A retrospective analysis of the audiological surveillance activities was performed for the period from January, 2013 to December, 2016. Well babies and neonatal intensive care unit (NICU) babies who received bilateral pass result during the newborn hearing screening and were neonatal intensive care unit one or more risk factor, were referred for targeted surveillance. The mean (SD) age of babies at the time of screening was 4 (2,1) weeks. The data of the present study were drawn from a database which included individual records for each child.

Since 2003, well-babies in Campania region are screened via two stage transient otoacoustic emission (TEOAE): The first in the course of the second or third day of life and the second between 3-4 weeks of age, if a refer result is obtained. TEOAE and automated auditory brainstem response (A-ABR) are reserved for infants under intensive care, prior to discharge. Infants who fail both screenings, either bilaterally or unilaterally, are referred to the nearest pediatric audiology service to perform a comprehensive audiology evaluation. In case of hearing impairment identification, a third level multidisciplinary diagnostic work-up together with appropriate management is provided by the regional reference center (RRC), at the audiology and vestibology unit of the neuroscience department of the university of Naples ‘Federico II’ [4]. Since 2013, this unit is also in charge of coordination of the audiological surveillance program according to the Position committee on infant hearing screening, 2007.

Children referred for surveillance appointment are accepted according to the following protocol: Audio-logical assessment in the third level center, every 6 months up to the age of 3 years and then annually up to the age of 6 years in the presence of risk factors such as cytomegalovirus (CMV) and rubella intrauterine infections; every 6 months up to the age of 3 years, in children affected by syndromes associated with progressive or late-onset hearing loss e.g., Pendred syndrome, distal tubular renal acidosis, Waardenburg syndrome (type II), branchio-otorenal syndrome, Usher syndrome (type II and III), Stickler syndrome, CHARGE syndrome, Down syndrome, Turner syndrome, Alport syndrome, neurodegenerative disorders such as Hunter syndrome, sensory-motor neuropathies such as Friedreich ataxia, Charcot-Marie-Tooth syndrome); every 6 months up to the age of 2 years in case of family history of progressive permanent infant hearing loss, severe asphyxia; at 9-12 months in the case of prolonged ventilation for more than 5 days, craniofacial anomalies including cleft palate and, audiological evaluation at the immediate third level center in every phase of childhood or adolescence in case of chemotherapy, trauma, culture positive infections associated with sensorineural hearing loss, ototoxic drugs, reports from family pediatricians or other health workers and educators, or meningitis.

The audiological evaluation is done as per the age group of children. Test battery is click-auditory brainstem responses (ABR), transient evoked oto-acoustic emissions (TEOE), tympanometry at 3-9 months; TEOE, tympanometry, visual reinforcement audiometry at 9-12 months; and visual reinforcement audiometry, conditio-ned play audiometry and tympanometry at 3.5 years.

Degrees of hearing loss is based on the Bureau International for Audiophonology (BIAP) [5] classi-fication viz normal (< 20 dB HL), mild (21-40 dB HL), moderate (41-70 dB HL), severe (71-90 dB HL) and profound (> 90 dB HL).

All families of children confirmed with hearing loss were offered a genetic evaluation and counselling. The evaluation, included a review of family history of specific genetic disorders or syndromes, genetic testing for gene mutations such as GJB2, GJB6 (connexin-26 and 30), and syndromes commonly associated with early-onset hearing loss.

Statistical analyses: Descriptive statistics were used, and Pearson chi-squared analyses were performed in order to identify variables of significance.

The flow of all children referred to the targeted surveillance program is shown in Fig. 1. A total of 165416 children were eligible for UNHS (158386 ‘well babies’ and 7030 ‘high risk’), of which 2752 (1.6%) children had a ‘refer’ result and underwent a comprehensive audiology evaluation. Another 2340 children had a ‘pass’ result, but with at least one risk factor and were referred for audiological surveillance program. Thus the recorded rate was 1.41% (2340/165416) in the period under investigation. With regards to individual risk factors, the largest proportion of referrals were generated from family history (35%), low birthweight (19.2%), prematurity (25.5%), severe hyperbilirubinemia (19%), prolonged ventilation (15%) and congenital infections (12.5%) (Table I).