The management of pediatric hearing loss caused by auditory neuropathy spectrum disorder

Purpose of review
Auditory neuropathy spectrum disorder (ANSD) is a condition in which auditory testing reveals normal
otoacoustic emissions, but auditory brainstem testing is abnormal or absent and speech discrimination is
poor. This constellation of findings ostensibly suggests that the cochlea is healthy and an abnormality of
conduction or processing of sound occurs along the nerve fibers. As more is learned about this condition, it
is becoming clear that ANSD describes heterogeneous, distinct clinical entities that must be taken into
account when devising treatment modalities.
Recent findings
Modern auditory testing, genetic testing, and neuroimaging can allow for an accurate understanding of the
location of the lesion causing ANSD in the auditory pathway. Contributing causes can include genetic
mutations, absent or deficient cochlear nerve, hypoxia and jaundice among others. Hearing aids can be
successful in the management of ANSD. Several studies suggest that cochlear implantation can lead to
successful hearing outcomes in a subset of this patient population.
Summary
Auditory neuropathy spectrum disorder represents a relatively rare but important diagnosis for clinicians.
Treatment for this condition includes hearing aids and FM systems in more mild cases, and cochlear
implants in severe cases. Cochlear implantation for many patients can lead to a good hearing outcomes
but the outcome can vary greatly depending on the underlying etiology of ANSD.
Keywords
auditory nerve dys-synchrony, auditory neuropathy spectrum disorder, cochlear implantation, sensorineural
hearing loss