Bobby A. Tajudeen

Speech production intelligibility of early implanted pediatric cochlear implant users.

OBJECTIVES To investigate the influence of age, and age-at-implantation, on speech production intelligibility in prelingually deaf pediatric cochlear implant recipients. METHODS Forty prelingually, profoundly deaf children who received cochlear implants between 8 and 40 months of age. Their age at testing ranged between 2.5 and 18 years. Children were recorded repeating the 10 sentences in the Beginners Intelligibility Test. These recordings were played back to normal-hearing listeners who were unfamiliar with deaf speech and who were instructed to write down what they heard. They also rated each subject for the intelligibility of their speech production on a 5-point rating-scale. The main outcome measures were the percentage of target words correctly transcribed, and the intelligibility ratings, in both cases averaged across 3 normal-hearing listeners. RESULTS The data showed a strong effect of age at testing, with older children being more intelligible. This effect was particularly pronounced for children implanted in the first 24 months of life, all of whom had speech production intelligibility scores of 80% or higher when they were tested at age 5.5 years or older. This was true for only 5 out of 9 children implanted at age 25-36 months. CONCLUSIONS Profoundly deaf children who receive cochlear implants in the first 2 years of life produce highly intelligible speech before the age of 6. This is also true for most, but not all children implanted in their third year.

Epidemiology and Treatment of Lacrimal Gland Tumors: A Population-Based Cohort Analysis

IMPORTANCE Primary tumors of the lacrimal gland are rare and are associated with substantial morbidity and mortality. The literature regarding these tumors is limited to case series and case reports. OBJECTIVE To examine the incidence, treatment, and overall survival (OS) and disease-specific survival (DSS) of patients with cancer of the lacrimal gland. DESIGN, SETTING, AND PARTICIPANTS Population-based cohort analysis using the Surveillance, Epidemiology, and End Results (SEER) database to identify patients with primary tumors of the lacrimal gland from 1973 to 2010. MAIN OUTCOMES AND MEASURES Overall survival and DSS. RESULTS A total of 321 patients with nonlymphoid tumors of the lacrimal gland were identified. The most common histological subtypes were adenoid cystic carcinoma (ACC) (32.1%) and squamous cell carcinoma (SCC) (29.9%). Survival analysis revealed a 5-year OS and DSS for all lacrimal gland tumors of 60% and 75%, respectively. On univariate analysis, low tumor grade (P = .04) and surgical treatment (P < .001) were associated with significantly better OS. For ACC tumors, surgery (P = .009), but not radiotherapy (P = .44), was found to significantly improve OS. For SCC tumors, surgical treatment significantly improved both OS (P < .001) and DSS (P = .004); radiation therapy also significantly improved OS (P = .03). Using a multivariable analysis model, age (hazard ratio [HR], 1.03 [95% CI, 1.01-1.04]; P < .001), surgery (HR, 0.43 [95% CI, 0.25-0.75]; P = .003), and T stage at presentation (HR, 1.18 [95% CI, 1.01-1.37]; P = .03) were found to be independent predictors of OS. For ACC alone, age (HR, 1.04 [95% CI, 1.02-1.06]; P < .001) and surgery (HR, 0.35 [95% CI, 0.13-0.91]; P = .03) were independent predictors of OS. For SCC, age (HR, 1.05 [95% CI, 1.02-1.09]; P = .005), surgical resection (HR, 0.31 [95% CI, 0.12-0.83]; P = .02), and radiation therapy (HR, 0.33 [95% CI, 0.14-0.80]; P = .01) were independent predictors of OS. CONCLUSIONS AND RELEVANCE Our study demonstrates that ACC is the most common malignant epithelial neoplasm of the lacrimal gland. Determinants of survival for tumors of the lacrimal gland include age at diagnosis and surgical therapy. Radiation therapy is associated with improved DSS in SCC but not in ACC.

Quantification of benign lesion regression as a function of 532-nm pulsed potassium titanyl phosphate laser parameter selection.

Although the potassium titanyl phosphate (KTP) laser is versatile, the variability in laser parameters for laryngeal pathologies and the lack of clinical efficacy data remain problematic. We provide preliminary data regarding these parameters for benign lesion regression. In addition, we describe a novel method for the quantification of the effects of the KTP laser on vocal fold (VF) lesions.

Importance of Tumor Grade in Esthesioneuroblastoma Survival: A Population-Based Analysis

IMPORTANCE There is a need for larger studies characterizing the effect of tumor grade on survival for patients with esthesioneuroblastoma. OBJECTIVE To investigate prognostic factors for survival in patients diagnosed with esthesioneuroblastoma, including emphasis on tumor grade. DESIGN, SETTING, AND PARTICIPANTS Retrospective, population-based cohort study of patients in the Surveillance, Epidemiology, and End Results (SEER) tumor registry who were diagnosed with esthesioneuroblastoma from January 1, 1973, to January 1, 2010. The last date of survival follow-up was 2013. MAIN OUTCOMES AND MEASURES Overall and disease-specific survival. RESULTS The cohort included 281 patients with a mean age of 52 years. There were 154 males (54.8%) and 127 females (45.2%). Kaplan-Meier analysis demonstrated an overall and disease-specific survival rate of 61% and 70% at 5 years and 50% and 64% at 10 years, respectively. Multivariable Cox regression analysis showed that advanced tumor grade and modified Kadish stage (hazard ratio, 4.930; 95% CI, 2.635-9.223; P = .001) portended worse disease-specific survival, and radiation therapy (hazard ratio, 0.499; 95% CI, 0.272-0.916; P = .03) improved disease-specific survival. Patients with low-grade tumors (grades I and II) demonstrated an overall and disease-specific survival rate of 84% and 92% at 5 years and 67% and 87% at 10 years, respectively. Multivariable analysis of low-grade tumors only revealed receiving surgery (P = .004) as an independent positive predictor of disease-specific survival. High-grade tumors (grades III and IV) demonstrated overall and disease-specific survival of 40% and 50% at 5 years and 34% and 43% at 10 years, respectively. Multivariable analysis of high-grade tumors showed modified Kadish stage (hazard ratio, 2.025; 95% CI, 1.430-2.866; P < .001) predicted worse disease-specific survival, and radiation therapy (hazard ratio, 0.433; 95% CI, 0.228-0.864; P = .02) independently predicted improved disease-specific survival. CONCLUSIONS AND RELEVANCE Here, to our knowledge, we report the largest study investigating prognostic factors for survival, with the inclusion of tumor grade, in patients diagnosed with esthesioneuroblastoma. Patients with high-grade tumors had substantially worse survival rates than patients with low-grade tumors. Multivariable analysis revealed only receiving surgery as an independent predictor of disease-specific survival for patients with low-grade tumors, while modified Kadish stage and postoperative radiation therapy were significant factors in predicting disease-specific survival in patients with high-grade tumors. This study highlights the growing evidence that tumor grade should be a key factor in predicting survival in patients with esthesioneuroblastoma, and that adjuvant radiation therapy improves survival rates among patients with high-grade, but not low-grade, tumors.

Esthesioneuroblastoma: An Update on the UCLA Experience, 2002–2013

Objectives To profile the clinical presentation and treatment results of esthesioneuroblastomas at the University of California, Los Angeles (UCLA), from 2002 to 2013. Design Retrospective review. Setting Tertiary academic institution. Participants Forty-one patients with esthesioneuroblastomas treated at UCLA. Main Outcome Measures Overall survival (OS) and recurrence-free survival (RFS). Results Thirty-six patients were included with a mean age of 50.1 years and a median duration of follow-up of 33 months. The 5-year RFS and OS were 54% and 82%, respectively. Modified Kadish stage was the only factor identified to affect OS. Multivariate analysis demonstrated that tumor grade was the only factor that had an independent impact on RFS. There was no statistical difference in survival among the surgical approaches chosen. Conclusions The updated data on the UCLA experience reveals that all three surgical approaches chosen provide comparable survival, although longer follow-up will be needed to ascertain if these findings hold true. The endoscopic approach had a statistically significant decrease in length of hospital stay and a trend toward reduced blood loss, intensive care unit admission, and complications. The modified Kadish staging was the only factor identified to predict OS. Multivariate analysis revealed that tumor grade was an independent predictor of recurrence; therefore, its importance should be emphasized in future staging systems.

Actinomycosis of the temporal bone and brain: case report and review of the literature.

Objectives Actinomycosis is a rare disease with a typically indolent course in the head and neck. During the modern era, only 12 cases within the ear and temporal bone and 75 intracranial cases have been reported. We present a case of actinomycosis of the petrous apex that led to meningitis and encephalitis. Methods The patient was a 12-year-old girl who presented with mental status changes. After 48 hours of treatment with empiric antibiotics for meningitis without improvement, imaging revealed an enhancing mass in the right petrous apex, destruction of the cochlea, meningeal enhancement, and left temporoparietal encephalitis. Results The initial therapy included broad-spectrum antibiotic, antifungal, and antiviral agents, as well as myringotomy and tympanostomy tube placement. When the patients clinical status worsened, she underwent subtotal petrosectomy with drainage of the petrous apex. The final pathologic findings were consistent with actinomycosis. Conclusions Actinomycosis is a rare infection in the temporal bone and central nervous system that can have a high mortality risk if not treated appropriately. Often, these bacteria do not grow well in culture, and diagnosis must be made on the basis of histopathologic features. Good clinical outcomes can be obtained with surgical debridement followed by long-term antibiotic treatment.

Significance of Tumor Stage in Sinonasal Undifferentiated Carcinoma Survival A Population-Based Analysis

Objective To describe the incidence and determinants of survival of patients with sinonasal undifferentiated carcinoma (SNUC) from 1973 to 2011 using the SEER database (Surveillance, Epidemiology, and End Results), with consideration of tumor stage based on the Kadish system. Study Design Retrospective database analysis. Setting Academic medical center. Subjects and Methods The SEER registry was utilized to calculate survival trends for 328 patients with SNUC between 1973 and 2011. Patient data were then analyzed with respect to histopathology, age, sex, race, subsite, modified Kadish stage, tumor size, and treatments rendered. Results The cohort was composed of 61.9% males with median age of 60 years. The median overall survival (OS) was 1.9 years. Most tumors presented in the nasal cavity, maxillary sinus, and ethmoid sinus (29.3%, 27.4%, 21%, respectively); 43.7% of patients received both surgical and radiation therapy. OS at 2, 5, and 10 years was 43%, 30%, and 25%, respectively. On univariate analysis, age, Kadish stage, and tumor size were associated with worse OS and disease-specific survival (DSS), while surgery and radiation therapy were associated with improved OS and DSS (all P < .05). On multivariate analysis, radiation therapy and lower Kadish stage were associated with improved OS and DSS, while younger age was additionally associated with improved OS (all P < .05). Conclusion SNUC is a rare but aggressive sinonasal malignancy. Tumor stage as determined by the Kadish system is associated with worse survival, with radiation therapy appearing to play a key role in therapeutic management.

Sinonasal mucosal melanoma: 20-year experience at a tertiary referral center

Sinonasal melanoma (SNM) is a rare cancer with extremely poor prognosis. Detecting melanoma on frozen section has historically been considered to be unreliable. A review of cases of sinonasal melanoma treated at a tertiary referral center was conducted to analyze treatment outcomes and identify prognostic factors for survival. In addition, an investigation was performed correlating sinonasal melanoma on frozen section and permanent analysis.

Malignant peripheral nerve sheath tumors of the head and neck: Demographics, clinicopathologic features, management, and treatment outcomes

OBJECTIVES To determine the epidemiology and prognostic indicators in patients with malignant peripheral nerve sheath tumors (MPNST) of the head and neck. MATERIALS AND METHODS The surveillance, epidemiology, and end results registry was reviewed for patients with head and neck MPNST from 1973 to 2011. Study variables included age, sex, race, tumor size, stage at presentation, and treatment modality. RESULTS There were 374 cases of head and neck MPNST identified. Mean age at diagnosis was 50.7 y ears; 60.2% of patients were male and 82.6% were white. After diagnosis, 38.8% of patients underwent surgery and radiation therapy and 48.1% underwent surgery alone. Kaplan-Meier analysis demonstrated overall (OS) and disease-specific survival (DSS) of 51% and 67% at 5 years. Multivariate Cox regression analysis showed that age (p=0.030), stage (p=0.002), surgery (p=0.037), and size (p<0.001) were predictors of OS, while stage (p<0.001) and size (p<0.001) were predictors of DSS. For stage I/II cancers, surgery (p=0.011) and size (p=0.010) were predictors of OS, and size (p=0.001) predicted DSS. For stage III/IV cancers, both radiotherapy (p=0.024, p=0.009) and size (p=0.001, p=0.001) predicted OS and DSS. For tumors ⩽5 cm, stage (p=0.031) predicted DSS. For tumors >5 cm, male gender (p=0.005), stage (p=0.001), surgery (p=0.003), and radiotherapy (p=0.050) were determinants of OS, and male gender (p=0.022), stage (p<0.001), and radiotherapy (p=0.002) were determinants of DSS. CONCLUSION Surgical resection confers survival benefit in patients with early stage MPNST, while radiotherapy improves survival in cases with metastatic disease. Surgery and radiotherapy are prognostically important in patients with tumors >5 cm.

Head and neck sarcomas: the UCLA experience

PURPOSE To profile the clinical presentation, subtype distribution, and treatment results of sarcomas of the head and neck at a single tertiary academic center over an 11-year period. MATERIALS AND METHODS A retrospective review was performed by examining the records and reviewing the pathology of 186 patients with head and neck sarcomas treated at UCLA Medical Center from 2000 to 2011. RESULTS The mean age of the study population was 49 ± 22 years. 58% of the patients were male and 42% were female. Median duration of follow-up for the entire group was 18.5 months. The most common presenting symptom was a mass lesion in 59.9% of patients. The nasal cavity/sinus was the most common presenting site seen in 22% of patients. Solitary fibrous tumor/hemangiopericytoma was the most common subtype. 15% of patients had evidence of prior radiation exposure. 26.3% of tumors were greater than 5 cm and 35.5% were high-grade. Margins were positive in 31.2% of patients. Lymph node metastasis was rare at 6.5%. Perineural invasion was identified in 6.5%. Among all subtypes, 5-year recurrence-free survival and overall survival were 50% and 49%, respectively. Multivariate analysis demonstrated that grade and margin status were predictors of recurrence-free survival while grade and age affected overall survival. CONCLUSIONS Head and neck sarcomas are a rare entity frequently presenting as a mass lesion. In our series, lesions tended to be high-grade with a significant portion of surgical specimens having positive margins. Grade and margin status were the most important predictors of survival.