The prevalence of hearing loss in newborns and infants is estimated between 1 to 3.47 cases per 1000 live births. Early diagnosis and rehabilitation of congenital hearing loss are mandatory in order to achieve a satisfactory linguistic and cognitive development. Without appropriate opportunities to learn language, these children will fall behind their normal hearing peers in communication, cognition, reading and socio-emotional development. After promising results, neonatal screening for hearing loss and audiological evaluation are becoming more extensively carried out. In planning universal neonatal hearing screening programs, transient evoked otoacoustic emissions and auditory brainstem responses are the gold standard for the screening and diagnosis program. However, there is no consensus regarding the use of audiometry and other electrophysiological tests (such as auditory steady-state responses) in current practices. Several screening and audiological assessment procedures have been described and advocated all around the world. But, a systematic scheme of performing diagnosis in the pediatric audiology population is lacking. A consensus conference was held at the International Federation of Oto-rhino-laryngological Societies Congress, in June 2017, to discuss the different current practices and to identify the best neonatal hearing screening and audiological assessment management. This article is intended to provide professionals with recommendations about the “best practice” based on consensus opinion of the session's speakers, and a review of the literature on the efficacy of various assessment options for children with hearing loss.