Thus, the aim of this study was to describe the appearance, distribution and possible clinically significant correlations of tissue remodeling markers (MMP-2, MMP-9, TIMP-2, TIMP-4), Sonic hedgehog gene protein (Shh), pro- and anti-inflammatory cytokines (IL–1, IL–10), transcription factor (NF-κβ), proliferation marker (Ki–67), angiogenetic factor (VEGF) and local tissue defensins (HβD–2, HβD–4) of the pediatric cholesteatoma tissue compared to control skin tissue. Here, SHH is linked to cholesteatoma.