Elsevier

Brain Research

Volume 847, Issue 2, 20 November 1999, Pages 352-356
Brain Research

Short communication
Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice

https://doi.org/10.1016/S0006-8993(99)02085-5Get rights and content

Abstract

Arylsulfatase A (ASA)-deficient mice constitute an animal model for the inherited lysosomal storage disease, metachromatic leukodystrophy (MLD). Brainstem auditory-evoked potentials (BAEPs) were recorded in control and ASA-deficient mice of 3, 6, 9 and 12 months. BAEPs were evoked in control mice of all ages studied, but were completely absent in ASA (−/−) mice of 9 and 12 months. A significant delay in the wave pattern was noted in 6-month-old ASA (−/−) mice. Histological examination and morphometric analysis showed that the decline of BAEPs in ASA (−/−) mice was paralleled by a decrease in spiral ganglion cell numbers.

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Acknowledgements

The excellent assistance of Ms. Marleen Geurden (Memory Clinic, Antwerp) and Ms. Dagmar Niemeier (Anatomy, Kiel) is gratefully acknowledged. The work of R.D. and P.P.D.D. was financially supported by the Born-Bunge Foundation, Antwerp University, NeuroSearch Antwerp, OCMW Antwerp-Medical Research Foundation and FWO-Flanders (grant no. G.0027.97). Dr. Rudi D'Hooge is a postdoctoral fellow of FWO-Flanders. The morphological investigations were financially supported by the Deutsche

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