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Abstract
Three patients with the syndrome of progressive severe deafness, associated with juvenile
onset of motor neurone disease, were examined using conventional audiometry and brainstem
auditory evoked response (BAER) recordings, and electrocochleographic recordings were
made in two of them.
Findings indicate that the deafness had resulted from loss of acoustic nerve fibers
and/or sensory cells of the spiral ganglion.
Keywords
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References
- Infantile amyotrophic lateral sclerosis of the family type.J. Nerv. Ment. Dis. 1894; (As quoted by): 707-716
- Progressive bulbar palsy in adults.in: Handbook of Clinical Neurology, Vol. 22 (System Disorders and Atrophies, Part II). North-Holland Publishing Co, Amsterdam1975: 111-156
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- Clinical patterns of motor neurone disease in younger age groups.Neurology (India). 1970; : 104
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Wadia, N.H. (1986) Personal communication.
Article info
Publication history
Accepted:
September 9,
1986
Received in revised form:
September 9,
1986
Received:
July 21,
1986
Identification
Copyright
© 1987 Published by Elsevier Inc.
