Perceptual characterization of children with auditory neuropathy

Objective: To characterize the perceptual abilities of a group of children with auditory neuropathy (AN)-type hearing loss, correlating results on a range of psychophysical tasks with open-set speech perception performance. Design: Frequency resolution, temporal resolution and frequency discrimination ability was assessed in a group of 14 children with AN. Data also were obtained from a cohort of matched subjects with sensorineural hearing loss, and from a group of normally hearing children. Results: Frequency resolution (notched noise masking) results for the AN subjects were equivalent to those of the normal-hearing subjects reflecting the "normal" outer hair cell function that characterizes the AN condition. Temporal resolution (TMTF) findings were, however, abnormal in many AN subjects and the degree of temporal disruption was correlated with speech discrimination (CNC) score. Frequency discrimination ability (for both fixed and frequency modulated stimuli) was also affected in those children with poor temporal resolution. Conclusions: The findings of this study indicate that the perceptual profiles of children with AN are quite different from those with sensorineural hearing loss. Where subjects in the latter group presented with impaired frequency resolution and normal temporal processing, the AN subjects typically showed normal frequency resolution and varying degrees of temporal disruption. The severity of this temporal abnormality, which appeared to affect both temporal resolution/amplitude modulation detection and the temporal aspects of frequency discrimination (such as phase locking), was strongly correlated to speech perception performance.