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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 Kimikaga-tky@umin.u-tokyo.ac.jp
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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

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