Bilateral Evoked Otoacoustic Emissions in a Child with Bilateral Profound Hearing Loss

Abstract

Recording of bilateral evoked otoacoustic emissions in a 3-year-old girl with bilateral profound hearing loss is presented. No component of auditory-evoked brain stem potentials was recorded. Findings on computed tomography of the inner ear were within normal limits. At retest 3 months later, these results were unchanged. Explanations for this particular condition are presented together with a review of the literature.

An Objective Method of Analyzing Cochlear Versus Noncochlear Patterns of Distortion‐Product Otoacoustic Emissions in Patients With Acoustic Neuromas

“…Clearly, noncochlear patterns of DPOAEs are not specific for acoustic neuromas or, for that matter, any IAC or CPA tumors, since a variety of pathological processes has been shown to result in OAEs suggestive of a peripheral auditory disconnection syndrome. 20–31,52,53 In fact, OAEs may be normal in the presence of severe behavioral thresholds in cases of nonorganic, or functional, hearing loss. 54 OAEs seem to be specific to outer hair cell function, suggesting that some noncochlear patterns may be obtained from ears with hearing loss‐related inner hair cell loss or, perhaps, even dysfunction in other cochlear structures.…”

“…Although there have been studies of both TEOAEs 32–42 and (to lesser extent) DPOAEs 43–45 in patients with acoustic neuromas, the comparisons between OAEs and behavioral thresholds have been subjective and nonuniform. Furthermore, although the authors of these investigations concluded that OAEs were not very sensitive, the results of these studies, and of the central auditory pathology case reports, 20–31 suggested that OAEs had a high specificity as a screening test for retrocochlear disease, when the emissions were better than expected for the degree of behavioral hearing loss.…”

“…A number of case reports involving patients with a variety of central auditory pathological processes have documented the presence of OAEs when the extent of behavioral threshold elevation would have suggested their absence. 19–30 Moreover, OAEs have been shown to be relatively resistant to destructive manipulations of the cochlear nerve and the central auditory structures, as long as those maneuvers do not affect the cochlea itself and its blood supply. For example, in animal experiments the eighth cranial nerve has been severed, with preservation of the internal auditory artery (IAA), which resulted in the loss of auditory brainstem responses (ABRs) without changes in DPOAE levels.…”

Profound Hearing Loss Attributable to Cochlear Nerve Disease: Diagnosis With Combination of Otoacoustic Emission and Magnetic Resonance Imaging

“…As for the findings of DPOAEs in patients with profound hearing loss, it has been reported that normal DPOAEs can be observed in deaf patients without any detectable wave in ABR 16‐18 . In those cases, the disorder could be at the level of the inner hair cells (IHCs), synapses between IHCs and the eighth auditory nerve fibers, or any combination thereof.…”

“…As for the findings of DPOAEk in patients with profound hearing loss, it has been reported that normal DPOAEs can be observed in deaf patients without any detectable wave in ABR. [16][17][18] In those cases, the disorder could be at the level of the inner hair cells (IHCs), synapses between IHCs and the eighth auditory nerve fibers, or any combination thereof. Based on the present study, in which a particular case showed significant levels of DPOAEs but no apparent auditory nerves in the 3DFT-CISS images, it should be considered that a majority of the cochlear nerve fibers may not be present in such cases of profound deafness with significant OAEs.…”