title: Hearing impairment in two boys with I-cell disease.
abstract: Auditory brainstem responses (ABR) were analyzed in 2 boys with I-cell disease. Both patients showed a prolonged latency of wave I, a normal I-V interpeak latency and an elevated threshold of wave V. The intensity-latency curve had a steep slope. These abnormal ABR findings reflect the presence of conductive hearing impairment associated with the cochlear component in I-cell disease.
authors.affiliation: Department of Pediatrics, Yamaguchi University School of Medicine, Ube.
authors.forename: T
authors.initials: T
authors.lastname: Ichiyama
authors.forename: T
authors.initials: T
authors.lastname: Hayashi
authors.forename: K
authors.initials: K
authors.lastname: Matsuo
mesh_terms: D016057:Evoked Potentials, Auditory, Brain Stem; D006311:Hearing Disorders / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008297:Male; D009081:Mucolipidoses / Q000150:complications*; D011930:Reaction Time; D012016:Reference Values