A.B.R. in Nager type acrofacial dysostosis syndrome.

The acrofacial dysostosis of nager is a very rare hereditary syndrome in which findings of mandibulofacial dysostosis are associated with defects of the limbs. The developmental defects of the mesoderm if accompany the neurectodermal malformations, result in mental retardation and sensorineural hearing impairment. The conductive type of hearing loss is mostly encountered in Nager's syndrome but here we present a case with sensorineural involvement, along with its analysis of auditory brainstem evoked response audiometry (ABR).