ObjectiveThe current study systematically reviewed the literature to compare auditory outcomes of patients treated for labyrinthine fistula (LF) based on characteristics of disease and surgical management.Databases ReviewedPubMed, Scopus, Web of Science.MethodsOriginal series (at least five cases) published from 2000 reporting management and hearing results of LF secondary to cholesteatoma were included. Proportion and odds-ratio (OR) meta-analyses were conducted through inverse variance random-effects models based on logit transformation.ResultsThe prevalence of LF is estimated to be 7% (95% confidence interval [CI], 5–9%). Fistulae involving the lateral semicircular canal (90%; 95% CI, 87–93%) and larger than 2 mm (53%; 95% CI, 43–64%) were common, whereas membranous involvement was less frequent (20%; 95% CI, 12–30%). Complete removal of the cholesteatoma matrix overlying the LF was mostly applied. Bone conduction (BC) preservation was frequently achieved (81%; 95% CI, 76–85%); new-onset postoperative anacusis was rarely reported (5%; 95% CI, 4–8%). A higher chance of BC preservation was associated with sparing the perilymphatic space (OR, 4.67; 95% CI, 1.26–17.37) or membranous labyrinth (OR, 4.56; 95% CI, 2.33–8.93), exclusive lateral semicircular canal involvement (OR, 3.52; 95% CI, 1.32–9.38), smaller size (<2 mm; OR, 3.03; 95% CI, 1.24–7.40), and intravenous steroid infusion (OR, 7.87; 95% CI, 2.34–26.42).ConclusionLF occurs in a significant proportion of patients with cholesteatoma. In the past two decades, complete removal of the cholesteatoma matrix followed by immediate sealing has been favored, supported by the high proportion of BC preservation. Hearing preservation depends primarily on characteristics of the LF, and specific surgical strategies should be pursued. Intraoperative and postoperative intravenous steroid infusion is recommended.