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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.

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.

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.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.

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.

BackgroundPaediatric hearing loss rates in Ghana are currently unknown.MethodsA cross-sectional study was conducted in peri-urban Kumasi, Ghana; children (aged 3–15 years) were recruited from randomly selected households. Selected children underwent otoscopic examination prior to in-community pure tone screening using the portable ShoeBox audiometer. The LittlEars auditory questionnaire was also administered to caregivers and parents.ResultsData were collected from 387 children. After conditioning, 362 children were screened using monaural pure tones presented at 25 dB. Twenty-five children could not be conditioned to behavioural audiometric screening. Eight children were referred based on audiometric screening results. Of those, four were identified as having hearing loss. Four children scored less than the maximum mark of 35 on the LittleEars questionnaire. Of those, three had hearing loss as identified through pure tone screening. The predominant physical finding on otoscopy was ear canal cerumen impaction.ConclusionPaediatric hearing loss is prevalent in Ghana, and should be treated as a public health problem warranting further evaluation and epidemiology characterisation.

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.

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 χ 2 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.

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.

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.

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.

Background Peripheral nerve sheath myxomas are benign tumors which primarily occur on the central face, arms, and shoulders. Here we present a rare case of nerve sheath myxoma in the external auditory canal (EAC) and review the differential diagnosis, relevant histopathological characteristics of this tumor, and considerations for management including workup for the associated Carney complex, an autosomal dominant disorder characterized by cardiac myxomas, endocrine tumors involving the thyroid and pituitary gland, and melanotic schwannomas. Case description A healthy 18-year-old male presented with an isolated soft-tissue mass in the right EAC. Otoscopic examination revealed a subepithelial 1.5‐cm soft-tissue mass within the lateral anterosuperior EAC. Audiometric testing for the right ear demonstrated a mild low frequency conductive loss with type C tympanogram. Contrasted computed tomography of the temporal bones revealed an ill-defined partially exophytic soft tissue density/mass in the right anterior lateral EAC. The patient subsequently underwent wide local excision and partial ear canal sleeve resection. Final pathology revealed peripheral nerve sheath myxoma and appropriate genetic and endocrine workup for Carney complex was performed. Conclusions This case highlights the value of thorough diagnosis and management of neoplasms of the EAC. Nerve sheath myxoma is an important consideration for otolaryngologists when evaluating EAC lesions given the risk of systemic involvement with high associated morbidity when associated with Carney complex. Appropriate syndromic workup and close follow-up for significant risk of recurrence is warranted.

This plain language summary explains Meniere'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: Meniere'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.

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.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.

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.

Objective Functional near-infrared spectroscopy (fNIRS) is an emerging noninvasive technology used to study cerebral cortex activity. Being virtually silent and compatible with cochlear implants has helped establish fNIRS as an important tool when investigating auditory cortex as well as cortices involved with hearing and language processing in adults and during child development. With respect to this review article, more recently, fNIRS has also been used to investigate central auditory plasticity following hearing loss and tinnitus or phantom sound perception. Methods Here, we review the currently available literature reporting the use of fNIRS in human studies with cochlear implants and tinnitus to measure human central auditory cortical circuits. We also provide the reader with detailed reviews of the technology and traditional recording paradigms/methods used in these auditory-based studies. Results The purpose of this review article is to summarize theoretical advancements in our understanding of the neurocognitive mechanisms underlying auditory processes and their plasticity through fNIRS research of human auditory performance with cochlear implantation and plasticity that may contribute to the central percepts of tinnitus. Conclusion fNIRS is an emerging noninvasive brain imaging technology that has wide reaching application that can be applied to human studies involving cochlear implants and tinnitus. Level of evidence N/A.

While cerebral arteriovenous malformations (AVM) are common, a presentation of this vascular lesion involving the internal auditory canal (IAC) is rare. We describe a case of an un-ruptured AVM within the right IAC of a 55-year-old male who presented following a whiplash injury and left-ear (contralateral to the lesion) unilateral non-pulsatile tinnitus. On magnetic resonance imaging (MRI), an incidental atypical linear enhancement suspicious for a vascular lesion within the right IAC was identified and investigated further using magnetic resonance angiography and venography (MRA/MRV), which illustrated serpentine flow voids that confirmed the presence of an AVM. Given the patient's intact hearing and rare location of the AVM, treatment options including watchful waiting, stereotactic radiosurgery, embolization, and microsurgery were discussed, of which observation was chosen.

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.

We previously demonstrated that bimodal stimulation (spinal trigeminal nucleus [Sp5] paired with best frequency tone) altered neural tone-evoked and spontaneous firing rates (SFRs) in primary auditory cortex (A1) 15 min after pairing in guinea pigs with and without noise-induced tinnitus. Neural responses were enhanced (+10 ms) or suppressed (0 ms) based on the bimodal pairing interval. Here we investigated whether bimodal stimulation leads to long-term (up to 2 h) changes in tone-evoked and SFRs and neural synchrony (correlate of tinnitus) and if the long-term bimodal effects are altered following noise exposure. To obviate the effects of permanent hearing loss on the results, firing rates and neural synchrony were measured three weeks following unilateral (left ear) noise exposure and a temporary threshold shift. Simultaneous extra-cellular single-unit recordings were made from contralateral (to noise) A1 and dorsal rostral belt (RB); an associative auditory cortical region thought to influence A1, before and after bimodal stimulation (pairing intervals of 0 ms; simultaneous Sp5-tone and +10 ms; Sp5 precedes tone). Sixty and 120 min after 0 ms pairing tone-evoked and SFRs were suppressed in sham A1; an effect only preserved 120 min following pairing in noise. Stimulation at +10 ms only affected SFRs 120 min after pairing in sham and noise-exposed A1. Within sham RB, pairing at 0 and +10 ms persistently suppressed tone-evoked and SFRs, while 0 ms pairing in noise markedly enhanced tone-evoked and SFRs up to 2 h. Together, these findings suggest that bimodal stimulation has long-lasting effects in A1 that also extend to the associative RB that is altered by noise and may have persistent implications for how noise damaged brains process multi-sensory information. Moreover, prior to bimodal stimulation, noise damage increased neural synchrony in A1, RB and between A1 and RB neurons. Bimodal stimulation led to persistent changes in neural synchrony in intact A1 and RB that were also altered by noise-exposure. Given that increases in neural synchrony following noise may be consistent with tinnitus onset, these data implicate that both A1 and RB may be involved in the etiology of phantom sound perception. These data also suggest that noise alters the effects of bimodal stimulation on neural synchrony in A1 and RB; an effect that may also lead to changes in tinnitus perception.

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

© Official journal of the American Academy of Otolaryngology-Head and Neck Surgery Foundation 2016. 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 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.

Objective Functional near‐infrared spectroscopy (fNIRS) is an emerging noninvasive technology used to study cerebral cortex activity. Being virtually silent and compatible with cochlear implants has helped establish fNIRS as an important tool when investigating auditory cortex as well as cortices involved with hearing and language processing in adults and during child development. With respect to this review article, more recently, fNIRS has also been used to investigate central auditory plasticity following hearing loss and tinnitus or phantom sound perception. Methods Here, we review the currently available literature reporting the use of fNIRS in human studies with cochlear implants and tinnitus to measure human central auditory cortical circuits. We also provide the reader with detailed reviews of the technology and traditional recording paradigms/methods used in these auditory‐based studies. Results The purpose of this review article is to summarize theoretical advancements in our understanding of the neurocognitive mechanisms underlying auditory processes and their plasticity through fNIRS research of human auditory performance with cochlear implantation and plasticity that may contribute to the central percepts of tinnitus. Conclusion fNIRS is an emerging noninvasive brain imaging technology that has wide reaching application that can be applied to human studies involving cochlear implants and tinnitus. Level of Evidence N/A

BackgroundMillions of people across the world suffer from disabling hearing loss. Appropriate interventions lead to improved speech and language skills, educational advancement, and improved social integration. A major limitation to improving care is identifying those with disabling hearing loss in low-resource countries.ObjectivesThis review article summarises information on currently available hearing screening platforms and technology available from published reports and the authors’ personal experiences of hearing loss identification in low-resource areas of the world. The paper reviews the scope and capabilities of portable hearing screening platforms, including the pros and cons of each technology and how they have been utilised in low-resource environments.ConclusionPortable hearing screening tools are readily available to assess hearing loss in low-resource areas. Each technology has advantages and limitations that should be considered when identifying the optimal methods to assess needs in each country.

Central auditory circuits are influenced by the somatosensory system, a relationship that may underlie tinnitus generation. In the guinea pig dorsal cochlear nucleus (DCN), pairing spinal trigeminal nucleus (Sp5) stimulation with tones at specific intervals and orders facilitated or suppressed subsequent tone-evoked neural responses, reflecting spike timing-dependent plasticity (STDP). Furthermore, after noise-induced tinnitus, bimodal responses in DCN were shifted from Hebbian to anti-Hebbian timing rules with less discrete temporal windows, suggesting a role for bimodal plasticity in tinnitus. Here, we aimed to determine if multisensory STDP principles like those in DCN also exist in primary auditory cortex (A1), and whether they change following noise-induced tinnitus. Tone-evoked and spontaneous neural responses were recorded before and 15 min after bimodal stimulation in which the intervals and orders of auditory-somatosensory stimuli were randomized. Tone-evoked and spontaneous firing rates were influenced by the interval and order of the bimodal stimuli, and in sham-controls Hebbian-like timing rules predominated as was seen in DCN. In noise-exposed animals with and without tinnitus, timing rules shifted away from those found in sham-controls to more anti-Hebbian rules. Only those animals with evidence of tinnitus showed increased spontaneous firing rates, a purported neurophysiological correlate of tinnitus in A1. Together, these findings suggest that bimodal plasticity is also evident in A1 following noise damage and may have implications for tinnitus generation and therapeutic intervention across the central auditory circuit.

A small sub-population of cells characterized by increased tumorigenic potential, ability to self-renew and to differentiate into cells that make up the tumor bulk, has been characterized in some (but not all) tumor types. These unique cells, namedcancer stem cells, are considered drivers of tumor progression in these tumors. The purpose of this work is to understand if cancer stem cells play a functional role in the tumorigenesis of salivary gland mucoepidermoid carcinomas. Here, we investigated the expression of putative cancer stem cell markers (ALDH, CD10, CD24, CD44) in primary human mucoepidermoid carcinomas by immunofluorescence, in vitro salisphere assays, and in vivo tumorigenicity assays in immunodeficient mice. Human mucoepidermoid carcinoma cells (UM-HMC-1, UM-HMC-3A, UM-HMC-3B) sorted for high levels of ALDH activity and CD44 expression (ALDHhighCD44high) consistently formed primary and secondary salispheres in vitro, and showed enhanced tumorigenic potential in vivo (defined as time to tumor palpability, tumor growth after palpability), when compared to ALDHlowCD44low cells. Cells sorted for CD10/CD24, and CD10/CD44 showed varying trends of salisphere formation, but consistently low in vivo tumorigenic potential. And finally, cells sorted for CD44/CD24 showed inconsistent results in salisphere formation and tumorigenic potential assays when different cell lines were evaluated. Collectively, these data demonstrate that salivary gland mucoepidermoid carcinomas contain a small population of cancer stem cells with enhanced tumorigenic potential and that are characterized by high ALDH activity and CD44 expression. These results suggest that patients with mucoepidermoid carcinoma might benefit from therapies that ablate these highly tumorigenic cells.

The dorsal cochlear nucleus is the first site of multisensory convergence in mammalian auditory pathways. Principal output neurons, the fusiform cells, integrate auditory-nerve inputs from the cochlea with somatosensory inputs from the head and neck. In previous work, we developed a guinea pig model of tinnitus produced by noise exposure and showed that the fusiform cells in these animals exhibited increased spontaneous activity and cross-unit synchrony, which are physiological correlates of tinnitus. Here, we delivered repeated bimodal auditory-somatosensory stimulation to the dorsal cochlear nucleus of guinea pigs with tinnitus, choosing a stimulus interval known to induce long-term depression (LTD). Twenty minutes per day of LTD-targeting bimodal (but not unimodal) stimulation reduced physiological and behavioral evidence of tinnitus in the guinea pigs after 25 days. Next, we applied the same bimodal treatment to 20 human subjects with tinnitus using a double-blinded, sham-controlled, crossover study. Twenty-eight days of LTD-targeted bimodal stimulation reduced tinnitus loudness and intrusiveness. Unimodal auditory stimulation did not deliver either benefit. Bimodal auditory-somatosensory stimulation that targets LTD in the dorsal cochlear nucleus may hold promise for suppressing chronic tinnitus, which reduces quality of life for millions of tinnitus sufferers worldwide.

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.

Tinnitus, or phantom sound perception, leads to increased spontaneous neural firing rates and enhanced synchrony in central auditory circuits in animal models. These putative physiologic correlates of tinnitus to date have not been well translated in the brain of the human tinnitus sufferer. Using functional near-infrared spectroscopy (fNIRS) we recently showed that tinnitus in humans leads to maintained hemodynamic activity in auditory and adjacent, non-auditory cortices. Here we used fNIRS technology to investigate changes in resting state functional connectivity between human auditory and non-auditory brain regions in normal-hearing, bilateral subjective tinnitus and controls before and after auditory stimulation. Hemodynamic activity was monitored over the region of interest (primary auditory cortex) and non-region of interest (adjacent non-auditory cortices) and functional brain connectivity was measured during a 60-second baseline/period of silence before and after a passive auditory challenge consisting of alternating pure tones (750 and 8000Hz), broadband noise and silence. Functional connectivity was measured between all channel-pairs. Prior to stimulation, connectivity of the region of interest to the temporal and fronto-temporal region was decreased in tinnitus participants compared to controls. Overall, connectivity in tinnitus was differentially altered as compared to controls following sound stimulation. Enhanced connectivity was seen in both auditory and non-auditory regions in the tinnitus brain, while controls showed a decrease in connectivity following sound stimulation. In tinnitus, the strength of connectivity was increased between auditory cortex and fronto-temporal, fronto-parietal, temporal, occipito-temporal and occipital cortices. Together these data suggest that central auditory and non-auditory brain regions are modified in tinnitus and that resting functional connectivity measured by fNIRS technology may contribute to conscious phantom sound perception and potentially serve as an objective measure of central neural pathology.

Tinnitus is the perception of sound in the absence of a physical sound stimulus. It is thought to arise from aberrant neural activity within central auditory pathways that may be influenced by multiple brain centers, including the somatosensory system. Auditory-somatosensory (bimodal) integration occurs in the dorsal cochlear nucleus (DCN), where electrical activation of somatosensory regions alters pyramidal cell spike timing and rates of sound stimuli. Moreover, in conditions of tinnitus, bimodal integration in DCN is enhanced, producing greater spontaneous and sound-driven neural activity, which are neural correlates of tinnitus. In primary auditory cortex (A1), a similar auditory-somatosensory integration has been described in the normal system (Lakatos et al., 2007), where sub-threshold multisensory modulation may be a direct reflection of subcortical multisensory responses (Tyll et al., 2011). The present work utilized simultaneous recordings from both DCN and A1 to directly compare bimodal integration across these separate brain stations of the intact auditory pathway. Four-shank, 32-channel electrodes were placed in DCN and A1 to simultaneously record tone-evoked unit activity in the presence and absence of spinal trigeminal nucleus (Sp5) electrical activation. Bimodal stimulation led to long-lasting facilitation or suppression of single and multi-unit responses to subsequent sound in both DCN and A1. Immediate (bimodal response) and long-lasting (bimodal plasticity) effects of Sp5-tone stimulation were facilitation or suppression of tone-evoked firing rates in DCN and A1 at all Sp5-tone pairing intervals (10, 20, and 40 ms), and greater suppression at 20 ms pairing-intervals for single unit responses. Understanding the complex relationships between DCN and A1 bimodal processing in the normal animal provides the basis for studying its disruption in hearing loss and tinnitus models. This article is part of a Special Issue entitled: Tinnitus Neuroscience.

The use of cochlear implantation to treat sensorineural hearing loss continues to evolve as an increasing number of both adult and pediatric patients undergo this life-changing surgery. As our understanding of electrical stimulation of the auditory system unfolds, the importance of proper cochleostomy placement and intracochlear electrode positioning continues to evolve. Currently, atraumatic intracochlear electrode array implantation into scala tympani appears to optimize performance while providing the opportunity for hearing preservation for the purposes of bimodal stimulation. With these objectives in mind, this article describes the authors' surgical approach to gain access to scala tympani with the intent of minimizing trauma to the underlying structures. This approach is based on the results of multiple clinical and anatomical studies as well as on data from various temporal bone experiments. Based herein, the authors perform either direct round window insertions or create a round window-related cochleostomy either with or without a bony partition. Blind drilling procedures on the convexity of the promontory are avoided because this can result in either scala vestibuli access or substantial intracochlear damage.

Sensorineural hearing loss during early childhood alters auditory cortical evoked potentials in humans and profoundly changes auditory processing in hearing-impaired animals. Multiple mechanisms underlie the early postnatal establishment of cortical circuits, but one important set of developmental mechanisms relies on the neuromodulator serotonin (5-hydroxytryptamine [5-HT]). On the other hand, early sensory activity may also regulate the establishment of adultlike 5-HT receptor expression and function. We examined the role of 5-HT in auditory cortex by first investigating how 5-HT neurotransmission and 5-HT2 receptors influence the intrinsic excitability of layer II/III pyramidal neurons in brain slices of primary auditory cortex (A1). A brief application of 5-HT (50 μM) transiently and reversibly decreased firing rates, input resistance, and spike rate adaptation in normal postnatal day 12 (P12) to P21 rats. Compared with sham-operated animals, cochlear ablation increased excitability at P12–P21, but all the effects of 5-HT, except for the decrease in adaptation, were eliminated in both sham-operated and cochlear-ablated rats. At P30–P35, cochlear ablation did not increase intrinsic excitability compared with shams, but it did prevent a pronounced decrease in excitability that appeared 10 min after 5-HT application. We also tested whether the effects on excitability were mediated by 5-HT2 receptors. In the presence of the 5-HT2-receptor antagonist, ketanserin, 5-HT significantly decreased excitability compared with 5-HT or ketanserin alone in both sham-operated and cochlear-ablated P12–P21 rats. However, at P30–P35, ketanserin had no effect in sham-operated and only a modest effect cochlear-ablated animals. The 5-HT2-specific agonist 5-methoxy- N, N-dimethyltryptamine also had no effect at P12–P21. These results suggest that 5-HT likely regulates pyramidal cell excitability via multiple receptor subtypes with opposing effects. These data also show that early sensorineural hearing loss affects the ability of 5-HT receptor activation to modulate A1 pyramidal cell excitability.

The optimal treatment for bilateral hearing loss continues to evolve as cochlear implant (CI) and hearing aid technologies advance, as does our understanding of the central auditory system. Ongoing discussions continue on the validity and feasibility of bilateral CI in terms of performance, justification of need, medical/surgical safety concerns, and economics. The purpose of this review article is to provide an update on the advantages and disadvantages of bilateral CI and to provide a discussion on timing (simultaneous vs. sequential), technology (bimodal vs. binaural) and feasibility. Binaural advantages are found in both adult and pediatric bilateral CI recipients, the greatest being the head shadow effect and improvements in localization and loudness summation. This theoretically offers an advantage over their unilateral implanted counterparts in terms of improved sound localization and enhanced speech perception under noisy conditions. Most investigators agree that bilateral stimulation during critical periods of development is paramount for optimizing auditory functioning in children. Currently, bilateral CI is widely accepted as a safe and effective means of bilateral auditory stimulation.

Previous studies from our laboratory have demonstrated that in an animal model of acute cervical spinal cord injury (SCI), respiratory function can be restored by theophylline. We also have shown that respiratory recovery occurs spontaneously after prolonged postinjury survival periods when a hemidiaphragm is paralyzed by an ipsilateral upper cervical (C2) spinal cord hemisection. Theophylline mediates functional recovery by central nervous system adenosine A1 receptor antagonism; however, it is unclear whether adenosine receptors are altered after prolonged postinjury periods and whether theophylline can further enhance restored respiratory function that occurs spontaneously.To assess putative effects of systemic theophylline administration on further enhancing spontaneous respiratory muscle recovery 4 months after C2 hemisection in rats and to determine whether adenosine A1 receptor mRNA expression is altered in these animals.Electrophysiologic assessment of respiratory activity in the phrenic nerves was conducted in C2 hemisected rats 4 months after hemisection under standardized conditions. Immediately thereafter, rats were killed and the cervical spinal cords were prepared for adenosine A1 receptor mRNA expression by in situ hybridization.Spontaneous recovery of respiratory activity in the ipsilateral phrenic nerve was detected in a majority (15/20) of C2 hemisected animals and amounted to 44.06% +/- 2.38% when expressed as a percentage of activity in the homolateral phrenic nerve in noninjured animals. At the optimal dosage used in the acute studies, theophylline (15 mg/kg) did not enhance, but rather unexpectedly blocked, recovered respiratory activity in 4 out of 5 animals tested. At dosages of 5 mg/kg and 2.5 mg/kg, the drug blocked recovered respiratory activity in 3 out of 4 and 3 out of 5 animals tested, respectively. Quantitative analysis of adenosine A1 receptor mRNA expression did not reveal a significant difference between experimental animals and sham-operated animals.The blockade or attenuation of spontaneously recovered respiratory activity following theophylline administration cannot be attributed to changes in adenosine A1 receptors because there were no significant differences in adenosine A1 mRNA expression with sham-operated animals. Lack of alteration in A1 mRNA expression 4 months after cervical SCI suggests that A1 receptor plasticity is not activated by chronic injury. Obliteration of spontaneous recovery with theophylline most likely involves a separate unknown mechanism. These findings suggest that there may be a limited therapeutic window for the clinical application of theophylline in SCI patients with respiratory deficits. Theophylline may be more effective clinically in the acute phase of injury rather than in the chronic phase.

Canal switch is a complication following canalith repositioning procedure (CRP) for posterior canal benign paroxysmal positional vertigo (BPPV). Instead of being returned to the utricle, the loose otoconia migrate into the superior or horizontal semicircular canal. Patients remain symptomatic, and treatment can be ineffective unless the switch is recognized and additional repositioning maneuvers directed toward the appropriate semicircular canal are performed. This report provides the first videographic documentation of canal switch involving conversion of unilateral posterior semicircular canal BPPV to geotropic horizontal canalithiasis.

Management options of vestibular schwannomas continue to evolve as surgical, radiation, and radiologic imaging techniques and technology change and improve over time. Currently, three options for these tumors exist and include primary surgical extirpation, stereotactic radiation, and observation with serial imaging. Skull base surgeons are charged with providing the patient with a comprehensive discussion of each option based on the inherit risks and benefits of each option to craft a management plan based on numerous factors that include age, tumor location, and size, hearing level at presentation and any medical comorbidities that may influence functional outcomes and quality of life. The purpose of this article was to review the surgical and non-surgical approaches to tumor management highlighting the risks and benefits and ongoing controversies between modalities regarding optimal functional outcomes. A particular emphasis is placed on hearing preservation and facial nerve function after treatment.

Involvement of the superior semicircular canal (SSC) in benign paroxysmal positional vertigo (BPPV) is rare. SSC BPPV is distinguished from the more common posterior semicircular canal (PSC) variant by the pattern of nystagmus triggered by the Dix-Hallpike position: down-beating torsional nystagmus in SSC BPPV versus up-beating torsional nystagmus in PSC BPPV. SSC BPPV may be readily treated at the bedside, which is a key component in excluding central causes of down-beating nystagmus. We present an unusual video case report believed to represent refractory SSC BPPV based on the pattern of nystagmus and the absence of any other central signs.

Cervical spinal cord injury leads to a disruption of bulbospinal innervation from medullary respiratory centers to phrenic motoneurons. Animal models utilizing cervical hemisection result in inhibition of ipsilateral phrenic nerve activity, leading to paralysis of the hemidiaphragm. We have previously demonstrated a role for serotonin (5-HT) as one potential modulator of respiratory recovery following cervical hemisection, a mechanism that likely occurs via 5-HT2A and/or 5-HT2C receptors. The present study was designed to specifically examine if 5-HT2A and/or 5-HT2C receptors are colocalized with phrenic motoneurons in both intact and spinal-hemisected rats. Adult female rats (250-350 g; n = 6 per group) received a left cervical (C2) hemisection and were injected with the fluorescent retrograde neuronal tracer Fluorogold into the left hemidiaphragm. Twenty-four hours later, animals were killed and spinal cords processed for in situ hybridization and immunohistochemistry. Using (35)S-labeled cRNA probes, cervical spinal cords were probed for 5-HT2A and 5-HT2C receptor mRNA expression and double-labeled using an antibody to Fluorogold to detect phrenic motoneurons. Expression of both 5-HT2A and 5-HT2C receptor mRNA was detected in motoneurons of the cervical ventral horn. Despite positive expression of both 5-HT2A and 5-HT2C receptor mRNA-hybridization signal over phrenic motoneurons, only 5-HT2A silver grains achieved a signal-to-noise ratio representative of colocalization. 5-HT2A mRNA levels in identified phrenic motoneurons were not significantly altered following cervical hemisection compared to sham-operated controls. Selective colocalization of 5-HT2A receptor mRNA with phrenic motoneurons may have implications for recently observed 5-HT2A receptor-mediated regulation of respiratory activity and/or recovery in both intact and injury-compromised states.

The present study was designed to determine if there are region-specific differences in serotonin (5-HT) neurotransmission and 5-HT receptor expression that may limit the stimulatory effects of the 5-HT releaser p-chloroamphetamine (pCA) on striatal neuropeptide gene expression to the posterior striatum (P-STR) during postnatal maturation. Sprague-Dawley rat brains from postnatal days (PND) 1–35 were processed for 5-HT2A and 5-HT2C receptor mRNA expression by in situ hybridization and monoamine analysis by HPLC. Within the P-STR, 5-HT2A receptor mRNA expression reached young adult (PND 35) levels by PND 3, while levels in the A-STR were significantly less (range: 1.43 ± 0.219–6.36 ± 0.478) than P-STR (5.36 ± 0.854–12.11 ± 1.08) at each respective age throughout the time course. 5-HT2C receptor mRNA expression reached young adult levels at PND 7 in the A-STR and by PND 3 in the P-STR. At each PND age 5-HT2C receptor mRNA levels within the P-STR were significantly less (6.23 ± 1.02–12.32 ± 0.427) than the A-STR (7.31 ± 1.65–26.84 ± 2.24). 5-HT content increased across the developmental time course within the P-STR (5.01 ± 0.327–15.7 ± 1.03 ng/mg protein) and A-STR (2.97 ± 0.223–11.2 ± 0.701 ng/mg protein). Four hours following injection (i.p.) of pCA (10 mg/kg), preprotachykinin (PPT) mRNA levels increased 89% in the P-STR but not the anterior (A-STR) striatum of the 3-week-old rat, which were prevented by preinjection (30 min, i.p.) of the 5-HT2 receptor antagonist ritanserin (1 mg/kg). Together, these data suggest that faster maturity of 5-HT2A receptor expression in the P-STR may be sufficient to convey the region-specific acute stimulatory effects of pCA on PPT mRNA transcription in the developing rodent striatum. These results provide further evidence that the influence of 5-HT on neuropeptide gene expression is far stronger in caudal vs. rostral striatal regions during postnatal development. Synapse 38:216–225, 2000. © 2000 Wiley-Liss, Inc.

Sixty days following neonatal dopamine depletion (>98%) with 6-hydroxydopamine, preprotachykinin and preprodynorphin mRNA levels were significantly reduced (67 and 78% of vehicle controls, respectively) in the anterior striatum as determined by in situ hybridization while preproenkephalin mRNA expression was elevated (133% of vehicle controls). Suppression of the serotonin hyperinnervation phenomenon in the dopamine-depleted rat with 5,7-dihydroxytryptamine yielded no significant alterations in reduced striatal preprotachykinin (66%) or preprodynorphin (64%) mRNA levels, while preproenkephalin mRNA expression remained significantly elevated (140%). These data suggest that striatal serotonin hyperinnervation does not contribute to the development of dysregulated striatal neuropeptide transmission in either direct or indirect striatal output pathways following neonatal dopamine depletion.