Imaging Characteristics of Children With Auditory Neuropathy Spectrum Disorder

Objective: To identify and define the imaging characteristics of children with auditory neuropathy spectrum disorder (ANSD). Design: Retrospective medical records review and analysis of both temporal bone computed tomographic (CT) and magnetic resonance images (MRI) in children with a diagnosis of ANSD. Setting: Tertiary referral center. Patients: One hundred eighteen children with the electrophysiologic characteristics of ANSD with available imaging studies for review. Interventions: Two neuroradiologists and a neurotologist reviewed each study, and consensus descriptions were established. Main Outcome Measures: The type and number of imaging findings were tabulated. Results: Sixty-eight (64%) MRIs revealed at least 1 imaging abnormality, whereas selective use of CT identified 23 (55%) with anomalies. The most prevalent MRI findings included cochlear nerve deficiency (n = 51; 28% of 183 nerves), brain abnormalities (n = 42; 40% of 106 brains), and prominent temporal horns (n = 33, 16% of 212 temporal lobes). The most prevalent CT finding from selective use of CT was cochlear dysplasia (n = 13; 31%). Conclusion: Magnetic resonance imaging will identify many abnormalities in children with ANSD that are not readily discernable on CT. Specifically, both developmental and acquired abnormalities of the brain, posterior cranial fossa, and cochlear nerves are not uncommonly seen in this patient population. Inner ear anomalies are well delineated using either imaging modality. Because many of the central nervous system findings identified in this study using MRI can alter the treatment and prognosis for these children, we think that MRI should be the initial imaging study of choice for children with ANSD.