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.

Reversing Synchronized Brain Circuits Using Targeted Auditory-Somatosensory Stimulation to Treat Phantom Percepts: A Randomized Clinical Trial.

Importance: Animal models have shown altered dorsal cochlear nucleus circuitry in animals that develop tinnitus; however, precise treatment using bisensory (auditory and somatosensory) stimuli can reverse altered neural patterns and lessen tinnitus. Objective: To confirm and extend the findings of a pilot study, which suggested an increased efficacy of bisensory stimulation, to a clinical trial with a greater duration and greater number of participants. Design, Setting, and Participants: This double-blind, crossover, single-center randomized clinical trial was conducted from March 2019, with a 3-month follow-up per participant ending in July 2022. Eligible adults were recruited from the University of Michigan Health System in Ann Arbor, Michigan. Eligibility criteria included bothersome tinnitus (Tinnitus Functional Index [TFI] score, ≥17 points), somatic tinnitus, normal to moderate hearing loss, and no other tinnitus treatments in the 6 months prior to the trial. Included participants were randomized to either treatment group 1, which received active (bisensory) treatment, or group 2, which received the control (auditory-only) treatment. Results were analyzed using intent-to-treat (ITT) and per protocol (PP) populations. Intervention: Precisely timed bisensory (combined auditory and somatosensory) treatment was delivered through a portable, custom, take-home device that was provided to each participant for daily, at-home treatments. Group 1 participants received 30 minutes per day of the bisensory treatment for 6 weeks, followed by a 6-week washout phase, and then 30 minutes per day of the auditory-only treatment followed by a second 6-week washout phase. Group 2 participants received the auditory-only treatment first, followed by a washout phase, and then the bisensory treatment followed by a second washout phase. Main Outcomes and Measures: Primary end points were changes in TFI score and tinnitus loudness level from baseline through week 6 and week 12. Results: Of 337 screened individuals, 99 (mean [SD] age, 47 [12.7] years; 59 males [60%]; 85 with non-Hispanic White [86%] race and ethnicity) were enrolled into the study and randomized to treatment group 1 (n = 49) or group 2 (n = 50). The active but not the control treatment resulted in clinically significant decreases in TFI scores at week 6 of phase 1 (ITT population: -12.0 [95% CI, -16.9 to -7.9] points; P < .001; PP population: -13.2 [95% CI, -16.0 to -10.5] points; P < .001). Decreases in tinnitus loudness level were greater than 6 dB sensation level (SL; >half as loud) at week 6 for the bisensory treatment group, with little effect for the auditory-only treatment control group at week 6 of phase 1 (ITT population: -5.8 [95% CI, -9.5 to -2.2] dB; P = .08; PP population: -7.2 [95% CI, -11.4 to -3.1] dB; P = .03), and up to 11 dB SL at week 12 of phase 2 (ITT population: -10.9 [95% CI, -15.2 to -6.5] dB; P = .001; PP population: -14.1 [95% CI, -18.4 to -9.8] dB; P < .001). Decreased tinnitus loudness level and TFI scores extended into the washout phase, indicating a prolonged treatment effect. Conclusions and Relevance: This trial found that precisely timed bisensory treatment using stimuli and timing developed in a validated animal model was effective for adults with somatic tinnitus. Prolonged reduction in tinnitus symptoms can result from using an extended treatment duration. Trial Registration: ClinicalTrials.gov Identifier: NCT03621735.

American Speech-Language-Hearing Association Clinical Practice Guideline on Aural Rehabilitation for Adults With Hearing Loss.

BACKGROUND: Hearing loss poses a significant public health challenge as a common chronic condition with many known side effects that are often worse when left untreated. Aural rehabilitation (AR) is an umbrella term that encompasses a range of interventions (e.g., informational counseling and perceptual training) designed to reduce deficits related to hearing loss that may stand alone or be used in combination as part of a holistic plan. PURPOSE: This evidence-based clinical practice guideline is intended to inform the implementation of person-centered AR to adults with hearing loss. Given the well-known benefits of sensory management, it was not included within the scope of this guideline. The recommendations aim to help clinicians, individuals with hearing loss, and other stakeholders make evidence-informed treatment decisions and improve clinical outcomes, as well as provide payers and policymakers with information detailing a comprehensive approach to AR. METHOD: The American Speech-Language-Hearing Association (ASHA) and a multidisciplinary panel of subject matter experts prioritized key clinical questions and outcomes that served as the foundation of the guideline. The clinical recommendations were based on a comprehensive systematic review and a meta-analysis of 85 studies published between 1978 and 2021. RECOMMENDATIONS: Given the current state of the evidence, resource considerations, patient acceptability, clinical feasibility, and the overall balance of benefits and harms, ASHA developed several evidence-based recommendations for the provision of AR to adults with hearing loss. Each recommendation is accompanied by a series of key practice points to support its implementation within a person-centered framework.

A Step towards Achieving Sustainable Otologic Surgery in Low-Resource Settings: A Cost Comparison between Shipping an Otologic versus Microscopic Surgical Setup.

Background: The advancement of otologic surgery in low-resource settings has been limited by the cost and transport of surgical equipment. This study compared the transportation costs of an otologic microscopic surgical setup (MSS) versus an endoscopic surgical setup (ESS) in low- and low to middle-income countries (LMICs) for surgical teaching. Methods: Dimensions of microscopes, endoscopes and associated surgical instruments were used to calculate shipping costs from Minneapolis, MN, USA to Kenya, Haiti and Sri Lanka. Results: The average cost of internationally shipping the ESS is less than the MSS in Kenya (ESS: USD 1344.03; MSS: USD 20,947.00; p = 0.370), Haiti (ESS: USD 549.11; MSS: USD 1679.00; p < 0.05) and Sri Lanka (ESS: USD 945.38; MSS: USD 8490.57; p = 0.377). Freight shipping was required for the MSS while the ESS can be packed into an international checked bag for USD 35.00 USD. Discussion: The ESS has fewer logistical barriers than the MSS, making the endoscope a feasible option for surgical teaching in LMICs.

Treatment and Prognosis of Inflammatory Pseudotumor of the Skull Base.

Background Inflammatory pseudotumor (IPT) of the skull base is a rare, locally destructive lesion managed with a variety of treatments. We explore the impact of treatment on outcome and assess the prognosis of IPT. Methods This is a retrospective review of IPT of the skull base at a tertiary academic medical center. The primary outcome was radiographic progression after treatment. Outcome versus tumor location was also examined and a prognostic model was developed using a logistic regression. Results The demographics of 21 patients with IPT are reported. Treatment consisted of corticosteroids (in 80.1% of patients), disease modifying antirheumatic drugs (DMARDs; 33.3%), surgical resection (28.6%), radiation (23.8%), antibiotics (14.3%), chemotherapy (rituximab; 9.5%), and antivirals (4.8%). At 50.7 months, 50.8% had radiographic progression. Local therapy trended toward having a better response than systemic therapy ( p = 0.60). IPT of the orbit required 2.4 treatment modalities, compared with 2.0 for pharyngeal IPT, and 1.3 for posterior skull base masses ( p = 0.14). A total of 75% orbital IPT underwent radiographic progression, compared with 71% of pharyngeal IPT and 50% of posterior skull base masses ( p = 0.62). Sixteen patients were used to create the logistic model of radiographic progression. The Cox-Snell R 2 was 0.71 ( p = 0.03). No individual variables were statistically significant. Conclusion To our knowledge, this is among the largest sample of cases describing the presentation, treatment, and prognosis of IPT of the skull base. Our data suggest that there may be an improved response with local therapy over systemic therapy and better prognosis among posterolateral skull base masses.

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.

Determining Cochlear Implant Candidacy in Adults: Limitations, Expansions, and Opportunities for Improvement.

The safety, efficacy, and success of cochlear implants (CIs) are well established and have led to changes in criteria used by clinicians to determine who should receive a CI. Such changes in clinical decision-making have out-paced the slower-occurring changes that have taken place with regulatory bodies' and insurers' indications. We review the historical development of indications for CIs, including those of the U.S. Food and Drug Administration (FDA), Medicare, Medicaid, and private insurers. We report on expansion to include patients with greater residual hearing, such as those who receive Hybrid and EAS devices, and report on recent FDA approvals that place less emphasis on the patient's best-aided condition and greater emphasis on the ear to be treated. This includes expansion of CIs to patients with single-side deafness and asymmetric hearing loss. We review changes in the test materials used to determine candidacy, including transition from sentences in quiet to sentences in noise to the recent use of monosyllabic words and cognitive screening measures. Importantly, we discuss the recent trend to recommend CIs despite a patient not meeting FDA or insurers' indications (a practice known as "off-label"), which serves as attestation that current indications need to be updated.

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.

Posttraumatic Dizziness: Navigating the Maze Towards Accurate Vestibular Diagnosis and Treatment.

OBJECTIVE: Highlight the importance of establishing a differential diagnosis to identify and treat multiple origins of dizziness in a patient following traumatic brain injury (TBI). PATIENT: 73-year-old man with TBI and temporal bone fracture developed posttraumatic bilateral multiple canal benign paroxysmal positional vertigo (BPPV). INTERVENTION: Multi-disciplinary diagnostic evaluation and vestibular rehabilitation (VR) treatment focused on canalith repositioning maneuvers (CRMs) and central adaptation. MAIN OUTCOME MEASURES: Diagnostic imaging, audiometric testing, clinical evaluation including video recordings of patterns of nystagmus, Dizziness Handicap Inventory (DHI). RESULTS: Systematic clinical examination identified multiple semicircular canal BPPV in addition to a suspected underlying unilateral hypofunction. Treatment focused on the appropriate CRMs and adaptation exercises. DHI scores improved significantly and patient returned to work and recreational activities. CONCLUSION: This Clinical Capsule Report highlights the importance of a comprehensive clinical evaluation of the TBI patient with dizziness when making an accurate diagnosis and treatment plan. Due to the complexity of differentiating between multiple canal BPPV in addition to other central and vestibular disorders, it is imperative for the clinician to have a clear understanding of nystagmus patterns for multicanal BPPV as well as other vestibular pathology.

Head and Neck Paragangliomas: Patterns of Otolaryngology Referrals for Genetic Testing Over 2 Decades.

OBJECTIVE: A large proportion of head and neck paragangliomas (HNPGLs) arise in patients with a genetic predisposition due to pathogenic variants in succinate dehydrogenase (SDHx) genes. Contemporary practice guidelines recommend consideration of referral for genetic testing for all patients with HNPGLs. We sought to assess adherence to these recommendations, factors associated with referral, and temporal trends in referral patterns by otolaryngologists over the past 2 decades. STUDY DESIGN: Retrospective cohort study. SETTING: Single tertiary care center. METHODS: All patients with newly diagnosed HNPGLs treated at a single academic center between 2000 and 2019 were included. Bivariable association of specific features of referral for genetic testing by treating surgeons were tested with χ2 and Wilcoxon rank-sum tests. Logistic regression was used to assess temporal trends in referral patterns overall and for specific clinical subgroups over time. RESULTS: Of 221 patients included, only 77 (34.8%) were referred for genetic testing. Factors associated with referral included young age, family history of paraganglioma, more recent year of diagnosis (ie, closer to study end date), tumor subsite (all P < .0001), and treatment by an otolaryngologist (vs vascular surgeon or neurosurgeon, P = .009). Overall, referral rates increased over time (P = .0002), but even in the most recent 5 years, only 51% of newly diagnosed patients were referred. CONCLUSION: Our analysis suggests that referral rates for genetic testing in patients with HNPGLs are growing yet are still largely based on young age, family history, and tumor subsite.

Tinnitus and auditory cortex; Using adapted functional near-infrared-spectroscopy to expand brain imaging in humans.

OBJECTIVES: Phantom sound perception (tinnitus) may arise from altered brain activity within auditory cortex. Auditory cortex neurons in tinnitus animal models show increased spontaneous firing rates. This may be a core characteristic of tinnitus. Functional near-infrared spectroscopy (fNIRS) has shown similar findings in human auditory cortex. Current fNIRS approaches with cap recordings are limited to ∼3 cm depth of signal penetration due to the skull thickness. To address this limitation, we present an innovative fNIRS approach via probes adapted to the external auditory canal. The adapted probes were placed deeper and closer to temporal lobe of the brain to bypass confining skull bone and improve neural recordings. METHODS: Twenty adults with tinnitus and 20 nontinnitus controls listened to periods of silence and broadband noise (BBN) during standard cap and adapted ear canal fNIRS neuroimaging. The evaluators were not blinded, but the protocol and postprocessing for the two groups were identical. RESULTS: Standard fNIRS measurements in participants with tinnitus revealed increased auditory cortex activity during silence that was suppressed during auditory stimulation with BBN. Conversely, controls displayed increased activation with noise but not during silence. Importantly, adapted ear canal fNIRs probes showed similar hemodynamic responses seen with cap probes in both tinnitus and controls. CONCLUSIONS: In this proof of concept study, we have successfully fabricated, adapted, and utilized a novel fNIRS technology that replicates established findings from traditional cap fNIRS probes. This exciting new innovation, validated by replicating previous and current cap findings in auditory cortex, may have applications to future studies to investigate brain changes not only in tinnitus but in other pathologic states that may involve the temporal lobe and surrounding brain regions. LEVEL OF EVIDENCE: NA.

Tinnitus and auditory cortex: using adapted functional near-infrared spectroscopy to measure resting-state functional connectivity.

OBJECTIVE: Tinnitus, phantom sound perception, arises from aberrant brain activity within auditory cortex. In tinnitus animal models, auditory cortex neurons show increased spontaneous firing and neural synchrony. In humans, similar hyperactivation in auditory cortex has been displayed with functional near-infrared spectroscopy (fNIRS). Resting-state functional connectivity (RSFC) or increased connectivity between brain regions has also been shown in tinnitus using fNIRS. However, current fNIRS technology utilizes infrared (IR)-sources and IR-detectors placed on the scalp that restricts (~3 cm depth IR penetration) signal capture to outer cerebral cortex due to skin and skull bone. To overcome this limitation, in this proof of concept study, we adapted fNIRS probes to fit in the external auditory canal (EAC) to physically place IR-probes deeper within the skull thereby extracting neural signals from deeper auditory cortex. METHODS: Twenty adults with tinnitus and 20 nontinnitus controls listened to periods of silence and broadband noise before and after 5 min of silence to calculate RSFC. Concurrent scalp probes over auditory cortex and an adapted probe placed in the right EAC were utilized. RESULTS: For standard probes, left and right auditory cortex in tinnitus showed increased RSFC to each other and to other nonauditory cortices. Interestingly, adapted fNIRS probes showed trends toward increased RSFC. CONCLUSION: While many areas for the adapted probes did not reach significance, these data using a highly innovative and newly created probe adapting fNIRS technology to the EAC substantiates our previously published data in human tinnitus and concurrently validates this technology as a useful and expanded brain imaging modality.

Treatment and Outcomes for Cutaneous Periauricular Basal Cell Carcinoma: A 16-Year Institutional Experience.

OBJECTIVE: To report a single institutional experience with the surgical management of cutaneous periauricular basal cell carcinoma. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary academic center. METHODS: Retrospective chart review of 71 patients diagnosed with periauricular basal cell carcinoma managed surgically from 2000 to 2016. Data were analyzed with descriptive statistics. RESULTS: The median age at diagnosis was 73.0 years (interquartile range, 13.0). Of all lesions, 2.8% (n = 2) were preauricular, 80.3% (n = 57) auricular, and 16.9% (n=12) postauricular. Auricular subsites included conchal bowl (36.6%, n = 26), helix (21.1%, n = 15), antihelix (1.4%, n = 1), peritragus (5.6%, n = 4), triangular fossa (1.4%, n = 1), external auditory canal (2.8%, n = 2), and lobule skin (1.4%, n = 1). Surgical approach included wide local excision (80.3%, n = 57), partial auriculectomy (8.5%, n = 6), and total auriculectomy or other combinations of surgical methods (11.3%, n = 8). Due to aggressive pathology, 3 cases required concurrent parotidectomy, neck dissection, ear canal sleeve resection, or mastoidectomy. In sum, 52.1% (n = 37) of cases had clear margins on first pass in the operating room; 25.4% (n = 18) required further resection; and 12.7% (n = 9) demonstrated final positive/overturned margins read as negative from the frozen sections. Reconstruction included full-thickness (25.4%, n = 18) or superficial-thickness (29.6%, n = 21) skin grafts and local flap reconstruction (25.4%, n = 18), while 5.6% (n = 4) required combinations of free flap and/or other reconstruction techniques; 14.1% (n = 10) did not undergo formal reconstruction. CONCLUSION: Periauricular basal cell carcinoma occurs in anatomically diverse locations in and around the ear, and multiple surgical methods are required for successful treatment.

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.