To examine the prevalence of auditory neuropathy in a group of infants at risk for hearing impairment and to present an overview of the clinical findings for affected children. Results for 20 subjects who showed repeatable cochlear microphonic potentials in the absence of click-evoked auditory brain stem responses are included in this study. Behavioral and steady state evoked potential thresholds were established in each case. Where possible, otoacoustic emission and speech perception results (unaided and aided) also were obtained. One in 433 (0.23%) of the children in our series had evidence of auditory neuropathy. The audiometric findings for these subjects varied significantly, with behavioral thresholds ranging from normal to profound levels. Discrimination skills were also variable. Approximately half of the subjects showed little understanding, or even awareness, of speech inputs in both the unaided and aided conditions. There were, however, a number of children who could score at significant levels on speech discrimination tasks and who benefited from the provision of amplification. The results suggest that auditory neuropathy is more common in the infant population than previously suspected. The effects of neuropathy on auditory function appear to be idiosyncratic, producing significant variations in both the detection and discrimination of auditory signals. As such, the management of children with this disorder must allow for individual differences.

Auditory neuropathy/dys-synchrony is a form of hearing impairment in which cochlear outer hair cell function is spared but neural transmission in the auditory pathway is disordered. This condition, or group of conditions with a common physiologic profile, accounts for approximately 7% of permanent childhood hearing loss and a significant (but as yet undetermined) proportion of adult impairment. This paper presents an overview of the mechanisms underlying auditory neuropathy/dys-synchrony-type hearing loss and the clinical profile for affected patients. In particular it examines the perceptual consequences of auditory neuropathy/dys-synchrony, which are quite different from those associated with sensorineural hearing loss, and considers currently available, and future management options.

The effects of inner ear abnormality on audibility have been explored since the early 20th century when sound detection measures were first used to define and quantify ‘hearing loss’. The development in the 1970s of objective measures of cochlear hair cell function (cochlear microphonics, otoacoustic emissions, summating potentials) and auditory nerve/brainstem activity (auditory brainstem responses) have made it possible to distinguish both synaptic and auditory nerve disorders from sensory receptor loss. This distinction is critically important when considering aetiology and management. In this review we address the clinical and pathophysiological features of auditory neuropathy that distinguish site(s) of dysfunction. We describe the diagnostic criteria for: (i) presynaptic disorders affecting inner hair cells and ribbon synapses; (ii) postsynaptic disorders affecting unmyelinated auditory nerve dendrites; (iii) postsynaptic disorders affecting auditory ganglion cells and their myelinated axons and dendrites; and (iv) central neural pathway disorders affecting the auditory brainstem. We review data and principles to identify treatment options for affected patients and explore their benefits as a function of site of lesion.

1) To investigate the unaided and aided speech perception abilities of children with auditory neuropathy (AN) and to compare their performance to children with sensorineural hearing loss. 2) To establish whether cortical event related potentials (ERPs) could be recorded in children with AN, and to determine the relationship between the presence of these responses and speech perception. Unaided and aided speech perception assessments (PBK words), and cortical-ERP testing was carried out in a group of 18 children with AN. Data also were obtained from a cohort of age and hearing level matched children with sensorineural hearing loss. The speech perception performance of the 15 children with AN able to complete a PBK-word assessment, fell into two distinct categories. The children either showed no open-set speech perception ability (7/15 cases), or performance levels similar to their sensorineural counterparts (8/15 cases). Approximately 50% of children with AN showed ERPs of normal latency, amplitude and morphology. In all cases, response presence (at normal latencies) was consistent with reasonable speech perception ability, and response absence was consistent with negligible speech perception. In approximately 50% of children with auditory neuropathy, the provision of amplification results in significant open-set speech perception improvements. The results confirm the previously published reports that speech perception ability cannot be reliably estimated from the behavioral audiogram in children with AN. Obligatory ERP test results may offer a means of predicting perceptual skills in newly diagnosed youngsters as the presence of ERPs (with age-appropriate latency and morphology) was correlated with significant open set speech perception abilities and amplification benefit. The absence of the ERP in contrast, indicated profound hearing disability evidenced by profound hearing loss and/or extremely poor speech perception.

To examine the relationship between auditory steady-state evoked potentials (SSEPs) and behavioral thresholds in sleeping subjects. 60 adults and children with hearing thresholds ranging from normal to profound were selected on the basis of appropriate audiograms. Behavioral audiograms were determined at the octave frequencies 250-4000 Hz. These behavioral thresholds were then compared with the SSEP thresholds obtained during natural sleep for adults, or natural or sedated sleep for children. A strong relationship between behavioral and SSEP thresholds was observed. The strength of the relationship increased with increasing frequency and increasing degree of the loss. On the basis of these data, the prediction of behavioral thresholds from SSEP levels was determined. It was found that the standard deviation of the error in this prediction decreased with increasing frequency and increasing degree of the loss. There was no significant age effect in the results obtained at any of the frequencies. The results suggest that the SSEP technique can be used as a predictor of behavioral thresholds in adults and children at the frequencies 250-4000 Hz.

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.