Cochlear nerve degeneration coincident with adrenocerebroleukodystrophy.
A 19-month-old boy had adrenocerebroleukodystrophy and unusually severe cochlear nerve degeneration and demyelination, a condition in advance of that expected from known disease entities. We report the only known temporal bone study in adrenocerebroleukodystrophy.
D000326:Adrenoleukodystrophy / Q000150:complications / Q000473:pathology*; D003056:Cochlear Nerve / Q000473:pathology*; D003711:Demyelinating Diseases / Q000150:complications / Q000473:pathology*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D013701:Temporal Bone / Q000473:pathology; D000160:Vestibulocochlear Nerve Diseases / Q000150:complications / Q000473:pathology*