Changing Management of Intravestibular Schwannomas in the Era of Cochlear Implantation for Single-Sided Deafness.

OBJECTIVE: Intralabyrinthine schwannomas (ILSs) are a rare cause of deafness. Patients with ILS confined to the semicircular canals and the vestibule (intravestibular schwannomas) are potential candidates for cochlear implantation for hearing rehabilitation, a new option for patients with unilateral hearing loss since the 2019 FDA approval of cochlear implant (CI) for single-sided deafness. In this report, we describe an evolving management approach for ILSs causing hearing loss. PATIENTS: Adults (≥18 years) who underwent simultaneous ILS resection and CI between January 2019 and June 2023 (n = 3). INTERVENTION: Transmastoid labyrinthectomy with simultaneous cochlear implantation. MAIN OUTCOME MEASURES: Hearing performance with cochlear implantation measured as CNC Word Recognition scores and AzBio Sentence scores. RESULTS: Three patients with ILS confined to the semicircular canals and vestibule underwent simultaneous tumor resection via labyrinthectomy with CI placement. In all cases, complete tumor resection and full CI insertion were achieved. No patients experienced postoperative complications. Patients 1 and 2 underwent 6- and 9-month postactivation testing, respectively, with CNC scores 64% to 80% and AzBio 81% to 99% in the implanted ears. Patient 3 scored 0% on CNC and AzBio testing at 3 months and deferred her 6-month audiometry. CONCLUSIONS: Patients with ILS confined to the vestibule and semicircular canals can be considered for simultaneous tumor resection and CI placement.

Role of the otologist/neurotologist in managing auricular and periauricular cutaneous malignancies: A 10-year otologic oncology experience.

Objective: Auricular/periauricular cutaneous malignancies can be challenging to manage surgically due to the complex anatomy of the region. Otologists/neurotologists have unique skillsets that are well-suited to surgically treat these patients. We aim to highlight the role of otologists and neurotologists in providing surgical care of patients with auricular and periauricular malignancies by describing the experience of a single fellowship-trained neurotologist over a 10-year period. Methods: Retrospective chart review of 387 patients with auricular and periauricular malignancy treated by a single neurotologist between 2012 and 2022 was completed. Tumor histology and procedures performed for each patient were extracted. Additional data was collected for a subset of 84 patients with complex cases requiring selective neck dissection, parotidectomy, lateral temporal bone resection, regional advancement or rotational flap reconstruction, and/or free tissue transfer reconstruction. Results: Within the series of 387 patients, squamous cell carcinoma was the most common histology (42.6%, n = 165), followed by basal cell carcinoma (40.8%, n = 158), and melanoma (9.8%, n = 38). Common surgical procedures included wide local excision (61.8%, n = 239), partial/sub-total auriculectomy 18.3% (n = 71), or total auriculectomy 5.2% (n = 20). Within the 84-patient subset, median age at diagnosis was 71.9 years. Dermatologists provided most patient referrals (50.0%, n = 42). Most common tumor locations included: auricular (58.3%, n = 49), pre-auricular (21.4%, n = 18), and parotid (27.4%, n = 23). Revision surgery occurred in 22.6% of cases (n = 19), of which 26.3% (n = 5) for positive margins and 31.6% (n = 6) for recurrence. Mean follow-up was 22.8 months. Disease-specific 5-year survival was 91%. Conclusions: We demonstrate the feasibility of an otologist/neurotologist incorporating the surgical management of auricular and periauricular malignancies into their practice. Level of Evidence: 4.

Paragangliomas of the head and neck: a contemporary review.

Head and neck paragangliomas (HNPGLs) are slow-growing, vascular, typically benign tumors whose growth may induce significant lower cranial nerve deficits. While most tumors arise sporadically, a significant portion is associated with defined genetic syndromes. While surgical resection has historically been the gold standard, management strategies have evolved with acknowledgement of high surgical morbidity, slow tumor growth rates, and technological advances. Conservative management approaches via observation and newer radiation therapy techniques have become more common. This review seeks to provide an update on contemporary management strategies for HNPGLs and future directions.

Targeted molecular characterization of external auditory canal squamous cell carcinomas.

HYPOTHESIS: Squamous cell carcinomas (SCC) of the external auditory canal (EAC) may harbor unique genomic alterations that may explain aggressive behavior and differentiate these tumors from cutaneous SCCs of other subsites. BACKGROUND: EAC SCCs arise in a non-ultraviolet-exposed region of the head and neck, are often locally aggressive and may metastasize to lymph nodes or distant sites. The genomic alterations underlying cutaneous SCC of other sites are well-documented; however, mutational profiles of EAC SCC are less well characterized and may contribute to the unique anatomic site, high rates of recurrence and tumor spread. We performed targeted sequencing of a cohort of primary EAC SCCs to identify recurring and potentially targetable genomic alterations. METHODS: Genomic DNA was extracted from formalin-fixed paraffin-embedded specimens of 7 EAC SCCs and subjected to targeted DNA sequencing using a 227-gene panel. Somatic alterations and gene copy number alterations were annotated using our validated, in-house bioinformatics pipelines. RESULTS: In our EAC SCCs, we found recurrent alterations in TP53 and genes of receptor tyrosine kinase (eg, EGFR, FGFR) and PI3K pathways (eg, PIK3CA), similar to cutaneous SCCs of other head and neck sites. We also observed a high frequency of telomerase reverse transcriptase amplification and DNA methyltransferase 1 alterations, both of which are rarely observed in cutaneous SCCs of other sites. CONCLUSION: These data represent the first step toward precise molecular characterization of EAC SCCs that may lead to an enhanced understanding of tumor biology and modernized precision medicine approaches for unique tumors.Level of Evidence: NA.

Re-evaluating the prevalence and factors characteristic of catecholamine secreting head and neck paragangliomas.

INTRODUCTION: We sought to characterize the prevalence and factors characteristic of head and neck paragangliomas (HNPGLs) that secrete catecholamines to inform best practices for diagnosis and management. METHODS: This was a retrospective cohort study from 2000 to 2020 at a single-institution tertiary centre. One-hundred fifty-two patients (182 tumours) with HNPGLs with at least one measurement of urine or plasma catecholamines and/or catecholamine metabolite levels prior to treatment were included. We differentiated and characterized those patients with increased level(s) of any nature and those with 'clinically significant' versus 'clinically insignificant' catecholamine production. RESULTS: Thirty-one (20.4%) patients had increased catecholamine and/or catecholamine metabolite levels. In most patients, these levels were ≤5-fold above the upper limit of the reference range. Four of these 31 patients with increased levels were ultimately found to have an additional catecholamine secreting mediastinal paraganglioma or pheochromocytoma. Fourteen of 31 patients with HNPGL were deemed clinically significant secretors of catecholamines based on hyper-adrenergic symptoms and/or profound levels of normetanephrines. This cohort was enriched for patients with paragangliomas of the carotid body or cervical sympathetic chain and those with SDHB genetic mutations. Ultimately, the prevalence of clinically significant catecholamine secreting Hangs was determined to be 9.2% and 7.7% based on a per-patient and per-tumour basis, respectively. CONCLUSIONS: The rate of catecholamine excess in the current cohort of patients with HNPGLs was higher than previously reported. Neuroendocrine tumours of any anatomic subsite may secrete catecholamines, although not all increased laboratory level(s) are indicative of clinically significant catecholamine secretion causing symptoms or warranting adrenergic blockade.

Clinical Practice Guideline: Ménière's Disease.

OBJECTIVE: Ménière's disease (MD) is a clinical condition defined by spontaneous vertigo attacks (each lasting 20 minutes to 12 hours) with documented low- to midfrequency sensorineural hearing loss in the affected ear before, during, or after one of the episodes of vertigo. It also presents with fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear. The underlying etiology of MD is not completely clear, yet it has been associated with inner ear fluid (endolymph) volume increases, culminating in episodic ear symptoms (vertigo, fluctuating hearing loss, tinnitus, and aural fullness). Physical examination findings are often unremarkable, and audiometric testing may or may not show low- to midfrequency sensorineural hearing loss. Conventional imaging, if performed, is also typically normal. The goals of MD treatment are to prevent or reduce vertigo severity and frequency; relieve or prevent hearing loss, tinnitus, and aural fullness; and improve quality of life. Treatment approaches to MD are many and typically include modifications of lifestyle factors (eg, diet) and medical, surgical, or a combination of therapies. PURPOSE: The primary purpose of this clinical practice guideline is to improve the quality of the diagnostic workup and treatment outcomes of MD. To achieve this purpose, the goals of this guideline are to use the best available published scientific and/or clinical evidence to enhance diagnostic accuracy and appropriate therapeutic interventions (medical and surgical) while reducing unindicated diagnostic testing and/or imaging.

Plain Language Summary: Ménière's Disease.

This plain language summary explains Ménière's (pronounced men-yerz) disease (MD) to patients. The summary applies to patients aged 18 years and older with a suspected diagnosis of definite or probable MD. It is based on the 2020 "Clinical Practice Guideline: Ménière's Disease." This guideline uses published research to best advise health care providers and patients on the history and physical examination of patients with MD and how to diagnose and treat them. The guideline includes recommendations that are explained in this summary. Recommendations may not apply to every patient but can be used to facilitate shared decision making between patients and their health care providers.

Clinical Practice Guideline: Ménière's Disease Executive Summary.

OBJECTIVE: Ménière's disease (MD) is a clinical condition defined by spontaneous vertigo attacks (each lasting 20 minutes to 12 hours) with documented low- to midfrequency sensorineural hearing loss in the affected ear before, during, or after one of the episodes of vertigo. It also presents with fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear. The underlying etiology of MD is not completely clear, yet it has been associated with inner ear fluid volume increases, culminating in episodic ear symptoms (vertigo, fluctuating hearing loss, tinnitus, and aural fullness). Physical examination findings are often unremarkable, and audiometric testing may or may not show low- to midfrequency sensorineural hearing loss. Imaging, if performed, is also typically normal. The goals of MD treatment are to prevent or reduce vertigo severity and frequency; relieve or prevent hearing loss, tinnitus, and aural fullness; and improve quality of life. Treatment approaches to MD are many, and approaches typically include modifications of lifestyle factors (eg, diet) and medical, surgical, or a combination of therapies. PURPOSE: The primary purpose of this clinical practice guideline is to improve the quality of the diagnostic workup and treatment outcomes of MD. To achieve this purpose, the goals of this guideline are to use the best available published scientific and/or clinical evidence to enhance diagnostic accuracy and appropriate therapeutic interventions (medical and surgical) while reducing unindicated diagnostic testing and/or imaging.

A Family With a Carotid Body Paraganglioma and Thyroid Neoplasias With a New SDHAF2 Germline Variant.

At least 30% of all pheochromocytomas (PCCs)/paragangliomas (PGLs) arise in patients with a germline predisposition syndrome. Variants in succinate dehydrogenase subunits A, B, C, and D (SDHA, SDHB, SDHC, and SDHD) are the most common pathogenic germline alterations. Few pathogenic variants have been reported in succinate dehydrogenase assembly factor 2 (SDHAF2). Here, we describe a 30-year-old female patient who presented with a left-sided neck mass, which was later characterized as a carotid body PGL. Genetic testing revealed a likely pathogenic SDHAF2 variant (c.347G>A;p.W116X). Two sisters carried the same pathologic variant, and screening protocols were recommended. Whole-body MRI revealed thyroid nodules; this testing was followed by fine-needle aspiration, which confirmed papillary thyroid carcinoma in one sister and a follicular adenoma in the other. The two sisters then underwent hemithyroidectomy and total thyroidectomy, respectively. Because evidence for pathogenic variants in SDHAF2 causing predisposition to PCC/PGL is limited, we discuss the challenges in mutational variant interpretation and decision making regarding screening for associated tumors.

Institutional Experience of Treatment and Outcomes for Cutaneous Periauricular Squamous Cell Carcinoma.

OBJECTIVES: To report our institutional experience, management, and outcomes of cutaneous periauricular squamous cell carcinoma (SCC). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary academic center. SUBJECTS: Patients undergoing treatment of cutaneous periauricular SCC from 2000 to 2016. RESULTS: A total of 112 patients had a median follow-up of 24.5 months, a mean ± SD age of 75.7 ± 10.6 years, and a strong male predominance (93.8%). Site distribution shows 87 (77.7%) auricular, 26 (23.2%) preauricular, and 10 (8.8%) postauricular lesions. Of auricular lesions, tumors involved the tragus (n = 3, 3.4%), helix/antihelix (n = 47, 54.0%), conchal bowl (n = 31, 35.6%), external auditory canal (n = 18, 16.1%), and lobule (n = 3, 3.4%). Most patients presented at stage I (52.7%) versus stages II (28.6%), III (6.3%), and IV (12.5%). Patients were largely treated surgically with primary tumor resection ranging from wide local excision to lateral temporal bone resection (± parotidectomy and neck dissection), with 17.0% and 5.4% receiving adjuvant radiation and chemoradiation, respectively. Metastatic spread was seen to the parotid (25.9%) and neck (26.8%), with most common cervical spread to level II. Overall survival, disease-specific survival, and disease-free survival at 3 years were 62%, 89%, and 56%, respectively. Nodal disease was associated with worse disease-specific survival (P < .001) and disease-free survival (P = .042). Pre- and postauricular sites were associated with worse overall survival (P = .007) relative to auricular sites. CONCLUSION: Among cutaneous SCC, periauricular subsites pose treatment challenges related to surrounding anatomy and represent a unique tumor population. The reported propensity toward recurrence and patterns of metastasis may better guide treatment of aggressive tumors to include regional nodal dissection.

Tinnitus Management in Lateral Skull Base Lesions.

Tinnitus, the phantom perception of sound in the absence of a physical sound source, is a complex problem with multiple etiologies. While most commonly presenting in a subjective fashion caused by measurable hearing loss, other etiologies including lateral skull base tumors that encroach on middle and inner ear structures can lead to phantom sound perception as well. In addition to discussing the basic background of tinnitus, here we also review current theories of etiology that include central auditory and nonauditory neural mechanisms and potential treatments that range from sound therapy to medications to cognitive and behavioral therapies and cranial nerve and brain stimulation. One main purpose of this article is to relate tinnitus causes to skull base tumors, surgical removal, and resultant sequelae, including damage to cranial nerves resulting in audiovestibular dysfunction. We also discuss the utility of microvascular decompression for both tumor and nontumor-associated tinnitus and the current literature regarding hearing preservation rates and tinnitus perception, where documented, with the three common treatment modalities employed for most lateral skull base tumors that includes watchful waiting with serial imaging, stereotactic radiosurgery and primary surgical resection using hearing preservation and hearing ablative approaches. The management of skull base tumors is a complex process that depending upon the approach and sequelae, may lead to manageable or worsening phantom sound perception that must be considered when discussing the multiple treatment options with patients.

Immediate and Long-term Hearing Outcomes With the Middle Cranial Fossa Approach for Vestibular Schwannoma Resection.

OBJECTIVE: To analyze the immediate postoperative and long-term hearing outcome data in patients who have undergone hearing preservation attempts with the middle cranial fossa (MCF) approach for the resection of sporadic vestibular schwannoma. STUDY DESIGN: Retrospective review of a surgical patient cohort. SETTING: Tertiary academic referral center. PATIENTS: Adult patients with unilateral sporadic vestibular schwannoma. INTERVENTION: Surgical treatment with a MCF approach. MAIN OUTCOME MEASURE: Comparison of pre- and postoperative audiometric data in accordance with the 1995 and 2012 American Academy of Otolaryngology/Head and Neck Surgery guidelines on reporting hearing outcomes and with a word recognition score only scale. Long-term hearing outcome data were analyzed in the following postoperative groups: 3 to 5, 6 to 8, 9 to 11, and 12+ years after surgery. RESULTS: From 1999 to 2016, 174 patients underwent the MCF approach and 155 of them met inclusion criteria for analysis of hearing outcome data. Class A or B hearing was preserved in 70% of the entire cohort after recovery. Seventy-one patients with measurable hearing met criteria for long-term data analysis. The rate of American Academy of Otolaryngology/Head and Neck Surgery class A or B hearing preservation was 82% at 3 to 5 years and declined thereafter. The rate of word recognition score class I or II hearing preservation was 98% at 3 to 5 years and declined less rapidly thereafter. Patients with preoperative Class A hearing had significantly higher rates of successful hearing preservation at all postoperative intervals. CONCLUSION: Delayed hearing loss occurs in a progressively increasing fashion but speech understanding remains durable for a majority of patients whose hearing is initially preserved with the MCF approach.

Superior Canal Dehiscence Syndrome Affecting 3 Families.

Importance: Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown. Objective: To describe 7 cases of SCDS across 3 families. Design, Setting, and Participants: This retrospective case series included 7 patients from 3 different families treated at a neurotology clinic at a tertiary academic medical center from 2010 to 2014. Patients were referred by other otolaryngologists or were self-referred. Each patient demonstrated unilateral or bilateral SCDS or near dehiscence. Interventions: Clinical evaluation involved body mass index calculation, audiometry, cervical vestibular evoked myogenic potential testing, electrocochleography, and multiplanar computed tomographic (CT) scan of the temporal bones. Zygosity testing was performed on twin siblings. Main Outcomes and Measures: The diagnosis of SCDS was made if bone was absent over the superior semicircular canal on 2 consecutive CT images, in addition to 1 physiologic sign consistent with labyrinthine dehiscence. Near dehiscence was defined as absent bone on only 1 CT image but with symptoms and at least 1 physiologic sign of labyrinthine dehiscence. Results: A total of 7 patients (5 female and 2 male; age range, 8-49 years) from 3 families underwent evaluation. Family A consisted of 3 adult first-degree relatives, of whom 2 were diagnosed with SCDS and 1 with near dehiscence. Family B included a mother and her child, both of whom were diagnosed with unilateral SCDS. Family C consisted of adult monozygotic twins, each of whom was diagnosed with unilateral SCDS. For all cases, dehiscence was located at the arcuate eminence. Obesity alone did not explain the occurrence of SCDS because 5 of the 7 cases had a body mass index (calculated as weight in kilograms divided by height in meters squared) less than 30.0. Conclusions and Relevance: Superior canal dehiscence syndrome is a rare, often unrecognized condition. This report of 3 multiplex families with SCDS provides evidence in support of a potential genetic contribution to the etiology. Symptomatic first-degree relatives of patients diagnosed with SCDS should be offered evaluation to improve detection of this disorder.

Clinical Practice Guideline: Improving Nasal Form and Function after Rhinoplasty.

Objective Rhinoplasty, a surgical procedure that alters the shape or appearance of the nose while preserving or enhancing the nasal airway, ranks among the most commonly performed cosmetic procedures in the United States, with >200,000 procedures reported in 2014. While it is difficult to calculate the exact economic burden incurred by rhinoplasty patients following surgery with or without complications, the average rhinoplasty procedure typically exceeds $4000. The costs incurred due to complications, infections, or revision surgery may include the cost of long-term antibiotics, hospitalization, or lost revenue from hours/days of missed work. The resultant psychological impact of rhinoplasty can also be significant. Furthermore, the health care burden from psychological pressures of nasal deformities/aesthetic shortcomings, surgical infections, surgical pain, side effects from antibiotics, and nasal packing materials must also be considered for these patients. Prior to this guideline, limited literature existed on standard care considerations for pre- and postsurgical management and for standard surgical practice to ensure optimal outcomes for patients undergoing rhinoplasty. The impetus for this guideline is to utilize current evidence-based medicine practices and data to build unanimity regarding the peri- and postoperative strategies to maximize patient safety and to optimize surgical results for patients. Purpose The primary purpose of this guideline is to provide evidence-based recommendations for clinicians who either perform rhinoplasty or are involved in the care of a rhinoplasty candidate, as well as to optimize patient care, promote effective diagnosis and therapy, and reduce harmful or unnecessary variations in care. The target audience is any clinician or individual, in any setting, involved in the management of these patients. The target patient population is all patients aged ≥15 years. The guideline is intended to focus on knowledge gaps, practice variations, and clinical concerns associated with this surgical procedure; it is not intended to be a comprehensive reference for improving nasal form and function after rhinoplasty. Recommendations in this guideline concerning education and counseling to the patient are also intended to include the caregiver if the patient is <18 years of age. Action Statements The Guideline Development Group made the following recommendations: (1) Clinicians should ask all patients seeking rhinoplasty about their motivations for surgery and their expectations for outcomes, should provide feedback on whether those expectations are a realistic goal of surgery, and should document this discussion in the medical record. (2) Clinicians should assess rhinoplasty candidates for comorbid conditions that could modify or contraindicate surgery, including obstructive sleep apnea, body dysmorphic disorder, bleeding disorders, or chronic use of topical vasoconstrictive intranasal drugs. (3) The surgeon, or the surgeon's designee, should evaluate the rhinoplasty candidate for nasal airway obstruction during the preoperative assessment. (4) The surgeon, or the surgeon's designee, should educate rhinoplasty candidates regarding what to expect after surgery, how surgery might affect the ability to breathe through the nose, potential complications of surgery, and the possible need for future nasal surgery. (5) The clinician, or the clinician's designee, should counsel rhinoplasty candidates with documented obstructive sleep apnea about the impact of surgery on nasal airway obstruction and how obstructive sleep apnea might affect perioperative management. (6) The surgeon, or the surgeon's designee, should educate rhinoplasty patients before surgery about strategies to manage discomfort after surgery. (7) Clinicians should document patients' satisfaction with their nasal appearance and with their nasal function at a minimum of 12 months after rhinoplasty. The Guideline Development Group made recommendations against certain actions: (1) When a surgeon, or the surgeon's designee, chooses to administer perioperative antibiotics for rhinoplasty, he or she should not routinely prescribe antibiotic therapy for a duration >24 hours after surgery. (2) Surgeons should not routinely place packing in the nasal cavity of rhinoplasty patients (with or without septoplasty) at the conclusion of surgery. The panel group made the following statement an option: (1) The surgeon, or the surgeon's designee, may administer perioperative systemic steroids to the rhinoplasty patient.

Clinical Practice Guideline: Improving Nasal Form and Function after Rhinoplasty Executive Summary.

Objective Rhinoplasty, a surgical procedure that alters the shape or appearance of the nose while preserving or enhancing the nasal airway, ranks among the most commonly performed cosmetic procedures in the United States, with >200,000 procedures reported in 2014. While it is difficult to calculate the exact economic burden incurred by rhinoplasty patients following surgery with or without complications, the average rhinoplasty procedure typically exceeds $4000. The costs incurred due to complications, infections, or revision surgery may include the cost of long-term antibiotics, hospitalization, or lost revenue from hours/days of missed work. The resultant psychological impact of rhinoplasty can also be significant. Furthermore, the health care burden from psychological pressures of nasal deformities/aesthetic shortcomings, surgical infections, surgical pain, side effects from antibiotics, and nasal packing materials must also be considered for these patients. Prior to this guideline, limited literature existed on standard care considerations for pre- and postsurgical management and for standard surgical practice to ensure optimal outcomes for patients undergoing rhinoplasty. The impetus for this guideline is to utilize current evidence-based medicine practices and data to build unanimity regarding the peri- and postoperative strategies to maximize patient safety and to optimize surgical results for patients. Purpose The primary purpose of this guideline executive summary is to provide evidence-based recommendations for clinicians who either perform rhinoplasty or are involved in the care of a rhinoplasty candidate, as well as to optimize patient care, promote effective diagnosis and therapy, and reduce harmful or unnecessary variations in care. The target audience is any clinician or individual, in any setting, involved in the management of these patients. The target patient population is all patients aged ≥15 years. The guideline is intended to focus on knowledge gaps, practice variations, and clinical concerns associated with this surgical procedure; it is not intended to be a comprehensive reference for improving nasal form and function after rhinoplasty. Recommendations in this guideline concerning education and counseling to the patient are intended to include the caregiver if the patient is <18 years of age. Action Statements The Guideline Development Group made the following recommendations: (1) Clinicians should ask all patients seeking rhinoplasty about their motivations for surgery and their expectations for outcomes, should provide feedback on whether those expectations are a realistic goal of surgery, and should document this discussion in the medical record. (2) Clinicians should assess rhinoplasty candidates for comorbid conditions that could modify or contraindicate surgery, including obstructive sleep apnea, body dysmorphic disorder, bleeding disorders, or chronic use of topical vasoconstrictive intranasal drugs. (3) The surgeon, or the surgeon's designee, should evaluate the rhinoplasty candidate for nasal airway obstruction during the preoperative assessment. (4) The surgeon, or the surgeon's designee, should educate rhinoplasty candidates regarding what to expect after surgery, how surgery might affect the ability to breathe through the nose, potential complications of surgery, and the possible need for future nasal surgery. (5) The clinician, or the clinician's designee, should counsel rhinoplasty candidates with documented obstructive sleep apnea about the impact of surgery on nasal airway obstruction and how obstructive sleep apnea might affect perioperative management. (6) The surgeon, or the surgeon's designee, should educate rhinoplasty patients before surgery about strategies to manage discomfort after surgery. (7) Clinicians should document patient satisfaction with their nasal appearance and with their nasal function at a minimum of 12 months after rhinoplasty. The guideline development group made recommendations against certain actions: (1) When a surgeon, or the surgeon's designee, chooses to administer perioperative antibiotics for rhinoplasty, he or she should not routinely prescribe antibiotic therapy for a duration >24 hours after surgery. (2) Surgeons should not routinely place packing in the nasal cavity of rhinoplasty patients (with or without septoplasty) at the conclusion of surgery. The panel group made the following statement an option: (1) The surgeon, or the surgeon's designee, may administer perioperative systemic steroids to the rhinoplasty patient.

Head and neck paragangliomas: A two-decade institutional experience and algorithm for management.

Objectives: Paragangliomas of the head and neck and cranial base are typically benign, slow-growing tumors arising within the jugular foramen, middle ear, carotid bifurcation, or vagus nerve proper. The objective of this study was to provide a comprehensive characterization of our institutional experience with clinical management of these tumors and posit an algorithm for diagnostic evaluation and treatment. Methods: This was a retrospective cohort study of patients undergoing treatment for paragangliomas of the head and neck and cranial base at our institution from 2000-2017. Data on tumor location, catecholamine levels, and specific imaging modalities employed in diagnostic work-up, pre-treatment cranial nerve palsy, treatment modality, utilization of preoperative angiographic embolization, complications of treatment, tumor control and recurrence, and hereditary status (ie, succinate dehydrogenase mutations) were collected and summarized. Results: The mean (SD) age of our cohort was 51.8 (±16.1) years with 123 (63.4%) female patients and 71 (36.6%) male patients. Catecholamine-secreting lesions were found in nine (4.6%) patients. Fifty-one patients underwent genetic testing, with mutations identified in 43 (20 SDHD, 13 SDHB, 7 SDHD, 1 SDHA, SDHAF2, and NF1). Observation with serial imaging, surgical extirpation, radiation, and stereotactic radiosurgery were variably employed as treatment approaches across anatomic subsites. Conclusion: An algorithmic approach to clinical management of these tumors, derived from our longitudinal institutional experience and current empiric evidence, may assist otolaryngologists, radiation oncologists, and geneticists in the care of these complex neoplasms. Level of Evidence: 4.

Comparison of second-echelon treatments for Ménière's disease.

IMPORTANCE: To compare the efficacy of treatments commonly offered to patients with Ménière's disease who fail conservative medical therapy including diuretics and a sodium-restricted diet. OBJECTIVES: This study compared three second-echelon treatments: the Meniett device, endolymphatic sac decompression, and intratympanic gentamicin injections to determine their comparative effectiveness and capacity to mitigate against the necessity of a surgical labyrinthectomy. DESIGN, SETTING, AND PARTICIPANTS: Retrospective observational study at an academic tertiary care center. Patients with Ménière's disease who failed primary medical management were evaluated after treatment with a Meniett device (n=20), endolymphatic sac decompression (n=23) or intratympanic gentamicin injections (n=17). Cases were included if auditory and vertigo control data were available before and a minimum of two years after treatment, in patients without previous otologic surgery or intratympanic injections. Average age ranged from 54 to 75 years. INTERVENTIONS: Use of the Meniett device, endolymphatic sac shunt decompression surgery or intratympanic gentamicin injections using variable doses and injection schedules. MAIN OUTCOMES AND MEASURES: Proportion of patients with vertigo control and hearing preservation by a modified version of the AAO-HNS criteria after second-echelon treatment, thus not requiring definitive labyrinthectomy. RESULTS: Despite endolymphatic sac surgery demonstrating a longer duration (61 months) prior to labyrinthectomy, no differences were found between the 3 treatment options in terms of patients going on to definitive labyrinthectomy or in the number of months of symptom relief following treatment. There was also no difference in residual auditory perception across the 3 groups. CONCLUSIONS AND RELEVANCE: No significant therapeutic differences were found between the studied second-echelon treatments for symptom relief of Ménière's disease.

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.

Transcanal labyrinthectomy for intractable vertigo after unilateral cochlear implantation.

OBJECTIVE: Document the use of transcanal labyrinthectomy to treat disabling attacks of vertigo after unilateral cochlear implantation. PATIENT: A 46-year-old woman with severe-profound bilateral sensorineural hearing loss secondary to enlarged vestibular aqueducts underwent cochlear implantation for her right ear with a Nucleus Freedom device. The surgery was uneventful, and postoperative imaging confirmed that the electrode was positioned properly. She developed episodic vertigo 10 to 14 days after the implant surgery, which failed to improve with aggressive vestibular rehabilitation therapy. Plugging of the round window for possible perilymphatic fistula did not relieve her symptoms. INTERVENTION: Right transcanal labyrinthectomy supplemented by filling the vestibule with gentamicin-soaked Gelfoam and then a customized vestibular rehabilitation program. MAIN OUTCOME MEASURE: Comparison of vestibular symptoms and cochlear implant performance before and after transcanal labyrinthectomy. RESULTS: The patient had immediate relief of symptoms, and the function of the cochlear implant was not adversely affected. CONCLUSION: Transcanal labyrinthectomy may be an effective method to ablate the vestibular end organ after unilateral cochlear implantation. It can offer relief of disabling vertigo without adversely affecting the performance of the implant.