Larry Lundy

The Role of Extraesophageal Reflux in Otitis Media in Infants and Children

Objectives/Hypothesis: Gastroesophageal reflux disease (GERD) is common in children, and extraesophageal reflux disease (EORD) has been implicated in the pathophysiology of otitis media (OM). We sought to 1) determine the incidence of pepsin/pepsinogen presence in the middle ear cleft of a large sample of pediatric patients undergoing myringotomy with tube placement for OM; 2) compare this with a control population of pediatric patients undergoing middle ear surgery (cochlear implantation) with no documented history of OM; 3) analyze potential risk factors for OM in children with EORD demonstrated by the presence of pepsin in the middle ear cleft; and 4) determine if pepsin positivity at the time of myringotomy with tube placement predisposes to posttympanostomy tube otorrhea.

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°C) maximum slow-phase velocities was smaller than those from warm water (44°C). Maximum slow-phase velocity distributions from cool (21°C) and warm (51°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.

The Effect of CO2 and KTP laser on the cat saccule and utricle

To assess the potential carbon dioxide (CO2) and potassium‐titanyl‐phosphate (KTP) laser‐related trauma to the saccule and utricle in a cat model.

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.

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.

The effect of repetition rate on air-conducted ocular vestibular evoked myogenic potentials (oVEMPs)

PURPOSE Ocular vestibular evoked myogenic potentials (oVEMPs) are used to describe utricular/superior vestibular nerve function; however, optimal recording parameters have not been fully established. This study investigated the effect of repetition rate on air-conducted oVEMPs. METHOD Ten healthy adults were evaluated using 500-Hz tone bursts (4-ms duration, Blackman gating, 122 dB pSPL). Four repetition rates were used (1.6, 4.8, 8.3, and 26.6 Hz) and resulting oVEMP response presence, amplitude, amplitude asymmetry, and n1/p1 latency were assessed. RESULTS Response presence was significantly reduced for 26.6 Hz using monaural stimulation and for 8.3 Hz and 26.6 Hz for binaural stimulation. For monaural stimulation using 1.6, 4.8, and 8.3 Hz, no significant differences were noted for amplitude or latency. Responses obtained using binaural stimulation demonstrated a significant effect of rate on amplitude, with 8.3 Hz producing significantly reduced amplitude. Binaural amplitudes were significantly larger than monaural contralateral responses but with reduced response presence. No significant differences were noted for latency or amplitude asymmetry. CONCLUSION Using repetition rates of approximately 5 Hz or less may produce more consistent oVEMP response presence with minimal effects on amplitude for monaural or binaural recordings.

Errors in Items and Algorithm in Questionnaire Used in Validation Study

Errors in Items and Algorithm in Questionnaire Used in Validation Study To the Editor We write to report a scoring algorithm error that was included in our article,1 “Development and Initial Validation of a Consumer Questionnaire to Predict the Presence of Ear Disease,” published online on August 3, 2017, and in the October 2017 issue of JAMA Otolaryngology–Head & Neck Surgery. In this study, we examined the validity of the Consumer Ear Disease Risk Assessment (CEDRA), a questionnaire for the self-assessment of risk of ear diseases associated with hearing loss. The error was discovered by a member of our research team who was preparing a digital version of the questionnaire used in our study. The error occurred in the analysis code for 3 of the 28 questionnaire items included in the scoring (questions 16, 18, and 19). For these 3 questions, the 4-item rating scales were dichotomized incorrectly. After correcting the algorithm for these questions, we found slightly different results, without any differences in statistical significance of these results. The overall results and conclusions remain unchanged. Specifically, after using the corrected algorithm, the following occurred: The odds ratio of the CEDRA score increased to 1.84 (95% CI, 1.56-2.17) from the previously reported 1.77 (95% CI, 1.51-2.07). As previously reported, a predicted probability of disease >.5 corresponded to a CEDRA score of 4. Using this cutoff, we obtained 90% (95% CI, 84%94%) sensitivity and 72% (95% CI, 59%-82%) specificity in our training sample (n = 246). The sensitivity and specificity of the training sample were previously erroneously reported to be 94% (95% CI, 89%-97%) and 61% (95% CI, 47%-73%), respectively. Applied to the test sample (n = 61), we found 76% (95% CI, 61%-87%) sensitivity and 80% (95% CI, 51%-95%) specificity, which were originally erroneously reported to be 85% (95% CI, 71%-93%) and 47% (95% CI, 22%-73%), respectively. The errors and corrections affect the data as reported in the Abstract, Methods, Results, and Discussion sections, and the Figure. We apologize for any confusion caused by the errors and have requested that the article be corrected online.2

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 patients 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.

Novel radiology method for investigating middle ear myoclonus

Neuroanatomy has traditionally been investigated through surgery, cadaveric exploration, and with histological tissue examination. Newer radiological techniques are noninvasive and include computed tomography (CT), magnetic resonance (MR) imaging, MR tractography, functional MRI (fMRI), and angiography (by means of contrasted CTor MR, or digital subtraction angiography [DSA]). Even under the best situations, these modalities can be limited by artifacts, resolution, resource utilization, and interpretation (Yousem and Grossman, 2010 ). We describe a new imagingmodality which assisted inmanagement of a case of “middle ear myoclonus.” A 62-year-old right-handed woman presented with 30 years of bilateral “ear clicking.” The clicking was synchronous with bilateral eye blinking/blepharospasm. Her medical history was only significant for otitis media as a child resulting in a left tympanostomy tube placement. Otherwise her social and family history was unremarkable. She had been treated with benzodiazepines without success. Examination demonstrated rhythmic bilateral tympanic membrane pulsations in conjunction with bilateral eye blinking. Middle ear myoclonus is a rare entity of abnormal, repetitive muscle contractions of the middle ear. The condition has been described both unilaterally and bilaterally, and with involvement of either the tensor tympani or stapedius muscles (Ellenstein et al., 2013). Associated blepharospasm is seen in rare cases and highlights the variability of possible lesion localization. Potential localization includes central or peripheral etiologies (basal ganglia, brainstem, or Guillain–Mollaret triangle for central, or trigeminal nerve affecting the tensor tympani, or the facial nerve affecting the stapedius and orbicularis muscles for peripheral). Management of vascular compression and irritation of cranial nerves can be amendable to microvascular decompression (MVD). Though the efficacy and safety evidence for MVD for middle ear myoclonus is sparse, there is data for MVD in patients with hemifacial spasm and trigeminal neuralgia, showing good efficacy (91.1% and 84.8–87.5% symptom relief, respectively), with complication rates <10%, including low risk of stroke or death (both <1.0%) (Miller and Miller, 2012; Qi et al., 2016; Phan et al., in press). We obtained an MRI and MR angiogram of her brain. T2-weighted sequences with thin slices through the cerebellopontine angle showed a close course of blood vessels and cranial nerve roots. The right superior cerebellar artery ran close to the root of the trigeminal nerve and a vein (possibly the lateral pontine vein or vein of the middle cerebellar peduncle) ran close to the root of the left facial nerve. Both of these can be normal anatomical variants or could cause bilateral mononeuropathies due to vascular irritation. The latter would likely not explain her bilateral symptoms (especially bilateral eye blinking). The MRI was unable to clearly elucidate the relationship between the vessels and nerve roots. We uploaded this MRI sequence into the Anatomage TableR . Using this tool, in conjunction with Anatomage Table software engineers, three-dimensional renderings were produced of the pons, cerebellum, pertinent cranial nerves, and blood vessels. These nerve tracts and blood vessel pathways were highlighted and colored (Supporting Information Videos 1 and Video 2). Using this technology, we found that there was no contact between these cranial nerve roots by their respective blood vessels. She did not need microvascular decompression, and her auditory symptoms resolved with bilateral tensor tympanectomy (with residual bilateral blepharospam). With the Anatomage Table, we were able to understand the anatomy of the posterior cranial fossa, cranial nerve roots, and posterior circulation of blood vessels. We do not wish to promulgate the Anatomage Table, but rather to illustrate how using nonconventional or nontraditional radiology modalities can help the management of difficult neuroanatomical cases. The technology helped us confirmed our suspicions that her pathology was likely not due to peripheral nerve irritation but rather the central pathology (dystonia). We recommend exploring further the utility of using new radiologic technology when working up and managing patients where neuroanatomy is a key feature with a localization dilemma.

Posterior fossa anatomy through novel radiology.

Neuroanatomy has traditionally been investigated through surgery, cadaveric exploration, and with histological tissue examination. Newer radiological techniques are noninvasive and include computed tomography (CT), magnetic resonance (MR) imaging, MR tractography, functional MRI (fMRI), and angiography (by means of contrasted CTor MR, or digital subtraction angiography [DSA]). Even under the best situations, these modalities can be limited by artifacts, resolution, resource utilization, and interpretation (Yousem and Grossman, 2010 ). We describe a new imagingmodality which assisted inmanagement of a case of “middle ear myoclonus.” A 62-year-old right-handed woman presented with 30 years of bilateral “ear clicking.” The clicking was synchronous with bilateral eye blinking/blepharospasm. Her medical history was only significant for otitis media as a child resulting in a left tympanostomy tube placement. Otherwise her social and family history was unremarkable. She had been treated with benzodiazepines without success. Examination demonstrated rhythmic bilateral tympanic membrane pulsations in conjunction with bilateral eye blinking. Middle ear myoclonus is a rare entity of abnormal, repetitive muscle contractions of the middle ear. The condition has been described both unilaterally and bilaterally, and with involvement of either the tensor tympani or stapedius muscles (Ellenstein et al., 2013). Associated blepharospasm is seen in rare cases and highlights the variability of possible lesion localization. Potential localization includes central or peripheral etiologies (basal ganglia, brainstem, or Guillain–Mollaret triangle for central, or trigeminal nerve affecting the tensor tympani, or the facial nerve affecting the stapedius and orbicularis muscles for peripheral). Management of vascular compression and irritation of cranial nerves can be amendable to microvascular decompression (MVD). Though the efficacy and safety evidence for MVD for middle ear myoclonus is sparse, there is data for MVD in patients with hemifacial spasm and trigeminal neuralgia, showing good efficacy (91.1% and 84.8–87.5% symptom relief, respectively), with complication rates <10%, including low risk of stroke or death (both <1.0%) (Miller and Miller, 2012; Qi et al., 2016; Phan et al., in press). We obtained an MRI and MR angiogram of her brain. T2-weighted sequences with thin slices through the cerebellopontine angle showed a close course of blood vessels and cranial nerve roots. The right superior cerebellar artery ran close to the root of the trigeminal nerve and a vein (possibly the lateral pontine vein or vein of the middle cerebellar peduncle) ran close to the root of the left facial nerve. Both of these can be normal anatomical variants or could cause bilateral mononeuropathies due to vascular irritation. The latter would likely not explain her bilateral symptoms (especially bilateral eye blinking). The MRI was unable to clearly elucidate the relationship between the vessels and nerve roots. We uploaded this MRI sequence into the Anatomage TableR . Using this tool, in conjunction with Anatomage Table software engineers, three-dimensional renderings were produced of the pons, cerebellum, pertinent cranial nerves, and blood vessels. These nerve tracts and blood vessel pathways were highlighted and colored (Supporting Information Videos 1 and Video 2). Using this technology, we found that there was no contact between these cranial nerve roots by their respective blood vessels. She did not need microvascular decompression, and her auditory symptoms resolved with bilateral tensor tympanectomy (with residual bilateral blepharospam). With the Anatomage Table, we were able to understand the anatomy of the posterior cranial fossa, cranial nerve roots, and posterior circulation of blood vessels. We do not wish to promulgate the Anatomage Table, but rather to illustrate how using nonconventional or nontraditional radiology modalities can help the management of difficult neuroanatomical cases. The technology helped us confirmed our suspicions that her pathology was likely not due to peripheral nerve irritation but rather the central pathology (dystonia). We recommend exploring further the utility of using new radiologic technology when working up and managing patients where neuroanatomy is a key feature with a localization dilemma.