Cholesteatoma, immunology and gender medicine

Primary cholesteatoma is a rare pathology of the ear in childhood, which is characterized by a high recurrence rate. The spread of keratinizing squamous epithelium in the middle ear space with subsequent bone destruction occurs in these cases, in contrast to acquired cholesteatoma, without a retraction pocket as a prerequisite of the disease. In order to better understand the pathogenesis and thus have the chance to develop better therapy concepts, we analyzed our patient population. In the past 10 years, 5 children with primary cholesteatoma have been treated in our clinic. All children were males (ages 3-8 years). 2 of 5 had bilateral cholesteatomas. An asymptomatic coagulation factor deficiency was also diagnosed in 3 of 5 patients (2 children with a disorder in the exogenous system and one with a disorder in the exogenous and endogenous systems). All children received interdisciplinary care. Based on our patient group, the increased risk of male patients developing this pathology can be confirmed. The association between ear pathology and coagulation factor deficiency is an indication that special attention should be paid to impaired hemostaseology in the affected children. Further work is necessary in the areas of therapy as well as the preventive measures for this pathology, in order to further optimize the treatment of these children.