8Why is Implementation of Newborn Hearing Screening Accelerating? Improved ScreeningTechniques/Equipment
9Acceptance By Policy Makers National Institutes of HealthAmerican Academy of PediatricsMaternal and Child Health BureauCenters for Disease Control & PreventionJoint Committee on Infant HearingAmerican Academy of AudiologyAmerican Speech-Language-Hearing AssociationNational Association of the Deaf
10Why is Implementation of Newborn Hearing Screening Accelerating? Improved ScreeningTechniques/EquipmentAcceptance byIncreased Number ofPolicy MakersSuccessful ProgramsPublicAwareness/Demand
11Why is Early Identification of Hearing Loss so Important? Hearing loss occurs more frequently than any other birth defect.
12Rate Per 1,000 of Permanent Childhood Hearing Loss in UNHS Programs Sample PrevalenceSite Size Per 1000Rhode Island (3/93 - 6/94) 16,Colorado (1/ /96) 41,New York (1/ /97) 69,Texas (1/94 - 6/97) ,Hawaii (1/ /96) 9,New Jersey (1/ /95) 15,
13Incidence per 10,000 of Congenital Defects/Diseases
14Why is Early Identification of Hearing Loss so Important? Hearing occurs more frequently than any other birth defect.Undetected hearing loss has serious negative consequences.
15Reading Comprehension Scores of Hearing and Deaf Students Grade EquivalentsAge in YearsSchildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.
16Effects of Unilateral Hearing Loss Normal HearingUnilateral Hearing LossKeller & Bundy (1980)Math(n = 26; age = 12 yrs)LanguagePeterson (1981)Math(n = 48; age = 7.5 yrs)LanguageBess & Thorpe (1984)Social(n = 50; age = 10 yrs)MathBlair, Peterson & Viehweg (1985)(n = 16; age = 7.5 yrs)LanguageMathCulbertson & Gilbert (1986)(n = 50; age = 10 yrs)LanguageSocialAverage Results0th10th20th30th40th50th60thMath = 30th percentilePercentile RankLanguage = 25th percentileSocial = 32nd percentile
17Effects of Mild Fluctuating Conductive Hearing Loss Teele, et al., 1990194 children followed prospectively from 0-7 years.Days child had otitis media between 0-3 years assessed during normal visits to physician.Data on intellectual ability, school achievement, and language competency individuallymeasured at 7 years by "blind" diagnosticians.Results for children with less than 30 days OME were compared to children with more than130 days adjusted for confounding variables.Effect Size forOutcome Measure Less vs. More OMEWISC-R Full Scale.62Metropolitan Achievement TestMath.48Reading.37Goldman Fristoe Articulation.43Teele, D.W., Klein, J.O., Chase, C., Menyuk, P., Rosner, B.A., and the Greater Boston Otitis media Study Group (1990).Otitis media in infancy and intellectual ability, school achievement, speech, and language at age 7 years.The Journalof Infectious Diseases,162,
18Why is Early Identification of Hearing Loss so Important? Hearing loss occurs more frequently than any other birth defect.Undetected hearing loss has serious negative consequences.There are dramatic benefits associated with early identification of hearing loss.
19Yoshinaga-Itano, et al., 1996Compared language abilities of hearing-impaired children identifiedbefore 6 months of age (n = 46) with similar children identified after 6months of age (n = 63).All children had bilateral hearing loss ranging from mild to profound,and normally-hearing parents.Language abilities measured by parent report using the MinnesotaChild Development Inventory (expressive and comprehension scales)and the MacArthur Communicative Developmental Inventories(vocabulary).Cross-sectional assessment with children categorized in 4 differentage groups.Yoshinaga-Itano, C., Sedey, A., Apuzzo, M., Carey, A., Day, D., & Coulter, D. (July 1996).The effect of earlyidentification on the development of deaf and hard-of-hearing infants and toddlers. Paper presented at theJoint Committee on Infant Hearing Meeting, Austin, TX.
20Expressive Language Scores for Hearing Impaired Children Identified Before and After 6 Months of Age353025Language Age in Months201510Identified BEFORE 6 Months5Identified AFTER 6 Months13-18 mos19-24 mos25-30 mos31-36 mos(n = 15/8)(n = 12/16)(n = 11/20)(n = 8/19)Chronological Age in Months
21Boys Town National Research Hospital Study of Earlier vs. Later 129 deaf and hard-of-hearing children assessed 2x each year.Assessments done by trained diagnostician as normal part of early intervention program.6Identified <6 mos (n = 25)5Identified >6 mos (n = 104)4Language Age (yrs)3184.108.40.206.220.127.116.11.24.8Age (yrs)Moeller, M.P. (1997).Personal communication,
22Good work, but I think we might need just a little more detail right Implementing Effective EHDI ProgramsoutThen amiracleoccursStartGood work,but I think we mightneed just a littlemore detail righthere.
23Is the Glass Half Empty or Half Full? More than 2.5 million babies are screened every year prior to dischargeLess than 30 hospitals with UNHS in 1993; compared with almost 2500 today37 states have passed legislation related to newborn hearing screeningOr half empty?1,500 hospitals are not yet screening for hearing lossAlmost 1.5 million babies are NOT screened every year prior to dischargeExisting legislation is of variable qualityFollow-up rates are often alarmingly lowSome hospitals have unacceptably high referral rates
24Status of Early Hearing Detection and Intervention (EHDI) in the United States Diagnosis before 3 monthsScreening before 1 monthIntervention before 6 monthsMedical HomeData Management and TrackingProgram Evaluation and Quality AssuranceFamily Support!!
25Status of EHDI Programs in the US: Universal Newborn Hearing Screening With over half of all babies are screened prior to discharge, has newborn hearing screening become the standard of care?There are hundreds of excellent programs regardless of the type of equipment or protocol usedMany programs are still struggling with high refer rates and poor follow-up
27Status of EHDI Programs in the United States Universal Newborn Hearing ScreeningEffective Tracking and Follow-up as a part of the Public Health System
28Purposes of an EHDI Data System ResearchProgram Improvementand Quality AssuranceScreeningDiagnosisInterventionMedical, Audiological andEducational
29Rate Per 1000 of Permanent Childhood Hearing Loss in UNHS Programs Sample Prevalence % of RefersSite Size Per with DiagnosisRhode Island (3/93 - 6/94) 16, %Colorado (1/ /96) 41, %New York (1/ /96) 27, %Utah (7/ /94) , %Hawaii (1/ /96) 9, %
30Tracking "Refers" is a Major Challenge (continued)Initial RescreenBirths Screened Refer Rescreen ReferRhode Island53,12152,6595,3974,575677(1/ /96)(99%)(10%)(85%)(1.3%)Hawaii10,5849,6051,204991121(1/ /96)(91%)(12%)(82%)(1.3%)New York28,95127,9381,9531,040245(1/96-12/96)(96.5%)(7%)(53%)(0.8%)
31Options for Developing an EHDI Patient/Data Management System Develop your ownModify an existing system, for exampleelectronic birth certificate, or“heelstick” data management systemPurchase an existing system
32Can EHDI Data Management be Combined with Heelstick? Both do initial screening of babies in the nursery prior to hospital dischargeBoth do outpatient screening for many babiesPoor follow-up is biggest challenge for hearing screeningHeelstick programs extremely successful with follow-upInfrastructure for Heelstick follow-up already existsEHDI information management looks deceptively simple at first glance.
33Issues to be Resolved Before Combining EHDI with Heelstick Follow-up Systems Recording results of EHDI on heelstick form is only the beginningTiming and procedures of data collection/entry are quite different for EHDIElectronic transfer of data from screening equipment to data formAvailability of staff with expertise in EHDI issues to do follow-upHospital staff need timely access to dataCosts of modifying data entry/ reporting systems and duplicate data entryEHDI information management looks deceptively simple at first glance.
34Efficiency of Early Hearing Detection and Intervention in a Statewide Evaluation (6 mos.)(n=43,547) (n=46,771) (n=23,307)Inpatient Refer Rates (state average) % 85.5% 87.5%10 most effective hospitals % 93.4% 93.7%10 least effective hospitals % 63.4% 74.4%Outpatient completion (state average) % 67.1% 68.3%10 most effective hospitals % 95.9% 94.7%10 least effective hospitals % 52.9% %Reported Completion of Diagnostic 133 of of of 110*Evaluations (state average) % 43.4% 40%% of babies who complete Diagnostic of of of 41*Eval & have permanent hearing loss % 39.4% 29.3%Number of babies still “in process”*only 3 months worth of data
35Status of EHDI Programs in the United States Universal Newborn Hearing ScreeningEffective Tracking and Follow-up as a part of the Public Health SystemAppropriate and Timely Diagnosis of the Hearing Loss
36State Coordinator’s Ratings of Obstacles to Effective EHDI Programs Serious or ExtremelySerious ObstacleShortage of qualified pediatric audiologists 49%Physicians don’t know enough about newbornhearing screening, diagnosis, and intervention %Unwillingness of third-party payersto reimburse for hearing screening %
37Status of EHDI Programs in the US: Audiological Diagnosis Equipment and techniques for diagnosis of hearing loss in infants continues to improveSevere shortages in experienced pediatric audiologists delays confirmation of hearing lossState coordinators estimate only 56.1% “receive diagnostic evaluations by 3 months of age
38Confirmation of Permanent Hearing Loss 35Coplan (1987)19Eissman et al. (1987)30Gustason (1987)30Meadow-Orlans (1987)24Yoshinago-Itano (1995)25Stein et al. (1990)31Mace et al. (1991)56O'Neil (1996)3Johnson et al. (1997)*3Vohr et al. (1998)*10203040506070Average Age in Months
39Status of EHDI Programs in the United States Universal Newborn Hearing ScreeningEffective Tracking and Follow-up as a part of the Public Health SystemAppropriate and Timely Diagnosis of the Hearing LossPrompt Enrollment in Appropriate Early Intervention
40Status of EHDI Programs in the US: Early Intervention Current system designed to serve infants with bilateral severe/profound losses---but, majority of those identified have mild, moderate, and unilateral lossesPart C of IDEA is severely under utilizedState Coordinators estimate:Only 53% of infants with hearing loss are enrolled in EI programs before 6 months of ageOnly 31% of states have adequate range of choices for EI programs
41Who is Eligible for Part C Services? Child has a profound, permanent sensorineural hearing loss in both ears (PTA>100 dB)Child has a profound, permanent sensorineural hearing loss in one ear (PTA>100dB), but normal hearing in the other earChild has a moderate, permanent sensorineural hearing loss in both ears (PTA=55dB)Child has a mild, permanent sensorineural hearing in both ears (PTA=35dB)Child has a mild, fluctuating conductive hearing loss (PTA=35dB) in both ears due to otitis media (ear infections)
42Hawai'i EHDI Progress Age of Identification and Intervention 60 50 40 Age in Months302010pre1992199319941995199619971998YearData from Hawai’I Zero to Three Project
43Status of EHDI Programs in the United States Universal Newborn Hearing ScreeningEffective Tracking and Follow-up as a part of the Public Health SystemAppropriate and Timely Diagnosis of the Hearing LossPrompt Enrollment in Appropriate Early InterventionA Medical Home for all Newborns
44What Is a Medical Home?A primary care physician provides care which is:AccessibleFamily-centeredComprehensiveContinuousCoordinatedCompassionateCulturally effective
45EHDI and the Medical Home Birthing HospitalAudiologyParent GroupsMental HealthPrimary ProviderChild/FamilyENT3rd Party PayersDeaf CommunityEarly Intervention ProgramsGeneticsServices for Hearing Loss
46Types of Hearing LossSensorineural versus Conductive versus MixedCongenital versus Acquired (prelingual or post lingual)Progressive versus non-progressiveSyndromic versus non-syndromicFamilial versus sporadic
48Common Forms of Syndromic Hearing Loss SyndromeMain Features (in addition to hearing loss)AlportKidney problemsBranchio-oto-renalNeck cysts and kidney problemsJervell and Lange-NielsenHeart problemsNeurofibromatosis Type 2Nerve tumors near the earPendredThyroid enlargementSticklerUnusual facial features, eye problems, arthritisUsherProgressive blindnessWaardenburgSkin pigment changes
49Benefits of Genetic Testing for Hearing Loss Determine the chances of hearing loss in subsequent childrenAvoid unecessary (and often costly) tests such as electroretinograms, temporal bone imaging, and electrocardigramsAnticipate potential health problemsMonitoring for myopia and early retinal detachment for Stickler syndromeRenal examinations can identify kidney problems in BORVitamin A therapy may be beneficial in slowing retinal degeneration in child with Usher syndromeTreatment of children with Jervell and Lange-Nielsen syndrome can minimize cardiac complicationsDispel misinformation and offer emotional support by allaying parental guilt
50Connexin 26A protein responsible for intracellular communication (transfer of ions between the hair cells in the cochlea and their support cells)Responsible for 20-30% of all congenital hearing lossSeveral different mutations35delG is found in 2-3% of all Caucasians of European descent167delT is found in 5% of Ashkenazi Jewish populationUsually recessive, occasionally dominantAlmost always results in hearing loss that is:CongenitalSevere-profoundNon-progressiveNon-syndromic
51Susceptibility to Aminoglycoside Ototoxicity mitochondrial mutation A1555G in the rRNA gene in combination with exposure to aminoglycoside antibiotics results in rapid onset of hearing lossprevalent in Chinese and other oriental ethnic groups but has also been found in Caucasians, Greeks, etc.
52Status of EHDI Programs in the United States Universal Newborn Hearing ScreeningEffective Tracking and Follow-up as a part of the Public Health SystemAppropriate and Timely Diagnosis of the Hearing LossPrompt Enrollment in Appropriate Early InterventionA Medical Home for all NewbornsCulturally Competent Family Support
53Emotions of Families with a Deaf or Hard of Hearing Baby (grief) Reactions to Unexpected Diagnosis(pressure) Urgency of Communication Decisions Search(confusion) Search for Experienced Professionals(isolation) Availability of Services and Support
54Communication Choices American Sign LanguageTotal CommunicationAuditory VerbalAuditory-OralCued Speech
55Information Wanted vs. Received by Parents at Hearing Loss ConfirmationMartin, George, O'Neal, & Daly (1987); *Sweetow & Barrager (1980)Degree of lossAuditory systemAmplificationEducational optionsSpeech/Lang devEtiologyHome activities*Written Information*Financial Support*Emotional Support*Parent Contacts*Referral Sources20406080100WantedReceived
56Parent’s Attitudes About Newborn Hearing Screening (Results of a Statewide Evaluation)After all hearing tests were completed, how did you feel?Strongly Agreeor AgreeWorried about my baby’s hearing %Confused about the results of screening tests %Glad hearing screening is done at this hospital 91%Confident the hearing tests were correct %Frustrated by how long it took to get results 13%Happy with the professional way screening was done 86%Confident about what I needed to do next %
57After all hearing tests were completed, how did you feel? If the analysis is limited to those whose babies did not pass the inpatient or outpatient screenAfter all hearing tests were completed, how did you feel?Strongly Agreeor Agreetotal group subgroupWorried about my baby’s hearing % 24%Confused about the results of screening tests % 24%Glad hearing screening is done at this hospital 91% 70%Confident the hearing tests were correct % 70%Frustrated by how long it took to get results 13% 28%Happy with the professional way screening was done 86% 76%Confident about what I needed to do next % 56%
58EHDI Materials Available from “State” Programs (n=54)General Screening Brochure statesWhat To Do If Your Baby Refers statesWhat To Do If Your Baby has a Hearing Loss 41 statesGuidelines for Audiologic Diagnostic Evaluations statesList of Qualified Pediatric Audiologists 39 statesBrochure about Genetics of Hearing Loss 7 statesFair or Excellent Availability of Materials inother Languages states
59Efforts by the Federal Government to Promote Early Identification of Hearing Loss Federal funding for research and program developmentNIH Consensus Development Conference in 1993Consortium for Universal Newborn Hearing Screening funded in 1993Marion Downs National Center for Infant Hearing Established in 1996National EHDI Technical Assistance System Established in 2000NIH and Dept of Educ Projects at Boys Town and University of North Carolina
60National EHDI Technical Assistance System EHDI Network members located in each of ten geographic regions
61National EHDI Assistance Network Region VIII(91% currently bornin UNHS hospitals)Terry FoustRegion V(26% currently bornin UNHS hospitals)Karen MunozRegion II(16% currently bornin UNHS hospitals)Beth PrieveIPuerto RicoVirgin IslandsRegion I(38% currently bornin UNHS hospitals)Antonia MaxonBVIIIXIIVRegion X(21% currently bornin UNHS hospitals)Curt WhitcombIIIVIIRegion III(49% currently bornin UNHS hospitals)Sean KastetterIXIVVI-Region IX(23% currently bornin UNHS hospitals)Randi WinstonYusnita WeiratherGuam, American Samoa,Marshall Islands, Palau,No. Mariana Islands,Fed. MicronesiaRegion IV(46% currently bornin UNHS hospitals)Faye McCollisterRegion VI(38% currently born in UNHS hospitals)Karen DittyPatti MartinRegion VII(33% currently bornin UNHS hospitals)Les Schmeltz= indicates the locations of MCHB Regional Offices
62Examples of Network Activities State-wide EHDI meetingsIndividualized TA with state EHDI programsTelephone Conference calls with State EDHI CoordinatorsAssist with development of state plans and grant applicationsRegional workshops on Diagnostic ABR6 weeks of on-line preparation2 day face-to-face workshop3 month follow-up practicum
63National EDHI Meetings Next meeting: February 24-26, 2002 (Atlanta)Speakers, panels, and round tablesState displaysProduct exhibits (commercial and non-profit)Networking opportunities
64National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regionsInformation dissemination and training
65Support for Program Implementation Implementation GuideBooklets for AAP and March of DimesMaterials posted atVideo tape for parentsEvaluation instruments and procedures
66Sound Ideas Newsletter Topical articles, suggestions for program improvementUpcoming eventsAvailable online or mailed
67National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regionsInformation dissemination and trainingWeb site (www.infanthearing.org)
70National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regionsInformation dissemination and trainingWeb site (www.infanthearing.org)Collaboration with other groups and agencies
71National EHDI Technical Assistance System (continued) Collaboration with Other Groups and AgenciesGroups actively promoting and assisting with EHDI activitiesAG Bell, NCHH, ASHA, AAA, JCIH, AAP, SKI-HI, ASDC, Boys Town, DSHPSHWARelevant groups whose main focus has been elsewhereNEC*TAS, Early Head Start, 0-3, Family Voices, NCCC, AMCHP, AHEC, March of Dimes, MCH Health Policy Center
72Collaboration with AAP AAP News articleAssisted with booklets for physicians and parentsCollaborated on implementation of recently funded EHDI InitiativeChapter ChampionsSpeaker’s KitBulletin BoardPhysician GuidelinesAnalysis of legislationNational survey of physicians
73“Take Home” Messages Deceptively simple—the devil is in the details EHDI is more than screeningMedical Home is where the action isThoughtful, ongoing, self appraisalYou’re not alone
74“I am a great believer in luck, and I find that the harder I work, the more I have of it.” ---Thomas Jefferson