Auditory neuropathy spectrum disorder: Examples of poor progress following cochlear implantation

Abstract The majority of babies with auditory neuropathy spectrum disorder (ANSD) spend 48 h or more in the Special Care Baby Unit (SCBU), and the current UK recommendations for the Newborn Hearing Screening Programme state that babies admitted to the SCBU should undergo hearing screening with transient evoked otoacoustic emissions together with automated auditory brainstem response audiometry, in order to identify babies affected with the condition. Current recommendations propose that individuals affected with ANSD should be considered candidates for cochlear implantation. It has been suggested that in patients with ANSD, the presence or absence of cortical electric response audiometry (CERA) responses can help to predict favourable or unfavourable prognosis in auditory language development and comprehension post implantation. We describe two individuals who had not been in SCBU, but came from the well baby population yet satisfied the diagnostic criteria for ANSD. These two patients underwent cochlear implantation, but made poor progress in auditory language development and speech understanding on subsequent follow-up. We suggest that ANSD should be considered in all cases of infants who undergo cochlear implantation but do not make subsequent good progress in auditory language development and speech understanding. We further suggest that CERA be performed prior to cochlear implantation in patients satisfying the diagnostic criteria for ANSD, as the results can assist in predicting post implantation progress in auditory language development and speech understanding.