Diagnosing Auditory Neuropathy
Diagnosis is possible after a test battery, that must necessarily include the following: the auditory brainstem response and otoacoustic emissions. Auditory brainstem response should be tested with both polarities (helps in identifying Cochlear Microphonics).
Auditory neuropathy is diagnosed when a person has present Otoacoustic Emissions and/or Cochlear Microphonics in combination with absent or abnormal Auditory Brainstem Response. Patients with Auditory Neuropathy Spectrum Disorders have to date never been shown to have normal middle ear muscle reflexes at 95 decibel dB or less despite having normal otoacoustic emissions.
Auditory Neuropathy can occur spontaneously, or in combination with diseases like Charcot-Marie-Tooth disease and Friedreich's ataxia.
It appears that regardless of the audiometric pattern(hearing thresholds) or of their function on traditional speech testing in quiet the vast majority of sufferers have very poor hearing in background noise situations.
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