Minimum intraoperative testing battery for cochlear implantation: the international practice trend.

PURPOSE: In cochlear implantation (CI) surgery, there are a wide variety of intraoperative tests available. However, no clear guide exists on which tests must be performed as the minimum intraoperative testing battery. Toward this end, we studied the usage patterns, recommendations, and attitudes of practitioners toward intraoperative testing. METHODS: This study is a multicentric international survey of tertiary referral CI centers. A survey was developed and administered to a group of CI practitioners (n = 34) including otologists, audiologists and biomedical engineers. Thirty six participants were invited to participate in this study based on a their scientific outputs to the literature on the intraoperative testing in CI field and based on their high load of CI surgeries. Thirty four, from 15 countries have accepted the invitation to participate. The participants were asked to indicate the usage trends, perceived value, influence on decision making and duration of each intraoperative test. They were also asked to indicate which tests they believe should be included in a minimum test battery for routine cases. RESULTS: Thirty-two (94%) experts provided responses. The most frequently recommended tests for a minimum battery were facial nerve monitoring, electrode impedance measurements, and measurements of electrically evoked compound action potentials (ECAPs). The perceived value and influence on surgical decision-making also varied, with high-resolution CT being rated the highest on both measures. CONCLUSION: Facial nerve monitoring, electrode impedance measurements, and ECAP measurements are currently the core tests of the intraoperative test battery for CI surgery.

International Consensus Statements on Intraoperative Testing for Cochlear Implantation Surgery.

OBJECTIVES: A wide variety of intraoperative tests are available in cochlear implantation. However, no consensus exists on which tests constitute the minimum necessary battery. We assembled an international panel of clinical experts to develop, refine, and vote upon a set of core consensus statements. DESIGN: A literature review was used to identify intraoperative tests currently used in the field and draft a set of provisional statements. For statement evaluation and refinement, we used a modified Delphi consensus panel structure. Multiple interactive rounds of voting, evaluation, and feedback were conducted to achieve convergence. RESULTS: Twenty-nine provisional statements were included in the original draft. In the first voting round, consensus was reached on 15 statements. Of the 14 statements that did not reach consensus, 12 were revised based on feedback provided by the expert practitioners, and 2 were eliminated. In the second voting round, 10 of the 12 revised statements reached a consensus. The two statements which did not achieve consensus were further revised and subjected to a third voting round. However, both statements failed to achieve consensus in the third round. In addition, during the final revision, one more statement was decided to be deleted due to overlap with another modified statement. CONCLUSIONS: A final core set of 24 consensus statements was generated, covering wide areas of intraoperative testing during CI surgery. These statements may provide utility as evidence-based guidelines to improve quality and achieve uniformity of surgical practice.

Clinical Practice Guideline: Age-Related Hearing Loss.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with explanation of the support in the literature, evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the guideline development group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life (QOL). (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related QOL at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

Clinical Practice Guideline: Age-Related Hearing Loss Executive Summary.

OBJECTIVE: Age-related hearing loss (ARHL) is a prevalent but often underdiagnosed and undertreated condition among individuals aged 50 and above. It is associated with various sociodemographic factors and health risks including dementia, depression, cardiovascular disease, and falls. While the causes of ARHL and its downstream effects are well defined, there is a lack of priority placed by clinicians as well as guidance regarding the identification, education, and management of this condition. PURPOSE: The purpose of this clinical practice guideline is to identify quality improvement opportunities and provide clinicians trustworthy, evidence-based recommendations regarding the identification and management of ARHL. These opportunities are communicated through clear actionable statements with an explanation of the support in the literature, the evaluation of the quality of the evidence, and recommendations on implementation. The target patients for the guideline are any individuals aged 50 years and older. The target audience is all clinicians in all care settings. This guideline is intended to focus on evidence-based quality improvement opportunities judged most important by the Guideline Development Group (GDG). It is not intended to be a comprehensive, general guide regarding the management of ARHL. The statements in this guideline are not intended to limit or restrict care provided by clinicians based on their experience and assessment of individual patients. ACTION STATEMENTS: The GDG made strong recommendations for the following key action statements (KASs): (KAS 4) If screening suggests hearing loss, clinicians should obtain or refer to a clinician who can obtain an audiogram. (KAS 8) Clinicians should offer, or refer to a clinician who can offer, appropriately fit amplification to patients with ARHL. (KAS 9) Clinicians should refer patients for an evaluation of cochlear implantation candidacy when patients have appropriately fit amplification and persistent hearing difficulty with poor speech understanding. The GDG made recommendations for the following KASs: (KAS 1) Clinicians should screen patients aged 50 years and older for hearing loss at the time of a health care encounter. (KAS 2) If screening suggests hearing loss, clinicians should examine the ear canal and tympanic membrane with otoscopy or refer to a clinician who can examine the ears for cerumen impaction, infection, or other abnormalities. (KAS 3) If screening suggests hearing loss, clinicians should identify sociodemographic factors and patient preferences that influence access to and utilization of hearing health care. (KAS 5) Clinicians should evaluate and treat or refer to a clinician who can evaluate and treat patients with significant asymmetric hearing loss, conductive or mixed hearing loss, or poor word recognition on diagnostic testing. (KAS 6) Clinicians should educate and counsel patients with hearing loss and their family/care partner(s) about the impact of hearing loss on their communication, safety, function, cognition, and quality of life. (KAS 7) Clinicians should counsel patients with hearing loss on communication strategies and assistive listening devices. (KAS 10) For patients with hearing loss, clinicians should assess if communication goals have been met and if there has been improvement in hearing-related quality of life at a subsequent health care encounter or within 1 year. The GDG offered the following KAS as an option: (KAS 11) Clinicians should assess hearing at least every 3 years in patients with known hearing loss or with reported concern for changes in hearing.

Skull Base Osteomyelitis: Historical Perspective, Diagnosis and Management Update.

SBO is a life-threatening disease that requires a high index of suspicion based on these patients complex underlying medical co-morbidities and clinician's acumen. Once a diagnosis is made, is it critical to communicate and work closely with other multidisciplinary teams (neuroradiology for appropriate choice of imaging study and interpretation; infectious disease for appropriate medical treatment and duration; internist to properly manage their underlying medical co-morbidities). Despite advances in imaging, the diagnosis is first made based on clinical judgment, appropriate culture, and tissue biopsy.

Myosin Mutations and Sudden Sensorineural Hearing Loss: Results of Whole Exome Sequencing.

OBJECTIVE: Idiopathic sudden sensorineural hearing loss (ISSNHL) affects 66,000 patients per year in the United States. Genetic mutations have been associated with progressive hearing loss; however, genetic mutations associated with ISSNHL have not been identified. METHODS: A prospective cohort study of adults older than 18 years presenting with ISSNHL at a tertiary academic medical center. Whole exome sequencing (WES) was conducted using Genome Analysis Toolkit best practices. An automated diagnostic screen employing a variety of models for pathogenicity was conducted across all genes with no specific targets. Candidate pathogenic variants were reviewed by a team of geneticists and clinicians. Variants were crossed-referenced with 92 known hearing loss associated genes. RESULTS: Twenty-nine patients with SSNHL were screened using WES. The average age of patients was 53 ± 17.1 years, and most patients were White (62%) and men (55%). The mean pure tone average was 64.8 ± 31.3 dB for the affected ear. Using a 0.1% allele frequency screen, 12 (41%) cases had a mutation in any of the nine selected myosin genes. When we restrict to singletons (allele frequency = 0%), 21% (n = 6) of cases have qualifying variants, whereas only 3.8% (n = 481) of 12,577 healthy controls carry qualifying variants (p < 0.01). Most mutations (80%) were missense mutations. Of the novel mutations, one was a frameshift mutation, and two were a stop-gained function. Three were missense mutations. CONCLUSION: Myosin mutations may be associated with ISSNHL. However, larger population screening is needed to confirm the association of myosin mutation with ISSNHL and steroid responsiveness.

Clinical Practice Guideline: Tympanostomy Tubes in Children (Update).

OBJECTIVE: Insertion of tympanostomy tubes is the most common ambulatory surgery performed on children in the United States. Tympanostomy tubes are most often inserted because of persistent middle ear fluid, frequent ear infections, or ear infections that persist after antibiotic therapy. All these conditions are encompassed by the term otitis media (middle ear inflammation). This guideline update provides evidence-based recommendations for patient selection and surgical indications for managing tympanostomy tubes in children. The guideline is intended for any clinician involved in managing children aged 6 months to 12 years with tympanostomy tubes or children being considered for tympanostomy tubes in any care setting as an intervention for otitis media of any type. The target audience includes specialists, primary care clinicians, and allied health professionals. PURPOSE: The purpose of this clinical practice guideline update is to reassess and update recommendations in the prior guideline from 2013 and to provide clinicians with trustworthy, evidence-based recommendations on patient selection and surgical indications for managing tympanostomy tubes in children. In planning the content of the updated guideline, the guideline update group (GUG) affirmed and included all the original key action statements (KASs), based on external review and GUG assessment of the original recommendations. The guideline update was supplemented with new research evidence and expanded profiles that addressed quality improvement and implementation issues. The group also discussed and prioritized the need for new recommendations based on gaps in the initial guideline or new evidence that would warrant and support KASs. The GUG further sought to bring greater coherence to the guideline recommendations by displaying relationships in a new flowchart to facilitate clinical decision making. Last, knowledge gaps were identified to guide future research. METHODS: In developing this update, the methods outlined in the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline Development Manual, Third Edition: A Quality-Driven Approach for Translating Evidence Into Action" were followed explicitly. The GUG was convened with representation from the disciplines of otolaryngology-head and neck surgery, otology, pediatrics, audiology, anesthesiology, family medicine, advanced practice nursing, speech-language pathology, and consumer advocacy. ACTION STATEMENTS: The GUG made strong recommendations for the following KASs: (14) clinicians should prescribe topical antibiotic ear drops only, without oral antibiotics, for children with uncomplicated acute tympanostomy tube otorrhea; (16) the surgeon or designee should examine the ears of a child within 3 months of tympanostomy tube insertion AND should educate families regarding the need for routine, periodic follow-up to examine the ears until the tubes extrude.The GUG made recommendations for the following KASs: (1) clinicians should not perform tympanostomy tube insertion in children with a single episode of otitis media with effusion (OME) of less than 3 months' duration, from the date of onset (if known) or from the date of diagnosis (if onset is unknown); (2) clinicians should obtain a hearing evaluation if OME persists for 3 months or longer OR prior to surgery when a child becomes a candidate for tympanostomy tube insertion; (3) clinicians should offer bilateral tympanostomy tube insertion to children with bilateral OME for 3 months or longer AND documented hearing difficulties; (5) clinicians should reevaluate, at 3- to 6-month intervals, children with chronic OME who do not receive tympanostomy tubes, until the effusion is no longer present, significant hearing loss is detected, or structural abnormalities of the tympanic membrane or middle ear are suspected; (6) clinicians should not perform tympanostomy tube insertion in children with recurrent acute otitis media who do not have middle ear effusion in either ear at the time of assessment for tube candidacy; (7) clinicians should offer bilateral tympanostomy tube insertion in children with recurrent acute otitis media who have unilateral or bilateral middle ear effusion at the time of assessment for tube candidacy; (8) clinicians should determine if a child with recurrent acute otitis media or with OME of any duration is at increased risk for speech, language, or learning problems from otitis media because of baseline sensory, physical, cognitive, or behavioral factors; (10) the clinician should not place long-term tubes as initial surgery for children who meet criteria for tube insertion unless there is a specific reason based on an anticipated need for prolonged middle ear ventilation beyond that of a short-term tube; (12) in the perioperative period, clinicians should educate caregivers of children with tympanostomy tubes regarding the expected duration of tube function, recommended follow-up schedule, and detection of complications; (13) clinicians should not routinely prescribe postoperative antibiotic ear drops after tympanostomy tube placement; (15) clinicians should not encourage routine, prophylactic water precautions (use of earplugs or headbands, avoidance of swimming or water sports) for children with tympanostomy tubes.The GUG offered the following KASs as options: (4) clinicians may perform tympanostomy tube insertion in children with unilateral or bilateral OME for 3 months or longer (chronic OME) AND symptoms that are likely attributable, all or in part, to OME that include, but are not limited to, balance (vestibular) problems, poor school performance, behavioral problems, ear discomfort, or reduced quality of life; (9) clinicians may perform tympanostomy tube insertion in at-risk children with unilateral or bilateral OME that is likely to persist as reflected by a type B (flat) tympanogram or a documented effusion for 3 months or longer; (11) clinicians may perform adenoidectomy as an adjunct to tympanostomy tube insertion for children with symptoms directly related to the adenoids (adenoid infection or nasal obstruction) OR in children aged 4 years or older to potentially reduce future incidence of recurrent otitis media or the need for repeat tube insertion.

Executive Summary of Clinical Practice Guideline on Tympanostomy Tubes in Children (Update).

OBJECTIVE: This executive summary of the guideline update provides evidence-based recommendations for patient selection and surgical indications for managing tympanostomy tubes in children. The summary and guideline are intended for any clinician involved in managing children aged 6 months to 12 years with tympanostomy tubes or children being considered for tympanostomy tubes in any care setting as an intervention for otitis media of any type. The target audience includes specialists, primary care clinicians, and allied health professionals. PURPOSE: The purpose of this executive summary is to provide a succinct overview for clinicians of the key action statements (recommendations), summary tables, and patient decision aids from the update of the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline: Tympanostomy Tubes in Children (Update)." The new guideline updates recommendations in the prior guideline from 2013 and provides clinicians with trustworthy, evidence-based recommendations on patient selection and surgical indications for managing tympanostomy tubes in children. This summary is not intended to substitute for the full guideline, and clinicians are encouraged to read the full guideline before implementing the recommended actions. METHODS: The guideline on which this summary is based was developed using methods outlined in the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline Development Manual, Third Edition: A Quality-Driven Approach for Translating Evidence Into Action," which were followed explicitly. The guideline update group represented the disciplines of otolaryngology-head and neck surgery, otology, pediatrics, audiology, anesthesiology, family medicine, advanced practice nursing, speech-language pathology, and consumer advocacy. ACTION STATEMENTS: Strong recommendations were made for the following key action statements: (14) Clinicians should prescribe topical antibiotic ear drops only, without oral antibiotics, for children with uncomplicated acute tympanostomy tube otorrhea. (16) The surgeon or designee should examine the ears of a child within 3 months of tympanostomy tube insertion AND should educate families regarding the need for routine, periodic follow-up to examine the ears until the tubes extrude.Recommendations were made for the following key action statements: (1) Clinicians should not perform tympanostomy tube insertion in children with a single episode of otitis media with effusion (OME) of less than 3 months' duration, from the date of onset (if known) or from the date of diagnosis (if onset is unknown). (2) Clinicians should obtain a hearing evaluation if OME persists for 3 months or longer OR prior to surgery when a child becomes a candidate for tympanostomy tube insertion. (3) Clinicians should offer bilateral tympanostomy tube insertion to children with bilateral OME for 3 months or longer AND documented hearing difficulties. (5) Clinicians should reevaluate, at 3- to 6-month intervals, children with chronic OME who do not receive tympanostomy tubes, until the effusion is no longer present, significant hearing loss is detected, or structural abnormalities of the tympanic membrane or middle ear are suspected. (6) Clinicians should not perform tympanostomy tube insertion in children with recurrent acute otitis media (AOM) who do not have middle ear effusion (MEE) in either ear at the time of assessment for tube candidacy. (7) Clinicians should offer bilateral tympanostomy tube insertion in children with recurrent AOM who have unilateral or bilateral MEE at the time of assessment for tube candidacy. (8) Clinicians should determine if a child with recurrent AOM or with OME of any duration is at increased risk for speech, language, or learning problems from otitis media because of baseline sensory, physical, cognitive, or behavioral factors. (10) The clinician should not place long-term tubes as initial surgery for children who meet criteria for tube insertion unless there is a specific reason based on an anticipated need for prolonged middle ear ventilation beyond that of a short-term tube. (12) In the perioperative period, clinicians should educate caregivers of children with tympanostomy tubes regarding the expected duration of tube function, recommended follow-up schedule, and detection of complications. (13) Clinicians should not routinely prescribe postoperative antibiotic ear drops after tympanostomy tube placement. (15) Clinicians should not encourage routine, prophylactic water precautions (use of earplugs or headbands, avoidance of swimming or water sports) for children with tympanostomy tubes.Options were offered from the following key action statements: (4) Clinicians may perform tympanostomy tube insertion in children with unilateral or bilateral OME for 3 months or longer (chronic OME) AND symptoms that are likely attributable, all or in part, to OME that include, but are not limited to, balance (vestibular) problems, poor school performance, behavioral problems, ear discomfort, or reduced quality of life. (9) Clinicians may perform tympanostomy tube insertion in at-risk children with unilateral or bilateral OME that is likely to persist as reflected by a type B (flat) tympanogram or a documented effusion for 3 months or longer. (11) Clinicians may perform adenoidectomy as an adjunct to tympanostomy tube insertion for children with symptoms directly related to the adenoids (adenoid infection or nasal obstruction) OR in children aged 4 years or older to potentially reduce future incidence of recurrent otitis media or the need for repeat tube insertion.

Impact of Underlying Diagnosis on Speech and Quality of Life Outcomes After Cochlear Implantation for Single-Sided Deafness.

OBJECTIVE: Our objective was to compare outcomes in speech and quality of life in those undergoing cochlear implantation for single-sided deafness (SSD), with the aim to characterize the clinical impact of underlying diagnosis in the affected ear and pre-operative hearing status. STUDY DESIGN: Prospective case series. SETTING: Academic Cochlear Implant Center. PATIENTS: 42 adult patients implanted with the diagnosis of SSD. INTERVENTIONS: Patients were evaluated at 3-, 6-, and 12-months post-operatively using AZBio sentence and speech, and consonant-nucleus-consonant (CNC) depending on appropriate testing level. Our previously validated Comprehensive Cochlear Implant Quality of Life (CCIQ) questionnaire was administered. MAIN OUTCOME MEASURES: Speech perception, quality of life. RESULTS: Subjects were stratified by the underlying diagnosis: Meniere's Disease (MD; n = 10), sudden sensorineural hearing loss (SSNHL; n = 13), and Other (eg TBI, acoustic neuroma, progressive, noise-induced; n = 19). Mean preoperative PTA of the implanted ear was 82dB ±â€Š17; that of the nonimplanted ear was 32dB ±â€Š17. SSNHL and MD demonstrated the highest speech perception score at 3 months (93 and 95%), and "Other" demonstrated the lowest scores at 88%. All 3 groups demonstrated nadir in speech scores at 6 months before improving at 12 months, but the "Other" diagnoses maintained the lowest speech testing across all time points. All 3 groups reported improved quality of life on CCIQ. CONCLUSIONS: Subjects with SSNHL and MD demonstrate excellent speech perception and quality of life outcomes after cochlear implantation for SSD. Subjects with "Other" diagnoses underlying their SSD demonstrated lower scores on speech testing but nonetheless reported improved quality of life.

Association of Audiometric Age-Related Hearing Loss With Depressive Symptoms Among Hispanic Individuals.

Importance: Age-related hearing loss is highly prevalent and has recently been associated with numerous morbid conditions of aging. Late-life depression is also prevalent and can be resistant to available treatments. Preliminary studies examining the association between hearing loss and late-life depression have been limited by subjective hearing measures, small sample sizes, and primarily white populations. Objective: To assess whether a cross-sectional association exists between objective audiometric hearing loss and depressive symptoms in older Hispanic adults. Design, Setting, and Participants: This cross-sectional study uses 2008-2011 Hispanic Community Health Study/Study of Latinos data collected in Miami, Florida, San Diego, California, Chicago, Illinois, or the Bronx, New York, from 5328 Hispanic adults 50 years or older who had exposure, outcome, and covariate data. Data analyses were conducted from March 2018 to September 2018. Exposure: Audiometric hearing loss (pure-tone average). Main Outcomes and Measures: Center for Epidemiologic Studies Depression Scale, 10-item version (CESD-10) score of 10 or higher, which indicates clinically significant depressive symptoms. Results: The median age (interquartile range) of the 5328 participants was 58 (53-63) years, and 3283 participants (61.6%) were female. The mean (SD) CESD-10 score was 7.7 (6.4). Of the 5328 included participants, 1751 (32.9%) had clinically significant depressive symptoms. The odds of having these symptoms increased 1.44 (95% CI, 1.27-1.63) times for every 20 dB of hearing loss, adjusting for hearing aid use, age, sex, educational level, study site, geographic background, cardiovascular disease, and antidepressant use. Compared with those for individuals with normal hearing (0 dB), the odds of having clinically significant depressive symptoms was 1.81 (95% CI, 1.48-2.22) times as high in individuals with mild hearing loss (median threshold, 32.5 dB), 2.38 (95% CI, 1.77-3.20) times as high in individuals with moderate hearing loss (median threshold, 47.5 dB), and 4.30 (95% CI, 2.61-7.09) times as high in individuals with severe hearing loss (median threshold, 80 dB). Conclusions and Relevance: Objective hearing loss appears to be associated with clinically significant depressive symptoms in older Hispanic people, with greater hearing loss seemingly associated with greater odds of having depressive symptoms. Given the high prevalence of untreated hearing loss in older adults, hearing loss may be a potentially modifiable risk factor for late-life depression.

Common Otologic Surgical Procedures: Clinical Decision-Making Pearls and the Role of Imaging.

Neuro-otologists rely on the expertise and judgment of a skilled neuroradiologist to identify radiographic abnormalities in the complicated regional anatomy of the temporal bone and middle and posterior fossa, and more importantly, to alert the surgeon to potential operative pitfalls. This article highlights some of the common otologic surgical procedures that stress this important dynamic. The surgical perspective on quick and effective clinical decision-making pearls to keep in mind during a thorough radiographic analysis of the ear and lateral skull base is presented.

Quality of Life after Sequential Bilateral Cochlear Implantation.

Objective Assess psychometric properties of the Comprehensive Cochlear Implant Questionnaire (CCIQ) as a tool for assessing changes in health-related quality of life (HRQoL) after receiving a second cochlear implant (CI2). Study Design Prospective study. Setting Academic cochlear implant center. Subjects and Methods The CCIQ is a 29-item questionnaire assessing the physical and psychosocial benefits of a CI2 based on a 5-point Likert scale. The CCIQ was administered with the Nijmegen Cochlear Implant Questionnaire and the Short Form-12 Patient Questionnaire (SF-12) to patients with a CI2. Speech perception was tested with the consonant-nucleus-consonant (CNC) word test and AzBio test. Results Of 56 patients, 32 (57.1%) completed the instruments sent by mail. Of the 32 patients, 22 (68.8%) completed retest CCIQs 6 weeks later. CCIQ scores demonstrated significantly increased HRQoL in all domains. Internal consistency was very good overall (Cronbach's α = 0.90), with all subdomains exceeding an alpha value of 0.70. Test-retest reliability was good, with an overall intraclass correlation of 0.62. The CCIQ showed a moderate correlation with the Nijmegen Cochlear Implant Questionnaire and a low correlation with the SF-12. Average CNC and AZBio scores significantly improved by 31% ± 29% and 34% ± 33%, respectively. Audiometric data were not significantly correlated with the CCIQ. Conclusion The CCIQ shows (1) good reliability and evidence of construct validity, (2) a significant increase in HRQoL and significantly improved speech perception after CI2, and (3) greater sensitivity at detecting CI2 improvements to HRQoL. This promising measure is quick and easy to administer and provides subjective assessments of HRQoL specifically for those with a CI2.

Cerebrospinal fluid leaks and encephaloceles of temporal bone origin: nuances to diagnosis and management.

OBJECTIVE: Temporal bone encephalocele has become less common as the incidence of chronic mastoid infection and surgery for this condition has decreased. As a result, the diagnosis is often delayed, and the encephalocele is often an incidental finding. This situation can result in serious neurologic complications with patients presenting with cerebrospinal fluid leak and meningitis. We review the occurrence of, characteristics of, and repair experience with temporal encephaloceles from 2000-2012. METHODS: We conducted a retrospective review of 32 patients undergoing combined mastoidectomy and middle cranial fossa craniotomy for the treatment of temporal encephalocele. RESULTS: The diagnosis of temporal encephalocele was made in all patients using high-resolution temporal bone computed tomography and magnetic resonance imaging. At the time of diagnosis, 12 patients had confirmed cerebrospinal fluid leak; other common presenting symptoms included hearing loss and ear fullness. Tegmen defect was most commonly due to chronic otitis media (n = 14). Of these patients, 8 had undergone prior mastoidectomy, suggesting an iatrogenic cause. Other etiologies included radiation exposure, congenital defects, and spontaneous defects. Additionally, 2 patients presented with meningitis; 1 patient had serious neurologic deficits resulting from venous infarction. CONCLUSIONS: The risk of severe neurologic complications after the herniation of intracranial contents through a tegmen defect necessitates prompt recognition and appropriate management. Computed tomography and magnetic resonance imaging aid in definitive diagnosis. A combined mastoid/middle fossa approach allows for sustainable repair with adequate exposure of defects and support of intracranial contents.

Facial nerve paralysis after pre-operative embolization of a paraganglioma.

Vascular tumors pose a challenging problem in treatment, as surgical planning can be extensive. Often times, pre-operative embolization is required to minimize blood loss during surgery. With the advent of new biochemical compounds, embolization modalities have evolved over the past decade. Although rare, side effects and complications of embolic materials have been cited sporadically in the literature. We present an interesting case of a patient afflicted with facial paralysis and other cranial neuropathies following embolization of a paraganglioma, along with the appropriate imaging that confirms the etiology of her paralysis.

Cost-effective analysis of unilateral vestibular weakness investigation.

OBJECTIVES: To evaluate the cost-effectiveness of obtaining a magnetic resonance imaging (MRI) in patients with abnormal electronystagmography (ENG) or videonystagmography (VNG) results. STUDY DESIGN: Retrospective chart review. SETTINGS: Academic specialty center. PATIENTS: Patients presenting with vertigo between January 1, 2010, and August 30, 2013. METHODS: Patients who fit the following abnormal criteria were included in the study: unilateral caloric weakness (≥20%), abnormal ocular motor testing, and nystagmus on positional testing. Patients with abnormal findings who then underwent MRI with gadolinium were evaluated. RESULTS: Of the 1,996 charts reviewed, there were 1,358 patients who met the inclusion criteria. The average age of these patients was 62 years (12-94 yr). The male:female ratio was approximately 1:2. Of the 1,358 patients, 253 received an MRI with the following pathologies: four vestibular schwannomas, three subcortical/periventricular white matter changes suspicious for demyelinating disease, four acute cerebellar/posterior circulation infarct, two vertebral artery narrowing, one pseudomeningocele of internal auditory canal, and two white matter changes indicative of migraines. The positive detection rate on MRI was 5.5% based on MRI findings of treatable pathologies causing vertigo. Average cost of an MRI is $1,200, thereby making the average cost of identifying a patient with a positive MRI finding $15,180. CONCLUSION: In our study, those patients with a positive MRI had a constellation of symptoms and findings (asymmetric sensorineural hearing loss, tinnitus, vertigo, and abnormal ENG/VNG). Cost-effectiveness can be improved by ordering an MRI only when clinical examination and VNG point toward a central pathology. Clinical examination and appropriate testing should be factored when considering the cost-effectiveness of obtaining an MRI in patients with abnormal ENG/VNG findings.

Dexamethasone's effect in the retrocochlear auditory centers of a noise-induced hearing loss mouse model.

OBJECTIVE: Examine prophylactic effects of dexamethasone (Dex) in retrocochlear auditory centers in a noise-induced hearing loss (NIHL) mouse model. STUDY DESIGN: Prospective animal study. SETTING: Academic research center. SUBJECTS AND METHODS: Thirty-two mice were divided into control, untreated, saline (2 and 10 µL), and Dex (2 and 10 µL) groups. Dex was applied intratympanically (IT) prior to 110 to 120 dB noise over 6 hours. Auditory brainstem response (ABR) and distortion product otoacoustic emission (DPOAE) were performed at 1 day, 1 week, 1 month, and 2 months. Retrocochlear neuronal cells were labeled with FluoroGold and counted. Hair cells of the organ of Corti were labeled with fluorescein isothiocyanate-conjugated phalloidin and counted. RESULTS: Auditory brainstem response thresholds of untreated NIHL, 2 and 10 µL IT saline, and 2 and 10 µL IT Dex were 21.7 ± 2.9 dB, 20 ± 0 dB, 20 ± 5 dB, 18.3 ± 2.9 dB, and 18.3 ± 2.9 dB, respectively. At 1-day post NIHL, all groups demonstrated profound hearing loss. At 2 weeks, 2 and 10 µL Dex thresholds improved to 47.5 ± 3.5 dB and 48.8 ± 18.9 dB, respectively, whereas the untreated and saline groups remained unchanged. Mean cell counts in the cochlear nucleus (CN), superior olivary complex (SOC), and lateral lemniscus (LL) of control mice were 1483 ± 190, 2807 ± 67, and 112 ± 20, respectively. After acoustic trauma, the untreated, saline, and 2 µL Dex groups yielded decreased neuronal counts in the SOC. In contrast, the 10 µL Dex group had 1883 ± 186 (CN), 2774 ± 182 (SOC), and 166 ± 18 (LL). There was sporadic hair cell loss for all traumatized groups. CONCLUSION: Our NIHL mouse model demonstrated dose-dependent Dex pretreatment otoprotection against NIHL with preservation of retrocochlear auditory neurons.

Comparison of cochlear implant performance after round window electrode insertion compared with traditional cochleostomy.

OBJECTIVE: To compare speech perception after undergoing round window (RW) electrode insertion with those who underwent traditional cochleostomy (C) cochlear implant (CI) surgery. STUDY DESIGN: Case series with chart review. SETTING: Academic cochlear implant center. SUBJECTS: Patients who met CI criteria with favorable intraoperative RW anatomy who underwent RW CI electrode insertion. Patients who underwent traditional C surgery were matched for age, duration of deafness, and preoperative hearing. METHODS: Postoperative speech performance using the consonant-nucleus-consonant (CNC) test, Northwestern University Children's Perception of Speech (NUCHIPS), and Hearing in Noise Test (HINT) between 3 and 48 months after CI. RESULTS: A total of 84 patients underwent RW insertion between 2004 and 2011. Of these, 55 had pre- and postoperative speech perception data for analysis. Average age of implantation ranged from 2.4 to 87.3 years (mean ± SD, 42.1 ± 24.2 years) for RW patients. Average age of implantation for matched traditional C patients ranged from 2.3 to 85.6 years (mean ± SD, 43.3 ± 25.0 years). No significant differences in postoperative CI speech perception scores were noted between the RW and C groups at 12 months post-CI (RW group, 55.28% ± 23.26% vs C group, 53.19% ± 24.14%; P = .70). CONCLUSION: Our study found that patients with favorable RW anatomy who underwent RW CI electrode insertion demonstrated comparable speech perception compared with the traditional cochleostomy insertion group.

Revision cochlear implant surgery in patients with suspected soft failures.

OBJECTIVE: To review our patient series who underwent revision cochlear implantation surgery, with special emphasis on the "soft failure" group. STUDY DESIGN: Retrospective chart review of cochlear implant revision surgeries from 1979 to 2008. An extensive review of these patients' medical, audiologic, and radiographic histories was performed. SETTING: Two tertiary care hospitals and 1 academic cochlear implant center. INTERVENTION: Explantation and reimplantation of cochlear implant, explanted device analysis, speech perception testing. MAIN OUTCOME MEASURES: Postoperative speech performance. RESULTS: Approximately 1,500 cochlear implant surgeries were performed from 1979 to 2008. Of these, 113 (7.53%) procedures in 98 patients were revision cases. The underlying reason for revision surgery was divided into 4 categories: 26 hard failures (23%), 31 medical failures (27.4%), 14 soft failures (12.4%), and 42 (37.2%) not classified/ambiguous cases. The last group was not categorized because of lack of available medical documentation or because of an ambiguous device failure analysis. The top 3 most common causes of hard failure were loss of hermiticity (8 patients [30.8%]), Vendor B defects (7 patients [26.9%]), and cracked casing (4 patients [15.4%]). The most common cause of medical failure was device extrusion (11 patients [35.5%]) followed by head trauma to the site of implantation (11 patients [35.5%]), and wound infection (5 patients [16.1%]). Fourteen patients (14.2%) were categorized as soft failures. All soft failure patients demonstrated a deterioration in pure-tone average and speech perception. Of the soft failure group, time to revision surgery was 4.7 years in contrast to 4.2 years for the hard failure group and 4.3 years for all revisions. An extensive review of patients' medical histories showed that 4 (28.5%) of the 14 patients had a previous diagnosis of meningitis. Two patients (14.3%) had evidence of inner ear malformations, and 2 patients (14.3%) had history of asthma. CONCLUSION: Our cochlear revision series are comparable to what is reported in the literature. However, an unexpected relationship between meningitis was identified among our soft failure group. More than one-quarter carried a history of meningitis. Moreover, nearly one-half of all soft failures had some form of inflammatory derangement. We used the soft failure criteria established by the 2005 Consensus Development Conference for our population analysis. Although we agree that audiologic data often are essential for defining soft failure, multiple patients in our series experienced pain that was severe enough to prevent a complete audiometric evaluation, therefore not rigorously fulfilling the criteria set forth by the 2005 Consensus. However, because their symptoms resolved after reimplantation, and their speech performance restored, we propose modifications of the current definition of "soft failure" to include these patients.

Cost analysis of asymmetric sensorineural hearing loss investigations.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to critically evaluate the typical cost of asymmetrical sensorineural hearing loss (ASNHL) work-up, and to compare the positive predictive value from this common presenting symptom. STUDY DESIGN: Retrospective chart review from two major otolaryngology centers. METHODS: We reviewed charts from patients presenting to New York Eye and Ear Infirmary between January 1, 2006 and December 31, 2006, and the University of Minnesota between December 1, 2002 and November 30, 2007 with ASNHL. Diagnostic information included magnetic resonance imaging (MRI) and serum laboratory values (antinuclear antibodies, erythrocyte sedimentation rate, Lyme, rapid plasma reagin, and thyroid-stimulating hormone). We calculated positive rate according to each item of diagnosis. To estimate cost-benefit, we further calculated the average cost for identifying a patient with a positive result. RESULTS: The total cost was $263,535, whereas the average cost for identifying a positive patient was $146,40.81. The total lab cost was $16,935 and the total imaging cost was $246,600. The average cost for identifying a positive patient based on MRI was $61,650 and $2,109 based on lab values. Of the 247 patients, only six patients (2.4%)-one patient with acoustic neuroma, two patients with syphilis, and three patients with Lyme--were identified with treatable pathology. CONCLUSIONS: A comprehensive ASNHL work-up may not be applicable to all patients. Laboratory serologic tests are highly cost effective in diagnosing treatable causes of ASNHL, such as syphilis and Lyme. Although radiographic imaging with MRI is not as cost effective, its value in detecting for acoustic neuroma is undeniable.