ABSTRACT
In this chapter, we will discuss cochlear implantation (CI), which is now widely employed in the rehabilitation of many forms of genetically determined deafness. We will point out the difficulties that may be encountered in implanting various inner ear dysplasias, the risks that might be encountered, and results that might be expected. We also look at the outcomes that one might reasonably hope for in the largest group of implanted children, those with recessively inherited connexin 26 mutation. The genetic causes of deafness are many and the numbers of individuals with the less common syndromes who have received CI are small; so information about outcomes in specific conditions is still scanty, if it exists at all. With the current state of our knowledge, therefore, this chapter must of necessity be somewhat rudimentary. Nevertheless, generalisations can be made about the pattern of phenotype that is likely to predict good or bad outcomes. The risks associated with comorbidity in certain deafness syndromes are touched upon. Finally the indications for the auditory brainstem implant (ABI) are discussed for that small group of patients who are not suitable for CI.
