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

Number of Hospitals Doing Universal Newborn Hearing Screening Number of Programs

Percentage of Births Screened for Hearing Before Discharge Number of Programs

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

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

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

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

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

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

Effects of Unilateral Hearing Loss Normal Hearing Unilateral Hearing Loss Keller & Bundy (1980) Math (n = 26; age = 12 yrs) Language Peterson (1981) Math (n = 48; age = 7.5 yrs) Language Bess & Thorpe (1984) Social (n = 50; age = 10 yrs) Math Blair, Peterson & Viehweg (1985) (n = 16; age = 7.5 yrs) Language Math Culbertson & Gilbert (1986) (n = 50; age = 10 yrs) Language Social Average Results 0th 10th 20th 30th 40th 50th 60th Math = 30th percentile Percentile Rank Language = 25th percentile Social = 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 .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.

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.

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

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

Is the Glass Half Empty or 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

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=7 Inpatient Screening Pass=920 Fail=80 Outpatient n=80 Diagnosis n=10 Pass=70 Fail=10 2 Stage OAE Hearing Loss=3 Normal Hearing=37 Diagnosis n=40 Inpatient Screening Fail=40 Pass=960 1 Stage AABR Diagnosis n=20 Inpatient Screening Pass=980 Fail=20 Hearing Loss=3 Normal Hearing=17 OAE / AABR 1 Stage

Protocols Used in Universal Newborn Hearing Screening Programs Screening Procedures Percent of newborns screened Before Hospital Discharge After Hospital Discharge 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% 6.4% OAE/ABR OAE/ABR 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 Research Program Improvement and Quality Assurance Screening Diagnosis Intervention Medical, Audiological and Educational

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 Number of states Pediatric Audiolgists per 10,000 Births per year

Confirmation of Permanent Hearing Loss 35 Coplan (1987) 19 Eissman et al. (1987) 30 Gustason (1987) 30 Meadow-Orlans (1987) 24 Yoshinago-Itano (1995) 25 Stein et al. (1990) 31 Mace et al. (1991) 56 O'Neil (1996) 3 Johnson et al. (1997)* 3 Vohr et al. (1998)* 10 20 30 40 50 60 70 Average Age in Months

Hawai'i EHDI Progress Age of Identification and Intervention 60 50 40 Age in Months 30 20 10 pre 1992 1993 1994 1995 1996 1997 1998 Year Data from Hawai’I Zero to Three Project

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

State Coordinator’s Ratings of Obstacles to Effective EHDI Programs Serious or Extremely Serious Obstacle Unwillingness of third-party payers to reimburse for hearing screening 28% Physicians don’t know enough about Hearing 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 Confirmation 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 20 40 60 80 100 Wanted Received