@article {Som:2016:2637-8329:332,
title = "Current Embryology of the Temporal Bone, Part II: the Middle and External Ears, the Statoacoustic and Facial Nerves, and When Things Go Developmentally Wrong",
journal = "Neurographics",
parent_itemid = "infobike://asnr/ng",
publishercode ="asnr",
year = "2016",
volume = "6",
number = "5",
publication date ="2016-09-01T00:00:00",
pages = "332-349",
itemtype = "ARTICLE",
issn = "2637-8329",
eissn = "2637-8329",
url = "https://asnr.publisher.ingentaconnect.com/content/asnr/ng/2016/00000006/00000005/art00008",
doi = "doi:10.3174/ng.5160174",
keyword = "PAX = Paired box transcription factor, PRX = Paired class homeobox gene, NT3 = Neurotrophin-3, a secreted growth factor, ET1 = Endothelin 1, s secreted signaling peptide, HES = Hairy and enhancer of split bHLH transcription factors, LFNG = Lunatic Fringe, a member of the fringe gene family of GlcNAc-transferases, which modify Notch signaling, Espin = Ectoplasmic specialization protein or autosomal recessive deafness protein, KSS = Kearns-Sayre syndrome, BRN3 = Brain-3, a POU domain transcription factor, NEUROD = Neuronal differentiation, a basic helix-loop helix transcription factor, DAN = Differential screening-selected gene aberrative in neuroblastoma, a BMP inhibitor, RA = Retinoic acid, GBX = Gastrulation and brain-specific homeobox protein, GATA = Transcription factors binding to the “GATA” DNA sequence, NOTCH = A transmembrane receptor for Delta and Serrate, mediating cell-cell interactions, WNT = Wingless/int1 family of secreted signaling molecules, MATH = Mammalian atonal homologue one (also known as Atoh1), a proneural protein, FOX = Forkhead box transcription factor, JAGGED =A ligand for the receptor Notch, SOX = Sry-related homeobox gene, HOX = A homeobox protein, MELAS = Mitochondrial encephalopathy, lactic acidosis and strokelike episode, SERRATE = Transmembrane ligand for NOTCH, BDNF = Brain-derived neurotrophic factor, DELTA = Single-pass transmembrane ligand for Notch, mediating cell-cell interactions, FGFR = Fibroblast growth factor receptor, DACH = Dachshund homolog, a nuclear factor, Myosin = A family of ATP-dependent motor proteins, CONNEXIN = A Gap junction protein, POU = Pou domain transcription factors, LUNATIC FRINGE = A gene whose role in embryonic development is to establish the anterior boundary of regions, MSX = Muscle segment homeobox gene, FGF = Fibroblast growth factor, SIX = Sine oculis homeobox transcription factor, DIX = Domain protein important in WNT and other protein-protein interactions, EYA = The EYA1 gene provides instructions for making a protein that plays a role in regulating the activity of other genes; it is a transcription factor or transcription coactivator, BEN = Bursal epithelium and neurons, a surface glycoprotein, EYA = Eyes absent homolog, a transcription factor, DLX = Distal-less family of homeobox protein, NUMB = Encodes a protein that plays a role in determining cell fates during development, MERRF = Myoclonic epilepsy and ragged red fibers, TM = Tympanic membrane, TGF = Transforming growth factor, MIDD = Maternally inherited diabetes and deafness, PENDRIN = A sodium-independent chloride/iodide transporter, HLH = Helix-loop-helix transcription factor, OTX = Orthodenticle-related homeobox gene, DLX = The Distal-less (Dlx) family of genes, BMP = Bone morphogenetic protein, TIMP = Tissue inhibitors of metalloproteinases, GSC = Goosecoid, a homeobox protein, SHH =Sonic hedgehog, a secreted signaling factor",
author = "Som, P.M. and Curtin, H.D. and Liu, K. and Mafee, M.F.",
abstract = "The development of the temporal bone is complex, with 3 distinctly different origins, namely, the inner ear derives from neuroectoderm, the middle ear comes from branchial arch mesoderm and endoderm, and the external ear arises from branchial ectoderm. The current understanding of how
these varied structures come together to form the normal functioning ear is the basis of this review. As we now are starting to gain insight into the molecular biology that drives this complex process, some molecular information is included in this review. This molecular biology is primarily
based on animal experiments and, not only provides a better understanding of how the normal anatomy develops, but provides insight into how the malformations of the temporal bone arise. The review was heavily illustrated to help the reader better understand the text. There also was a section
that described the major congenital-related abnormalities that have known associated gene malfunctions.Learning Objective: The reader will understand the development of the ear and how it can lead to the congenital variations that are encountered in a radiology practice.",
}