The Colorado newborn hearing screening project, 1992-1999: on the threshold of effective population-based universal newborn hearing screening.

OBJECTIVE Although previous studies have documented the feasibility and benefits of universal newborn hearing screening in selected hospitals, none have reviewed the effectiveness of regionally mandated participation of large numbers of hospitals with variable levels of motivation to succeed. The purpose of this study was to measure hospital participation and overall screening success in a statewide program for universal newborn hearing screening and to track improvements in program establishment and outpatient follow-up over time. METHODS Four Colorado hospitals began voluntarily performing hearing screening before hospital discharge on all newborns in 1992. By 1996, 26 Colorado hospitals were participating in universal newborn hearing screening. The publication of screening results from these early years served as a catalyst for legislation requiring increased hospital participation in establishing universal screening programs. Data systems were subsequently developed to improve statistical tracking and follow-up. Eight years' worth of cumulative study data as well as the results from calendar year 1999 (the year of greatest hospital participation) were reviewed for collective measures of successful screening and follow-up. Three hospitals did not initiate newborn hearing screening programs until after the study period ended in 1999. Of the 57 hospitals that were screening newborns in 1999, the chosen method of screening at 52 hospitals was automated auditory brainstem response testing; 3 hospitals used otoacoustic emission testing, and the remaining 2 hospitals used 2-stage screening. Hearing loss was defined as a threshold of 35 decibels or greater in 1 or both ears at the time of confirmatory testing. RESULTS During the full 8-year study period, 1992 to 1999, 148 240 newborns were screened. A total of 291 infants who were born during the study period received a diagnosis of congenital hearing loss. In this cohort of 291 children, the cumulative frequency of bilateral hearing loss was 71% (range: 48%-94% by calendar year), the frequency of sensorineural hearing loss was 82% (range: 67%-88%), and the frequency of 1 or more risk factors was 47% (range: 37%-61%). During calendar year 1999, a total of 63 590 births were recorded at 60 birthing hospitals in Colorado. The families of 263 (0.4%) of these newborns refused newborn hearing screening. Of the remaining 63 327 newborns, 87% (55 324 infants) were screened for hearing acuity before hospital discharge, a far greater percentage than the 19% of all newborns screened during the first 5 years of voluntary hospital participation, and approaching the American Academy of Pediatrics's recommendation of 95% of newborns completing hospital-based testing in a successful screening program. As a result of this statewide hearing screening program, congenital hearing loss was diagnosed in 86 Colorado newborns during 1999, representing an occurrence rate of approximately 1 affected child in every 650 newborns. In this group of 86 infants, 59 had bilateral sensorineural hearing loss, 17 had unilateral sensorineural hearing loss, 4 had bilateral conductive hearing loss, and 6 had unilateral conductive hearing loss. Mild hearing loss was present in 6 infants, moderate hearing loss was present in 42 infants, severe hearing loss was present in 33 infants, and profound hearing loss was present in the remaining 5 infants. Only 32 of the 86 affected newborns in 1999 had 1 or more risk factors for hearing loss subsequently identified. After failing an initial hospital-based screening at 1 of the 57 participating hospitals in 1999, 2.3% of infants screened (1283 newborns) were referred for follow-up testing, easily exceeding the standard of <4% recommended by the American Academy of Pediatrics. Similarly, the false-positive rate of 2.2% during 1999 exceeded the recommended standard of <3%. Of the infants who failed their initial screening, 76% (978 infants) had documented follow-up testing to confirm or exclude congenital hearing loss, a percentage significantly improved from a follow-up rate of 48% during the first 5 years of screening, although not yet achieving the standard of 95% recommended by the American Academy of Pediatrics. Nine participating hospitals, however, were able to document appropriate follow-up for 95% or more of the infants who failed their initial screening tests. The median age of diagnosis of congenital hearing loss during 1999 was 2.1 months; 71% of affected infants were identified by 3 months of age (the recommended standard for age of diagnosis), and 92% of affected newborns were identified by 5 months of age. Measures of screening success were compared for large, mid-sized, and small hospitals. Increasing hospital size, as measured by the number of births per year, was associated with an increasing percentage of newborns who were successfully screened. It was notable that smaller hospital size was associated with increased referral rates for follow-up testing, whereas larger hospital size was associated with the highest recapture rate for follow-up testing. CONCLUSIONS Universal screening for congenital hearing loss is demonstrated to be feasible in a large regional effort of legislatively mandated participation. The success of such an endeavor is dependent on educational efforts for community professionals, commitment on the part of program planners, and data systems that more accurately track and recall infants who fail initial hospital-based screening.