title: Brooke-Spiegler syndrome complicated by unilateral hearing loss.
abstract: 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.
authors.affiliation: Department of Dermatology, Maastricht University Center for Molecular Dermatology, and GROW-School for Oncology and Developmental Biology, University Hospital Maastricht, The Netherlands. lparre@sder.azm.nl
authors.forename: Lizelotte J M T
authors.initials: LJ
authors.lastname: Parren
authors.forename: Boris
authors.initials: B
authors.lastname: Bauer
authors.forename: Henning
authors.initials: H
authors.lastname: Hamm
authors.forename: Jorge
authors.initials: J
authors.lastname: Frank
mesh_terms: D000328:Adult; D003528:Carcinoma, Adenoid Cystic / Q000150:complications*; D018280:Carcinoma, Skin Appendage / Q000150:complications*; D004428:Ear Neoplasms / Q000150:complications*; D046088:Hearing Loss, Unilateral / Q000209:etiology*; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications*; D010641:Phenotype; D012535:Scalp; D012878:Skin Neoplasms / Q000150:complications*; D013577:Syndrome