HEARING LOSS IN INFANTS AND YOUNG CHILDREN:

PRESENTING PATIENTSInfants and children no older than 3 years of age with possible hearing loss initially indicated by:

• Parent concern• Didnotpassnewbornhearingscreening• Oneormoreriskfactorsforhearingloss,regardlessofscreeningresult(SeeRiskIndicatorssection)• Audiologicorothertestresultssuggestinghearinglossinoneorbothears

No child is too young to test:Delayinobtaininginterventionservicescancompromise languagedevelopmentandreducecommunicationandschoolperformance.Remember 1-3-6:

By 1 month of age |Obtainhearingscreennolaterthan1monthofageifnohearingscreeninghasbeendone.By 3 months of age | Referimmediatelytopediatricaudiologistif child did not pass hearing screening and audiologic evaluation1 has not been completed.Hearingstatusshouldbeconfirmedbyaudiologicevaluationnolaterthan3monthsofage.By 6 months of age | VerifyentryintoEarlyIntervention2servicesnolaterthan6monthsofage if hearing loss is confirmed.

If child passes hearing screening but displays risk factors, including caregiver concern: Assessriskfactor(s)andplanaudiologicmonitoringwithcaretakers.Late-onsethearinglosscan occuratanytimeafterbirthdespiteapassedhearingscreenatbirth3.

Considerations for Pediatric Primary Care Providers

KEY PRACTICE POINTS• Referallinfantswhodonotpasshearingscreeningfordiagnosticaudiologicevaluation.• Onceanydegreeofhearinglossisdiagnosed(including“transient”hearinglossassociatedwith

cleftpalate),referraltoEarlyInterventionmustbemadewithin7workingdaysofconfirmationofhearingloss.

• Ongoingcareforallhearinglosses(“transient”andpermanent)byanaudiologistisessential,inadditiontomedicalcare.

• Forchildrenwhopassedtheirbirth-screenbutareatriskforhearingloss:ensurethatadiagnosticaudiologicevaluationiscompletedatleastonceby24to30monthsofage4.

• Regardlessofprevioushearingscreeningoutcomes,allinfantswithorwithoutriskfactorsshouldreceivecontinuedtrackingofauditoryskills,languagemilestones,andmiddleearstatusduringwell-childvisitsinthemedicalhome.

RECOMMENDATIONS FOR CLINIC VISITHISTORY

• Reviewallmedicalrecordsandcasehistoryinformationforriskfactorsassociatedwithcongenitalhearinglossand/orlate-onsetorprogressivehearingloss(SeebelowRiskIndicatorsection).

• Reviewaudiologicrecordtoassessneedforfurtheraudiologictesting.• Elicitwhetherthereisparentalconcernaboutthechild’shearing.• Payspecialattentiontodysmorphicfeatures,andpresenceofearpitsorbranchialcysts.• Ifhearinglossetiologyremainsundeterminedconsiderreferringfamilytoageneticspecialistto

determinepossiblegeneticetiology.

PHYSICAL EXAM & REFERRAL• Everyinfantwithconfirmedhearinglossshouldbeevaluatedbyanotolaryngologistwhohas

knowledgeofpediatrichearinglossandhaveatleastonevisualacuityexaminationbyanophthalmologistwhoisexperiencedinevaluatinginfants.

• VerifywithparentsifreferraltoEarlyInterventionhasbeenmade2.• Alsoaskparentsifreferraltoparentsupporthasbeendone.ManystateEarlyHearingDetection

&Intervention(EHDI)programshaveparentsupportresourceshttp://www.infanthearing.org/states/index.html

TREATMENT• Hearingaidfittingshouldtakeplacewithinonemonthofdiagnosis,ifthe

child’sparents/caregivershavechosenhearingaids.• Medicalclearancebyanotolaryngologistforhearingaidfittingshould

notbedelayedwhileothermedicalexamsareon-going,norshouldsuchclearancebedelayeduntiladiagnosisofetiologyismade.

• Hearingaidfittingwhenthereischronicmiddleeareffusionshouldbeconsideredifotheroptionssuchasmyringotomyortubeinsertionarenotpossible,especiallywhenassociatedwithcleftpalateandDownSyndrome.

RISK INDICATORSRisk indicators5 associated with permanent congenital, delayed-onset, or progressive hearing loss in childhood: Risk indicators that are marked with a “§” are of greater concern for delayed-onset hearing loss.

1. Caregiverconcern§regardinghearing,speech,language,ordevelopmentaldelay.

2. Familyhistory§ofpermanentchildhoodhearingloss.

3. Neonatalintensivecareofmorethan5daysoranyofthefollowingregardlessoflengthofstay:ExtracorporealMembraneOxygenation(ECMO)§,assistedventilation,exposuretoototoxicmedications(gentamicinandtobramycin)orloopdiuretics(furosemide/Lasix),andhyperbilirubinemiathatrequiresexchangetransfusion.Any child who has been in the neonatal intensive care unit (NICU) for 5 or more days requires an Automated Auditory Brainstem Response (AABR) by an audiologist

4. Inuteroinfections,suchasCytomegalovirus(CMV)§,herpes,rubella,syphilis,andtoxoplasmosis.

5. Craniofacialanomalies,includingthosethatinvolvethepinna,earcanal,eartags,earpits,andtemporalboneanomalies.

6. Physicalfindings,suchaswhiteforelock,thatareassociatedwithasyndromeknowntoincludeasensorineuralorpermanentconductivehearingloss.

7. Syndromesassociatedwithhearinglossorprogressiveorlate-onsethearingloss,§suchasneurofibromatosis,osteopetrosis,andUshersyndrome;otherfrequentlyidentifiedsyndromesincludeWaardenburg,Alport,Pendred,andJervellandLange-Nielson.

8. Neurodegenerativedisorders§,suchasHuntersyndrome,orsensorymotorneuropathies,suchasFriedreichataxiaandCharcot-Marie-Toothsyndrome.

9. Culture-positivepostnatalinfectionsassociatedwithsensorineuralhearingloss§,includingconfirmedbacterialandviral(especiallyherpesvirusesandvaricella)meningitis.

10.Headtrauma,especiallybasalskull/temporalbonefracture§thatrequireshospitalization.

11.Chemotherapy§.

WEBSITES

FOOTNOTES

TO LOCATE: • Pediatric audiology services -EarlyHearingDetection&Intervention-PediatricAudiologyLinksto

Services(EHDI-PALS)http://ehdipals.org • State Early Hearing Detection and Intervention Programs - NationalCenterforHearingAssessment

andManagementhttp://www.infanthearing.org/states

OTHER RESOURCES• AmericanAcademyofAudiologywww.audiology.org • AmericanAcademyofFamilyPhysiciansPatient-CenteredMedicalHomeChecklist

http://www.aafp.org/practice-management.html• AmericanAcademyofOtolaryngologyHeadandNeckSurgerywww.entnet.org • AmericanAcademyofPediatricsNewbornandInfantHearingScreeningActivities

http://www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/PEHDIC/Pages/Early-Hearing-Detection-and-Intervention.aspx

• AmericanSpeech-Language-HearingAssociation(ASHA)www.asha.org • CDCEarlyHearingDetectionandIntervention(EHDI)www.cdc.gov/ncbddd/ehdi/• *JointCommitteeonInfantHearing(JCIH)www.jcih.org

CS248844

1. A complete audiologic evaluation for infants consists of (may vary depending on age, circumstances, diagnosis):

• Acousticimmittance(tympanometryandacousticreflexthresholds{high-frequencyprobetoneforinfantsunder6monthsofage})

• Otoacousticemissions(DistortionProductorTransient)• AuditoryBrainstemResponse(Air-andbone-conduction)forspecificfrequencies(inparticular,500Hzand

2000Hz,othersastimepermits).ABRprotocolstoassesspossibleAuditoryNeuropathywhenindicated.• AuditorySteady-StateResponseasasecondarymeasure(aftertone-burstABR).• BehavioralAudiometryatage-anddevelopmentally-appropriatestages.

2. Early Intervention according to Part C of IDEA 2004istheprocessofprovidingservices,educationandsupporttoyoungchildrenwhoaredeemedtohave

• anestablishedcondition,• adiagnosedphysicalormentalcondition(withahighprobabilityofresultinginadevelopmentaldelay),• anexistingdelayor• achildwhoisat-riskofdevelopingadelayor• specialneedthatmayaffecttheirdevelopmentorimpedetheireducation.

http://ectacenter.org/partc/partc.asp http://pediatrics.aappublications.org/content/early/2013/03/18/peds.2013-0008.citation

3. Newborn Hearing Screening: Lost to Documented Follow up Considerations for the Medical Home, AAPNewsAug.2013.http://www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/pehdic/pages/early-hearing-detection-and-intervention.aspx

4. Guidelines for Rescreening in the Medical Home Following a “Do Not Pass” Newborn Hearing Screening,AAPhttp://www.aap.org/en-us/advocacy-and-policy/aap-health-initiatives/PEHDIC/Documents/Rescreening_Guidelines.PDF

5. Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs: JointCommitteeonInfantHearing.PediatricsVol.120No.4October1,2007pp.898-921(doi:10.1542/peds.2007-2333)http://pediatrics.aappublications.org/content/120/4/898.full?ijkey=oj9BAleq21OlA&keytype=ref&siteid=aapjournals