title: Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice.
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.
authors.affiliation: Laboratory of Neurochemistry and Behaviour, Born-Bunge Foundation, and Memory Clinic, Neurology Department, Middelheim Hospital, University of Antwerp, Universiteitsplein 1, Antwerp, Belgium.
authors.forename: R
authors.initials: R
authors.lastname: D'Hooge
authors.forename: R
authors.initials: R
authors.lastname: Coenen
authors.forename: V
authors.initials: V
authors.lastname: Gieselmann
authors.forename: R
authors.initials: R
authors.lastname: Lüllmann-Rauch
authors.forename: P P
authors.initials: PP
authors.lastname: De Deyn
mesh_terms: 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*
chemical_list: D002553:Cerebroside-Sulfatase