CT findings of the temporal bone in CHARGE syndrome: aspects of importance in cochlear implant surgery
| Autor: | van Conny Ravenswaaij-Arts, Henriëtte E. Westerlaan, Berit M. Verbist, Rolien Free, Ronald J.C. Admiraal, Francka J. J. Kloostra, Annemarie Vesseur, Emmanuel A. M. Mylanus |
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| Přispěvatelé: | Perceptual and Cognitive Neuroscience (PCN), Clinical Cognitive Neuropsychiatry Research Program (CCNP) |
| Jazyk: | angličtina |
| Předmět: |
Male
ANOMALIES medicine.medical_treatment Otology CHILDREN Sensory disorders Donders Center for Medical Neuroscience [Radboudumc 12] Otolaryngology CHARGE syndrome 0302 clinical medicine Cochlear implant Temporal bone Child 030223 otorhinolaryngology CHD7 GENE INNER-EAR Oval window General Medicine Anatomy ASSOCIATION Middle Aged Cochlear Implantation Facial nerve Cochlea DNA-Binding Proteins Facial Nerve SEMICIRCULAR CANALS medicine.anatomical_structure Child Preschool Middle ear Female Radiology Rare cancers Radboud Institute for Health Sciences [Radboudumc 9] Adult Adolescent Ear Middle Mastoid 03 medical and health sciences medicine Genetics otorhinolaryngologic diseases Humans Inner ear MALFORMATIONS Retrospective Studies SPECTRUM Round window business.industry MUTATIONS DNA Helicases Infant Newborn Infant medicine.disease Cochlear Implants Round Window Ear Otorhinolaryngology sense organs Tomography X-Ray Computed business 030217 neurology & neurosurgery PETROSQUAMOSAL SINUS |
| Zdroj: | European Archives of Oto-Rhino-Laryngology, 273(12), 4225-4240. SPRINGER European Archives of Oto-Rhino-Laryngology, 273, 4225-4240 European Archives of Oto-Rhino-Laryngology European Archives of Oto-Rhino-Laryngology, 273(12), 4225-4240 European Archives of Oto-Rhino-Laryngology, 273, 12, pp. 4225-4240 |
| ISSN: | 0937-4477 |
| DOI: | 10.1007/s00405-016-4141-z |
| Popis: | Contains fulltext : 167636.pdf (Publisher’s version ) (Open Access) To provide an overview of anomalies of the temporal bone in CHARGE syndrome relevant to cochlear implantation (CI), anatomical structures of the temporal bone and the respective genotypes were analysed. In this retrospective study, 42 CTs of the temporal bone of 42 patients with CHARGE syndrome were reviewed in consensus by two head-and-neck radiologists and two otological surgeons. Anatomical structures of the temporal bone were evaluated and correlated with genetic data. Abnormalities that might affect CI surgery were seen, such as a vascular structure, a petrosquamosal sinus (13 %), an underdeveloped mastoid (8 %) and an aberrant course of the facial nerve crossing the round window (9 %) and/or the promontory (18 %). The appearance of the inner ear varied widely: in 77 % of patients all semicircular canals were absent and the cochlea varied from normal to hypoplastic. A stenotic cochlear aperture was observed in 37 %. The middle ear was often affected with a stenotic round (14 %) or oval window (71 %). More anomalies were observed in patients with truncating mutations than with non-truncating mutations. Temporal bone findings in CHARGE syndrome vary widely. Vascular variants, aberrant route of the facial nerve, an underdeveloped mastoid, aplasia of the semicircular canals, and stenotic round window may complicate cochlear implantation. |
| Databáze: | OpenAIRE |
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