Based upon the known association of cleft palate and middle ear disease, a study was undertaken to determine the incidence and type of middle ear pathology associated with velar anomalies exclusive of cleft palate which may produce congenital palatal pharyngeal incompetence. The range of velar anomalies encountered was subdivided into congenital palatal incompetence Type 1 (clinically manifested by one or more of a triad of visible palatal anomalies including submucous deficiency of the hard palate, bifid uvula, and a diastasis of velar musculature) and congenital palatal incompetence Type 2 (no visible velar anomalies but radiographic anomalies of the velopharyngeal region such as short or thin velum and/or enlarged nasopharyngeal dimensions consequent to vertebral and skull base anomalies). Middle ear disease was assessed separately in CPI Types 1 and 2 in order to differentiate the effects upon middle ear function between overt and occult velar anomalies. Middle ear disease was more frequent in CPI Type 1 than in CPI Type 2. The predominant otopathologic finding was serous otitis media, paralleling the type associated with cleft palate. Tympanic membrane atrophy, tympanosclerosis and tympanic membrane perforation, often considered sequelae of chronic serous otitis media, were noted infrequently. This investigation supports the concept that middle ear disease frequently occurs with congenital palatal incompetence as it does with cleft palate.