Discrepancy between auditory brainstem responses, auditory steady-state responses, and auditory behavior in two patients with Pelizaeus-Merzbacher disease.
We reported two cases of Pelizaeus-Merzbacher disease. Both cases visited our hospital manifesting horizontal nystagmoid movements present from birth, and delayed motor development. Magnetic resonance imaging of the brain showed diffuse dysmyelination of the cerebral white matter, and auditory brainstem response showed waves I and II but absence of all subsequent components. Conditioned orientation reflex (COR) audiometry showed poor reactions in an infantile case whose development was severely retarded, and who spoke no meaningful words. Auditory steady-state response (ASSR) was a helpful tool for identifying her auditory ability; thereafter, her communication skills improved naturally. The other case was mildly developmentally retarded, and the results of COR audiometry and ASSR were considered the same level. The discrepancy between results of these hearing tests may arise under the influence of developmental level of the case.
Tanaka
Manabu
M
Division of Neurology, Saitama Children's Medical Center, Saitama, Japan. a1099655@pref.saitama.lg.jp
Hamano
Shin-Ichiro
S
Sakata
Hideaki
H
Adachi
Nodoka
N
Kaga
Kimitaka
K
Osaka
Hitoshi
H
Kurosawa
Kenji
K
D000161:Acoustic Stimulation; D001300:Audiometry, Evoked Response / Q000379:methods*; D001309:Auditory Threshold / Q000502:physiology*; D001921:Brain / Q000473:pathology; D001933:Brain Stem / Q000503:physiopathology; D002675:Child, Preschool; D041321:Chromosomes, Human, X / Q000235:genetics; D002658:Developmental Disabilities / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D020440:Gene Duplication; D006801:Humans; D007805:Language Development Disorders / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D018991:Myelin Proteolipid Protein / Q000235:genetics; D009759:Nystagmus, Pathologic / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D020371:Pelizaeus-Merzbacher Disease / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D012729:Sex Chromosome Aberrations; D012815:Signal Processing, Computer-Assisted
D018991:Myelin Proteolipid Protein