{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T05:27:40Z","timestamp":1793424460166,"version":"4.5.0"},"reference-count":51,"publisher":"Wiley","issue":"S11","license":[{"start":{"date-parts":[[2022,3,18]],"date-time":"2022-03-18T00:00:00Z","timestamp":1647561600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:sec>\n                    <jats:title>Objective\/Hypothesis<\/jats:title>\n                    <jats:p>Sensorineural hearing loss (SNHL) is a common sequela of congenital cytomegalovirus (cCMV), potentially exacerbating neurocognitive delay. The objectives of this study were to assess: (1) age at which SNHL in children with cCMV; (2) stimulability of the auditory system in children with cCMV following cochlear implantation (CI); and (3) whether features of magnetic resonance imaging (MRI) potentially are predictive of hearing outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>In this retrospective study of a prospectively acquired cohort, 123 children with cCMV who were referred for hearing loss at a single tertiary referral hospital over 20\u2009years were compared with an unmatched comparative group of 90 children with GJB2\u2010related deafness. Outcome measures were results of newborn hearing screening (NHS), behavioral audiograms, and, in a subgroup of cochlear implant (CI) users, responses from the auditory nerve and brainstem evoked by CI at initial activation, as well as lesional volume of FLAIR\u2010hyperintense signal alterations on MRI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All but 3 of 123 children with cCMV had confirmed and persistent SNHL. At birth, 113 children with cCMV underwent NHS, 31 (27%) passed in both ears and 23 (20%) passed in one ear (no NHS data in 10 children). At the first audiologic assessment, 32 of 123 (26%) had normal hearing bilaterally; 35 of 123 (28%) had unilateral SNHL; and 57 of 123 (46%) had bilateral SNHL. More than half (67 of 123, 54%) experienced hearing deterioration in at least one ear. Survival analyses suggested that 60% of children who developed SNHL did so by 2.5\u2009years and 80% by 5\u2009years. In the children who passed NHS in one or both ears, 50% developed hearing loss by 3.5\u2009years in the ear, which passed unilaterally (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a023 ears), and 50% by 5\u2009years in bilateral passes (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a062 ears). Hearing loss was significant enough in all but one child with isolated high\u2010frequency loss for rehabilitation to be indicated. Hearing thresholds in individual ears were in the CI range in 83% (102 of 123), although duration of deafness was sufficient to preclude implantation at our center in 13 children with unilateral SNHL. Hearing aids were indicated in 16% (20 of 123). Responses from the auditory nerve and brainstem to initial CI stimulation were similar in children with cCMV\u2010related SNHL compared with GJB2\u2010related SNHL. Characteristic white matter changes on MRI were seen in all children with cCMV\u2010related SNHL (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a091), but the lesion volume in each cortical hemisphere did not predict degree of SNHL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>cCMV\u2010related SNHL is often not detected by NHS but occurs with high prevalence in early childhood. Electrophysiological measures suggest equivalent stimulability of the auditory nerve and brainstem with CI in children with cCMV and GJB2\u2010related SNHL. Hyperintense white matter lesions on FLAIR MRI are consistently present in children with cCMV\u2010related SNHL but cannot be used to predict its time course or degree. Combined, the data show early and rapid deterioration of hearing in children with cCMV\u2010related SNHL with potential for good CI outcomes if SNHL is identified and managed without delay. Findings support universal newborn screening for cCMV followed by careful audiological monitoring.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Level of Evidence<\/jats:title>\n                    <jats:p>\n                      3\n                      <jats:italic>Laryngoscope<\/jats:italic>\n                      , 132:S1\u2013S24, 2022\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lary.30108","type":"journal-article","created":{"date-parts":[[2022,3,18]],"date-time":"2022-03-18T08:06:19Z","timestamp":1647590779000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Hearing Instability in Children with Congenital Cytomegalovirus: Evidence and Neural Consequences"],"prefix":"10.1002","volume":"132","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9226-4510","authenticated-orcid":false,"given":"Sharon L.","family":"Cushing","sequence":"first","affiliation":[{"name":"Department of Otolaryngology: Head &amp; Neck Surgery Hospital for Sick Children Toronto Ontario Canada"},{"name":"Department of Otolaryngology: Head &amp; Neck Surgery, Faculty of Medicine University of Toronto Toronto Ontario Canada"},{"name":"Archie's Cochlear Implant Laboratory Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia L.","family":"Purcell","sequence":"additional","affiliation":[{"name":"C.S. Mott Children's Hospital Michigan Medicine Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicky","family":"Papaiaonnou","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology: Head &amp; Neck Surgery Hospital for Sick Children Toronto Ontario Canada"},{"name":"Department of Otolaryngology: Head &amp; Neck Surgery, Faculty of Medicine University of Toronto Toronto Ontario Canada"},{"name":"Department of Communication Disorders Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jaina","family":"Neghandi","sequence":"additional","affiliation":[{"name":"Archie's Cochlear Implant Laboratory Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maya","family":"Daien","sequence":"additional","affiliation":[{"name":"Archie's Cochlear Implant Laboratory Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan I.","family":"Blaser","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology: Head &amp; Neck Surgery, Faculty of Medicine University of Toronto Toronto Ontario Canada"},{"name":"Department of Diagnostic Imaging, Division of Paediatric Neuroradiology Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birgit","family":"Ertl\u2010Wagner","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Paediatric Neuroradiology Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Wagner","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Paediatric Neuroradiology Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Sheng","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, Division of Paediatric Neuroradiology Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4125-4053","authenticated-orcid":false,"given":"Adrian L.","family":"James","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology: Head &amp; 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