A Case of Respiratory Epithelial Adenomatoid Hamartoma (REAH) in a Patient with History of Radiation Exposure.

Respiratory epithelial adenomatoid hamartomas (REAHs) are becoming a more commonly recognized otolaryngologic tumor and are often misdiagnosed as inverted papilloma. Here, we present such a case in a patient with history of previous radiation exposure. Otolaryngologists and pathologists should be aware of the mucinous histological appearance of REAH to help differentiate from other growths. Given our patient's history, an association between REAH and previous radiation exposure is worth consideration.

Dopamine D1A directly interacts with otoferlin synaptic pathway proteins: Ca2+ and phosphorylation underlie an NSF-to-AP2mu1 molecular switch.

Dopamine receptors regulate exocytosis via protein-protein interactions (PPIs) as well as via adenylyl cyclase transduction pathways. Evidence has been obtained for PPIs in inner ear hair cells coupling D1A to soluble N-ethylmaleimide-sensitive factor (NSF) attachment protein receptor (SNARE)-related proteins snapin, otoferlin, N-ethylmaleimide-sensitive factor (NSF), and adaptor-related protein complex 2, mu 1 (AP2mu1), dependent on [Ca2+] and phosphorylation. Specifically, the carboxy terminus of dopamine D1A was found to directly bind t-SNARE-associated protein snapin in teleost and mammalian hair cell models by yeast two-hybrid (Y2H) and pull-down assays, and snapin directly interacts with hair cell calcium-sensor otoferlin. Surface plasmon resonance (SPR) analysis, competitive pull-downs, and co-immunoprecipitation indicated that these interactions were promoted by Ca2+ and occur together. D1A was also found to separately interact with NSF, but with an inverse dependence on Ca2+ Evidence was obtained, for the first time, that otoferlin domains C2A, C2B, C2D, and C2F interact with NSF and AP2mu1, whereas C2C or C2E do not bind to either protein, representing binding characteristics consistent with respective inclusion or omission in individual C2 domains of the tyrosine motif YXXΦ. In competitive pull-down assays, as predicted by KD values from SPR (+Ca2+), C2F pulled down primarily NSF as opposed to AP2mu1. Phosphorylation of AP2mu1 gave rise to a reversal: an increase in binding by C2F to phosphorylated AP2mu1 was accompanied by a decrease in binding to NSF, consistent with a molecular switch for otoferlin from membrane fusion (NSF) to endocytosis (AP2mu1). An increase in phosphorylated AP2mu1 at the base of the cochlear inner hair cell was the observed response elicited by a dopamine D1A agonist, as predicted.

Comparative analysis of quality-of-life metrics after endoscopic surgery for sinonasal neoplasms.

BACKGROUND: The objective of this study was to evaluate the postoperative quality of life (QOL) after endoscopic resection of sinus and skull base neoplasms using validated outcomes measures and to perform correlation of the various metrics to better discern their efficacy. Prospective QOL data collection and retrospective chart review were performed. METHODS: QOL data were collected using the 20-item Sino-Nasal Outcome Test (SNOT-20), Anterior Skull Base Questionnaire (ASBQ), European Quality-of-Life-5 Dimension (EQ-5D) questionnaire, and Lund-Kennedy endoscopic (LKE) score in 71 patients with sinonasal and skull base tumors. RESULTS: The mean age was 53 years and mean follow-up was 14.5 months at the time QOL data were collected. Benign and malignant tumors represented 39 (54.9%) and 32 (45.1%) cases, respectively. Twenty malignancies (62.5%) were stage T3 or T4, and 23 required postoperative chemotherapy and radiation (CRT). Factors indicating worsened postoperative QOL included malignant histopathology, T3 or T4 tumors, and the use of postoperative CRT (p < 0.05). There was a strong correlation of ASBQ with EQ-5D and SNOT-20 scores (r < -0.5) and a moderate correlation between the SNOT-20 and EQ-5D (r > 0.3), and the LKE had moderate correlation with SNOT-20 (r > 0.3) and weak correlation to the ASBQ (r > -0.3) and EQ-5D (r < 0.3). CONCLUSION: Patients who have undergone endoscopic resection of sinonasal tumors have quantifiable QOL changes as measured by various validated metrics. This study shows that concurrent use of these instruments may better discern QOL outcomes after endoscopic tumor surgery.

Section for Residents and Fellows-in-Training Leaders: Where Are They Now?

The purpose of this study was to examine the experiences of prior governing council (GC) members of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) Section for Residents and Fellows-in-Training (SRF) and assess the impact of early Academy involvement. A survey was conducted via email on all prior AAO-HNS SRF GC members. The AAO-HNS SRF has elected 52 GC members since its 2003 inception. Each member served an average of 1.5 year-long terms. The mean time since completion of training is 4.1 years. A subspecialty fellowship was pursued in 86%. Fifty-seven percent practice in academic settings, with 3 members advancing to subspecialty division director within their department. More than half (58%) have served on an AAO-HNS committee, and most are frequent attendees of the annual meeting. All prior members felt involvement in the SRF GC was beneficial, enabling them to gain leadership skills and deeper understanding of the specialty.

Clinical practice guideline: Bell's palsy.

OBJECTIVE: Bell's palsy, named after the Scottish anatomist, Sir Charles Bell, is the most common acute mono-neuropathy, or disorder affecting a single nerve, and is the most common diagnosis associated with facial nerve weakness/paralysis. Bell's palsy is a rapid unilateral facial nerve paresis (weakness) or paralysis (complete loss of movement) of unknown cause. The condition leads to the partial or complete inability to voluntarily move facial muscles on the affected side of the face. Although typically self-limited, the facial paresis/paralysis that occurs in Bell's palsy may cause significant temporary oral incompetence and an inability to close the eyelid, leading to potential eye injury. Additional long-term poor outcomes do occur and can be devastating to the patient. Treatments are generally designed to improve facial function and facilitate recovery. There are myriad treatment options for Bell's palsy, and some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, numerous diagnostic tests available are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy. PURPOSE: The primary purpose of this guideline is to improve the accuracy of diagnosis for Bell's palsy, to improve the quality of care and outcomes for patients with Bell's palsy, and to decrease harmful variations in the evaluation and management of Bell's palsy. This guideline addresses these needs by encouraging accurate and efficient diagnosis and treatment and, when applicable, facilitating patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell's palsy. The guideline is intended for all clinicians in any setting who are likely to diagnose and manage patients with Bell's palsy. The target population is inclusive of both adults and children presenting with Bell's palsy. ACTION STATEMENTS: The development group made a strong recommendation that (a) clinicians should assess the patient using history and physical examination to exclude identifiable causes of facial paresis or paralysis in patients presenting with acute-onset unilateral facial paresis or paralysis, (b) clinicians should prescribe oral steroids within 72 hours of symptom onset for Bell's palsy patients 16 years and older, (c) clinicians should not prescribe oral antiviral therapy alone for patients with new-onset Bell's palsy, and (d) clinicians should implement eye protection for Bell's palsy patients with impaired eye closure. The panel made recommendations that (a) clinicians should not obtain routine laboratory testing in patients with new-onset Bell's palsy, (b) clinicians should not routinely perform diagnostic imaging for patients with new-onset Bell's palsy, (c) clinicians should not perform electrodiagnostic testing in Bell's palsy patients with incomplete facial paralysis, and (d) clinicians should reassess or refer to a facial nerve specialist those Bell's palsy patients with (1) new or worsening neurologic findings at any point, (2) ocular symptoms developing at any point, or (3) incomplete facial recovery 3 months after initial symptom onset. The development group provided the following options: (a) clinicians may offer oral antiviral therapy in addition to oral steroids within 72 hours of symptom onset for patients with Bell's palsy, and (b) clinicians may offer electrodiagnostic testing to Bell's palsy patients with complete facial paralysis. The development group offered the following no recommendations: (a) no recommendation can be made regarding surgical decompression for patients with Bell's palsy, (b) no recommendation can be made regarding the effect of acupuncture in patients with Bell's palsy, and (c) no recommendation can be made regarding the effect of physical therapy in patients with Bell's palsy.

Clinical practice guideline: Bell's Palsy executive summary.

The American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) has published a supplement to this issue featuring the new Clinical Practice Guideline: Bell's Palsy. To assist in implementing the guideline recommendations, this article summarizes the rationale, purpose, and key action statements. The 11 recommendations developed encourage accurate and efficient diagnosis and treatment and, when applicable, facilitate patient follow-up to address the management of long-term sequelae or evaluation of new or worsening symptoms not indicative of Bell's palsy. There are myriad treatment options for Bell's palsy; some controversy exists regarding the effectiveness of several of these options, and there are consequent variations in care. In addition, there are numerous diagnostic tests available that are used in the evaluation of patients with Bell's palsy. Many of these tests are of questionable benefit in Bell's palsy. Furthermore, while patients with Bell's palsy enter the health care system with facial paresis/paralysis as a primary complaint, not all patients with facial paresis/paralysis have Bell's palsy. It is a concern that patients with alternative underlying etiologies may be misdiagnosed or have an unnecessary delay in diagnosis. All of these quality concerns provide an important opportunity for improvement in the diagnosis and management of patients with Bell's palsy.

Comparison of two minimally invasive techniques for treating chronic rhinosinusitis in the pediatric population.

OBJECTIVE: To compare two minimally invasive techniques for the treatment of chronic rhinosinusitis in young children. BACKGROUND: Chronic rhinosinusitis (CRS) is a common diagnosis in young children. Maxillary sinus aspiration & irrigation with adenoidectomy (MSI) followed by an extended course of oral antibiotics has been shown to be an alternative to functional endoscopic sinus surgery. However, since MSI is not performed under direct visualization, it has inherent risk. This study analyzes the techniques of MSI and endoscopically guided middle meatus cultures & antral biopsy with adenoidectomy (EGC) in the (1) diagnosis of bacterial infection by culture, (2) time to resolution using double antibiotic therapy, and (3) associated morbidity of the two procedures. METHODS: The medical records at Wayne State University, Department of Otolaryngology Head & Neck Surgery were reviewed from 2004 to 2010. All children who presented with CRS who underwent MSI or EGC were included in this retrospective case series. RESULTS: Patients presented with a history of cough, nasal discharge, and congestion. The mean age was 3.7 years. Symptom duration prior to treatment was 7.4 months in the 64 patients who underwent MSI and 9.1 months in the 46 patients who underwent EGC. MSI identified bacteria in 80% of patients compared to 73% in EGC patients (p=0.45). The MSI group underwent antibiotic treatment for 8.7 weeks and achieved symptom resolution in 8.7 weeks compared to 6.9 weeks and 4.9 weeks respectively in the EGC group (p=0.08 and 0.01). However, if patients presented with snoring or cough, time to resolution of symptoms was significantly lower in patients undergoing EGC versus MSI (p=0.02 and p=0.01, respectively). One patient who underwent MSI experienced epistaxis requiring nasal packing, and two patients had pseudoproptosis following irrigation that resolved spontaneously shortly thereafter. No complications were reported in the EGC group. CONCLUSION: EGC is an effective treatment for young children with CRS. EGC and MSI are equally effective in obtaining diagnostic cultures. EGC decreases time to symptom resolution, and it lowers the risk of complication when compared to MSI.

Frozen-section biopsy analysis for acute invasive fungal rhinosinusitis.

OBJECTIVE: To evaluate sensitivity and specificity of frozen-section biopsy in the diagnosis of acute invasive fungal rhinosinusitis (AIFRS). STUDY DESIGN AND SETTING: Retrospective chart review of all patients treated for AIFRS at the University of Miami between 1993 and 2005. Twenty patients with the clinical diagnosis of AIFRS were identified. Histopathological data were collected to evaluate the use of frozen-section biopsy to diagnose the disease. RESULTS: Permanent pathology sections were positive for AIFRS in all specimens. Frozen-section biopsies were positive for fungal features in all but five cases (four with Mucor and one with Aspergillus). High positive predictive values were found for both fungal types and for both sets of patients (bedside and intraoperative). CONCLUSION: Frozen-section biopsy is a useful tool for rapid and effective diagnosis in patients with suspected AIFRS. A positive frozen-section result is highly predictive of invasive disease and merits prompt surgical intervention.