No abstract

Objectives In the management of two related patients with multicentric glomus jugulare tumors, given the incidence of 1:30,000 with approximately 20% familial cases, our objective was to review the genetic characteristics and inheritance patterns of these tumors and to determine what molecular genetic screening possibilities exist for the phenotypically normal family members. In addition, our aim was to review the incidence of various multicentric paraganglioma (PGL) tumor location combinations. Methods Molecular genetic linkage analysis testing was performed on the 2 patients and 14 other unaffected family members. We report the results of this screening and review the literature on the incidence and genetics of paragangliomas. Results The inheritance pattern in the literature demonstrates autosomal dominant transmission with maternal imprinting (inactivation). The proclivity for multicentric origin increases to 26% in familial cases, as reflected in our patients. In addition to the two patients, four unaffected family members demonstrated the presence of the disease haplotype at chromosome band 11q23, which indicates a very high likelihood of developing a paraganglioma, given the highly penetrant nature of the disease. Conclusions It is clear that the familial PGL gene locus is situated at chromosome 11q23. The gene itself and its exact degree of penetrance, however, still await identification. Since early detection of paragangliomas reduces the incidence of morbidity and mortality, genotypic analysis as a screening tool in families of affected patients should play a front‐line diagnostic role, leading to more timely and cost‐effective patient management.

The cases of 67 patients consecutively operated on for acoustic tumors are reported. Preoperative records consist of tumor size and the position of the fourth ventricle on computerized tomography (CT) scans. An analysis is made of the immediate postoperative and current function of the facial nerves. There is a statistically significant relationship between the number of weak or paralyzed seventh nerves and displacement of the fourth ventricle (P less than .05). It is now possible to accurately measure acoustic tumors on preoperative CT scans. The position of the fourth ventricle can also be clearly seen. The current standard of diagnosis and management of acoustic tumors should include a cursor measurement of tumor size on CT scan and a notation of the position of the fourth ventricle. Adoption of this method of reporting will permit meaningful evaluation of treatment for acoustic tumors. This analysis illustrates the importance of accurate measurements in reporting and evaluating surgical results.

Increasing popularity of the retrosigmoid approach would have one believe this is the preferred posterior fossa approach for a vestibular nerve section. The authors take issue with this. The antesigmoid posterior lateral retrolabyrinthine approach is a gentle craniotomy, easily performed and with low morbidity. A review of 70 consecutive cases revealed no procedural alteration because of surgical exposure, an absence of meningitis, no VIIth nerve weakness, minimal headache, average hospital stay of five days, and only one patient with a significant CSF leak. Hyperventilation anesthesia, patience during delicate VIIIth nerve exposure, incorporation of abdominal fat into dural closure sutures and prophylactic antibiotics have made this operation easy for the patient…and the surgeon. Don't abandon it!