Alarcon score | n | Neuroimaging | Hearing | |||||
Modality | N | Abnormalities (n) | Normal | Unilateral SNHL | Bilateral SNHL | |||
0 No abnormalities | 8 | cUS | 6 | None | 1 | 5 | 2 | |
MRI | 1 | |||||||
Both | 1 | |||||||
1 Mild abnormalities | 34 | cUS | 18 | Single calcifications (1), LSV (11), GLC (5), mild VM (8) | 1 | 18 | 15 | |
MRI | 2 | Mild VM (1), (multi) focal WM signal abnormality (2) | ||||||
Both | 14 |
cUS
LSV (6) GLC (9) Single calcification (2) Mild VM (1) |
MRI
GLC (1) (Multi) focal WM signal abnormality (9) | |||||
2 Moderate abnormalities | 3 | cUS | 0 | 0 | 1 | 2 | ||
MRI | 1 | Mild VM, diffuse WM signal abnormality | ||||||
Both | 2 |
cUS
LSV (2) GLC (1) |
MRI
Severe VM (1) (Multi)focal WM signal abnormality (2) Temporal lobe involvement (2) | |||||
3 Severe abnormalities | 3 | cUS | 0 | 0 | 1 | 2 | ||
MRI | 2 | (Multi)focal WM signal abnormality (1) PMG (2) | ||||||
Both | 1 |
cUS
LSV Mild VM Extensive calcification Atrophy Suspected PMG Dysgenesis of CC |
MRI
Mild VM Diffuse WM signal abnormality PMG Dysgenesis of CC |
Alarcon classification: 0=none of the following abnormalities; 1=single punctate periventricular calcification, LSV, caudothalamic germinolysis, VM (excluding severe) and/or focal/multifocal WM signal abnormality on MRI; 2=multiple discrete periventricular calcifications, paraventricular GLC, severe VM, diffuse WM signal abnormality and/or temporal lobe involvement; 3=extensive calcifications, brain atrophy, abnormal gyration, cortical malformation, dysgenesis of the CC and/or cerebellar hypoplasia.
CC, corpus callosum; cUS, cranial ultrasound; GLC, germinolytic cyst; LSV, lenticulostriate vasculopathy; PMG, polymicrogyria; SNHL, sensorineural hearing loss; VM, ventriculomegaly; WM, white matter.