Zapala, David A.

Almost since the inception of the modern-day electroacoustic audiometer a century ago the results of pure-tone audiometry have been characterized by an audiogram. For almost as many years, clinicians and researchers have sought ways to distill the volume and complexity of information on the audiogram. Commonly used approaches have made use of pure-tone averages (PTAs) for various frequency ranges with the PTA for 500, 1000, 2000 and 4000 Hz (PTA4) being the most widely used for the categorization of hearing loss severity. Here, a three-digit triad is proposed as a single-number summary of not only the severity, but also the configuration and bilateral symmetry of the hearing loss. Each digit in the triad ranges from 0 to 9, increasing as the level of the pure-tone hearing threshold level (HTL) increases from a range of optimal hearing (< 10 dB Hearing Level; HL) to complete hearing loss (≥ 90 dB HL). Each digit also represents a different frequency region of the audiogram proceeding from left to right as: (Low, L) PTA for 500, 1000, and 2000 Hz; (Center, C) PTA for 3000, 4000 and 6000 Hz; and (High, H) HTL at 8000 Hz. This LCH Triad audiogram-classification system is evaluated using a large United States (U.S.) national dataset (N = 8,795) from adults 20 to 80 + years of age and two large clinical datasets totaling 8,254 adults covering a similar age range. Its ability to capture variations in hearing function was found to be superior to that of the widely used PTA4.

Abstract Background We describe a 42-year-old patient with reported “shaking” vision referred due to concerns of possible vestibular system dysfunction. The patient has known history of Usher syndrome type I, bilateral cochlear implants, and severe vision impairment. Purpose This case describes an unusual nystagmus previously only reported in individuals with central demyelinating disorders, significant light deprivation, or in congenital/early-onset visual pathway impairment. Research Design Case study. Data Collection and Analysis Retrospective chart review of vestibular function. Results Vestibular function was likely absent in this case. There was no evidence of vestibular (jerk) nystagmus for sinusoidal harmonic acceleration stimuli or repeatable responses for cervical vestibular evoked myogenic potentials. Significant pendular low-amplitude high-frequency oscillations of approximately 6 Hz were present for horizontal and vertical tracings throughout testing. Conclusion Nystagmus may not always be associated with vestibular system impairment. In this case, the patient's reported “shaking” vision was attributed to pendular low-amplitude high-frequency nystagmus and hypothesized to relate to long-standing significant vision impairment. This presentation is unusual in adults and has historically been associated with individuals with significant central pathology or in those with long duration light deprivation.

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.

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.

Galvanic stimulation has long been used as a nonmechanical means of activating the vestibular apparatus through direct action on the vestibular nerve endings. This stimulation has been reported to be safe, but no studies have examined the potential changes in the corresponding cochlear receptors. The aim of the present study was to evaluate the effect of galvanic vestibular stimulation (GVS) on distortion product otoacoustic emissions (DPOAEs). Fourteen subjects underwent DPOAEs during several conditions of GVS. The DPOAEs ranged from ∼ 1 kHz to ∼ 8 kHz at 65/55 dB for f1/f2 and with an f2/f1 ratio of 1.2. The subjects were evaluated at 10 stimulation conditions that ranged from −2.0 mA to +2.0 mA for each frequency. Statistical analysis showed no significant differences in DPOAE amplitudes for all conditions with and without GVS. Results also showed no significant differences between DPOAE amplitudes before and after GVS. Multivariate analysis found subject variability in DPOAE amplitude, which was not thought to be GVS related. Results indicated that GVS produced neither temporary nor permanent changes in DPOAEs.

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.

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.

Objectives: The International Classification of Functioning, Disability, and Health (ICF) Core Sets for Hearing Loss (CSHL) consists of short lists of categories from the entire ICF classification that are thought to be the most relevant for describing the functioning of persons with hearing loss. A comprehensive intake that covers all factors included in the ICF CSHL holds the promise of developing a tailored treatment plan that fully complements the patient’s needs. The Comprehensive CSHL contains 117 categories and serves as a guide for multiprofessional, comprehensive assessment. The Brief CSHL includes 27 of the 117 categories and represents the minimal spectrum of functioning of persons with HL for single-discipline encounters or clinical trials. The authors first sought to benchmark the extent to which Audiologist (AUD) and Otorhinolaryngologist (ORL) discipline-specific intake documentation, as well as Mayo Clinic’s multidisciplinary intake documentation, captures ICF CSHL categories. Design: A retrospective study design including 168 patient records from the Department of Otorhinolaryngology/Audiology of Mayo Clinic in Jacksonville, Florida. Anonymized intake documentation forms and reports were selected from patient records filed between January 2016 and May 2017. Data were extracted from the intake documentation forms and reports and linked to ICF categories using pre-established linking rules. “Overlap,” defined as the percentage of ICF CSHL categories represented in the intake documentation, was calculated across document types. In addition, extra non–ICF CSHL categories (ICF categories that are not part of the CSHL) and extra constructs (constructs that are not part of the ICF classification) found in the patient records were described. Results: The total overlap of multidisciplinary intake documentation with ICF CSHL categories was 100% for the Brief CSHL and 50% for the Comprehensive CSHL. Brief CSHL overlap for discipline-specific documentation fell short at 70% for both AUD and ORL. Important extra non–ICF CSHL categories were identified and included “sleep function” and “motor-related functions and activities,” which mostly were reported in relation to tinnitus and vestibular disorders. Conclusion: The multidisciplinary intake documentation of Mayo Clinic showed 100% overlap with the Brief CSHL, while important areas of nonoverlap were identified in AUD- and ORL-specific reports. The ICF CSHL provides a framework for describing each hearing-impaired individual’s unique capabilities and needs in ways currently not documented by audiological and otological evaluations, potentially setting the stage for more effective individualized patient care. Efforts to further validate the ICF CSHL may require the involvement of multidisciplinary institutions with commonly shared electronic health records to adequately capture the breath of the ICF CSHL.