title: Correlating auditory evoked and brainstem histologic abnormalities in infantile Gaucher's disease.
abstract: In an infant with Gaucher's disease, brainstem auditory evoked responses were abnormal on three separate occasions. Only waves I and II were typically present at standard intensity settings, although a third peak was elicited at 88 dB. Histologic studies demonstrated absence of neurons in the cochlear nuclei and a hypoplastic superior olivary complex. This neurophysiologic-pathologic correlation supports contentions that both brainstem auditory evoked response (BAER) waves I and II arise from the auditory nerve and that it may be capable of generating additional peaks.
mesh_terms: D001933:Brain Stem / Q000473:pathology / Q000503:physiopathology*; D005072:Evoked Potentials, Auditory; D005776:Gaucher Disease / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D011930:Reaction Time