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The relationship between central peak cone density, mfERG amplitude topography, and foveal hypoplasia (FH) grade was calculated using Spearman rank correlation. Parafoveal cone densities among a subset of PWA were compared to a previously reported control group.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All PWA had variably below average peak cone density, decreased BCVA, flattened mfERG topography, and FH. Higher peak cone density significantly correlated with higher peripheral mfERG amplitudes (\n                      <jats:italic>r<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.73,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0049), but not central (\n                      <jats:italic>r<\/jats:italic>\n                      <jats:sup>2<\/jats:sup>\n                      \u2009=\u20090.25), and did not correlate with steeper (more normal) mfERG topography (\n                      <jats:italic>r<\/jats:italic>\n                      <jats:sup>\n                        <jats:italic>2<\/jats:italic>\n                      <\/jats:sup>\n                      \u2009=\u20090.031). Parafoveal cone densities did not greatly differ outside of the central 2\u00b0 (within Ring 1) between PWA and those of\u00a0previously reported controls.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The association of increased foveal cone packing with elevated peripheral, but not central mfERG amplitudes, suggests aberrant post-receptoral circuitry in the macula. The presence of excess connections between cone photoreceptors and bipolar cells, similar to the unrefined circuitry seen in fetal retinae, may underly the electrical dysfunction seen in these PWA, and explain why, even with milder FH and denser cone packing, normal visual acuity is often not achieved.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10633-026-10130-w","type":"journal-article","created":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T03:02:30Z","timestamp":1783047750000},"page":"223-234","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The relationship between foveal anatomy and retinal function in oculocutaneous albinism"],"prefix":"10.1007","volume":"153","author":[{"given":"Uma","family":"Balakrishnan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niamh","family":"Wynne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wanda","family":"Pfeifer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erica","family":"Woertz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Carroll","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlene V.","family":"Drack","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,7,3]]},"reference":[{"key":"10130_CR1","doi-asserted-by":"publisher","first-page":"1653","DOI":"10.1016\/j.ophtha.2011.01.028","volume":"118","author":"MG Thomas","year":"2011","unstructured":"Thomas MG, Kumar A, Mohammad S, Proudlock FA, Engle EC, Andrews C, Chan WM, Thomas S, Gottlob I (2011) Structural grading of foveal hypoplasia using spectral-domain optical coherence tomography a predictor of visual acuity? 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Drack, Spark Therapeutics (Research grant), InVision 2020 (Research grant), Fighting Blindness Canada (Research grant), Meira GTx (Collaborator).","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.","order":2,"name":"Ethics","label":"Statement of human rights","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"No animals were used in this study; it is a human study, performed with IRB approval for the human subjects.","order":3,"name":"Ethics","label":"Statement on the welfare of animals","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","label":"Informed consent","group":{"name":"EthicsHeading","label":"Declarations"}}]}}