Abstract
Otoacoustic emissions (OAEs) were evaluated in 51 ears of 30 patients with a severe
auditory brainstem response (ABR) waveform abnormality. Thirteen ears showed no ABR
to click sound of higher intensity than 100 dBSPL (group 1). Fourteen ears exhibited
only wave V or a decreased amplitude pattern of ABR (group 2). Twenty-four ears showed
a predominant wave I or no wave III pattern (group 3). Almost all the ears with absent
ABR showed no OAE, which strongly suggested hearing loss of cochlear origin, although
one patient with alternating hemiplegia of childhood exhibited definite OAEs and auditory
reactions without ABR. One patient with mitochondrial myopathy, encephalopathy, lactic
acidosis, and strokelike episodes (MELAS) and her mother in group 2 had OAE abnormalities,
which also suggested mild to severe hearing impairment. When OAEs are present, an
accompanying ABR abnormality may be produced by brainstem dysfunction of the underlying
disorder such as Pelizaeus–Merzbacher disease. There was a significant relationship
(χ-square test P<0.001) between the positivity of the distortion product OAE response and the clinical
auditory reactions in 24 patients, although their ABR abnormalities did not reflect
hearing impairment directly. Careful examination of both audiometry and OAEs might
be necessary for further assessment of the hearing function in pediatric patients
with neurological disorders and specific auditory nerve disease.
Keywords
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Article Info
Publication History
Accepted:
April 7,
2000
Received:
January 11,
2000
Identification
Copyright
© 2000 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.