A former preterm infant who required prolonged ventilation and exchange transfusion for hyperbilirubinaemia fails newborn hearing screening bilaterally. At follow up, otoacoustic emissions are present bilaterally but the auditory brainstem response is absent. What is the most likely diagnosis?

• A Auditory neuropathy spectrum disorder ✓
• B Bilateral severe cochlear hair cell loss
• C Bilateral auditory nerve aplasia
• D Congenital cholesteatoma

Explanation

Auditory neuropathy spectrum disorder shows preserved outer hair cell function, hence recordable otoacoustic emissions, with disordered neural conduction shown by absent or severely abnormal ABR. Risk factors include prematurity, hyperbilirubinaemia requiring exchange transfusion, hypoxia, and intensive care admission. In true sensory hair cell loss the otoacoustic emissions would be absent, which distinguishes it from the key. Management includes visual communication support and consideration of cochlear implantation.

Reference: Cummings Otolaryngology: Head and Neck Surgery, 7th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.