Predictors of hearing functional outcome following surgery for cerebellopontine angle meningioma.

OBJECTIVE: Cerebellopontine angle (CPA) meningiomas can affect hearing function and require expeditious treatment to prevent permanent hearing loss. The authors sought to determine the factors associated with functional hearing outcome in CPA meningioma patients treated with surgery and/or radiation therapy in the form of either stereotactic radiosurgery or stereotactic radiation therapy. METHODS: Consecutive patients with CPA meningiomas who had presented at our hospital from 2008 to 2018 were identified through retrospective chart review. Hearing function (as defined by pure tone average (PTA) and speech discrimination score (SDS) on Audiogram) was assessed before and after surgery for CPA meningioma. Audiograms with PTA > 50 dB and SDS < 69% were defined as poor hearing functional outcome. Multivariable Cox Proportional Hazards Regression Model was used to assess the associations between pre-operative hearing functional assessment and post-operative hearing functional outcomes. RESULTS: The study cohort included 31 patients (80.6% females, with a mean age of 61.3 ± 15.2 years) with a median clinical follow-up of 5 months (range: 1 week-98 months). The mean pre-operative PTA and SDS were 23.8 ± 11.2 dB and 64.4 ± 22.2% respectively. At the last visit, there was significant hearing recovery, with an improvement of 29.7 ± 18.0 dB (p < 0.001) and 87.6 ± 17.8% (p < 0.001) in PTA and SDS respectively. After adjusting for age, gender, tumor volume, location, and tumor classification, Multivariable Cox Proportional Hazards Regression Model was conducted which revealed that patients undergoing surgery through retro sigmoid approach [Hazards Ratio (HR): 32.1, 95% Confidence Interval (CI): 2.11-491.0, p = 0.01] and gross total resection (GTR) (HR: 2.99, 95% CI: 1.09-9.32, p = 0.05) had significantly higher risk of poor hearing functional outcome compared to petrosal approach and near/subtotal resection. Moreover, patients with poor preoperative hearing had 85% higher chance of poor hearing functional outcome postoperatively (HR: 0.15, 95%CI: 0.03-0.59, p = 0.007). CONCLUSION: Postoperative improvement in hearing is a reasonable expectation following surgery for CPA meningioma. Preoperative hearing, surgical approach and extent of surgical resection are predictive factors of postoperative hearing function outcome and can therefore aid in identification of patients at higher risk of hearing loss.

Meniere's disease: Medical management, rationale for vestibular preservation and suggested protocol in medical failure.

Meniere's disease is a peripheral audiovestibular disorder characterized by vertigo, hearing loss, tinnitus, and aural fullness. Management of these symptoms includes medical and surgical treatment. Many patients with Meniere's disease can be managed using nonablative therapy, such as intratympanic steroids and endolymphatic shunt surgery, prior to ablative techniques such as intratympanic gentamicin. Recognition of concurrent migraine symptoms may aid in medical therapy and also underscore the importance of preserving vestibular function where possible. The goal of this review is to explain the importance of nonablative therapy options and discuss treatment protocols after medical failure.

The effect of increased cost-sharing on low-value service use.

We examine the effect of a value-based insurance design (VBID) program implemented at a large public employer in the state of Oregon. The program substantially increased cost-sharing for several healthcare services likely to be of low value for most patients: diagnostic services (e.g., imaging services) and surgeries (e.g., spinal surgeries for pain). Using a difference-in-differences design coupled with granular, administrative health insurance claims data over the period 2008-2012, we estimate the change in low-value service use among beneficiaries before and after program implementation relative to a comparison group not exposed to the VBID. Our findings suggest that the VBID significantly reduced the use of targeted services, with an implied elasticity of demand of -0.22. We find no evidence that the VBID led to substitution to non-targeted services or increased overall healthcare costs. However, we also observe no evidence that the program led to cost-savings.

Benefits of Cochlear Implantation for Single-Sided Deafness: Data From the House Clinic-University of Southern California-University of California, Los Angeles Clinical Trial.

OBJECTIVES: Cochlear implants (CIs) have been shown to benefit patients with single-sided deafness (SSD) in terms of tinnitus reduction, localization, speech understanding, and quality of life (QoL). While previous studies have shown cochlear implantation may benefit SSD patients, it is unclear which point of comparison is most relevant: baseline performance before implantation versus performance with normal-hearing (NH) ear after implantation. In this study, CI outcomes were assessed in SSD patients before and up to 6 mo postactivation. Benefits of cochlear implantation were assessed relative to binaural performance before implantation or relative to performance with the NH ear alone after implantation. DESIGN: Here, we report data for 10 patients who completed a longitudinal, prospective, Food and Drug Administration-approved study of cochlear implantation for SSD patients. All subjects had severe to profound unilateral hearing loss in one ear and normal hearing in the other ear. All patients were implanted with the MED-EL CONCERTO Flex 28 device. Speech understanding in quiet and in noise, localization, and tinnitus severity (with the CI on or off) were measured before implantation (baseline) and at 1, 3, 6 mo postactivation of the CI processor. Performance was measured with both ears (binaural), the CI ear alone, and the NH ear alone (the CI ear was plugged and muffed). Tinnitus severity, dizziness severity, and QoL were measured using questionnaires administered before implantation and 6 mo postactivation. RESULTS: Significant CI benefits were observed for tinnitus severity, localization, speech understanding, and QoL. The degree and time course of CI benefit depended on the outcome measure and the reference point. Relative to binaural baseline performance, significant and immediate (1 mo postactivation) CI benefits were observed for tinnitus severity and speech performance in noise, but localization did not significantly improve until 6 mo postactivation; questionnaire data showed significant improvement in QoL 6 mo postactivation. Relative to NH-only performance after implantation, significant and immediate benefits were observed for tinnitus severity and localization; binaural speech understanding in noise did not significantly improve during the 6-mo study period, due to variability in NH-only performance. There were no correlations between behavioral and questionnaire data, except between tinnitus visual analog scale scores at 6 mo postactivation and Tinnitus Functional Index scores at 6 mo postactivation. CONCLUSIONS: The present behavioral and subjective data suggest that SSD patients greatly benefit from cochlear implantation. However, to fully understand the degree and time course of CI benefit, the outcome measure and point of comparison should be considered. From a clinical perspective, binaural baseline performance is a relevant point of comparison. The lack of correlation between behavioral and questionnaire data suggest that represent independent measures of CI benefit for SSD patients.

Endoscope-assisted middle fossa craniotomy for resection of inferior vestibular nerve schwannoma extending lateral to transverse crest.

OBJECTIVE The authors describe their results using an endoscope as an adjunct to microsurgical resection of inferior vestibular schwannomas (VSs) with extension into the fundus of the internal auditory canal below the transverse crest. METHODS All patients who had undergone middle fossa craniotomy for VSs performed by the senior author between September 2014 and August 2016 were prospectively enrolled in accordance with IRB policies, and the charts of patients undergoing surgery for inferior vestibular nerve tumors, as determined either on preoperative imaging or as intraoperative findings, were retrospectively reviewed. Age prior to surgery, side of surgery, tumor size, preoperative and postoperative pure-tone average, and speech discrimination scores were recorded. The presence of early and late facial paralysis, nerve of tumor origin, and extent of resection were also recorded. RESULTS Six patients (all women; age range 40-65 years, mean age 57 years) met these criteria during the study period. Five of the 6 patients underwent gross-total resection; 1 patient underwent a near-total resection because of a small amount of tumor that adhered to the facial nerve. Gross-total resection was facilitated using the operative endoscope in 2 patients (33%) who were found to have additional tumor visible only through the endoscope. All patients had a House-Brackmann facial nerve grade of II or better in the immediate postoperative period. Serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery class A or B) was preserved in 3 of the 6 patients. CONCLUSIONS Endoscope-assisted middle fossa craniotomy for resection of inferior vestibular nerve schwannomas with extension beyond the transverse crest is safe, and hearing preservation is feasible.

Growth hormone-secreting pituitary macroadenoma presenting concurrently with non-Hodgkin's lymphoma and responding to doxorubicin treatment: case report and review of the literature.

We present a case report of a patient who presented with nausea and vomiting, as well as acromegalic features. Following testing, a pituitary adenoma and mediastinal non-Hodgkin's lymphoma were diagnosed. Following two cycles of R-CHOP chemotherapy, imaging showed significant decrease in size of the sellar tumor. Following resection of tumor, both frozen and permanent section revealed only necrotic material. Further research into the potential utility of doxorubicin for the treatment of recurrent or refractory pituitary adenomas may be warranted.

Early Communication Development of Children with Auditory Brainstem Implants.

The auditory brainstem implant (ABI) is an auditory sensory device that is surgically placed on the cochlear nucleus of the brainstem for individuals who are deaf but unable to benefit from a cochlear implant (CI) due to anatomical abnormalities of the cochlea and/or eighth nerve, specific disease processes, or temporal bone fractures. In the United States, the Food and Drug Administration has authorized a Phase I clinical trial to determine safety and feasibility of the ABI in up to 10 eligible young children who are deaf and either derived no benefit from the CI or were anatomically unable to receive a CI. In this paper, we describe the study protocol and the children who have enrolled in the study thus far. In addition, we report the scores on speech perception, speech production, and language (spoken and signed) for five children with 1-3 years of assessment post-ABI activation. To date, the results indicate that spoken communication skills are slow to develop and that visual communication remains essential for post-ABI intervention.

Extrusion of a cochlear implant positioner through the tympanic membrane in a pediatric patient: management of a delayed complication.

OBJECTIVE: This study aimed to present a case of an extruded Advanced Bionics positioner, with the goal of educating a new generation of otologic surgeons on management of the delayed complications associated with this cochlear implant model. METHODS: Retrospective case report in a pediatric patient. RESULTS: We observed the extrusion of an Advanced Bionics positioner 10 years after cochlear implantation. CONCLUSION: Awareness of the Advanced Bionics positioner as a component of certain historical models of cochlear implants is important as the delayed complications associated with this model may still be encountered. Management of an extruded positioner should include precautions against infectious complications, repair of the defect, and assessment of the patient's performance with the cochlear implant.

An atlas of gross and histologic lesions and immunohistochemical immunoreactivity during the temporal progression of aerosolized Lassa virus induced hemorrhagic fever in cynomolgus macaques.

Lassa virus (LASV) causes an acute multisystemic hemorrhagic fever in humans known as Lassa fever, which is endemic in several African countries. This manuscript focuses on the progression of disease in cynomolgus macaques challenged with aerosolized LASV and serially sampled for the development and progression of gross and histopathologic lesions. Gross lesions were first noted in tissues on day 6 and persisted throughout day 12. Viremia and histologic lesions were first noted on day 6 commencing with the pulmonary system and hemolymphatic system and progressing at later time points to include all systems. Immunoreactivity to LASV antigen was first observed in the lungs of one macaque on day 3 and appeared localized to macrophages with an increase at later time points to include immunoreactivity in all organ systems. Additionally, this manuscript will serve as a detailed atlas of histopathologic lesions and disease progression for comparison to other animal models of aerosolized Arenaviral disease.

Auditory brainstem implantation after unsuccessful cochlear implantation of children with clinical diagnosis of cochlear nerve deficiency.

OBJECTIVES: We compared the perceptual auditory abilities of 21 children with suspected cochlear nerve deficiency (CND) and a surgically verified absent cochlear nerve (CN) who first underwent cochlear implantation (CI) and subsequently underwent auditory brainstem implantation (ABI). METHODS: In this retrospective cohort study, from 2000 to 2011, 21 children initially underwent CI at an outside institution and failed to progress in their perceptual auditory abilities. Before CI, all of the children had severe to profound sensorineural hearing loss and a diagnosis of CND. Magnetic resonance imaging (MRI) documented an absent CN in 13 children and a small CN in 8 children. We performed explantation of the cochlear implant and simultaneous ABI on the same side. We performed MRI if no previous MRI results were available. All surgical videos were reviewed to determine the presence or absence of the CN. Measures of the patients' perceptual auditory abilities obtained after CI and after ABI were converted to the Category of Auditory Performance (CAP) scale. RESULTS: At surgery, all patients demonstrated an absent CN. After CI, all patients had a CAP score of 2 or less (mean, 0.52 +/- 0.68). After ABI, all patients had a CAP score of 2 or more (mean, 4.33 +/- 1.68); the improvement was statistically significant (p < 0.001). The complication rates were similar for CI and ABI. CONCLUSIONS: In this cohort of patients who had poor performance after CI, ABI achieved significantly improved performance as measured by the CAP and was shown to successfully rehabilitate hearing. Cases of a small CN may in reality represent an absent CN. Although this cohort was selected from patients with failed CI, the results have implications for the selection of device for patients with CND, in that ABI is a potential alternative to CI in select cases. In patients who fail to progress with intensive rehabilitation with CI or who have no progression in evoked auditory brainstem response, ABI must be considered early.

Impact of the collaborative care model on medical spending.

OBJECTIVES: The collaborative care model integrates mental health care into primary care. In 2017, CMS created new billing codes to reimburse collaborative care. We measured the impact of a program supported by these codes on medical spending. STUDY DESIGN: Quasi-experimental. METHODS: We identified a commercially insured and managed Medicare sample of 825 patients who received collaborative care services in 8 primary care practices. We used propensity score matching to match treated patients to potential controls, resulting in 569 patients per group. We performed a difference-in-differences regression analysis to evaluate the impact of collaborative care on total medical spending, including medical, psychiatric, and pharmaceutical claims. RESULTS: Collaborative care patients' mean total medical cost began to fall after a patient's third month in the program and fell below the mean cost of control patients at month 7. Difference-in-differences regressions indicate a nonsignificant savings in total medical cost of $29.35 per member per month for patients in collaborative care compared with matched controls (95% CI, -$226.52 to $167.82). Treated members incurred $34.11 (95% CI, $31.95-$36.27) higher primary care costs that were directly attributed to collaborative care, $19.91 (95% CI, $4.84-$34.98) higher costs for other mental or behavioral health care, and a nonsignificant reduction of $91.34 (95% CI, -$319.32 to $136.63) in inpatient costs. CONCLUSIONS: Modest spending on collaborative care services to address the behavioral health needs of patients did not increase overall health care costs. This is the first economic study of a collaborative care program supported by the new billing codes.

Lassa antiviral LHF-535 protects guinea pigs from lethal challenge.

LHF-535 is a small molecule antiviral currently in development for the treatment of Lassa fever, a zoonotic disease endemic in West Africa that generates significant morbidity and mortality. Current treatment options are inadequate, and there are no approved therapeutics or vaccines for Lassa fever. LHF-535 was evaluated in a lethal guinea pig model of Lassa pathogenesis, using once-daily administration of a fixed dose (50 mg/kg/day) initiating either 1 or 3 days after inoculation with a lethal dose of Lassa virus. LHF-535 reduced viremia and clinical signs and protected all animals from lethality. A subset of surviving animals was rechallenged four months later with a second lethal challenge of Lassa virus and were found to be protected from disease. LHF-535 pharmacokinetics at the protective dose in guinea pigs showed plasma concentrations well within the range observed in clinical trials in healthy volunteers, supporting the continued development of LHF-535 as a Lassa therapeutic.

Bilateral Auditory Brainstem Implants in Patients With Neurofibromatosis 2.

OBJECTIVE: To discuss indications for bilateral auditory brainstem implants (ABIs), compare audiometric outcomes of unilateral vs bilateral ABIs, and determine if patients have improved outcomes with addition of a second-side implant. STUDY DESIGN: Retrospective review of 24 patients with neurofibromatosis 2 (NF2) who underwent sequential placement of ABIs from 1989 to 2019. SETTING: Tertiary referral center. METHODS: Charts were reviewed for indication for second-side surgery, use of implants, and audiometric outcomes. Implants placed in the past 30 years were included in the study. Northwestern University Children's Perception of Speech (NU-CHIPS) and/or City University of New York (CUNY) sentence scores were compared in unilateral and bilateral conditions. RESULTS: Indications for a second-side implant included first-side implants with severe nonauditory symptoms (11), marginal audiometric results (9), outdated technology (2), or deterioration of first side (2). Seven patients are bilateral users and 1 patient discontinued bilateral use after a year due to no significant improvement over unilateral use. One patient with initial bilateral use was lost to follow-up. Thirteen patients are unilateral users due to nonaudiometric side effects or poor audiometric outcomes with the first side. Two patients are complete nonusers. Seventy-five percent had improved audiometric outcomes after the second-side implant, and 20% had stable findings. CONCLUSIONS: Second-side ABIs should be consider in patients with poor performance from a first-side implant. Most patients demonstrate subjective improvement with the second ABI. More research is needed for better objective assessments of improvements.

Role of cerebral digital subtraction angiography in the evaluation of pulse synchronous tinnitus.

OBJECTIVES: The aim of this study was to evaluate the value of digital subtraction angiography (DSA) in the diagnostic evaluation of a highly selected patient population presenting with pulse-synchronous tinnitus (PST). METHODS: We retrospectively reviewed the charts of all patients referred for evaluation of possible vascular etiology of pulsatile tinnitus. Patients were evaluated with regards to presenting signs, comorbidities, non-invasive imaging results, angiographic findings and outcomes. RESULTS: Fifteen patients underwent cerebral DSA. Dural arteriovenous fistula (dAVF) was identified in six patients, and five patients had other significant vascular pathology identified on DSA. Seven patients with 'negative' non-invasive imaging were found to have significant pathology on DSA. CONCLUSIONS: Catheter angiography may have a significant yield in appropriately selected patients presenting with pulse synchronous tinnitus.

MRI evaluation of axillary neurovascular bundle: Implications for minimally invasive proximal humerus fracture fixation.

BACKGROUND: Percutaneous fixation of proximal humeral fractures places the axillary nerve and posterior humeral circumflex artery at risk for injury. Safe operative zones for the axillary nerve are described based on external measurements from anatomic landmarks, but no study to date has incorporated advanced imaging to help guide surgical procedures in the region of the axillary neurovascular bundle (ANVB). We sought to define the location and trajectory of the ANVB in relation to osseous landmarks using magnetic resonance imaging (MRI) measurements. METHODS: Retrospective review of 750 consecutive MRI studies was performed with 55 imaging studies meeting inclusion criteria for patient positioning, image alignment, and quality. Five measurements were performed including the distance from mid-lateral acromion to lateral ANVB, mid-lateral acromion to medial ANVB, greater tuberosity to lateral ANVB, vertical distance between inferior anatomic neck and lateral ANVB, and angle the ANVB crosses the humerus. Height, gender, and age were recorded. Analysis was performed using ANOVA and Pearson correlation tests. RESULTS: The lateral ANVB was below the inferior articular margin of the humeral head by an average of 12.9 ± 3.9 mm and within a 22 mm window. It was an average of 57.4 ± 5.1 mm from the lateral mid-acromion, and 34.7 ± 4.3 mm below the greater tuberosity. The angle formed by the ANVB crossing the humerus averaged 19.5 ± 3.9 degrees upward from medial to lateral. Height and gender directly impacted measurements. CONCLUSIONS: The use of the inferior humeral head articular margin provides a radiographic landmark to aid intraoperative lateral ANVB assessment which may be helpful during percutaneous fracture fixation.

A wider influence: The impact of formal humanitarian otology training on otology-neurotology fellows.

OBJECTIVES: Long-term commitment to humanitarian surgical outreach requires the opportunity, resources, and time to participate, but perhaps more importantly, it requires a preceding successful outreach experience. The Accreditation Council for Graduate Medical Education (ACGME) expects physician trainees to achieve six Core Competencies: patient care, medical knowledge, interpersonal and communication skills, professionalism, practice-based learning and improvement, and systems-based practice. This study investigates the challenges and benefits of a supervised humanitarian experience with a focus on ACGME Core Competencies, future global outreach, and commitment to education. STUDY DESIGN: Physician survey and program curriculum review. METHODS: Participants in a supervised humanitarian educational experience at a single Otology-Neurotology Fellowship Program between the years of 2006 and 2019 were surveyed. Barriers to participation, challenges, benefits, prior and ongoing humanitarian efforts, and education endeavors were discussed. RESULTS: Challenges including time away from fellowship, logistical difficulties with Resident Review Committee recognition of operative case volume, and civil unrest were encountered. Benefits within all six ACGME Core Competencies were achieved. International collaboration with local providers, patients, and families with diverse socioeconomic and cultural backgrounds allowed bidirectional education while striving for safe, innovative solutions in resource-poor environments. CONCLUSIONS: A supervised education experience with a sustainable humanitarian otology program offers Neurotology fellows an early positive experience near the end of their formal training. ACGME Professionalism goals were achieved through building collegial relationships with local otolaryngologists and staff, while fostering a sense of responsibility to assist those in developing nations. Participation correlated with an ongoing commitment to humanitarian efforts and education post-fellowship. LEVEL OF EVIDENCE: 4.

Simultaneous Cochlear and Auditory Brainstem Implantation in Children With Severe Inner Ear Malformations: Initial Surgical and Audiological Results.

OBJECTIVE: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear. INTERVENTION(S): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear. MAIN OUTCOME MEASURES: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed. RESULTS: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome. CONCLUSIONS: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.

Single vertical incision for Baha implant surgery: preliminary results.

OBJECTIVES: A single vertical skin incision with subcutaneous tissue removal is a cosmetic alternative for Baha implant placement. We aimed to determine the preliminary complication rate using a 4-cm vertical skin incision. STUDY DESIGN: Retrospective review. SUBJECTS AND METHODS: Vertical incision Baha implant placements from January 2006 to August 2007 were reviewed. Complications including skin irritation, skin overgrowth, and implant extrusion were tallied. A total of 71 patients underwent surgery, with a mean follow-up time of 7 months. RESULTS: There were five minor complications (three cases of skin irritation, one wound infection requiring oral antibiotics, one postoperative hematoma) and seven major complications (one case of irritation requiring abutment removal, six cases of skin overgrowth or infection requiring flap revision), for a total complication rate of 16.9 percent. CONCLUSIONS: A single vertical incision for Baha implant placement has a complication rate similar to that of traditional dermatome use in this preliminary study. Patients with thick scalps or risk factors for hypertrophic scarring may require longer abutments.

Early Clinical and Economic Outcomes of Prophylactic and Acute Pathologic Fracture Treatment.

INTRODUCTION: Pathologic fractures often contribute to adverse events in metastatic bone disease, and prophylactic fixation offers to mitigate their effects. This study aims to analyze patient selection, complications, and in-hospital costs that are associated with prophylactic fixation compared with traditional acute fixation after completed fracture. MATERIALS AND METHODS: The Nationwide Inpatient Sample database was queried from 2002 to 2014 for patients with major extremity pathologic fractures. Patients were divided by fixation technique (prophylactic or acute) and fracture location (upper or lower extremity). Patient demographics, comorbidities, complications, hospitalization length, and hospital charges were compared between cohorts. Preoperative variables were analyzed for potential confounding, and χ2 tests and Student's t tests were used to compare fixation techniques. RESULTS: Cumulatively, 43,920 patients were identified, with 14,318 and 28,602 undergoing prophylactic and acute fixation, respectively. Lower extremity fractures occurred in 33,582 patients, and 10,333 patients had upper extremity fractures. A higher proportion of prophylactic fixation patients were white ( P = .043), male ( P = .046), age 74 years or younger ( P < .001), and privately insured ( P < .001), with decreased prevalence of obesity ( P = .003) and/or preoperative renal disease ( P = .008). Prophylactic fixation was also associated with decreased peri- and postoperative blood transfusions ( P < .001), anemia ( P < .001), acute renal failure ( P = .010), and in-hospital mortality ( P = .031). Finally, prophylactic fixation had decreased total charges (-$3,405; P = .001), hospitalization length ( P = .004), and extended length of stay (greater than 75th percentile; P = .012). CONCLUSION: Prophylactic fixation of impending pathologic fractures is associated with decreased complications, hospitalization length, and total charges, and should be considered in appropriate patients.

Correction of progressive hearing loss in superior canal dehiscence syndrome.

A 44-year-old woman presented with typical vestibular symptoms of superior semicircular canal dehiscence syndrome (SSCDS). In addition, the patient experienced a rapidly progressive mixed hearing loss in the affected ear prior to surgical intervention that was unresponsive to oral steroid administration. Following middle fossa craniotomy with repair of the dehiscence, the patient's mixed hearing loss resolved to normal levels with no air-bone gap. In this report, we discuss the possible etiology of this rapidly progressive hearing loss and its implications on the differential diagnosis of patients with new onset mixed hearing losses. We also contrast the index case of progressive mixed loss with the more frequent conductive hearing loss seen in SSCDS.