Prevalence and risk factors for auditory neuropathy spectrum disorder in a screened newborn population at risk for hearing loss.

Objective: To determine the prevalence of Auditory Neuropathy Spectrum Disorder (ANSD) among infants with sensorineural hearing loss (SNHL) and discuss the risk factors.

Methods: The study group was comprised of children diagnosed with ANSD. Criteria for inclusion in the study group were as follows: permanent sensorineural hearing loss (SNHL); normal transient evoked or distortion product OAEs; absent or elevated middle-ear muscle reflexes (MEMR); severely abnormal or absent ABR waveform. All newborns were screened via a two-stage protocol, involving OAEs and ABR for the repeatedly referred cases and all neonates with one or more risk factors.

Results: From 2002 to 2011, 9419 infants whose hearing ability was uncertain or who had risk factors for hearing loss were investigated. From this population, 352 were diagnosed with SNHL. Of these 352 children, 18 (5.1%) were diagnosed with ANSD. In the ANSD group, prematurity and low birth weight (<1500 g) were observed in 5 cases; ototoxical medication in 8 cases; mechanical ventilation in excess of 5 days in 5 cases. Hyperbilirubinemia was observed in 7 cases, but severe hyperbilirubinemia requiring an exchange transfusion was not observed; 4 patients had no risk factors, 6 patients had only one risk factor, and the 8 remaining had two or more risk factors.

Conclusion: Auditory Neuropathy Spectrum Disorder is not an extremely rare hearing disorder. Screening for hearing loss via OAEs, MEMR and ABR examinations concurrently are essential for the accurate diagnosis of ANSD.