Prevalence, behavioural, and management outcomes of infants with auditory neuropathy spectrum disorder
1Nottingham University Hospitals NHS Trust, Nottingham, UK.
Aim:
To provide an overview of electrophysiological and behavioural outcomes from a large UK centres data set on children diagnosed with auditory neuropathy (ANSD) between 2002 and March 2018.
Method:
A systematic audit was undertaken, collating the electrophysiological data from auditory brainstem response (ABR) follow-up, risk factors, and later behavioural results/management. These were then compared to look for trends between groups. The study sample consisted of 118 925 infants born, with 46 (0.039%, 0.39 per 1000 births) being diagnosed with congenital ANSD (39 bilateral, seven unilateral).
Results:
Twenty-nine per cent of ears with ANSD had short latency components on ABR testing. Forty-four per cent of ears with present cochlear microphonics but absent transient-evoked otoacoustic emissions (TE-OAE) and no ABR went on to have profound behavioural hearing threshold levels. All but one child went on to show a hearing loss on behavioural testing. ANSD was not confined to the population from neonatal intensive care units: there were three bilateral and five unilateral cases in the typically developing infant population.
Interpretation:
The incidence of ANSD is higher in this sample than that reported previously in the literature. Children who had cochlear microphonics with absent ABR and absent TE-OAE had significantly worse later behavioural outcomes than other patterns of electrophysiological results.
