Auditory Neuropathy Clinical Presentation

Updated: May 15, 2018
  • Author: Wayne T Shaia, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Two thirds of the children with auditory neuropathy/auditory dyssynchrony (AN/AD) demonstrate risk factors associated with perinatal hearing loss (see Pathophysiology). AN/AD should be suspected in any child with slightly-to-severely abnormal hearing thresholds and severe speech and language delay out of proportion with the presumed hearing loss. In addition, adults with mildly-to-moderately abnormal pure tone thresholds and speech discrimination scores out of proportion with suspected hearing loss should undergo additional evaluation by an otologist or neurootologist. This evaluation should include a further audiologic workup with auditory brainstem response (ABR) and otoacoustic emission (OAE) testing to rule out the presence of AN/AD or other retrocochlear processes. [5]

The natural history of AN/AD is varied. Reports have included no change, improvement, and deterioration in children's ability to hear. Patients with AN/AD and associated hyperbilirubinemia have a more profound initial hearing loss but also have a greater tendency for improvement. Children with fever-associated hearing loss consistent with AN have also shown some resilience in their hearing loss. Understand that, although some recovery in the auditory system has been reported, most children affected by AN/AD continue to display abnormal pure tone averages and ABR test results that requires a lifelong commitment by the child, family, speech pathologist, and audiologist.