[6]. Virtually all individuals with CHARGE syndrome have recurrent otitis media with effusion in their first year of life. Approximately two-thirds of individuals clinically diagnosed with CHARGE syndrome have a mutation in the Chd7 gene [7], encoding the chromodomain helicase DNA-binding protein 7 (CHD7). Recent evidence suggests that Chd7 functions as a regulator of genes which play a role in cell-lineage specification in the nucleus and as a positive regulator of rRNA biogenesis in the nucleolus [8], [9], [10]. This evidence concerns the gene CHD7 and otitis media with effusion.