The audiological journey and early outcomes of twelve infants with auditory neuropathy spectrum disorder from birth to two years of age

AbstractObjective: To describe the audiological journey of a group of infants with auditory neuropathy spectrum disorder (ANSD) following the fitting of hearing aids, and to investigate the potential benefits of including cortical auditory-evoked potentials (CAEPs) and a measure of functional auditory behaviour during early audiological management. Design: Results from chart revision of estimated hearing threshold, early behavioural testing, parental observation, and functional auditory behaviour assessments were described, and compared to visual reinforcement audiometry (VRA) thresholds obtained at a mean corrected age of 10 months (SD 3). The relationship with CAEPs and functional performance was examined. Study sample: The study included 12 infants diagnosed with ANSD and fitted with amplification. Results: The estimated 4FA at a mean corrected age of four months (SD5) was within ± 10 dB of VRA results in 75% of infants when unaided and aided behavioural observation audiometry (BOA), together with unai...