RESUMO
OBJECTIVE: There is a need to highlight individual prognostic factors determining hearing loss in enlarged (wide) vestibular aqueduct, as currently clinicians cannot counsel parents about the expected clinical course, nor provide individualized hearing rehabilitation plans following identification at newborn screening. We apply a novel methodology to specifically outline and assess the accuracy of prognostic factors reporting for hearing loss in enlarged vestibular aqueduct. DATA SOURCES: A preferred reporting items for systematic reviews and meta-analyses compliant systematic review (Prospero ID: CRD42019151199), with searches applied to Medline, EMBASE, and Cochrane. Studies with longitudinal design were included between 1995 and 2019. STUDY SELECTION: The CHARMS-PF tool was used to assess robustness of prognostic factor study designs. DATA EXTRACTION: The QUIPS tool was used to assess for individual study risk of bias. DATA SYNTHESIS & RESULTS: Seventy papers were suitable for data extraction. In the six studies with low risk of bias, the domains of enlarged vestibular aqueduct (EVA) morphology, age, hearing thresholds, sex, head trauma, and genotype provided exploratory prognostic factors for hearing loss associated with enlarged vestibular aqueduct. Overall, study heterogeneity and risk of bias precluded reporting by forest plots and meta-analysis. CONCLUSIONS: The majority of exploratory prognostic factor studies for hearing loss associated with enlarged vestibular aqueduct are hampered by risk of bias. However, this systematic review identifies potential independent prognostic factors which should be measured, and adjusted for, in subsequent confirmatory studies utilizing multivariate analysis. This would determine the true independent prognostic effects associated with hearing loss in enlarged vestibular aqueduct, while facilitating prognostic model development and the ability to predict individual hearing loss trajectory.