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.

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.

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.

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.

Dizziness handicap after cartilage cap occlusion for superior semicircular canal dehiscence.

OBJECTIVE: To evaluate the change in self-reported dizziness handicap after surgical repair using the cartilage cap occlusion technique in cases of superior canal dehiscence (SCD). STUDY DESIGN: Repeated measures, retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Twenty patients over a 2-year period who underwent surgical repair of SCD using the cartilage cap occlusion technique. INTERVENTION: Therapeutic. MAIN OUTCOME MEASURE: Preoperative and postoperative Dizziness Handicap Inventory (DHI) questionnaires were completed (median, interquartile range). RESULTS: Preoperative (48, 28-56) and postoperative (33, 19-50) total scores were not significantly different. Scores for patients with moderate/severe preoperative DHI scores (DHI, >30; n = 14) demonstrated significant change (p = 0.001, Wilcoxon paired sample test), whereas those with mild scores did not (DHI, ≤ 30; n = 6; p = 0.67). CONCLUSION: Change in DHI score is variable. As described by DHI score, patients with higher preoperative handicap may demonstrate significant improvement after surgery, whereas those with mild handicap may not. These results are similar to previous reports and indicate that the cartilage cap occlusion technique may provide an alternative to middle fossa craniotomy approach for surgical management of symptomatic SCD.

Pure-tone hearing asymmetry: a logistic approach modeling age, sex, and noise exposure history.

BACKGROUND: Asymmetric hearing loss (AHL) can be an early sign of vestibular schwannoma (VS). However, recognizing VS-induced AHL is challenging. There is no universally accepted definition of a "medically significant pure-tone hearing asymmetry," in part because AHL is a common feature of medically benign forms of hearing loss (e.g., age- or firearm-related hearing loss). In most cases, the determination that an observed AHL does not come from a benign cause involves subjective clinical judgment. PURPOSE: Our purpose was threefold: (1) to quantify hearing asymmetry distributions in a large group of patients with medically benign forms of hearing loss, stratifying for age, sex, and noise exposure history; (2) to assess how previously proposed hearing asymmetry calculations segregate tumor from nontumor cases; and (3) to present the results of a logistic regression method for defining hearing asymmetry that incorporates age, sex, and noise information. RESEARCH DESIGN: Retrospective chart review. STUDY SAMPLE: Five thousand six hundred and sixty-one patients with idiopathic, age- or noise exposure-related hearing loss and 85 untreated VS patients. DATA COLLECTION AND ANALYSIS: Audiometric, patient history, and clinical impression data were collected from 22,785 consecutive patient visits to the audiology section at Mayo Clinic in Florida from 2006 to 2009 to screen for eligibility. Those eligible were then stratified by VS presence, age, sex, and self-reported noise exposure history. Pure-tone asymmetry distributions were analyzed. Audiometric data from VS diagnoses were used to create four additional audiograms per patient to model the hypothetical development of AHL prior to the actual hearing test. The ability of 11 previously defined hearing asymmetry calculations to distinguish between VS and non-VS cases was described. A logistic regression model was developed that integrated age, sex, and noise exposure history with pure-tone asymmetry data. Regression model performance was then compared to existing asymmetry calculation methods. RESULTS: The 11 existing pure-tone asymmetry calculations varied in tumor detection performance. Age, sex, and noise exposure history helped to predict benign forms of hearing asymmetry. The logistic regression model outperformed existing asymmetry calculations and better accounted for normal age-, sex-, and noise exposure-related asymmetry variability. CONCLUSIONS: Our logistic regression asymmetry method improves the clinician's ability to estimate risk of VS, in part by integrating categorical patient history and numeric test data. This form of modeling can enhance clinical decision making in audiology and otology.

Bilateral multicanal benign paroxysmal positional vertigo coexisting with a vestibular schwannoma: case report.

We describe a rarely encountered case of coexisting bilateral multicanal benign paroxysmal positional vertigo (BPPV) and vestibular schwannoma in a 56-year-old woman. The patient had presented with a 10-year history of dizziness and imbalance, and her vestibular findings were perplexing. We decided on a working diagnosis of BPPV and began treatment. After several months of canalith repositioning maneuvers had failed to resolve her symptoms, we obtained magnetic resonance imaging, which revealed the presence of the vestibular schwannoma. This case serves as a reminder of the importance of differentiating between central and peripheral vestibular disorders, as well as central and anterior canal BPPV-induced down-beating nystagmus in order to establish the correct diagnosis and initiate appropriate treatment.

Takotsubo cardiomyopathy and canalith repositioning procedure for benign paroxysmal positional vertigo.

BACKGROUND: Takotsubo cardiomyopathy, also known as left ventricular apical ballooning syndrome, ampulla cardiomyopathy, or transient left ventricular dysfunction is characterized by chest pain, electrocardiographic changes, transient left ventricular apical aneurysm, and normal coronary arteries. Takotsubo is a round-bottomed, narrow-necked Japanese octopus trap and lends its name to takotsubo cardiomyopathy because of its resemblance to echocardiographic and ventricular angiographic images of the left ventricle in this condition. This appearance takes its source from peculiar, transient regional systolic dysfunction involving the left ventricular apex and mid-ventricle with hyperkinesis of the basal left ventricular segments. Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigo caused by peripheral vestibular dysfunction. The symptoms of BPPV are attributed to intralabyrinthine particles, presumed displaced otoconia. Thus, the treatment recommended for BPPV is head repositioning maneuvers. PURPOSE: To present the first takotsubo cardiomyopathy case in the English literature related to BPPV undergoing canalith repositioning procedure. CONCLUSION: This report will provide additional information for physicians encountering acute-onset chest pain and vertigo. It will also expand the spectrum of clinical correlates of the increasingly well recognized but poorly understood syndrome, takotsubo cardiomyopathy.

A comparison of water and air caloric responses and their ability to distinguish between patients with normal and impaired ears.

OBJECTIVES: The caloric test is a mainstay of modern vestibular assessment. Yet caloric test methods have not been well standardized, and normal response values have not been universally agreed upon. The air caloric test has been particularly problematic. In this article, we present our efforts to establish a population-based description of the caloric response evoked by water and air stimuli at both cool and warm temperatures. DESIGN: Data were collected from a retrospective record review of patients who underwent caloric testing at Mayo Clinic Jacksonville between 2002 and 2006. Two subgroups were identified. One group was found to have no vestibulopathy after comprehensive medical investigation. The second group was found to have severe bilateral vestibular weakness; this diagnosis was based on medical evaluation and objective test results. Caloric response distributions and associated probability estimates were developed from each group. RESULTS: A total of 2587 medical records were found to contain caloric response data. Of these, 693 patients met the criteria to be classified as having no identifiable vestibulopathy (otologically normal patients with normal caloric responses). Sixty-eight patients met the criteria for bilateral vestibular weakness (reduced or absent rotatory chair responses). Our analysis yielded the following results: (1) there were differences between nystagmus distributions across stimuli. On average, the magnitude of cool water (30 degrees C) maximum slow-phase velocities was smaller than those from warm water (44 degrees C). Maximum slow-phase velocity distributions from cool (21 degrees C) and warm (51 degrees C) air stimuli were more similar to each other than were responses to water stimuli and fell between the water distributions. (2) Combined metrics (combined eye speed and total eye speed) were comparable for water and air stimuli. (3) Response distributions from otologically normal patients were different from those of patients with bilateral vestibular weakness. (4) Derived probability estimates allowed for quantification of caloric response normal limits, sensitivity, specificity, and error rates. CONCLUSIONS: Current bithermal test methods assume an equivalence of caloric response strength from warm and cool stimuli. Our results show standard cool and warm water stimuli provoke substantially different response magnitudes, with warm stimuli provoking stronger responses. When calibrated as described herein, air stimuli perform comparably with water stimuli for bithermal caloric test purposes, with more uniform and less variable response distributions. Both air- and water-based tests were able to distinguish between normal and abnormally weak ears with sensitivity and specificity values between 0.82 and 0.84. We advocate for the calibration of all caloric stimuli based on the test's statistical performance and not arbitrary assumptions about stimulus equivalence.

Simultaneous acute superior nerve neurolabyrinthitis and benign paroxysmal positional vertigo.

An acutely vertiginous 47-year-old woman presented to the emergency department with simultaneous acute left neurolabyrinthitis and left posterior canal benign paroxysmal positional vertigo (BPPV). Gaze nystagmus from the neurolabyrinthitis hampered diagnosis of the BPPV. However, once the BPPV was identified and treated, the patient's subjective vertigo improved rapidly. Concomitant BPPV should not be overlooked when a diagnosis of acute neurolabyrinthitis is made in the emergency department.

Pediatric and adult cochlear implantation.

The frequency of cochlear implantation has increased tremendously over the past decade. Cochlear implantation is often performed as an outpatient procedure and is considered an acceptable treatment for severe to profound sensorineural hearing loss in patients who are refractory to conventional hearing augmentation. Imaging plays an important part in the work-up of cochlear implant candidates, and an understanding of imaging evaluation procedures is essential. The radiologist must be familiar with imaging findings that contraindicate implantation (absence of the cochlea or cochlear nerve) and with those that could significantly alter surgery (facial nerve dehiscence, cochlear ossification). It is also imperative to be familiar with the growing number of imaging options (particularly magnetic resonance [MR] imaging pulse sequences) to optimize evaluation of cochlear implant candidates. Imaging choices will be substantially influenced by the manufacturer of the computed tomographic scanner or MR imager. Radiologists will assume an expanding role in evaluating affected patients as the frequency of cochlear implantation continues to increase.