FRCS (ORL-HNS) · SECTION 1 SBA

Auditory neuropathy pattern

An adult reports disproportionate difficulty understanding speech in noise. Pure-tone thresholds are mildly impaired, otoacoustic emissions are present, but auditory brainstem responses are absent and acoustic reflexes are abnormal. What diagnosis best explains this pattern?

CHOOSE ONE ANSWER

Best of five

  1. A. Otosclerosis
  2. B. Auditory neuropathy spectrum disorder
  3. C. Ménière's disease
  4. D. Superior semicircular canal dehiscence
  5. E. Presbycusis alone
ANSWER AND REASONING

B. Auditory neuropathy spectrum disorder

Preserved outer-hair-cell activity with disordered neural synchrony and disproportionately poor speech perception is characteristic of auditory neuropathy spectrum disorder. Management requires specialist audiology and may include remote-microphone systems, hearing aids or cochlear implantation according to functional benefit.

Why every option is right or wrong

A. Otosclerosis: Otosclerosis produces a conductive or mixed pattern and does not characteristically preserve emissions with absent neural responses.

B. Auditory neuropathy spectrum disorder: Preserved outer-hair-cell activity with disordered neural synchrony and disproportionately poor speech perception is characteristic of auditory neuropathy spectrum disorder. Management requires specialist audiology and may include remote-microphone systems, hearing aids or cochlear implantation according to functional benefit.

C. Ménière's disease: Ménière's causes fluctuating cochlear loss with episodic vertigo rather than this electrophysiological dissociation.

D. Superior semicircular canal dehiscence: A third-window disorder produces sound/pressure symptoms and characteristic conductive physiology, not absent ABR with preserved emissions.

E. Presbycusis alone: Age-related cochlear loss does not account for the defining neural-synchrony pattern.

What if the scenario changed?

If otoacoustic emissions were absent with a conventional sloping cochlear loss and proportionate speech scores, ordinary sensorineural hearing loss would be more likely.

EDUCATIONAL USE

Independent Clinora educational preparation material; not official JCIE content, an accredited programme, clinical advice, or a substitute for local policy and specialist judgement.

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