Esther X. Vivas

Treatment outcomes in patients treated with CyberKnife radiosurgery for vestibular schwannoma.

Objective To evaluate tumor control, hearing, tinnitus, and balance outcomes of patients treated with CyberKnife (CK) radiosurgery for vestibular schwannoma (VS). Study Design Retrospective series review. Setting Tertiary referral center. Patients All patients treated with CK radiosurgery for vestibular schwannoma by a multidisciplinary radiosurgical team from August 2005 to November 2011. The median age was 59 years, and mean follow-up was 40 months. Seventy-three patients were treated (63 primary radiosurgery and 10 postsurgical). Interventions CK radiosurgery, serial MRI imaging, comprehensive audiometry, Tinnitus Handicap Inventory (THI) scores, and Activities-Specific Balance Confidence Scale (ABC). Main Outcome Measures Tumor control defined as 2 mm linear growth or lower or less than 20% increase in tumor volume (TV), measured in cubic centimeter, after a minimum of 12 months of monitoring, audiogram profiles, THI, and ABC surveys. Results Of those treated with CK as primary modality, 83% had 0- to 2-mm growth (tumor control or stable) and 17% grew greater than 2 mm. Of the tumors that were stable, 29% shrank 2 mm or greater. Volumetric analysis found that 74% of tumors had less than 20% TV growth, whereas 26% exhibited 20% or greater increase in TV. Of those deemed stable, 65% shrank 20% or greater TV; 95% of patients did not need additional surgical intervention, 3 required salvage surgery and 1 underwent additional radiosurgery. The majority of patients started with Class D hearing, but of those with Class A or B hearing before treatment, 53.5% maintained serviceable hearing at 3 years of follow-up. The pretreatment and posttreatment median THI Grades were both 1. The pretreatment and posttreatment ABC scores were unchanged at 81%. Conclusion The LINAC-based CK (18 Gy over 3 fractions at 80% isodose line) provides tumor control rates comparable to other forms of radiosurgery. Analysis for tumor growth was positive for 17% using maximum linear diameters and 26% with a volumetric workstation. This discrepancy is consistent with previous reports where volumetric models were found to be more sensitive in establishing growth. Serviceable hearing was comparable to previous SRS and SRT reports with an overall hearing preservation of 53.5%. This number was 77% in those with pre-Class A hearing. SRS did not affect pretreatment tinnitus or vestibular function.

ICP, BMI, surgical repair, and CSF diversion in patients presenting with spontaneous CSF otorrhea.

Objective To assess intracranial pressure (ICP), body mass index (BMI), surgical repair, and cerebrospinal fluid (CSF) diversion in patients presenting with spontaneous CSF otorrhea. Study Design Retrospective series review. Setting Tertiary referral center. Patients Thirty-two patients were treated surgically from 2004 to 2013 for spontaneous CSF otorrhea by the principal investigators. Patients with a history of chronic ear disease and cholesteatoma, previous mastoid surgery, head trauma, or iatrogenic injury were excluded. Average age was 56 years. Twenty-two patients (69%) were female. Intervention(s) Middle fossa repair, transmastoid repair, lumbar puncture, V-P shunt, L-P shunt, and magnetic resonance imaging. Main Outcome Measure(s) Patients underwent middle fossa or transmastoid repair of tegmen defects. Intracranial pressures were determined with lumbar puncture at time of surgical repair or shortly after surgery. CSF diversion procedures were performed in patients who were found to have elevated ICP, which was not controlled medically, presented with recurrent leak or had ICP of 25 cm or greater of H2O. Preoperative BMI was calculated. Results Thirty-two patients underwent 37 operations. Average BMI was 35.0 kg/m2 (median, 34.7; range, 18.7–53.2 kg/m2). There were 21 repairs on the left and 16 on the right. The majority underwent a middle fossa craniotomy for repair (27/32). Two patients had bilateral repairs. Three patients (8%) underwent revision surgery, of which, 2 had untreated intracranial hypertension (ICP 24.5 and 24 cm H2O). ICP measurements were available for 29 patients. The mean ICP was 23.4 cm H2O (median, 24; range, 13–36 cm H20). Twenty-two patients (69%) had ICP of 20 cm or greater of H20; of those, 13 had an ICP of 25 cm or greater of H20. Seventeen patients (53%) underwent CSF diversion procedures. Conclusion Our findings of elevated ICP and BMI in patients presenting with spontaneous CSF otorrhea are consistent with previous reports in the literature. The percentage of patients that underwent CSF diversion procedures was high at 53% and represents an aggressive stance in managing elevated ICP in a population that may be at risk for subsequent leaks.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Hearing Preservation Outcomes in Patients With Sporadic Vestibular Schwannomas

Abstract Please see the full‐text version of this guideline (https://www.cns.org/guidelines/guidelines‐management‐patients‐vestibular‐schwannoma/chapter_3) for the target population of each recommendation listed below. STEREOTACTIC RADIOSURGERY Question 1: What is the overall probability of maintaining serviceable hearing following stereotactic radiosurgery utilizing modern dose planning, at 2, 5, and 10 yr following treatment? Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is moderately high probability (>50%‐75%) of hearing preservation at 2 yr, moderately high probability (>50%‐75%) of hearing preservation at 5 yr, and moderately low probability (>25%‐50%) of hearing preservation at 10 yr. Question 2: Among patients with AAO‐HNS (American Academy of Otolaryngology‐Head and Neck Surgery hearing classification) class A or GR (Gardner‐Robertson hearing classification) grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing following stereotactic radiosurgery, utilizing modern dose planning, at 2, 5, and 10 yr following treatment? Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is a high probability (>75%‐100%) of hearing preservation at 2 yr, moderately high probability (>50%‐75%) of hearing preservation at 5 yr, and moderately low probability (>25%‐50%) of hearing preservation at 10 yr. Question 3: What patient‐ and tumor‐related factors influence progression to nonserviceable hearing following stereotactic radiosurgery using ≤13 Gy to the tumor margin? Recommendation: Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled regarding the probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut‐points reported, smaller tumor size, marginal tumor dose ≤12 Gy, and cochlear dose ≤4 Gy. Age and sex are not strong predictors of hearing preservation outcome. MICROSURGERY Question 4 What is the overall probability of maintaining serviceable hearing following microsurgical resection of small to medium‐sized sporadic vestibular schwannomas early after surgery, at 2, 5, and 10 yr following treatment? Recommendation Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled that there is a moderately low probability (>25%‐50%) of hearing preservation immediately following surgery, moderately low probability (>25%‐50%) of hearing preservation at 2 yr, moderately low probability (>25%‐50%) of hearing preservation at 5 yr, and moderately low probability (>25%‐50%) of hearing preservation at 10 yr. Question 5 Among patients with AAO‐HNS class A or GR grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing following microsurgical resection of small to medium‐sized sporadic vestibular schwannomas early after surgery, at 2, 5, and 10 yr following treatment? Recommendation Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled that there is a moderately high probability (>50%‐75%) of hearing preservation immediately following surgery, moderately high probability (>50%‐75%) of hearing preservation at 2 yr, moderately high probability (>50%‐75%) of hearing preservation at 5 yr, and moderately low probability (>25%‐50%) of hearing preservation at 10 yr. Question 6 What patient‐ and tumor‐related factors influence progression to nonserviceable hearing following microsurgical resection of small to medium‐sized sporadic vestibular schwannomas? Recommendation Level 3: Individuals who meet these criteria and are considering microsurgical resection should be counseled regarding the probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut‐points reported, smaller tumor size commonly less than 1 cm, and presence of a distal internal auditory canal cerebrospinal fluid fundal cap. Age and sex are not strong predictors of hearing preservation outcome. CONSERVATIVE OBSERVATION Question 7 What is the overall probability of maintaining serviceable hearing with conservative observation of vestibular schwannomas at 2, 5, and 10 yr following diagnosis? Recommendation Level 3: Individuals who meet these criteria and are considering observation should be counseled that there is a high probability (>75%‐100%) of hearing preservation at 2 yr, moderately high probability (>50%‐75%) of hearing preservation at 5 yr, and moderately low probability (>25%‐50%) of hearing preservation at 10 yr. Question 8 Among patients with AAO‐HNS class A or GR grade I hearing at baseline, what is the overall probability of maintaining serviceable hearing with conservative observation at 2 and 5 yr following diagnosis? Recommendation Level 3: Individuals who meet these criteria and are considering stereotactic radiosurgery should be counseled that there is a high probability (>75%‐100%) of hearing preservation at 2 yr, and moderately high probability (>50%‐75%) of hearing preservation at 5 yr. Insufficient data were available to determine the probability of hearing preservation at 10 yr for this population subset. Question 9 What patient and tumor‐related factors influence progression to nonserviceable hearing during conservative observation? Recommendation Level 3: Individuals who meet these criteria and are considering observation should be counseled regarding probability of successful hearing preservation based on the following prognostic data: the most consistent prognostic features associated with maintenance of serviceable hearing are good preoperative word recognition and/or pure tone thresholds with variable cut‐points reported, as well as nongrowth of the tumor. Tumor size at the time of diagnosis, age, and sex do not predict future development of nonserviceable hearing during observation. The full guideline can be found at: https://www.cns.org/guidelines/guidelines‐manage‐ment‐patients‐vestibular‐schwannoma/chapter_3.

Otopolyposis With Middle Ear Allergic Mucin in a Patient With Allergic Fungal Rhinosinusitis

Objective: The purpose of this study is to report a case of otopolyposis and middle ear allergic mucin in a patient with allergic fungal rhinosinusitis (AFRS) and no history of middle ear disease and introduce these as possible otologic manifestations of the AFRS. Methods: A case of a 31-year-old female with the aforementioned findings is reported. A review of the pertinent literature was performed. Results: We report a case of a 31-year-old female with a history of AFRS but no history of middle ear disease or hearing loss who presented to our institution complaining of aural fullness. Physical exam was significant for middle ear masses of unknown etiology. Surgical exploration revealed the presence of allergic mucin and middle ear polyposis histologically identical to tissue sampled during prior sinonasal surgeries at the same institution. Aspiration of the middle ear space did not resolve the otologic symptoms. Conclusion: Otopolyposis and middle ear allergic mucin are extremely rare but possible otologic manifestations of AFRS. We encourage otolaryngologists to consider this in the clinical differential diagnosis of patients with a history of AFRS with new onset otologic symptoms.

Spontaneous Labyrinthine Hemorrhage: A Case Series

Objectives To describe patient characteristics, audiometric outcomes, and magnetic resonance imaging (MRI) signal patterns in patients with suspected labyrinthine hemorrhage. Study Design Retrospective review. Setting Tertiary medical center. Subjects and Methods Radiology database was queried for terms related to labyrinth hemorrhage or labyrinthitis and then selected for patients in which labyrinthine hemorrhage was suspected in the report. Eleven patients were identified and all treated at our institution. Blinded assessment of temporal bone MRI by 2 experienced neuroradiologists was performed and interrater reliability assessed. Patient demographics, medical comorbidities, and audiometric outcomes are described. Results Of the 11 patients identified, the median patient age was 60 years; 7 were female and 4 male. Ten of 11 patients presented with unilateral sudden sensorineural hearing loss (SNHL), and 8 of 11 had associated vertigo. One patient experienced vertigo without hearing loss. Of those presenting with sudden SNHL, 82% were left with nonserviceable American Academy of Otolaryngology—Head and Neck Surgery class D hearing. Interrater reliability for detecting T1 signal abnormalities was moderate but very good for detecting fluid attenuation inversion recovery (FLAIR) signal abnormalities. Most patients had existing hypertension. Average follow-up was 13.3 months. Conclusion We present the largest cohort of patients with radiographic diagnosis of labyrinthine hemorrhage using T1 and FLAIR signal abnormalities on MRI. Most patients presented with a profound unilateral sudden SNHL that did not recover. Our findings are consistent with prior reports that abnormal FLAIR signal on MRI is a reliable marker for detecting inner ear injury and can potentially be used as a marker for poor prognosis.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Otologic and Audiologic Screening for Patients With Vestibular Schwannomas

QUESTION 1 What is the expected diagnostic yield for vestibular schwannomas when using a magnetic resonance imaging (MRI) to evaluate patients with previously published definitions of asymmetric sensorineural hearing loss? TARGET POPULATION These recommendations apply to adults with an asymmetric sensorineural hearing loss on audiometric testing. RECOMMENDATION Level 3: On the basis of an audiogram, it is recommended that MRI screening on patients with ≥10 decibels (dB) of interaural difference at 2 or more contiguous frequencies or ≥15 dB at 1 frequency be pursued to minimize the incidence of undiagnosed vestibular schwannomas. However, selectively screening patients with ≥15 dB of interaural difference at 3000 Hz alone may minimize the incidence of MRIs performed that do not diagnose a vestibular schwannoma. QUESTION 2 What is the expected diagnostic yield for vestibular schwannomas when using an MRI to evaluate patients with asymmetric tinnitus, as defined as either purely unilateral tinnitus or bilateral tinnitus with subjective asymmetry? TARGET POPULATION These recommendations apply to adults with subjective complaints of asymmetric tinnitus. RECOMMENDATION Level 3: It is recommended that MRI be used to evaluate patients with asymmetric tinnitus. However, this practice is low yielding in terms of vestibular schwannoma diagnosis (<1%). QUESTION 3 What is the expected diagnostic yield for vestibular schwannomas when using an MRI to evaluate patients with a sudden sensorineural hearing loss? TARGET POPULATION These recommendations apply to adults with a verified sudden sensorineural hearing loss on an audiogram. RECOMMENDATION Level 3: It is recommended that MRI be used to evaluate patients with a sudden sensorineural hearing loss. However, this practice is low yielding in terms of vestibular schwannoma diagnosis (<3%). The full guideline can be found at: https://www.cns.org/guidelines/guidelines‐management‐patients‐vestibular‐schwannoma/chapter_2.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Intraoperative Cranial Nerve Monitoring in Vestibular Schwannoma Surgery

FACIAL NERVE MONITORING Question 1 Does intraoperative facial nerve monitoring during vestibular schwannoma surgery lead to better long‐term facial nerve function? Target Population This recommendation applies to adult patients undergoing vestibular schwannoma surgery regardless of tumor characteristics. Recommendation Level 3: It is recommended that intraoperative facial nerve monitoring be routinely utilized during vestibular schwannoma surgery to improve long‐term facial nerve function. Question 2 Can intraoperative facial nerve monitoring be used to accurately predict favorable long‐term facial nerve function after vestibular schwannoma surgery? Target Population This recommendation applies to adult patients undergoing vestibular schwannoma surgery. Recommendation Level 3: Intraoperative facial nerve can be used to accurately predict favorable long‐term facial nerve function after vestibular schwannoma surgery. Specifically, the presence of favorable testing reliably portends a good long‐term facial nerve outcome. However, the absence of favorable testing in the setting of an anatomically intact facial nerve does not reliably predict poor long‐term function and therefore cannot be used to direct decision‐making regarding the need for early reinnervation procedures. Question 3 Does an anatomically intact facial nerve with poor electromyogram (EMG) electrical responses during intraoperative testing reliably predict poor long‐term facial nerve function? Target Population This recommendation applies to adult patients undergoing vestibular schwannoma surgery. Recommendation Level 3: Poor intraoperative EMG electrical response of the facial nerve should not be used as a reliable predictor of poor long‐term facial nerve function. COCHLEAR NERVE MONITORING Question 4 Should intraoperative eighth cranial nerve monitoring be used during vestibular schwannoma surgery? Target Population This recommendation applies to adult patients undergoing vestibular schwannoma surgery with measurable preoperative hearing levels and tumors smaller than 1.5 cm. Recommendation Level 3: Intraoperative eighth cranial nerve monitoring should be used during vestibular schwannoma surgery when hearing preservation is attempted. Question 5 Is direct monitoring of the eighth cranial nerve superior to the use of far‐field auditory brain stem responses? Target Population This recommendation applies to adult patients undergoing vestibular schwannoma surgery with measurable preoperative hearing levels and tumors smaller than 1.5 cm. Recommendation Level 3: There is insufficient evidence to make a definitive recommendation. The full guideline can be found at: https://www.cns.org/guidelines/guidelines‐manage‐ment‐patients‐vestibular‐schwannoma/chapter_4.

Idiopathic, Infectious and Reactive Lesions of the Ear and Temporal Bone

A number of infectious, inflammatory and idiopathic lesions develop within otologic tissues that may share similar clinical and/or microscopic features. This review first provides a working classification for otitis externa, and then otitis media and includes two recently described entities, eosinophilic otitis media and otitis media with ANCA-associated vasculitis. Next, the microscopic findings of a spectrum of otopathologic conditions are described, including post-inflammatory conditions such as tympanosclerosis and aural polyps, an overview of animate aural foreign body as well as iatrogenic aural foreign body reactions. Finally, a review of fungal disease affecting the ear with a brief synopsis of Candida auris, a recently described and virulent organism, is presented.

Internal auditory canal meningocele-perilabyrinthine/translabyrinthine fistula: Case report and imaging.

The case of a 17‐year‐old patient with progressive unilateral sensorineural hearing loss and temporal bone malformations concerning for internal auditory canal meningocele with translabyrinthine/perilabyrinthine cerebrospinal fluid fistula is presented with associated computed tomography and magnetic resonance imaging. As the second reported case of an unruptured internal auditory canal meningocele with translabyrinthine/perilabyrinthine fistula, the case presents several clinically relevant points for otologists, neurotologists, and neuroradiologists. Although rare, it is an additional entity to consider as a cause of unilateral sensorineural hearing loss and may pose a risk for developing meningitis and possible “gushing” of cerebrospinal fluid should surgical intervention be attempted. Laryngoscope, 126:1931–1934, 2016