Diagnosis and treatment of temporal paragangliomas

Introduction: Paragangliomas are usually benign tumours stemming from the n eural crest which may have neuroendocrine activity. Because of hypervascula risation they usually lead to pulsatile tinnitus. Local expansion initially causes middle ear and later inner ear hearing loss, and cranial nerve dysf unction. Patients/methods: Signs and symptoms, demographic data, tumour stages, trea tments 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 preope ratively, usually followed by selective embolisation of tumour vessels, in 7 cases with additional permanent occlusion of the internal carotid artery. These 9 rumours were removed through an infratemporal approach with partia l petrosectomy, preservation of the inner ear and facial nerve rerouting, f ollowed in 8 cases by middle ear occlusion. The patient with a type D-2 tum our had a two-stage otological and neurosurgical operation. One patient wit h type C-3 tumour only underwent radiation therapy. 4 patients have had no treatment so far: 2 had small type A tumours and one a type C-3 tumour but contraindications for surgery. A last patient with a slowly growing type D- 2 tumour cannot be operated on due to general health problems. Results: All patients with type A and B rumours had normal to subnormal pos toperative hearing and normal facial nerve function. In one case with a typ e C tumour the middle ear reconstruction led to normal hearing thresholds. After rerouting of the facial nerve, transient palsy tvas noted in 6 out of 9 cases. In 3 patients a slight permanent impairment of facial nerve funct ion (Hwouse-Brackman II) remained. 4 patients herd some dysfunction of cran ial nerves IX-XII postoperatively. After a followup of up to 8 years (mean 5 years) no recurrence was observed in the surgically treated patients. The patient treated lay radiation had further tumour growth and another treatm ent is being planned. Among the 4 patients followed without treatment, no t umour growth has been observed up to now in 3 patients. Discussion: Our study confirms that the well standardised conservative surg ical treatment allows reliable removal of paragangliomas in most cases, and involves low morbidity.