EHDI: An Amazing Journey

Transcription

1 EHDI: An Amazing Journey Jackson Roush, PhD Professor & Director Division of Speech and Hearing Sciences Director, North Carolina LEND Program Carolina Institute for Developmental Disabilities UNC School of Medicine Hear n Now Conference 2016

2 For every 100,000 births 2 adrenal hypoplasia (X-linked genetic disorder characterized by adrenal insufficiency) 3 PKU (autosomal recessive metabolic disease due to absence of a single enzyme) 28 hypothyroid 47 sickle cell disease 314 with permanent hearing loss of some degree in one or both ears

3 Number per 10,000 Prevalence Rate in Comparison to Other Congenital Conditions (Per 10,000) Hearing loss Cleft lip or palate Down syndrome Limb defects Spina bifida Sickle cell anemia PKU Congenital Condition Type NCHAM;

4 Behavioral Observation Screening ( s)

5 Newborn hearing screening based on behavioral observation (early 1970 s) Dr. Marion Downs

6 High Risk Register The High Risk Register was developed to identify babies with increased likelihood of hearing loss, based on a review of the case history and hospital records (JCIH, 1972).

7 The Crib-o-gram (late 1970 s to early 1980 s, better control of stimulus and response but lacked the specificity needed for efficient screening) Pat Roush calibrating the Crib-o-gram at Denver Children s Hospital in preparation for screening a high-risk infant

8 Auditory Brainstem Response (ABR) Screening Physiologic (not behavioral) screening method ABR was the first screening method to combine high sensitivity and high specificity Early instruments were large and difficult to move around; note supra-aural earphone.

9 NIH Consensus Statement March, 1993 Infants w/ HL should be identified and treated by 6 months of age Universal screening for HL should be performed prior to 3 months of age, preferably before hospital discharge (with the aim of identifying all congenital hearing loss)

10 Bess and Paradise Commentary Universal Screening for Infant Hearing Impairment: Not Simple, Not Risk -Free, Not Necessarily Beneficial, and Not Presently Justified (Pediatrics 98:2, , 1994) Highly controversial; triggered a debate that lasted over two years

11 Northern and Hayes Rebuttal Universal Screening for Infant Hearing Impairment: Necessary, Beneficial, and Justifiable (Audiology Today, May, 1994). Dozens of articles and letters to the editor followed...

12 Joint Committee on Infant Hearing (1994) Endorsed the goal of universal infant hearing screening But more data needed

13 American Academy of Pediatrics: Task Force on Newborn and Infant Hearing Pediatrics 103 (2): , 1999 Endorsed the implementation of universal newborn hearing screening Recommended guidelines for screening, follow-up, and evaluation

14 Paradise (1999) was still unconvinced: Limited evidence supporting the benefits of early identification Predictive value of currently available screening methods is too low Mandated universal hearing screening is premature Efforts should be focused on high risk infants

15 JCIH Year 2000 Position Statement All infants should have access to hearing screening within the first month, using a physiologic screening measure (NICU: before discharge) Infants with permanent HL should be diagnosed by age 3 months Intervention should be provided by 6 months of age

16 US Preventive Services Task Force Statement (2001) USPSTF is an independent panel of experts in primary care and prevention that systematically reviews the evidence of effectiveness and develops recommendations for clinical services. The Task Force concluded that the hypothesis that early intervention is a predictor of language acquisition is plausible, but the current studies do not establish that screening low-risk newborns is the important factor. JAMA, 286 (16), 2001

17 JCIH Year 2007 Position Statement Expanded definitions and inclusion criteria Recognized the growing body of scientific evidence to support early identification Reaffirmed 1-3-6

18 US Preventive Services Task Force Statement (July, 2008) Recommended: The USPSTF recommends that clinicians provide newborn hearing screening to eligible patients. The USPSTF found at least fair evidence that [the service] improves important health outcomes and concludes that benefits outweigh harms. uspstf/gradespre.htm#brec

19 What s the current status? NHS is now considered a standard of care Performed in all 50 states and US Territories Over 40 states have legislative mandates Many challenges remain but we are no longer debating whether NHS is necessary and appropriate

20 K. White, 2012

21 Image: marchofdimes.org

22 Dr. Bruce Weber with Pat Roush conducting a diagnostic ABR on an infant at Duke Hospital, 1989

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24 Images: Boys Town National Research Hospital, University of South Carolina, Baltimore Sun

25 1 3 6 Images: Boys Town National Research Hospital. UNC Hospitals, University of South Carolina

26 EHDI: An Amazing Journey It s good to have an end to journey toward; but it s the journey that matters, in the end. - Ernest Hemingway

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