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Auditory neuropathy (ANSD): test profile, types, screening and management
In auditory neuropathy the outer hair cells function (OAE or CM present) but neural transmission is not synchronised (ABR absent or grossly abnormal). The result: hearing that ranges from normal to profound, and speech understanding far worse than the audiogram predicts.
Why it matters in the exam
The test profile the bank builds on: OAE and/or CM present; ABR absent or grossly abnormal; acoustic reflexes absent; poor word recognition, especially in noise; thresholds that may change from day to day. Risk factors: NICU stay, severe jaundice (hyperbilirubinaemia), prematurity, lack of oxygen and mutations such as OTOF. The presynaptic type affects the inner hair cell and its synapse (for example OTOF), and cochlear-implant results are usually good; the postsynaptic type affects the nerve itself (for example an absent or thin cochlear nerve), where implant benefit is limited — so imaging is needed.
In newborn screening, OAE alone misses ANSD — a false negative — so under JCIH NICU infants are screened with AABR. In management the ABR does not estimate threshold, so the plan rests on behavioural thresholds (in an infant — from a developmental age that allows VRA) and functional monitoring of language and speech. Start a hearing-aid trial when behavioural thresholds justify it, add a remote microphone and visual support, and consider a cochlear implant when progress is inadequate. In some infants, especially after jaundice, things improve with maturation over the first two years. The difference from APD: in APD the ABR is normal.
Key points
- OAE or CM present, ABR absent, reflexes absent.
- Speech understanding worse than expected, especially in noise.
- Risk: NICU, jaundice, prematurity, lack of oxygen, OTOF.
- AABR screening in the NICU; OAE alone misses it.
- Manage by behavioural thresholds and function; implant by progress and lesion type.
Summary table
| Area | Finding or principle | Meaning |
|---|---|---|
| OAE and CM | Present | The outer cells work |
| ABR | Absent or grossly abnormal | The nerve is not synchronised; no threshold estimate |
| Reflexes and recognition | Reflexes absent; word recognition worse than expected | Marked difficulty in noise |
| Risk factors | NICU, severe jaundice, prematurity, lack of oxygen, OTOF | AABR screening in the NICU |
| Presynaptic | Inner hair cell and synapse (OTOF) | Good results with an implant |
| Postsynaptic | Auditory nerve (absent or thin cochlear nerve) | Limited implant benefit; imaging is a must |
| Management | Behavioural thresholds and functional monitoring; aid trial; implant by progress | The ABR does not set gain |
A free exam question
An infant admitted to the NICU with severe jaundice has hearing tests. OAEs are normal and the cochlear microphonic (CM) is present, but the ABR is absent. What is the most likely diagnosis?
- A. Conductive hearing loss
- B. Cochlear (outer hair cell) hearing loss
- C. Auditory neuropathy spectrum disorder (ANSD)
- D. Normal hearing
In the full version for subscribers
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Sources
- Guidelines Development Conference on the Identification and Management of Infants with Auditory Neuropathy, 2008 (Como) — Consensus guidelines
- Starr, Picton, Sininger, Hood & Berlin, Brain, 1996 — Auditory neuropathy
- Joint Committee on Infant Hearing, Journal of Early Hearing Detection and Intervention, 2019 — Year 2019 Position Statement
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.