The Detection Of Misrouting In Albinism: Evaluation of Different VEP Procedures in a Heterogeneous Cohort

Charlotte C. Kruijt, Gerard C. de Wit, Herman E. Talsma, Nicoline E. Schalij-Delfos, Maria M. van Genderen

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    Purpose: To investigate the optimal procedures for multichannel visually evoked potentials (VEPs) to detect misrouting in albinism subjects.

    Methods: Investigations were done in a phenotypically heterogeneous group of 180 albinism subjects and 187 controls with and without ocular pathology. We retrospectively compared standard flash VEP (fVEP), high-frequency fVEP with a handheld device (hh fVEP), pattern-onset VEP (poVEP), and short-onset acuity sweep VEP. The diagnostic power of these stimuli were estimated by calculating the area under the curve (AUC). Subjects were divided in three age groups (<3, 3-6 [toddler], and ≥6 years). Subjects ≥6 years of age were further divided in two visual acuity groups (≤0.3 logMAR and >0.3 logMAR).

    Results: The optimal stimulus was hh fVEP, standard fVEP, and poVEP 60' for subjects <3, 3-6, and ≥6 years of age, respectively. In subjects ≥6 years old with poor visual acuity, the area under the curve of fVEP was almost equal to that of poVEP 60'.

    Conclusions: For the optimal detection of misrouting with multichannel VEP recordings, we recommend using a high-frequency hh fVEP in children <3 years of age, standard fVEP in toddlers, and poVEP 60' in subjects ≥6 years of age. fVEP can also be used in the oldest age group for subjects with visual acuity of >0.3 logMAR. Remarkably, some albinism subjects showed misrouting on full-field stimulation but normal routing of the central retina, suggesting that not the whole line of decussation is shifted temporally.

    Original languageEnglish
    Pages (from-to)3963-3969
    Number of pages7
    JournalInvestigative ophthalmology & visual science
    Issue number12
    Publication statusPublished - 3 Sept 2019


    • Albinism, Ocular/diagnosis
    • Area Under Curve
    • Child
    • Child, Preschool
    • Cohort Studies
    • Evoked Potentials, Visual/physiology
    • Female
    • Humans
    • Male
    • Optic Chiasm/pathology
    • Optic Nerve Diseases/diagnosis
    • Photic Stimulation
    • Reproducibility of Results
    • Retrospective Studies
    • Sensitivity and Specificity
    • Vision Disorders/diagnosis
    • Visual Acuity
    • Visual Pathways/pathology


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