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To evaluate long-term hearing changes and surgical outcomes for middle ear cholesteatoma with labyrinthine fistula following complete surgical removal of the cholesteatoma matrix.Consecutive samples of 43 patients who underwent 1-stage complete resection of the cholesteatoma matrix overlying a labyrinthine fistula were obtained. Immediate and long-term hearing changes were analyzed in association with fistula size. Accuracy of various diagnostic examinations was assessed.Immediately postsurgery, the average bone conduction threshold (43.34 dB) did not differ significantly from the preoperative value (36.66 dB, P = .083). There were also minimal changes thereafter (45.63 dB) without recurrent case over an average follow-up time of 38.3 months (range, 17-69 months). More than 10 dB hearing loss was found in 7 patients with a case of intraoperative perilymph leakage (2.3%), although 5 of them had had preoperative air conduction threshold above 90 dB. Their fistulas were significantly larger than those of patients without hearing loss (P = .027).Although caution is required for total removal of a large fistula, owing to increased risk of postoperative hearing deterioration, 1-stage complete resection of cholesteatoma matrix on labyrinthine fistula could be effective in disease control and long-term hearing preservation.