In this issue of The Journal, Holster et al from Sophia Children's Hospital Erasmus Medical Center in Rotterdam report results of a retrospective cohort study over almost a decade of neonatal hearing screening tests, and follow-up of infants with positive tests with formal auditory brainstem response (ABR) testing, physical examination, and tympanometry. During the study period, the method of screening was changed from a behavioral observation test to otoacoustic emission (OAE) testing for healthy infants, and to automated ABR (AABR) test for infants in the neonatal intensive care unit (NICU). These screening tests are in line with recommendations of the American Academy of Pediatrics.
The first remarkable strength of the study is that more than 98% of infants born in the Netherlands had screening performed. And in Rotterdam, more than 98% of infants referred because of failed screening tests actually were seen and evaluated. Authors ascribe adherence with follow-up to frequent communications between screeners from well-baby clinics and audiologists, which included monthly discussion of all referred patients at joint meetings.
The investigation showed that OAE and AABR testing had remarkably higher positive predictive value than the 2% for behavioral observation screening. The current program also led to intervention at a younger age than previously (8 months vs. 15-18 months). Using current screening methods, 58% of infants who failed screening tests had sensoneural hearing loss (SNHL) and 20% had permanent conductive hearing loss. Not surprisingly, positive predictive value of screening for SNHL was higher for failing NICU screening (71%) than failing well-baby clinic screening (54%).
Although genetic (syndromic and nonsyndromic) etiologies were pursued—and found—in some infants, intrauterine infections were pursued only for clinically apparent reasons. With these limitations, just over one-half of infants with hearing loss had etiology determined, which was predominantly genetic.
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