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dc.contributor.authorHanemann, Clemens Oliver
dc.contributor.authorEmmanouil, B
dc.contributor.authorHouston, R
dc.contributor.authorMay, A
dc.contributor.authorRamsden, JD
dc.contributor.authorHalliday, D
dc.contributor.authorParry, A
dc.contributor.authorMackeith, S
dc.date.accessioned2018-11-27T14:13:37Z
dc.date.issued2019-04
dc.identifier.issn0023-852X
dc.identifier.issn1531-4995
dc.identifier.urihttp://hdl.handle.net/10026.1/12913
dc.description.abstract

<jats:sec><jats:title>Objectives/Hypothesis</jats:title><jats:p>This study set out to describe the progression of hearing loss in patients with neurofibromatosis type 2 (NF2), treated in a quaternary multidisciplinary clinic. It also aimed to compare hearing loss across patients grouped according to a known genetic severity score to explore its utility for prognostication.</jats:p></jats:sec><jats:sec><jats:title>Study Design</jats:title><jats:p>Retrospective cohort study.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>We conducted a study of 147 patients with confirmed NF2 diagnosis for a mean observational period of 10 years. Pure‐tone average (PTA), optimum discriminations scores (ODS), and genotype data were collected. Patients were classified according to hearing class (American Academy of Otolaryngology), their candidacy for auditory implantation (UK National NF2 consensus) and grouped by genetic severity as: 1 = tissue mosaic, 2A = mild classic, 2B = moderate classic, and 3 = severe. Survival analysis investigated the effect of genetic severity on the age of loss of serviceable hearing.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Genetic severity was a significant predictor of hearing outcomes such as ODS, hearing classification, and maximum annual PTA deterioration. Although the overall median age of loss of serviceable hearing was 78 years, there was significant variation according to the genetic severity; the median for severe patients was 32 years compared to a median of 80 for tissue mosaic patients.</jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p>This is the first description of long‐term hearing outcomes in a clinical setting across a large heterogeneous cohort of patients with NF2. The results highlight the potential importance and benefit of considering the genetic severity score of patients when undertaking treatment decisions, as well as planning future natural history studies.</jats:p></jats:sec><jats:sec><jats:title>Level of Evidence</jats:title><jats:p>2c <jats:italic>Laryngoscope</jats:italic>, 129:974–980, 2019</jats:p></jats:sec>

dc.format.extent974-980
dc.format.mediumPrint-Electronic
dc.languageen
dc.language.isoen
dc.publisherWiley
dc.subjectNeurofibromatosis 2
dc.subjectgenetic severity
dc.subjectnatural history
dc.subjecthearing loss
dc.subjectacoustic neuroma
dc.subjectvestibular schwannoma
dc.titleProgression of Hearing Loss in Neurofibromatosis Type 2 According to Genetic Severity
dc.typejournal-article
dc.typeJournal Article
plymouth.author-urlhttps://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000462650400046&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=11bb513d99f797142bcfeffcc58ea008
plymouth.issue4
plymouth.volume129
plymouth.publication-statusPublished
plymouth.journalThe Laryngoscope
dc.identifier.doi10.1002/lary.27586
plymouth.organisational-group/Plymouth
plymouth.organisational-group/Plymouth/Faculty of Health
plymouth.organisational-group/Plymouth/Faculty of Health/Peninsula Medical School
plymouth.organisational-group/Plymouth/REF 2021 Researchers by UoA
plymouth.organisational-group/Plymouth/REF 2021 Researchers by UoA/UoA01 Clinical Medicine
plymouth.organisational-group/Plymouth/Research Groups
plymouth.organisational-group/Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED)
plymouth.organisational-group/Plymouth/Research Groups/Institute of Translational and Stratified Medicine (ITSMED)/CBR
plymouth.organisational-group/Plymouth/Users by role
plymouth.organisational-group/Plymouth/Users by role/Academics
plymouth.organisational-group/Plymouth/Users by role/Researchers in ResearchFish submission
dc.publisher.placeUnited States
dcterms.dateAccepted2018-08-29
dc.rights.embargodate2019-11-19
dc.identifier.eissn1531-4995
dc.rights.embargoperiodNot known
rioxxterms.versionofrecord10.1002/lary.27586
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserved
rioxxterms.licenseref.startdate2019-04
rioxxterms.typeJournal Article/Review


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