title: VEP analysis methods in children with optic nerve hypoplasia: relationship to visual acuity and optic disc diameter.
abstract: OBJECTIVE
  Assessing vision in young children with optic nerve hypoplasia (ONH) is challenging due to multi-directional infantile nystagmus, the range of optic nerve loss, and cognitive delay. This study examined visual evoked potential (VEP) responses and averaging techniques in children with ONH. The assumption is that EEG epochs with inconsistent temporal phase would be associated with nystagmus, signal reduction due to axon loss, and visual inattention.
  
  
  METHODS
  A retrospective chart review was performed on 44 children (average age 2.2 years; SD 1.9). Optic disc diameter was estimated by ophthalmoscopy. Visual function was measured under binocular viewing and then compared to the eye with the larger optic disc to exclude secondary amblyopia. Visual acuity was measured by Teller cards or by recognition optotypes, and both measures were converted into log minimum angle of resolution (logMAR). VEPs were recorded to onset/offset of horizontal gratings and to reversing checkerboards. Signal-to-noise ratios (SNRs) were estimated from phase consistency across epochs in the Fourier domain. VEPs were also averaged after (1) correction of epochs for phase shifts across a limited bandwidth, or (2) selection of only epochs showing phase consistency.
  
  
  RESULTS
  Optic disc diameter, logMAR, VEP amplitudes, and VEP SNR were all significantly inter-correlated. Optic disc diameter correlated best with VEP SNR (Spearman rho = 0.82; p < 0.001). Age-corrected logMAR correlated with optic disc diameter and VEP SNR (Spearman rho = -0.695 and 0.70, respectively; p < 0.001). VEP latency poorly correlated with optic disc diameter or logMAR. Correction of phase shifts or selection of epochs based on phase consistency significantly increased VEP amplitude and SNR for children with optic disc diameters <1000 microns. Correction of phase inconsistency did not improve the correlation of VEP parameters with optic disc diameter or with logMAR.
  
  
  CONCLUSIONS
  In ONH, the size of the optic nerve is correlated with VEP SNR and logMAR. The results imply a direct relationship between the reduction in optic nerve axons and generalized reduction in visual function. Our calculation of VEP SNR provides objective assessment of optic nerve function that is independent of subjective scoring of VEP peaks.
authors.affiliation: Division of Ophthalmology, OA.5.345, Roger H. Johnson Vision Lab, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA. john.kelly@seattlechildrens.org.|Department of Ophthalmology, University of Washington School of Medicine, Seattle, WA, 98105, USA. john.kelly@seattlechildrens.org.
authors.forename: John P
authors.initials: JP
authors.lastname: Kelly
authors.affiliation: Division of Ophthalmology, OA.5.345, Roger H. Johnson Vision Lab, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA.|Department of Otolaryngology, University of Washington School of Medicine, Seattle, WA, USA.
authors.forename: James O
authors.initials: JO
authors.lastname: Phillips
authors.affiliation: Division of Ophthalmology, OA.5.345, Roger H. Johnson Vision Lab, Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA.|Department of Ophthalmology, University of Washington School of Medicine, Seattle, WA, 98105, USA.
authors.forename: Avery H
authors.initials: AH
authors.lastname: Weiss
mesh_terms: D000550:Amblyopia / Q000503:physiopathology; D002675:Child, Preschool; D004569:Electroencephalography; D005074:Evoked Potentials, Visual / Q000502:physiology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009759:Nystagmus, Pathologic / Q000503:physiopathology; D009887:Ophthalmoscopy; D009898:Optic Disk / Q000473:pathology*; D009900:Optic Nerve / Q000002:abnormalities* / Q000503:physiopathology; D009901:Optic Nerve Diseases / Q000151:congenital* / Q000503:physiopathology; D012189:Retrospective Studies; D059629:Signal-To-Noise Ratio; D014792:Visual Acuity / Q000502:physiology*
keywords: Children; Nystagmus; Optic nerve hypoplasia; Signal-to-noise ratio; Visual evoked potential