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

Objective: 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 unaided and aided parental observations was combined. Infants with a greater proportion of CAEPs present had higher PEACH scores.
Conclusions: Delaying amplification until VRA results were available would have led to a significant period of auditory deprivation for infants in this study group. None of the assessments could accurately determine hearing thresholds when used in isolation, however when used in combination clinicians were able to obtain sufficient information to fit hearing aids early, and identify infants requiring closer monitoring.