Mythbusters! The Truth about Common Misconceptions in Cochlear Implantation.

Cochlear implantation (CI) is the preferred method of hearing rehabilitation when patients cannot perform well with traditional amplification. Unfortunately, there are still significant misconceptions around this life-changing intervention. The goal of this article is to address some of the most common myths around CI surgery. After reading this article, the learner will be able to explain the utility of CI in patients with residual hearing and recognize that insurance coverage is widespread. The reader will be able to list common risks associated with this well-tolerated procedure including anesthetic risk and the risk of vestibular dysfunction. Additionally, the reader will be able to identify the significant positive impact of CI on patients' quality of life. Finally, the reader will identify that many patients can safely have an MRI scan after implantation, including nearly all contemporary recipients.

The Future of Telemedicine: Revolutionizing Health Care or Flash in the Pan?

The meteoric rise of telemedicine early in the COVID-19 pandemic might easily be mistaken for an ephemeral trend-one reaching its zenith in a moment of crisis. To the contrary, momentum has been mounting for telehealth over decades. The recent increase in telecare reveals its potential to deliver efficient, patient-centered, high-quality care in an increasingly technology-dependent landscape. Prior to COVID-19, surgeons lagged behind medical counterparts in embracing telemedicine; however, the pragmatic imperatives for remote care of patients and changes to Medicare removed key barriers to adoption. Otolaryngology-head and neck surgery has innovated across subspecialties, leading in COVID-19 scholarship and year-over-year publications on telemedicine. Yet, improved access to subspecialists is tempered by a digital divide that threatens to exacerbate disparities. Otolaryngology is poised to lead the transformation of procedural specialties while ensuring equitable care.

Pediatric vestibular testing: Tolerability of test components in children.

INTRODUCTION: Objective of the study is to define rates of successful completion of components of pediatric vestibular testing (VT). METHODS: Retrospective review of VT performed on patients less than 18 years of age from 2004 to 2015. RESULTS: 188 pediatric patients (mean age: 13.9 ±â€¯3.56 years old, range 2-17 years) underwent testing. Thirty-five (18.6%) had abnormal test results. Pediatric patients unable to complete all aspects of VT could still complete an average of 7.9 ±â€¯4.0 of 12 test components. The optokinetic tracking test was the most commonly omitted component of the vestibular tests. In a multivariate analysis, failure to perform Nylen-Barany positional testing (χ2 27.5, p < 0.0001) or Dix-Hallpike (5.66, p = 0.0174) testing was associated with inability to obtain final diagnosis on VT. CONCLUSIONS: Interpretable VT may be obtained in most children, even in those that do not tolerate the full testing protocol. Spontaneous and gaze-evoked nystagmus testing maybe considered as part of initial testing protocol before attempting less well-tolerated components such as bithermal calorics or components that require VNG goggles.

Ultra Long-Term Audiometric Outcomes in the Treatment of Vestibular Schwannoma With the Middle Cranial Fossa Approach.

OBJECTIVE: Define the long-term audiometric outcomes of vestibular schwannomas treated using the middle cranial fossa (MCF) approach. STUDY DESIGN: Retrospective records review. SETTING: University-based tertiary referral center. PATIENTS: Patients undergoing treatment of small vestibular schwannomas using the MCF approach. INTERVENTION(S): MCF exposure and treatment of small vestibular schwannomas. MAIN OUTCOME MEASURE(S): Demographic and audiometric variables were assessed. RESULTS: Thirteen subjects demonstrated audiometric data for review. The average time between surgery and the most recent audiometric testing was 14 years (range 10-18 yr). The mean baseline pure-tone average (PTA) was 36 dB HL and the most recent PTA was 59 dB HL in the operated ear. The mean baseline PTA was 16 dB HL and the most recent PTA was 37 dB HL in the nonoperated ear. The mean change from baseline to most recent follow-up was a threshold elevation of 22 and 19 dB HL in the operated and nonoperated ears, respectively. The mean baseline speech discrimination score (SDS) was 83% and the most recent SDS was 73% in the operated ear. The mean baseline SDS was 98% and the most recent SDS was 94% in the nonoperated ear. The mean changes from baseline to most recent follow-up were 10 and 0% SDS depression in the operated and nonoperated ears, respectively. The rates of changes in PTA and SDS were not statistically different between the operated and nonoperated ears. CONCLUSIONS: Surgically preserved hearing is maintained in the majority of patients more than 10 years following surgery. PTA and SDS changes were similar between the ipsilateral and contralateral ears.

Clinical Practice Guideline (Update): Earwax (Cerumen Impaction)

Objective This update of the 2008 American Academy of Otolaryngology-Head and Neck Surgery Foundation cerumen impaction clinical practice guideline provides evidence-based recommendations on managing cerumen impaction. Cerumen impaction is defined as an accumulation of cerumen that causes symptoms, prevents assessment of the ear, or both. Changes from the prior guideline include a consumer added to the development group; new evidence (3 guidelines, 5 systematic reviews, and 6 randomized controlled trials); enhanced information on patient education and counseling; a new algorithm to clarify action statement relationships; expanded action statement profiles to explicitly state quality improvement opportunities, confidence in the evidence, intentional vagueness, and differences of opinion; an enhanced external review process to include public comment and journal peer review; and 3 new key action statements on managing cerumen impaction that focus on primary prevention, contraindicated intervention, and referral and coordination of care. Purpose The primary purpose of this guideline is to help clinicians identify patients with cerumen impaction who may benefit from intervention and to promote evidence-based management. Another purpose of the guideline is to highlight needs and management options in special populations or in patients who have modifying factors. The guideline is intended for all clinicians who are likely to diagnose and manage patients with cerumen impaction, and it applies to any setting in which cerumen impaction would be identified, monitored, or managed. The guideline does not apply to patients with cerumen impaction associated with the following conditions: dermatologic diseases of the ear canal; recurrent otitis externa; keratosis obturans; prior radiation therapy affecting the ear; previous tympanoplasty/myringoplasty, canal wall down mastoidectomy, or other surgery affecting the ear canal. Key Action Statements The panel made a strong recommendation that clinicians should treat, or refer to a clinician who can treat, cerumen impaction, defined as an accumulation of cerumen that is associated with symptoms, prevents needed assessment of the ear, or both. The panel made the following recommendations: (1) Clinicians should explain proper ear hygiene to prevent cerumen impaction when patients have an accumulation of cerumen. (2) Clinicians should diagnose cerumen impaction when an accumulation of cerumen, as seen on otoscopy, is associated with symptoms, prevents needed assessment of the ear, or both. (3) Clinicians should assess the patient with cerumen impaction by history and/or physical examination for factors that modify management, such as ≥1 of the following: anticoagulant therapy, immunocompromised state, diabetes mellitus, prior radiation therapy to the head and neck, ear canal stenosis, exostoses, and nonintact tympanic membrane. (4) Clinicians should not routinely treat cerumen in patients who are asymptomatic and whose ears can be adequately examined. (5) Clinicians should identify patients with obstructing cerumen in the ear canal who may not be able to express symptoms (young children and cognitively impaired children and adults), and they should promptly evaluate the need for intervention. (6) Clinicians should perform otoscopy to detect the presence of cerumen in patients with hearing aids during a health care encounter. (7) Clinicians should treat, or refer to a clinician who can treat, the patient with cerumen impaction with an appropriate intervention, which may include ≥1 of the following: cerumenolytic agents, irrigation, or manual removal requiring instrumentation. (8) Clinicians should recommend against ear candling for treating or preventing cerumen impaction. (9) Clinicians should assess patients at the conclusion of in-office treatment of cerumen impaction and document the resolution of impaction. If the impaction is not resolved, the clinician should use additional treatment. If full or partial symptoms persist despite resolution of impaction, the clinician should evaluate the patient for alternative diagnoses. (10) Finally, if initial management is unsuccessful, clinicians should refer patients with persistent cerumen impaction to clinicians who have specialized equipment and training to clean and evaluate ear canals and tympanic membranes. The panel offered the following as options: (1) Clinicians may use cerumenolytic agents (including water or saline solution) in the management of cerumen impaction. (2) Clinicians may use irrigation in the management of cerumen impaction. (3) Clinicians may use manual removal requiring instrumentation in the management of cerumen impaction. (4) Last, clinicians may educate/counsel patients with cerumen impaction or excessive cerumen regarding control measures.

Clinical Practice Guideline (Update): Earwax (Cerumen Impaction) Executive Summary

The American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) has published a supplement to this issue of Otolaryngology-Head and Neck Surgery featuring the updated Clinical Practice Guideline: Earwax (Cerumen Impaction). To assist in implementing the guideline recommendations, this article summarizes the rationale, purpose, and key action statements. The 11 recommendations emphasize proper ear hygiene, diagnosis of cerumen impaction, factors that modify management, evaluating the need for intervention, and proper treatment. An updated guideline is needed due to new evidence (3 guidelines, 5 systematic reviews, and 6 randomized controlled trials) and the need to add statements on managing cerumen impaction that focus on primary prevention, contraindicated intervention, and referral and coordination of care.

Safety of Monopolar Electrocautery in Patients With Cochlear Implants.

OBJECTIVE: The outcomes of 2 patients with cochlear implants (CIs) who underwent adenotonsillectomy (AT) with inadvertent use of monopolar cautery are presented. The safety data regarding monopolar cautery use in CI recipients is also reviewed. STUDY DESIGN: This is a retrospective case series of 2 CI recipients that underwent AT with monopolar cautery and literature review of electrocautery safety in the setting of CI. RESULTS: Two patients with CIs underwent AT with use of monopolar cautery inadvertently by surgeons that do not routinely perform cochlear implants as part of his or her clinical practice. Patient 1 was a 9-year-old female who had AT for obstructive sleep apnea (OSA) after undergoing unilateral CI for profound congenital sensorineural hearing loss (SNHL) 8 years ago. Patient 2 was a 7-year-old female who underwent AT for OSA 4 months after undergoing unilateral CI for congenital SNHL. Both patients had no immediate signs of complications with their CI use postoperatively. Both patients demonstrated unchanged postoperative neural response telemetry and behavioral audiometric testing. Patient 1 continues to have no CI-related complications 3.5 years after the procedure. Patient 2 has been followed for at least 3 months by audiometric testing and 10 months by otolaryngologist with no CI-related complications. CONCLUSION: Although animal and cadaveric studies suggest that monopolar cautery may be safely used in patients with cochlear implants, there have been no in vivo human studies that have evaluated the risk to the patient or implant. This is a report of a small, unintended experience with 2 patients, both of whom exhibit no complications or changes to CI function thus far.

p75NTR is highly expressed in vestibular schwannomas and promotes cell survival by activating nuclear transcription factor κB.

Vestibular schwannomas (VSs) arise from Schwann cells (SCs) and result from the loss of function of merlin, the protein product of the NF2 tumor suppressor gene. In contrast to non-neoplastic SCs, VS cells survive long-term in the absence of axons. We find that p75(NTR) is overexpressed in VSs compared with normal nerves, both at the transcript and protein level, similar to the response of non-neoplastic SCs following axotomy. Despite elevated p75(NTR) expression, VS cells are resistant to apoptosis due to treatment with proNGF, a high affinity ligand for p75(NTR) . Furthermore, treatment with proNGF protects VS cells from apoptosis due to c-Jun N-terminal kinase (JNK) inhibition indicating that p75(NTR) promotes VS cell survival. Treatment of VS cells with proNGF activated NF-κB while inhibition of JNK with SP600125 or siRNA-mediated knockdown reduced NF-κB activity. Significantly, proNGF also activated NF-κB in cultures treated with JNK inhibitors. Thus, JNK activity appears to be required for basal levels of NF-κB activity but not for proNGF-induced NF-κB activity. To confirm that the increase in NF-κB activity contributes to the prosurvival effect of proNGF, we infected VS cultures with Ad.IκB.SerS32/36A virus, which inhibits NF-κB activation. Compared with control virus, Ad.IκB.SerS32/36A significantly increased apoptosis including in VS cells treated with proNGF. Thus, in contrast to non-neoplastic SCs, p75(NTR) signaling provides a prosurvival response in VS cells by activating NF-κB independent of JNK. Such differences may contribute to the ability of VS cells to survive long-term in the absence of axons.

Upper airway manifestations of granulomatosis with polyangiitis.

Upper airway manifestations, particularly sinonasal manifestations, are encountered frequently in granulomatosis with polyangiitis (GPA). Nasal endoscopy often reveals crusting, friable erythematous mucosa, and granulation. Up to 25% of patients may have a "saddle-nose" deformity as cartilage destruction worsens. Treatment is often complicated by loss of mucociliary function and necrosis, leading to refractory symptoms. Culture-directed antibiotics, topical antibiotic and saline irrigations, and occasional debridement of adherent crusts can reduce the frequency of sinonasal exacerbations and improve obstructive symptoms. Surgery should be reserved for patients unresponsive to maximal medical therapy. Saddle-nose reconstruction is possible in highly selected patients and can improve nasal breathing and resolve anosmia. Up to 20% of patients with GPA have subglottic stenosis; patients with respiratory symptoms should undergo laryngoscopy to assess the presence of subglottic narrowing. Although systemic manifestations of GPA are managed by immunosuppressive therapy, most patients with subglottic stenosis of GPA require surgical management (ie, endoscopic dilation, endoscopic or laser excision, surgical resection followed by reconstruction).

The Hybrid cochlear implant: a review.

The Hybrid S or 'short-electrode' cochlear implant was developed to treat patients with a severe to profound hearing loss limited to the high frequencies. The short electrode is implanted into just the base or high-frequency region of the cochlea, with the goal of preserving residual low-frequency hearing. As a result, electric stimulation can be combined with acoustic stimulation in the same ear (and the opposite ear); this is one instance of 'acoustic plus electric' (A + E) stimulation. In this paper, we will review the latest findings from the first two stages of the clinical trial for the Hybrid concept in the United States. Generally, we will review surgical techniques, clinical trial criteria, residual hearing preservation, improvements in speech perception in quiet, and predictive factors for patient benefit. We will also discuss the significant benefit of A + E stimulation for speech perception in noise and musical measures of melody and instrument recognition, as well as valuable insights into central auditory nervous system plasticity gained from the use of a very short electrode array.

Neurolymphomatosis mimicking chemotherapy-induced ototoxicity.

OBJECTIVE: To present an unusual cause of bilateral sensorineural hearing loss mimicking chemotherapy-induced ototoxicity. PATIENT: A 76-year-old woman with bilateral sudden sensorineural hearing loss after chemotherapy for non-Hodgkin lymphoma was referred for cochlear implantation. INTERVENTIONS: Prednisone and acyclovir were administered empirically during the course of the diagnostic evaluation. MAIN OUTCOME MEASURES: Diagnostic tests were obtained, including audiometry, positron emission tomography/computed tomographic imaging, and magnetic resonance imaging scan of the brain with gadolinium. RESULTS: Audiometry revealed bilateral profound sensorineural hearing loss without improvement after steroid and acyclovir treatment. Postchemotherapy positron emission tomography/computed tomographic imaging obtained 1 week after onset of hearing loss suggested the lymphoma to be in remission. However, magnetic resonance imaging obtained a few weeks later, concurrent with progression of symptoms to include facial palsy and numbness, demonstrated enhancement and thickening of multiple cranial nerves, consistent with neurolymphomatosis (malignant lymphocytic infiltration of nerves). CONCLUSION: Particular vigilance must be given to the potential for malignant invasion of the peripheral nervous system in the context of new-onset sensorineural hearing loss after chemotherapy. Such a neurologic deficit should not be assumed to be chemotherapy induced even if the hearing loss is bilaterally symmetric and the only neurologic abnormality.

Membrane depolarization inhibits spiral ganglion neurite growth via activation of multiple types of voltage sensitive calcium channels and calpain.

The effect of membrane electrical activity on spiral ganglion neuron (SGN) neurite growth remains unknown despite its relevance to cochlear implant technology. We demonstrate that membrane depolarization delays the initial formation and inhibits the subsequent extension of cultured SGN neurites. This inhibition depends directly on the level of depolarization with higher levels of depolarization causing retraction of existing neurites. Cultured SGNs express subunits for L-type, N-type, and P/Q type voltage-gated calcium channels (VGCCs) and removal of extracellular Ca(2+) or treatment with a combination of L-type, N-type, and P/Q-type VGCC antagonists rescues SGN neurite growth under depolarizing conditions. By measuring the fluorescence intensity of SGNs loaded with the fluorogenic calpain substrate t-butoxy carbonyl-Leu-Met-chloromethylaminocoumarin (20 microM), we demonstrate that depolarization activates calpains. Calpeptin (15 microM), a calpain inhibitor, prevents calpain activation by depolarization and rescues neurite growth in depolarized SGNs suggesting that calpain activation contributes to the inhibition of neurite growth by depolarization.