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Background: The World Health Organization (WHO) reported in 2013 that approximately 360 million people have disabling hearing loss, of which 32 million are children. A major consequence of hearing loss is a disability in communication: development of spoken language is often delayed in children with hearing loss, or absent in total deafness. Such children can benefit from a range of interventions such as hearing aids, cochlear implants, and educational and social support. Hearing screening can prevent the most severe consequences of hearing loss, but it is preferable first to consider populations with a high probability of developing hearing impairment. The present study assessed the incidence of hearing impairment through ABR in infants at high risk at Casa Sollievo della Sofferenza Hospital in southern Italy. Material and methods: The paper reports the results of auditory brain stem response (ABR) testing over a period of 3 years on infants born with a history of infantile respiratory distress syndrome (IRDS), otoacoustic emission failure at hearing screening, low birth-weight (<1800 g), family history of deafness, hyperbiluribinemia, premature birth, congenital infection (cytomegalovirus), or craniofacial anomalies. Results: Of 549 infants tested, 469 (85.4%) showed normal hearing, 51 (9.3%) suffered mild hearing impairment, 6 (1.1%) had moderate hearing impairment, 8 (1.5%) had severe hearing loss, and 15 (2.7%) failed to show any response at maximum intensity levels in both ears. For the hearing loss categories, the most important risk factors were found to be (in order of importance): craniofacial malformation, CMV infection, and familial factors. Conclusions: ABR is an important clinical tool in the identification and quantification of hearing impairment. In neonatal screening it is preferable first to consider populations with a high probability of developing hearing losses. [ABSTRACT FROM AUTHOR]

Background: Congenital cytomegalovirus (cCMV) infection is the most frequent non-genetic cause of sensorineural hearing-loss (SNHL) (i.e., hearing loss due to a cochlear and/or auditory nerve damage). It is widely accepted that SNHL at birth, when associated to cCMV symptomatic infection involving the central nervous system, benefits from antiviral therapy started in the neonatal period. Conversely, there is no consensus for antiviral treatment in congenitally infected infants diagnosed with isolated SNHL (i.e., SNHL in an otherwise asymptomatic infant) at birth. Our aim was to assess the frequency and the auditory outcome of isolated SNHL at birth due to auditory neuropathy (AN) (i.e., SNHL in a patient with normal cochlear function and auditory nerve dysfunction) in infants with cCMV infection., Methods: We retrospectively reviewed the clinical history of 60 infants, born at term, with cCMV asymptomatic infection, without additional risk factors for SNHL, and exhibiting bilateral "pass" otoacustic emissions (OAE). None of them underwent antiviral therapy. Hearing thresholds were assessed by means of Auditory Brainstem Responses (ABR). AN affected children were followed up until possible normalization of the hearing thresholds or definitive diagnosis of AN. Each infant diagnosed with monolateral or bilateral AN was classified according to the worst ear threshold., Results: In our population, the first ABR was performed at a mean age of 5.00 ± 2.79 (SD) months and AN was diagnosed in 16/60 (26.67%) infants; in 4 infants the AN was defined as mild (4/4 monolateral), moderate in 11 (5/11 bilateral), and severe in 1 (bilateral). The mean age at first ABR was 3.69 ± 2.80 (SD) months in the 16 babies with AN and 5.48 ± 2.66 (SD) months in the 44 infants with normal hearing (p = 0.007). All AN cases spontaneously recovered a normal auditory threshold over time. The mean length of the audiological follow-up was 32.44 ± 17.58 (SD) months (range 5-60 months)., Conclusion: A delayed maturation of the auditory pathways should be considered when a mild/moderate isolated AN at birth is detected in cCMV infected infants. Prospective studies conducted on larger populations, and with a longer audiological follow-up, are needed to confirm our findings.