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.

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.

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.

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.

Hydroxyapatite cement in temporal bone surgery: a 10 year experience.

OBJECTIVES: To describe the indications for successful use of hydroxyapatite cement (HAC) in temporal bone surgery. STUDY DESIGN: Retrospective case review. METHODS: One hundred nine temporal bone defects related to surgical approaches to the skull base, infection, neoplasms, or congenital defect in 102 adults and children were corrected using HAC over a 10-year period. The results and complications were obtained through retrospective chart review. RESULTS: Ninety-seven percent of temporal bone defects were successfully repaired using HAC, and the reconstruction remained stable over the course of this study. There was one case of postoperative cerebrospinal fluid leak. Wound infection occurred in three patients, which required reoperation and removal of the biomaterial. CONCLUSIONS: HAC is a biomaterial that should be used as the primary method to reconstruct temporal bone defects. Proper use of this biomaterial results in restoration of the integrity of the temporal bone and elimination of cerebrospinal fluid leak as a postoperative complication.

Efficacy of the 2-staged procedure in the management of cholesteatoma.

OBJECTIVE: To demonstrate the efficacy of intact canal wall procedure coupled with a second-stage exploration for the treatment of cholesteatoma. DESIGN: Retrospective case study of patients with cholesteatomas treated with staged surgical extirpation. A minimum of 6 months' postoperative follow-up time was required for inclusion into the study. SETTING: Tertiary academic referral center. Patients A total of 35 adult and pediatric patients, ranging from 9 to 65 years of age, who underwent 2-stage procedures for removal of cholesteatomas. INTERVENTIONS: Two-stage procedures, separated by 6 months, performed with posterior tympanotomy approaches. MAIN OUTCOME MEASURES: The presence or absence of cholesteatoma on second-stage look and the subsequent surgical treatment for recurrent cholesteatoma. The overall hearing results after the completion of the 2-staged procedure were calculated. RESULTS: Disease was controlled in 26 (74%) of the patients. Residual and/or recurrent cholesteatomas were found in 9 (26%) of the patients during the second-stage operation. Of these patients, 5 (14% of the total group) ultimately required conversion to canal-wall-down procedure. Average hearing gain at the completion of the second-stage procedure was 9 dB. CONCLUSIONS: A planned 2-stage procedure that uses the posterior tympanotomy approach for the control of cholesteatoma is an effective technique. This approach offers significant potential for hearing preservation and restoration.

Acoustic neuroma. Assessment and management.

Acoustic neuroma results from abnormal proliferation of Schwann cells. These tumors originate in the region of Scarpa's ganglion at the junction of peripheral and central myelin of the vestibular nerve located in the internal auditory canal (IAC). The bony confine of the IAC houses the VII and the VIII cranial nerves. The presence of tumor mass compresses these structures. The growing tumor mass may also prolapses into the cerebellopontine angle (CPA). With continued growth, the tumor eventually compresses on the brain stem and cerebellum. Despite the benign nature of these tumors, the clinical course of this disease may be fraught with complications.

Rapid growth of acoustic neuromas after stereotactic radiotherapy in type 2 neurofibromatosis.

We describe a rare complication of stereotactic radiotherapy for large acoustic neuromas in a patient with type 2 neurofibromatosis. We retrospectively reviewed the case of a 14-year-old girl who had been referred to our tertiary care center. Prior to referral, the patient had been evaluated for hoarseness. During the work-up, magnetic resonance imaging (MRI) detected two large bilateral acoustic neuromas and two bilateral jugular foramen tumors. The patient was diagnosed with type 2 neurofibromatosis, and she underwent stereotactic radiotherapy for treatment of the two acoustic neuromas; the jugular foramen tumors were not irradiated. The patient's post-treatment course was complicated by hydrocephalus and symptoms of brainstem compression, which required urgent surgical intervention. Follow-up MRI 7 months following radiotherapy demonstrated a rapid growth of the acoustic neuromas, but no appreciable change in the size of the jugular foramen neuromas. These findings suggest that the radiotherapy might have been the cause of the rapid growth of the acoustic neuromas. To our knowledge, no such report has been published in the literature, and this phenomenon might be unique. Our findings suggest that radiotherapy might not be the optimal first-line treatment for acoustic neuromas in patients with type 2 neurofibromatosis.