Interpret a referred newborn hearing screen — OSCE

Candidate brief

You have this station to interpret a set of paediatric hearing-test results and to counsel the parents of a 5-week-old infant referred on the newborn hearing screen. Read the four tests together — the audiogram, the tympanogram, the otoacoustic emissions and the auditory brainstem response — identify the pattern they produce, explain the diagnosis and the next steps in plain language, and demonstrate that you understand why an OAE-only screen can miss the diagnosis and what the 1-3-6 timeline means for this family. [11] [2]

Key teaching and management objectives

Begin by reading the results together. The otoacoustic emissions are robustly present at all frequencies on both ears, which confirms that the cochlear outer hair cells are functioning. The tympanograms are normal type A on both ears, which excludes a middle-ear effusion as the cause of an absent emission — important, because a conductive loss can dampen OAE. The right-ear auditory brainstem response shows no reproducible wave V even at high stimulus levels, while the cochlear microphonic is preserved. The pattern of preserved OAE with an absent ABR and a preserved cochlear microphonic is the signature of auditory neuropathy spectrum disorder. [11] [1]

Explain to the parents in plain language that the cochlea — the sensory part of the ear — is working (which is why the emissions are present), but that the nerve pathway that carries the signal from the cochlea to the brain is not conducting a synchronous signal on the right (which is why the brainstem response is absent). Make clear that this is not a simple hearing loss that an OAE-only screen would have caught — the emissions were preserved, so the outer hair cells looked normal — and that the two-stage screen with automated ABR is what allowed the diagnosis to be made. [11] [2]

State the operational plan. The child is referred to a multidisciplinary team led by paediatric audiology and ENT. The management is individualised: the team weighs hearing aids calibrated to the ABR thresholds, FM or remote-microphone systems to improve the signal-to-noise ratio, and, where speech perception remains poor despite a trial of amplification, cochlear implantation, which can be highly effective when the lesion is at the synapse. Set the timeline within the 1-3-6 milestones: the screen was done, the diagnosis is being made at five weeks (within the three-month target), and intervention will be in place by six months. Enrol the family in early intervention services and give them a named contact. [11] [2]

Close with honest counselling and a safety-net. The trajectory and the benefit from each intervention in auditory neuropathy are individualised, so the family should expect close monitoring rather than a single fixed plan; the hearing will be tracked with behavioural and electrophysiological testing over the first years. Confirm the left ear is functioning normally and explain how the team will support binaural development. Invite questions and check the parents' understanding. [11] [1]

Marking domains

• Interpretation of the four tests together (4 marks). Identifies the pattern of preserved OAE, normal tympanogram, absent ABR with preserved cochlear microphonic as auditory neuropathy spectrum disorder; explains why the cochlear microphonic and the preserved OAE localise the lesion to the nerve or synapse rather than the hair cells.
• Understanding of screening limitations (2 marks). Explains that an OAE-only screen would have reported a pass because the outer hair cells are intact, and that the two-stage protocol with automated ABR is what detected the condition.
• Operational plan and timeline (3 marks). Names the multidisciplinary team (paediatric audiology, ENT, early intervention); states the 1-3-6 milestones; describes the individualised role of hearing aids, FM systems and cochlear implantation.
• Counselling and safety-net (2 marks). Communicates the diagnosis in plain, honest language without catastrophising; confirms the normal-functioning left ear; checks understanding and offers a named contact and follow-up. [11] [2]