@article {Som:2016:2637-8329:250,
title = "Current Embryology of the Temporal Bone, Part I: the Inner Ear",
journal = "Neurographics",
parent_itemid = "infobike://asnr/ng",
publishercode ="asnr",
year = "2016",
volume = "6",
number = "4",
publication date ="2016-07-01T00:00:00",
pages = "250-265",
itemtype = "ARTICLE",
issn = "2637-8329",
eissn = "2637-8329",
url = "https://asnr.publisher.ingentaconnect.com/content/asnr/ng/2016/00000006/00000004/art00008",
doi = "doi:10.3174/ng.4160166",
keyword = "SHH = sonic hedgehog, a secreted signaling factor, GSC = Goosecoid, a homeobox protein, MSX = Muscle segment homeobox gene, RA = retinoic acid, TGF = transforming growth factor, OTX = Orthodenticle-related homeobox gene, HES = Hairy and enhancer of split bHLH transcription factors, SOX = Sry-related homeobox gene, NEUROD = Neuronal differentiation, a basic helix-loop helix transcription factor, FGF = Fibroblast growth factor, PENDRIN = A sodium-independent chloride/iodide transporter, GBX = Gastrulation and brain-specific homeobox protein, BEN = Bursal epithelium and neurons, a surface glycoprotein, GATA = Transcription factors binding to the “GATA” DNA sequence, HLH = Helix-loop-helix transcription factors, DELTA = Single-pass transmembrane ligand for Notch, mediating cell-cell interactions, PAX = Paired box transcription factors, DAN = Differential screening-selected gene aberrative in neuroblastoma, a BMP inhibitor, Espin = Ectoplasmic specialization protein or autosomal recessive deafness protein, DLX = The Distal-less (Dlx) family of genes, NT3 = Neurotrophin-3, a secreted growth factor, EYA = Eyes absent homolog, a transcription factor, PRX = paired class homeobox gene, 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, NUMB = Encodes a protein that plays a role in determining cell fates during development, DIX = Domain protein important in WNT and other protein-protein interactions, bHLH = Basic helix-loop helix, Endothelin = A secreted signaling peptide, FGFR = Fibroblast growth factor receptor, JAGGED = A ligand for the receptor Notch, HOX = A homeobox protein, Myosin = A family of ATP-dependent motor proteins, BMP = Bone morphogenetic protein, BRN3 = Brain-3, a POU domain transcription factor, TIMP = Tissue inhibitors of metalloproteinases, LUNATIC FRINGE = A gene whose role in embryonic development is to establish the anterior boundary of regions, DACH = Dachshund homolog, a nuclear factor, POU = Pou domain transcription factors, LFNG = Lunatic Fringe, a member of the fringe gene family of GlcNAc-transferases, which modify Notch signaling, FOX = Forkhead box transcription factor, BDNF = Brain derived neurotrophic factor, WNT = wingless/int1 family of secreted signaling molecules, MATH = Mammalian atonal homologue 1 (also known as Atoh1), a proneural protein, SERRATE = transmembrane ligand for NOTCH, DLX = Distal-less family of homeobox protein, NOTCH = A transmembrane receptor for Delta and Serrate, mediating cell-cell interactions, SIX = sine oculis homeobox transcription 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 was the basis of this review. Because 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 also provides insight into how the malformations of the temporal bone arise. The review is heavily illustrated to help the reader better understand the text. There is also a section
that describes the major congenital-related abnormalities that have known associated gene malfunctions. This review has 2 parts due to the length and complexity of the material. Part I discusses the inner ear embryology. Part II discusses the middle and external ears, pneumatization of the
temporal bone, the neonatal temporal bone, the statoacoustic and facial nerves, and a discussion of when things go developmentally wrong.Learning Objective: The reader will understand the development of the inner ear and how it can lead to the congenital variations that are encountered
in a radiology practice.",
}