Cochlear Nerve Degeneration Coincident With Adrenocerebroleukodystrophy

Abstract
• 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. (Arch Otolaryngol 102:722-726, 1976)

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