There is an increased prevalence of otitis media and associated hearing loss in patients with isolated cleft or submucous cleft palates, palatal abnormalities which are well known components of velo-cardio-facial syndrome (VCFS). The records of 166 VCFS patients were reviewed for the purpose of documenting the prevalence of middle ear disease and associated conductive hearing loss in patients with VCFS. Patients were divided according to age, type of palatal abnormality and presence or absence of chromosomal deletion using fluorescence hybridization test. Results indicated that the prevalence of middle ear disease in patients with VCFS was 47% and the prevalence of hearing loss was 44%, the majority of which was conductive and due to middle ear disease (74%) and 11% of which was sensorineural. There was no statistical difference between groups using the chi2-test with regard to age, type of palatal abnormality and presence of chromosomal deletion. The prevalence of conductive hearing loss secondary to middle ear disease in VCFS is comparable to the prevalence found in isolated cases of cleft palate.