Gorham-stout disease of the temporal bone

Objectives: 1) Present data regarding Gorham Stout disease as manifested in the temporal bone; 2) review the literature regarding this rare bone disorder. Methods: A case report from a tertiary university hospital is presented. A literature search using the terms "Gorham Stout disease and head and neck," "massive osteolysis," and "temporal bone" was undertaken. Results: A 29 year old female with a diagnosis of Gorham Stout disease was identified. She complained of aural fullness and tinnitus bilaterally. Demineralization and moth eaten changes of the osseous structures of the skull base and posterior fossa were prominent. The left mastoid air cells were opacified and erosion extended to the left jugular foramen, left hypoglossal canal, left stylomastoid process, and left Eustachian tube. The histopathology and radiographic findings are presented. Conclusion: Gorham Stout disease is a rare disease of the bone and is also known as massive osteolysis. Less than 200 cases have been reported in the world literature. This disease follows a rapidly progressive course resulting in resorption and replacement of bone with dense fibrous tissue. It typically presents in childhood or adolescence. Involvement within the head and neck is frequent but typically involves the calvarial bones. Involvement of the temporal bone is very rare. A review of the literature with a focus on head and neck manifestations of the disease and their management is presented.