Auditory neuropathy spectrum disorder: predictive value of radiologic studies and electrophysiologic tests on cochlear implant outcomes and its radiologic classification

Abstract

Conclusion: The width of the bony cochlear nerve canal (BCNC) and the size of the cochlear nerve are reliable predictors of long-term speech perception abilities for children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI). In addition, electrical stapedial reflex (ESR) and electrical compound action potential (ECAP) also have considerable value in predicting postoperative speech perception abilities in these children. Objectives: To assess whether speech perception abilities after CI in children with ANSD can be predicted from the results of radiologic studies and electrophysiologic tests. Methods: Fifteen children with ANSD underwent CI. The width of the BCNC and the size of the cochlear nerve were measured using preoperative CT and MRI. The results of early postoperative ESR, ECAP, and implant evoked electrical auditory brainstem response were reviewed. The latest speech perception test scores were also reviewed. Results: Radiologic findings of normal BCNC and normal cochlear nerve correlated with excellent speech perception abilities after CI. A narrow or obliterated BCNC and a deficient cochlear nerve correlated with poor speech perception abilities. Children with good speech perception abilities showed robust responses on ESR and ECAP, but there were no responses from any of the children with poor speech perception abilities.