X-linked Adrenoleukodystrophy Initially Presenting with Severe Deafness

Abstract

90 -linked adrenoleukodystrophy (ALD) is the most common peroxisomal disorder characterized by progressive demyelination of the central nervous system and adrenal insufficiency. Poor school performance, attention deficit, behavioral changes, severe visual and hearing impairment are major clinical findings. Hypoglycemia and/or episodes of salt loss, skin hyperpigmentation, quadriplegia and cerebral ataxia may also occur.1