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Age Impairs Corneal Sensitivity and Reflex Tearing in Congenital Aniridia

Title: Age Impairs Corneal Sensitivity and Reflex Tearing in Congenital Aniridia
Authors: Acosta, María Carmen; Náray, Annamária; Csidey, Mária; Maka, Erika; Nagy, Zoltán Zsolt; Jávorszky, Eszter; Zobor, Ditta; Tory, Kálmán; Cortón, Marta; Kovács, Illés; Gallar, Juana; Szentmáry, Nóra
Contributors: Ministerio de Ciencia e Innovación; Ministerio de Ciencia, Innovación y Universidades; Conselleria de Innovación, Universidades, Ciencia y Sociedad Digital, Generalitat Valenciana
Source: Cornea ; ISSN 0277-3740 1536-4798
Publisher Information: Ovid Technologies (Wolters Kluwer Health)
Publication Year: 2026
Description: Purpose: To characterize, for the first time, corneal sensory nerve functionality in congenital aniridia by assessing mechanical and cold corneal sensitivity, reflex tearing, and basal blinking and tearing. Methods: A cohort of fourteen pediatric and adult patients with PAX6 -related aniridia was enrolled in the study. Mechanical sensitivity (noncontact gas esthesiometer) and cold sensitivity (4°C drops) were quantified using a 0 to 10 verbal numerical scale (VNS). Spontaneous blinking, basal, and CO 2 -induced reflex tearing were also measured. Data were stratified by age, aniridia-associated keratopathy (AAK), and PAX6 mutation type—reduced expression or loss-of-function (LOF) genetic variants—and compared with healthy controls (n = 9). Results: Mechanical sensitivity to high-intensity stimuli was reduced in patients with aniridia, particularly in adults (2.2 ± 0.3 VNS, P = 0.02), those with severe AAK (2.4 ± 0.4 VNS, P = 0.09), and patients with LOF mutations (2.4 ± 0.2 VNS, P = 0.05) compared with controls (3.9 ± 0.5 VNS). Cold sensitivity was also reduced in adult patients (5.5 ± 0.5 vs. 3.8 ± 0.6 VNS, P = 0.05) and severe AAK (3.2 ± 0.8 VNS, P = 0.01). Reflex tearing values exhibited variability but trended lower in patients than controls, and tearing reserve (reflex minus basal tearing) was significantly reduced (12.7 ± 3.8 vs. 1.9 ± 1.9 mm, P = 0.02). Basal blinking and tearing were not significantly different. Conclusions: These findings suggest impaired mechano-nociceptors, polymodal nociceptors, and cold thermoreceptors function in congenital aniridia, with severity increasing with age and AAK grade. LOF mutation results show a trend with greater deficits than reduced expression variants, highlighting a critical role of PAX6 in corneal sensory integrity.
Document Type: article in journal/newspaper
Language: English
DOI: 10.1097/ico.0000000000004117
DOI: 10.1097/ICO.0000000000004117
Availability: https://doi.org/10.1097/ico.0000000000004117; https://journals.lww.com/10.1097/ICO.0000000000004117
Rights: http://creativecommons.org/licenses/by-nc-nd/4.0/
Accession Number: edsbas.8B13E401
Database: BASE