[Diagnosis and treatment of temporal paragangliomas: an analysis of 21 cases treated in Bern between 1992 and 1999].

INTRODUCTION: Paragangliomas are usually benign tumours stemming from the neural crest which may have neuroendocrine activity. Because of hypervascularisation they usually lead to pulsatile tinnitus. Local expansion initially causes middle ear and later inner ear hearing loss, and cranial nerve dysfunction.

PATIENTS/METHODS: Signs and symptoms, demographic data, tumour stages, treatments and outcomes were analysed for the 21 patients treated at our clinic between 1992 and 1999. Among 16 surgically treated patients 3 type A and 4 type B tumours were treated by mastoidectomy and middle ear reconstruction. For the 8 type C and one type D tumours, angiography was performed preoperatively, usually followed by selective embolisation of tumour vessels, in 7 cases with additional permanent occlusion of the internal carotid artery. These 9 tumours were removed through an infratemporal approach with partial petrosectomy, preservation of the inner ear and facial nerve rerouting, followed in 8 cases by middle ear occlusion. The patient with a type D2 tumour had a two-stage otological and neurosurgical operation. One patient with type C3 tumour only underwent radiation therapy. 4 patients have had no treatment so far: 2 had small type A tumours and one a type C3 tumour but contraindications for surgery. A last patient with a slowly growing type D2 tumour cannot be operated on due to general health problems.

RESULTS: All patients with type A and B tumours had normal to subnormal postoperative hearing and normal facial nerve function. In one case with a type C tumour the middle ear reconstruction led to normal hearing thresholds. After rerouting of the facial nerve, transient palsy was noted in 6 out of 9 cases. In 3 patients a slight permanent impairment of facial nerve function (House-Brackman II) remained. 4 patients had some dysfunction of cranial nerves IX-XII postoperatively. After a follow-up of up to 8 years (mean 5 years) no recurrence was observed in the surgically treated patients. The patient treated by radiation had further tumour growth and another treatment is being planned. Among the 4 patients followed without treatment, no tumour growth has been observed up to now in 3 patients.

DISCUSSION: Our study confirms that the well standardised conservative surgical treatment allows reliable removal of paragangliomas in most cases, and involves low morbidity.