Glomus jugulare tumour presenting with isolated accessory nerve palsy

Glomus tumours of the skull base are rare, and most frequently present with symptoms of hearing loss and tinnitus. Diagnosis is often delayed due to the slow growth of the tumour. We describe the previously unreported occurrence of a glomus tumour presenting with a unilateral accessory nerve palsy.     

Glomus jugulare tumor.

A review has been made of the current recommendations for treatment of glomus tumors involving the ear. Thirty cases treated in our clinic have been reviewed, comprised of 8 glomus tympanicum and 22 glomus jugulare tumors. We recommend surgical excision as primary treatment for glomus tympanicum tumors and high voltage radiotherapy as primary treatment for glomus jugulare tumors. Visible residual tumor following adequate radiotherapy may remain unchanged for many years. Some cases that do not show a response to high voltage radiation may be suitable for secondary radical surgical treatment. Fortunately, we have not yet encountered a case of glomus jugulare tumor with nonresponse to high voltage radiation in the doses recommended. Long term follow-up is important.     

Glomus jugulare tumors with intracranial extension.

A glomus jugulare tumor with posterior fossa extension needs the expertise of the neuro-otologic surgeon and neurosurgeon to obtain total tumor removal. A one-stage combined otologic and neurosurgical procedure is presented for removal of base of skull tumors posterior to the carotid artery. Twelve cases of this type of tumor have been seen at The Cleveland Clinic. Two cases are presented in detail to illustrate the applicability of this procedure. In cases with infection around the temporal portion of the tumor, tumor extension around or anterior to the carotid artery, or extensive involvement of the posterior fossa, the procedure should be performed in two stages.     

Glomus tympanicum and glomus jugulare tumors

The ideal management of most paragangliomas is complete surgical excision. Because of technical advances, issues of resectability have given way to issues of functional outcome and postsurgical quality of life. This article reviews the surgical strategy for craniocervical paragangliomas. Intracranial extension, defect reconstruction, and cranial nerve rehabilitation are addressed.