Herpes simplex virus of the nose masquerading as invasive fungal sinusitis: A pediatric case series.

The management of invasive fungal sinusitis differs greatly from the management of herpes simplex virus (HSV) of the nose in immunocompromised patients. However, the diagnosis may be uncertain and a delay in treatment can lead to mortality. Here we describe the successful medical management of a series of immunocompromised pediatric patients with HSV lesions of the nose with the initial concern for invasive fungal sinusitis. The diagnosis of HSV herpes was supported by positive polymerase chain reaction (PCR) testing of the nasal lesion. To our knowledge, these are the first cases described in the pediatric literature, emphasizing the need to include this entity on the differential.

Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease.

BACKGROUND: Autoimmune inner ear disease (AIED) is a rare disease that results in progressive sensorineural hearing loss. Patients with AIED initially respond to corticosteroids; however, many patients become unresponsive to this treatment over time, and there is no effective alternative therapy for these individuals. METHODS: We performed a phase I/II open-label, single-arm clinical trial of the IL-1 receptor antagonist anakinra in corticosteroid-resistant AIED patients. Given that the etiology of corticosteroid resistance is likely heterogeneous, we used a Simon 2-stage design to distinguish between an unacceptable (≤10%) and an acceptable (≥30%) response rate to anakinra therapy. Subjects received 100 mg anakinra by subcutaneous injection for 84 days, followed by a 180-day observational period. RESULTS: Based on patient responses, the Simon 2-stage rule permitted premature termination of the trial after 10 subjects completed the 84-day drug period, as the target efficacy for the entire trial had been achieved. Of these 10 patients, 7 demonstrated audiometric improvement, as assessed by pure tone average (PTA) and word recognition score (WRS). In these 7 responders, reduced IL-1ß plasma levels correlated with clinical response. Upon discontinuation of treatment, 3 subjects relapsed, which correlated with increased IL-1ß plasma levels. CONCLUSION: We demonstrated that IL-1ß inhibition in corticosteroid-resistant AIED patients was effective in a small cohort of patients and that IL-1ß plasma levels associated with both clinical hearing response and disease relapse. These results suggest that a larger phase II randomized clinical trial of IL-1ß inhibition is warranted. TRIAL REGISTRATION: ClinicalTrials.gov NCT01267994. FUNDING: NIH, Merrill & Phoebe Goodman Otology Research Center, and Long Island Hearing & Speech Society.

Resident Research Experience and Career Path Association: A National Survey of Recent Otolaryngology Graduates.

OBJECTIVE: To determine whether the research rotation experience affects the career path of otolaryngology residents. STUDY DESIGN/SETTING: Two web-based surveys were disseminated by the AAO-HNS; one to current and former resident trainees and the other to current residency program directors. SUBJECTS AND METHODS: A web-based survey was disseminated to all AAO-HNS members classified as otolaryngology residents or residency graduates within the last 6 years, regarding their research rotation and its potential influence on their career path. A second web-based survey was delivered simultaneously to program directors to evaluate their perception of the need for research in a training program and their role in the rotation. Chi-square tests for independence as well as multivariate analyses were conducted to determine whether aspects of the resident research rotation related to career path. RESULTS: The resident survey was completed by 350 respondents (25% response rate), and 39 program directors completed the second survey (37% response rate). Multiple factors were examined, including federal funding of faculty, mentorship, publications prior to residency, success of research project measured by publication or grant submission, and type of research. Multivariate analyses revealed that factors most predictive of academic career path were intellectual satisfaction and presence of a T32 training grant within the program (P < .05). CONCLUSION: The composition and quality of the residency research rotation vary across institutions. Factors that enhance stronger intellectual satisfaction and the presence of T32 grant, which demonstrates an institution's commitment to research training, may promote pursuit of a career in academia versus private practice.

Otolaryngology resident selection: do rank lists matter?

OBJECTIVES: To examine the relationship between National Residency Matching Program (NRMP) rank list position and future otolaryngology residency performance. STUDY DESIGN: Cohort study. METHODS: Eight consecutive residency classes (starting 2001-2008; 4 residents/y) were reviewed. Three hundred and thirty-three applicants (40.6 applicants/y) were interviewed, and 316 (94.9%) were ranked. Residents matching with our program were divided 3 different ways: into quarters, thirds, and halves, based on their rank order. Correlation coefficients were obtained to compare resident rank number and rank group (quarter, third, half) to faculty evaluation, coresident evaluation, and in-service score. Chi-square tests were conducted comparing rank group to chief resident selection and annual teaching award. RESULTS: Resident NRMP rank number was not significantly correlated with faculty evaluation, coresident evaluation, or in-service exam score (-0.21 < r < 0.05; P > .28). There was also no significant correlation between resident quarter, third, or half rank group and faculty evaluation; coresident evaluation; or in-service exam score (-0.29 < r < 0.10; P > .13). Chi-square analysis found no relationship between resident rank group and chief resident (P > .35) or teaching award (P > .13) selection. CONCLUSIONS: Applicant rank number and rank group did not correlate with performance of this otolaryngology residency cohort as assessed by faculty evaluation, coresident evaluation, in-service exam score, or selection for chief resident or the annual teaching award. Resident selection committees should consider reallocating manpower hours from creating rank order to recruiting applicants and selecting interview candidates.

Mentoring in otolaryngology training programs.

OBJECTIVE: The Accreditation Council for Graduate Medical Education's focus on outcome-based training has made the mentoring process critical for resident education. It is unknown how otolaryngology training programs mentor residents. Our objective was to determine the current state of mentoring in otolaryngology training programs and describe resident perceptions of mentoring. STUDY DESIGN: Cross-sectional survey. SETTING: Accredited U.S. otolaryngology training programs. SUBJECTS AND METHODS: All U.S. otolaryngology residents and program directors were contacted via e-mail with a link to an online survey. RESULTS: Of the 1411 residents contacted, 27.7 percent responded, representing 71 of the 103 accredited otolaryngology programs. Of the 103 program directors contacted, 37.9 percent responded. Of these programs, 26 had formal mentoring programs, 45 did not have formal mentoring programs, and 12 programs were listed in both categories. Fifty-one percent of male residents and 49 percent of female residents had mentors. The most important mentor characteristics were personality match, good clinical role model, and similar subspecialty interests. Least important characteristics were race, gender, and age. Twenty-six percent of residents felt that mentoring was critical to their training, while 63 percent of residents listed mentoring as important but not critical. Programs with fewer faculty and residents were less likely to offer formal mentoring (P = 0.007 and 0.054, respectively). Of residents who did not have mentors, 80 percent lacked a mentor because their residency had no formal mentoring program. CONCLUSION: Residents perceive mentoring as important, and formal mentoring programs should be incorporated into otolaryngology training programs.

Jugular foramen fibromatosis in a 3-month-old male.

A 3-month-old male with a chief complaint of episodic choking with feeds and a hoarse cry is presented. Left eye ptosis and asymmetric soft palate elevation were detected on physical examination. Fiberoptic examination showed a left vocal fold paresis and pooling of secretions in the pyriform sinuses. MRI demonstrated an ill-defined lesion at the left jugular foramen extending into the left carotid sheath. A fine needle biopsy revealed spindle shaped cells consistent with fibromatosis. The histopathology of fibromatosis and the differential diagnosis of jugular foramen masses in children will be described. To our knowledge, this represents the earliest reported case of fibromatosis in the jugular foramen.

Pediatric hemophilic pseudotumor of the paranasal sinus.

OBJECTIVES/HYPOTHESIS: Hemophilic pseudotumors are rare clinical entities in otolaryngology. An unusual case of a pediatric hemophilic pseudotumor of the paranasal sinus in a previously undiagnosed hemophiliac is presented. STUDY DESIGN: Case report. METHODS: A 6-month-old, otherwise healthy boy was admitted for evaluation of a rapidly expanding left-sided cheek mass. The patient's initial presentation, imaging workup, and intraoperative and postoperative courses are discussed. RESULTS: Imaging workup revealed a large, heterogeneous, dense lesion in the patient's left-side maxillary sinus with distortion of the orbital floor and hard palate. Because of the concern for an aggressive malignancy, a biopsy was performed. After a Caldwell-Luc approach, a large amount of greenish fluid leaked from an otherwise empty maxillary sinus. The patient continued to bleed from his intraoral incision postoperatively and required two packed erythrocyte transfusions. Subsequent hematological testing led to a diagnosis of severe hemophilia type A with factor VIII levels less than 1% of normal. After treatment with recombinant coagulation factors hemostasis was achieved, and the patient clinically improved. CONCLUSION: The study presents the first report of a pediatric hemophilic pseudotumor in the paranasal sinuses leading to a diagnosis of hemophilia. It is a rare entity characterized by a cycle of repeated hemorrhage with subsequent encapsulation of the coagulum leading to pressure-induced distortion of adjacent bone. In the correct clinical setting, hemophilic pseudotumor should be considered in the differential diagnosis of a rapidly expanding mass in children.

Obstructive congenital epulis: prenatal diagnosis and perinatal management.

OBJECTIVE: To describe a multidisciplinary approach for delivery room management of congenital epulis. STUDY DESIGN: Case reports. METHODS: Reporting of two cases of congenital epulis and review of the literature. RESULTS: Antepartum ultrasonography demonstrated massive intraoral masses in two fetuses whereby concerns regarding the patency of the airway at birth necessitated development of a multidisciplinary team of maternal-fetal medicine, neonatal-perinatal medicine, anesthesiology, and otolaryngology. Surgical excision was performed before delivery in one infant and after complete delivery in the other without a need for endotracheal intubation and general anesthesia. Feeding was started early, and both infants were discharged after brief hospital stays. Pathological findings were consistent with congenital epulis. Differential diagnosis and options for surgical intervention are discussed, including ex utero intrapartum treatment. CONCLUSIONS: A multidisciplinary approach to antenatally identified congenital intraoral masses facilitates care at birth. Surgical treatment in this milieu may be simple and complete at the time of delivery.

Recurrent attacks of facial nerve palsy as the presenting sign of leukemic relapse.

OBJECTIVE: To present an unusual case of recurrent facial palsy resulting from acute leukemic infiltration of the parotid gland. STUDY DESIGN: Case report. METHODS: An 11-year-old boy who had been treated for acute lymphoblastic leukemia (ALL) from 3 to 6 years of age presented with intermittent left facial nerve palsy with concurrent ipsilateral parotid fullness. The initial findings at diagnosis and workup are presented, and the disease progression and resolution with therapy are documented. RESULTS: The patient had been off therapy when this finding developed. A workup for central and viral etiologies for the facial palsy was unrevealing. Biopsy of the parotid gland demonstrated a lymphoblastic leukemic infiltrate. The patient was placed on a chemotherapy protocol for relapsed leukemia, resulting in complete resolution of the facial palsy. CONCLUSION: Isolated facial nerve dysfunction, albeit rare, has been documented as a sign of central nervous system involvement in leukemia, but until now this presentation has not been described in the setting of leukemic relapse presenting with acute infiltration of the parotid gland.