Faculty Bibliography

The vast majority of paragangliomas are benign. It is unclear what percentage is malignant but it has been estimated between 6% and 10% of those reported. Despite their distinct radiologic and pathologic appearance, there is no reliable way to distinguish benign from malignant paragangliomas preoperatively. There are no absolute pathologic criteria for malignancy. The accepted criteria for determining malignancy preoperatively are spread to regional lymph nodes or distant metastasis.There are predictors of malignancy. They include location of the paraganglioma (vagal paragangliomas are malignant more often than any other paraganglioma), pain as a presenting symptom, recent rapid growth, and the succinate dehydrogenase B subunit mutation. The 5-year survival rate for malignant paraganglioma has been estimated to be around 60%. As the presence of lymph node metastasis could be the only sign of malignancy, it is recommended that lymph nodes be removed during the resections of these tumors. In the case of paragangliomas presenting in the neck, levels II and III are easily approached and should be sampled. In cases where there is preoperative involvement of lymph nodes by paraganglioma, a modified neck dissection is indicated. It should address minimally levels II through IV

Testicular varicocele, which is defined as the dilation of the veins draining the testicle, has long been associated with a detrimental effect on testicular function. Despite a lack of high-quality, prospective data, recent evidence has shed light on potential links between varicocele and male infertility and serum testosterone levels. Similarly, varicocele repair has increasingly been shown to have a beneficial impact on pregnancy rates, semen parameters, and on improving serum testosterone in adult men. Numerous studies have assessed the optimal technique for varicocele repair and the bulk of the evidence has shown the microsurgical inguinal/subinguinal approach to have the highest success rates, the lowest overall complication rates, and the lowest recurrence rates. The management of varicocele in adolescents remains a clinical conundrum, but contemporary evidence suggests early deleterious effects of varicocele on testicular function in some patients. Well-designed prospective trials are critical to delineate the true impact and role of varicocele repair on male infertility and hypogonadism in adult and adolescent men.

Vagal paragangliomas are nonchromaffin tumors that originate from paraganglionic chemoreceptor cell bodies associated with the vagus nerve. Although both surgical excision and radiation therapy have been reported to be effective treatment modalities, the effectiveness of one over the other remains unclear. There are several surgical approaches that have been described. As with any surgical disease, the utility of a technique over another has been justified at some point in time. Clearly, there has been a shift away from the more radical approaches with the primary goal of surgery being complete extirpation of the tumor along with avoidance of iatrogenic cranial neuropathies and preservation and restoration of cranial nerve function. In addition, early rehabilitation of speech and swallowing dysfunction results in improved functional outcomes and reduced hospital stay and morbidity

BACKGROUND:Status dystonicus (SD) is a rare and potentially life-threatening complication of primary or secondary dystonia, characterized by acute worsening of dystonic movements. There is no consensus regarding optimal treatment, which may be medical and/or surgical. METHODS:We present our experience with pallidal deep brain stimulation (DBS) in 5 DYT1-positive patients with SD and provide a review of the literature to examine optimal management. RESULTS:Of the 5 patients treated with pallidal DBS, all experienced postoperative resolution of their dystonic crisis within a range of 1-21 days. Long-term follow-up resulted in 1 patient returning to preoperative baseline, 3 patients improving from baseline, and 1 patient making a complete recovery. Of the 28 SD patients (including our 5 patients) reported in the literature who were treated with DBS or ablative surgery, 26 experienced cessation of their dystonic crisis with a return to baseline function and, in most cases, clinical improvement. CONCLUSION:DBS is an effective therapeutic modality for the treatment of SD. In addition to the long-term benefits of stimulation, early and aggressive treatment may improve the overall outcome.

OBJECTIVE: The aim of this case series was to assess the impact of auditory rehabilitation with cochlear implantation on the cognitive function of elderly patients over time. DESIGN: This is a longitudinal case series of prospective data assessing neurocognitive function and speech perception in an elderly cohort pre- and post-implantation. SETTING: University cochlear implant center. PARTICIPANTS: The patients were post-lingually deafened elderly female (mean, 73.6 years; SD, 5.82; range, 67-81 years) cochlear implant recipients (n=7). MEASUREMENTS: A neurocognitive battery of 20 tests assessing intellectual function, learning, short- and long-term memory, verbal fluency, attention, mental flexibility, and processing speed was performed prior to and 2-4.1 years (mean, 3.7) after cochlear implant (CI). Speech perception testing using Consonant-Nucleus-Consonant words was performed prior to implantation and at regular intervals postoperatively. Individual and aggregate differences in cognitive function pre- and post-CI were estimated. Logistic regression with cluster adjustment was used to estimate the association (%improvement or %decline) between speech understanding and years from implantation at 1 year, 2 years, and 3 years post-CI. RESULTS: Improvements after CI were observed in 14 (70%) of all subtests administered. Declines occurred in five (25%) subtests. In 55 individual tests (43%), post-CI performance improved compared to a patient's own performance before implantation. Of these, nine (45%) showed moderate or pronounced improvement. Overall, improvements were largest in the verbal and memory domains. Logistic regression demonstrated a significant relationship between speech perception and cognitive function over time. Five neurocognitive tests were predictive of improved speech perception following implantation. CONCLUSION: Comprehensive neurocognitive testing of elderly women demonstrated areas of improvement in cognitive function and auditory perception following cochlear implantation. Multiple neurocognitive tests were strongly associated with current speech perception measures. While these data shed light on the complex relationship between hearing and cognition by showing that CI may slow the expected age-related cognitive decline, further research is needed to examine the impact of hearing rehabilitation on cognitive decline.