additional information:additional information:

– Family choiceFamily choice

– other disabilities/on-other disabilities/on-going medical issuesgoing medical issues

– performanceperformance

PWG, 1996PWG, 1996

• Binaural fitting unless clear Binaural fitting unless clear contraindicationcontraindication

• Behind-the-ear style of choiceBehind-the-ear style of choice

PWG, 1996PWG, 1996

Pre-Selection - Pre-Selection - Children’s Hearing Aids Should Include:Children’s Hearing Aids Should Include:

Pre-Selection - Pre-Selection - Children’s Hearing Aids Should Include:Children’s Hearing Aids Should Include:

• Flexible response characteristicsFlexible response characteristics

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

PWG, 1996PWG, 1996

Directional Microphone TechnologyDirectional Microphone Technology

• Improves directional hearing abilitiesImproves directional hearing abilities– Hear parent speaking from front; noise from Hear parent speaking from front; noise from

shopping mall at back of child reduced.shopping mall at back of child reduced.

Multiple Memory Hearing InstrumentsMultiple Memory Hearing Instruments

• Allows storage of Allows storage of more than one more than one electroacoustic electroacoustic response settingresponse setting

• Allows switching Allows switching between memories for between memories for various listening various listening situations situations

Digital Signal Processing (DSP) TechnologyDigital Signal Processing (DSP) Technology

• Newer hearing aids that use digital Newer hearing aids that use digital processing of incoming processing of incoming

• No studies, to date, demonstrate better No studies, to date, demonstrate better performance of digital instruments over performance of digital instruments over conventional hearing aids in either adults conventional hearing aids in either adults or childrenor children

• Considerably more expensiveConsiderably more expensive

• Safety-related features: tamper Safety-related features: tamper resistantresistant– battery compartmentbattery compartment– volume controlvolume control

• Physical fit (size) and colorPhysical fit (size) and color

• Earmolds made of soft materialEarmolds made of soft material

PWG, 1996PWG, 1996

What Other Features Should What Other Features Should Children’s Hearing Aids Include?Children’s Hearing Aids Include?

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

• Use computerized prescriptive fitting procedureUse computerized prescriptive fitting procedure• Requires only minimal threshold data to begin, so Requires only minimal threshold data to begin, so

fitting can begin early.fitting can begin early.

From: Seewald, 2003

Prescriptive Fitting ProcedurePrescriptive Fitting Procedure

• Incorporate simple probe Incorporate simple probe microphone measurementsmicrophone measurements

• Allow audiologist to:Allow audiologist to:– Pre-select the response Pre-select the response

characteristics of the hearing characteristics of the hearing aidsaids

– Refine or ‘individualize’ the Refine or ‘individualize’ the hearing aid for the unique hearing aid for the unique acoustic characteristics of acoustic characteristics of each infant’s ear each infant’s ear

– Verify that the prescriptive Verify that the prescriptive frequency-gain and output frequency-gain and output targets have been achievedtargets have been achieved

From: www.babyhearing.org

Goals of FM FittingGoals of FM Fitting• Child hears primary talker at level that is Child hears primary talker at level that is

consistently audible above the background consistently audible above the background noisenoise

• Child able to monitor his/her own voiceChild able to monitor his/her own voice

• Child hears voices of others who are not Child hears voices of others who are not wearing the FM microphonewearing the FM microphone

Lewis et al., 1998 (Phonak AG)Lewis et al., 1998 (Phonak AG)

• Infants and young children with Infants and young children with congenital/early onset hearing loss congenital/early onset hearing loss – Are learning language for the first timeAre learning language for the first time– Have greater difficulty understanding Have greater difficulty understanding

speech in background noise than adultsspeech in background noise than adults– Require a more audible (intense) signal Require a more audible (intense) signal

than adults to understand speechthan adults to understand speech

Why Does the Acoustic Climate (of the Home, Daycare Setting, etc.) Need to be Considered?

1m1m

2m2m

3m3m

4m4m

5m5m

6m6m

7m7m

8m8m

9m9m

10m10m

11m11m

12m12m

Fail Neonatal ScreeningFail Neonatal Screening

Fail Outpatient screeningFail Outpatient screening

FS-ABR,EOAE AC & BC, tympsFS-ABR,EOAE AC & BC, tymps

Repeat FS-ABR, EOAE,RECD withRepeat FS-ABR, EOAE,RECD withinsert, for HA selection, tympsinsert, for HA selection, tymps

Behavioral & tympsBehavioral & tymps(with mold to insert coupling)(with mold to insert coupling)

Behavioral & tympsBehavioral & tymps(with mold-to-insert coupling)(with mold-to-insert coupling)

Behavioral & tymps Behavioral & tymps (with mold-to-insert coupling)(with mold-to-insert coupling)

Behavioral & tympsBehavioral & tymps(with mold-to-insert coupling)(with mold-to-insert coupling)

Mold impressions, EI ProgramMold impressions, EI Program

HA FittingHA Fitting

HA Check & (molds)HA Check & (molds)Review habilitation, language milestonesReview habilitation, language milestones

RECD, RECD, HA modification, (molds)HA modification, (molds)Review habilitation, language milestonesReview habilitation, language milestones

RECD, RECD, HA modification, (molds)HA modification, (molds)Review habilitation, language milestonesReview habilitation, language milestones

RECD, HA Check, (molds)RECD, HA Check, (molds)Validation measures, language milestonesValidation measures, language milestonesSet habilitation goals for year 2Set habilitation goals for year 2

Counseling; medical/ENT referralCounseling; medical/ENT referralBegin processes for HA procurementBegin processes for HA procurement

Observe auditory behaviors & tympsObserve auditory behaviors & tymps

CounselingCounseling

Gravel, 2000Gravel, 2000

Qualifications of Qualifications of Audiologists and Facilities Audiologists and Facilities

• No facility should fit hearing aids to children No facility should fit hearing aids to children if it lacks the equipment for behavioral, if it lacks the equipment for behavioral,

electrophysiologic, electroacoustic, and probe-electrophysiologic, electroacoustic, and probe-microphone/real-ear evaluation.microphone/real-ear evaluation.

• Facilities that lack the expertise or equipment Facilities that lack the expertise or equipment should establish consortialshould establish consortial

arrangements with centers that do.arrangements with centers that do.

PWG, 1996PWG, 1996

Considerations in Determining the Considerations in Determining the Appropriateness of Cochlear Appropriateness of Cochlear

Implantation including Risks, Benefits & Implantation including Risks, Benefits & TimingTiming

GravelGravel

What are the Candidacy Criteria for a What are the Candidacy Criteria for a Cochlear Implant?Cochlear Implant?

• Limited benefit from conventional amplification Limited benefit from conventional amplification following a minimum of 3-6 months use following a minimum of 3-6 months use – May be sooner following deafness from meningitisMay be sooner following deafness from meningitis

• Profound hearing loss: Profound hearing loss: – 12-18 months 12-18 months

• Severe-to-Profound hearing lossSevere-to-Profound hearing loss– >18 months>18 months

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

From: www.babyhearing.org

Cochlear ImplantsCochlear Implants

• Acoustic signal picked up by microphone located in headset Acoustic signal picked up by microphone located in headset worn at ear levelworn at ear level

• Cord carries sound from microphone to a speech processor Cord carries sound from microphone to a speech processor • Speech processor digitizes sound into coded signalsSpeech processor digitizes sound into coded signals• Coded signals sent up to the transmitting coilCoded signals sent up to the transmitting coil• Coil sends coded signals as FM radio signals to CI under the Coil sends coded signals as FM radio signals to CI under the

skinskin• CI delivers electrical energy to the electrode array inserted into CI delivers electrical energy to the electrode array inserted into

the cochleathe cochlea• Electrodes along the array stimulate remaining nerve fibers in Electrodes along the array stimulate remaining nerve fibers in

cochleacochlea

Cochlear Corp. 2002

Selecting a Cochlear Implant CenterSelecting a Cochlear Implant Center• Experienced cochlear implant teamExperienced cochlear implant team

– Audiologist, speech-language pathologist, surgeonAudiologist, speech-language pathologist, surgeon– Others: educator of the deaf, psychologist, social workerOthers: educator of the deaf, psychologist, social worker

• Comprehensive program covering eligibility, surgery, Comprehensive program covering eligibility, surgery, device activation, and long-term habilitation.device activation, and long-term habilitation.– Multi-disciplinary, family centered approach. Multi-disciplinary, family centered approach. – Knowledgeable regarding deafness, child development, Knowledgeable regarding deafness, child development,

and speech, language and auditory developmentand speech, language and auditory development– Offers intervention program and continued Offers intervention program and continued – Follow-up for changes in cochlear implant mappingFollow-up for changes in cochlear implant mapping

BenefitsBenefits

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

Listening to Parental Concern About Listening to Parental Concern About Delayed Language DevelopmentDelayed Language Development

Gravel & VohrGravel & Vohr

Listening to parent concern about Listening to parent concern about language developmentlanguage development

• Parent concerns about hearing, speech, Parent concerns about hearing, speech, language, or developmental delays are language, or developmental delays are strong predictors of an actual problem.strong predictors of an actual problem.

• Providers must avoid statements like Providers must avoid statements like “Babies develop at different rates. Lets take “Babies develop at different rates. Lets take another look in about 6 months”another look in about 6 months”

Clinical signs of Hearing LossClinical signs of Hearing Loss

• Delayed early language milestonesDelayed early language milestones

• Unintelligible speechUnintelligible speech

• Uncharacteristic voice patternsUncharacteristic voice patterns

• Child turns TV volume very loudChild turns TV volume very loud

• Child only responds to loud sounds/words Child only responds to loud sounds/words or in a very quiet environmentor in a very quiet environment

Myths about hearing and early speech Myths about hearing and early speech language delaylanguage delay

• We don’t have to worry because:We don’t have to worry because:• His older brothers and sisters talk for himHis older brothers and sisters talk for him• Boys develop speech much later than girlsBoys develop speech much later than girls• Twins always have language delaysTwins always have language delays• Grandma says that her Dad did not speak until Grandma says that her Dad did not speak until

he was 3 years old.he was 3 years old.• She has great motor milestonesShe has great motor milestones• I know he hears because he gets upset every I know he hears because he gets upset every

time I turn on the vacuum.time I turn on the vacuum.

Assessment of language delayAssessment of language delay

• Administer a speech language screenAdminister a speech language screen

• Check middle ear status for MEE: Rx Check middle ear status for MEE: Rx

• MEE for > 3 m refer to otolaryngologyMEE for > 3 m refer to otolaryngology

• If receptive /expressive delay refer back to If receptive /expressive delay refer back to audiology for repeat diagnosticaudiology for repeat diagnostic

• Refer to early intervention for speech Refer to early intervention for speech language therapylanguage therapy

• Assess for other possible dx: PDD, autismAssess for other possible dx: PDD, autism

• Continue to follow-up on speech/language Continue to follow-up on speech/language

It is important to respond to concerns about language It is important to respond to concerns about language immediately !immediately !

Most children with delays of speech and Most children with delays of speech and language respond to appropriate medical, language respond to appropriate medical, audiologic, and educational interventions.audiologic, and educational interventions.

A successful early screening, identification, A successful early screening, identification, and intervention program will ultimately and intervention program will ultimately permit every child with HL to develop to permit every child with HL to develop to his/her potential.his/her potential.

• All in-hospital screening failures should receive All in-hospital screening failures should receive follow-upfollow-up– Failure rate at discharge, once high has decreased to Failure rate at discharge, once high has decreased to

2% or less.2% or less.– Probability 1 in 5 that infant who fails NHS has hearing Probability 1 in 5 that infant who fails NHS has hearing

lossloss

• Regardless of screening outcome, if parent is Regardless of screening outcome, if parent is concerned regarding hearing or speech-language concerned regarding hearing or speech-language development, child should be referred for development, child should be referred for audiologic evaluationaudiologic evaluation

Question-and-AnswerQuestion-and-Answer

Early Hearing Detection and Intervention Early Hearing Detection and Intervention CME Teleconference Series, Part IICME Teleconference Series, Part II

November 12, 2003November 12, 200312:00 – 1:00 pm, Central Standard Time12:00 – 1:00 pm, Central Standard Time

TopicsTopics: : Implementing newly developed AAP guidelines; important Implementing newly developed AAP guidelines; important referrals; the role of early intervention; primary care’s role referrals; the role of early intervention; primary care’s role in coordination of services; parenting issues and reactions; in coordination of services; parenting issues and reactions; knowledge of and support for communication choices; cost knowledge of and support for communication choices; cost and reimbursement issues; and national resources.and reimbursement issues; and national resources.

FacultyFaculty: Al Mehl and Mary Pat Moeller: Al Mehl and Mary Pat Moeller

Early Hearing Detection and Intervention – The Role of the Primary Care Physician AAP CME Teleconference, Part I October 15, 2003.

Documents

type of hearing loss

types of hearing loss

hearing loss siterate

sensory loss

hearing screen

db frequency

late onset hearing loss

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