Hindawi Publishing Corporation Case Reports in Pediatrics Volume 2013, Article ID 591350, 3 pages http://dx.doi.org/10.1155/2013/591350 Case Report A Case of Monozygotic Twins: The Value of Discordant Monozygotic Twins in Goldenhar Syndrome—OMIM%164210 K. N. Venkateshwara Prasad, 1 Arvind Rajha, 1 and Pradeep Kumar Vegi 2 1 Department of Paediatrics, Sri Devaraj Urs Medical College, Kolar, Karnataka 563101, India 2 Scientifc Research Laboratory-AHS, Department of Biochemistry, Sri Devaraj Urs Academy of Higher Education and Research, Kolar, Karnataka 563101, India Correspondence should be addressed to Pradeep Kumar Vegi; lifesresearch7@gmail.com Received 21 June 2013; Accepted 18 July 2013 Academic Editors: N. Arslan, J. Kobr, M. Tzouf, and P. Visrutaratna Copyright © 2013 K. N. Venkateshwara Prasad et al. Tis is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Goldenhar syndrome is a rare developmental disorder characterised by hemifacial microsomia, epibulbar tumours, ear malfo- rmation, and vertebral anomalies. As monozygotic (MZ) twins are believed to be genetically identical, discordance for disease phenotype between MZ twins varies with craniofacial anomalies, cardiac, vertebral, and central nervous system defects sporadically. We report a case of monozygotic female twins discordant for Goldenhar syndrome with hemifacial microsomia and the dysplasia of auricular pinna. 1. Introduction Goldenhar syndrome is one of the rare craniofacial anomalies with incidence of 1 : 3500 to 1 : 5600 births [1]. It is unilat- eral in 70–80% of the cases [2] as oculoauriculo vertebral (OAV) dysplasia or hemifacial microsomia (OMIM%164210) or facioauriculo vertebral sequence [3]. Nevertheless, the origin of the OAVS is unclear but states as complex and heterogeneous in condition. Two pathophysiologic mecha- nisms have been proposed for the OAVS: a reduced blood fow and focal hemorrhage in the development region of the frst and second branchial arches around 30 to 45 days of pregnancy, in the blastogenesis period. Tese mechanisms explain the outer ear abnormalities in this spectrum, as the frst branchial arch gives rise to the anterior ear primordium and the second branchial arch originates the posterior ear primordium. Also, the outer ear canal derives from the dorsal portion of the frst branchial clef[4]. Although external ear anomalies have been described in OAVS patients—to the point of being inclusion criteria—middle and especially inner ear alterations have received little attention in the literature [4, 5]. As the function of variability in clinical pre- sentations, there are patients who are aficted with minimal clinical manifestations, predominantly facial asymmetry and dysplasia of the external ear [6]. Tis case demonstrates the heterogeneity of spectrums with multifactorial features with frequent alterations. 2. Case Report A twenty-year-old 2nd gravida healthy mother delivered female twins per vaginum dated on 19-11-2012, following 36 weeks of uneventful gestation in a rural tertiary care—R L Jalappa Hospital, India. Amongst the twins, the frst female twin baby was born by assisted breach delivery and cried soon afer the birth with 1.84 kgs by weight, 47 cms in length, and 32 cms head circumference. On examination, the baby was referred as late-preterm baby with an uneventful neonatal period and normal examination. Te second female twin baby born by vertex presentation and cried soon afer birth; Apgar score at 1 minute was 8/10, weighing 1.94 kgs, 47 cms by length, and 30 cms head circumference. Te placenta was completely expelled, monochorionic and diamniotic. On clinical examination, the 2nd twin baby showed the following abnormal features as shown in Figures 1 and 2. Facial features: hypoplasia of the right malar region, microg- nathia, and hypertelorism. Ears: bilateral accessory tags, right