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Hearing impairment in two boys with I-cell disease
T Ichiyama1, T Hayashi, K Matsuo
1Department of Pediatrics, Yamaguchi University School of Medicine, Ube.
Brain & Development
|September 1, 1991
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.