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Background The head and neck is the most common site of mucosal melanoma, a cancer with poor prognosis. In contrast to cutaneous melanoma, mucosal melanoma of the head and neck (MMHN) is uncommon, with limited data regarding outcomes and prognostic factors drawn from small, single-institution case series. In order to identify factors predictive of survival, we analyzed MMHN outcomes in a large US cohort. Methods MMHN cases (n = 815) diagnosed in the USA between 1973 and 2007 were analyzed in the Surveillance, Epidemiology, and End Results registry, and cause of death was individually determined in 778 (95.5%) cases. Kaplan–Meier survival analysis and Cox proportional hazards regression were used to analyze prognostic variables. Results Disease-specific survival status was determined in 778 (95.5%) cases. The 5- and 10-year rates of overall survival (OS) were 25.2 and 12.2%; disease-specific survival (DSS), 32.4 and 19.3%. On multivariable analysis, anatomic primary site was an independent predictor of OS and DSS, with tumors in the nasal cavity and oral cavity associated with survival superior to tumors in the nasopharynx and paranasal sinuses. Age > 70 years, tumor size, nodal status, and distant metastasis status were additional independent predictors of poorer survival. Conclusions In this large cohort of patients with MMHN, we have identified several novel factors robustly predictive of overall and melanoma-specific survival.

Objective-1) To investigate whether children implanted in the first year of life show higher levels of speech perception than later-implanted children, when compared at the same ages, and 2) to investigate the time course of sensitive periods for developing speech perception skills. More specifically, to determine whether faster gains in speech perception are made by children implanted before 1-yr-old relative to those implanted at 2 or 3 years.Methods-A retrospective analysis was completed on 117 patients with congenital profound bilateral sensorineural hearing loss, with no additional identified disabilities, who were implanted in the first, second, or third year of life. The outcome measure consisted of developmental curves showing LNT word identification scores as a function of age.Results-Children implanted within the first year of life have a mean advantage of 8.2% LNT-easy word scores over those implanted in the second year (p<0.001) and a 16.8% advantage in LNT-easy word scores over those implanted in the third year of life (p<0.001). These advantages remained statistically significant after accounting for gender, residual hearing and bilateral cochlear implant use. When speech perception scores were expressed as a function of "hearing age" rather than chronological age, however, there were no significant differences among the three groups Conclusions-There is a clear advantage for earlier implanted children over later implanted children when compared at the same age, but not when compared at the same time after implantation with regards to speech perception. Thus, the sensitive period for developing word identification seems to extend at least until age 3.

To investigate the characteristics associated with survival in esthesioneuroblastoma and to determine whether the modified Kadish staging system can predict outcome. Design: Retrospective population-based cohort study. Subjects: All patients in the Surveillance, Epidemiology, and End Results tumor registry diagnosed as having esthesioneuroblastoma (1973-2002). Main Outcome Measures: The modified Kadish stage and the overall and disease-specific survival rates were determined. Results: The cohort included 311 patients with a mean age of 53 years and a unimodal age distribution. The overall 5-and 10-year survival rates were 62.1% and 45.6%, respectively. The modified Kadish staging system was applied to 261 patients. Kaplan-Meier analysis showed the overall and disease-specific survival rates at 10 years to be 83.4% and 90%, respectively, for patients with stage A disease; 49% and 68.3% for patients with stage B disease; 38.6% and 66.7% for patients with stage C disease; and 13.3% and 35.6% for patients with stage D disease. Log-rank test comparisons found Kadish stage (PϽ.01), treatment modality (PϽ.002), lymph node status (PϽ.01), and age at diagnosis (PϽ.001) to be significant predictors of survival. Cox regression analysis confirmed that Kadish stage remained a significant predictor of diseasespecific survival. Conclusion: The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma.

A cochlear implant (CI) is a surgically implanted device for the treatment of severe to profound sensorineural hearing loss in children and adults. It works by transducing acoustic energy into an electrical signal, which is used to stimulate surviving spiral ganglion cells of the auditory nerve. The past 2 decades have witnessed an exponential rise in the number of CI surgeries performed. Continual developments in programming strategies, device design, and minimally traumatic surgical technique have demonstrated the safety and efficacy of CI surgery. As a result, candidacy guidelines have expanded to include both pre and postlingually deaf children as young as 1 year of age, and those with greater degrees of residual hearing. A growing proportion of patients are undergoing CI for off-label or nontraditional indications including single-sided deafness, retrocochlear hearing loss, asymmetrical sensorineural hearing loss (SNHL) in adults and children with at least 1 ear that is better than performance cut-off for age, and children less than 12 months of age. Herein, we review CI design, clinical evaluation, indications, operative technique, and outcomes. We also discuss the expanding indications for CI surgery as it relates to lateral skull base pathology, comparing CI to auditory brainstem implants, and address the concerns with obtaining magnetic resonance imaging (MRI) in CI recipients.

Object The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation. Methods Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise. Results Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15–120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants. Conclusions Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.

Object The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.). Methods The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded. Results Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction). Conclusions Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.

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