Prevalence, risk factors, and audiological characteristics of auditory neuropathy

The objective of this study was to determine the prevalence, risk factors, and audiological characteristics of auditory neuropathy spectrum disorder (ANSD) in the pediatric population.
A retrospective review of medical charts was conducted for children visiting two hospitals in Saudi Arabia.
Medical records of 1025 patients with sensorineural hearing loss (SNHL) were reviewed. We analyzed the databases for results of audiological examinations, risk factors, and outcomes of intervention including hearing aid (HA) and cochlear implantation (CI).
Out of 1025 children with SNHL, 101 patients (9.85%) were identified to have ANSD. Audiological characteristics of the ANSD group revealed a severe-to-profound degree of hearing loss, all showed type A tympanogram and absent reflexes, absent auditory brainstem response (ABR) findings with present cochlear microphonic while otoacoustic emissions were absent in 54.5% of patients. The most prevalent risk factors for ANSD in this group were family history of hearing loss, consanguinity, hyperbilirubinemia, and low birth weight. Pure tone and speech detection thresholds improved significantly with CI compared to HA use in this sample of patients with ANSD.
This study shows that ANSD is not extremely rare among Saudi children with severe to profound hearing loss, with a prevalence of 9.85%.