Single-cell transcriptomic atlas reveals increased regeneration in diseased human inner ear balance organs.

Mammalian inner ear hair cell loss leads to permanent hearing and balance dysfunction. In contrast to the cochlea, vestibular hair cells of the murine utricle have some regenerative capacity. Whether human utricular hair cells regenerate in vivo remains unknown. Here we procured live, mature utricles from organ donors and vestibular schwannoma patients, and present a validated single-cell transcriptomic atlas at unprecedented resolution. We describe markers of 13 sensory and non-sensory cell types, with partial overlap and correlation between transcriptomes of human and mouse hair cells and supporting cells. We further uncover transcriptomes unique to hair cell precursors, which are unexpectedly 14-fold more abundant in vestibular schwannoma utricles, demonstrating the existence of ongoing regeneration in humans. Lastly, supporting cell-to-hair cell trajectory analysis revealed 5 distinct patterns of dynamic gene expression and associated pathways, including Wnt and IGF-1 signaling. Our dataset constitutes a foundational resource, accessible via a web-based interface, serving to advance knowledge of the normal and diseased human inner ear.

The Safety and Efficacy of Hydroxyapatite Repair of Cholesteatoma Skull Base Defects.

Objective: To investigate the possibility of hydroxyapatite as a safe and effective alternative to currently used bioavailable materials for repair of tegmen defects and labyrinthine fistulas in the setting of cholesteatoma. Study Design: Retrospective study. Setting: Tertiary-level-care hospital. Methods: Electronic medical records of patients 18+ years undergoing cholesteatoma-removal surgery between 2013 and 2022 were reviewed. Results: Twenty-two patients diagnosed with cholesteatoma who underwent repair of either a tegmen defect or labyrinthine fistula using hydroxyapatite were evaluated. There were 17 canal wall up (CWU) and 5 canal wall down (CWD) surgeries. The cholesteatoma recidivism rate was 18.2% (n = 4) and the recurrence rate was 4.5% (n = 1). To ensure that these rates were similar to cholesteatoma-removal surgeries in which hydroxyapatite was not used, a 22 age, gender, and operative technique-matched cohort was evaluated. For patients with CWU surgeries, the rate of recurrence and recidivism were identical between both cohorts (0% and 23.5%, respectively; P = 1 for both). While in CWD surgeries, there was a nonstatistically significant difference in the recurrence as there was only 1 patient with recurrence in the hydroxyapatite group (P = 1). Three (13.5%) patients in the hydroxyapatite group had a local infection and 1 (4.5%) had a subacute mastoid infection. All patients with semicircular canal fistulas had consistent bone lines on postoperative audiograms, with no worsening sensorineural hearing loss. Conclusion: In our cohort, hydroxyapatite was safe and successful in repairing skull base defects and inner-ear fistulas in the setting of cholesteatoma with a low rate of postoperative infection and no evidence of a higher rate of cholesteatoma recurrence. Further studies are needed to assess population generalizability.

Hydroxyapatite Use in Repair of Lateral Skull Base CSF Leaks Via Transmastoid Approach: When Does It Work?

OBJECTIVE: The objective of this study is to evaluate the efficacy and outcomes of using a transmastoid approach with hydroxyapatite cement to repair lateral skull base cerebrospinal fluid (CSF) leaks. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary-level care hospital. PATIENTS: Surgical patients 18 years or older between 2013 and 2022 with spontaneous CSF leak. INTERVENTIONS: Trans-mastoid approach for skull base repair using hydroxyapatite cement. MAIN OUTCOME MEASURES: Failure rate of repair; location and size of defect, patient demographic factors. RESULTS: Of the 60 total defects (55 patients, 5 bilateral repairs) that underwent CSF leak repair using hydroxyapatite cement, the success rate was 91.66% (55 successful repairs). The average defect size in unsuccessful repairs was 1.15 cm compared with 0.71 cm for successful repairs. In addition, 80% (4/5) of the failed repairs were in the tegmen tympani region. Higher failure rate was noted in women (3/5) and in former smokers (4/5). Average time to recurrent symptoms was 1.75 years in the failed repair cohort. Of the patients with failed repairs, 4/5 were prescribed acetazolamide before their second procedure with successful second repair. In addition, five patients experienced postoperative headaches, three (5.4%) of whom required placement of VP shunts to relieve increased intracranial pressure. Two patients (3.6%) had complications of either infection or hearing loss. CONCLUSIONS: Transmastoid approach utilizing hydroxyapatite is a successful approach for CSF leak repair, with a low complication and failure rate. Women, prior smoking history, and larger defects in the tegmen tympani region may need alternative materials or approach for repair. Long follow-up is warranted as recurrence of symptoms might be delayed. In cases of benign intracranial hypertension, adjuvant treatment with either acetazolamide or VP shunt placement may prevent failures.

Progression of Hearing Loss in Observed Non-Growing Vestibular Schwannoma.

OBJECTIVE: To assess hearing outcomes in observed vestibular schwannoma (VS) with focus on non-growing tumors. STUDY DESIGN: Retrospective review. SETTING: Two tertiary neurotology centers. PATIENTS AND INTERVENTIONS: Patients with sporadic VS undergoing at least 3 years' observation. MAIN OUTCOME MEASURES: Changes in pure tone averages (PTA) and word recognition scores (WRS) normalized to the contralateral ear. RESULTS: During the study period, 39 of 105 included patients (37.1%) had tumor growth. Patients with tumor growth had a mean normalized increase in PTA of 8.0 dB HL ( p = 0.008) corresponding to a normalized average worsening of their PTA of 1.8 dB per year. Patients with non-growing tumors less than 5 mm in maximal dimension did not have significant ongoing normalized hearing loss ( p > 0.05). Patients with non-growing tumors more than or equal to 5 mm had a mean normalized increase in PTA of 7.4 dB HL ( p = 0.001) corresponding to an average of 2.0 dB HL per year, which was similar to the loss observed in growing tumors regardless of size ( p > 0.05). Normalized decline in PTA of at least 5 dB HL was seen in 72% of patients with growing tumors, 53% of patients with nongrowing tumors more than or equal to 5 mm, and 38% of patients with non-growing tumors less than 5 mm. CONCLUSIONS: A long-term analysis of hearing outcomes in observed vestibular schwannoma is presented. With observation, VS more than or equal to 5 mm is associated with continued hearing loss even without tumor growth, while non-growing tumors less than 5 mm are not associated with continuing hearing loss. These data inform expectations for observed VS for providers and patients.

The Clinical Implications of Spontaneous Hemorrhage in Vestibular Schwannomas.

Background Spontaneous hemorrhage into vestibular schwannomas (VSs) is rare and can render more rapid symptom onset and a seemingly poorer prognosis for an otherwise benign pathology. We describe our series of hemorrhagic VS (HVSs) and systematically reviewed the literature to better understand relevant clinical factors and outcomes. Methods Retrospective case review series and systematic review of the literature using PRISMA guidelines. Results Fifty-three patients with HVS met inclusion criteria. Compared with historical data for all VS, patients with HVS had relatively higher rates of perioperative mortality, significant preoperative facial weakness, and harbored relatively larger tumors. Regardless of the extent of resection (EOR), surgery for HVS resulted in significant improvement of facial weakness ( p = 0.041), facial numbness ( p < 0.001), vertigo ( p < 0.001), and headache ( p < 0.001). Patients with facial weakness tended to have larger tumors ( p = 0.058) on average and demonstrated significant improvement after surgery, irrespective of EOR ( p < 0.01). The use of blood-thinning medications did not affect patient health outcome. Histopathology of HVS samples showed an increased number of dilated/ectatic thin-walled vascular channels, reflective of potentially increased vascular permeability and hypervascularity. Conclusion HVS may be an aggressive subgroup of VS, associated with a surprisingly high mortality rate. When features of HVS are identified on imaging, these patients should be treated expeditiously, especially given that facial nerve dysfunction, which is identified in more than half of patients with HVS, appears to be reversible. Overall, this study has significant implications in the management of VS, raising awareness of a small, but highly morbid subgroup.

Surgical Outcomes After Conservative Resection of Vestibular Schwannoma in the Elderly.

OBJECTIVE: To assess outcomes after surgery for vestibular schwannoma in patients over 70 years of age. STUDY DESIGN: Retrospective chart review. SETTING: Two tertiary otology and neurotology centers. PATIENTS AND INTERVENTIONS: Patients undergoing primary surgery for vestibular schwannoma between 2007 and 2018. MAIN OUTCOME MEASURES: Postoperative complications and surgical outcomes. RESULTS: A total of 452 patients met inclusion criteria, 31 of whom (6.9%) were over 70 years of age. Age ranged from 18 to 90 years with a mean of 53 years. Elderly patients were more likely to have pre-existing hypertension (58.1% versus 34.0%, p = 0.007) and diabetes mellitus (19.4% versus 7.4%, p = 0.02). Elderly patients were less likely to undergo gross total resections of their tumors (35.5% versus 60.6%, p = 0.05) although they were not statistically significantly more likely to undergo subtotal (<95%) resections (25.8% versus 14.7%, p > 0.05). Elderly patients were also less likely to undergo second stage procedures (0% versus 9.5%, p = 0.04). There were no significant differences between elderly and non-elderly patients in the rates of any complications, ultimate facial nerve function, or duration of surgery. No patients over 70 years of age expired within 1 year of surgery. CONCLUSIONS: Conservative surgery for vestibular schwannoma in appropriately selected elderly patients is appropriate and safe, given adequate consideration to risk-benefit analysis and goals of care.

Association Between Body Mass Index and Complications in Acoustic Neuroma Surgery.

OBJECTIVES: Elevated body mass index (BMI) is a risk factor for surgical complications, but data in acoustic neuroma surgery are conflicting and limited to small single-institution studies. This work evaluates associations between BMI and complications in surgery for acoustic neuroma (AN). STUDY DESIGN: Retrospective review. SETTING: Two tertiary otology referral institutions. SUBJECTS AND METHODS: Patients undergoing surgery for AN. Univariate and multivariate analysis of association between BMI and complications was performed using two-tailed t tests and binary logistic regression. RESULTS: BMI ranged from 18.0 kg/m2 to 63.9 kg/m2 with mean of 29.2 kg/m2 among 362 included patients. High BMI was associated with increased risk of cerebrospinal fluid (CSF) leak (p = 0.003) and need for revision surgery within 6 months (p = 0.03). CSF leak occurred in 11.6% of obese patients (BMI ≥ 30.0) and 5.1% of patients with BMI < 30.0. There was no association between BMI and post-operative intracranial hemorrhage, wound infection, or incomplete resection (p > 0.05). Multivariate analysis revealed BMI was associated with CSF leak (odds ratio 1.11 per BMI point, p = 0.002) and need for revision surgery (odds ratio 1.07 per BMI point, p = 0.02) independent of age, gender, tumor size, or surgical approach. CONCLUSIONS: The largest series to date investigating BMI in acoustic surgery is presented. Elevated BMI is strongly associated with CSF leak and need for revision surgery but not with other complications. Obese or overweight patients undergoing acoustic neuroma resection should be counseled of their increased risk of CSF leak.

Evolving Role of Non-Total Resection in Management of Acoustic Neuroma in the Gamma Knife Era.

OBJECTIVE: To examine patients with residual tumor after vestibular schwannoma (VS) resection with focus on need for further therapy, including stereotactic radiosurgery (SRS) and revision surgery. STUDY DESIGN AND SETTING: Retrospective review at two tertiary otology referral centers. PATIENTS AND INTERVENTION: Patients undergoing primary surgery for VS from 2007 to 2017. MAIN OUTCOME MEASURE: Degree of resection and need for further treatment. RESULTS: Of 289 patients undergoing surgery, 38 (13.1%) underwent subtotal resections (<95% of tumor resected) and 77 (26.6%) underwent near-total resections (≥95% but <100%). Patients with any residual tumor had larger tumors preoperatively (mean estimated volume 6.3 cm versus 2.1 cm, p < 0.0005) but were otherwise clinically and demographically similar to the population as a whole. Further treatment (surgery or SRS) was needed in 4.6, 14.3, and 50.0% of patients after gross total, near-total, and subtotal resections, respectively (p < 0.0005). Patients undergoing additional therapy had larger residual tumors (median post- to preoperative estimated volume ratio 0.09 versus 0.01, p < 0.0005). Patients undergoing subtotal and near-total resections had poorer facial function at ultimate follow up than those undergoing gross total resections (p = 0.001), likely due to larger tumors and more difficult resections. Literature review revealed higher rates of gross total resection as well as facial palsy in the pre-SRS era. CONCLUSION: Residual tumor following VS resection is more common today than in the pre-SRS era. Availability of SRS may encourage leaving residual tumor intraoperatively to preserve neural structures. Current surgical strategies decrease surgical morbidity but necessitate further treatment in over 10% of cases.

Hydroxyapatite cement cranioplasty for translabyrinthine surgery: A single institution experience.

OBJECTIVE: To assess complication rates in hydroxyapatite cement (HAC) cranioplasty for translabyrinthine acoustic surgery compared with historical controls. METHODS: Retrospective chart review of patients undergoing de novo translabyrinthine surgery with HAC cranioplasty without concurrent adipose tissue graft or additional material between 2010 and 2017 at a tertiary otology center. RESULTS: Fifty-two patients underwent HAC cranioplasty during the study period. The average length of follow-up was 30.5 months. HAC cranioplasty was associated with acceptable rates of cerebrospinal fluid leak (3.8%), wound or intracranial infection (5.8%), need for unplanned revision surgery (9.6%), and all complications (15.3%). All complications occurred within 5 months of surgery. No delayed wound infections or implant extrusions occurred. CONCLUSION: HAC cranioplasty has an acceptable complication profile for translabyrinthine surgery and is a viable alternative to abdominal fat grafting without associated donor site morbidity. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:206-211, 2020.

Natural History of Facial Weakness Following Surgery of the Cerebellopontine Angle: A Tertiary Care Cohort.

OBJECTIVE: Facial function is a key outcome in surgery of the cerebellopontine angle (CPA). This study describes the natural history of facial weakness after CPA surgery. STUDY DESIGN AND SETTING: Retrospective study of two tertiary centers. PATIENTS AND INTERVENTION: Patients undergoing surgery for CPA tumors from 2003 to 2017 with preoperatively normal facial function and without subsequent surgical or adjuvant therapy. MAIN OUTCOME MEASURE: Serial facial nerve examinations using the House-Brackmann (HB) scale. RESULTS: Of 301 patients examined, 149 (49.5%) had facial weakness postoperatively and 74.5% of these occurred within 24 hours. Of patients with HB-1 function within 24 hours after surgery, 95.3% had HB-1 function and 100% had HB-3 or better function at last follow-up. Of patients with HB-4 or worse function within 24 hours after surgery, 22.6% ultimately achieved HB-1 function and 83.9% ultimately achieved HB-3 or better function. By 180 days postoperatively, 83.7% of patients' facial function had stabilized. At last follow-up, 3.0% of patients had facial function poorer than HB-3, which was associated with subtotal resections (12.9% vs. 4.4% for near total and 0.6% for gross total resections, p = 0.002), aspirin use (10.6% vs. 1.6%, p = 0.001), larger tumors (p < 0.0005), longer surgery (p < 0.002), and immediate versus delayed postoperative facial weakness (p = 0.002). CONCLUSIONS: Facial function over time after CPA surgery is presented. While facial function immediately after surgery correlates with future function, delayed weakness or improvement is common. These data inform patient counseling both preceding and following surgery.

Reliability of clinical diagnosis of masses of the cerebellopontine angle: A retrospective multi-institutional study.

OBJECTIVES: To assess the accuracy of pre-operative diagnosis of masses of the cerebellopontine angle (CPA) when compared to surgical pathology. DESIGN: Retrospective chart review. PARTICIPANTS: Patients who underwent surgery for CPA masses at two tertiary care institutions from 2007 to 2017. MAIN OUTCOME MEASURES: Percent concordance between pre-operative and surgical pathologic diagnosis; sensitivity, specificity, positive predictive value, and negative predictive value for predicted diagnoses. RESULTS: Concordance between pre-operative diagnosis and surgical pathology was 93.2% in 411 sampled patients. Concordance was 57.9% for masses other than vestibular schwannoma. Prediction of vestibular schwannoma and meningioma had high positive (0.95 and 0.97, respectively) and negative (0.76 and 0.99, respectively) predictive values. Prediction of facial neuroma had sensitivity of 0.13 and positive predictive value of 0.25. Headache (p = 0.001) and facial weakness (p = 0.003) were significantly associated with different pathologic profiles. Hearing loss was associated with differences in diagnostic prediction (p = 0.02) but not with differences in surgical pathology (p > 0.05). CONCLUSIONS: Comparison between pre-operative predicted diagnosis and surgical pathology for cerebellopontine angle masses is presented. Vestibular schwannoma and meningioma were effectively identified while rarer CPA masses including facial neuroma were rarely identified correctly. Clinicians caring for patients with CPA masses should be mindful of diagnostic uncertainty which may lead to changes in treatment plan or prognosis.

Semicircular canal dehiscence among idiopathic intracranial hypertension patients.

OBJECTIVES/HYPOTHESIS: The cause of superior semicircular canal dehiscence (SSCD) is unknown. Because of a demonstrated association with tegmental defects and obesity, some have suggested idiopathic intracranial hypertension (IIH) could contribute by eroding the bone over the canal and resulting in SSCD. However, an association between IIH and SSCD has not previously been evaluated. Our objective was to evaluate an association between IIH and SSCD. STUDY DESIGN: Retrospective cohort. METHODS: A retrospective study was performed of opening pressures for consecutive patients presenting at a lumbar puncture clinic between August 2012 and October 2015. Imaging for patients who also had thin-sectioned computed tomography (CT) imaging was reviewed for the presence of radiographic SSCD. Association between IIH and SSCD was evaluated using the Student t test and multivariate logistic regression. RESULTS: One hundred twenty-one patients had both a lumbar puncture performed and thin-sectioned CT imaging available, of which 24 patients (19.8%) met the criteria for IIH with an opening pressure >25 cm H2 O. The remaining 97 patients (80.2%) did not have elevated opening pressures and served as the control cohort. None of the 24 patients with IIH had radiographic SSCD, whereas eight of the 97 patients (8.2%) without IIH had radiographic SSCD. The average opening pressure in patients without radiographic SSCD was 20.2 cm H2 O compared to 19.3 cm H2 O in patients with radiographic SSCD (P = .521). In multivariate logistic regression controlling for age, body mass index, gender, and comorbidities (hypertension, diabetes, hyperlipidemia), opening pressure was not a significant predictor of radiographic SSCD. CONCLUSIONS: The results of this retrospective pilot study do not suggest an association between IIH and SSCD. LEVEL OF EVIDENCE: 3b. Laryngoscope, 128:1196-1199, 2018.

Posterior semicircular canal dehiscence secondary to jugular enlargement.

Semicircular canal dehiscence is an important entity often presenting with symptoms of noise or pressure induced vertigo, autophony, aural fullness and conductive hearing loss. Due to its varied presentation and mimic of other otologic conditions its consideration is of increased importance. Within we report a case of right sided posterior semicircular canal dehiscence secondary to an enlarged and high jugular bulb. The results of this observation indicate a need for patients with auditory symptoms and high riding jugular bulbs to be evaluated for possible posterior semicircular canal dehiscence.

The correlation between obesity, obstructive sleep apnea, and superior semicircular canal dehiscence: a new explanation for an increasingly common problem.

OBJECTIVE: To investigate rates of obesity and obstructive sleep apnea (OSA) in patients with a diagnosis of superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective cohort study. SETTING: Two tertiary referral centers. PATIENTS: Thirty-one patients with SSCD were identified from patient records at Yale between January 1, 2003 and August 1, 2013 and from the University of Cincinnati between November 1, 2008 and November 1, 2013. The control cohort consisted of 100 consecutive adult patients who obtained high-resolution CT imaging of their temporal bones at Yale University for any reason. INTERVENTIONS: CT images were reviewed by two authors in double blind fashion and patient data was analyzed statistically. MAIN OUTCOME MEASURES: Rates of OSA, body mass index (BMI), and presence of tegmental defects in patients with SSCD were compared to the control cohort. RESULTS: The 31 patients with SSCD demonstrated higher BMIs [SSCD avg. 31.62, standard deviation (SD) 8.6 vs. no SSCD 28.01, SD 6.3, P = 0.036], rates of OSA (SSCD 29.03% vs. no SSCD 7.00, P = 0.001), and rates of tegmental defects (SSCD avg. 64.5% vs. no SSCD 16%, P = 1.24 × 10(-7)), in comparison to the control cohort. SSCD was found in 6 of 100 consecutively reviewed adult CT scans and in 0 of 41 scans obtained in those under 17 years of age. CONCLUSIONS: Patients with SSCD demonstrated higher BMIs, higher rates of OSA, and were more likely to have accompanying tegmental defects. These results may support a possible causality between increased intracranial pressure and the formation of superior semicircular canal dehiscence.

Differences in otosclerotic and normal human stapedial osteoblast properties are normalized by alendronate in vitro.

OBJECTIVE: Identify and compare phenotypic properties of osteoblasts from patients with otosclerosis (OSO), normal bones (HOB), and normal stapes (NSO) to determine a possible cause for OSO hypermineralization and assess any effects of the bisphosphonate, alendronate. STUDY DESIGN: OSO (n = 11), NSO (n = 4), and HOB (n = 13) cultures were assayed for proliferation, adhesion, mineralization, and gene expression with and without 10(-10)M-10(-8)M alendronate. SETTING: Academic hospital. METHODS: Cultures were matched for age, sex, and passage number. Cell attachment and proliferation + alendronate were determined by Coulter counting cells and assaying tritiated thymidine uptake, respectively. At 7, 14, and 21 days of culture + alendronate, calcium content and gene expression by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) were determined. RESULTS: OSO had significantly more cells adhere but less proliferation than NSO or HOB. Calcification was significantly increased in OSO compared to HOB and NSO. NSO and HOB had similar cell adhesion and proliferation rates. A dose-dependent effect of alendronate on OSO adhesion, proliferation, and mineralization was found, resulting in levels equal to NSO and HOB. All cultures expressed osteoblast-specific genes such as RUNX2, alkaline phosphatase, type I collagen, and osteocalcin. However, osteopontin was dramatically reduced, 9.4-fold at 14 days, in OSO compared to NSO. Receptor activator of nuclear factor κB ligand/osteoprotegerin (RANKL/OPG), important in bone resorption, was elevated in OSO with decreased levels of OPG levels. Alendronate had little effect on gene expression in HOB but in OSO increased osteopontin levels and decreased RANKL/OPG. CONCLUSIONS: OSO cultures displayed properties of hypermineralization due to decreased osteopontin (OPN) and also had increased RANKL/OPG, which were normalized by alendronate.

Cochlear implantation after resection of an intralabyrinthine schwannoma.

Intralabyrinthine schwannomas are rare tumors of the distal ends of the cochlear and vestibular nerve. Their presence can be debilitating secondary to symptoms of hearing loss, vertigo, tinnitus, and imbalance. Currently, treatment to restore hearing in those who have become profoundly deaf is not attempted. Additionally, resection in patients with functioning hearing is rare, as the surgery assures deafness. We report the first case demonstrating the feasibility of resection of an intralabyrinthine schwannoma with immediate cochlear implantation. This technique addresses the patients hearing status by taking into account advancing technology, allowing for an improved quality of life.

Large jugular bulb abnormalities involving the middle ear.

OBJECTIVE: Jugular bulb abnormalities (JBA), such as jugular bulb diverticula (JBD) or large jugular bulbs, rarely present in the middle ear. We review a large series of temporal bone histopathologic specimens to determine their prevalence and present a series of cases of JB abnormalities involving the middle ear (JBME) that shed light on the probable mechanism for their development. PATIENTS: 1,579 unique temporal bone specimens and individuals with radiographically-diagnosed JBME. INTERVENTION: Histopathologic and clinical review of temporal bone specimens and patient presentations, radiographic findings, treatments and outcomes. MAIN OUTCOME MEASURE: Shared characteristics of JBME. RESULTS: There were 17 cases of JBME in 1,579 temporal bone (1.1%), of which, 15 involved the inferior mesotympanum below the level of the round window membrane (RWM), whereas 2 encroached upon the RWM or ossicles. In addition, 4 clinical cases of large JBME extending above RWM were encountered; these occurred in both sexes with ages spanning from young to old (7-66 yr). They presented with conductive hearing loss (n = 3), ear canal mass (n = 1), and intraoperative bleeding (n = 1). Radiologically, they had multiple diverticula of the JB on the side with JBME, with 1 patient demonstrating growth on serial imaging studies. All patients who underwent additional imaging had marked hypoplastic contralateral transverse sinus. CONCLUSION: JBME abnormalities are rare, present across age groups, and may demonstrate serial growth over time. They are usually associated with multiple other diverticula within the same JB. Our clinical series suggests that JBME's development and uniquely aggressive behavior results from contralateral transverse sinus outflow obstruction.

Use of intravenous immunoglobulin to treat chronic bilateral otomastoiditis in the setting of rituximab induced hypogammaglobulinemia.

The temporal bone may be affected by a variety of systemic pathology because the disease nature, location, and extent determine the symptoms. Middle ear and mastoid infections may be the initial clinical manifestation of autoimmune and acquired immunodeficiency disorders. Rituximab, an anti-CD20 chimeric antibody, has become increasingly popular as a therapeutic agent for patients with a wide range of autoimmune disorders refractory to standard treatments. Normal levels of immunoglobulin levels are usually maintained during and after rituximab therapy, and clinical trials to date have shown no statistically significant increase of serious infections among patients with autoimmune diseases being treated with rituximab (Cohen SB, Emery P, Greenwald MW, Dougados M, Furie RA, Genovese MC, et al, for the REFLEX Trial Group. Rituximab for rheumatoid arthritis refractory to anti-tumor necrosis factor therapy: results of a multicenter, randomized, double-blind, placebo-controlled, phase III trial evaluating primary efficacy and safety at 24 weeks. Arthritis Rheum. 2006;54:2793-2806. Edwards JC, Szczepanski L, Szechinski J, Filipowicz-Sosnowska A, Emery P, Close DR, et al. Efficacy of B-cell-targeted therapy with rituximab in patients with rheumatoid arthritis. N Engl J Med. 2004;350:2572-2581). However, there have been several reports of opportunistic infections associated with rituximab (Kelesidis T, Daikos G, et al. Does rituximab increase the incidence of infectious complications? A narrative review. Int J Infect Dis 2011;15:e2-e16. Teichmann LL, Woenckhaus M, Vogel C, et al. Fatal Pneumocystis pneumonia following rituximab administration for rheumatoid arthritis. Rheumatology 2008;47:1256-1257), as well as cases of it accelerating the presentation of hypogammaglobulinemia (Diwakar L, Gorrie S, et al. Does rituximab aggravate pre-existing hypogammaglobulinaemia? J Clin Pathol 2010;63:275-277). Humoral immune defects can cause persistent acute and serous otitis media, with the development of chronic suppurative otitis media refractory to medical and surgical therapy (Sasaki CT, Askenase P, Dwyer J, et al. Chronic ear infection in the immunodeficient patient. Arch Otolaryngol 1981;107:82). Here, we describe the first presentation, diagnostic workup, and treatment with intravenous immunoglobulin of chronic bilateral otomastoiditis in the setting of rituximab-induced hypogammaglobulinemia.

Relationship between obesity, obstructive sleep apnea, and spontaneous cerebrospinal fluid otorrhea.

OBJECTIVES/HYPOTHESIS: To investigate the relationship between obesity, obstructive sleep apnea (OSA), and spontaneous cerebrospinal fluid (CSF) otorrhea. STUDY DESIGN: Retrospective review of patients presenting with the diagnosis of CSF otorrhea during a 15-year period to a tertiary care medical center. METHODS: Patient records were reviewed and the following data were extracted: age, sex, height, weight, past medical/surgical history, laterality of otorrhea, findings at operation, and method of repair. Otorrhea was considered spontaneous in the absence of trauma, otologic surgery, infection, and neoplasm. RESULTS: Of the 29 adult patients identified, 14 patients presented with CSF otorrhea that was spontaneous in nature. The average body mass index among these patients was 35.2 +/- 8, which was higher than the average in the nonspontaneous group, 28.5 +/- 5 (P = .01). Diagnoses of OSA were also more common in the spontaneous group (n = 4) compared with the nonspontaneous group (n = 0). One case of bilateral spontaneous CSF leaks was recorded. The majority of patients were repaired with hydroxyapatite cement through a transmastoid approach. There were no recurrences. CONCLUSIONS: In this series, patients with spontaneous CSF otorrhea were more likely to be severely and morbidly obese than were patients with nonspontaneous otorrhea. OSA was also more common in these patients. The findings from this study support an association between obesity and spontaneous CSF leaks. Patients presenting with spontaneous CSF otorrhea should therefore be screened for OSA and signs of increased intracranial pressure.

Revision surgery for vertigo.

When confronted with vertigo after an otologic procedure, a surgeon first must identify the functional status of the inner ear by performing auditory and vestibular testing. Using this information in conjunction with knowledge of the primary disease process for which the initial procedure was performed, the surgeon can make a rational selection of the procedure required to eliminate vertigo. This article outlines a systematic approach to the selection of the appropriate revision procedure and discusses the specific advantages and disadvantages of these procedures used to control vertigo.