Better defining best-aided condition: The role of hearing aids on cochlear implantation qualification rates.

OBJECTIVE: Describe the effect of hearing aid type used during cochlear implantation evaluation on qualification rates. METHODS: Consecutive adult patients at an academic cochlear implant program undergoing cochlear implantation evaluation were identified to determine cochlear implantation qualification rate according to history of hearing aid use and type of hearing aid used during evaluation. RESULTS: 609 patients met criteria. 90.1% of patients reported prior use of a hearing aid, and 77.4% reported current use of a hearing aid. Patients were most likely to undergo cochlear implantation evaluation utilizing their own personal hearing aids exclusively (61.6%) followed by loaner hearing aids fitted at the time of the evaluation (28.2%). White patients were more likely to be tested using personal hearing aids (OR = 2.60, 95% CI 1.43 to 4.71). Married patients were more likely to be current hearing aid users (OR 1.62, 95% CI 1.04 to 2.51) and were more likely to be tested using personal hearing aids (OR = 1.68, 95% CI 1.10 to 2.56). Patients with a history of any hearing aid use (OR = 2.50, 95% CI 1.42 to 4.40) and current hearing aid use (OR = 1.62, 95% CI 1.06 to 2.49) were more likely to qualify for cochlear implantation. Patients tested using personal hearing aids were 1.5 times more likely to qualify for cochlear implantation (95% CI 0.99 to 2.27). CONCLUSION: History of hearing amplification and current amplification predict cochlear implant qualification. Hearing aids fitted at the time of cochlear implantation evaluation may result in lower qualification rates.

Endoscopic Removal of an Epithelial Cyst of the Zygoma.

This study elaborates a brief overview of epithelial cysts in the bones of the skull and describes an unusual patient with an epidermoid cyst of the zygoma. This report focuses on an endoscopic preauricular infratemporal fossa approach for resection of a left epidermoid cyst of the zygoma. Preoperative magnetic resonance imaging and computed tomography imaging as well as intraoperative endoscopic images and movie were demonstrated. The epithelial cyst was successfully removed from the patient using a minimally invasive approach utilizing endoscopes. No complications were encountered. The possible causes of this rare presentation and minimal surgical removal utilizing endoscopes were discussed in this study. According to the authors, this is the first reported study of endoscopic removal of an epidermoid cyst from the zygomatic root. The patient's previous surgical history of a tympanoplasty could have contributed to the unusual location of the lesion.

Management of Complications in Vestibular Schwannoma Surgery.

Microsurgical removal of acoustic neuroma has advanced tremendously; however, complications still occur. Facial nerve injury is the most common detrimental complication and should take precedence over gross tumor removal in cases where there is an unfavorable tumor-facial nerve interface. Cerebrospinal fluid leakage can occur even with meticulous closure techniques and is generally treatable with either lumbar-subarachnoid drainage or revision wound closure. Meningitis is a serious complication that requires a high index of suspicion in the postoperative period. Other less common complications include intraoperative and postoperative vascular injuries. Early identification and treatment can prevent devastating outcomes.

Impact of Cochlear Dose on Hearing Preservation Following Stereotactic Radiosurgery in Treatment of Vestibular Schwannomas: A Multi-Center Study.

OBJECTIVE: Stereotactic radiosurgery (SRS) is a well-established treatment for vestibular schwannomas (VS). Hearing loss remains a main morbidity of VS and its treatments, including SRS. The effects of radiation parameters of SRS on hearing remain unknown. The goal of this study is to determine the effect of tumor volume, patient demographics, pretreatment hearing status, cochlear radiation dose, total tumor radiation dose, fractionation, and other radiotherapy parameters on hearing deterioration. METHODS: Multicenter retrospective analysis of 611 patients who underwent SRS for VS from 1990-2020 and had pre- and post-treatment audiograms. RESULTS: Pure tone averages (PTAs) increased and word recognition scores (WRSs) decreased in treated ears at 12-60 months while remaining stable in untreated ears. Higher baseline PTA, higher tumor radiation dose, higher maximum cochlear dose, and usage of single fraction resulted in higher post radiation PTA; WRS was only predicted by baseline WRS and age. Higher baseline PTA, single fraction treatment, higher tumor radiation dose, and higher maximum cochlear dose resulted in a faster deterioration in PTA. Below a maximum cochlear dose of 3 Gy, there were no statistically significant changes in PTA or WRS. CONCLUSIONS: Decline of hearing at one year in VS patients after SRS is directly related to maximum cochlear dose, single versus 3-fraction treatment, total tumor radiation dose, and baseline hearing level. The maximum safe cochlear dose for hearingtbrowd preservation at one year is 3 Gy, and the use of 3 fractions instead of one fraction was better at preserving hearing.

Diagnosing Pulsatile Tinnitus: A Review of 251 Patients.

OBJECTIVE: To analyze the underlying etiologies, presenting characteristics, and diagnostic workup of patients with pulsatile tinnitus (PT). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: All patients who received a diagnostic workup for PT from January 01, 2015 and May 31, 2020. MAIN OUTCOME MEASURE: Diagnostic rate of imaging studies. RESULTS: Among 251 patients with PT, the most common etiologies included neoplasms (16%), arteriopathies (14%), venopathies (8.5%), middle/inner ear pathology (9.0%), or idiopathic (50%). Patients with identifiable etiologies of PT more often had hypertension, obesity, vision changes, ipsilateral asymmetric hearing loss, or an abnormal otologic examination. Only 18.5% of patients without those characteristics had an identifiable etiology of PT. The most commonly ordered diagnostic studies were magnetic resonance imaging with contrast (n = 146), MR angiography (MRA) (n = 105), CT angiography (CTA) (n = 84), computed tomography (CT) without contrast (n = 76), and MR Venogram (MRV) (n = 62). Magnetic resonance imaging with contrast and CT without contrast preferentially identified patients with nonvascular etiologies of PT, while MRA and CTA identified patients with vascular etiologies of PT. MRV did not demonstrate high diagnostic rate for either type of PT. No difference in diagnostic rate was found between MR-based or CT-based imaging. CONCLUSIONS: Patients who lack a history of hypertension, obesity, vision changes, ipsilateral asymmetric hearing loss, or an abnormal otologic examination are less likely to have an identifiable cause for PT. In cases where a specific etiology was identified, MR-based imaging (MRI with contrast and MRA) or CT-based imaging (CT without contrast and CTA) were equally efficacious in identifying that etiology. MR-based imaging is preferred for neoplasms, while CT-based imaging is preferred for semicircular canal dehiscence.

Patient Factors Predicting Success in Lateral Graft Tympanoplasty.

Objective: To compare the effects of preoperative medical comorbidities and operative findings on the success of lateral graft tympanoplasty. Study Design: Retrospective chart review. Setting: Tertiary medical center. Patients: Ninety-six patients undergoing lateral graft tympanoplasty from December 2008 to November 2020 with at least 2 months follow-up were included. Patient demographics, comorbidities including smoking status, intraoperative findings, and healing, and hearing outcomes were recorded. Interventions: Lateral graft tympanoplasty. Main Outcome Measures: The primary outcome was perforation closure. Secondary outcomes were postoperative complications and change in air-bone gap (ABG). Results: Ninety-nine ears (mean age 40.94 ± 18.44 years) were included. Tympanic membrane perforation closure was achieved in 92 (92.9%) ears. Perforation closure was not associated with diabetes (P > 0.99), smoking (P > 0.99), or the presence of cholesteatoma at the time of lateral graft tympanoplasty (P = 0.10). Increased age (odds ratio [OR] = 1.04, P = 0.31) was also not correlated with tympanic membrane closure rate. An absent malleus resulted in a higher rate of lateralization (31.3% versus 2.1%; OR = 18.41, 95% confidence interval [CI] = 3.09-95.95, P = 0.001) but not blunting (12.5% versus 4.8%; OR = 0.24, 95% CI = 0.49-12.93, P = 0.24). The mean ABG improved 6.82 ± 11.33 dB (P < 0.01). History of prior tympanoplasty was associated with smaller ABG improvement following surgery (ß = 4.038, R2 = 0.262, P = 0.04) but not perforation closure (OR = 3.25, 95% CI = 0.63-16.81, P = 0.24). Conclusions: Diabetes, active smoking, and advancing age were not associated with adverse healing in patients undergoing lateral graft tympanoplasty. Lateralization was more common with an absent malleus.

Transcanal Endoscopic Versus Microscopic Tympanoplasty: Is There a Difference in Perforation Closure Rates?

Objective: To compare closure rates of endoscopic and microscopic tympanoplasty (MT) as influenced by perforation size, perforation location, and graft position. Study Design: Retrospective chart review. Setting: Tertiary university medical center. Patients: Adult patients who underwent tympanoplasty by a fellowship-trained neurotologist from January 2010 to December 2019, had at least 2 months of follow-up, and had a tympanic perforation with no cholesteatoma before surgery. Interventions: Transcanal endoscopic tympanoplasty (ET) or MT. Main Outcome Measures: The primary outcome is postoperative closure of the tympanic membrane perforation as assessed using otomicroscopy at the last follow-up appointment. Results: Two-hundred and eleven patients-98 in the transcanal ET group and 113 in the MT group-were identified. Tympanic membrane closure rates were not significantly different between the ET and MT groups (79.6% and 84.1% respectively; P = 0.473), and further multivariable analysis revealed that closure rates for ET relative to MT had an insignificant odds ratio (0.56; P = 0.144). Similar analyses also found no significant difference between the 2 methods in subsets of perforation size (small, large, subtotal/total), perforation location (anterior, posterior, inferior), and graft position (underlay, overlay). Conclusions: ET resulted in similar rates of postoperative closure rates compared with the microscopic technique.

Audiometric Outcomes Comparing Endoscopic Versus Microscopic Ossiculoplasty.

OBJECTIVE: To assess endoscopic and microscopic ossiculoplasty audiometric outcomes. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic center. PATIENTS: Adult patients who underwent ossiculoplasty with either partial ossicular replacement prosthesis (PORP) or total ossicular replacement prosthesis (TORP) from 2010 to 2019 with at least 1 year of audiometric follow-up were included. INTERVENTIONS: Endoscopic or microscopic ossiculoplasty. MAIN OUTCOME MEASURES: Postoperative air-bone gap (ABG) after at least 1 year. RESULTS: A total of 198 patients, 53.5% female, and a median age of 47.5 years, met inclusion criteria. 64.1% of patients were reconstructed with a PORP, and 31.8% were reconstructed using an endoscopic approach. The median audiometric follow-up was 27 months. The median postoperative ABG was 16.9 dB overall, 15.6 dB for PORP reconstruction, and 19.4 dB for TORP reconstruction (PORP versus TORP, p = 0.002). For TORP reconstructions, the median ABG for both endoscopic and microscopic TORP was 19.4 dB ( p = 0.92). For PORP reconstructions, the median ABG for endoscopic PORP was 12.3 dB compared with 16.3 dB for microscopic PORP ( p = 0.02). Using multivariate linear regression to predict postoperative PORP ABG, and controlling for age, prior ossiculoplasty, middle ear mucosal disease (granulation, fibrosis, polyposis), middle ear atelectasis, myringitis, contralateral middle ear disease, and use of byte prostheses, endoscopic PORP reconstruction was associated with improvement in ABG over the microscopic approach by 4.4 dB ( p = 0.04). CONCLUSIONS: For PORP ossiculoplasty procedures, endoscopic ossiculoplasty is associated with improved postoperative ABG compared with microscopic ossiculoplasty.

Endoscopic Versus Microscopic Pediatric Tympanoplasty: Is There a Difference Between Closure Rates and Hearing Outcomes?

OBJECTIVE: To compare closure rates and hearing outcomes of microscopic and endoscopic tympanoplasty in pediatric patients. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary university medical center. PATIENTS: Pediatric patients who underwent tympanoplasty surgery by a fellowship-trained neurotologist between 2010 and 2019 with a minimum of 2 months of follow-up, a tympanic membrane perforation, and no preoperative cholesteatoma. INTERVENTIONS: Transcanal endoscopic tympanoplasty or microscopic tympanoplasty (MT) surgery. MAIN OUTCOME MEASURES: The primary outcome is postoperative closure of the tympanic membrane perforation, assessed using otomicroscopy at the last follow-up appointment. Secondary outcomes include operative time and changes in the air-bone gap (ABG) and pure-tone average (PTA). RESULTS: Two hundred eleven tympanoplasty operations were analyzed: 121 in the transcanal endoscopic ear surgery (TEES) group and 90 in the MT group. Tympanic membrane closure rates were no different between the two groups (TEES, 82.6%; MT, 88.9%; p = 0.24), and no significant association was found on multivariable analysis (TEES: odds ratio, 0.8; p = 0.61). Both groups showed improvements in the 4-month PTA and ABG and the 12-month PTA, but the 12-month ABG only improved in the TEES group ( p < 0.01). The TEES group had a shorter average operative time (109.8 versus 123.5 min; p = 0.03) and less need for a postauricular incision (2.5% versus 93.3%; p < 0.01). CONCLUSION: In pediatric tympanoplasty, TEES gives similar membrane closure and hearing outcomes as the microscopic technique, with less operative time and less need for a postauricular incision.

Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2.

OBJECTIVE: The aim of this study is to investigate facial nerve outcomes after microsurgical resection in neurofibromatosis type 2 (NF2) compared to sporadic tumors. STUDY DESIGN: Single institutional retrospective chart review. SETTING: Tertiary referral center. METHODS: All adult patients with NF2 vestibular schwannoma (VS) or sporadic VS who underwent microsurgical resection from 2008 to 2019 with preoperative magnetic resonance imaging (MRI) and 1 year of postsurgical follow-up were included. The primary outcome measure was postoperative House-Brackmann (HB) facial nerve score measured at first postoperative visit and after at least 10 months. RESULTS: In total, 161 sporadic VSs and 14 NF2 VSs met inclusion criteria. Both median tumor diameter (NF2, 33.5 mm vs sporadic, 24 mm, P = .0011) and median tumor volume (NF2, 12.4 cm3 vs sporadic, 2.9 cm3, P = .0005) were significantly greater in patients with NF2. The median follow-up was 24.9 months (range, 12-130.1). Median facial nerve function after 1 year for patients with NF2 was HB 3 (range, 1-6) compared to HB 1 (range, 1-6) for sporadic VS (P = .001). With multivariate logistic regression, NF2 tumors (odds ratio [OR] = 13.9, P = .001) and tumor volume ≥3 cm3 (OR = 3.6, P = .025) were significantly associated with HB ≥3 when controlling for age, sex, extent of tumor resection, translabyrinthine approach, and prior radiation. CONCLUSION: Tumor volume >3 cm3 and NF2 tumors are associated with poorer facial nerve outcomes 1 year following microsurgical resection.

The Association of Vestibular Schwannoma Volume With Facial Nerve Outcomes After Surgical Resection.

OBJECTIVE: To explore the relationship between tumor size and facial nerve outcomes following vestibular schwannoma (VS) resection. STUDY DESIGN: Single institutional retrospective chart review of all adult patients with untreated sporadic VS who underwent surgical resection from 2008 to 2018 with preoperative magnetic resonance imaging (MRI) and 1 year of follow-up. The primary outcome measure was facial nerve outcome as assessed by the House-Brackmann facial nerve grading system. RESULTS: One hundred sixty-seven patients, 54.5% female, with a median age of 49 years (20-76 years), were identified who underwent VS resection. Surgical resection was performed by translabyrinthine (76.7%), middle cranial fossa (14.4%), retrosigmoid (7.2%), and transpromontorial (1.8%) approaches. The median tumor diameter and volume were 25.3 mm (range: 4.1-47.1 mm) and 3.17 cm3 (range: 0.01-30.6 cm3 ), respectively. The median follow-up was 24.2 months (range: 12-114.2 months). Gross total resection was performed in 79% of cases, with residual tumor identified on MRI in 17% of cases. For patients with tumors <3 cm3 , 92.7% had grade 1 or 2 facial function after at least 1 year follow-up, compared to 81.2% for those with tumors >3 cm3 (univariate logistic regression OR = 2.9, P = .03). Tumor volume >3 cm3 was predictive of facial weakness on multivariate regression analysis (OR = 7.4, P = .02) when controlling for surgical approach, internal auditory canal extension, anterior extension, age, gender, and extent of resection. CONCLUSIONS: Tumor volume >3 cm3 is associated with worse facial nerve outcomes 12 months following surgical resection. LEVEL OF EVIDENCE: IV Laryngoscope, 131:E1328-E1334, 2021.

A Novel Surgical Technique for the Management of Cerebrospinal Fluid Gusher Encountered During Cochlear Implantation.

: Cerebrospinal fluid (CSF) gusher encountered during cochlear implantation are most commonly encountered in the setting of an inner ear abnormality. Repair of the gusher is essential to prevent CSF egress postoperatively and to decrease the risk of meningitis. Various methods to repair a CSF gusher have been described, including tight packing of the cochleostomy with fascia, lumbar drainage, and Eustachian tube packing with ear canal overclosure. We describe a novel and simple technique using a fascia ring placed around the cochlear implantation electrode (Cochlear Corporation, CI522) as a means to treat CSF gusher. The fascia is slid down the electrode after insertion, allowing circumferential coverage of the defect. The following video will provide an overview of patients at risk for CSF gusher and demonstrate the technique described above.SDC video link: http://links.lww.com/MAO/B52.

Revision pediatric cochlear implantation in a large tertiary center since 1986.

Objective: To identify the rate of cochlear implantation (CI) re-implantation and assess audiologic outcomes. Methods: Demographic, audiometric, radiographic, and clinical data were collected from the medical records of the first 834 pediatric patients (age < 18) who underwent CI at a tertiary-care center. Results: Reviewing the first 834 pediatric patients who underwent CI between 1986 and 2013, 33 (4%) children have required re-implantation. Seven (0.8%) of these required a second re-implantation, for a total of 40 total revision surgeries (4.8%) and 21.1% of patients who underwent revision required multiple re-implantations. The mean age at initial CI was 3.5 years old, with identification of the failure an average of 2.7 years later (range, 0.1-10.1 years). The most common indications for re-implantation CI were unknown etiology (58%), vendor recall (18%), and trauma (9%). Twenty-three (88.5%) of the 26 patients who underwent only one re-implantation surgery achieved a complete insertion with both procedures. Eleven (91.7%) of 12 patients who had open set speech after their initial procedure maintained this after re-implantation surgery. Eight (38.1%) of 21 patients who did not have open-set speech after their initial implantation achieved open set speech with CI re-implantation. Conclusion: CI re-implantation is not common in the pediatric population. However, given the time-sensitive nature of speech/language development in children, with the right indications, CI re-implantation can be performed safely without compromising audiologic outcomes. However, re-implanted patients have an increased risk they will require re-implantation again in the future.

Can You Hear Me Now? The Impact of Hearing Loss on Patient Health Literacy.

OBJECTIVE: To elucidate the impact of hearing loss on patient health literacy. STUDY DESIGN: Prospective, cross-sectional study. SETTING: Academic otology practice at a university hospital. PATIENTS: Consecutive, adult, English-speaking patients. MAIN OUTCOME MEASURES: Inadequate health literacy, defined as a composite score of less than or equal to nine on the brief health literacy screen (BHLS), was compared with patient hearing data utilizing the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification. Secondary outcome measures included comparisons of inadequate BHLS scores according to patient demographic and clinical information. RESULTS: There were 300 consecutive adult (>18 years old) patients evaluated with the BHLS at a university otology practice between February and March 2019. The median patient age was 60-years (range, 18-91 yr), a slight majority (160, 53.3%) were women, and most patients were White (241, 86.7%) and non-Hispanic (260, 91.6%). Overall, 9.7% of patients were found to have inadequate health literacy. Men had higher rates of inadequate health literacy as compared with women (13.6% versus 6.3%, odds ratio [OR] = 2.35, 95% confidence interval [CI] 1.06-5.25). Audiometric data was available for 284 (95%) patients, of which 235 (82.7%) had class A or B hearing and 49 (17.3%) had class C or D hearing. Patients with Class C or D hearing had a lower median composite BHLS score compared with patients with Class A or B hearing (11.6 versus 13.6, p < 0.0001) and an increased rate of inadequate health literacy (28.6% versus 4.7%, OR = 8.15, 95% CI 3.42-19.37). Increased age, female sex, and better hearing were independent predictors of higher BHLS scores on multivariable analysis. CONCLUSIONS: Hearing loss is an independent risk factor for inadequate health literacy. Providers should be aware of this risk and consider implementing strategies to improve counseling for this at-risk group of patients.

Identifying Disadvantaged Groups for Cochlear Implantation: Demographics from a Large Cochlear Implant Program.

OBJECTIVE: To identify demographic predictors of patients undergoing cochlear implantation evaluation and surgery. METHODS: Consecutive adult patients between 2009 and 2018 who underwent cochlear implantation evaluation at a university cochlear implantation program were retrospectively identified to determine (1) cochlear implantation qualification rate and (2) pursuit of surgery rate with respect to age, gender, race, primary spoken language, marital status, insurance type, and distance to the cochlear implantation center. RESULTS: A total of 823 cochlear implantation evaluations were analyzed. Overall, 76.3% of patients qualified for cochlear implantation and 61.5% of these patients pursued surgery. Age was the only independent predictor for cochlear implantation qualification, such that, for each year younger, the odds of qualifying for cochlear implantation increased by 2.5% (OR 0.98; 95% CI: 0.96-0.99). Age, race, marital status, and insurance type were each independent predictors of the decision to pursue surgery. The odds of pursuing surgery increased by 2.8% for each year younger (OR 1.03; 95% CI: 1.01-1.05). Compared to White patients, non-Whites were half as likely to pursue surgery (OR 0.47; 95% CI: 0.25-0.88). Single (OR 0.49; 95% CI: 0.26-0.94) and widowed patients (OR 0.46; 95% CI: 0.23-0.95) were about half as likely to pursue surgery as compared to married patients. Patients with military insurance were 13 times more likely to pursue surgery as compared to patients with Medicare (OR 13.0; 95% CI: 1.67-101.4). CONCLUSION: Younger age is an independent predictor for a higher cochlear implantation qualification rate, suggesting the possibility for delayed candidacy referral. Rate of surgical pursuit in qualified cochlear implantation candidates is lower for racial minorities, single and widowed patients, and older patients.

Diagnosis and management of spontaneous cerebrospinal fluid fistula and encephaloceles.

PURPOSE OF REVIEW: To describe the current state in the diagnosis and management of spontaneous cerebrospinal fluid (sCSF) fistula and encephaloceles. RECENT FINDINGS: The increased incidence of obesity has resulted in more cases of sCSF fistula and encephaloceles. Obesity results in increased intracranial pressure and a greater chance of developing a sCSF fistula or encephalocele. Obstructive sleep apnea can also result in transient increase in intracranial pressure and has been shown to be common in patients with sCSF fistula. Treatment of CSF fistula is usually necessary because of the increased risk of meningitis. The use of hydroxyapatite bone cements to repair the temporal bone defects has been described with a high success rate of closing the fistula and a low complication rate. Concurrent superior semicircular canal dehiscent can be seen in up to 15% of cases and should be suspected during the surgical approach to avoid potential sensorineural hearing loss and chronic imbalance. SUMMARY: sCSF fistula and encephaloceles are an uncommon cause of hearing loss, middle ear effusion, and otorrhea, but should be recognized and repaired because of the risk of meningitis.

Middle fossa approach for spontaneous cerebrospinal fluid fistula and encephaloceles.

PURPOSE OF REVIEW: The aim of this article is to describe the middle fossa craniotomy (MFC) approach for the repair of cerebrospinal (CSF) fistula and encephaloceles. RECENT FINDINGS: The MFC approach has a greater than 93% success rate for managing CSF fistula and encephaloceles located along the tegmen tympani and tegmen mastoideum. Posterior fossa defects cannot be managed by an MFC approach. Multilayer repair with the combination of soft tissue and durable substances is preferred. Hydroxyapatite bone cement provides a durable repair of thinned or absent areas of bone with a low risk of infection. Concurrent management of symptomatic superior semicircular canal dehiscence may be readily performed. Small keyhole craniotomies with the utilization of the endoscope are possible as a means to minimize temporal lobe retraction. SUMMARY: MFC repair of CSF fistula and encephaloceles is a highly effective approach for the repair of tegmen mastoideum and tegmen tympani defects.

Cochlear Implantation in Patients With Neurofibromatosis Type 2.

OBJECTIVE: To describe cochlear implantation (CI) outcomes in patients with neurofibromatosis type 2 (NF2). STUDY DESIGN: Retrospective case series. SETTING: A multidisciplinary NF2 clinic at a university hospital. PATIENTS/INTERVENTIONS: Patients with NF2 who underwent CI. MAIN OUTCOME MEASURES: Pre- and postimplantation audiometric data, including pure-tone average (PTA) and AzBio Sentence scores. RESULTS: Eight patients with NF2 underwent CI. The mean age at implantation was 28.6 years (range: 17-63 yr) and six were female. The average length of deafness before CI was 3.5 years (range: 0.3-10 yr). Two patients underwent previous microsurgical resection via middle fossa craniotomy and one patient was treated with stereotactic radiotherapy before CI. Two tumors were growing at the time of CI, five tumors were not growing for an average period of 3.8 years (range: 1-6 yr), and one tumor had undergone previous gross total resection. Mean preoperative unaided PTA was 103.1 dB (range: 81.3-115 dB) and all preoperative AzBio scores were 0% in the ear to be implanted. These improved to a mean postoperative PTA of 30.9 dB (range: 12.5-43.8 dB) and mean postoperative AzBio score of 20% (range: 0-82%) with an average follow-up length of 16.6 months (range: 1.4-27.6 mo). Data logging demonstrated that six patients were daily CI users and two were nonusers, one of whom had normal hearing in the contralateral ear. CONCLUSIONS: CI is an effective option for rehabilitating hearing loss in patients with NF2; however, patients with normal contralateral hearing or poor follow-up do not perform as well.

Long-Term Effects of Bevacizumab on Vestibular Schwannoma Volume in Neurofibromatosis Type 2 Patients.

Introduction Bevacizumab offers a medical treatment that may slow the growth of vestibular schwannomas (VS) and possibly preserve hearing in patients with neurofibromatosis type 2 (NF2). This study aims to investigate the effect of long-term bevacizumab treatment on VS progression. Methods Demographic, clinical, audiometric, and radiographic data were collected from the medical records of NF2 patients treated with bevacizumab at a tertiary medical center. Results Eleven tumors from seven NF2 patients treated with bevacizumab were analyzed. The median age was 17 years (range: 12-47 years). Median bevacizumab treatment time was 33 months (range: 12-74 months). Of five patients with serviceable hearing pretreatment, one (20%) maintained serviceable hearing during bevacizumab therapy. Significantly slower growth rates for both tumor diameters and tumor volumes were identified during active bevacizumab treatment. Median tumor diameters and volumes during active bevacizumab treatment were 0 cm/year (range: -0.13-0.17 cm/year) and 0.1 cm 3 /year (range: -0.92-0.41), compared with 0.37 cm/year (range: 0-1.5 cm/year, p = 0.0011) and 1.38 cm 3 /year (range: 0.013-3.74), respectively, without bevacizumab treatment ( p = 0.0263). Reduced tumor progression was noted with bevacizumab treatment utilizing both linear greatest diameter (hazard ratio 0.16, p = 0.006) and segmentation volumes (hazard ratio 0.15, p = 0.023). Complications of bevacizumab treatment included fatigue (43%), nausea/vomiting (43%), hypertension (43%), epistaxis (29%), and proteinuria (29%). One subject had a cerebrovascular accident detected on screening magnetic resonance imaging without symptoms or neurological sequelae. Discussion Bevacizumab may reduce tumor growth rate and the risk of progression based on both volumetric and linear measurements.

Recidivism After Endoscopic Treatment of Cholesteatoma.

OBJECTIVE: To investigate the recidivism rate of cholesteatoma treated via endoscopic ear surgery (EES), either via transcanal endoscopic ear surgery or endoscopic assisted tympanomastoidectomy compared with a microscopic postauricular approach. STUDY DESIGN: Retrospective chart review. SETTING: Academic otology practice. PATIENTS: Adult patients (18 years and older) with at least 11 months of surgical follow-up who were treated for cholesteatoma via endoscopic techniques or microscopic postauricular approach. INTERVENTION: Use of the endoscope for cholesteatoma dissection. MAIN OUTCOME MEASURE: Residual or recurrent cholesteatoma identified at second look surgery or postoperative diffusion-weighted magnetic resonance imaging. RESULTS: Fifty-nine patients treated for cholesteatoma via endoscopic techniques and 35 patients treated via microscopic postauricular approach were analyzed. The endoscopic group required significantly fewer mastoid procedures (28% versus 80%, p-value 0.001). Postoperative changes in median ABG (5 dB versus 3.75 dB, p = 0.9519), median PTA (6.875 dB versus 1.25 dB, p = 0.3864), and median word recognition score (0% versus 0%, p = 0.3302) were not significantly different between the EES and microscopic surgery groups. Median operative times were not significantly different between the two groups (182 min endoscopic versus 174 min microscopic, p-value 0.66). The rate of residual disease (17% EES versus 17% microscopic, p = 0.959) or disease recurrence (18% endoscopic versus 20% microscopic, p = 0.816) were not significantly different between the two groups. CONCLUSIONS: EES is an effective option for cholesteatoma management with similar rates of recurrent or residual disease as compared with the more traditional microscopic postauricular approach in these samples.