Bilateral middle ear cholesterol granuloma in familial hypercholesterolemia

Cholesterol granuloma (CG) is a histologic description of foreign body giant cell formation toward cholesterol crystals. The majority of temporal bone CG is unilateral and most common in the petrous apex. Middle ear CG is usually the result of underlying ear diseases. Primary middle ear CG is very rare. Most reported CG has not been associated with familial hypercholesterolemia (FH). FH, an autosomal dominant disorder, manifests as high levels of serum cholesterol and low density lipoprotein (LDL) cholesterol. We report a rare case of FH and bilateral aggressive primary middle ear CG. This publication has been approved by the IRB, Hospital Alor Setar.