Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice.
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.
D'Hooge
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R
Laboratory of Neurochemistry and Behaviour, Born-Bunge Foundation, and Memory Clinic, Neurology Department, Middelheim Hospital, University of Antwerp, Universiteitsplein 1, Antwerp, Belgium.
Coenen
R
R
Gieselmann
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V
Lüllmann-Rauch
R
R
De Deyn
P P
PP
D000367:Age Factors; D000818:Animals; D002553:Cerebroside-Sulfatase / Q000235:genetics* / Q000378:metabolism; D016057:Evoked Potentials, Auditory, Brain Stem / Q000235:genetics*; D007966:Leukodystrophy, Metachromatic / Q000235:genetics* / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic; D013136:Spiral Ganglion / Q000378:metabolism / Q000473:pathology*
D002553:Cerebroside-Sulfatase