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BACKGROUND Cervical fusion surgery is associated with adjacent-level degeneration, but surgical and technical factors are difficult to dissociate from the mechanical effects of the fusion itself. OBJECTIVE To determine the effect of fusion on adjacent-level degeneration in unoperated patients using a cohort of patients with congenitally fused cervical vertebrae. METHODS We identified 96 patients with incidental single-level cervical congenital fusion on computed tomography imaging. We compared these patients to an age-matched control cohort of 80 patients without congenital fusion. We quantified adjacent-level degeneration through direct measurements of intervertebral disk parameters as well as the validated Kellgren & Lawrence classification scale for cervical disk degeneration. Ordinal logistic regression and 2-way analysis of variance testing were performed to correlate extent of degeneration with the congenitally fused segment. RESULTS Nine hundred fifty-five motion segments were analyzed. The numbers of patients with C2-3, C3-4, C4-5, C5-6, and C6-7 congenitally fused segments were 47, 11, 11, 17, and 9, respectively. We found that patients with congenital fusion at C4-C5 and C5-C6 had a significantly greater extent of degeneration at adjacent levels compared with the degree of degeneration at the same levels in control patients and in patients with congenital fusion at other cervical levels, even while controlling for expected degeneration and age. CONCLUSION Taken together, our data suggest that congenitally fused cervical spinal segments at C4-C5 and C5-C6 are associated with adjacent-level degeneration independent of fixation instrumentation. This study design removes surgical factors that might contribute to adjacent-level degeneration.