DISEASE PAGE

Auditory Neuropathy Spectrum Disorder

The signature dissociation — present OAE with an abnormal or absent ABR.

The OAE signature

  • Emissions are present and frequently of normal or even high amplitude — despite abnormal behavioural thresholds.[3]
  • The defining picture: a normal OAE alongside an absent or grossly abnormal auditory brainstem response.[2]
0 ms20 ms
— Normal ear— Auditory Neuropathy Spectrum Disorder
Simulated TEOAE comparison. Educational signal model — not recorded patient data.

Audiogram companion

The pure-tone audiogram below accompanies the OAE signature. Reading the two together — what the threshold shows and what the emission shows — is the core diagnostic skill.

0204060801002505001k2k4k8kFrequency (Hz)
○ Right — PTA 43 dB (Moderate)✕ Left — PTA 47 dB (Moderate)
Pure-tone audiogram companion. dB HL increases downward, following clinical convention. Illustrative thresholds — not recorded patient data.

Why the emission looks this way

  • The OAE probes only the pre-neural cochlea. In ANSD the outer hair cells are intact, so emissions persist; the lesion lies in inner hair cell, synaptic, or neural transmission, which the OAE cannot see.[2]

TEACHING POINT

A present OAE never excludes serious hearing loss. ANSD is the reason newborn screening pairs OAE with automated ABR — an OAE-only protocol would miss it entirely.[3]

Sources for this page are listed on the References page. Browse all condition patterns from the atlas home.