Interstitial deletion of 4p15.32p16.3 in a boy with minor anomalies, hearing loss, borderline intelligence, and oligodontia

We describe an Ii-year-old boy of Saudi origin with an interstitial deletio n in the short arm of chromosome 4 (p15.32p16.3) as determined by G-banding and fluorescent in situ hybridization. His clinical manifestations were si milar but not identical to previously reported cases of interstitial deleti on in the same chromosomal region, and were not those associated with Wolf- Hirschhorn syndrome, The boy had normal facial characteristics, short statu re, minor anomalies of hands and feet, amblyopia of the right eye, bilatera l hearing loss, and hypotonia. On developmental testing, he had borderline intelligence, with a severe sensory integration and motor planning disorder , and severe deficits in the communication domain. In addition, he had seve re oligodontia affecting his secondary dentition. This finding supports the presence of one or more genes involved in dentition in this chromosomal re gion. Am. J. Med, Genet, 86:316-320, 1999. (C) 1999 Wiley-Liss, Inc.