A neonate fails universal newborn hearing screening on both sides. Auditory brainstem response (ABR) at one month shows absent wave I at 95 dB nHL bilaterally but present cortical auditory evoked potentials (CAEPs). The most likely diagnosis is:
- A Auditory neuropathy spectrum disorder (ANSD) ✓
- B Bilateral profound sensorineural hearing loss — cochlear origin
- C Bilateral otitis media with effusion causing conductive loss
- D Central auditory processing disorder
Explanation
Auditory neuropathy spectrum disorder (ANSD) is characterised by absent or abnormal ABR (specifically wave I from the auditory nerve) with preserved or present otoacoustic emissions (OAEs) and/or present cochlear microphonics, indicating normal outer hair cell function. The dissociation — abnormal ABR but present cortical responses — confirms disruption at the level of the auditory nerve/inner hair cell synapse, the hallmark of ANSD. This diagnosis has profound implications for management, as cochlear implants can bypass the disordered nerve synchrony and provide excellent outcomes.
Reference: Dhingra Diseases of Ear, Nose and Throat, 7th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.