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However, auditory neural dysfunction, its perceptual consequences and implications for patient management are not established. Similarly, the association with vestibular impairment has not yet been explored. This case\u2013control study investigated in 12 adults with genetically confirmed m.3243A&amp;gt;G adults [9 females; 45.5 \u00b1 16.3 years (range 18\u201366); 47.1 \u00b1 21.5 hearing level, dB] compared with 12 age, sex and hearing level-matched controls with sensory (cochlear level) hearing loss [9 females; 46.6 \u00b1 11.8 years (range 23\u201359); 47.7 \u00b1 25.4 hearing level, dB]. Participants underwent a battery of electroacoustic, electrophysiologic and perceptual tests, which included pure tone audiometry, otoacoustic emissions, auditory brainstem responses, auditory temporal processing measures, monaural\/binaural speech perception, balance and vestibular testing and self-reported questionnaires (dizziness and hearing disability). Our findings showed evidence of auditory neural abnormality and perceptual deficits greater than expected for cochlear pathology. Compared with matched controls with sensory hearing loss, adults with mitochondrial disease harbouring m.3243A&amp;gt;G had abnormal electrophysiologic responses from the VIII nerve and auditory brainstem (P = 0.005), an impaired capacity to encode rapidly occurring acoustic signal changes (P = 0.005), a reduced ability to localize sound sources (P = 0.028) and impaired speech perception in background noise (P = 0.008). Additionally, vestibular dysfunction (P = 0.011), greater perceived dizziness (P = 0.001) and reduced stance time (balance, P = 0.009) were also seen in participants with m.3243A&amp;gt;G mitochondrial disease when compared with matched counterparts. This pilot study revealed that auditory evaluation including evoked potential responses from the auditory nerve\/brainstem and speech perception in noise tests should form an important part of the management for individuals with m.3243A&amp;gt;G-related mitochondrial disease. Those presenting with hearing impairment and symptoms concerning balance and dizziness should undergo vestibular testing and appropriate management.<\/jats:p>","DOI":"10.1093\/braincomms\/fcae361","type":"journal-article","created":{"date-parts":[[2024,10,14]],"date-time":"2024-10-14T08:20:37Z","timestamp":1728894037000},"source":"Crossref","is-referenced-by-count":0,"title":["Auditory and vestibular function in mitochondrial patients harbouring the m.3243A&amp;gt;G variant"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6402-6435","authenticated-orcid":false,"given":"Renae J","family":"Stefanetti","sequence":"first","affiliation":[{"name":"Faculty of Medical Sciences, Wellcome Centre for Mitochondrial Research, Translational and Clinical Research Institute, Newcastle University , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"National Institute for Health and Care Research Newcastle Biomedical Research Centre, The Medical School , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"NHS Highly Specialised Service for Rare Mitochondrial Disorders of Adults and Children, Newcastle upon Tyne Hospitals NHS Foundation Trust , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane","family":"Newman","sequence":"additional","affiliation":[{"name":"Faculty of Medical Sciences, Wellcome Centre for Mitochondrial Research, Translational and Clinical Research Institute, Newcastle University , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"National Institute for Health and Care Research Newcastle Biomedical Research Centre, The Medical School , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"NHS Highly Specialised Service for Rare Mitochondrial Disorders of Adults and Children, Newcastle upon Tyne Hospitals NHS Foundation Trust , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alasdair P","family":"Blain","sequence":"additional","affiliation":[{"name":"Faculty of Medical Sciences, Wellcome Centre for Mitochondrial Research, Translational and Clinical Research Institute, Newcastle University , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"National Institute for Health and Care Research Newcastle Biomedical Research Centre, The Medical School , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donella","family":"Chisari","sequence":"additional","affiliation":[{"name":"Department of Audiology & Speech Pathology, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne , Parkville, VIC 3010 ,","place":["Australia"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gr\u00e1inne S","family":"Gorman","sequence":"additional","affiliation":[{"name":"Faculty of Medical Sciences, Wellcome Centre for Mitochondrial Research, Translational and Clinical Research Institute, Newcastle University , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"National Institute for Health and Care Research Newcastle Biomedical Research Centre, The Medical School , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]},{"name":"NHS Highly Specialised Service for Rare Mitochondrial Disorders of Adults and Children, Newcastle upon Tyne Hospitals NHS Foundation Trust , Newcastle upon Tyne, NE2 4HH ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gary","family":"Rance","sequence":"additional","affiliation":[{"name":"Department of Audiology & Speech Pathology, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne , Parkville, VIC 3010 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