Delayed Visual Maturation Associated With Auditory Neuropathy/Dyssynchrony

Abstract

Delayed visual maturation is a term used to describe infants who initially seem blind but subsequently have a marked improvement. The mechanism of visual loss and the subsequent improvement remains unknown. Auditory neuropathy/dyssynchrony is a condition of hearing impairment associated with absent or severely abnormal brainstem auditory evoked potentials but normal cochlear functions as measured by otoacoustic emissions. In this report, a 9-month-old infant who had no visual fixation for the first 3 months of life and congenital hearing impairment is described. Her brainstem auditory evoked potential study at 2.5 months of age showed no response to click stimuli presented at 90 dB nHL, whereas her otoacoustic emissions were normal. Subsequently, her vision and hearing improved. A brainstem auditory evoked potential study at 9 months of age showed reproducible waveforms. This case suggests the need for a detailed hearing evaluation of children with delayed visual maturation. Furthermore, this case highlights the need for follow-up brainstem auditory evoked potential testing prior to pursuing any audiologic intervention. (J Child Neurol 2003; 18: 358—361).