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dc.contributor.authorBiggs, Kirsty
dc.contributor.authorCrundwell, Gemma
dc.contributor.authorMetcalfe, Christopher
dc.contributor.authorMuzaffar, Sayed
dc.contributor.authorMonksfield, Peter
dc.contributor.authorBance, Manohar
dc.date.accessioned2022-05-19T01:03:09Z
dc.date.available2022-05-19T01:03:09Z
dc.date.issued2022-04
dc.identifier.issn2378-8038
dc.identifier.other35434312
dc.identifier.otherPMC9008175
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/337295
dc.description.abstractObjective: Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods: Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence-Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results: Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty-nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion: The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence: NA.
dc.languageeng
dc.publisherWiley
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/
dc.sourcenlmid: 101684963
dc.sourceessn: 2378-8038
dc.subjectSystematic review
dc.subjectAudiology
dc.subjectBranchiootorenal Syndrome
dc.subjectOtology/neurotology
dc.titleAnatomical and audiological considerations in branchiootorenal syndrome: A systematic review.
dc.typeArticle
dc.date.updated2022-05-19T01:03:09Z
prism.endingPage563
prism.issueIdentifier2
prism.publicationNameLaryngoscope Investig Otolaryngol
prism.startingPage540
prism.volume7
dc.identifier.doi10.17863/CAM.84710
dcterms.dateAccepted2021-12-11
rioxxterms.versionofrecord10.1002/lio2.749
rioxxterms.versionVoR
rioxxterms.licenseref.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/
dc.contributor.orcidBiggs, Kirsty [0000-0003-2993-9495]
dc.contributor.orcidCrundwell, Gemma [0000-0001-5798-4264]
dc.contributor.orcidMetcalfe, Christopher [0000-0002-8790-2722]
dc.contributor.orcidMuzaffar, Sayed [0000-0003-3065-0269]
dc.contributor.orcidMonksfield, Peter [0000-0001-7343-7105]
dc.contributor.orcidBance, Manohar [0000-0001-8050-3617]
dc.identifier.eissn2378-8038
cam.issuedOnline2022-02-08


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Attribution-NonCommercial-NoDerivatives 4.0 International
Except where otherwise noted, this item's licence is described as Attribution-NonCommercial-NoDerivatives 4.0 International