研究目的
To study transition zones from normal to abnormal retina in Usher syndrome IB (USH1B) caused by myosin 7A (MYO7A) mutations.
研究成果
In USH1B-MYO7A, the constriction rate of EZ extent depends on the initial eccentricity of the transition. Ellipsoid zone edges in the macula correspond to large local changes in cone vision, but extramacular EZ edges show more pronounced losses on rod-based vision tests. It is advisable to use not only the EZ line but also other structural and functional parameters for estimating natural history of disease and possible therapeutic effects in future clinical trials of USH1B-MYO7A.
研究不足
The study was limited by the small sample size and the use of a linear model for progression, which may not fully capture the complexity of disease progression. Additionally, the spatial distribution of transition zones was not isotropic, and the relationship between structure and function varied across different retinal regions.
1：Experimental Design and Method Selection:
Optical coherence tomography (OCT) was used to segment scattering layers in the outer retina of patients with USH1B due to MYO7A mutations. Static perimetry was measured under dark-adapted (DA) and light-adapted (LA) conditions.
2：Sample Selection and Data Sources:
Sixteen patients (ages 2–42 years) with USH1B and known MYO7A mutations were included. A group of normal subjects (n ? 28, ages 5–58 years) were also studied.
3：List of Experimental Equipment and Materials:
Spectral-domain (SD) OCT system (RTVue-100; Optovue, Inc., Fremont, CA, USA) and time-domain OCT (OCT1 and OCT3; Carl Zeiss Meditec, Dublin, CA, USA) were used.
4：Experimental Procedures and Operational Workflow:
The OCT protocol included 4.5- or 9-mm line scans along horizontal and vertical meridians crossing the fovea and extending to 9 mm into the periphery. Each scan was repeated three or more times. Postacquisition processing of OCT data was performed with custom programs (MatLab 7.5; MathWorks, Natick, MA, USA).
5：5- or 9-mm line scans along horizontal and vertical meridians crossing the fovea and extending to 9 mm into the periphery. Each scan was repeated three or more times. Postacquisition processing of OCT data was performed with custom programs (MatLab 5; MathWorks, Natick, MA, USA). Data Analysis Methods:
5. Data Analysis Methods: Quantitation of retinal layers was performed manually as a function of eccentricity every 0.58 using published methods.
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