Article révisé par les pairs
Résumé : Despite the availability of published recommendations for the management of Auditory Neuropathy/Auditory Dyssynchrony, numerous questions remain unanswered and different opinions can still be found on the subject Moreover, the entity is characterized by an important inter- and even intra- individual variability. This makes prognosis particularly difficult The experience accumulated in our rehabilitation center comes from eleven children diagnosed at an early, pre-lingual stage and having auditory deficits ranging from moderate-severe to profound hearing loss. They have been followed-up for at least six years. The results indicate that a major determinant of the level of language development is the presence or absence of associated handicap(s), particulany neurological deficits, but also psycho-social problems and simultaneous exposure to two or more languages. Only four out of the eleven children reached a level of language development appropriate for their ages (Linguistic Quotient around 100%). None of them had an associated handicap whereas the seven others, each of them being afflicted with at least one additional handicap, achieved Linguistic Quotient values between 36 and 67 %. No correlation between the results and the level of auditory deficit or the type of prosthetic aid (amplification or cochlear implantation) was found. We still have a lot to learn about the numerous factors affecting the linguistic development of children affected with Auditory Neuropathy/Auditory Dyssynchrony. A comprehensive, international data bank as projected in chapter XVII should be of a great help in this regard.

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