Cochlear Nerve Degeneration Coincident With Adrenocerebroleukodystrophy

Igarashi, Makoto; Jg, Neely; Pf, Anthony; Br, Alford

doi:10.1001/archotol.1976.00780170040003

Cited by 8 publications

(3 citation statements)

References 5 publications

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“…It appeared that the nerve was suffering from gradual degeneration. In a case of adrenocerebroleukodystrophy that we have experienced [Igarashi et al, 1976], the cochlear nerve was severely and selectively degenerated and the vestibular nerve was intact. The cochlear nerve can be involved in presbycusis, too.…”

Section: Discussionmentioning

confidence: 96%

Temporal Bone Findings in Friedreich’s Ataxia

Igarashi

Miller

O‐Uchi

et al. 1982

ORL

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Temporal bones from a case of Friedreich’s ataxia were studied. The diagnosis was confirmed neuropathologically. Severe reduction of the cochlear nerve fibers and spiral ganglion cells was found, whereas the pathology of the vestibular nerve was less marked and the facial nerve was normal. Among the different branches of vestibular nerve, the ampullary nerves were more involved than the macular nerves.

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Section: Discussionmentioning

confidence: 96%

Temporal Bone Findings in Friedreich’s Ataxia

Igarashi

Miller

O‐Uchi

et al. 1982

ORL

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“…Except with acoustic tumours the etiology of sensorineural hearing loss rarely has been shown to be localized outside the cochlea. Hearing loss due to a lesion primarily affecting the first order neuron or the higher connections of the auditory system has been presumed to occur in neural presbyacusis (v Fieandt and Saxen, 1937;Schuknecht, 1964), erythroblastosis fetali's (Dublin, 1951;Gerrand, 1952), hereditary ataxia (Spoendlin, 1974) and adrenoleucodystrophy (Igarashi etal, 1976). Temporal bone and brain-stem studies of these cases have revealed advanced loss of cochlear neurons.…”

Section: Discussionmentioning

confidence: 99%

Eighth nerve alcoholic neuropathy: A case report with light and electron microscopic findings

1981

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A 55-year-old man with symptoms of hearing loss, balance disturbance, and facial weakness was treated by translabyrinthine eighth cranial nerve trans-section. The patient also had a history and physical signs of chronic alcoholism and peripheral polyneuropathy. Results of laboratory tests revealed concomitant nutritional deficiency. Histopathological examination of the eighth nerve showed extensive degeneration of both myelinated and unmyelinated nerve fibers in both cochlear and vestibular divisions. Light and electron microscopic features of the degenerative process were compatible with previously described pathology of experimentally induced Wallerian-like degeneration. Alcoholic neuropathy as the cause of hearing loss and balance disturbance—and probably facial paresis—is suggested.

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“…Neuropathologic data to explain the nature of this deafness have been extremely meager. One case of NALD showed severe atrophy of the sensory epithelium and the tectorial membrane (36), while a single case of IRD showed again severe atrophy of the sensory epithelium, and the stria vascularis (117). No morphologic abnormalities have been reported in the relevant central neurons and pathways.…”

Section: Neuronal Degenerations In Peroxisomal Disordersmentioning

confidence: 94%

Peroxisomal Disorders: Genotype, Phenotype, Major Neuropathologic Lesions, and Pathogenesis

1998

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Neurological dysfunction is a prominent feature of most peroxisomal disorders. Enormous progress in defining their gene defects has been achieved. The genes and gene products, peroxins (PEX), in five of the complementation groups have been defined. These studies confirm that Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease (IRD) are a disease continuum. The gene defect in adreno‐leukodystrophy (ALD) / adrenomyeloneuropathy (AMN) involves an integral peroxisomal membrane protein. Neuropathologic lesions are of three major classes: (i) abnormalities in neuronal migration or differentiation, (ii) defects in the formation or maintenance of central white matter, and (iii) postdevelopmental neuronal degenerations. The central white matter lesions are those of: (i) inflammatory demyelination, (ii) non‐inflammatory dysmyelination, and (iii) nonspecific reductions in myelin volume or staining with or without reactive astrocytosis. The neuronal degenerations are of two major types: (i) the axonopathy of AMN involving ascending and descending tracts of the spinal cord, and (ii) cerebellar atrophy in rhizomelic chondrodysplasia punctata and probably IRD. We postulate that the abnormal fatty acids in peroxisomal disorders, particularly very long chain fatty acids and phytanic acid, are incorporated into cell membranes and perturb their microenvironments resulting in dysfunction, atrophy and death of vulnerable cells. The advent of mouse models for ZS and ALD is anticipated to provide even greater pathogenetic insights into the peroxisomal disorders.

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Cochlear Nerve Degeneration Coincident With Adrenocerebroleukodystrophy

Cited by 8 publications

References 5 publications

Temporal Bone Findings in Friedreich’s Ataxia

Temporal Bone Findings in Friedreich’s Ataxia

Eighth nerve alcoholic neuropathy: A case report with light and electron microscopic findings

Peroxisomal Disorders: Genotype, Phenotype, Major Neuropathologic Lesions, and Pathogenesis

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