auditory neuropathy spectrum disorder
A hearing condition in which outer hair cell function is preserved but neural synchrony along the auditory nerve is disrupted, causing speech perception difficulties disproportionate to pure-tone thresholds.
Full Definition
Auditory neuropathy spectrum disorder (ANSD) is characterised by present otoacoustic emissions or cochlear microphonics alongside absent or abnormal auditory brainstem responses, indicating intact outer hair cell function but disrupted auditory nerve or inner hair cell activity. Patients typically experience speech understanding difficulties that are far worse than the degree of hearing loss suggested by their audiogram. The condition can arise from hyperbilirubinemia, prematurity, genetic mutations, or anoxia. Editorial references should use the full term on first use; 'ANSD' is the accepted abbreviation thereafter. Editors must distinguish this from sensorineural hearing loss, with which it is frequently confused in lay copy.
Usage
Usage note: Always expand the full term on first use; abbreviate as ANSD thereafter. Do not conflate with sensorineural hearing loss.
In Context
- "The clinical report confirmed auditory neuropathy spectrum disorder based on present cochlear microphonics and an absent ABR." — Audiological diagnostic report
- "Children diagnosed with ANSD may present with apparently normal hearing on initial screening, making editorial accuracy on this distinction critical." — Patient education brochure