Intracranial ceruminous gland adenocarcinoma

Patients with adenocarcinoma of the ceruminous gland arising in the middle ear have a typical syndrome consisting of unilateral hearing loss, otalgia, facial paresis, and a middle-ear mass. Adjacent cranial nerves also may be affected. Some patients may have an ipsilateral cerebellar ataxia if the lesion extends into the cerebellopontine angle and compresses the cerebellar hemisphere. Obstructive hydrocephalus may occur secondary to obstruction of the aqueduct or fourth ventricle. The initial clinical findings may be those of a jugular foramen syndrome. These lesions are usually slow-growing and may be associated with a very prolonged clinical course. Although the tumors are rare, the physician should be aware of their existence if proper care is to be given.