Abstract
We can now distinguish, in part, between nerve deafness and hair cell deafness through the use of otoacoustic emissions. We can also assess the efferent system by carefully quantifying the effects of contralateral stimulation on these same otoacoustic emissions. The suppression of transient evoked emissions by continuous contralateral white noise is an ostensibly small effect of 2 or 3 dB when studied over a 20-msec window. However, when subjected to microstructural analysis, the effect can exceed 6 to 8 dB in the zones from 10 to 20 msec after the stimulus has subsided. Temporal and spectral analyses reveal robust effects of contralateral lateral stimulation, although in any given normal subject it may be difficult to separate middle ear effects from efferent effects. Evidence is strong that the efferent effect is mediated in part by cholinergic — primarily nicotinic — receptors in the outer hair cell. However, a unique type of patient, who shows nearly normal pure-tone audiograms and absent ABRs, shows virtually no contralateral suppression of transient evoked emissions. Some other patients, with symptoms of Charcot-Marie-Tooth disease, may paradoxically show extremely poor audiograms, but perfectly normal evoked emissions along with absent contralateral suppression. The ABR, along with middle ear muscle reflexes and masking level differences, are all absent in these patients; we therefore think they have a disorder that desynchronizes most of their primary auditory nerve fibers and thereby disconnects them from any efferent activity or masking cancellation. The existence of such an auditory disorder, characterized by severe dysfunction in speech comprehension — especially when listening in noise—suggests that what appears to be a “central auditory imperception” might stem instead from a systemic peripheral primary neuropathy.
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