Genomics and Hearing Loss: Toward a New Standard of Care?

Abstract

The presence of outer hair cell (OHC) function is one of the main characteristics of Auditory Neuropathy/Dys-Synchrony (AN/AD) and represents one of the salient features used in its diagnosis. OHC function can be measured with otoacoustic emission (OAE) testing or by the recording of the cochlear microphonic. OHC function seems to remain intact over years in most patients with AN/AD, however, a significant number of patients have abnormal OAEs, while others will loose them over time. Hence, patients with AN/AD can present with various degrees of OHC function which can be misleading in the course of their diagnosis or management There is no clear relationship between OAE presence or absence and hearing abilities in individuals with AN/AD. Since the natural cochlear amplifier functions normally in the majority of patients, management with hearing aids remains controversial. In addition, hearing aids have not yet proven to be useful for speech and language development even in cases in which the OAEs are absent or abnormal. Disappearance of OAEs can be an epiphenomenon (hearing aid use or ototoxic factors for example) but seems to be linked directly to the progression of AN/AD in specific cases.

Publication Title

Cahiers De L Audition

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