Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome)

H Skarżyński, M Porowski… - International journal of …, 2009 - Elsevier
The oculoauriculovertebral dysplasia is a rare congenital malformation that is characterized
by a hemifacial microsomia with ocular abnormalities and coexisting disorders in the spinal …

Fgf3 is required for dorsal patterning and morphogenesis of the inner ear epithelium

EP Hatch, CA Noyes, X Wang, TJ Wright, SL Mansour - 2007 - journals.biologists.com
The inner ear, which contains sensory organs specialized for hearing and balance,
develops from an ectodermal placode that invaginates lateral to hindbrain rhombomeres (r) …

Oculo-auriculo-vertebral spectrum: going beyond the first and second pharyngeal arch involvement

B Davide, M Renzo, G Sara, L Elisa, M Rodica, T Irene… - Neuroradiology, 2017 - Springer
Introduction Intracranial vascular, bone, and brain abnormalities might be observed in oculo-
auriculo-vertebral spectrum (OAVS) patients even though these structures do not derive …

Goldenhar's syndrome: congenital hearing deficit of conductive or sensorineural origin? Temporal bone histopathologic study

AW Scholtz, JH Fish III, K Kammen-Jolly… - Otology & …, 2001 - journals.lww.com
Background Oculoauriculovertebral dysplasia (OAVD)(Goldenhar's syndrome) is a
congenital syndrome with ipsilateral deformity of the ear and face, epibulbar lipodermoids …

Inner ear abnormalities in patients with Goldenhar syndrome

S Bisdas, M Lenarz, T Lenarz, H Becker - Otology & Neurotology, 2005 - journals.lww.com
Objective: The objective of this study is to investigate the inner ear malformations in patients
with Goldenhar syndrome and to hypothesize the potential embryopathogenesis of these …

Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities

F Hennersdorf, N Friese, H Löwenheim… - Otology & …, 2014 - journals.lww.com
Objective Goldenhar syndrome is a developmental disorder presenting with orofacial and
vertebral anomalies, which are also accompanied by abnormalities in other organs. We …

[HTML][HTML] Elongation Factor 1 alpha1 and Genes Associated with Usher Syndromes Are Downstream Targets of GBX2

DA Roeseler, S Sachdev, DM Buckley, T Joshi, DK Wu… - PloS one, 2012 - journals.plos.org
Gbx2 encodes a DNA-binding transcription factor that plays pivotal roles during
embryogenesis. Gain-and loss-of-function studies in several vertebrate species have …

CT and MRI of the semicircular canals in the normal and diseased temporal bone

M Lemmerling, B Vanzieleghem, I Dhooge… - European …, 2001 - Springer
Imaging of the semicircular canals specifically is part of the imaging process of the temporal
bone in general. The semicircular canals are easily seen on CT images and 3DFT-CISS …

Unilateral semicircular canal aplasia in Goldenhar's syndrome

MM Lemmerling, BD Vanzieleghem… - American journal …, 2000 - Am Soc Neuroradiology
A patient with Goldenhar's syndrome (oculoauriculovertebral dysplasia) and unilateral
aplasia of all semicircular canals is presented. This is the first report of such a finding and …

The differential sensitivities of inner ear structures to retinoic acid during development

D Choo, JL Sanne, DK Wu - Developmental biology, 1998 - Elsevier
In order to examine the mechanisms that underlie development of the inner ear, the normal
processes were perturbed using all-trans-retinoic acid (RA). By implanting a resin exchange …