Evaluation of clinical predictors of postoperative outcomes in tegmen defect patients with and without concurrent superior semicircular canal dehiscence and cerebrospinal fluid leak.

OBJECTIVES: Tegmen and superior semicircular canal defects have been well studied, yet the factors contributing to their onset and progression are widely debated. The clinical utility of intraoperative intracranial pressure measurements has yet to be tested. This report aims to use intraoperative opening pressure and concurrent superior semicircular canal dehiscence (SSCD) to analyze factors influencing disease course and clinical outcomes in patients with tegmen dehiscence. METHODS: A retrospective analysis of 61 patients who underwent tegmen defect repair was performed. Multiple variables of interest including body mass index (BMI), presence of SSCD, presence of dural venous sinus stenosis, opening pressure, and acetazolamide therapy use were recorded. The cohort was divided into those with or without concurrent SSCD and those presenting with or without cerebrospinal fluid (CSF) leak for analysis. RESULTS: A linear relationship between opening pressure and BMI (p = 0.009) was noted; however, intraoperative opening pressure was not associated with disease outcome. Concurrent SSCD was present in 25 % of patients, while 62 % presented with CSF leak. The concurrent SSCD group exhibited higher opening pressure, higher likelihood of having dural sinus stenosis, and higher likelihood of being discharged on acetazolamide. The CSF leak group had higher likelihood of obstructive sleep apnea and persistent symptoms. CONCLUSIONS: In patients undergoing tegmen defect repair, concurrent SSCD suggests increased disease severity. The presence of preoperative CSF leak predicts persistent symptoms following repair. BMI is linearly correlated with intracranial pressure in these patients.

Probabilities of Isolated and Co-Occurring Vestibular Disorder Symptom Clusters Identified Using the Dizziness Symptom Profile.

OBJECTIVES: Dizziness is among the most common reasons people seek medical care. There are data indicating patients with dizziness, unsteadiness, or vertigo may have multiple underlying vestibular disorders simultaneously contributing to the overall symptoms. Greater awareness of the probability that a patient will present with symptoms of co-occurring vestibular disorders has the potential to improve assessment and management, which could reduce healthcare costs and improve patient quality of life. The purpose of the current investigation was to determine the probabilities that a patient presenting to a clinic for vestibular function testing has symptoms of an isolated vestibular disorder or co-occurring vestibular disorders. DESIGN: All patients who are seen for vestibular function testing in our center complete the dizziness symptom profile, a validated self-report measure, before evaluation with the clinician. For this retrospective study, patient scores on the dizziness symptom profile, patient age, and patient gender were extracted from the medical record. The dizziness symptom profile includes symptom clusters specific to six disorders that cause vestibular symptoms, specifically: benign paroxysmal positional vertigo, vestibular migraine, vestibular neuritis, superior canal dehiscence, Meniere disease, and persistent postural perceptual dizziness. For the present study, data were collected from 617 participants (mean age = 56 years, 376 women, and 241 men) presenting with complaints of vertigo, dizziness, or imbalance. Patients were evaluated in a tertiary care dizziness specialty clinic from October 2020 to October 2021. Self-report data were analyzed using a Bayesian framework to determine the probabilities of reporting symptom clusters specific to an isolated disorder and co-occurring vestibular disorders. RESULTS: There was a 42% probability of a participant reporting symptoms that were not consistent with any of the six vestibular disorders represented in the dizziness symptom profile. Participants were nearly as likely to report symptom clusters of co-occurring disorders (28%) as they were to report symptom clusters of an isolated disorder (30%). When in isolation, participants were most likely to report symptom clusters consistent with benign paroxysmal positional vertigo and vestibular migraine, with estimated probabilities of 12% and 10%, respectively. The combination of co-occurring disorders with the highest probability was benign paroxysmal positional vertigo + vestibular migraine (~5%). Probabilities decreased as number of symptom clusters on the dizziness symptom profile increased. The probability of endorsing vestibular migraine increased with the number of symptom clusters reported. CONCLUSIONS: Many patients reported symptoms of more than one vestibular disorder, suggesting their symptoms were not sufficiently captured by the symptom clusters used to summarize any single vestibular disorder covered by the dizziness symptom profile. Our results indicate that probability of symptom clusters indicated by the dizziness symptom profile is comparable to prior published work on the prevalence of vestibular disorders. These findings support use of this tool by clinicians to assist with identification of symptom clusters consistent with isolated and co-occurring vestibular disorders.

Scuba Diving-Induced Inner-Ear Pathology: Imaging Findings of Superior Semicircular Canal and Tegmen Tympani Dehiscence.

BACKGROUND Superior semicircular canal dehiscence is an inner-ear pathology which presents with vertigo, disequilibrium, and hearing loss. Although the exact etiology of superior semicircular canal dehiscence is unknown, it is thought that an increase in middle-ear pressure disrupts a thin overlying temporal bone. Superior semicircular canal dehiscence is frequently seen in association with dehiscence of the tegmen tympani, which overlies the middle ear. Here, we present a case report of a 52-year-old Puerto Rican man with vertigo, dizziness, vomiting, and mild hearing loss associated with superior semicircular canal and tegmen tympani dehiscence after performing improper scuba diving techniques. CASE REPORT A 52-year-old Puerto Rican man presented to the emergency department with vertigo, dizziness, vomiting, and mild hearing loss in the right ear. The symptoms began shortly after scuba diving with inadequate decompression techniques on ascent. He was treated with recompression therapy with mild but incomplete improvement in symptoms. Bilateral temporal magnetic resonance imaging was suggestive of segmental dehiscence of the right superior semicircular canal and tegmen tympani. High-resolution computed tomography of the temporal bone confirmed right superior semicircular canal and tegmen tympani dehiscence with an intact left inner ear. CONCLUSIONS The increased inner-ear pressure that occurs during scuba diving can lead to dehiscence of the superior semicircular canal and tegmen tympani, causing vertigo and hearing loss. Performance of improper diving techniques can further increase the risk of dehiscence. Therefore, appropriate radiologic evaluation of the inner ear should be performed in such patients.

Superior canal dehiscence syndrome: A review.

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages.

Trans-mastoid plugging of superior semicircular canal dehiscence: long-term follow-up.

OBJECTIVE: To evaluate the long-term outcomes of trans-mastoid plugging of superior semicircular canal dehiscence (SSCD), focusing on complicated cases. METHODS: In this cohort study, we included all patients who underwent trans-mastoid plugging of SSCD between 2009 and 2019. We evaluated the symptoms (autophony, sound-/pressure-induced vertigo, disequilibrium, aural fullness and pulsatile tinnitus) before and 1 year after surgery in the medical records. We systematically assessed the current symptoms 6.2 ± 3 years postoperative (range 2.2-12.3 years) using questionnaires sent by post and validated by telephone interviews. We also documented any complications and the need for further procedures. We compared pure tone and speech audiometry before and 1 year after surgery. Finally, the degree of mastoid pneumatisation and mastoid tegmen anatomy were reviewed on preoperative CT scans. RESULTS: We included 24 ears in 23 patients. No complications were recorded, and none required a second procedure for SSCD. Following surgery, oscillopsia and Tullio phenomena resolved in all patients. Hyperacusis, autophony, and aural fullness were also settled in all patients except one. Balance impairment persisted to some degree in 35% of patients. No deterioration over the years was reported regarding the above symptoms. On average, bone conduction pure tone average pre- and 1 year postoperative were 13.7 ± 17 and 20.5 ± 18 dB, respectively (P = 0.002). Air bone gaps were reduced from 12.7 ± 8 to 5.9 ± 6 (P = 0.001). Two patients had a significant sclerotic mastoid, three had a prominent low-lying mastoid tegmen, and two had both. Anatomy had no effect on outcome. CONCLUSION: Trans-mastoid plugging of SSCD is a reliable and effective technique which achieves long-lasting symptom control, even in cases with sclerotic mastoid or low-lying mastoid tegmen.

Relationship Between Superior Semicircular Canal Dehiscence Volume with Clinical Symptoms: Case Series.

BACKGROUND: Superior semicircular canal dehiscence (SSCD) is an osseous defect of the arcuate eminence of the petrosal temporal bone. Patients typically present with auditory and vestibular symptoms, such as hearing loss and disequilibrium. Using advanced imaging segmentation techniques, we evaluated whether the volume of SSCD correlated with preoperative symptoms and postoperative outcomes. METHODS: Our laboratory previously described a novel method of quantifying the size of an SSCD via manual segmentation. High-resolution computed tomography images of the temporal bones were imported into a specialized segmentation software. The volume of the dehiscence was outlined on consecutive slices of the coronal and axial planes via a single-pixel-thick paintbrush tool and was then calculated according to the number of nonzero image voxels. RESULTS: This study included 111 patients (70 women and 41 men; mean age, 55.1 years; age range, 24-87 years) with a total of 164 SSCDs. Mean postoperative follow-up time was 5.2 months (range, 0.03-59.5 months). The most common preoperative and postoperative symptoms were tinnitus (n = 85) and dizziness (n = 45), respectively. Surgery resulted in improvement of symptoms in most patients. The average volume of 164 SSCDs was 1.3 mm3. SSCD volume was not significantly associated with either preoperative symptoms or postoperative outcomes. CONCLUSIONS: Advances in imaging techniques have allowed increased visualization of SSCD. Further research will be necessary to evaluate the potential correlation of volume of the dehiscence with clinical variables.

Dural Contact to the Malleus Head in Patients with Superior Semicircular Canal Dehiscence (SSCD): Case Series and Review of SSCD and Tegmen Defects.

OBJECTIVE: Report a series of cases in which patients have concomitant superior semicircular canal dehiscence (SSCD) and a dehiscent tegmen tympani with Dural contact to the malleus head (DCMH). METHODS: An analysis of radiologic and audiologic data in 4 patients who presented with SSCD and DCMH at a tertiary care institution. A pertinent literature review was performed. RESULTS: Four patients (5 ears) had SSCD and DCMH. In 3 patients with unilateral DCMH, the mean maximum air-bone gap was 15 dB in the ear with DCMH compared to 50 dB in the ear without DCMH. Of the 5 ears with DCMH, the mean air conduction threshold at 250 Hz was 17 dB compared to 42 dB in the 3 ears without DCMH. CONCLUSIONS: We report the findings of DCMH in a series of 4 patients with bilateral SSCD. This limited series suggests that ears with SSCD and DCMH have less of an air-bone gap than would be expected, as 1 would expect an additive effect of DCMH and SSCD on the air-bone gap.

Influence of Semicircular Canal Dehiscence on Cochlear Implant Outcome.

INTRODUCTION: Semicircular canal dehiscence (SCD) is defined as a defect of the bone overlying the semicircular canal. It has a relatively high prevalence of 3% in the general population, which makes it likely that a certain number of patients receiving a cochlear implant (CI) would have it. However, little is known about the influence of SCD on the CI outcome. Therefore, the aim of this study was to determine the influence of SCD on CI outcome with regard to short- and long-term word perception and hearing preservation. METHODS: This study was a retrospective analysis of postoperative word perception ability in the electric-only condition after 6, 12, and ≥18 months and of hearing preservation 4 weeks after surgery in CI recipients with and without SCD. All patients received a preoperative 1.5- or 3-tesla magnetic resonance imaging. RESULTS: Fifty-five patients were included. Forty-eight patients (87%) had no SCD, and 7 patients (13%) had SCD. Mean postoperative word perception scores were 66% in the non-SCD group versus 50% in the SCD group (p = 0.17) after 6 months, 74 versus 64% (p = 0.28) after 12 months, and 77 versus 73% (p = 0.62) after 18 or more months. The mean postoperative hearing loss in patients with functional residual hearing before surgery (n = 34) was 22 dB in the non-SCD group versus 31 dB in the SCD group (p = 0.15). CONCLUSIONS: CI outcome is comparable between recipients without and with SCD. Specifically, hearing preservation rate and word perception ability in the electric-only condition seem not affected by SCD. The rate of progress of word perception ability in the first 12 months after cochlear implantation is not influenced by SCD.

Multiple Co-Occurring Vestibular Disorders Identified Using the Dizziness Symptom Profile.

Purpose The purpose of the current investigation was to determine the interrelations among vestibular disorders from a data set generated from the patient perspective as compared to previous data generated from the physician's perspective. Method The data for the current investigation originated from a previously published study describing the development of the Dizziness Symptom Profile (DSP; Jacobson et al., 2019). The DSP is a 31-item patient self-report tool designed to help primary care physicians in the development of a differential diagnosis using the patient's level of agreement with each dizziness and symptom-related statement. Responses to these items converge on common vestibular diagnoses and were previously found to agree with ear specialist differential diagnoses 70.3% of the time. Data were collected for 131 subjects (M age = 56.7 years, 72 women) seen for evaluation in a tertiary dizziness specialty clinic. For this study, the data were analyzed using descriptive statistics to determine the frequency of single diagnoses, multiple diagnoses, co-occurring diagnoses, and patterns of co-occurrence. Results Results indicated that 52.7% of patients endorsed a single vestibular diagnosis and 47.3% endorsed two or more vestibular diagnoses. Benign paroxysmal positional vertigo (BPPV) and vestibular migraine were the most common single diagnoses and also the most common co-occurring diagnoses. As the number of diagnoses endorsed on the DSP increased, so did the percentage of time that BPPV and vestibular migraine would occur. Conclusions Results support and extend the work of others but using data generated from the perspective of the patient. A slight majority of patients endorsed a single disorder, but almost as many patients endorsed more than one vestibular diagnosis. BPPV and vestibular migraine were the most common single vestibular diagnoses and also the most common co-occurring vestibular diagnoses; vestibular migraine was more common when multiple diagnoses were endorsed. Results suggest it is common for patients to volunteer symptoms that cannot be explained by a single vestibular diagnosis. This finding is in agreement with physician-generated diagnosis data. Clinicians should consider the possibility of co-occurring diagnoses in complicated patients or in patients who are not responding optimally to management of a single vestibular disorder. The DSP is a tool that encourages clinicians to consider multiple co-occurring vestibular disorders as the source of patient complaints.

Inner-Ear Disorders Presenting with Air-Bone Gaps: A Review.

Air-bone gaps (ABGs) are commonly found in patients with conductive or mixed hearing loss generally due to outer- and/or middle-ear diseases such as otitis externa, tympanic membrane perforation, interruption or fixation of the ossicular chain, and chronic suppurative otitis media. ABGs can also be found in correlation with inner-ear disorders, such as endolymphatic hydrops, enlarged vestibular aqueduct syndrome, semicircular canal dehiscence, gusher syndrome, cochlear dehiscence, and Paget disease's as well cerebral vascular anomalies including dural arteriovenous fistula. The typical clinical presentation of inner-ear conditions or cerebral vascular anomalies causing ABGs includes audiological and vestibular symptoms like vertigo, oscillopsia, dizziness, imbalance, spinning sensation, pulsatile or continuous tinnitus, hyperacusis, autophony, auricular fullness, Tullio's phenomenon, and Hennebert's sign. Establishing a definitive diagnosis of the underlying condition in patients presenting with an ABG is often challenging to do and, in many patients, the condition may remain undefined. Results from an accurate clinical, audiological, and vestibular evaluation can be suggestive for the underlying condition; however, radiological assessment by computed tomography and/or magnetic resonance imaging is mandatory to confirm any diagnostic suspicion. In this review, we describe and discuss the most recent updates available regarding the clinical presentation and diagnostic workup of inner-ear conditions that may present together with ABGs.

Symptoms, Audiometric and Vestibular Laboratory Findings, and Imaging in a Concurrent Superior Canal Dehiscence Syndrome and Vestibular Schwannoma: A Case Report.

BACKGROUND: Dizziness is a common complaint that can arise from multiple systems in the body. Objective vestibular tests are used to understand the underlying function of the vestibular system and whether or not it may be contributing to the dizziness symptoms experienced by the patient. Even when comprehensive case history is consistent with an otologic etiology, audiometric and vestibular tests are ordered to objectively characterize inner ear function to help further refine the differential diagnoses and aid in guiding treatment options. Few reports in the literature describe audiometric and vestibular results in patients with multiple concurrent otologic etiologies. PURPOSE: This case provides a description of audiometric, vestibular, and imaging results in a case of concurrent bilateral superior canal dehiscence, right-sided vestibular schwannoma, and right-sided posterior canal benign paroxysmal positional vertigo. The patient's symptoms and laboratory findings are described in detail and, where appropriate, highlight challenges that may arise in interpretation. RESEARCH DESIGN: A case report. RESULTS: The patient presented for evaluation of dizziness, asymmetric hearing loss, and autophony. Comprehensive audiometric evaluation shows asymmetric sensorineural hearing loss and an air-bone gap at 250 Hz in the right ear. Vestibular evaluation shows right caloric asymmetry along with abnormal cervical vestibular- and ocular vestibular-evoked myogenic potentials, with the left ear showing results consistent with the third-window pathology. CONCLUSIONS: Comprehensive assessment of symptoms and critical thinking while performing testing are necessary when examining multiple concurrent otologic etiologies in a patient. Knowledge of anticipated test results and physiology may help the audiologist to synthesize results and make appropriate clinical recommendations as part of the multidisciplinary team.