Evaluation of saccular and inferior vestibular nerve function in children with auditory neuropathy spectrum disorder

The aim of the current work was to evaluate the function of the saccule and inferior vestibular nerve in children with auditory neuropathy spectrum disorder (ANSD) by recording the cervical-evoked myogenic potentials (C-VEMP) on those children and to compare C-VEMP results in ANSD children of pre-lingual onset to those in ANSD children of post-lingual onset.

The study included 38 ANSD children of pre-lingual onset, 16 ANSD children of post-lingual onset, and 20 control children. All participant children were subjected to C-VEMP testing using 500 Hz tone burst stimuli.

The vast majority of ANSD children of pre-lingual onset (35 out of 38; 92.1%) had bilateral intact C-VEMP response with C-VEMP parameters (amplitude, asymmetric ratio, latency, and inter-aural latency difference) that were not statistically different than those in the control children. Only three children had bilateral absent C-VEMP response. On the other hand, the majority of ANSD children of post-lingual onset (11 out of 16; 68.75%) had bilateral absent C-VEMP response. The remaining five children had bilateral intact C-VEMP response with C-VEMP parameters that were not statistically different than those in the control children.

The pathology of ANSD spares the saccule and inferior vestibular nerve in the vast majority of ANSD children of pre-lingual onset, while it involves them in the majority of ANSD of post-lingual onset reflecting different site(s) of lesion between the two ANSD categories. Such results have important clinical implications as regards to the management of ANSD in children.