Audiologic profile of infants at risk: Experience of a Western Sicily tertiary care centre

Objective: To identify the incidence of sensorineural hearing loss (SNHL) on infant at risk and to classify the degree and type of hearing loss describing the main causes associated in Western Sicily. To compare single TEOAE and combined TEOAE/ABR techniques studying the referral rate, the false-positive and false-negative rates through concordance test (κ coefficient), sensitivity (TPR) and specificity (TNR) for each protocol. Methods: From January 2010 to June 2011, 412 infants at risk, ranging from 4 to 20 weeks of life, transferred to Audiology Department of Palermo from the births centers of Western Sicily, underwent to audiological assessment with TEOAE, tympanometry and ABR. The following risk factors were studied: family history of SNHL, consanguinity, low birth weight, prematurity, cranio-facial abnormality and syndromes associated to SNHL, respiratory distress, intensive care in excess of 5 days (NICU), pregnant maternal diseases, perinatal sepsis or meningitis, hyperbilirubinemia, ototoxic drugs administration. Results: Forty-seven infants (11.41%) were diagnosed with SNHL; median corrected age at final audiological diagnosis was 12 weeks. SNHL resulted moderate in 44.68%, severe in 10.64% and profound in 21 cases with a significant difference in family history and NICU infants (p < 0.0001). As the number of coexisting risk factors increases, the percentage value of SNHL in infants (χ 2 = 12.31, p = 0.01, r 2 = 0.98) and the degree of hearing loss (χ 2 = 13.40, p = 0.0095, r = 0.92) also increase. The study of single TEOAE and combined TEOAE/ABR showed a statistical difference (χ 2 = 14.89, p < 0.001) with a low concordance value (κ = 0.87) confirming the importance of combined techniques for NICU group (κ = 0.86) where four cases (0.97%) of auditory neuropathy were diagnosed. Conclusion: This study demonstrates the necessity to implement a neonatal hearing screening program in Western Sicily because of the high percentage of SNHL in infants at risk. Family history of HL is an independent significant risk factor for SNHL easily diagnosed through single TEOAE technique. Combined TEOAE/ABR is the gold standard for NICU babies which are at risk for auditory neuropathy. Coexisting risk factors are an additional risk factor for HL.