Lemire et al. (1998) described a 9-year-old girl, the daughter of consanguineous Mennonite parents, who had a seemingly unique multiple congenital anomaly syndrome consisting of short stature, auditory canal atresia, mandibular hypoplasia, and skeletal abnormalities. The skeletal defects ... Lemire et al. (1998) described a 9-year-old girl, the daughter of consanguineous Mennonite parents, who had a seemingly unique multiple congenital anomaly syndrome consisting of short stature, auditory canal atresia, mandibular hypoplasia, and skeletal abnormalities. The skeletal defects consisted of bilateral humeral hypoplasia, delayed ossification of the pubic rami, and previously unreported humeroscapular synostosis. Either autosomal recessive inheritance or new autosomal dominant mutation were considered plausible. The child was the youngest of 6 children. The face was round with somewhat broad forehead, deep-set eyes, downslanting palpebral fissures, micrognathia, and small mouth with prominent incisors. The ears showed a simple, dysplastic pinnae with atretic ear canals. Ter Heide et al. (2002) described a child with auditory canal atresia and scapulohumeral synostosis who had previously been reported by Schrander-Stumpel et al. (1998). The girl was the fifth child of first-cousin Afghan parents and was noted to have simply formed external ears with bilateral auditory canal atresia, short humeri, and clubfeet. Her external genitalia were hypoplastic and ventrally displaced with the urethra ending within the vagina. She also had severe mandibular hypoplasia, proximally implanted thumbs, delayed ossification of the pubic rami, and dysplastic hips. Chromosomal studies and mental development were normal.