Recent work shows that FBXO11 mutations (Online Mendelian Inheritance in Man number 607871) are associated with intellectual disability (Fritzen et al., 2018) and variable neurodevelopmental disorder (Gregor et al., 2018); it is noteworthy that 3 of 20 children in the latter cohort had otitis media, but a background level of spontaneous chronic otitis media might be expected in this age group. Here, FBXO11 is linked to chronic otitis media.