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Universal Newborn Hearing Screening What It Is and How It Happens Karl White, Ph.D. National Center for Hearing Assessment and Management Utah State University.

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Presentation on theme: "Universal Newborn Hearing Screening What It Is and How It Happens Karl White, Ph.D. National Center for Hearing Assessment and Management Utah State University."— Presentation transcript:

1 Universal Newborn Hearing Screening What It Is and How It Happens Karl White, Ph.D. National Center for Hearing Assessment and Management Utah State University www.infanthearing.org

2 Early Hearing Detection and Interveniton (EHDI) Programs

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5 Percentage of Newborns Screened for Hearing in the United States (Dec 2001). Percentage of Births Screened 90%+ 21 - 50% 1 - 20% 3 51 - 90%

6 States with Legislative Mandates Related to Universal Newborn Hearing Screening Status of UNHS Legislative Mandates States with mandates No mandate No mandate, but statewide programs

7 National Universal Newborn Hearing Screening Programs Austria United Kingdom Poland Flemish Belgium Singapore Canada (Ontario)

8 Improved Screening Techniques/Equipment Why is Implementation of Newborn Hearing Screening Accelerating?

9 Acceptance By Policy Makers National Institutes of Health American Academy of Pediatrics Maternal and Child Health Bureau Centers for Disease Control & Prevention Joint Committee on Infant Hearing American Academy of Audiology American Speech-Language-Hearing Association National Association of the Deaf

10 Improved Screening Techniques/Equipment Acceptance by Policy Makers Why is Implementation of Newborn Hearing Screening Accelerating? Increased Number of Successful Programs Public Awareness/Demand

11 Why is Early Identification of Hearing Loss so Important? Hearing loss occurs more frequently than any other birth defect.

12 Rate Per 1,000 of Permanent Childhood Hearing Loss in UNHS Programs Sample Prevalence Site Size Per 1000 Rhode Island (3/93 - 6/94) 16,3951.71 Colorado (1/92 - 12/96) 41,9762.56 New York (1/95 - 12/97) 69,7611.95 Texas (1/94 - 6/97) 52,5082.15 Hawaii (1/96 - 12/96) 9,6054.15 New Jersey (1/93 - 12/95) 15,7493.30

13 Incidence per 10,000 of Congenital Defects/Diseases

14 Why is Early Identification of Hearing Loss so Important? Hearing occurs more frequently than any other birth defect. Undetected hearing loss has serious negative consequences.

15 Reading Comprehension Scores of Hearing and Deaf Students Age in Years Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press. Grade Equivalents

16 Effects of Unilateral Hearing Loss Math Language Math Language Social Math Language Math Language Social 0th10th20th30th40th50th60th Percentile Rank Normal HearingUnilateral Hearing Loss Keller & Bundy (1980) (n = 26; age = 12 yrs) Peterson (1981) (n = 48; age = 7.5 yrs) Bess & Thorpe (1984) (n = 50; age = 10 yrs) Blair, Peterson & Viehweg (1985) (n = 16; age = 7.5 yrs) Culbertson & Gilbert (1986) (n = 50; age = 10 yrs) Average Results Math = 30th percentile Language = 25th percentile Social = 32nd percentile

17 Effects of Mild Fluctuating Conductive Hearing Loss Teele, et al., 1990 194 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 individually measured at 7 years by "blind" diagnosticians. Results for children with less than 30 days OME were compared to children with more than 130 days adjusted for confounding variables. Effect Size for Outcome Measure Less vs. More OME WISC-R Full Scale.62 Metropolitan Achievement Test Math.48 Reading.37 Goldman Fristoe Articulation.43 Teele, 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 Journal of Infectious Diseases,162, 685-694.

18 Why 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.

19 Yoshinaga-Itano, et al., 1996 Compared language abilities of hearing-impaired children identified before 6 months of age (n = 46) with similar children identified after 6 months 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 Minnesota Child Development Inventory (expressive and comprehension scales) and the MacArthur Communicative Developmental Inventories (vocabulary). Cross-sectional assessment with children categorized in 4 different age groups. Yoshinaga-Itano, C., Sedey, A., Apuzzo, M., Carey, A., Day, D., & Coulter, D. (July 1996).The effect of early identification on the development of deaf and hard-of-hearing infants and toddlers. Paper presented at the Joint Committee on Infant Hearing Meeting, Austin, TX.

20 13-18 mos (n = 15/8) 19-24 mos (n = 12/16) 25-30 mos (n = 11/20) 31-36 mos (n = 8/19) 0 5 10 15 20 25 30 35 Identified BEFORE 6 Months Identified AFTER 6 Months Expressive Language Scores for Hearing Impaired Children Identified Before and After 6 Months of Age Chronological Age in Months Language Age in Months

21 0.81.21.82.22.83.23.84.24.8 0 1 2 3 4 5 6 Identified <6 mos (n = 25) Identified >6 mos (n = 104) Age (yrs) Language Age (yrs) Boys Town National Research Hospital Study of Earlier vs. Later Moeller, M.P. (1997).Personal communication, moeller@boystown.org 129 deaf and hard-of-hearing children assessed 2x each year. Assessments done by trained diagnostician as normal part of early intervention program.

22 Good work, but I think we might need just a little more detail right here. Implementing Effective EHDI Programs Then a miracle occurs out Start

23 Half full? More than 2.5 million babies are screened every year prior to discharge Less than 30 hospitals with UNHS in 1993; compared with almost 2500 today 37 states have passed legislation related to newborn hearing screening Or half empty? 1,500 hospitals are not yet screening for hearing loss Almost 1.5 million babies are NOT screened every year prior to discharge Existing legislation is of variable quality Follow-up rates are often alarmingly low Some hospitals have unacceptably high referral rates Is the Glass Half Empty or Half Full?

24 Status of Early Hearing Detection and Intervention (EHDI) in the United States Screening before 1 month Diagnosis before 3 months Intervention before 6 months Medical Home Data Management and Tracking Program Evaluation and Quality Assurance Family Support!!

25 Status 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 used Many programs are still struggling with high refer rates and poor follow-up

26 Typical UNHS Screening Protocols (example for 1,000 newborns) Hearing Loss=3 Normal Hearing=37 Diagnosis n=40 Inpatient Screening Fail=40 Pass=960 1 Stage AABR Hearing Loss=3 Normal Hearing=7 Inpatient Screening Pass=920 Fail=80 Outpatient Screening n=80 Diagnosis n=10 Pass=70 Fail=10 2 Stage OAE Diagnosis n=20 Inpatient Screening Pass=980 Fail=20 Hearing Loss=3 Normal Hearing=17 1 Stage OAE / AABR

27 Status of EHDI Programs in the United States Universal Newborn Hearing Screening Effective Tracking and Follow-up as a part of the Public Health System

28 Purposes of an EHDI Data System Screening Research DiagnosisIntervention Medical, Audiological and Educational Program Improvement and Quality Assurance

29 Rate Per 1000 of Permanent Childhood Hearing Loss in UNHS Programs Sample Prevalence % of Refers Site Size Per 1000 with Diagnosis Rhode Island (3/93 - 6/94) 16,3951.71 42% Colorado (1/92 - 12/96) 41,9762.56 48% New York (1/96 - 12/96) 27,9381.65 67% Utah (7/93 - 12/94) 4,0122.99 73% Hawaii (1/96 - 12/96) 9,6054.15 98%

30 Tracking "Refers" is a Major Challenge (continued) Initial Rescreen Births Screened Refer Rescreen Refer Rhode Island53,12152,659 5,397 4,575 677 (1/93 - 12/96) (99%) (10%) (85%) (1.3%) Hawaii10,5849,605 1,204 991 121 (1/96 - 12/96) (91%) (12%) (82%) (1.3%) New York28,95127,938 1,953 1,040 245 (1/96-12/96) (96.5%) (7%) (53%) (0.8%)

31 Options for Developing an EHDI Patient/Data Management System Develop your own Modify an existing system, for example electronic birth certificate, or heelstick data management system Purchase an existing system

32 Can EHDI Data Management be Combined with Heelstick? Both do initial screening of babies in the nursery prior to hospital discharge Both do outpatient screening for many babies Poor follow-up is biggest challenge for hearing screening Heelstick programs extremely successful with follow-up Infrastructure for Heelstick follow-up already exists

33 Issues to be Resolved Before Combining EHDI with Heelstick Follow-up Systems Recording results of EHDI on heelstick form is only the beginning Timing and procedures of data collection/entry are quite different for EHDI Electronic transfer of data from screening equipment to data form Availability of staff with expertise in EHDI issues to do follow-up Hospital staff need timely access to data Costs of modifying data entry/ reporting systems and duplicate data entry

34 Efficiency of Early Hearing Detection and Intervention in a Statewide Evaluation 1999 2000 2001(6 mos.) (n=43,547) (n=46,771) (n=23,307) Inpatient Refer Rates (state average) 85.2%85.5%87.5% 10 most effective hospitals 92.8%93.4%93.7% 10 least effective hospitals 70.7%63.4%74.4% Outpatient completion (state average) 70.1%67.1%68.3% 10 most effective hospitals 94.5%95.9%94.7% 10 least effective hospitals 45.3%52.9%58.08% Reported Completion of Diagnostic 133 of 357 165 of 380 41 of 110* Evaluations (state average) 37.3%43.4%40% % of babies who complete Diagnostic 33 of 133 65 of 165 12 of 41* Eval & have permanent hearing loss 24.8%39.4%29.3% Number of babies still in process 224 215 69 *only 3 months worth of data

35 Status of EHDI Programs in the United States Universal Newborn Hearing Screening Effective Tracking and Follow-up as a part of the Public Health System Appropriate and Timely Diagnosis of the Hearing Loss

36 State Coordinators Ratings of Obstacles to Effective EHDI Programs Serious or Extremely Serious Obstacle Shortage of qualified pediatric audiologists49% Physicians dont know enough about newborn hearing screening, diagnosis, and intervention 41% Unwillingness of third-party payers to reimburse for hearing screening 28%

37 Status of EHDI Programs in the US: Audiological Diagnosis Equipment and techniques for diagnosis of hearing loss in infants continues to improve Severe shortages in experienced pediatric audiologists delays confirmation of hearing loss State coordinators estimate only 56.1% receive diagnostic evaluations by 3 months of age

38 Average Age in Months 3 3 35 19 30 24 25 31 56 Coplan (1987) Eissman et al. (1987) Gustason (1987) Meadow-Orlans (1987) Yoshinago-Itano (1995) Stein et al. (1990) Mace et al. (1991) O'Neil (1996) Johnson et al. (1997)* Vohr et al. (1998)* 010203040506070 Confirmation of Permanent Hearing Loss

39 Status of EHDI Programs in the United States Universal Newborn Hearing Screening Effective Tracking and Follow-up as a part of the Public Health System Appropriate and Timely Diagnosis of the Hearing Loss Prompt Enrollment in Appropriate Early Intervention

40 Status 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 losses Part C of IDEA is severely under utilized State Coordinators estimate: – Only 53% of infants with hearing loss are enrolled in EI programs before 6 months of age –Only 31% of states have adequate range of choices for EI programs

41 Who 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 ear Child 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)

42 Hawai'i EHDI Progress Age of Identification and Intervention Data from HawaiI Zero to Three Project pre1992199319941995199619971998 Year 0 10 20 30 40 50 60 Age in Months IdentificationIntervention

43 Status of EHDI Programs in the United States Universal Newborn Hearing Screening Effective Tracking and Follow-up as a part of the Public Health System Appropriate and Timely Diagnosis of the Hearing Loss Prompt Enrollment in Appropriate Early Intervention A Medical Home for all Newborns

44 What Is a Medical Home? A primary care physician provides care which is: Accessible Family-centered Comprehensive Continuous Coordinated Compassionate Culturally effective

45 EHDI and the Medical Home Parent Groups Mental Health Birthing Hospital Audiology Primary Provider Child/Family ENT Genetics Early Intervention Programs 3rd Party Payers Deaf Community Services for Hearing Loss

46 Types of Hearing Loss Sensorineural versus Conductive versus Mixed Congenital versus Acquired (prelingual or post lingual) Progressive versus non-progressive Syndromic versus non-syndromic Familial versus sporadic

47 ~50% Environmental Genetic ~70% ~30% CMV meningitis rubella prematurity head trauma asphyxiation ototoxicity hyperbilirubin other infections Syndromic Alport Norrie Pendred Usher Waardenburg Branchio-oto-renal Jervell and Lange-Nielsen Non-syndromic Autosomal dominant Autosomal recessive X-Linked Mitochondrial 21% 77% ~1% Congenital Hearing Loss What Causes Hearing Loss?

48 Common Forms of Syndromic Hearing Loss Syndrome Main Features (in addition to hearing loss) AlportKidney problems Branchio-oto-renalNeck cysts and kidney problems Jervell and Lange-NielsenHeart problems Neurofibromatosis Type 2Nerve tumors near the ear PendredThyroid enlargement SticklerUnusual facial features, eye problems, arthritis UsherProgressive blindness WaardenburgSkin pigment changes

49 Benefits of Genetic Testing for Hearing Loss Determine the chances of hearing loss in subsequent children Avoid unecessary (and often costly) tests such as electroretinograms, temporal bone imaging, and electrocardigrams Anticipate potential health problems –Monitoring for myopia and early retinal detachment for Stickler syndrome –Renal examinations can identify kidney problems in BOR –Vitamin A therapy may be beneficial in slowing retinal degeneration in child with Usher syndrome –Treatment of children with Jervell and Lange-Nielsen syndrome can minimize cardiac complications Dispel misinformation and offer emotional support by allaying parental guilt

50 Connexin 26 A 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 loss Several different mutations 35delG is found in 2-3% of all Caucasians of European descent 167delT is found in 5% of Ashkenazi Jewish population Usually recessive, occasionally dominant Almost always results in hearing loss that is : Congenital Severe-profound Non-progressive Non-syndromic

51 Susceptibility to Aminoglycoside Ototoxicity mitochondrial mutation A1555G in the rRNA gene in combination with exposure to aminoglycoside antibiotics results in rapid onset of hearing loss prevalent in Chinese and other oriental ethnic groups but has also been found in Caucasians, Greeks, etc.

52 Status of EHDI Programs in the United States Universal Newborn Hearing Screening Effective Tracking and Follow-up as a part of the Public Health System Appropriate and Timely Diagnosis of the Hearing Loss Prompt Enrollment in Appropriate Early Intervention A Medical Home for all Newborns Culturally Competent Family Support

53 Emotions 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

54 Communication Choices American Sign Language Total Communication Auditory Verbal Auditory-Oral Cued Speech

55 Wanted Received

56 Parents Attitudes About Newborn Hearing Screening (Results of a Statewide Evaluation) After all hearing tests were completed, how did you feel? Strongly Agree or Agree Worried about my babys hearing 11% Confused about the results of screening tests 10% Glad hearing screening is done at this hospital91% Confident the hearing tests were correct 91% Frustrated by how long it took to get results13% Happy with the professional way screening was done86% Confident about what I needed to do next 88%

57 If the analysis is limited to those whose babies did not pass the inpatient or outpatient screen After all hearing tests were completed, how did you feel? Strongly Agree or Agree total group subgroup Worried about my babys hearing 11%24% Confused about the results of screening tests 10%24% Glad hearing screening is done at this hospital91%70% Confident the hearing tests were correct 91%70% Frustrated by how long it took to get results13%28% Happy with the professional way screening was done86%76% Confident about what I needed to do next 88%56%

58 EHDI Materials Available from State Programs (n=54) General Screening Brochure 39 states What To Do If Your Baby Refers 35 states What To Do If Your Baby has a Hearing Loss41 states Guidelines for Audiologic Diagnostic Evaluations 30 states List of Qualified Pediatric Audiologists39 states Brochure about Genetics of Hearing Loss 7 states Fair or Excellent Availability of Materials in other Languages 34 states

59 Efforts by the Federal Government to Promote Early Identification of Hearing Loss Federal funding for research and program development NIH Consensus Development Conference in 1993 Consortium for Universal Newborn Hearing Screening funded in 1993 Marion Downs National Center for Infant Hearing Established in 1996 National EHDI Technical Assistance System Established in 2000 NIH and Dept of Educ Projects at Boys Town and University of North Carolina

60 National EHDI Technical Assistance System EHDI Network members located in each of ten geographic regions

61 Region I (38% currently born in UNHS hospitals) Antonia MaxonB Region IV (46% currently born in UNHS hospitals) Faye McCollister Region III (49% currently born in UNHS hospitals) Sean Kastetter Region VI (38% currently born in UNHS hospitals) Karen Ditty Patti Martin Region VII (33% currently born in UNHS hospitals) Les Schmeltz Region IX (23% currently born in UNHS hospitals) Randi Winston Yusnita Weirather Region II (16% currently born in UNHS hospitals) Beth Prieve Region V (26% currently born in UNHS hospitals) Karen Munoz Region X (21% currently born in UNHS hospitals) Curt Whitcomb Region VIII (91% currently born in UNHS hospitals) Terry Foust National EHDI Assistance Network - VIII IX VII VI V IV III II I X Guam, American Samoa, Marshall Islands, Palau, No. Mariana Islands, Fed. Micronesia Puerto Rico Virgin Islands = indicates the locations of MCHB Regional Offices

62 Examples of Network Activities State-wide EHDI meetings Individualized TA with state EHDI programs Telephone Conference calls with State EDHI Coordinators Assist with development of state plans and grant applications Regional workshops on Diagnostic ABR –6 weeks of on-line preparation –2 day face-to-face workshop –3 month follow-up practicum

63 National EDHI Meetings Next meeting: February 24-26, 2002 (Atlanta) Speakers, panels, and round tables State displays Product exhibits (commercial and non-profit) Networking opportunities

64 National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regions Information dissemination and training

65 Support for Program Implementation Implementation Guide Booklets for AAP and March of Dimes Materials posted at www.infanthearing.org www.infanthearing.org Video tape for parents Evaluation instruments and procedures

66 Sound Ideas Newsletter Topical articles, suggestions for program improvement Upcoming events Available online or mailed

67 National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regions Information dissemination and training Web site (www.infanthearing.org)www.infanthearing.org

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69 www. babyhearing.org

70 National EHDI Technical Assistance System (continued) EHDI Network members located in each of the MCHB regions Information dissemination and training Web site (www.infanthearing.org)www.infanthearing.org Collaboration with other groups and agencies

71 National EHDI Technical Assistance System (continued) Groups actively promoting and assisting with EHDI activities –AG Bell, NCHH, ASHA, AAA, JCIH, AAP, SKI-HI, ASDC, Boys Town, DSHPSHWA Relevant groups whose main focus has been elsewhere –NEC*TAS, Early Head Start, 0-3, Family Voices, NCCC, AMCHP, AHEC, March of Dimes, MCH Health Policy Center Collaboration with Other Groups and Agencies

72 Collaboration with AAP AAP News article Assisted with booklets for physicians and parents Collaborated on implementation of recently funded EHDI Initiative –Chapter Champions –Speakers Kit –Bulletin Board –Physician Guidelines Analysis of legislation National survey of physicians

73 Take Home Messages Deceptively simplethe devil is in the details EHDI is more than screening Medical Home is where the action is Thoughtful, ongoing, self appraisal Youre 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

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