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Objectives: (1) Determine perinatal medical factors associated with auditory neuropathy spectrum disorder (ANSD). (2) Compare risk factors associated with ANSD to those in sensorineural hearing loss (SNHL).Methods: Retrospective case-control study of children diagnosed with ANSD at an academic tertiary care center between July 2009 and January 2014. Inclusion criteria for the ANSD group were normal otoacoustic emissions, absent or elevated middle-ear muscle reflexes, and severely abnormal or absent auditory brainstem response waveforms. The ANSD group was compared with 2 age-and sex-matched control groups:(1) normal-hearing children and (2) children with SNHL.Results: Twenty-two patients (15 male, 7 female) met ANSD inclusion criteria, and were matched to 2 control groups as described above. Case and control groups were assessed for the following perinatal risk factors: prematurity, low birth weight, hyperbilirubinemia, mechanical ventilation, and administration of ototoxic antibiotics. On multivariate logistic regression between ANSD and normal-hearing groups, hyperbilirubinemia (P = .006) and administration of ototoxic antibiotics (P = .004) were significantly associated with an increased odds ratio (OR) toward developing ANSD. Comparing ANSD and SNHL groups, the only significant risk factor for development of ANSD was hyperbilirubinemia (P = .005, odds ratio = 12).Conclusions: ANSD, a clinical spectrum of disease united by normal cochlear function but abnormal auditory pathway transduction, remains an enigmatic entity. In the present study, matched comparisons between ANSD versus normal-hearing and SNHL control groups identified perinatal hyperbilirubinemia and ototoxic antibiotics as significant risk factors. Diligent hearing evaluation to diagnose ANSD in infancy, particularly among patients with a perinatal history of hyperbilirubinemia, is critical.

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