{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T03:00:06Z","timestamp":1760238006267,"version":"build-2065373602"},"reference-count":45,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2022,5,30]],"date-time":"2022-05-30T00:00:00Z","timestamp":1653868800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background: Children with non-syndromic hereditary sensorineural hearing loss (SNHL) provide an opportunity to explore the impact of hearing on brain development. Objective: This study investigates volumetric differences of key hearing-related structures in children with gap junction protein beta 2 GJB2-related SNHL compared to controls. Materials and methods: Ninety-four children with SNHL (n = 15) or normal hearing (n = 79) were studied using automated volumetric segmentation. Heschl\u2019s gyrus (HG), anterior HG (aHG), planum temporale (PT), medial geniculate nucleus (MGN), and nucleus accumbens (NA) were analyzed relative to total brain volume (TBV) at two different age groups: (1) 7\u201312 months and (2) 13 months\u201318 years. Two-sided t-tests were used to evaluate differences between groups. Differences were considered significant if p &lt; 0.007. Results: Significantly smaller aHG-to-TBV ratios were found in 13-month-to-18-year-old patients (p &lt; 0.0055). HG-, PT-, MGN-, and NA-to-TBV ratios were smaller in the same age group, without reaching a significant level. Conversely, HG- and NA-to-TBV were larger in the younger age group. No significant differences were found between the groups for age and TBV. Conclusions: In this exploratory volumetric analysis of key hearing-related structures, we observed age-related changes in volume in children with GJB2-related SNHL.<\/jats:p>","DOI":"10.3390\/children9060800","type":"journal-article","created":{"date-parts":[[2022,5,30]],"date-time":"2022-05-30T10:05:14Z","timestamp":1653905114000},"page":"800","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Volumetric Analysis of Hearing-Related Structures of Brain in Children with GJB2-Related Congenital Deafness"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6501-839X","authenticated-orcid":false,"given":"Matthias W.","family":"Wagner","sequence":"first","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5S 1A1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sharon L.","family":"Cushing","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head & Neck Surgery, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Otolaryngology, Head & Neck Surgery, University of Toronto, Toronto, ON M5S 1A1, Canada"},{"name":"Archie\u2019s Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makabongwe","family":"Tshuma","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5S 1A1, Canada"},{"name":"Imaging Department, British Columbia Children\u2019s Hospital, Vancouver, BC V6H 3N1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen A.","family":"Gordon","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology, Head & Neck Surgery, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Otolaryngology, Head & Neck Surgery, University of Toronto, Toronto, ON M5S 1A1, Canada"},{"name":"Archie\u2019s Cochlear Implant Laboratory, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Communications Disorders, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Birgit B.","family":"Ertl-Wagner","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5S 1A1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Logi","family":"Vidarsson","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, ON M5G 1X8, Canada"},{"name":"Department of Medical Imaging, University of Toronto, Toronto, ON M5S 1A1, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,5,30]]},"reference":[{"key":"ref_1","first-page":"S70","article-title":"Newborn hearing screening programs: Overview","volume":"34","author":"Hyde","year":"2005","journal-title":"J. Otolaryngol."},{"key":"ref_2","unstructured":"Shearer, A.E., Hildebrand, M.S., and Smith, R.J. (2021, May 12). Hereditary Hearing Loss and Deafness Overview. GeneReviews\u00ae[Internet], Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1434\/."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1016\/j.mjafi.2015.07.003","article-title":"Genetics of non syndromic hearing loss","volume":"71","author":"Venkatesh","year":"2015","journal-title":"Med. J. Armed Forces India"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"16094","DOI":"10.1038\/nrdp.2016.94","article-title":"Congenital hearing loss","volume":"3","author":"Korver","year":"2017","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"12638","DOI":"10.1523\/JNEUROSCI.2559-11.2011","article-title":"Hearing loss in older adults affects neural systems supporting speech comprehension","volume":"31","author":"Peelle","year":"2011","journal-title":"J. Neurosci."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1007\/s10162-012-0332-5","article-title":"Auditory cortex signs of age-related hearing loss","volume":"13","author":"Eckert","year":"2012","journal-title":"J. Assoc. Res. Otolaryngol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.neuroscience.2013.12.010","article-title":"Diffusion tensor imaging and MR morphometry of the central auditory pathway and auditory cortex in aging","volume":"260","author":"Profant","year":"2014","journal-title":"Neuroscience"},{"key":"ref_8","doi-asserted-by":"crossref","unstructured":"Rigters, S.C., Bos, D., Metselaar, M., Roshchupkin, G.V., Baatenburg de Jong, R.J., Ikram, M.A., Vernooij, M.W., and Goedegebure, A. (2017). Hearing Impairment Is Associated with Smaller Brain Volume in Aging. Front. Aging Neurosci., 9.","DOI":"10.3389\/fnagi.2017.00002"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"107823","DOI":"10.1016\/j.heares.2019.107823","article-title":"Cortical thickness of left Heschl\u2019s gyrus correlates with hearing acuity in adults\u2014A surface-based morphometry study","volume":"384","author":"Neuschwander","year":"2019","journal-title":"Hear Res."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"597","DOI":"10.3389\/fnins.2019.00597","article-title":"Assessing Cerebral White Matter Microstructure in Children with Congenital Sensorineural Hearing Loss: A Tract-Based Spatial Statistics Study","volume":"13","author":"Jiang","year":"2019","journal-title":"Front. Neurosci."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"10049","DOI":"10.1073\/pnas.1730169100","article-title":"A morphometric analysis of auditory brain regions in congenitally deaf adults","volume":"100","author":"Emmorey","year":"2003","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1093\/cercor\/bhq164","article-title":"Morphometric differences in the Heschl\u2019s gyrus of hearing impaired and normal hearing infants","volume":"21","author":"Smith","year":"2011","journal-title":"Cereb. Cortex"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.brainres.2011.09.057","article-title":"Cortical thickness analysis and optimized voxel-based morphometry in children and adolescents with prelingually profound sensorineural hearing loss","volume":"1430","author":"Li","year":"2012","journal-title":"Brain Res."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1","DOI":"10.13104\/imri.2015.19.1.1","article-title":"Reduced gray matter volume of auditory cortical and subcortical areas in congenitally deaf adolescents: A voxel-based morphometric study","volume":"19","author":"Tae","year":"2015","journal-title":"Investig. Magn. Reson. Imaging"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"E1022","DOI":"10.1073\/pnas.1717603115","article-title":"Neural preservation underlies speech improvement from auditory deprivation in young cochlear implant recipients","volume":"115","author":"Feng","year":"2018","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.ijdevneu.2019.05.009","article-title":"The left lateral occipital cortex exhibits decreased thickness in children with sensorineural hearing loss","volume":"76","author":"Shiohama","year":"2019","journal-title":"Int. J. Dev. Neurosci."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"565445","DOI":"10.3389\/fnhum.2020.565445","article-title":"Alteration of Cortical and Subcortical Structures in Children with Profound Sensorineural Hearing Loss","volume":"14","author":"Qu","year":"2020","journal-title":"Front. Hum. Neurosci."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1038\/s41467-019-08350-7","article-title":"Medial geniculate body and primary auditory cortex differentially contribute to striatal sound representations","volume":"10","author":"Chen","year":"2019","journal-title":"Nat. Commun."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/S0165-0173(98)00047-2","article-title":"The planum temporale: A systematic, quantitative review of its structural, functional and clinical significance","volume":"29","author":"Shapleske","year":"1999","journal-title":"Brain Res. Brain Res. Rev."},{"key":"ref_20","first-page":"493","article-title":"Uses and abuses of hearing loss classification","volume":"23","author":"Clark","year":"1981","journal-title":"Asha"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1006\/nimg.1998.0395","article-title":"Cortical surface-based analysis: I. Segmentation and surface reconstruction","volume":"9","author":"Dale","year":"1999","journal-title":"Neuroimage"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1006\/nimg.1998.0396","article-title":"Cortical surface-based analysis: II: Inflation, flattening, and a surface-based coordinate system","volume":"9","author":"Fischl","year":"1999","journal-title":"Neuroimage"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"3887","DOI":"10.1038\/s41598-020-60609-y","article-title":"TASH: Toolbox for the Automated Segmentation of Heschl\u2019s gyrus","volume":"10","author":"Schneider","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"116946","DOI":"10.1016\/j.neuroimage.2020.116946","article-title":"Infant FreeSurfer: An automated segmentation and surface extraction pipeline for T1-weighted neuroimaging data of infants 0\u20132 years","volume":"218","author":"Iglesias","year":"2020","journal-title":"Neuroimage"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"143","DOI":"10.3389\/fnhum.2020.00143","article-title":"Investigating the Added Value of FreeSurfer\u2019s Manual Editing Procedure for the Study of the Reading Network in a Pediatric Population","volume":"14","author":"Beelen","year":"2020","journal-title":"Front. Hum. Neurosci."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1016\/j.neuroimage.2006.01.021","article-title":"An automated labeling system for subdividing the human cerebral cortex on MRI scans into gyral based regions of interest","volume":"31","author":"Desikan","year":"2006","journal-title":"Neuroimage"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.neuroimage.2010.06.010","article-title":"Automatic parcellation of human cortical gyri and sulci using standard anatomical nomenclature","volume":"53","author":"Destrieux","year":"2010","journal-title":"Neuroimage"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"171","DOI":"10.3389\/fnins.2012.00171","article-title":"101 Labeled Brain Images and a Consistent Human Cortical Labeling Protocol","volume":"6","author":"Klein","year":"2012","journal-title":"Front. Neurosci."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1016\/j.neuroimage.2012.01.021","article-title":"FreeSurfer","volume":"62","author":"Fischl","year":"2012","journal-title":"Neuroimage"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"S69","DOI":"10.1016\/j.neuroimage.2004.07.016","article-title":"Sequence-independent segmentation of magnetic resonance images","volume":"23","author":"Fischl","year":"2004","journal-title":"Neuroimage"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/S0896-6273(02)00569-X","article-title":"Whole brain segmentation: Automated labeling of neuroanatomical structures in the human brain","volume":"33","author":"Fischl","year":"2002","journal-title":"Neuron"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1016\/j.neuroimage.2018.08.012","article-title":"A probabilistic atlas of the human thalamic nuclei combining ex vivo MRI and histology","volume":"183","author":"Iglesias","year":"2018","journal-title":"Neuroimage"},{"key":"ref_33","unstructured":"Bonferroni, C. (1936). Teoria Statistica delle Classi e Calcolo delle Probabilita, Pubblicazioni del R Istituto Superiore di Scienze Economiche e Commericiali di Firenze. Available online: https:\/\/methods.sagepub.com\/reference\/encyc-of-research-design\/n455.xml."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"988","DOI":"10.2106\/00004623-199307000-00004","article-title":"Acute fractures and dislocations of the cervical spine in children and adolescents","volume":"75","author":"McGrory","year":"1993","journal-title":"J. Bone Jt. Surg. Am."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"8174","DOI":"10.1073\/pnas.0402680101","article-title":"Dynamic mapping of human cortical development during childhood through early adulthood","volume":"101","author":"Gogtay","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1093\/cercor\/bhh156","article-title":"Postnatal cortical development in congenital auditory deprivation","volume":"15","author":"Kral","year":"2005","journal-title":"Cereb. Cortex"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1002\/(SICI)1096-9861(19971020)387:2<167::AID-CNE1>3.0.CO;2-Z","article-title":"Regional differences in synaptogenesis in human cerebral cortex","volume":"387","author":"Huttenlocher","year":"1997","journal-title":"J. Comp. Neurol."},{"key":"ref_38","unstructured":"Bourgeois, J.-P., Goldman-Rakic, P.S., and Rakic, P. (2000). Formation, elimination and stabilization of synapses in the primate cerebral cortex. The New Cognitive Neurosciences, MIT Press."},{"key":"ref_39","unstructured":"Smith, R.J., and Jones, M.K.N. (2021, May 31). Nonsyndromic Hearing Loss and Deafness, DFNB1. GeneReviews\u00ae[Internet], Available online: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1272\/."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"E34","DOI":"10.1002\/lary.24332","article-title":"GJB2-associated hearing loss: Systematic review of worldwide prevalence, genotype, and auditory phenotype","volume":"124","author":"Chan","year":"2014","journal-title":"Laryngoscope"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1002\/biof.150","article-title":"Ca2+ homeostasis defects and hereditary hearing loss","volume":"37","author":"Mammano","year":"2011","journal-title":"Biofactors"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.brainres.2009.02.008","article-title":"Diverse deafness mechanisms of connexin mutations revealed by studies using in vitro approaches and mouse models","volume":"1277","author":"Dinh","year":"2009","journal-title":"Brain Res."},{"key":"ref_43","first-page":"1","article-title":"Alterations of grey matter asymmetries in adolescents with prelingual deafness: A combined VBM and cortical thickness analysis","volume":"31","author":"Li","year":"2013","journal-title":"Restor. Neurol. Neurosci."},{"key":"ref_44","doi-asserted-by":"crossref","unstructured":"Heinen, R., Bouvy, W.H., Mendrik, A.M., Viergever, M.A., Biessels, G.J., and de Bresser, J. (2016). Robustness of Automated Methods for Brain Volume Measurements across Different MRI Field Strengths. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0165719"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1016\/j.neuroimage.2008.10.037","article-title":"Reliability of MRI-derived cortical and subcortical morphometric measures: Effects of pulse sequence, voxel geometry, and parallel imaging","volume":"44","author":"Wonderlick","year":"2009","journal-title":"Neuroimage"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/6\/800\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:21:53Z","timestamp":1760138513000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/9\/6\/800"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,5,30]]},"references-count":45,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2022,6]]}},"alternative-id":["children9060800"],"URL":"https:\/\/doi.org\/10.3390\/children9060800","relation":{},"ISSN":["2227-9067"],"issn-type":[{"type":"electronic","value":"2227-9067"}],"subject":[],"published":{"date-parts":[[2022,5,30]]}}}