Paragangliomas are vascular tumors thought to stem from (chemoreceptor) cells responsible for sensing blood pressure and oxygenation. These cells are found throughout the temporal bone and neck, so tumors can develop in the middle ear, around the jugular vein, and near the skull base near the vagus nerve. Since the vagus nerve controls voice box movement, swallowing, palatal elevation and many digestive tract functions, these areas can be adversely affected. The most common location for head and neck region glomus tumors is where the carotid artery divides. Tumors there are known as carotid body paragangliomas. It is believed that head and neck glomus tumors are pathologically similar to adrenal gland tissue tumors, known as pheochromocytomas.
A head or neck glomus tumor typically presents with hearing loss and/or throbbing/pulsating tinnitus in which the patient “hears their pulse in their ear.” Depending on the location of the tumor, patients sometimes report a slow-growing neck mass. Patients occasionally report drainage from the ear, facial weakness, voice changes, numbness or asymmetry of the tongue, and weakness of the shoulder. It is not uncommon for the tumor to be visible behind the eardrum on physical examination.
Treatment of these tumors involves surgery or radiation therapy. In the past, surgical removal was considered the gold standard, but risks associated with the surgery and potential neurological deficits following surgery have led many patients to favor radiation therapy. Although radiation therapy stops tumor growth in over 90% of patients, the long-term success of glomus tumor radiation therapy is still unknown. Treatment options are made on a case-by-case basis. Discussion with our neurotologist will help guide your treatment. Glomus tumor treatment often involves a team approach which may include a head and neck surgeon, a medical oncologist, a radiation oncologist, a neurosurgeon, and sometimes, a geneticist.