Brooke-Spiegler syndrome complicated by unilateral hearing loss

Brooke-Spiegler syndrome is an autosomal dominant tumor predisposition disorder. The disease is characterized by the occurrence of multiple skin appendage tumors, including cylindroma, trichoepithelioma, and spiradenoma. In some patients, tumors cover the entire head circumference, thereby causing disfigurement and other complications. Here, we report on a man with multiple cylindroma that were distributed in a turban tumor-like fashion. One of these neoplasms arose in the meatus externus of the right ear leading to unilateral hearing loss, a complication that has been documented only on few occasions in this disease.