| First Author | D'Hooge R | Year | 1999 |
| Journal | Brain Res | Volume | 847 |
| Issue | 2 | Pages | 352-6 |
| PubMed ID | 10575108 | Mgi Jnum | J:58600 |
| Mgi Id | MGI:1349262 | Doi | 10.1016/s0006-8993(99)02085-5 |
| Citation | D'Hooge R, et al. (1999) Decline in brainstem auditory-evoked potentials coincides with loss of spiral ganglion cells in arylsulfatase A-deficient mice. Brain Res 847(2):352-6 |
| abstractText | 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. |
