CLINICAL CASE 3 OF 8

Absent ABR with present OAEs in a toddler

Vignette

An 18-month-old referred for delayed speech has present OAEs in both ears, but the click ABR is absent/grossly abnormal with preserved cochlear microphonic — a picture of auditory neuropathy spectrum disorder. The family is anxious about whether the child will ever use spoken language. You record cortical responses to speech sounds.

Findings

0100200300400latency (ms)Cortical response
Despite the absent ABR, a cortical response is present — reduced and slightly delayed, but repeatable.

Diagnostic question

How should the present cortical response be interpreted in this ANSD picture?

References