Relationship of vitamin D levels with clinical presentation and recurrence of BPPV in a Southeastern United States institution.

OBJECTIVE: To examine the relationship of 25hydroxyvitamin D serum levels with BPPV incidence and recurrence rates. METHODS: A retrospective cross-sectional, case-controlled study with follow-up phone survey was performed on patients diagnosed with BPPV between 05/2017-05/2020, who had available 25hydroxyvitamin D serology. Patients were seen at a multidisciplinary, vestibular-focused, neurotology clinic at a tertiary referral center. Controls consisted of subjects from the National Health and Nutrition Examination Survey (NHANES), and a locoregional age, sex, and race-matched group of patients from our institution. RESULTS: Our BPPV cohort consisted of 173 patients (mean age 66.2 ± 11.8 years), who were predominately female (75.7%) and Caucasian (76.3%). Almost all age subgroups (BPPV, NHANES, and locoregional groups) ≤60 years old had insufficient levels of vitamin D. However, the overall BPPV cohort had a significantly higher vitamin D level than the NHANES control (31.4 ± 16.5 v. 26.0 ± 11.2 ng/mL, d=0.474 [0.323, 0.626]). There was no significant difference when compared to the overall locoregional control (31.4 ± 20.5 ng/mL). Migraines were significantly correlated to increased BPPV recurrence rates on univariate (beta=0.927, p=0.037, 95% CI: [0.057, 1.798]) and multiple regression analyses (beta=0.231, 95% CI: [0.024, 2.029], p=0.045). Furthermore, patients with BPPV recurrences had significantly lower levels of vitamin D at initial presentation when compared to patients with no recurrences (29.0 ± 12.0 v. 37.6 ± 18.3 ng/mL, d=0.571[0.139,1.001]). CONCLUSION: Many BPPV patients in our cohort had insufficient vitamin D levels, and patients with BPPV recurrences had insufficient and significantly lower vitamin D levels than those without. As a readily available and affordable supplement, vitamin D may be used as an adjunct treatment but prospective studies should be done to confirm if it can prevent or reduce recurrence.

Lateral Modified Brandt-Daroff Exercises: A Novel Home Treatment Technique for Horizontal Canal BPPV.

OBJECTIVES: Brandt-Daroff exercises (BDEs) are commonly used as an at-home treatment for posterior canalithiasis, but their efficacy in the treatment of benign paroxysmal positional vertigo (BPPV) of the horizontal canal (HC-BPPV) has not been previously studied. Using biomechanical model simulation, we investigated modifications that may optimize BDE use for HC-BPPV treatment. MATERIALS AND METHODS: The BPPV Viewer, a three-dimensional model of the human labyrinth, was used to analyze BDE for HC-BPPV treatment. While moving the model through sequential BDE positions, the expected position of otoliths was demonstrated. Treatment steps were adjusted to maximize otolith movement around the canal circumference without compromising otolith repositioning into the semicircular duct's anterior arm. All adjustments were integrated into lateral modified BDEs (LMBDEs) presented here. RESULTS: By implementing several modifications, BDE can effectively treat HC-BPPV. Model simulation indicates tilting the head 20° upward in the lateral position, instead of 45° specified by the original technique, which significantly increases displacement of otoliths originating from the horizontal duct's anterior and intermediate segments. LMBDE can be performed as a direct two-step sequence without pausing in the upright position before switching sides. If the affected ear is known, positioning the head 45° below horizontal on the unaffected side as a third treatment step can promote actual canal evacuation. These treatment enhancements increase circumferential otolith movement around the canal and may promote horizontal canal evacuation. CONCLUSION: LMBDEs are a modification of BDE that may increase their effectiveness for use in patients with HC-BPPV. This safe treatment adjunct between office visits may promote long-term symptom reduction.

BPPV Viewer: A downloadable 3D BPPV model for study of otolith disease.

OBJECTIVE: To develop a downloadable three-dimensional (3D) study tool of the membranous labyrinth in order to facilitate the study of benign paroxysmal positional vertigo (BPPV). BACKGROUND: The diagnosis and treatment of BPPV depend on an understanding of the anatomy of the vestibular labyrinth and its position relative to the head. To date, many illustrations have been made to explain principles of diagnosis and treatment of BPPV, but few have been based on anatomical studies of the membranous labyrinth. METHODS: A previously reported 3D model of a human labyrinth was transposed to a 3D development software to allow the creation of markers along the semicircular ducts and utricle. These markers represent otoliths at different positions during movement of the model within the 3D environment. User-friendly tools were created to navigate the model, to allow clear documentation and communication of studied problems, and to study the model across relevant planes. The final model can be downloaded and is available for general useat https://bppvviewer.com/download/. RESULTS: The model allows visualization of true membranous labyrinth anatomy in both ears simultaneously. The dependent portion of each semicircular duct, the planes of the cristae, and the position of the utricle can easily be visualized in any head position. Moveable markers can mark the expected progress of otolith debris with changes in head position and images can be captured to document simulations in various draw styles. CONCLUSION: This simple model could offer insights that lead to more accurate diagnosis and treatment of BPPV. It may also be useful as a tool to teach BPPV.

Care Gaps and Recommendations in Vestibular Migraine: An Expert Panel Summit.

Vestibular migraine (VM) is an increasingly recognized pathology yet remains as an underdiagnosed cause of vestibular disorders. While current diagnostic criteria are codified in the 2012 Barany Society document and included in the third edition of the international classification of headache disorders, the pathophysiology of this disorder is still elusive. The Association for Migraine Disorders hosted a multidisciplinary, international expert workshop in October 2020 and identified seven current care gaps that the scientific community needs to resolve, including a better understanding of the range of symptoms and phenotypes of VM, the lack of a diagnostic marker, a better understanding of pathophysiologic mechanisms, as well as the lack of clear recommendations for interventions (nonpharmacologic and pharmacologic) and finally, the need for specific outcome measures that will guide clinicians as well as research into the efficacy of interventions. The expert group issued several recommendations to address those areas including establishing a global VM registry, creating an improved diagnostic algorithm using available vestibular tests as well as others that are in development, conducting appropriate trials of high quality to validate current clinically available treatment and fostering collaborative efforts to elucidate the pathophysiologic mechanisms underlying VM, specifically the role of the trigemino-vascular pathways.

Short CRP for Anterior Canalithiasis: A New Maneuver Based on Simulation With a Biomechanical Model.

Introduction/Objective: Anterior canalithiasis is an uncommon and challenging diagnosis. This is due in part to the difficulty of defining the affected side, the extreme positioning required to carry out described therapeutic maneuvers, and the infrequent use of specific maneuvers. Our objective is to present a new treatment alternative for anterior canalithiasis which is based on the well-known canalith repositioning procedure (CRP) described by Epley and which is used routinely in the treatment of both posterior and anterior canalithiasis. Analysis of the standard CRP for anterior canalithiasis with a biomechanical model validates that this new maneuver is an enhanced treatment option for anterior canalithiasis. We call the new maneuver the "short CRP." Methods: A previously published 3D biomechanical model of the human labyrinths for the study of BPPV was used to analyze the conventional CRP in the treatment of anterior canalithiasis. The expected position of free otoliths near the anterior ampulla of the anterior semicircular duct was followed while recreating the sequential positions of the CRP. Although the standard CRP was possibly effective, certain enhancements were evident that could increase successful repositioning. These enhancements were incorporated into the modification of the CRP presented here as the "short CRP" for anterior canalithiasis. Results: The traditional CRP used for posterior canalithiasis can also be used for anterior canalithiasis. Although in the traditional CRP the head hangs 30° below horizontal, our simulation shows that a 40° head-hang below horizontal is an enhancement and may ensure progression of anterior otolith debris. Elimination of Position 4 of the classic CRP, in which the face is turned 45° toward the floor, was also seen as an enhancement as this position is predicted to cause retrograde movement of otoliths back into the anterior canal if the patient tucks the chin in position 4 or when sitting up. Conclusion: A modification of the CRP called the "short CRP" can be used to treat anterior canalithiasis. Model analysis predicts possible increased efficacy over the standard CRP. Model analysis of existing BPPV treatments is a valuable exercise for examination and can lead to realistic enhancements in patient care.

Cochlear implantation in a patient with mucopolysaccharidosis IVA.

Mucopolysaccharidosis IVA (OMIM 253000; also known as Morquio A syndrome) is associated with skeletal, airway, and hearing abnormalities. Cochlear implantation is an effective intervention for patients with severe-to-profound hearing loss. Patients can gain substantial improvement in auditory performance, speech perception, and their quality of life from cochlear implantation. Although severe progressive sensorineural hearing loss is a common feature of mucopolysaccharidosis IVA, no detailed description of cochlear implantation for mucopolysaccharidosis IVA has been reported. To review the effectiveness and special considerations associated with cochlear implantation in patients with mucopolysaccharidosis IVA, we here report the case of cochlear implantation in mucopolysaccharidosis IVA by a multidisciplinary team. A retrospective chart review was conducted on a 34-year-old female with mucopolysaccharidosis IVA, who received a cochlear implant. Audiometric thresholds, speech perception scores, and cochlear implant processor mapping information were reviewed during the first 12 months following cochlear implantation. The results of audiological tests indicate improved hearing thresholds as well as remarkable enhancement of speech perception skills over 12 months of cochlear implant use. Cochlear implantation improved auditory performance in a mucopolysaccharidosis IVA patient with postlingually severe-to-profound sensorineural hearing loss. The benefits of cochlear implantation could be meaningful for other Morquio patients with progressive hearing loss, although the risks of surgery and anesthesia should be carefully considered by a multidisciplinary team of experts during the cochlear implant candidacy process.

Patterns of Migraine Disease in Otolaryngology: A CHEER Network Study.

Objective To evaluate the prevalence of migraine disease in an otolaryngologic cohort and migraine-related otologic and sinonasal symptoms in this population. Study Design Cross-sectional study utilizing the CHEER (Creating Healthcare Excellence through Education and Research) network for recruitment. Setting Patients were recruited in a cross-sectional and pragmatic manner in 14 CHEER sites between June 2015 and March 2017 (9 academic, 5 community based). Subjects and Methods Patients were included if they were aged ≥18 years and seen for any concern that was not head and neck cancer. Patients with any history of brain abnormality or headaches that began within 2 weeks of a medical illness, trauma, or head injury were excluded. Patients were screened for migraine with a validated instrument. If they screened positive on the Migraine Assessment Tool (MAT+), the subjects also filled out validated and custom questionnaires for sinonasal, otologic, and migraine-specific symptoms. Results Of 1458 patients screened, 235 (16.1%) screened positive for migraine (MAT+), which is higher than general population (13%, P < .001). The MAT+ group was significantly younger (47.2 vs 55.6 years of age, P < .001) and predominantly women (80.0% vs 55.9%, P < .001). The MAT+ cohort commonly reported ear- and sinus-related symptoms, such as tinnitus (70.5%), ear pressure (61.9%), balance problems (82%), facial pressure (85%), and rhinorrhea (49.9%). There were significantly higher levels of sinus burden with higher levels of dizziness handicap, Jonckheere-Terpstra test = 11,573.00, z = 7.471, P < .001. Conclusion Migraine disease has a higher prevalence in an otolaryngologic cohort than in the general population, presenting with a high rate of sinonasal and otologic symptoms that may be due to or exacerbated by migraines.

Qualitative analysis of the Dix-Hallpike maneuver in multi-canal BPPV using a biomechanical model: Introduction of an expanded Dix-Hallpike maneuver for enhanced diagnosis of multi-canal BPPV.

INTRODUCTION/OBJECTIVE: Multiple canal BPPV can be a diagnostic challenge to the clinician. This is due in part to the complex anatomy of the labyrinth but also to complex and often simultaneous ocular responses that result from stimulation of multiple canals during traditional diagnostic testing. Our objective was to analyze the Dix-Hallpike maneuver used in the diagnosis of BPPV to look for patterns of simultaneous canal response and to develop a diagnostic maneuver that will allow separation of canal responses in multiple canal BPPV. METHODS: A previously created and published 3D biomechanical model of the human labyrinths for the study of BPPV was used to analyze and compare the position and movement of otoliths in the Dix-Hallpike maneuver as well as in a proposed expanded version of the traditional Dix-Hallpike maneuver. RESULTS: The traditional Dix-Hallpike maneuver with the head hanging may promote movement of otoliths in 5 of the six semicircular canals. The Dix-Hallpike maneuver with the head lowered only to the horizontal position allows for otoconia in only the lowermost posterior canal to fall to the most gravity dependent position. This position allows for minimal or no movement of otoconia in the contralateral posterior canal, or in either superior canal. Turning the head ninety degrees to the opposite side while still in the horizontal position will provoke otolith movement in only the contralateral posterior canal. The superior canals can then be examined for free otolith debris by extending the neck to a head-hanging position. These positions may be assumed directly from one to the next in the lying position. There seems to be no advantage to sitting up between positions. CONCLUSION: The Dix-Hallpike maneuver may cause simultaneous movement of otoliths present in multiple canals and create an obstacle to accurate diagnosis in multi-canal BPPV. An expanded Dix-Hallpike maneuver is described which adds intermediate steps with the head positioned to the right and left in the horizontal position before head-hanging. This expanded maneuver has helped to isolate affected semi-circular canals for individual assessment in multiple canal BPPV.

A 3D benign paroxysmal positional vertigo model for study of otolith disease.

OBJECTIVE: To develop a three-dimensional study tool of the membranous labyrinth in order to study the pathophysiology, diagnostic workup and treatment of benign paroxysmal positional vertigo (BPPV). BPPV is the most common cause of peripheral vertigo. Its diagnosis and treatment depend on an understanding of the anatomy of the vestibular labyrinth and its position relative to the head. To date, many illustrations have been made to explain principals of diagnosis and treatment of BPPV, but few have been based on anatomical studies of the membranous labyrinth. METHODS: A cadaveric human membranous labyrinth was axially sectioned at 20 µm resolution, stained and segmented to create a high-resolution digital model. The model was cloned to create an enantiomeric pair of labyrinths. These were associated a 3D model of a human skull, segmented from MRI data, and were oriented according to established anatomic norms. Canal markers representing otoliths were created to mark canalith position during movement of the model within the 3D environment. RESULTS: The model allows visualization of true membranous labyrinth anatomy in both ears simultaneously. The dependent portion of each semicircular duct and of the utricle can easily be visualized in any head position. Moveable markers can mark the expected progress of otolith debris with changes in head position and images can be captured to document simulations. The model can be used to simulate pathology as well as diagnostic maneuvers and treatment procedures used for BPPV. The model has great potential as a teaching tool. CONCLUSION: A simple model based on human anatomy has been created to allow careful study of BPPV pathophysiology and treatment. Going forward, this tool could offer insights that may lead to more accurate diagnosis and treatment of BPPV.

Normative inner ear volumetric measurements.

In the current study, we attempted to determine normative inner ear volumetric measurements generated from three-dimensional computed tomography (CT) images. In addition, we investigated a correlation between the axial length and the volume of the labyrinth and discussed clinical outcomes of this correlation. Amira 5.2.2 software was used to create three-dimensional isosurface images of the human labyrinth using two-dimensional CT images from 35 anatomically normal patients. With the three-dimensional labyrinths, complete dimensional analysis was performed to gain insight into both the volume and the greatest axial length of the inner ear. Paired t test and Pearson correlation were used. Our volume of the inner ear inquiry reported a mean volume of 221.5 with SD of 24.3 µL (0.228 µL for males and 0.218 µL for females). The length showed a mean of 1.713 cm with SD of 0.064 cm (1.753 cm for males and 1.695 cm for females). The length was used to estimate the volume, and the estimates were within 10% of the measured volume 74.3% of the time. Normative volumetric measurements of the inner ear can be obtained by using three-dimensional CT Imaging by Amira 5.2.2 software. There was a statistically significant positive correlation between the axial length of the labyrinth and the volume of the labyrinth. The axial length of the labyrinth could be used to estimate the volume of the labyrinth, which may be clinically important to estimate the concentration of the drug distributed in the inner ear.

Inflammatory pseudotumor of the nasopharynx with spread along the trigeminal nerve.

Inflammatory pseudotumor of the nasopharynx is a rare diagnosis that is often misinterpreted as carcinoma or lymphoma. It has been referred to as a tumefactive fibroinflammatory lesion, idiopathic pseudotumor, and fibrosing inflammatory pseudotumor. We present a rare case of a 40-year-old African American female from Kenya with inflammatory pseudotumor of the nasopharynx with perineural spread of disease along the trigeminal nerve to discuss the diagnosis and treatment of such an uncommon entity.

Anastomoses of the vestibular, cochlear, and facial nerves.

The internal auditory canal (IAC) is 10 to 17 mm in length, and the facial nerve and vestibulocochlear nerve, which consist of the cochlear nerve, the superior vestibular nerve, and the inferior vestibular nerve, run together in the IAC packaged in dura mater. Oort first described the vestibulocochlear anastomoses in 1918, which is important for the understanding of the pathogenesis and pathophysiology of otologic disorders. The current study documents the existence of vestibulofacial and vestibulocochlear neural connections and topographical relationship of the nerves as part of a radiologic evaluation of 73 human temporal bones from brainstem to the lateral portion of IAC.

Histopathology of the temporal bone in a case of superior canal dehiscence syndrome.

OBJECTIVES: We describe the histopathologic findings in the temporal bones of a patient who had, during life, received a diagnosis of superior canal dehiscence (SCD) syndrome. METHODS: The patient was found to have SCD syndrome at 59 years of age. She became a temporal bone donor, and died of unrelated causes at 62 years of age. Both temporal bones were prepared in celloidin and examined by light microscopy. RESULTS: The patient developed bilateral aural fullness, pulsatile tinnitus, and difficulty tolerating loud noises after minor head trauma at 53 years of age. The symptoms were worse on the right. She also had Valsalva-induced dizziness and eye movements, as well as sound-induced dizziness (more prominent on the right). Audiometry showed a small air-bone gap of 10 dB in the right ear. Vestibular evoked myogenic potential testing showed an abnormally low threshold of 66 dB on the right, and a computed tomography scan showed dehiscence of the superior canal on the right. Histopathologic examination of the right ear showed a 1.4 x 0.6-mm dehiscence of bone covering the superior canal. Dura was in direct contact with the endosteum and the membranous duct at the level of the dehiscence. No osteoclastic process was evident within the otic capsule bone surrounding the dehiscence. The left ear showed thin but intact bone over the superior canal. Both ears showed focal microdehiscences of the tegmen tympani and tegmen mastoideum. The auditory and vestibular sense organs on both sides appeared normal. No endolymphatic hydrops was observed. CONCLUSIONS: The findings were consistent with the hypothesis put forth by Carey and colleagues that SCD may arise from a failure of postnatal bone development, and that minor trauma may disrupt thin bone or stable dura over the superior canal.

Transmastoid middle fossa craniotomy repair of superior semicircular canal dehiscence using a soft tissue graft.

OBJECTIVE: To describe the successful surgical treatment of 5 cases of superior semicircular canal dehiscence via a transmastoid middle fossa craniotomy using a soft tissue graft. DESIGN: Case report. SETTING: Private practice otologic referral center. RESULTS: All patients have experienced reduction in auditory and vestibular symptoms. Pulsatile tinnitus and autophony are now absent in the operated ears. Chronic disequilibrium is subjectively improved. Patients with sound evoked eye movements no longer have sound sensitivity on the operated side. Head thrust testing indicates no obstruction of the operated superior canal in all patients with normal head thrust preoperatively. Audiometry is unchanged from preoperation, and cervical vestibular-evoked myogenic potential thresholds have increased on the operated side in 4 of 4 patients. Three patients had dehiscence at the superior petrosal sinus inaccessible to standard middle fossa repair. All patients were discharged to home the morning after surgery. CONCLUSION: Transmastoid craniotomy repair of the superior semicircular canal dehiscence using a soft tissue graft offers numerous advantages over traditional surgical approaches and can be performed safely in the outpatient setting. The strategy is particularly useful in patients with dehiscence at the superior petrosal sinus. This article will review our strategy and discuss the advantages and disadvantages of the different surgical treatments used for patients with severe symptoms from superior canal dehiscence.

Otalgia associated with migraine.

OBJECTIVE: To investigate the association of otalgia and migraine. STUDY DESIGN: Retrospective survey with evaluation of otalgia response to migraine treatment. Only patients with minimum symptom duration of 3 months, who accepted migraine treatment and had a minimum follow-up of 3 months, were included. SETTING: Single neurotology practice. SUBJECTS: All patients with otalgia in whom other causes of otalgia had been excluded and who were treated with migraine therapies. INTERVENTION: Standard first-line abortive and prophylactic migraine therapies. MAIN OUTCOME MEASURES: Specific clinical data, as well as pretreatment and posttreatment severity scores, were gathered. Response to treatment was assessed by comparing pretreatment and posttreatment symptom scores using paired t test. RESULTS: A total of 26 patients were included. Ninety-two percent responded to migraine therapy with improved symptom frequency, severity, and duration (p < 0.001). Median duration of symptoms was 5 years. Mean delay to response was 2.3 weeks, and mean follow-up was 20 months. Otalgia was the chief complaint in 77%. Pain was dull in 35%, sharp in 19%, throbbing in 19%, and mixed in 27%. Sixty-five percent demonstrated triggerability of otalgia. A total of 65% had International Headache Society migraine. Patients responded to many classes of migraine preventive and abortive medications. CONCLUSION: Otalgia of unclear cause can be related to migraine mechanisms. Our group showed a high prevalence of migraine characteristics, including headache, migraine-associated symptoms, patterns of triggerability, and response to migraine treatment. Clinical criteria for diagnosis of migraine-associated otalgia are suggested for future prospective study.

Clinical experience with symptomatic superior canal dehiscence in a single neurotologic practice.

OBJECTIVE: To discuss the range of signs and symptoms of patients with superior canal dehiscence syndrome identified in a single neurotologic practice. STUDY DESIGN: Retrospective chart review of 35 patients diagnosed with superior canal dehiscence from April 2000 to June 2007. RESULTS: All patients had evidence of superior canal dehiscence on computed tomography. These 35 patients represented 0.56% of new patients seen over a five-year period. The incidence of clinical symptoms and signs is compared to other published series. The mean vestibular evoked myogenic potential thresholds of affected ears are significantly lower than the mean vestibular evoked myogenic potential thresholds of unaffected ears. To date, 5 of 35 patients have undergone transmastoid occlusion of the superior semicircular canal with significant improvement in clinical symptoms in 4 of 5 cases. CONCLUSION: Not all patients with a diagnosis of superior canal dehiscence syndrome will have classic symptoms and signs. A high index of suspicion with careful clinical examination and properly performed ancillary testing is required to confirm this diagnosis.

A virtual-reality approach for the treatment of benign paroxysmal positional vertigo.

Benign Paroxysmal Positional Vertigo (BPPV) is a common cause of dizziness caused by debris, which has collected within the semicircular canals of the inner ear. Stereoscopic representations of the human labyrinth are constructed and incorporated into a downloadable viewing platform to allow visualization of straightforward and complex variations of BPPV.