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
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Developmental changes of distortion product and transient evoked otoacoustic emissions in different age groups.Brain Dev. [in press];
- A case of alternating hemiplegia of childhood with cerebellar atrophy.Pediatr Neurol. 1998; 19: 65-68
- Auditory neuropathy.Brain. 1996; 119: 741-753
- Auditory nerve disease of both ears revealed by auditory brainstem responses, electrocochleography and otoacoustic emissions.Scand Audiol. 1996; 25: 233-238
- Auditory neuropathy: a report on three cases with early onset and major neonatal illnesses.Electroenceph clin Neurophysiol. 1997; 104: 17-22
- Auditory neuropathy in childhood.Laryngoscope. 1998; 108: 1374-1377
- Cochlear origin of hearing loss in MELAS syndrome.Ann Neurol. 1998; 43: 350-359
- Auditory perception and language in a patient with classical Pelizaeus–Merzbacher disease.Jpn J Logopedics and Phoniatrics. 1992; 33 ([in Japanese]): 317-324
- Otoacoustic emissions and brainstem auditory potentials in children with neurological afflictions.Brain Dev. 1994; 16: 213-218
- Characteristic evoked potentials in childhood-onset dentatorubal-pallidoluysian atrophy.Brain Dev. 1996; 18: 389-393
- Differences in evoked potential characteristics between DRPLA patients and patients with progressive myoclonic epilepsy: preliminary findings indicating usefulness for differential diagnosis.Epilepsy Res. 1999; 37: 3-11
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.
