Long-term natural history of ellipsoid zone width in USH2A-retinopathy.

ABSTRACT:

Aims

To investigate the long-term natural history of ellipsoid zone (EZ) width in USH2A-retinopathy.

Methods

EZ width measurements from optical coherence tomography were retrospectively obtained from 110 eyes of 55 participants with molecularly confirmed biallelic USH2A-retinopathy. We used a hierarchical Bayesian method to construct and compare different mathematical models describing the long-term decline of EZ width.

Results

Compared with linear and quadratic models, exponential decline best represented the long-term loss of EZ width based on the deviance information criterion score. Log-transformed EZ width declined linearly over 30 years of inferred disease duration (median: 0.063 (IQR: 0.040-0.086) log (µm)/year). Compared with the raw EZ width dec

SUBMITTER: Heyang M 

PROVIDER: S-EPMC11866300 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature