Occipital meningocele and Mondini deformity of the cochlea

We present two cases of a previously unreported association between occipital meningocele and Mondini deformity of the cochlea. The probability of the association is supported by the intimate relationship between the development of these structures; the existence of dysembryoplastic factors that can cause both abnormalities; and the universal existence of cochlear abnormalities in anencephalic foetuses. The importance of considering the association is emphasized, in terms of early identification and referral of children with hearing loss, and avoidance of duplication of investigations.