Diagnosis and Management of Patients with Neurofibromatosis Type 2 : Experience of 16 Cases

ABSTRACT

Neurofibromatosis type 2(NF-2) is a dominantly inherited disorder characterized by the occurrence of bilateral acoustic neurinomas and the frequent association of other central nervous system tumors. We present a retrospective review of 16 patients with NF-2 who were treated at our hospital from 1984 to 1995. In 13 cases, the diagnoses of NF-2 were based on the criteria developed at the Consensus Development Conference of National Institute of Health in the United States, and in another 3 cases, the criterias of NF-2 were not fully satisfied, but the diagnoses of NF-2 were highly suspected. The average age of the patients was 27.6 years, ranging from 13 years to 56 years. The most common symptom was hearing difficulty;intervals between symptom onset and deafness ranged from 8 months to 6 years(mean2.9 years.) One family of NF-2 was documented, consisting of a sister, a brother and their mother. Nine patients underwent operations on unilateral acoustic neurinomas;these were subtotally removed in eight patient s and totally in one patient. Among these patients, five were deaf on the ipsilateral side at surgery. Among the other four patients with useful hearing before surgery, hearing was preserved to preoperative status in two patients. Four patients with diagnoses of meningioma, received operations to relieve mass effect with subtotal removal in two patients and total removal in the other two. Early diagnosis and treatment are the most important in the management planning of patients with NF-2 for reasons of early manifestation and rapid progression of the disease.