Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review
Background and Purpose: Cognitive impairment has been observed in patients with bilateral vestibular loss (BVL) and in patients with sensorineural hearing loss (SNHL). DFNA9 is an autosomal dominant disorder that causes a combination of both sensory deficits by the 3rd to 5th decade. We therefore hy...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-01-01
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| Series: | Frontiers in Neuroscience |
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| Online Access: | http://journal.frontiersin.org/article/10.3389/fnins.2017.00735/full |
| _version_ | 1811268741315428352 |
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| author | Jonas De Belder Stijn Matthysen Annes J. Claes Annes J. Claes Griet Mertens Griet Mertens Paul Van de Heyning Paul Van de Heyning Vincent Van Rompaey Vincent Van Rompaey |
| author_facet | Jonas De Belder Stijn Matthysen Annes J. Claes Annes J. Claes Griet Mertens Griet Mertens Paul Van de Heyning Paul Van de Heyning Vincent Van Rompaey Vincent Van Rompaey |
| author_sort | Jonas De Belder |
| collection | DOAJ |
| description | Background and Purpose: Cognitive impairment has been observed in patients with bilateral vestibular loss (BVL) and in patients with sensorineural hearing loss (SNHL). DFNA9 is an autosomal dominant disorder that causes a combination of both sensory deficits by the 3rd to 5th decade. We therefore hypothesize a combined detrimental effect on cognition. The aim of this systematic review was to identify studies related to DFNA9 in general and its relationship with cognitive impairment more specifically.Materials and Methods: Several databases including Medline, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, ISI Web of Knowledge, and Web of Science were searched to accumulate information about DFNA9-mutations, including phenotype, genotype, pathophysiology, quality of life (QOL), and imaging in general and cognitive function more specifically. A qualitative analysis was performed on the 55 articles that qualified.Results: The clinical features of DFNA9 are different along the 24 COCH mutations, described up to now. Vestibular symptoms generally present themselves a few years after SNHL onset in mutations associated with the vWFA-domain although they can precede SNHL onset in other mutations associated with the LCCL-domain. QoL has not been studied extensively in DFNA9, although scarce work is available on the positive impact of cochlear implantation to rehabilitate hearing. No studies were found evaluating cognition in DFNA9 patients.Conclusion: Although cognitive impairment has been demonstrated in patients with hearing loss as well as in patients with BVL, no studies have been reported on the combination of both sensory deficits, such as in DFNA9. Further research is warranted to correlate otovestibular status with cognition. |
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| issn | 1662-453X |
| language | English |
| last_indexed | 2024-04-12T21:28:21Z |
| publishDate | 2018-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neuroscience |
| spelling | doaj.art-b98c78c9e33c4551b82d1b54bda9ca6a2022-12-22T03:16:07ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2018-01-011110.3389/fnins.2017.00735280120Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic ReviewJonas De Belder0Stijn Matthysen1Annes J. Claes2Annes J. Claes3Griet Mertens4Griet Mertens5Paul Van de Heyning6Paul Van de Heyning7Vincent Van Rompaey8Vincent Van Rompaey9Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumFaculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumFaculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumFaculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumFaculty of Medicine and Health Sciences, University of Antwerp, Antwerp, BelgiumDepartment of Otorhinolaryngology and Head and Neck Surgery, Antwerp University Hospital, Edegem, BelgiumBackground and Purpose: Cognitive impairment has been observed in patients with bilateral vestibular loss (BVL) and in patients with sensorineural hearing loss (SNHL). DFNA9 is an autosomal dominant disorder that causes a combination of both sensory deficits by the 3rd to 5th decade. We therefore hypothesize a combined detrimental effect on cognition. The aim of this systematic review was to identify studies related to DFNA9 in general and its relationship with cognitive impairment more specifically.Materials and Methods: Several databases including Medline, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, ISI Web of Knowledge, and Web of Science were searched to accumulate information about DFNA9-mutations, including phenotype, genotype, pathophysiology, quality of life (QOL), and imaging in general and cognitive function more specifically. A qualitative analysis was performed on the 55 articles that qualified.Results: The clinical features of DFNA9 are different along the 24 COCH mutations, described up to now. Vestibular symptoms generally present themselves a few years after SNHL onset in mutations associated with the vWFA-domain although they can precede SNHL onset in other mutations associated with the LCCL-domain. QoL has not been studied extensively in DFNA9, although scarce work is available on the positive impact of cochlear implantation to rehabilitate hearing. No studies were found evaluating cognition in DFNA9 patients.Conclusion: Although cognitive impairment has been demonstrated in patients with hearing loss as well as in patients with BVL, no studies have been reported on the combination of both sensory deficits, such as in DFNA9. Further research is warranted to correlate otovestibular status with cognition.http://journal.frontiersin.org/article/10.3389/fnins.2017.00735/fullDFNA9cognitionsystematic reviewquality of lifelabyrinth diseases |
| spellingShingle | Jonas De Belder Stijn Matthysen Annes J. Claes Annes J. Claes Griet Mertens Griet Mertens Paul Van de Heyning Paul Van de Heyning Vincent Van Rompaey Vincent Van Rompaey Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review Frontiers in Neuroscience DFNA9 cognition systematic review quality of life labyrinth diseases |
| title | Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review |
| title_full | Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review |
| title_fullStr | Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review |
| title_full_unstemmed | Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review |
| title_short | Does Otovestibular Loss in the Autosomal Dominant Disorder DFNA9 Have an Impact of on Cognition? A Systematic Review |
| title_sort | does otovestibular loss in the autosomal dominant disorder dfna9 have an impact of on cognition a systematic review |
| topic | DFNA9 cognition systematic review quality of life labyrinth diseases |
| url | http://journal.frontiersin.org/article/10.3389/fnins.2017.00735/full |
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