Confirmation of deafness in infancy

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Abstract
AIM To assess delay in confirming hearing impairment in infants identified by universal neonatal screening and to investigate the causes. PATIENTS Infants identified from 25 199 babies screened from January 1992 to December 1997. METHODS A two stage transient evoked oto-acoustic emission test (TEOAE), with a threshold auditory brainstem response (ABR) recording undertaken on those who failed. The screen identified infants with a permanent congenital hearing impairment (PCHI) averaging 40 dBnHL or worse in the best ear. Those with less impairment were also ascertained. The positive predictive value (PPV) of the ABR test and measures of delay between identification and eventual diagnosis were analysed. RESULTS A targeted PCHI was found in 1.18/1000 neonates. The PPV of the ABR for confirming a targeted PCHI was 100% when the ABR threshold was ⩾ 80 dBnHL. Nine of 11 infants with this threshold had severe or profound permanent deafness. The delay from ABR to audiological certainty was about 1 month—diagnosis was confirmed around 3 months. There was uncertainty when the ABR was 40–80 dBnHL. The PPV was 60% and 8% when the ABR thresholds were 70 dBnHL and 50 dBnHL, respectively. 85 of 111 infants with ABR thresholds in this range had a temporary conductive impairment. Their early diagnosis depended upon the type and degree of hearing impairment and diagnosis was delayed to about 8 months in these infants. CONCLUSIONS Hearing impairments identified by universal screening are delayed in all but those with severe or profound bilateral PCHI. This delay can be reduced by applying in early infancy a battery of audiological tests and requires further exploration. Useful implementation of universal neonatal hearing screens depends on the ability to confirm both the type and degree of deafness in early infancy Screening may be relatively straightforward but audiological diagnosis at this age is not Severe or profound deafness can be identified in early infancy with minimal delay but moderate deafness is confirmed on average at 8 months Targeting moderate deafness in early infancy demands that audiological tests are developed to confirm a permanent hearing impairment in those identified by the screen