How is ANSD diagnosed?

Health professionals, including otolaryngologists (ear, nose, and throat doctors), pediatricians, and audiologists, use a combination of methods to diagnose ANSD. These include tests of auditory brainstem response (ABR) and otoacoustic emissions (OAE). The hallmark of auditory neuropathy is a negligible or very abnormal ABR reading together with a normal hair cell function either through an OAE or cochlear microphonic measurement from the ABR.

An ABR measures brain wave activity in response to sound using electrodes that are placed on the person’s head and ears. An OAE test uses a small, very sensitive microphone inserted into the ear canal to monitor the faint sounds produced by the outer hair cells in response to stimulation by a series of clicks. ABR and OAE testing are painless and can be used for newborn babies and infants as well as older children and adults. Other tests may also be used as part of a more comprehensive evaluation of an individual’s hearing and speech-perception abilities.