Nager syndrome: an update of speech and hearing characteristics

Nager acrofacial dysostosis is a rare syndrome of unknown etiology combining mandibular and thumb/radial hypoplasia. Seven patients evaluated in this study had histories of early respiratory and feeding problems, micrognathia and absent velum, atretic ear canals and conductive hearing loss, upper and lower limb malformations, normal intelligence, and speech/language delays and disorders. These findings, with few exceptions, were consistent with the findings in previously published and unpublished case histories of patients with Nager syndrome. Recommended rehabilitative strategies include prespeech feeding activities (especially if gastrostomy tubes are present), oral language stimulation, individualized speech/language therapy, and early audiologic evaluation and amplification.