Hostname: page-component-78c5997874-94fs2 Total loading time: 0 Render date: 2024-11-05T11:03:24.463Z Has data issue: false hasContentIssue false
  • English
  • Français

Brainstem pathology of infantile Gaucher's disease with only wave I and II of auditory brainstem response

Published online by Cambridge University Press:  29 June 2007

Kimitaka Kaga ,
Masae Ono ,
Kazuhiro Yakumaru ,
Masao Owada  and
Toshlo Mizutani
Kimitaka Kaga*
Affiliation:
Department of Otolaryngology, University of Tokyo, Tokyo, Japan
Masae Ono
Affiliation:
Division of Pediatrics, Tokyo Teishin General Hospital, Tokyo, Japan
Kazuhiro Yakumaru
Affiliation:
Division of Pediatrics, Tokyo Teishin General Hospital, Tokyo, Japan
Masao Owada
Affiliation:
Department of Pediatrics, Nihon University, Tokyo, Japan
Toshlo Mizutani
Affiliation:
Department of Pathology, Tokyo Metropolitan Geriatric Medical Center, Tokyo, Japan
*
Address for correspondence: Dr K. Kaga, M.D., Department of Otolaryngology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Toyko 113, Japan. Fax: +81-3-3814-9486 email [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

We studied the auditory brainstem response (ABR) and neuropathology in a female infant who died at six months of age because of typical infantile Gaucher's disease. The patient was hospitalized for hepatosplenomegaly and failure to thrive. Her ABR showed only waves I and II.

The neuropathological study disclosed that: (1) Gaucher's cells were found in the perivascular region of the cerebrum and anterior ventral nucleus of the thalamus. (2) Gliosis was found in the dorsal part of the brainstem rather than the ventral part. (3) Neuronal cells in the superior olivary nucleus were lost, and marked gliosis was found in the cochlear nucleus. The disappearance of wave III and later waves of ABR could be supported by these pathological findings.

Keywords

Evoked responses, auditoryGaucher's disease
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 112 , Issue 11 , November 1998 , pp. 1069 - 1073
Copyright
Copyright © JLO (1984) Limited 1998

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Achor, L. J., Starr, A. (1980) Auditory brainstem responses in the cat. I. Intracranial and extracranial recordings. Electroencephalography and Clinical Neurophysiology 48: 154–173.CrossRefGoogle ScholarPubMed
Buchwald, J., Huang, C.-M. (1975) Far field acoustic response: origins in the cat. Science 189: 382–384.CrossRefGoogle ScholarPubMed
Kaga, M., Azuma, C., Imamura, T., Murakami, T. (1982) Auditory brainstem response (ABR) in infantile Gaucher's disease. Neuropediatrics 13: 207–210.CrossRefGoogle ScholarPubMed
Kaga, K., Shinoda, Y., Suzuki, J.-I. (1997) Origin of auditory brainstem responses in cats: whole brainstem mapping, and a lesion and HRP study of the inferior colliculus. Acta Otolaryngologica (Stockh) 117: 197–201.CrossRefGoogle Scholar
Lacey, D. J., Terplan, K. (1984) Correlating auditory evoked and brainstem histological abnormalities in infantile Gaucher's disease. Neurology 34: 539–541.CrossRefGoogle ScholarPubMed
Lake, B. D. (1984) Gaucher disease, 4th Edition (Adames, J. H., Corsellis, J. A. N., Duchen, L. W. eds.) Edward Arnold Ltd, London. pp. 522–526.Google Scholar
Melcher, J. R., Guinan, J. J. Jr., Knudson, I. M., Kiang, N. Y. S. (1996) Generators of the brainstem auditory evoked potential in cat. II. Correlating lesion sites with waveform changes. Hearing Research 93: 28–51.CrossRefGoogle ScholarPubMed
Møller, A. R., Jannetta, P. J. (1983) Auditory evoked potentials recorded from the cochlear nucleus and its vicinity in man. Journal of Neurosurgery 59: 1013–1018.CrossRefGoogle ScholarPubMed
Møller, A. R., Jannetta, P. J., Møller, M. B. (1981) Neural generators of brainstem evoked potentials. Results from human intracranial recordings. Annals of Otology, Rhinology and Laryngology 90: 591–596.CrossRefGoogle ScholarPubMed
Starr, A., Hamilton, A. E. (1976). Correlation between confirmed sites of neurologic lesions and abnormalities of far-field auditory bramstem responses. Electroencephalography and Clinical Neurophysiology 41: 595–608.CrossRefGoogle ScholarPubMed