Clinical Utility of the Standardized Word Recognition Score.

OBJECTIVES: An unexpectedly low word recognition (WR) score may be taken as evidence of increased risk for retrocochlear tumor. We sought to develop evidence for or against using a standardized WR (sWR) score in detecting retrocochlear tumors. The sWR is a z score expressing the difference between an observed WR score and a Speech Intelligibility Index-based predicted WR score. We retrospectively compared the sensitivity and specificity of pure-tone asymmetry-based logistic regression models that incorporated either the sWR or the raw WR scores in detecting tumor cases. Two pure-tone asymmetry calculations were used: the 4-frequency pure-tone asymmetry (AAO) calculation of the American Academy of Otolaryngology-Head and Neck Surgery and a 6-frequency pure-tone asymmetry (6-FPTA) calculation previously optimized to detect retrocochlear tumors. We hypothesized that a regression model incorporating the 6-FPTA calculation and the sWR would more accurately detect retrocochlear tumors. DESIGN: Retrospective data from all patients seen in the audiology clinic at Mayo Clinic in Florida in 2016 were reviewed. Cases with retrocochlear tumors were compared with a reference group with noise- or age-related hearing loss or idiopathic sensorineural hearing loss. Two pure-tone-based logistic regression models were created (6-FPTA and AAO). Into these base models, WR variables (WR, sWR, WR asymmetry [WRΔ], and sWR asymmetry [sWRΔ]) were added. Tumor detection performance for each regression model was compared twice: first, using all qualifying cases (61 tumor cases; 2332 reference group cases), and second, using a data set filtered to exclude hearing asymmetries greater than would be expected from noise-related or age-related hearing loss (25 tumor cases; 2208 reference group cases). The area under the curve and the DeLong test for significant receiver operating curve differences were used as outcome measures. RESULTS: The 6-FPTA model significantly outperformed the AAO model-with or without the addition of WR or WRΔ variables. Including sWR into the AAO base regression model significantly improved disease detection performance. Including sWR into the 6-FPTA model significantly improved disease detection performance when large hearing asymmetries were excluded. In the data set that included large pure-tone asymmetries, area under the curve values for the 6-FPTA + sWR and AAO + sWR models were not significantly better than the base 6-FPTA model. CONCLUSIONS: The results favor the superiority of the sWR computational method in identifying reduced WR scores in retrocochlear cases. The utility would be greatest where undetected tumor cases are embedded in a population heavily representing age- or noise-related hearing loss. The results also demonstrate the superiority of the 6-FPTA model in identifying tumor cases. The 2 computational methods may be combined (ie, the 6-FPTA + sWR model) into an automated tool for detecting retrocochlear disease in audiology and community otolaryngology clinics. The 4-frequency AAO-based regression model was the weakest detection method considered. Including raw WR scores into the model did not improve performance, whereas including sWR into the model did improve tumor detection performance. This further supports the contribution of the sWR computational method for recognizing low WR scores in retrocochlear disease cases.

Supralabyrinthine air cell is not present in superior semicircular canal dehiscence.

Objectives: Superior semicircular canal dehiscence (SSCD) is defined as a defect in the bone overly the superior semicircular canal (SSC). The purpose of this study is to evaluate the pre-operative imaging examinations of patients who have undergone SSCD repair. We hypothesize that these patients will not have a supralabyrinthine air cell on the side of surgery. Material and Methods: Our group retrospectively reviewed 50 consecutive pre-operative computed tomography (CT) temporal bone examinations who had confirmed SSCD on intraoperative examination and underwent repair for the presence of a supralabyrinthine air cell. Results: 100% of patients who had confirmed SSCD on intraoperative examination had no supralabyrinthine air cell on pre-operative CT of the temporal bone. Conclusion: This study shows that a supralabyrinthine air cell is not present in SSCD. When a supralabyrinthine air cell is present, the roof of the SSC is intact. CT and magnetic resonance imaging (MRI) are often performed together to evaluate for SSCD and exclude other etiologies. We propose that if a supralabyrinthine air cell is seen on MRI, no CT is necessary, thus avoiding unnecessary radiation exposure and additional imaging costs to the patient.

New bone formation over dehiscent semicircular canal with cartilage cap.

PURPOSE: Cartilage cap resurfacing is a method to seal a superior semicircular canal dehiscence. The purpose of this study was to evaluate the detection of new bone formation after surgical placement of a cartilage cap over a dehiscent semicircular canal. METHODS: In this retrospective review, two neuroradiologists blinded to each other's interpretation reviewed the temporal bones of 20 patients, five of which had a pre-operative computed tomography (CT) exam which was interpreted as unilateral superior semicircular canal dehiscence and with new bone formation following repair on follow-up CT. There were also 15 control subjects. Each neuroradiologist was blinded to history, including post-operative changes, and asked to determine if there was a dehiscence or no dehiscence. RESULTS: Out of the 15 controls, there was 100% inter-observer agreement. On the five post-operative patients, there was agreement in 4/5 that there was no dehiscence post-operatively and 1/5 agreement of dehiscence post-operatively, but ectopic bone adjacent to the dehiscence. CONCLUSION: Our results indicate that new bone formation can be seen at the site of cartilage cap placement over the dehiscence and be interpreted as bony closure of the dehiscence.

Initial Observation among Patients with Vestibular Schwannoma.

Introduction Vestibular schwannomas (VS) are slow growing tumors. Although there are a wide variety of available treatment options, these tumors are often initially observed. We aimed to establish the presenting symptoms and outcomes of patients treated with initial observation at our institution. Methods The medical records of patients with radiographically diagnosed VS were reviewed from 1989 to 2018. Actuarial estimates of radiographic tumor control and freedom of local therapy were calculated and compared using Cox regression analyses. Results A total of 360 patients were diagnosed with VS at our institution from 1989 through 2018 with a median age of 59.9 years. After radiographic diagnosis, 243 patients (67.5%) opted for initial observation. Local control at 1, 5, and 10 years was 91, 67, and 58%, respectively. On multivariable analysis, factors associated with shorter time to radiographic tumor progression included younger patient age ( p = 0.016) and tumors with an extracanalicular component ( p = 0.032). Regarding time until definitive treatment only larger baseline American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) tumor size predicted for earlier initiation of therapy ( p < 0.001), although this was restricted to tumors with an extracanalicular component ( p = 0.004), as opposed to purely internal auditory canal tumors ( p = 0.839). Conclusion Many patients who were initially observed continued to have satisfactory local control rates at 10 years. In patients with extracanalicular tumors, larger AAO-HNS tumor measurements were associated with earlier radiographic tumor progression and shorter time to local therapy, with 7 mm serving as a potential threshold value for extracanalicular tumors. Younger patients and tumors with primarily an extracanalicular portion may warrant closer observation.

Superior Semicircular Canal Dehiscence : Covering Defects in Understanding from Clinical to Radiologic Evaluation.

Superior semicircular canal dehiscence alters the normal fluid mechanics of the vestibulocochlear system and can be a debilitating condition. This article reviews the current understanding of the bony labyrinthine defect, including symptoms, etiology, surgical approach, as well as preoperative and postoperative imaging pearls.

American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Foundation Guide to Enhance Otologic and Neurotologic Care During the COVID-19 Pandemic.

: This combined American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Surgery Foundation document aims to provide guidance during the coronavirus disease of 2019 (COVID-19) on 1) "priority" of care for otologic and neurotologic patients in the office and operating room, and 2) optimal utilization of personal protective equipment. Given the paucity of evidence to inform otologic and neurotologic best practices during COVID-19, the recommendations herein are based on relevant peer-reviewed articles, the Centers for Disease Control and Prevention COVID-19 guidelines, United States and international hospital policies, and expert opinion. The suggestions presented here are not meant to be definitive, and best practices will undoubtedly change with increasing knowledge and high-quality data related to COVID-19. Interpretation of this guidance document is dependent on local factors including prevalence of COVID-19 in the surgeons' local community. This is not intended to set a standard of care, and should not supersede the clinician's best judgement when managing specific clinical concerns and/or regional conditions.Access to otologic and neurotologic care during and after the COVID-19 pandemic is dependent upon adequate protection of physicians, audiologists, and ancillary support staff. Otolaryngologists and associated staff are at high risk for COVID-19 disease transmission based on close contact with mucosal surfaces of the upper aerodigestive tract during diagnostic evaluation and therapeutic procedures. While many otologic and neurotologic conditions are not imminently life threatening, they have a major impact on communication, daily functioning, and quality of life. In addition, progression of disease and delay in treatment can result in cranial nerve deficits, intracranial and life-threatening complications, and/or irreversible consequences. In this regard, many otologic and neurotologic conditions should rightfully be considered "urgent," and almost all require timely attention to permit optimal outcomes. It is reasonable to proceed with otologic and neurotologic clinic visits and operative cases based on input from expert opinion of otologic care providers, clinic/hospital administration, infection prevention and control specialists, and local and state public health leaders. Significant regional variations in COVID-19 prevalence exist; therefore, physicians working with local municipalities are best suited to make determinations on the appropriateness and timing of otologic and neurotologic care.

Multiple meningiomas: does quantity matter? a population-based survival analysis with underlined age and sex differences.

INTRODUCTION: Intracranial meningiomas rarely present with multiple lesions. To the best of our knowledge, current literature regarding meningiomatosis (MM) is mostly comprised of small case series and individual reports. Hence, survival outcome data are limited. The Objective of this study is to explore the influence of sex, age, and number of lesions on overall survival (OS) in patients with MM. METHODS: We obtained demographic and clinical data from the surveillance, epidemiology, and end results program (SEER) on adult patients diagnosed with meningiomas from 1975 to 2017. Univariable and multivariable analyses were conducted to assess whether number of lesions, age, and sex had a significant influence on OS. RESULTS: 99,918 cases were included. Results showed that MM patients had a significantly decreased OS when compared to patients with a single lesion (median OS of 94 and 180 months, respectively; p < 0.001). Further analysis showed a progressive decrease on OS for every additional lesion; 2 (HR 1.659 [CI 95% 1.612-1.708], p < 0.001), 3 (HR 1.877 [CI 95% 1.773-1.988], p < 0.001), and ≥ 4 (HR 2.116 [CI 95% 1.886-2.373], p < 0.001). When assessing for sex differences, female patients had increased OS (HR 0.778 [CI 95% 0.743-0.815], p < 0.001) and decreased risk of developing MM (HR 0.809 [CI 95% 0.784-0.835], p < 0.001). CONCLUSION: Increasing number of meningiomas has a significant negative impact on OS, with a progressive decrease on survival for every additional lesion. Furthermore, female patients had increased OS and decreased risk to develop MM.

Clinical implications of nervus intermedius variants in patients with geniculate neuralgia: Let anatomy be the guide.

Geniculate neuralgia (GN) is an uncommon, but severe, condition that is characterized by excruciating paroxysmal pain in the seventh cranial nerve's cutaneous distribution of general somatic afferent fibers carried through the nervus intermedius (NI). GN becomes a surgical disease in refractory cases of pain after exhaustive medical management. Surgical intervention in the form of microvascular decompression and nerve sectioning has been investigated with good patient outcomes. Despite this, there are limited guidelines on either technique's appropriateness in specific operative scenarios. In our 30-year experience in GNs surgical management, we have found that a detailed knowledge of the NIs anatomy, variants, and intraoperative surgical anatomic findings are the key to choosing the most appropriate intervention, and may provide the answer to why some patients fail to experience pain relief after surgery. These anatomic variants also may explain why many patients commonly do not experience side effects related to the visceral efferent and special afferent fibers after nerve sectioning.

Observation or stereotactic radiosurgery for newly diagnosed vestibular schwannomas: A systematic review and meta-analysis.

INTRODUCTION: Vestibular schwannomas (VS) are benign tumors with a slow growth rate. There exists controversy regarding whether patients should receive upfront observation, SRS, FSRT, or surgery at the time of diagnosis. For patients declining resection, this systematic review evaluates the risks and benefits between observation and SRS upon diagnosis of VS. METHODS: Published studies on VS (including acoustic neuromas) were systematically reviewed for clinical series including patients with newly/recently diagnosed unilateral VS. Studies that included patients with previous treatment for the VS or focused on patients with neurofibromatosis (or other genetic conditions) were excluded. Review articles and systematic reviews were excluded but reviewed for relevant references that would otherwise meet search criteria. RESULTS: Most patients electing observation underwent their first surveillance scan 6 months after initial diagnosis, then annually or every-other-year thereafter. Follow up was similar for patients receiving radiosurgery. The follow up period varied between studies as not all studies published at a specific time point. Observation alone conferred a radiographic tumor control rate of 65% and serviceable hearing in 71.3% at by the end of the follow up period in the reviewed studies, and 34% of patients initially opting for observation went on to ultimately elect for treatment. Initial radiosurgery resulted in a tumor control rate of 97% and serviceable hearing rate of 73.8% at by the end of the follow up period. Radiosurgery resulted in improved tumor control at the end of the follow up period (p < 0.0001), and serviceable hearing did not statistically differ (p = 0.69). There is an early risk of progressive hearing decline on patients who chose initial observation. CONCLUSION: Based on the available published data, observation after initial diagnosis is appropriate for many patients with VS without symptomatic brainstem compression. However, initial observation does risk early progressive hearing dysfunction and patients with serviceable hearing at diagnosis may benefit from early therapy. Long term follow up is critical, and an algorithm to better risk-stratify patients with newly diagnosed VS is underway.

Trends in the initial management of vestibular schwannoma in the United States.

INTRODUCTION: Vestibular schwannomas are benign tumors of the 8th cranial nerve. Initial treatment options include active surveillance, surgery, and/or radiation therapy. We analyzed the United States National Cancer Database (NCDB) for patients with vestibular schwannomas and evaluated the initial management trends after diagnosis. METHODS: We queried the NCDB for patients with vestibular schwannomas, excluding patients who did not have schwannomas of the vestibulocochlear nerve. Categorical and continuous variables were analyzed, and multivariate Cox regression analyses were performed to investigate for predictors of initial local therapy at diagnosis. All statistical analyses were performed using commercially available software (SPSS, Version 22; SPSS Inc., Chicago, IL). RESULTS: A total of 28,446 patients met the inclusion criteria. In this cohort, 7351 (25.8%) underwent observation, 12,362 (43.5%) underwent surgical resection, 7785 (27.4%) underwent SRS, 824 (2.9%) underwent EBRT, and 124 (0.4%) underwent RT NOS. On multivariate analysis, younger age, increased distance to treating facility, Charlson/Deyo score of 1, primary payer insurance, facility location and facility type (academic or cancer center) (p < 0.001) were all factors that predicted patients undergoing initial definitive treatment. CONCLUSION: Age, distance to treating facility, Charlson/Deyo score, primary payer, facility location, and facility type are factors that influence initial treatment for patients with vestibular schwannoma. Clinical stratification systems are needed to identify which patients would benefit most from initial local therapy versus active surveillance.

CEDRA: A Tool to Help Consumers Assess Risk for Ear Disease.

This article introduces the Consumer Ear Disease Risk Assessment (CEDRA) tool. CEDRA is a brief questionnaire designed to screen for targeted ear diseases. It offers an opportunity for consumers to self-screen for disease before seeking a hearing device and may be used by clinicians to help their patients decide the appropriate path to follow in hearing healthcare. Here we provide highlights of previously published validation in the context of a more thorough description of CEDRA's development and implementation. CEDRA's sensitivity and specificity, using a cut-off score of 4 or higher, was 90% and 72%, respectively, relative to neurotologist diagnoses in the initial training sample used to create the scoring algorithm (n = 246). On a smaller independent test sample (n = 61), CEDRA's sensitivity and specificity were 76% and 80%, respectively. CEDRA has readability levels similar to many other patient-oriented questionnaires in hearing healthcare, and informal reports from pilot CEDRA-providers indicate that the majority of patients can complete it in less than 10 min. As the hearing healthcare landscape changes and provider intercession is no longer mandated, CEDRA provides a measure of safety without creating a barrier to access.

Outcomes of large vestibular schwannomas following subtotal resection: early post-operative volume regression and facial nerve function.

INTRODUCTION: Subtotal resection (STR) of vestibular schwannoma (VS) tumors remains controversial and little is known regarding post-operative volume changes. METHODS: Authors retrospective reviewed the medical records from January 1st 2002 to January 1st 2018, for all patients who had undergone primary STR of large VS at a single tertiary academic institution. RESULTS: Our series consists of 34 patients with a mean age of 53.9 (median 53; range 21-87) years that had STR of their VS tumor. The mean pre-operative tumor diameter and volume was 3.9 cm (median 3.0 cm; range 1.6-6.0 cm) and 11.7 cm3 (median 9.6 cm3; range 2.8-44.3 cm3), respectively, with a mean extent of resection of 86% (median 90%; range 53-99%). The mean radiographic and clinical follow-up was 40 months (range 6-120 months) and 51 months (range 7-141 months), respectively. 85% of patients had optimal House-Brackmann (HB) scores (Grade 1 & 2) immediately post-operatively, and 91% at 1 year; 94% of patients had normal (HB 1) at last follow-up. There was significant regression of residual tumor volume at 1 year (p = 0.006) and 2 years (p = 0.02), but not at 3 years (p = 0.08), when compared to the prior year. There was significant regression of size over time, with a mean slope estimate of - 0.70 units per year (p < 0.001). CONCLUSION: Excellent clinical facial nerve outcomes can be obtained with STR of large VS tumors. Maximal reduction in tumor size occurs at 2-year post-operatively. Thus, in patients undergoing surgery for large VS, STR and a "watch and wait" strategy is a reasonable treatment option that may optimize facial nerve outcomes.

Intratympanic Dexamethasone via Saturated Gelfoam for Idiopathic Sudden Sensorineural Hearing Loss.

The duration of contact time of intratympanic steroids at the round window is a variable that can potentially affect the ultimate concentration within the cochlea. By placing Gelfoam saturated with dexamethasone directly over the round window, contact time is prolonged and potentially increases the dexamethasone concentration within the cochlea. This technique is simple, readily available with standard instruments and ingredients, and easily done in the office.

A Retrospective Estimate of Ear Disease Detection Using the "Red Flags" in a Clinical Sample.

OBJECTIVES: The purpose of this study was to evaluate the specificity and sensitivity of two red flag protocols in detecting ear diseases associated with changes in hearing. DESIGN: The presence of red-flag symptoms was determined in a chart review of 307 adult patients from the Mayo Clinic Florida Departments of Otorhinolaryngology and Audiology. Participants formed a convenience sample recruited for a separate study. Neurotologist diagnosis was the criterion for comparisons. RESULTS: Of the 251 patient files retained for analysis, 191 had one or more targeted diseases and 60 had age- or noise-related hearing loss. Food and Drug Administration red flags sensitivity was 91% (confidence interval [CI], 86 to 95%) and specificity was 72% (CI, 59 to 83%). American Academy of Otolaryngology-Head and Neck Surgery red flags sensitivity was 98% (CI, 95 to 99%) and specificity was 20% (CI, 11 to 32%). CONCLUSIONS: Stakeholders must determine which diseases are meaningful contraindications for hearing aid use and whether these red-flag protocols have acceptable levels of sensitivity and specificity. As direct-to-consumer models of hearing devices increase, a disease detection method that does not require provider intercession would be useful.

Mastoid Epidermoid Tumor and Associated Dural Arteriovenous Fistula with Venous Sinus Occlusion.

BACKGROUND: Dural arteriovenous fistula (DAVF) is an abnormal vascular connection between arterial and venous channels within dura mater. Although DAVFs have been linked to other types of intracranial tumors, this is the first case reporting the association between DAVF and an epidermoid tumor. CASE DESCRIPTION: A middle-aged patient with chronic headache presented with Borden type II DAVF draining into the right transverse sigmoid junction and was also found to have an epidermoid tumor over the right mastoid. The patient underwent staged embolization of the fistula through both transvenous and transarterial routes. Continuous intraoperative venous pressure monitoring confirmed marked reduction in intracranial venous pressure, and the patient's symptoms completely resolved. However, the fistula still remained. The residual DAVF was then surgically disconnected, and the epidermoid tumor was resected in the same procedure. CONCLUSIONS: This case demonstrates a DAVF can be associated with an epidermoid tumor. Tumor can compromise the venous outflow, which can then lead to intracranial venous hypertension and development of the DAVF. Venous pressure monitoring offers an objective method to verify resolution of venous hypertension, which might correlate with resolution of clinical symptoms.

Development and Initial Validation of a Consumer Questionnaire to Predict the Presence of Ear Disease.

Importance: The already large population of individuals with age- or noise-related hearing loss in the United States is increasing, yet hearing aids remain largely inaccessible. The recent decision by the US Food and Drug Administration to not enforce the medical examination prior to hearing aid fitting highlights the need to reengineer consumer protections when increasing accessibility. A self-administered tool to estimate ear disease risk would provide disease surveillance without posing an unreasonable barrier to hearing aid procurement. Objective: To develop and validate a consumer questionnaire for the self-assessment of risk for ear diseases associated with hearing loss. Design, Setting, and Participants: The questionnaire was developed using established methods including expert opinion to validate and create questions, and cognitive interviews to ensure that questions were clear to respondents. Exploratory structural equation modeling, logistic regression, and receiver operating characteristic curve analysis were used to determine sensitivity and specificity with blinded neurotologist opinion as the criterion for evaluation. Patients 40 to 80 years old with ear or hearing complaints necessitating a neurotologic examination and a control group of participants with a diagnosis of age- or noise-related hearing loss participated at the Departments of Otorhinolaryngology and Audiology of Mayo Clinic Florida. Main Outcomes and Measures: Sensitivity and specificity of the prototype questionnaire to identify individuals with targeted diseases. Results: Of 307 participants (mean [SD] age, 62.9 [9.8] years; 148 [48%] female), 75% (n = 231) were enrolled with targeted disease(s) identified on neurotologic assessment and 25% (n = 76) with age- or noise-related hearing loss. Participants were randomly divided into a training sample (80% [n = 246; 185 with disease, 61 controls]) and a test sample (20% [n = 61; 46 with disease, 15 controls]). Using a simple scoring method, a sensitivity of 94% (95% CI, 89%-97%) and specificity of 61% (95% CI, 47%-73%) were established in the training sample. Applying this cutoff to the test sample resulted in 85% (95% CI, 71%-93%) sensitivity and 47% (95% CI, 22%-73%) specificity. Conclusions and Relevance: This is the first self-assessment tool designed to assess an individual's risk for ear disease. Our preliminary results demonstrate a high sensitivity to disease detection. A further validated and refined version of this questionnaire may serve as an efficacious tool for improving access to hearing health care while minimizing the risk for missed ear diseases.

Facial Nerve Schwannomas: Review of 80 Cases Over 25 Years at Mayo Clinic.

OBJECTIVE: To elucidate the long-term clinical behavior, treatment, and outcomes of sporadic facial nerve schwannoma (FNS) in a large cohort of patients managed in the post-magnetic resonance imaging era. PATIENTS AND METHODS: Retrospective review at a single tertiary health care system (January 1, 1990, through December 31, 2015), evaluating 80 consecutive patients with sporadic FNS. RESULTS: Ninety-eight patients with FNS were identified; 10 with incomplete data and 8 with neurofibromatosis type 2 were excluded. The remaining 80 patients (median age, 47 years; 58% women) were analyzed. Forty-three (54%) patients presented with asymmetrical hearing loss, 33 (41%) reported facial paresis, and 21 (26%) reported facial spasm. Seventeen (21%) exhibited radiologic features mimicking vestibular schwannoma, 14 (18%) presented as a parotid mass, and 5 (6%) were discovered incidentally. Factors predictive of facial nerve paresis or spasm before treatment were female sex and tumor involvement of the labyrinthine/geniculate and tympanic facial nerve segments. The median growth rate among growing FNS was 2.0 mm/y. Details regarding clinical outcome according to treatment modality are described. CONCLUSION: In patients with FNS, female sex and involvement of the labyrinthine/geniculate and tympanic segments of the facial nerve predict a higher probability of facial paresis or spasm. When isolated to the posterior fossa or parotid gland, establishing a preoperative diagnosis of FNS is challenging. Treatment should be tailored according to tumor location and size, existing facial nerve function, patient priorities, and age. A management algorithm is presented, prioritizing long-term facial nerve function.

Identifying and Prioritizing Diseases Important for Detection in Adult Hearing Health Care.

PURPOSE: The purpose of this research note is to identify and prioritize diseases important for detection in adult hearing health care delivery systems. METHOD: Through literature review and expert consultation, the authors identified 195 diseases likely to occur in adults complaining of hearing loss. Five neurotologists rated the importance of disease on 3 dimensions related to the necessity of detection prior to adult hearing aid fitting. RESULTS: Ratings of adverse health consequences, diagnostic difficulty, and presence of nonotologic symptoms associated with these diseases resulted in the identification of 104 diseases potentially important for detection prior to adult hearing aid fitting. CONCLUSIONS: Current and evolving health care delivery systems, including direct-to-consumer sales, involve inconsistent means of disease detection vigilance prior to device fitting. The first steps in determining the safety of these different delivery methods are to identify and prioritize which diseases present the greatest risk for poor health outcomes and, thus, should be detected in hearing health care delivery systems. Here the authors have developed a novel multidimensional rating system to rank disease importance. The rankings can be used to evaluate the effectiveness of alternative detection methods and to inform public health policy. The authors are currently using this information to validate a consumer questionnaire designed to accurately identify when pre- fitting medical evaluations should be required for hearing aid patients.