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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Research ArticlePediatric Neuroimaging
Open Access

Characteristic Cochlear Hypoplasia in Patients with Walker-Warburg Syndrome: A Radiologic Study of the Inner Ear in α-Dystroglycan–Related Muscular Disorders

G. Talenti, C. Robson, M.S. Severino, C.A. Alves, D. Chitayat, H. Dahmoush, L. Smith, F. Muntoni, S.I. Blaser and F. D’Arco
American Journal of Neuroradiology November 2020, DOI: https://doi.org/10.3174/ajnr.A6858
G. Talenti
aDepartment of Diagnostics and Pathology (G.T.), Neuroradiology Unit, Verona University Hospital, Verona, Italy
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C. Robson
bDivision of Neuroradiology (C.R.), Department of Radiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
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M.S. Severino
cNeuroradiology Unit (M.S.S.), Istituti di Ricovero e Cura a Carattere Scientifico, Istituto Giannina Gaslini, Genova, Italy
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C.A. Alves
dDepartments of Radiology and Division of Neuroradiology (C.A.A.), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
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D. Chitayat
eThe Prenatal Diagnosis and Medical Genetics Program (D.C.), Department of Obstetrics and Gynecology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada
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H. Dahmoush
fDepartment of Radiology (H.D.), Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California
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L. Smith
gDental and Maxillofacial Surgery Department (L.S.), Great Ormond Street Hospital, London, UK
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F. Muntoni
hDubowitz Neuromuscular Centre (F.M.), UCL Great Ormond Street Institute of Child Health and Great Ormond Street Hospital, London, UK
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S.I. Blaser
iDivision of Neuroradiology (S.I.B.), Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada
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F. D’Arco
jNeuroradiology Unit (F.D.), Department of Radiology, Great Ormond Street Hospital for Children, National Health Service Trust, London, UK
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Article Information

DOI 
https://doi.org/10.3174/ajnr.A6858
PubMed 
33122211

Published By 
American Journal of Neuroradiology
Print ISSN 
0195-6108
Online ISSN 
1936-959X
History 
  • Received June 6, 2020
  • Accepted after revision August 16, 2020
  • Published online November 12, 2020.

Article Versions

  • Latest version (October 29, 2020 - 07:58).
  • You are currently viewing a Latest version of this article (November 12, 2020 - 07:53).
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Copyright & Usage 
© 2020 by American Journal of Neuroradiology Indicates open access to non-subscribers at www.ajnr.org

Author Information

  1. G. Talentia,
  2. C. Robsonb,
  3. M.S. Severinoc,
  4. C.A. Alvesd,
  5. D. Chitayate,
  6. H. Dahmoushf,
  7. L. Smithg,
  8. F. Muntonih,
  9. S.I. Blaseri and
  10. F. D’Arcoj
  1. aDepartment of Diagnostics and Pathology (G.T.), Neuroradiology Unit, Verona University Hospital, Verona, Italy
  2. bDivision of Neuroradiology (C.R.), Department of Radiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
  3. cNeuroradiology Unit (M.S.S.), Istituti di Ricovero e Cura a Carattere Scientifico, Istituto Giannina Gaslini, Genova, Italy
  4. dDepartments of Radiology and Division of Neuroradiology (C.A.A.), Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
  5. eThe Prenatal Diagnosis and Medical Genetics Program (D.C.), Department of Obstetrics and Gynecology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada
  6. fDepartment of Radiology (H.D.), Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California
  7. gDental and Maxillofacial Surgery Department (L.S.), Great Ormond Street Hospital, London, UK
  8. hDubowitz Neuromuscular Centre (F.M.), UCL Great Ormond Street Institute of Child Health and Great Ormond Street Hospital, London, UK
  9. iDivision of Neuroradiology (S.I.B.), Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada
  10. jNeuroradiology Unit (F.D.), Department of Radiology, Great Ormond Street Hospital for Children, National Health Service Trust, London, UK
  1. Please address correspondence to Felice D’Arco, MD, Neuroradiology Unit, Department of Radiology, Great Ormond St Hospital for Children NHS Trust, Gr. Ormond Street, WC1N3JH – London, United Kingdom; e-mail: felice.d'arco{at}gosh.nhs.uk
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  • The Cochlea in Branchio-Oto-Renal Syndrome: An Objective Method for the Diagnosis of Offset Cochlear Turns
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  • The cochlear basal turn as a very preserved region in cochlear hypoplasias: radiological and embryological considerations from a cohort of 125 patients
    Antonio Messina, Emma Clement, Bernadette De Bakker, Robert Nash, Giovanni Morana, Shivaram Avula, Steve Connor, Felice D’Arco
    Neuroradiology 2025
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G. Talenti, C. Robson, M.S. Severino, C.A. Alves, D. Chitayat, H. Dahmoush, L. Smith, F. Muntoni, S.I. Blaser, F. D’Arco
Characteristic Cochlear Hypoplasia in Patients with Walker-Warburg Syndrome: A Radiologic Study of the Inner Ear in α-Dystroglycan–Related Muscular Disorders
American Journal of Neuroradiology Nov 2020, DOI: 10.3174/ajnr.A6858

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Characteristic Cochlear Hypoplasia in Patients with Walker-Warburg Syndrome: A Radiologic Study of the Inner Ear in α-Dystroglycan–Related Muscular Disorders
G. Talenti, C. Robson, M.S. Severino, C.A. Alves, D. Chitayat, H. Dahmoush, L. Smith, F. Muntoni, S.I. Blaser, F. D’Arco
American Journal of Neuroradiology Nov 2020, DOI: 10.3174/ajnr.A6858
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Cited By...

  • Subtle Malformation of the Cochlear Apex and Genetic Abnormalities: Beyond the "Thorny" Cochlea
  • The Cochlea in Branchio-Oto-Renal Syndrome: An Objective Method for the Diagnosis of Offset Cochlear Turns
  • Crossref (11)
  • Google Scholar

This article has been cited by the following articles in journals that are participating in Crossref Cited-by Linking.

  • Congenital muscular dystrophies: What is new?
    Alberto A. Zambon, Francesco Muntoni
    Neuromuscular Disorders 2021 31 10
  • A single-cell level comparison of human inner ear organoids with the human cochlea and vestibular organs
    Wouter H. van der Valk, Edward S.A. van Beelen, Matthew R. Steinhart, Carl Nist-Lund, Daniel Osorio, John C.M.J. de Groot, Liang Sun, Peter Paul G. van Benthem, Karl R. Koehler, Heiko Locher
    Cell Reports 2023 42 6
  • Genetics of Inner Ear Malformations: A Review
    Davide Brotto, Flavia Sorrentino, Roberta Cenedese, Irene Avato, Roberto Bovo, Patrizia Trevisi, Renzo Manara
    Audiology Research 2021 11 4
  • The spectrum of cochlear malformations in CHARGE syndrome and insights into the role of the CHD7 gene during embryogenesis of the inner ear
    Martin A. Lewis, Amy Juliano, Caroline Robson, Emma Clement, Robert Nash, Kaukab Rajput, Felice D’Arco
    Neuroradiology 2023 65 4
  • Syndromic Hearing Loss in Children
    Martin Lewis, Caroline D. Robson, Felice D‘Arco
    Neuroimaging Clinics of North America 2023 33 4
  • Etiology of Childhood Profound Sensorineural Hearing Loss: The Role of Hearing Loss Gene Panel Testing
    Kaukab Rajput, Umar Akhtar, Waheeda Pagarkar, Sarah Rajput, Claire Walder, Felice D'Arco, Lesley Cochrane, Robert Nash, Maria Bitner‐Glindzicz, Rohani Omar
    Otolaryngology–Head and Neck Surgery 2024 171 5
  • Cochlear Implant in cochlear Hypoplasia type 4 with Chiari Malformation- Case Report
    Neeraj Suri, Anshu Singh, Diva Sharma
    Indian Journal of Otolaryngology and Head & Neck Surgery 2023 75 3
  • Pediatric Neuroradiology
    Ana Filipa Geraldo, Andrea Rossi, Mariasavina Severino
    2021
  • Subtle Malformation of the Cochlear Apex and Genetic Abnormalities: Beyond the “Thorny” Cochlea
    F. D’Arco, A. Biswas, E. Clement, K. Rajput, A.F. Juliano
    American Journal of Neuroradiology 2023 44 1
  • The Cochlea in Branchio-Oto-Renal Syndrome: An Objective Method for the Diagnosis of Offset Cochlear Turns
    A.F. Juliano, F. D’Arco, J. Pao, S. Picariello, E. Clement, G. Moonis, C.D. Robson
    American Journal of Neuroradiology 2022 43 11

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