{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T22:29:12Z","timestamp":1782512952087,"version":"3.54.5"},"reference-count":34,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2024,12,2]],"date-time":"2024-12-02T00:00:00Z","timestamp":1733097600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Graeme Clark Chair in Audiology &amp; Speech Science"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Chiari malformation is a condition involving caudal descent of the hindbrain which herniates the cerebellar tonsils through the foramen magnum. The purpose of this study was to quantify auditory deficits in an affected individual and to explore the hypothesis that cerebellar malformation specifically disrupts binaural processing. Methods: We present audiometric, electrophysiologic, imaging and auditory perceptual findings for a 17-year-old female with Chiari 1 malformation and for a cohort of 35 hearing- and age-matched controls. Results: The patient presented with auditory deficit consistent with cerebellar disorder\u2014that is, an impaired ability to judge the duration of auditory stimuli. In addition, she showed evidence of abnormal binaural processing affecting her capacity to localise sound sources to optimise speech perception in background noise. The provision of a remote microphone listening device was successful in improving her perceptual ability to normal levels. Conclusions: Despite normal sound detection ability, this child with Chiari 1 malformation suffered functional hearing deficits severe enough to impact everyday listening\/communication and educational progress. These limitations were ameliorated through auditory intervention.<\/jats:p>","DOI":"10.3390\/jcm13237311","type":"journal-article","created":{"date-parts":[[2024,12,3]],"date-time":"2024-12-03T11:48:56Z","timestamp":1733226536000},"page":"7311","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Binaural Processing Deficits in a Child with Chiari Malformation (Type 1)"],"prefix":"10.3390","volume":"13","author":[{"given":"Gary","family":"Rance","sequence":"first","affiliation":[{"name":"Department of Audiology and Speech Pathology, The University of Melbourne, Parkville VIC 3052, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6540-3765","authenticated-orcid":false,"given":"Julien","family":"Zanin","sequence":"additional","affiliation":[{"name":"Department of Audiology and Speech Pathology, The University of Melbourne, Parkville VIC 3052, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,12,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1023\/A:1023948921381","article-title":"Chiari type I malformation with or without syringomyelia: Prevalence and genetics","volume":"12","author":"Speer","year":"2003","journal-title":"J. Genet. Couns."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"Goldschagg, N., Feil, K., Ihl, F., Krafczyk, S., Kunz, M., Tonn, J.C., Strupp, M., and Peraud, A. (2017). Decompression in Chiari malformation: Clinical, ocular motor, cerebellar, and vestibular outcome. Front. Neurol., 8.","DOI":"10.3389\/fneur.2017.00292"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"74","DOI":"10.5606\/tftrd.2019.2003","article-title":"Posturographic examination of body balance in patients with Chiari type I malformation and correlation with the presence of syringomyelia and degree of cerebellar ectopia","volume":"65","author":"Palamar","year":"2019","journal-title":"Turk. J. Phys. Med. Rehabil."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Famili, H.P., Zalewski, C.K., Ibrahimy, A., Mack, J., Cantor, F., Heiss, J.D., and Brewer, C.C. (2023). Audiovestibular Findings in a Cohort of Patients with Chiari Malformation Type I and Dizziness. J. Clin. Med., 12.","DOI":"10.3390\/jcm12082767"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1097\/00129492-200109000-00020","article-title":"Otologic manifestations of Chiari I malformation","volume":"22","author":"Sperling","year":"2001","journal-title":"Otol. Neurotol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1017\/S0022215100123370","article-title":"Otoneurological manifestations in Chiari-I malformation","volume":"107","author":"Albers","year":"1993","journal-title":"J. Laryngol. Otol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1152\/jn.1944.7.6.331","article-title":"Receiving areas of the tactile, auditory, and visual systems in the cerebellum","volume":"7","author":"Snider","year":"1944","journal-title":"J. Neurophysiol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1007\/BF00234933","article-title":"Electrical responses of the auditory area of the cerebellar cortex to acoustic stimulation","volume":"26","author":"Altman","year":"1976","journal-title":"Exp. Brain Res."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1162\/jocn.1989.1.2.136","article-title":"Timing functions of the cerebellum","volume":"1","author":"Ivry","year":"1989","journal-title":"J. Cogn. Neurosci."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e67303","DOI":"10.7554\/eLife.67303","article-title":"Temporo-cerebellar connectivity underlies timing constraints in audition","volume":"10","author":"Stockert","year":"2021","journal-title":"Elife"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1111\/j.1749-6632.2002.tb07576.x","article-title":"The cerebellum and event timing","volume":"978","author":"Ivry","year":"2002","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1038\/nrn1764","article-title":"What makes us tick? Functional and neural mechanisms of interval timing","volume":"6","author":"Buhusi","year":"2005","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1159\/000255341","article-title":"Auditory perception in individuals with Friedreich\u2019s ataxia","volume":"15","author":"Rance","year":"2010","journal-title":"Audiol. Neurotol."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Kim, S.-G., Mueller, K., Lepsien, J., Mildner, T., and Fritz, T.H. (2019). Brain networks underlying aesthetic appreciation as modulated by interaction of the spectral and temporal organisations of music. Sci. Rep., 9.","DOI":"10.1038\/s41598-019-55781-9"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1412","DOI":"10.1093\/brain\/aws085","article-title":"Auditory function in children with Charcot\u2013Marie\u2013Tooth disease","volume":"135","author":"Rance","year":"2012","journal-title":"Brain"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1093\/brain\/119.3.741","article-title":"Auditory neuropathy","volume":"119","author":"Starr","year":"1996","journal-title":"Brain"},{"key":"ref_17","unstructured":"Pratt, S., and Lightfoot, G. (2012). Physiological mechanisms underlying MLRs and cortical EPs. Translational Perspectives in Auditory Neuroscience: Hearing Across the Life Span\u2014Assessment and Disorders, Plural Publishing, Inc."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e2136842","DOI":"10.1001\/jamanetworkopen.2021.36842","article-title":"Auditory dysfunction among individuals with neurofibromatosis type 1","volume":"4","author":"Rance","year":"2021","journal-title":"JAMA Netw. Open"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"377","DOI":"10.3766\/jaaa.19.5.2","article-title":"The Listening in Spatialized Noise\u2013Sentences Test (LISN-S): Comparison to the prototype LISN and results from children with either a suspected (central) auditory processing disorder or a confirmed language disorder","volume":"19","author":"Cameron","year":"2008","journal-title":"J. Am. Acad. Audiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1080\/14992020400050014","article-title":"The Speech, Spatial and Qualities of Hearing Scale (SSQ)","volume":"43","author":"Gatehouse","year":"2004","journal-title":"Int. J. Audiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1177\/0883073812448963","article-title":"Auditory processing deficits in children with Friedreich ataxia","volume":"27","author":"Rance","year":"2012","journal-title":"J. Child Neurol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1006\/brln.1997.1826","article-title":"Categorical speech perception in cerebellar disorders","volume":"60","author":"Ackermann","year":"1997","journal-title":"Brain Lang."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"3050","DOI":"10.1152\/jn.00985.2004","article-title":"Perceptual consequences of disrupted auditory nerve activity","volume":"93","author":"Zeng","year":"2005","journal-title":"J. Neurophysiol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1808","DOI":"10.1016\/j.cortex.2012.11.012","article-title":"Fundamental deficits of auditory perception in Wernicke\u2019s aphasia","volume":"49","author":"Robson","year":"2013","journal-title":"Cortex"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.heares.2007.01.007","article-title":"The role of auditory cortex in the formation of auditory streams","volume":"229","author":"Micheyl","year":"2007","journal-title":"Hear. Res."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/S0378-5955(01)00362-8","article-title":"Auditory brain stem responses evoked by lateralized clicks: Is lateralization extracted in the human brain stem?","volume":"163","author":"Riedel","year":"2002","journal-title":"Hear. Res."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1309","DOI":"10.1152\/jn.00901.2005","article-title":"Sensitivity to interaural time differences in the dorsal nucleus of the lateral lemniscus of the unanesthetized rabbit: Comparison with other structures","volume":"95","author":"Kuwada","year":"2006","journal-title":"J. Neurophysiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/0006-8993(82)90897-6","article-title":"Projections from the cochlear nucleus to the cerebellum","volume":"244","author":"Huang","year":"1982","journal-title":"Brain Res."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1152\/physrev.1964.44.3.432","article-title":"Teleceptive components of the cerebellar function","volume":"44","author":"Fadiga","year":"1964","journal-title":"Physiol. Rev."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/0031-9384(71)90091-6","article-title":"Behavior after cerebellar lesions in cats and monkeys","volume":"6","author":"Peters","year":"1971","journal-title":"Physiol. Behav."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1121\/1.2812596","article-title":"The effects of environmental and classroom noise on the academic attainments of primary school children","volume":"123","author":"Shield","year":"2008","journal-title":"J. Acoust. Soc. Am."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"5415","DOI":"10.1007\/s00405-022-07445-0","article-title":"Extended wear hearing aids: A comparative, pilot study","volume":"11","author":"Gazia","year":"2022","journal-title":"Eur Arch Otorhinolaryngol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.heares.2015.07.006","article-title":"Central auditory processing in patients with spinocerebellar ataxia","volume":"327","author":"Zeigelboim","year":"2015","journal-title":"Hear. Res."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1007\/s12311-023-01519-3","article-title":"Altered binaural hearing in pre-ataxic and ataxic mutation carriers of spinocerebellar ataxia type 3","volume":"23","author":"Jacobi","year":"2024","journal-title":"Cerebellum"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/23\/7311\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:44:30Z","timestamp":1760114670000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/23\/7311"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,2]]},"references-count":34,"journal-issue":{"issue":"23","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["jcm13237311"],"URL":"https:\/\/doi.org\/10.3390\/jcm13237311","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,2]]}}}