The Status of Early Hearing Detection and Intervention in the United States Karen Ditty National Center for Hearing Assessment and Management Utah State.

The Status of Early Hearing Detection and Intervention in the United States

Karen DittyNational Center for Hearing Assessment and Management

Utah State University

www.infanthearing.org

Number of Hospitals Doing Universal Newborn Hearing Screening

3 3 11 26 60 120 243462

712934

1816

2384

0200400600800

1000120014001600180020002200240026002800

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Percentage of Births Screened for Hearing Before Discharge

3

1525

65 67

010

2030

4050

6070

8090

100

Jan

-93

Jul-9

3

Jan

-94

Jul-9

4

Jan

-95

Jul-9

5

Jan

-96

Jul-9

6

Jan

-97

Jul-9

7

Jan

-98

Jul-9

8

Jan

-99

Jul-9

9

Jan

-00

Jul-0

0

Jan

-01

Jul-0

1

Jan

-02

Jul-0

2

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s

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

.

Percentage of Newborns Screened for Hearing Loss in the United States

(Dec 2001)

.Percentage of Births

Screened

90%+

21 - 50%1 - 20%

3

51 - 90%

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

30

12 11

6 52 1

0

10

20

30

40

Hearing Loss

Cleft lip or palate

Down Syndrome

Limb defects

Spina bifida

Sickle Cell Anemia

PKU

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.

Reading Comprehension Scores of Hearing and Deaf Students

1.0

2.0

3.0

4.0

5.0

6.0

7.0

8.0

9.0

10.0

8 9 10 11 12 13 14 15 16 17 18

Deaf

Hearing

Age in Years

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

Gra

de

Eq

uiv

alen

ts

Effects of Unilateral Hearing Loss

MathLanguage

MathLanguage

Social

MathLanguage

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.

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.

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)

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

Lan

gu

age

Ag

e in

Mo

nth

s

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

Implementing Effective EHDI Programs

Then amiracleoccurs

out

Start

• Half full? More than 21/2 million babies are screened every year prior to

discharge

Less than 30 hospitals with UNHS in 1993; compared with more than 2400 today

36 states have passed legislation related to newborn hearing screening

Or half empty? 1,400+ hospitals are not yet screening for hearing loss

More than a 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 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

Status of EHDI Programs in the US:Universal Newborn Hearing Screening

• With 2/3 all babies screened prior to discharge, newborn hearing screening is becoming 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

tag

eA

AB

R

Hearing Loss=3Normal Hearing=7

InpatientScreening Pass=920

Fail=80 OutpatientScreening

n=80

Diagnosisn=10

Pass=70

Fail=10

2 S

tag

eO

AE

Diagnosisn=20

InpatientScreening

Pass=980

Fail=20 Hearing Loss=3

Normal Hearing=17

1 S

tag

eO

AE

/ A

AB

R

Protocols Used in Universal Newborn Hearing Screening Programs

Screening Procedures

Before Hospital Discharge After Hospital Discharge Percent of newborns

screened

OAE ------- 11.6%ABR ------- 23.3%OAE/ABR ------- 6.7%

OAE OAE 21.4%OAE ABR 4.2%ABR OAE 2.8%

ABR ABR 23.2%

OAE/ABR OAE/ABR 6.4%

Other protocol ---------- 0.3%

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 Improvement and 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%)

Tracking and Data Management

• 75% of states report at least some hospitals submit data to state about results of their screening program

• For those getting data, information was submitted for 62% of the births in last quarter of 2001

• 33% of submissions do not include identifying information --- making follow-up by state impossible

• Only 17% of states currently have any kind of linkage with other data systems (eg, Vital Statistics, metabolic, EI, Immunizations)

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

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 56.1% “receive diagnostic evaluations by 3 months of age

Availability of Pediatric Audiolgists

2

13

9

6 65

0

5

10

15

20

0-2.00

2.01 to 4.00

4.01 to 6.00

6.01 to 10.00

10.01 to 14.00

14.01 or more

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Pediatric Audiolgists per 10,000 Births per year

Average Age in Months

3

3

35

19

30

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)*

0 10 20 30 40 50 60 70

Confirmation of Permanent Hearing Loss

Hawai'i EHDI ProgressAge of Identification and Intervention

Data from Hawai’I Zero to Three Project

pre 1992 1993 1994 1995 1996 1997 1998

Year

0

10

20

30

40

50

60

Ag

e in

Mon

ths

Identification Intervention

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

Early Intervention• Part C of IDEA is an under used

resource

• 96% of state coordinators know who the Part C coordinator is

• 74% of states have someone on the IDEA Interagency Coordinating Council with experience / expertise in hearing loss with infants

• State Coordinators estimate:

– 53% of infants with hearing loss are enrolled in EI programs before 6 months of age

– 31% of states have adequate range of choices for type of EI programs

Percentage of State Coordinators Who Rate Early

Intervention Programs in the State as Good or Excellent

For children with:

bilateral severe/profound losses 63%

bilateral mild/moderate losses 56%

unilateral losses 46%

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

What Is a Medical Home?

• A primary care physician provides care which is:

• Accessible

• Family-centered

• Comprehensive

• Continuous

• Coordinated

• Compassionate

• Culturally effective

AAP Task Force on Newborn Infant Hearing

• Endorses implementation of universal newborn hearing screening

• Defines standards for:– Screening– Tracking & Follow-up– Identification & Intervention– Program Evaluation

• Encourages AAP Chapters to provide leadership in developing statewide programs

EHDI and the Medical Home

Parent Groups

Mental Health

Birthing Hospital

Audiology

Primary Provider

Child/Family

ENT

GeneticsEarly

Intervention Programs

3rd Party Payers

Deaf Community

Services for Hearing Loss

State Coordinator’s Ratings of Obstacles to

Effective EHDI Programs Serious or Extremely Serious Obstacle

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

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

Shortage of qualified pediatric audiologists 49%

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

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

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