b) Prelingual profound deafness in children and central plasticity
In cases of prelingual deafness, anatomical and physiological lesions are found all along the hearing pathways though the actual cause of deafness is restricted to the inner ear. Sound deprivation is responsible for significant alterations in the brain stem and cerebral hemispheres. These alterations are due to cerebral plasticity which compensates for an anomalous situation. For example, in animals blinded at birth a kind of colonization of the visual cortex by the hearing function occurs (5). The younger the subject is, the more important the role plasticity has; with age, the phenomenomn of adaptation becomes irreversible. An early restoration of the hearing function is able to avoid the alterations created by sensorial deprivation. Treatment of deafness should therefore be carried out before that critical point after which anatomical alteration becomes definitive, even when the hearing function is restored. We were able to demonstrate this several years ago (6). In guinea pigs deafened at birth, after several weeks we observed atrophy of the hearing centres of the brain stem. Such atrophy can be largely avoided by a cochlear implant which can supply the cerebral centres with sound information necessary for their development. This preventative effect of the cochlear implant does not, however, occur in cases in which auditory stimulation is not supplied early enough, that is, before the critical age of 45 days in guinea pigs which corresponds in humans to aprox. 4-5 years old. If the restoration of the hearing function is too late, the cerebral structure is already altered by deafness and is incapable of modifying itself further for the eventual development of language. For this reason, we maintain that the restoration of the hearing function in children through cochlear implants should be considered as urgent. We can thus formulate three fundamental concept which are linked together: 1) The notion of a critical period in which the treatment of profound deafness with cochlear implants is truly effective; 2) The notion of a time limit, variable, but set well before the onset of adolescence, after which such treatment loses its efficacy; 3) The notion of urgent therapy.