To determine what factors are associated with newborn hearing loss. We retrospectively reviewed hospital records of all neonates found on screening to have hearing loss as well as a cohort with normal audiometric findings from January 1998 through December 2000. The two groups were compared for various maternal, obstetric, and perinatal variables. Sixty-four neonates had hearing loss. The controls consisted of 270 neonates with normal findings. There were no differences between groups when compared for various maternal and perinatal variables. The mean gestational age (35.9 ± 5.9 weeks versus 37.8 ± 3.1 weeks, P = .01) and birth weight (2698 ± 1173 g versus 3195 ± 816 g, P = .002) were significantly lower in the hearing-loss group. Only 32 of the 64 neonates (50%) had one or more of the ten clinical neonatal high-risk factors for newborn hearing loss. On multivariable analysis, very low birth weight of 1500 g or less (odds ratio [OR] 7; 95% confidence interval [CI] 3, 18; P = .001), 5-minute Apgar scores less than 7 (OR 5; 95% CI 2, 18; P = .009), positive family history of congenital deafness (OR 7; 95% CI 2, 197; P = .02), and structural and chromosomal anomalies (OR 64; 95% CI 14, 292; P = .001) were independently associated with the development of newborn hearing loss. Congenital structural and chromosomal anomalies appear to be the most significantly associated risk factors for the development of newborn hearing loss. Very low birth weight, low Apgar scores, and family history are also independently associated with newborn hearing loss. However, most infants with hearing loss have no clinical risk factors.