Improvement in Performance of the Central Auditory System in Type 1 Neurofibromatosis

Abstract

Background:The aim was to electrophysiologically evaluate changes in the peripheral and central auditory systems in individuals with neurofibromatosis type 1 who underwent formal auditory training. Material and methods:The sample consisted of 6 individuals aged between 9 and 15 years. The procedures involved anamnesis, meatoscopy, brainstem auditory potentials and long-latency evoked potentials, and behavioral tests of central auditory processing. All individuals were given 9 weekly sessions of formal auditory training.Results: After therapeutic intervention we observed improved performance in auditory closure (p = 0.014) and figure-background discrimination for verbal sounds (p = 0.025). There were no significant changes in brainstem auditory evoked potentials. However, the P300 of the longlatency auditory evoked potential showed significant differences for the left ear in terms of latencies of waves N2 (p = 0.05) and P3 (p = 0.05) and the amplitude of N2-P3 (p = 0.05). Conclusions: Therapeutic intervention by means of formal auditory training is effective in rehabilitating central auditory processing disorder.There were improved responses in both behavioral and electrophysiological assessments, which were maintained for at least 4 months.