title: Effect of Sensorineural Hearing Loss on Neurocognitive Functioning in Pediatric Brain Tumor Survivors.
abstract: BACKGROUND
  Intensified therapy with platinum-based regimens for pediatric brain tumors has dramatically increased the number of pediatric brain tumor survivors (PBTS) but frequently causes permanent sensorineural hearing loss (SNHL). Although neurocognitive decline in PBTS is known to be associated with radiation therapy (RT), SNHL represents a potential additional contributor whose long-term impact has yet to be fully determined.
  
  
  METHODS
  The neurocognitive impact of significant SNHL (Chang scale ≥ 2b) in PBTS was assessed through a retrospective cohort study of audiograms and neurocognitive testing. Scores for neurocognitive domains and subtest task performance were analyzed to identify specific strengths and weakness for PBTS with SNHL.
  
  
  RESULTS
  In a cohort of PBTS (n = 58) treated with platinum therapy, significant SNHL was identified in more than half (55%, n = 32/58), of which the majority required hearing aids (72%, 23/32). RT exposure was approximately evenly divided between those with and without SNHL. PBTS were 6.7 ± 0.6 and 11.3 ± 0.7 years old at diagnosis and neurocognitive testing, respectively. In multivariate analyses adjusted for RT dose, SNHL was independently associated with deficits in intelligence, executive function, and verbal reasoning skills. Subtests revealed PBTS with SNHL to have poor learning efficiency but intact memory and information acquisition.
  
  
  CONCLUSIONS
  SNHL in PBTS increases the risk for severe therapy-related intellectual and neurocognitive deficits. Additional prospective investigation in malignant brain tumors is necessary to validate these findings through integration of audiology and neurocognitive assessments and to identify appropriate strategies for neurocognitive screening and rehabilitation specific to PBTS with and without SNHL.
authors.affiliation: Jonathan Jaques Children's Cancer Center, Miller Children's Hospital Long Beach, Long Beach, California.|Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA.|Keck School of Medicine of University of Southern California, Los Angeles, California.
authors.forename: Etan
authors.initials: E
authors.lastname: Orgel
authors.affiliation: Keck School of Medicine of University of Southern California, Los Angeles, California.|Division of Neurology, Children's Hospital Los Angeles, Los Angeles, CA.|Clinical Translational Science Institute, Children's Hospital Los Angeles, Los Angeles, CA.
authors.forename: Sharon H
authors.initials: SH
authors.lastname: O'Neil
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA.|Keck School of Medicine of University of Southern California, Los Angeles, California.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Kayser
authors.affiliation: Division of Rehabilitative Medicine at the Children's Hospital Los Angeles, Los Angeles, California.
authors.forename: Bea
authors.initials: B
authors.lastname: Smith
authors.affiliation: Jonathan Jaques Children's Cancer Center, Miller Children's Hospital Long Beach, Long Beach, California.
authors.forename: Teddi L
authors.initials: TL
authors.lastname: Softley
authors.affiliation: Jonathan Jaques Children's Cancer Center, Miller Children's Hospital Long Beach, Long Beach, California.
authors.forename: Sandra
authors.initials: S
authors.lastname: Sherman-Bien
authors.affiliation: Jonathan Jaques Children's Cancer Center, Miller Children's Hospital Long Beach, Long Beach, California.
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Counts
authors.affiliation: Jonathan Jaques Children's Cancer Center, Miller Children's Hospital Long Beach, Long Beach, California.
authors.forename: Devin
authors.initials: D
authors.lastname: Murphy
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA.|Keck School of Medicine of University of Southern California, Los Angeles, California.
authors.forename: Girish
authors.initials: G
authors.lastname: Dhall
authors.affiliation: Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA.|Keck School of Medicine of University of Southern California, Los Angeles, California.
authors.forename: David R
authors.initials: DR
authors.lastname: Freyer
mesh_terms: D000124:Achievement; D001299:Audiometry; D001932:Brain Neoplasms / Q000150:complications / Q000188:drug therapy*; D002648:Child; D015331:Cohort Studies; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000139:chemically induced* / Q000503:physiopathology*; D006801:Humans; D007360:Intelligence; D008297:Male; D008568:Memory; D019965:Neurocognitive Disorders / Q000175:diagnosis* / Q000503:physiopathology; D009483:Neuropsychological Tests; D012189:Retrospective Studies
keywords: brain tumors; chemotherapy neurotoxicity; late effects; neuro-oncology; psychosocial