Update on Newborn Hearing Screening NIDCD. National Goals for Hearing Screening (1-3-6) 1, 2 All infants will access hearing screening using a physiologic

Update on Newborn Update on Newborn Hearing ScreeningHearing Screening

NIDCD

National Goals for National Goals for Hearing Screening (1-3-Hearing Screening (1-3-

6)6)1, 21, 2 All infants will access hearing screening using All infants will access hearing screening using

a physiologic measure a physiologic measure – no later than 1 month of ageno later than 1 month of age

All infants not passing initial screening and All infants not passing initial screening and subsequent rescreening should have subsequent rescreening should have confirmatory audiological and medical confirmatory audiological and medical evaluations evaluations – no later than 3 months of ageno later than 3 months of age

All infants with confirmed permanent hearing All infants with confirmed permanent hearing loss should receive early intervention as soon loss should receive early intervention as soon as possibleas possible– no later than 6 months of ageno later than 6 months of age

Prerequisites for a Prerequisites for a Population Screening Population Screening

ProgramProgram Condition sufficiently frequent in Condition sufficiently frequent in

screened populationscreened population Condition serious or fatal without Condition serious or fatal without

interventionintervention Condition must be treatable or Condition must be treatable or

preventablepreventable Effective follow-up program Effective follow-up program

possiblepossible

YES

YES

YES

YES

Why is early identification of Why is early identification of hearing loss important?hearing loss important?

Hearing loss is the most common birth conditionHearing loss is the most common birth condition

Incidence of Congenital Conditions Incidence of Congenital Conditions

(Per 10,000)(Per 10,000)

0

5

10

15

20

25

30

35

Hearing loss Cleft lip orpalate

Downsyndrome

Limb defects Spina bifida Sickle cellanemia

PKU

Congenital Condition Type

Nu

mb

er

pe

r 1

0,0

00

Prevalence of Hearing LossPrevalence of Hearing Loss

Prevalence estimates vary across Prevalence estimates vary across studies studies

Estimated that 1 to 3 per 1000 infants Estimated that 1 to 3 per 1000 infants will have permanent sensorineural will have permanent sensorineural hearing losshearing loss3, 43, 4

– 1/1000 from the well baby nursery 1/1000 from the well baby nursery – 10/1000 from the NICU10/1000 from the NICU

Rate increases to 6/1000 by school Rate increases to 6/1000 by school ageage44

– Need for surveillanceNeed for surveillance

What does it sound like to What does it sound like to have a hearing loss?have a hearing loss?

Normal hearing

Mild hearing loss

Moderate hearing loss

Severe hearing loss


Previous methods for detecting Previous methods for detecting hearing loss have been ineffectivehearing loss have been ineffective– High risk screening failed to identify High risk screening failed to identify

~ 50% of the infants with hearing ~ 50% of the infants with hearing lossloss

– Large retrospective cohort studyLarge retrospective cohort study5, 6: : mean age of diagnosis 21.6 monthsmean age of diagnosis 21.6 months

– Similar findings reported in USSimilar findings reported in US7,8,97,8,9

Newborn hearing Newborn hearing screening is effectivescreening is effective Large, good-quality cohort study Large, good-quality cohort study

conducted in UKconducted in UK1010

53,781 babies; 25,609 born during 53,781 babies; 25,609 born during NHS eraNHS era

2-step screening (OAE + ABR)2-step screening (OAE + ABR)– Sensitivity = 0.92Sensitivity = 0.92– Specificity = 0.98Specificity = 0.98

Lower refer rates with qualified Lower refer rates with qualified examinersexaminers1111


Hearing loss is the most common Hearing loss is the most common birth conditionbirth condition

Previous methods for detecting Previous methods for detecting hearing loss have been ineffectivehearing loss have been ineffective

Undetected hearing loss can Undetected hearing loss can delay speech, language, social & delay speech, language, social & academic developmentacademic development

Vocabulary Development Vocabulary Development in Infantsin Infants12, 1312, 13

0

50

100

150

200

250

300

350

400

12 mos 14 mos 16 mos 18 mos 24 mos

Age

Nu

mb

er

of

Ex

pre

ss

ive

Wo

rds

NH Boys

NH Girls

Toddlers with Hearing Loss

Delays in babble also observed 14, 1514, 15

Reading Comprehension in Reading Comprehension in Children with Mild-Mod Loss Children with Mild-Mod Loss

1616

0

20

40

60

80

100

120

140

Grade 1 Grade 4

Academic Grade

Re

ad

ing

Co

mp

reh

en

sio

n S

tan

da

rd S

co

re

Normal Hearing

Hearing Loss


Early identification and Early identification and intervention can make a intervention can make a differencedifference

Effects of Age of Effects of Age of Identification on Language Identification on Language DevelopmentDevelopment1717

Prospective, longitudinal study of Prospective, longitudinal study of early-identified infantsearly-identified infants

30 children with mild-profound hearing 30 children with mild-profound hearing loss (HL) compared to 96 normal loss (HL) compared to 96 normal hearing (NH) controlshearing (NH) controls

Children identified Children identified < < 3 months had 3 months had stronger language development at 12-stronger language development at 12-16 months than those identified > 3 16 months than those identified > 3 monthsmonths

Children with HL were delayed Children with HL were delayed compared to NH infantscompared to NH infants

Effects of Age of Identification Effects of Age of Identification on Language Developmenton Language Development1818

Language Quotients at Three Years of Age by Age of Identification Category

0

10

20

30

40

50

60

70

80

90

100

0-6 mos 7-12 mos 13-18 mos 19-24 mos 25-34 mos

Ages of Identification

La

ng

ua

ge

Qu

oti

en

t S

co

re Average range

Vocabulary at Age Five by Vocabulary at Age Five by Age of InterventionAge of Intervention1919

Id Age: 8%

Family

Involvement:

37%

Significant Predictors:

Average range

Evidence that Early Evidence that Early MattersMatters

8-year follow up to Wessex (UK) trial8-year follow up to Wessex (UK) trial1010

– 120 children with permanent HL (from 120 children with permanent HL (from population-based cohort of 157,000 infants)population-based cohort of 157,000 infants)

Speech-language outcomes at school age Speech-language outcomes at school age (Mean = 7.9 years)(Mean = 7.9 years)

Children with HL confirmed Children with HL confirmed < < 9 mos had 9 mos had better receptive and expressive language better receptive and expressive language scores than later identified children scores than later identified children – Speech scores were equivalent in the 2 Speech scores were equivalent in the 2

groupsgroups

American Academy of Pediatrics American Academy of Pediatrics (AAP)(AAP)

Endorsed implementation of Endorsed implementation of universal newborn hearing universal newborn hearing screening in 1999screening in 1999

Defined standards for:Defined standards for:– ScreeningScreening– Tracking & Follow-upTracking & Follow-up– Identification & InterventionIdentification & Intervention– Program EvaluationProgram Evaluation

Encouraged AAP Chapters to Encouraged AAP Chapters to provide leadership in physician provide leadership in physician education and newborn screening education and newborn screening in their statesin their states

Early Hearing Detection Early Hearing Detection and Intervention (EHDI)and Intervention (EHDI)

Endorsed by:Endorsed by:– AAP, National Institutes of Health, AAP, National Institutes of Health,

Maternal and Child Health, Centers for Maternal and Child Health, Centers for Disease Control, Joint Committee on Disease Control, Joint Committee on Infant Hearing & in 2008, the USPSTF Infant Hearing & in 2008, the USPSTF

As of 2005, all 50 states implemented As of 2005, all 50 states implemented statewide EHDI programsstatewide EHDI programs

As of 2006, an average of 95.7% of As of 2006, an average of 95.7% of newborns were screened nationallynewborns were screened nationally

Status of Hearing Status of Hearing ScreeningScreening

in Nebraska (as of 10/08)in Nebraska (as of 10/08)

99.5% of newborns are being 99.5% of newborns are being screened screened

68/69 hospitals are screening68/69 hospitals are screening Refer rate is 2.3%Refer rate is 2.3% 54 infants with permanent 54 infants with permanent

HL were diagnosed in 2007HL were diagnosed in 2007

Contact:Contact:[email protected]@dhhs.ne.go

vv

Hearing Screening Hearing Screening TechniquesTechniques

Otoacoustic emissions (OAE)Otoacoustic emissions (OAE)

Auditory brainstem response Auditory brainstem response (ABR)(ABR)

Two stage screening (OAE + ABR)Two stage screening (OAE + ABR)

Otoacoustic EmissionsOtoacoustic Emissions

Sounds are presented to the ear canal and a small microphone measures the response in the ear canal

Average test time is 5-15 minutes/baby

Auditory Brainstem Auditory Brainstem ResponseResponse

Sounds are presented and surface electrodes measure brainstem activity

Average test time 20 min/baby

OAE + ABROAE + ABR

All babies are screened using OAEs

Those babies who fail the OAE screening receive an ABR screening prior to leaving the hospital

Average test time/baby (25-35 min)

Reduces refer rate; useful when follow up is likely to be difficult or costly

Initial cost of equipment is higher than OAE or ABR screening alone, but follow-up costs are less

2007 JCIH Position on 2007 JCIH Position on ScreeningScreening22

NICUNICU– >5 days in NICU>5 days in NICU– ABR should be ABR should be

included to screen for included to screen for neural lossneural loss

– Rescreen BOTH ears, Rescreen BOTH ears, even if only one ear even if only one ear failsfails

– Non pass – refer to Non pass – refer to AudiologistAudiologist

– Readmission – Readmission – rescreen before rescreen before dischargedischarge

Well baby nurseryWell baby nursery– Screen with OAE or Screen with OAE or

ABRABR– Repeat screen when Repeat screen when

necessary before necessary before dischargedischarge

– When using 2 step When using 2 step protocol test order protocol test order should be OAE then should be OAE then ABRABR

– Rescreen BOTH ears, Rescreen BOTH ears, even if only one ear even if only one ear fails fails

Cost effectivenessCost effectiveness

Within each hospital, the Within each hospital, the optimum approach will depend optimum approach will depend upon the number of births/year, upon the number of births/year, the availability of trained the availability of trained personnel for testing 365 personnel for testing 365 days/year, follow-up services in days/year, follow-up services in the area, and expected loss to the area, and expected loss to follow-up rate.follow-up rate.

Screening CostsScreening Costs2222

Characteristics of a good Characteristics of a good screening programscreening program

Refer rate of 1.5-5.0% in well baby Refer rate of 1.5-5.0% in well baby nursery and slightly lower in the NICU nursery and slightly lower in the NICU (resulting from 2-stage screening in (resulting from 2-stage screening in the hospital)the hospital)– 5.0% = 400 babies per 8000 births5.0% = 400 babies per 8000 births

Ongoing training and monitoring Ongoing training and monitoring program for personnelprogram for personnel

Structured plan for follow up Structured plan for follow up Ability to track program performance Ability to track program performance

(important for quality assurance and (important for quality assurance and for JCAHO requirements)for JCAHO requirements)

What if a baby fails UNHS?What if a baby fails UNHS?

Failure rates range from 1.5-5.0% in Failure rates range from 1.5-5.0% in good screening programsgood screening programs

Most babies who fail the initial Most babies who fail the initial screening will actually have normal screening will actually have normal hearing hearing – For 10 babies that refer, 1 is expected For 10 babies that refer, 1 is expected

to have permanent hearing lossto have permanent hearing loss

System challenges: System challenges: Loss to Follow Up Loss to Follow Up2323

8 New York hospitals, 8 New York hospitals, – 28% infants who did not pass in-hospital 28% infants who did not pass in-hospital

screening failed to returnscreening failed to return– Loss to follow up is as high as 50% in Loss to follow up is as high as 50% in

some statessome states Return rates better for in-hospital fails Return rates better for in-hospital fails

than in-hospital missesthan in-hospital misses

Medical Home: Medical Home: Strategies to Promote Strategies to Promote

Follow Up Follow Up At prenatal visit, encourage families to At prenatal visit, encourage families to

identify you as follow-up care locationidentify you as follow-up care location Inform hospital to facilitate Inform hospital to facilitate

communication of results communication of results Provide checkbox on newborn well Provide checkbox on newborn well

child form/patient chart for hearing child form/patient chart for hearing screening results & risk factorsscreening results & risk factors

Set up tracking system for infants who Set up tracking system for infants who do not pass hearing screeningdo not pass hearing screening

Counseling ParentsCounseling Parents

Effective communication of results to Effective communication of results to families has an influence on follow up families has an influence on follow up behaviorsbehaviors

Balance between reassurance and Balance between reassurance and importance of follow up testingimportance of follow up testing

““Your child may or may not have a Your child may or may not have a hearing loss…but let’s be sure about it. If hearing loss…but let’s be sure about it. If further testing shows hearing loss, the further testing shows hearing loss, the earlier we get started helping the child, earlier we get started helping the child, the better.”the better.”

Counseling Parents Counseling Parents Following ScreeningFollowing Screening

Follow Up TestingFollow Up Testing

Referral for follow-up testing Referral for follow-up testing – Repeat OAE and/or ABR screeningRepeat OAE and/or ABR screening

If a hearing loss is still suspected…If a hearing loss is still suspected…– Referral to a Referral to a pediatricpediatric audiologist audiologist

Experienced in testing infants & childrenExperienced in testing infants & children Has appropriate equipment to test infantsHas appropriate equipment to test infants

– Frequency specific ABR to estimate Frequency specific ABR to estimate degree and configuration of hearing degree and configuration of hearing lossloss

Early testing can avoid need for sedationEarly testing can avoid need for sedation

Counseling Parents Counseling Parents Following DiagnosisFollowing Diagnosis

Components of a Components of a Comprehensive Audiological Comprehensive Audiological

EvaluationEvaluation History History Assessment of hearing sensitivity (ABR) Assessment of hearing sensitivity (ABR) Rule out middle ear pathology; refer to Rule out middle ear pathology; refer to

ENT physician if appropriate ENT physician if appropriate Initiate amplification Initiate amplification Refer to local early intervention Refer to local early intervention

programprogram Provide support via other parents of Provide support via other parents of

children with hearing losschildren with hearing loss PCP helps to coordinate child’s follow up PCP helps to coordinate child’s follow up

care in their practicecare in their practice

JCIH 2007 Follow Up JCIH 2007 Follow Up GuidelinesGuidelines22

EHDI systems should be family-EHDI systems should be family-centeredcentered

Families should have:Families should have:– Access to information on all Access to information on all

treatment optionstreatment options– Counseling regarding hearing lossCounseling regarding hearing loss

Child and family should have:Child and family should have:– Immediate access to hearing Immediate access to hearing

technologiestechnologies

AmplificationAmplification

Hearing aids can be Hearing aids can be fitted as young as 1 fitted as young as 1 month of agemonth of age

Importance of Importance of Intervention in Intervention in

OutcomesOutcomes

Early Identification needs to Early Identification needs to be paired with early, be paired with early,

appropriate and consistent appropriate and consistent interventions.interventions.

3 year old with moderate-severe loss: Inconsistent

Intervention

Child A

3 year old with moderate-severe loss: Consistent early

identification

Child B

3 year old with mild-moderate loss: Identified at 3 years, 3

monthsPre-intervention sample

Child C – 3 years

5 year old with mild-moderate loss: Identified at 3 years, 3

monthsPost- intervention sample

Child C – 5 years

Roles of the Medical HomeRoles of the Medical Home

Understand testing results at screening and Understand testing results at screening and diagnostic phases & implications for follow updiagnostic phases & implications for follow up

Assure follow-up screening; refer for Assure follow-up screening; refer for diagnostic and medical specialty evaluationsdiagnostic and medical specialty evaluations

Support family in understanding severity & Support family in understanding severity & type of hearing losstype of hearing loss

Refer to early intervention Refer to early intervention Offer partnership with parents to identify and Offer partnership with parents to identify and

develop a plan of health and habilitative caredevelop a plan of health and habilitative care

Optimal Surveillance in Optimal Surveillance in the Medical Home (JCIH, the Medical Home (JCIH,

2007)2007)22

At each visit consistent with AAP At each visit consistent with AAP periodicity schedule monitor for:periodicity schedule monitor for:– Auditory skills, middle ear statusAuditory skills, middle ear status– Developmental milestones (validated Developmental milestones (validated

global screening tool)global screening tool)– Parental concernsParental concerns

If concerns, refer for pediatric If concerns, refer for pediatric audiology and speech-language audiology and speech-language pathology evaluationspathology evaluations

Optimal Surveillance in Optimal Surveillance in the Medical Home (JCIH, the Medical Home (JCIH, 2007)2007)22

If hearing loss is diagnosed, refer siblings If hearing loss is diagnosed, refer siblings of infant for audiological evaluationof infant for audiological evaluation

Refer infants with any RISK indicators for Refer infants with any RISK indicators for audiological assessment by 24-30 audiological assessment by 24-30 months of agemonths of age

Carefully assess middle ear status at all Carefully assess middle ear status at all well child visits; refer for otologic well child visits; refer for otologic evaluation if persistent middle ear evaluation if persistent middle ear effusion lasts for 3 months+effusion lasts for 3 months+

Risk Indicators for permanent Risk Indicators for permanent congenital, delayed onset or congenital, delayed onset or

progressive hearing lossprogressive hearing loss22

Caregiver concerns* about hearing, speech, Caregiver concerns* about hearing, speech, language, developmentlanguage, development

Family history* of permanent childhood Family history* of permanent childhood hearing losshearing loss

NICU stay > 5 days or any of following NICU stay > 5 days or any of following (regardless of length of stay):(regardless of length of stay):

– ECMO assisted ventilation*ECMO assisted ventilation*– Ototoxic medications (gentimycin, tobramycin)Ototoxic medications (gentimycin, tobramycin)– Loop diuretics (furosemide, Lasix)Loop diuretics (furosemide, Lasix)– Hyperbilirubinemia reguiring exchange transfusionHyperbilirubinemia reguiring exchange transfusion

In Utero infections (cmv*, herpes, rubella, In Utero infections (cmv*, herpes, rubella, syphillis, toxoplasmosissyphillis, toxoplasmosis

* = greater risk for delayed onset HL



Caregiver concerns* Caregiver concerns* – about hearing, speech, language, developmentabout hearing, speech, language, development

Family history* Family history* – of permanent childhood hearing lossof permanent childhood hearing loss

NICU stay > 5 days or any of following NICU stay > 5 days or any of following (regardless of length of stay):(regardless of length of stay):– ECMO assisted ventilation*ECMO assisted ventilation*– Ototoxic medications (gentimycin, tobramycin)Ototoxic medications (gentimycin, tobramycin)– Loop diuretics (furosemide, Lasix)Loop diuretics (furosemide, Lasix)– Hyperbilirubinemia reguiring exchange Hyperbilirubinemia reguiring exchange

transfusiontransfusion

JCIH, 2007 * = greater risk for delayed onset HL



In Utero infections In Utero infections – CMV*, herpes, rubella, syphilis, CMV*, herpes, rubella, syphilis,

toxoplasmosistoxoplasmosis Craniofacial anomaliesCraniofacial anomalies Physical findings (e.g. white forelock)Physical findings (e.g. white forelock) Syndromes* involving hearing lossSyndromes* involving hearing loss

– Neurofibromatosis, osteopetrosis, Usher, Neurofibromatosis, osteopetrosis, Usher, Waardenburg, Alport, Pendred, Jervell & Waardenburg, Alport, Pendred, Jervell & Lange-NielsonLange-Nielson




Neurodegenerative disordersNeurodegenerative disorders– Hunter syndromeHunter syndrome– Sensory motor neuropathies (Frieidrich Sensory motor neuropathies (Frieidrich

ataxia, Charcot-Marie-Tooth)ataxia, Charcot-Marie-Tooth) Culture positive postnatal infections Culture positive postnatal infections

associated with HL*associated with HL*– Herpes, varicella, meningitisHerpes, varicella, meningitis

Head trauma (basal skull, temporal Head trauma (basal skull, temporal bone)*bone)*

Chemotherapy*Chemotherapy*


Medical WorkupMedical Workup

Complete prenatal & perinatal historyComplete prenatal & perinatal history Family Hx of onset of HL < age 30Family Hx of onset of HL < age 30 Physical for stigmata, ear tabs, cleftPhysical for stigmata, ear tabs, cleft

palate, cardiac, sketetal, palate, cardiac, sketetal, microcephalymicrocephaly

Refer to ENT/CT of temporal bonesRefer to ENT/CT of temporal bones Refer to Genetics and OphthalmologyRefer to Genetics and Ophthalmology Other: CMV, EKG, Developmental Other: CMV, EKG, Developmental

evaluationevaluation

CI Candidacy CriteriaCI Candidacy Criteria

3-6 month trial with hearing 3-6 month trial with hearing aids; lack of benefitaids; lack of benefit

Profound loss 90+dB (12 to Profound loss 90+dB (12 to 18 mos); >18 mos, Severe-18 mos); >18 mos, Severe-to-Profound 70 dB+to-Profound 70 dB+

No medical No medical contraindicationscontraindications

Rehab setting encouraging Rehab setting encouraging auditoryauditory

Family factors (motivation, Family factors (motivation, expectations)expectations)

Goals of Early Goals of Early InterventionIntervention Home based servicesHome based services Optimally, providers have experience & training with the Optimally, providers have experience & training with the

population and work to:population and work to:– Establish partnerships with familiesEstablish partnerships with families– Promote family competence & confidence in Promote family competence & confidence in

parenting childparenting child– Support family in providing a language-rich Support family in providing a language-rich

environment in everyday routinesenvironment in everyday routines– Support family to become informed decision makers Support family to become informed decision makers

for the childfor the child– Conduct ongoing assessments of outcomes Conduct ongoing assessments of outcomes

Adjust interventions as necessary to optimize outcomesAdjust interventions as necessary to optimize outcomes– Promote family access to formal and informal Promote family access to formal and informal

supportssupports– Provide culturally competent servicesProvide culturally competent services

Resources:Resources:

Early Early InterventionIntervention

Parent-to-ParentParent-to-Parent

Physician supportPhysician support

Contact State EHDI Contact State EHDI Coordinator – see Coordinator – see www.infanthearing.orgwww.infanthearing.org

www.nectac.org

www.handsandvoices.org www.beginningssvsc.com www.babyhearing.org

www.aap.org www.medicalhomeinfo.orwww.medicalhomeinfo.or

gg

Physician ResourcesPhysician Resources

http://www.medicalhomeinfo.org/screening/hearing.html

http://www.cdc.gov/ncbddd/dd/ddhi.htm

ALSO: hearing loss module on http://www.pedialink.org

www.babyhearing.orgwww.babyhearing.org

www.infanthearing.orgwww.infanthearing.org

www.infanthearing.org

Chapter Champion Chapter Champion ContactContact

For more information…For more information… Chapter Champion contact Chapter Champion contact

information and additional State information and additional State resources (like EHDI program) resources (like EHDI program) should be listed hereshould be listed here

ContributorsContributors

Mary Pat Moeller, Ph.D., BTNRHMary Pat Moeller, Ph.D., BTNRH Pat Stelmachowicz, Ph.D., BTNRHPat Stelmachowicz, Ph.D., BTNRH Don Uzendoski, M.D., AAP Chapter Don Uzendoski, M.D., AAP Chapter

Champion, BTNRHChampion, BTNRH Leisha Eiten, AUD, BTNRHLeisha Eiten, AUD, BTNRH Staci Gray, PA, BTNRHStaci Gray, PA, BTNRH Susan Wiley, M.D., AAP Chapter Susan Wiley, M.D., AAP Chapter

Champion, Cincinnati Children’s Champion, Cincinnati Children’s HospitalHospital

Roger Harpster, BTNRHRoger Harpster, BTNRH Diane Schmidt, BTNRHDiane Schmidt, BTNRH Skip Kennedy, BTNRHSkip Kennedy, BTNRH Dr. Karl White, Ph.D., NCHAMDr. Karl White, Ph.D., NCHAM Michelle Esquivel, AAPMichelle Esquivel, AAP

Project Supported by the National Institute on Deafness and Other Communication Disorders

(NIDCD/NIH) R25 DC04559; R25 DC006460

For additional information about this presentation orFor additional information about this presentation or

Universal NewbornUniversal Newborn Hearing ScreeningHearing Screening

contact: contact:

Boys Town National Research HospitalBoys Town National Research Hospital555 No. 30555 No. 30thth St. St.

Omaha, NE 68131Omaha, NE 68131

Dr. Mary Pat MoellerDr. Mary Pat Moeller(402) 452-5068(402) 452-5068

E-mail: [email protected]: [email protected]

Documents

Update on Newborn Hearing Screening NIDCD. National Goals for Hearing Screening (1-3-6) 1, 2 All infants will access hearing screening using a physiologic