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Auditory brain-stem responses in adrenomyeloneuropathy.

A M Grimes, M L Elks, G Grunberger

Archives of Neurology

|September 1, 1983

Summary

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Auditory brain-stem response testing effectively detects neurologic abnormalities in adrenomyeloneuropathy patients. This method is reliable even before clinical symptoms appear, aiding early diagnosis.

Area of Science:

Neurology
Audiology
Genetics

Background:

Adrenomyeloneuropathy (AMN) is an X-linked peroxisomal disorder.
AMN primarily affects the spinal cord and peripheral nerves, leading to progressive disability.
Early detection of neurological involvement is crucial for management.

Observation:

Three patients with confirmed adrenomyeloneuropathy underwent comprehensive audiologic evaluations.
Peripheral hearing was normal in two patients; one exhibited mild high-frequency hearing loss.
Auditory brain-stem responses (ABRs) were abnormal in all three subjects.

Findings:

ABRs revealed significant abnormalities, including absent responses above wave I in one patient.
Prolonged intervals between waves I-III and III-V were observed in the other two patients.

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These ABR findings indicate central auditory pathway dysfunction.

Implications:

ABR testing is a valuable, non-invasive tool for identifying neurological dysfunction in AMN.
ABR can detect subclinical auditory pathway involvement, aiding in early diagnosis.
This diagnostic approach can guide timely therapeutic interventions and patient monitoring.