Abstract

The combined findings of "normal evoked otoacoustic emissions (EOAE) and absent or markedly disturbed auditory evoked potentials from the brainstem and/or cortex" has been named auditory neuropathy in the English literature. The pure tone thresholds in these patients range from mild to severe loss. The loss of speech comprehension is out of proportion in comparison to the pure tone threshold elevation. This combination of findings suggests that cochlear function and in particular outer hair cell function, is normal in these patients but also suggests that the inner hair cell / VIII nerve functional unit is abnormal. Disruption in neural synchrony has previously been postulated as a reasonable explanation for the absent auditory brainstem response while otoacoustic emissions are preserved. Because the long-latency auditory evoked potentials, not having the stringent synchrony requirements of the ABR, may also be missing, the term synchrony - disruption may be somewhat misleading. The literature about auditory neuropathy is reviewed here: We now know that a set of salient features distinguishes these patients from the majority of patients with sensorineural hearing loss or other described syndromes. The symptoms defining auditory neuropathy are mild, moderate or severe elevation of auditory thresholds to pure tone stimuli by air and bone conduction, absent to severely abnormal ABRs to high level stimuli, present otoacoustic emissions (that do not suppress with contralateral noise), word recognition ability poorer than expected from the pure tone hearing loss configuration (in cases with mild to moderate hearing threshold elevation) and absent acoustic reflexes to both ipsilateral and contralateral tones. The entire set of findings, consistently seen in these patients, supports the importance of the crosscheck principle in diagnostic evaluation especially in children with delayed and/or impaired language acquisition. It is of ponderous importance that this disorder is not being confused with sensory hearing loss.