title: Audiological chronological findings in children with congenital anomalies of the central nervous system.
abstract: OBJECTIVE
  To determine the frequency of hearing impairment in children with congenital anomalies of the central nervous system (CNS) by using detailed audiological evaluation methods.
  
  
  METHODS
  The patients were 78 children with congenital anomalies of the CNS with a mean age of 29.5 months. They had been observed for a mean period of 38.5 months. Hearing levels were evaluated behavioral observation audiometry (BOA), visual reinforcement audiometry (VRA) and distortion product otoacoustic emissions (DPOAEs) were performed. Auditory brainstem responses (ABRs) and computed tomography (CT) scans of the temporal bone were performed in the cases in which the minimum response levels (MRLs) were above 30 dBHL. All cases were assessed in terms of developmental age.
  
  
  RESULTS
  A total of 14.1% (11/78) of the children with congenital anomalies of the CNS were initially diagnosed with bilateral sensorineural hearing loss (SNHL). However, the hearing levels of nine of them improved by the time of the last diagnosis. Therefore, the patients with bilateral SNHL were only 2.6% (2/78) of the total patients with congenital anomalies of the CNS at last diagnoses. As shown by our results, many children with bilateral SNHL at initial diagnosis showed improved ABR thresholds and behavioral hearing thresholds with age. In this series, the use of hearing aids was arranged for six patients. However, four patients stopped using hearing aids when their hearing threshold levels improved. In two cases, there were no changes in hearing levels and the children continued using hearing aids.
  
  
  CONCLUSIONS
  Our results suggest that hearing level recovery can occur in some children with CNS anomalies. Confirmation of hearing loss in children with congenital anomalies of the CNS takes a long time. There are improvements in hearing loss during the observation period. Therefore periodic assessment of hearing is important.
authors.affiliation: Department of Otolaryngology, Hokkaido Medical Center for Child Health and Rehabilitation, Teine-ku, Sapporo, Japan. bxe03114@s9.dion.ne.jp
authors.forename: Kae
authors.initials: K
authors.lastname: Kitagawa
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Mitsuzawa
authors.forename: Tomoko
authors.initials: T
authors.lastname: Shintani
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Go
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Himi
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001299:Audiometry; D001921:Brain / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006310:Hearing Aids; D006311:Hearing Disorders / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed