universal newborn hearing screening what it is and how it happens karl white, ph.d. national center...

Universal Newborn Hearing ScreeningWhat It Is and How It Happens

Karl White, Ph.D.National Center for Hearing Assessment and Management

Utah State University

www.infanthearing.org

Early Hearing Detection and Interveniton (EHDI) Programs

Hospitals with Universal Newborn Hearing Screening Programs

1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001

100.0%

Percentage of Newborns Screened for Hearing Prior to Hosptial Discharge

100.0%

Percentage of Newborns Screened for Hearing in the United States

(Dec 2001)

.Percentage of Births

Screened

21 - 50%1 - 20%

51 - 90%

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

National Universal Newborn Hearing Screening Programs

• Austria

• United Kingdom

• Poland

• Flemish Belgium

• Singapore

• Canada (Ontario)

Improved ScreeningTechniques/Equipment

Why is Implementation of Newborn Hearing Screening Accelerating?

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

Improved ScreeningTechniques/Equipment

Acceptance byPolicy Makers

Why is Implementation of Newborn Hearing Screening Accelerating?

Increased Number ofSuccessful Programs

PublicAwareness/Demand

Why is Early Identification of Hearing Loss so Important?

• Hearing loss occurs more frequently than any other birth defect.

Rate Per 1,000 of Permanent Childhood Hearing Loss in UNHS

ProgramsSample Prevalence

Site Size Per 1000

Rhode Island (3/93 - 6/94) 16,395 1.71

Colorado (1/92 - 12/96) 41,976 2.56

New York (1/95 - 12/97) 69,761 1.95

Texas (1/94 - 6/97) 52,508 2.15

Hawaii (1/96 - 12/96) 9,605 4.15

New Jersey (1/93 - 12/95) 15,749 3.30

Incidence per 10,000 of Congenital Defects/Diseases

6 52 1

Hearing Loss

Cleft lip or palate

Down Syndrome

Limb defects

Spina bifida

Sickle Cell Anemia

• Hearing occurs more frequently than any other birth defect.

• Undetected hearing loss has serious negative consequences.

Reading Comprehension Scores of Hearing and Deaf Students

8 9 10 11 12 13 14 15 16 17 18

Hearing

Age in Years

Schildroth, A. N., & Karchmer, M. A. (1986). Deaf children in America, San Diego: College Hill Press.

Effects of Unilateral Hearing Loss

MathLanguage

Social

MathLanguage

Social

0th 10th 20th 30th 40th 50th 60th

Percentile Rank

Normal Hearing Unilateral 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 ResultsMath = 30th percentile

Language = 25th percentileSocial = 32nd percentile

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 .62Metropolitan Achievement Test

Math .48Reading .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.

• 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.

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.

13-18 mos(n = 15/8)

19-24 mos(n = 12/16)

25-30 mos(n = 11/20)

31-36 mos(n = 8/19)

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

0.8 1.2 1.8 2.2 2.8 3.2 3.8 4.2 4.80

Identified <6 mos (n = 25)

Identified >6 mos (n = 104)

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.

Good work,but I think we mightneed just a little more detail righthere.

Implementing Effective EHDI Programs

Then amiracleoccurs

• 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?

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!!

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

Typical UNHS Screening Protocols(example for 1,000 newborns)

Hearing Loss=3

Normal Hearing=37

Diagnosisn=40

InpatientScreening

Fail=40

Pass=9601 S

Hearing Loss=3Normal Hearing=7

InpatientScreening Pass=920

Fail=80 OutpatientScreening

Diagnosisn=10

Pass=70

Fail=10

Diagnosisn=20

InpatientScreening

Pass=980

Fail=20 Hearing Loss=3

Normal Hearing=17

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

Purposes of an EHDI Data System

Screening

Research

Diagnosis InterventionMedical, Audiological and

Educational

Program Improvementand Quality Assurance

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) 16,395 1.71 42%

Colorado (1/92 - 12/96) 41,976 2.56 48%

New York (1/96 - 12/96) 27,938 1.65 67%

Utah (7/93 - 12/94) 4,012 2.99 73%

Hawaii (1/96 - 12/96) 9,605 4.15 98%

Tracking "Refers" is a Major Challenge(continued)

Initial Rescreen Births Screened Refer Rescreen Refer

Rhode Island 53,121 52,659 5,397 4,575 677 (1/93 - 12/96) (99%) (10%) (85%) (1.3%)

Hawaii 10,584 9,605 1,204 991 121(1/96 - 12/96) (91%) (12%) (82%) (1.3%)

New York 28,951 27,938 1,953 1,040 245 (1/96-12/96) (96.5%) (7%) (53%) (0.8%)

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

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

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

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

• Appropriate and Timely Diagnosis of the Hearing Loss

State Coordinator’s Ratings of Obstacles to

Effective EHDI Programs Serious or Extremely Serious Obstacle

Shortage of qualified pediatric audiologists 49%

Physicians don’t know enough about newbornhearing screening, diagnosis, and intervention 41%

Unwillingness of third-party payersto reimburse for hearing screening 28%

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

Average Age in Months

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)*

0 10 20 30 40 50 60 70

Confirmation of Permanent Hearing Loss

• Prompt Enrollment in Appropriate Early Intervention

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

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)

Hawai'i EHDI ProgressAge of Identification and Intervention

Data from Hawai’I Zero to Three Project

pre 1992 1993 1994 1995 1996 1997 1998

Identification Intervention

• A Medical Home for all Newborns

What Is a Medical Home?

• A primary care physician provides care which is:

• Accessible

• Family-centered

• Comprehensive

• Continuous

• Coordinated

• Compassionate

• Culturally effective

EHDI and the Medical Home

Parent Groups

Mental Health

Birthing Hospital

Audiology

Primary Provider

Child/Family

GeneticsEarly

Intervention Programs

3rd Party Payers

Deaf Community

Services for Hearing Loss

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

Environmental

Genetic

CMV meningitisrubella prematurityhead trauma asphyxiationototoxicity hyperbilirubinother infections Syndromic

Alport NorriePendred Usher

WaardenburgBranchio-oto-renal

Jervell and Lange-Nielsen

Non-syndromic

Autosomal dominant

Autosomal recessive

X-Linked

Mitochondrial

Congenital Hearing Loss

What Causes Hearing Loss?

Common Forms of Syndromic Hearing Loss

Syndrome Main Features (in addition to hearing loss)

Alport Kidney problems

Branchio-oto-renal Neck cysts and kidney problems

Jervell and Lange-Nielsen Heart problems

Neurofibromatosis Type 2 Nerve tumors near the ear

Pendred Thyroid enlargement

Stickler Unusual facial features, eye problems, arthritis

Usher Progressive blindness

Waardenburg Skin pigment changes

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

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

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.

• A Medical Home for all Newborns

• Culturally Competent Family Support

Emotions of Families with a Deaf orHard 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

Communication Choices

• American Sign Language

• Total Communication

• Auditory Verbal

• Auditory-Oral

• Cued Speech

Information Wanted vs. Received by Parents at Hearing Loss ConfirmationInformation Wanted vs. Received by Parents

Martin, George, O'Neal, & Daly (1987); *Sweetow & Barrager (1980)

Degree of loss

Auditory system

Amplification

Educational options

Speech/Lang dev

Etiology

Home activities

*Written Information

*Financial Support

*Emotional Support

*Parent Contacts

*Referral Sources

0 20 40 60 80 100

Wanted

Received

Parent’s 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 baby’s hearing 11%

Confused about the results of screening tests 10%

Glad hearing screening is done at this hospital 91%

Confident the hearing tests were correct 91%

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 88%

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 Agree or Agree

total group subgroupWorried about my baby’s hearing 11% 24%

Confused about the results of screening tests 10% 24%

Glad hearing screening is done at this hospital 91% 70%

Confident the hearing tests were correct 91% 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 88% 56%

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 Loss 41 states

Guidelines for Audiologic Diagnostic Evaluations 30 states

List of Qualified Pediatric Audiologists 39 states

Brochure about Genetics of Hearing Loss 7 states

Fair or Excellent Availability of Materials in other Languages 34 states

Efforts by the Federal Government toPromote 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

National EHDI Technical Assistance System

• EHDI Network members located in each of ten geographic regions

Region I(38% currently bornin UNHS hospitals)

Antonia MaxonB

Region IV(46% currently bornin UNHS hospitals)

Faye McCollister

Region III(49% currently bornin UNHS hospitals)

Sean Kastetter

Region VI(38% currently born in UNHS hospitals)

Karen DittyPatti Martin

Region VII(33% currently bornin UNHS hospitals)

Les Schmeltz

Region IX(23% currently bornin UNHS hospitals)

Randi WinstonYusnita Weirather

Region II(16% currently bornin UNHS hospitals)

Beth Prieve

Region V(26% currently bornin UNHS hospitals)

Karen Munoz

Region X(21% currently bornin UNHS hospitals)

Curt Whitcomb

Region VIII(91% currently bornin UNHS hospitals)

Terry Foust

National EHDI Assistance Network

Guam, American Samoa,Marshall Islands, Palau,

No. Mariana Islands,Fed. Micronesia

Puerto RicoVirgin Islands

= indicates the locations of MCHB Regional Offices

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

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

National EHDI Technical Assistance System (continued)

• EHDI Network members located in each of the MCHB regions

• Information dissemination and training

Support for Program Implementation

• Implementation Guide

• Booklets for AAP and March of Dimes

• Materials posted at www.infanthearing.org

• Video tape for parents

• Evaluation instruments and procedures

Sound Ideas Newsletter

• Topical articles, suggestions for program improvement

• Upcoming events

• Available online or mailed

• Web site (www.infanthearing.org)

www.infanthearing.org

www.babyhearing.org

• Web site (www.infanthearing.org)

• Collaboration with other groups and agencies

• 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

Collaboration with AAP• AAP News article

• Assisted with booklets for physicians and parents

• Collaborated on implementation of recently funded EHDI Initiative

– Chapter Champions

– Speaker’s Kit

– Bulletin Board

– Physician Guidelines

• Analysis of legislation

• National survey of physicians

“Take Home” Messages

• Deceptively simple—the devil is in the details

• EHDI is more than screening

• Medical Home is where the action is

• Thoughtful, ongoing, self appraisal

• You’re not alone

“I am a great believer in luck, and I find that the harder I work, the more I have of it.” ---Thomas Jefferson

universal newborn hearing screening what it is and how it happens karl white, ph.d. national center...

hearing assessment

percentile slide

deaf slide

undetected hearing loss

early hearing detection

percentile language

language competency

percentile social

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