Clinical Case · clinician

A child who passed newborn screening now struggling in noise

History

A 6-year-old has marked difficulty understanding speech in noise despite only a mild audiometric loss. Otoacoustic emissions are robustly present bilaterally. The auditory brainstem response is grossly abnormal. Tympanometry is type A.

Findings

Pure-tone audiogram

0204060801002505001k2k4k8kFrequency (Hz)Hearing level (dB HL)
○ Right ear✕ Left ear

Acoustic reflex grid

Stim. Right
Stim. Left
Probe Right
Absent
Absent
Probe Left
Absent
Absent

Reflexes are absent in all four conditions. With the other findings, this is most consistent with: