Congenital and acquired deafness in clefting and craniofacial syndromes

A study of 284 craniofacial defect patients determined factors associated with or predisposing to acquired or congenital hearing loss. Complete otolaryngic and audiologic evaluation was done at the initial assessment of a large group of patients. From there, a smaller number who had had adequate workup and followup was selected. The patients were classified into seven clefting categories, microtia/artresia, facial defects, cranial defects and miscellaneous anomalies of the head and neck. X-ray findings, those present at middle ear surgery and those studied at autopsy, are summarized. The incidence of hearing loss is 88 per cent. The likelihood of congenital hearing loss increases with the number of defects. Certain unifying concepts that may help identify a specific child as being in urgent need of otologic and audiologic testing are presented.