Nager Acrofacial Dysostosis Syndrome: A Newborn with Bilateral Hearing Impairment

Introduction Nager acrofacial dysostosis (NAFD) comprises defects of craniofacial region and limbs (mostly upper) with variable associated anomalies. This syndrome was recognized as a specific entity by Nager and deReynier1, Craniofacial features include malar hypoplasia, micrognathia, low set posteriorly rotated ears, preauricular tag, atresia of external ear canal. The limb deformities in the Nager syndrome consists of hypoplasia to aplasia of thumb, with or without radius, proximal radioulnar synostosis and short forearm2,3. Conductive deafness usually bilateral and problems with articulation is also noted in this syndrome2. Occasional abnormalities may comprise genitourinary abnormality, hypoplastic 1st rib, downward pointing of ribs, lower lid coloboma and cleft lip4.