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Objectives/Hypothesis The aims of this study were to investigate short- and long-term rates of hearing preservation after cochlear implantation and identify factors that impact hearing preservation. Study Design Retrospective review. Methods Patients undergoing cochlear implantation with conventional-length electrodes and air-conduction thresholds ≤80 dB HL at 250 Hz preoperatively were included. Hearing preservation was defined as air-conduction thresholds ≤80 dB HL at 250 Hz. Results The sample included 196 patients (225 implants). Overall, the rate of short-term hearing preservation was 38% (84/225), with 18% (33/188) of patients preserving hearing long term. Multivariate analysis showed better preoperative hearing was predictive of hearing preservation at short (odds ratio [OR]: 0.93, 95% confidence interval [CI]: 0.91-0.95, P < .001) and long-term follow-up (OR: 0.94, 95% CI: 0.91-0.97, P < .001). Lateral wall electrodes and mid-scala electrodes had 3.4 (95% CI: 1.4-8.6, P = .009) and 5.6-times (95% CI: 1.8-17.3, P = .003) higher odds of hearing preservation than perimodiolar arrays at short-term follow-up, respectively. Long-term data revealed better hearing preservation for lateral wall (OR: 7.6, 95% CI: 1.6-36.1, P = .01), but not mid-scala (OR: 3.1, 95% CI: 0.4-23.1, P = .28), when compared to perimodiolar electrodes. Round window/extended round window (RW/ERW) approaches were associated with higher rates of long-term hearing preservation (21%) than cochleostomy approaches (0%) (P = 0.002) on univariate analysis. Conclusions Better preoperative residual hearing, lateral wall electrodes, and RW/ERW approaches are predictive of higher rates of long-term functional hearing preservation. Level of Evidence 4 Laryngoscope, 2017

Background One study has investigated postoperative growth rates following subtotal resection of petroclival meningiomas utilizing linear measurements, which are insensitive to the multidimensional complex growth of meningiomas, to estimate tumor volume. Objective To describe petroclival meningioma growth patterns following less-than-complete resection utilizing volumetric analysis and to identify variables associated with tumor progression. Methods Patients with surgically resected WHO grade I petroclival meningiomas were retrospectively reviewed (1999-2015). Image analysis software was utilized to perform volumetric analyses of tumor size and growth on serial MRI studies. The impact of preoperative and postoperative variables on tumor growth after subtotal resection was analyzed. An increase in tumor volume of at least 20% was defined as "tumor growth." Results Twenty-three patients had immediate preoperative and serial postoperative MRI studies available for review. The mean preoperative tumor volume was 20.9 cm3 (range 0.4-54.6). The mean extent of resection was 75.5% (range 31.5%-100.0%). At a mean follow-up of 24.8 mo, 12 tumors (66.7%) exhibited radiological tumor growth, while 6 tumors did not change in size. The median annual volumetric growth rate was 2.82 cm3/yr (range -0.34 to 10.1). Extent of resection and immediate postoperative tumor volume were significantly correlated with the annual volumetric growth rate following resection. At last follow-up, 3 (13%) patients required further intervention. Conclusion The majority of petroclival meningiomas exhibit growth following subtotal resection. Extent of resection is strongly associated with risk for disease progression following surgery.

Objectives 1) Examine angular insertion depths (AID) and scalar location of Med-El (GmbH Innsbruck, Austria) electrodes; and 2) determine the relationship between AID and audiologic outcomes controlling for scalar position. Study Design Retrospective review. Methods Postlingually deafened adults undergoing cochlear implantation with Flex 24, Flex 28, and Standard electrode arrays (Med-El) were identified. Patients with preoperative and postoperative computed tomography scans were included so that electrode location and AID could be determined. Outcome measures were 1) speech perception in the cochlear implant (CI)-only condition, and 2) short-term hearing preservation. Results Forty-eight implants were included; all electrodes (48 of 48) were positioned entirely within the scala tympani. The median AID was 408° (interquartile [IQ] range 373°–449°) for Flex 24, 575° (IQ range 465°–584°) for Flex 28, and 584° (IQ range 368°–643°) for Standard electrodes (Med-El). The mean postoperative CNC score was 43.7% ± 21.9. A positive correlation was observed between greater AID and better CNC performance (r = 0.48, P < 0.001). Excluding patients with postoperative residual hearing, a strong correlation between AID and CNC persisted (r = 0.57, P < 0.001). In patients with preoperative residual hearing, mean low-frequency pure-tone average (PTA) shift was 27 dB ± 14. A correlation between AID and low-frequency PTA shift at activation was noted (r = 0.41, P = 0.04). Conclusion Favorable rates of scala tympani insertion (100%) were observed. In the CI-only condition, a direct correlation between greater AID and CNC score was noted regardless of postoperative hearing status. Deeper insertions were, however, associated with worse short-term hearing preservation. When patients without postoperative residual hearing were analyzed independently, the relationship between greater insertion depth and better performance was strengthened. Level of Evidence 4. Laryngoscope, 127:2352–2357, 2017

Hypothesis An image-guided robotic system can safely perform the bulk removal of bone during the translabyrinthine approach to vestibular schwannoma (VS). Background The translabyrinthine approach to VS removal involves extensive manual milling in the temporal bone to gain access to the internal auditory canal (IAC) for tumor resection. This bone removal is time consuming and challenging due to the presence of vital anatomy (e.g., facial nerve) embedded within the temporal bone. A robotic system can use preoperative imaging and segmentations to guide a surgical drill to remove a prescribed volume of bone, thereby preserving the surgeon for the more delicate work of opening the IAC and resecting the tumor. Methods Fresh human cadaver heads were used in the experiments. For each trial, the desired bone resection volume was planned on a preoperative computed tomography (CT) image, the steps in the proposed clinical workflow were undertaken, and the robot was programmed to mill the specified volume. A postoperative CT scan was acquired for evaluation of the accuracy of the milled cavity and examination of vital anatomy. Results In all experimental trials, the facial nerve and chorda tympani were preserved. The root mean squared surface accuracy of the milled cavities ranged from 0.23 to 0.65 mm and the milling time ranged from 32.7 to 57.0 minute. Conclusion This work shows feasibility of using a robot-assisted approach for VS removal surgery. Further testing and system improvements are necessary to enable clinical translation of this technology.

Objective To quantify the potential effectiveness of cochlear implantation for tinnitus suppression in patients with single-sided deafness using the Tinnitus Handicap Inventory. Methods The study included 12 patients with unilateral tinnitus who were undergoing cochlear implantation for single-sided deafness. The Tinnitus Handicap Inventory was administered at the patient's cochlear implant candidacy evaluation appointment prior to implantation and every cochlear implant follow-up appointment, except activation, following implantation. Patient demographics and speech recognition scores were also retrospectively recorded using the electronic medical record. Results A significant reduction was found when comparing Tinnitus Handicap Inventory score preoperatively (61.2 ± 27.5) to the Tinnitus Handicap Inventory score after three months of cochlear implant use (24.6 ± 28.2, p = 0.004) and the Tinnitus Handicap Inventory score beyond 6 months of CI use (13.3 ± 18.9, p = 0.008). Further, 45% of patients reported total tinnitus suppression. Mean CNC word recognition score improved from 2.9% (SD 9.4) pre-operatively to 40.8% (SD 31.7) by 6 months post-activation, which was significantly improved from pre-operative scores (p = 0.008). Conclusion The present data is in agreement with previously published studies that have shown an improvement in tinnitus following cochlear implantation for the large majority of patients with single-sided deafness.

To determine the accuracy of a modern navigation system in temporal bone surgery. While routine in other specialties, navigation has had limited use in the temporal bone due to issues of accuracy, perceived impracticality, and value.Prospective observational study.Temporal bone laboratory.Eighteen cadaveric specimens were dissected after rigid fiducials were implanted and computed tomography scans were obtained. Target registration and target localization errors were then measured at various points.The mean overall target registration error was 0.48 ± 0.29 mm. The mean target localization error was 0.54 mm at the sinodural angle, 0.48 mm at the lateral semicircular canal, 0.55 mm at the round window, 0.39 mm at the oval window, and 0.52 mm at the second genu of the facial nerve.A modern navigation system demonstrated submillimeter accuracy for all points of interest. Its use in clinical as well as training settings has yet to be fully elucidated.

Objective This meta-analysis compares the efficacy and safety of endoscopic and microscopic approaches to tympanoplasty and stapes surgery, two common middle ear procedures. Data sources A comprehensive electronic search of PubMed/MEDLINE, Scopus, Web of Science, and Cochrane Library was conducted for studies published from the year 1960 through June 2018. Article selection and screening proceeded according to the strategies outlined in the standard Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. Methods Studies were assessed for quality using the Newcastle-Ottawa Scale and the Jadad scale depending on the presence of randomization. Bias was analyzed using funnel plots. For each outcome measure, a forest plot was generated and a pooled relative risk or risk difference was calculated to assess significance. Results Twenty-one studies met the inclusion criteria, 16 tympanoplasty (1,323 ears) and 5 stapes surgery (283 ears). The nonrandomized studies scored moderately well but the randomized studies were of low quality with respect to the Newcastle-Ottawa Scale and the Jadad Scale, respectively. With respect to tympanoplasty, statistical analysis demonstrated that the endoscopic approach had significantly lower canalplasty rates, better cosmetic outcomes, and shorter operative times. Furthermore, endoscopic compared to microscopic methods were similar in terms of audiological outcome, graft success rate, and complication rate. Among stapes surgeries, the endoscopic approach demonstrated a significantly decreased incidence of postoperative pain and chorda tympani injury but was similar to the microscopic approach with respect to audiological outcome, postoperative dizziness, and operative time. Conclusions Though the merits of endoscopic techniques are becoming more well known, there are still concerns regarding their efficacy and safety. An analysis of the current literature suggests that audiological, functional, and safety outcomes are similar, if not superior, for the endoscopic approach to both tympanoplasty and stapes surgery compared to the microscopic approach. With respect to tympanoplasty, patients undergoing the endoscopic approach had lower canalplasty rates, better cosmetic outcomes, and shorter operative times. Among stapes surgeries, patients in the endoscopic group had a lower incidence of postoperative pain and injury to the chorda tympani. This meta-analysis of the current evidence supports the use of endoscopic techniques for tympanoplasty and stapes surgery.

OBJECTIVES To determine whether image guidance improves dissection quality among residents in the temporal bone lab and to gauge their reactions to its use. METHODS Prospective cohort study of nine otolaryngology residents who dissected cadaveric temporal bones with and without image guidance in the temporal bone laboratory. Quality of dissection was measured with the Welling scale. A survey was administered assessing their interactions with the navigation system. RESULTS There was no significant difference in quality of dissection for the majority of structures or number of major errors with use of image guidance. Dissection quality of the tegmen (4.67 vs. 5.67, P = .034) and sinodural angle (0.83 vs. 1.56, P = .005) was superior in the non-navigation specimens. The majority of residents reported increased confidence with locating several critical anatomic structures and found the system intuitive to use. Nearly all thought it would be helpful in revision cases or for aural atresia. CONCLUSION Image guidance did not improve quality of temporal bone dissection or number of major errors in this cohort of residents, but the majority found it intuitive and increased confidence in finding several structures. Navigation systems may play an important adjunctive role in resident education and further work is required to determine its optimal use. LEVEL OF EVIDENCE NA Laryngoscope, 130:E368-E371, 2020.

To describe and assess intraoperative and postoperative outcomes in the insertion of osseointegrated auditory implants with a newly designed surgical instrumentation set through a punch type technique.Retrospective case series.Patients who underwent bone anchored auditory implant surgery using the Minimally Invasive Ponto Surgery (Oticon Medical, Somerset, NJ) surgical set through a punch technique at nine neurotology tertiary referral based practices were identified. Demographic data, skin thickness at implant site, implant used, duration of surgery, adverse intraoperative events, and postoperative outcomes were recorded.Seventy-five patients comprised the study cohort (32 males, 43 females). Most patients (57. 3%) were aged 51 to 75 years while 30.7% of the cohort comprised those aged 18 to 50 years and 12% were over 75 years. All but two patients received 4 mm fixtured implants and 68% received the Oticon Medical BioHelix implant. Two patients received 3 mm fixture implants and 32% received the Oticon Medical Wide Ponto implant. Mean surgical time was 12.2 minutes (6-45 min, standard deviation of 6.88 min). In three instances, surgery was converted to a linear incision to control brisk bleeding. Skin condition was Holgers 0 to 1 in 91.8%, while 5.5% had Holgers 2, and 2.7% had Holgers 3 at the first postoperative visit. At second postoperative visit, 94.3% had Holgers 0 to 1, 4.3% had Holgers 2, and 1.4% had Holgers 3. All instances of adverse skin reactions were treated with topical or systemic antibiotics and/or local debridement. There were no instances of implant loss. One patient had his implant traumatically displaced to a 45-degree angle necessitating implant replacement at a second site.Punch technique placement of osseointegrated auditory implants using the Minimally Invasive Ponto Surgery surgical set represents a safe technique that further simplifies a progressively minimally invasive surgery.

Objectives: To analyze hearing outcomes following Type 3 tympanoplasty with stapes columella grafting after canal wall down mastoidectomy and determine disease recurrence rates in patients undergoing this procedure. Methods: This retrospective cohort analysis examines patients undergoing Type 3 tympanoplasty with stapes columella grafting following canal wall down mastoidectomy for cholesteatoma at a tertiary care center from 2005 to 2015. Patient charts were reviewed for demographic data, diagnosis, and operative details. Patients were included in statistical analysis if they were found to have undergone the aforementioned procedure. Evaluation of hearing improvement was made by comparing preoperative air-bone gap (ABG) and ABG at follow-up at 6 months and 1 year postoperatively. Results: Nineteen patients met criteria for this study. Erosion of the otic capsule, posterior fossa plate, or tegmen was noted in 37% of cases, highlighting disease severity. Eighteen (95%) had undergone prior otologic surgery. Mean time to short-term follow-up was 6 ± 3 months. The average short-term ABG was 26 ± 11 dB HL; 26% achieved an ABG Conclusion: In some patients undergoing canal wall down mastoidectomy for advanced or recurrent cholesteatoma, Type 3 tympanoplasty with stapes columella grafting yields marginal hearing benefit. This type of reconstruction is a viable option in this challenging patient cohort, particularly as it is associated with low rates of revision surgery.

Objectives This study characterizes primary petroclival meningioma growth rates, before intervention, using volumetric analysis. In addition, predictors of growth are analyzed. Methods Patients with previously untreated petroclival meningiomas were retrospectively reviewed (1999-2015). Image analysis software was used to perform volumetric analyses of tumor size and growth. Three-dimensional segmentation volumetric analyses were compared with volumes estimated utilizing three orthogonal dimensions. Tumor growth was defined as a 15% increase in volume. Results Thirty-four patients who underwent at least two magnetic resonance imaging (MRI) studies before intervention were included. The mean age was 55.2 years, and 65.7% were women. The mean tumor volume at presentation was 5.6 cm (range, 0.1-25.8 cm) as determined from segmentation volumetric analysis. At a mean follow-up of 44.5 months (range, 3.7-125.1 mo), 88.2% of tumors grew. The mean annual volumetric growth rate was 2.38 cm/yr (-0.63 to 25.9 cm/yr). Tumor volume, T2 hyperintensity within the tumor, peritumoral edema, and ataxia and/or cerebellar symptoms at presentation were all significantly associated with greater rates of tumor growth. Ultimately, 10 (29.4%) patients underwent treatment during the follow-up period. Conclusion Our experience demonstrates that the vast majority (88%) of untreated petroclival meningiomas grow; the mean volumetric growth rate was noted to be 2.38 cm/yr. We found a significant association between increased growth rate and larger tumor size at diagnosis, T2 hyperintensity within the tumor, peritumoral edema, and the presence of ataxia and/or cerebellar symptoms.

Objectives As indications for cochlear implantation have expanded to include patients with more residual hearing, increasing emphasis has been placed on minimally traumatic electrode insertion. Histopathologic evaluation remains the gold standard for evaluation of cochlear trauma, but advances in imaging techniques have allowed clinicians to determine scalar electrode location in vivo. This review will examine the relationship between scalar location of electrode arrays and audiologic outcomes. In addition, the impact that surgical approach, electrode design, and insertion depth have on scalar location will be evaluated. Data Sources: PubMed literature review Review Methods: A review of the current literature was conducted to analyze the relationship between scalar location of cochlear implant electrode arrays and speech perception outcomes. Further, data were reviewed to determine the impact that surgical variables have on scalar electrode location. Results Electrode insertions into the scala tympani are associated with superior speech perception and higher rates of hearing preservation. Lateral wall electrodes, and round window/extended round window approaches appear to maximize the likelihood of a scala tympani insertion. It does not appear that deeper insertions are associated with higher rates of scalar translocation. Conclusion Superior audiologic outcomes are observed for electrode arrays inserted entirely within the scala tympani. The majority of clinical data demonstrate that lateral wall design and a round window approach increase the likelihood of a scala tympani insertion. Level of Evidence N/A.

Objective Patients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time. Study Design Retrospective review at two tertiary care academic referral centers. Methods Adults analyzed were diagnosed with SSCD by high-resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms. Patients with other potential causes of hearing impairment were excluded. Results A total of 40 ears in 30 adult patients (median age: 59 years; 63% female) were analyzed. Median audiometric follow-up was 23 months (range 1–136 months). None experienced a sudden hearing loss over the follow-up period. In patients with audiometric follow-up of at least 20 months (median 34 months), the median change in air-conduction pure tone average and air–bone gap was 0.9 decibels (dB) per year (interquartile range [IQR] 0–2.1) and 0.7 dB per year (IQR 0–2.0), respectively. Speech discrimination scores did not differ when comparing median initial (100%) and median final (98%) scores (P = 0.77). There was no statistically significant change in bone-conduction thresholds at 0.5, 1, 2, and 4 kHz over the period of observation. Conclusion The risk of progressive hearing loss with observed SSCD is low during short- and intermediate-term follow-up. Further studies are necessary to determine whether late hearing loss occurs. Such information may be critical toward patient counseling regarding the need for and timing of surgery. Level of Evidence 4. Laryngoscope, 127:1181–1186, 2017

Objective The middle fossa floor lacks reliable surface landmarks. In cases of superior semicircular canal dehiscence (SSCD), multiple skull base defects may be present, further confounding the location of the labyrinth. Misidentification of the SSCD during surgery may lead to treatment failure or sensorineural hearing loss. Anecdotally, the authors have observed the distance from the lateral edge of the craniotomy to the SSCD to be consistently 1 inch. Herein, we present radiologic evidence of this practical and clinically useful relationship. Patients All patients at two tertiary care academic referral centers with high-resolution temporal bone computed tomography (CT) evidence of SSCD were retrospectively reviewed. Intervention(s) Review of high-resolution temporal bone CT. Main outcome measures The horizontal distance from the outer cortex of the squama temporalis immediately superior to the bony external auditory canal (approximating lateral edge of craniotomy) to the SSCD was measured in the coronal plane by two independent reviewers. Results A total of 151 adult ears with SSCD were analyzed. A Shapiro-Wilk goodness-of-fit test confirmed that measurements were normally distributed. Pearson inter-rater correlation was 0.95, confirming very strong agreement. The mean distance between the outer cortex of the squama temporalis and SSCD was 25.9 mm, or 1.02 inches. Sixty-eight percent of the SSCD population would fall between 0.92 and 1.12 inches and 95% would lie between 0.83 and 1.21 inches. Conclusions The horizontal distance from the outer cortex of the squama temporalis to the SSCD consistently approximates 1 inch. This easily remembered distance can aid surgeons in locating or confirming the SSCD during middle fossa surgery.

Objectives To test the hypothesis that bipolar electrical impedance measurements in perimodiolar cochlear implants (CIs) may be used to differentiate between perimodiolar insertion technique favoring proximity to the modiolus or lateral wall. Study Design and Methods Bipolar impedances are a measure of electrical resistance between pairs of electrode contacts in a CI. Stimulation is through biphasic pulses at fixed frequency. Impedance measurements were made in real time through sequential sampling of electrode pairs. Perimodiolar electrodes were inserted in temporal bones using one of two techniques: 1) In the standard insertion technique (SIT), the electrode array slides along the lateral wall during insertion. 2) In the Advance Off Stylet (Cochlear Ltd. Sydney) technique (AOS), the electrode maintains modiolar contact throughout the insertion process. A set of 22 insertions were performed in temporal bone specimens using perimodiolar electrode arrays with both AOS and SIT. Buffered saline was used as a substitute for natural perilymph based on similar electrical conductivity properties. Impedance with and without stylet removal were recorded with a 30-second sampling window at final insertion depth. Results There is a significant difference in bipolar impedance measures between AOS and SIT, with impedances rising in measurements with stylet removal. Evaluation was based on two-sided analysis of variance considering technique and electrode with P < 0.025. Conclusion Bipolar electrical impedance can be used to detect relative motion toward the modiolus inside the cochlea. This detection method has the potential to optimize intraoperative placement of perimodiolar electrode arrays during implantation. We anticipate that this will result in lower excitation thresholds and improved hearing outcome. Level of Evidence N/A. Laryngoscope, 2016

OBJECTIVE To analyze disease presentation, treatment, and clinical course of a consecutive series of patients with primary cerebellopontine angle (CPA) epidermoids. PATIENTS Forty-seven consecutive patients with previously untreated CPA epidermoids. INTERVENTION(S) Observation and microsurgery. MAIN OUTCOME MEASURES Disease- and treatment-associated morbidity, recurrence. RESULTS Forty-seven patients (mean age 39 years; 53% women) were analyzed and the average duration of follow-up was 42 months. The most common presenting symptom was headache (27; 57%); 13 (28%) exhibited preoperative asymmetric sensorineural hearing loss, 3 (6%) facial nerve paresis, and 3 (6%) hemifacial spasm. Thirteen patients (28%) were initially observed over a mean interval of 56 months; however, five experienced disease progression requiring operation. Thirty-nine patients (83%) underwent surgical resection; 18 (46%) received gross total, 5 (13%) near total, and 16 (41%) aggressive subtotal resection. Three patients (8%) recurred at a median of 53 months; two after subtotal and one after gross total resection. Ninety-three percent of patients with useful hearing maintained serviceable hearing following treatment and one patient (3%) experienced mild long-term postoperative facial nerve paresis (HB II/VI). All patients with preoperative facial nerve paresis recovered normal function postoperatively. There were no episodes of stroke or death. CONCLUSIONS Surgical intervention is effective in alleviating symptoms of cranial neuropathy and brainstem compression from CPA epidermoids. Gross total resection is preferred; however, aggressive subtotal removal should be considered with adherent or extensive disease as reoperation rates are low, even in the setting of aggressive subtotal resection. Conservative observation with serial imaging is a viable initial strategy in asymptomatic or minimally symptomatic patients.

We sought to assess the complication rates following cochlear implantation in canal wall down mastoid cavities in adults and children.A systematic review of English articles from PubMed, Web of Science, EMBASE, and the Cochrane Library. Additional studies were identified by reviewing the reference lists of the originally identified studies.Studies were included that reported on surgical outcomes following cochlear implantation in canal wall down mastoid cavities. Two authors, using predefined data fields, independently reviewed all articles while tabulating study quality indicators. A meta-analysis of proportions was conducted on select cohorts to determine weighted complication rates.Forty-two articles were included that accounted for 424 patients and described surgical outcomes following cochlear implantation in canal wall down mastoid cavities. In a comparison of the postoperative complication rates, overclosing the external auditory meatus had significantly fewer global complications than did maintaining a canal wall down mastoid cavity (P = .027). In a comparison of the complication rates (1) between staging the cochlear implantation and performing external auditory meatus overclosure simultaneously with cochlear implantation and (2) between pediatric and adult cochlear implantation recipients with external auditory meatus overclosure, there were no significant differences (P = .085 and P = .92, respectively).Overclosing the external auditory meatus at the same time of cochlear implantation leads to significantly fewer complications when compared with maintaining a canal wall down mastoid cavity with soft tissue coverage of the electrode array.

Cochlear implantation in canal wall down mastoid (CWD) cavities is fraught with the issue of how best to manage the mastoid cavity. Decision points include whether the external auditory meatus should be overclosed, whether the implantation should be staged, or even if the eustachian tube should be plugged. Given these options, we sought to describe our experience of cochlear implantation in CWD cavities. Among evaluation of subjects ≥18 years of age, 9 had cochlear implants placed in CWD mastoid cavities, 7 of which had the external auditory meatus overclosed, while 2 maintained open cavities following implantation. With an average follow-up of 36.7 months (median, 22.8 months), 2 subjects who had overclosure developed meatal dehiscences requiring further intervention, while no complications were observed in the 2 patients who maintained open cavities.

Introduction Traumatic cerebrospinal fluid (CSF) fistulae can be a challenging neurosurgical disease, often requiring complicated surgical intervention. Case Presentation A 54-year-old man presented with a gunshot wound to the head with complex injury to the skull base and significant CSF leakage from multiple sites. A single surgery was performed using a combined Neurosurgery, Neurotology, and Rhinology team, which was successful in repairing the multiple skull base defects and preventing further CSF leak. Discussion Trauma to the skull base is a common inciting factor for the development of CSF fistulae. Endoscopic approaches are often preferred for repairing these defects, but craniotomy remains a viable option that may be required in more complex cases. A combined approach has not been described previously, but was successful for this severe multifocal defect. Conclusion A multidisciplinary approach allowed for a combined intervention that addressed both the anterior and middle fossae fistulae simultaneously. This limited the potential infectious complications of continued CSF leak and allowed for early rehabilitation.

SUMMARYDuring the past decade, robotics for cochlear implant electrode array insertion has been limited to manipulation assistance. Going beyond manipulation assistance, this paper presents the new concept ofperception augmentationto detect and warn against theonsetof intracochlear electrode array tip folding. Thisonlinefailure detection method uses a combination of intraoperative electrode insertion force data and a predictive model of insertion force profile progression as a function of insertion depth. The predictive model uses statistical characterization of insertion force profiles during normal robotic electrode array insertions as well as the history of intra-operative insertion forces. Online detection of onset of tip folding is achieved using the predictive model as an input into a support vector machine classifier. Results show that the detection of tip folding onset can be achieved with an accuracy of 88% despite the use of intra-operative insertion force data representing incomplete insertion. This result is significant because it allows the surgeon or robot to choose a corrective action for preventing intra-cochlear complications.

This study explores the current literature regarding associations between phosphodiesterase-5 (PDE-5) inhibitors and ototoxicity and provides a detailed summary and discussion of the findings.A comprehensive electronic search of PubMed/MEDLINE, Scopus, and Cochrane Library for studies published from database inception through March 21, 2018.Basic science articles, epidemiological studies, randomized controlled trials, cohort studies, case reports, reviews, meta-analyses, press releases, and newsletters were included. The PRISMA search strategy was used to select papers. Search terms are included in the appendix (http://links.lww.com/MAO/A733).Twenty-two articles met the inclusion criteria. Among case reports, there were a total of nine patients, all male, with an average age of 57.4 years (37-79 years, SD = 13.87 years). Of the cases of hearing loss, 25% (2/8 cases) were bilateral and 75% (6/8) were unilateral; 22% (2/9) were associated with tinnitus; and 33% (3/9) had accompanying vestibular symptoms (including vertigo and dizziness). Among multipatient studies, all prospective studies failed to find a significant association between ototoxicity and PDE-5 inhibitor use. Results of the retrospective studies were also heterogeneous. Many key molecules in the PDE-5 inhibition pathway have been demonstrated to exist in the cochlea. However, mirroring the clinical studies, the basic science mechanisms have suggested both ototoxic and otoprotective effects.Currently, the literature is inconclusive regarding the interaction between PDE-5 inhibitor use and ototoxicity. Future study such as a double-blinded placebo controlled randomized trial with audiometric assessment would provide more sound evidence. Similarly, a unified molecular model is necessary.

Herein we discuss the various vestibular disorders found in the pediatric population. The etiologies are wide ranging and include labyrinthine, otologic, neurologic, psychiatric, and ophthalmologic etiologies.

Objectives To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. Study design Multicenter retrospective review. Setting Three tertiary referral centers. Patients All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. Intervention Surgical repair or observation. Main outcome measure Radiographic findings and clinical symptoms were analyzed. Results Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. Conclusions SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

We present a rare intracranial vagal schwannoma along with its preoperative, intraoperative, and postoperative course.

Introduction Hearing loss in children is a common entity worldwide. We examined the prevalence and etiology of hearing loss among primary school children in Mbarara, Uganda. Methods Cross-sectional study in primary school children aged 5–14 was performed to determine the prevalence of hearing loss. Ugandan primary school children were screened for disabling hearing loss (threshold >30 dB) and confirmatory audiometry was performed on those who failed the screening. Results There were 639 children screened. Thirty-five (5.5%) of children screened failed and were referred for further testing. Two children were lost to follow-up. The percentage of children with true hearing loss was 3.1%. Conclusions The incidence of failed hearing screening and hearing loss in Mbarara, Uganda is similar to other populations. Hearing loss is a significant problem in Uganda and efforts should be made for primary, secondary, and tertiary prevention of hearing loss.

Objectives To evaluate the effectiveness of resorbable mesh cranioplasty at reducing postoperative cerebrospinal fluid (CSF) leak and pseudomeningocele formation after translabyrinthine tumor resection. Study design Case series with chart review. Setting Tertiary academic referral center. Patients Fifty-three consecutive cases using a resorbable mesh cranioplasty after translabyrinthine tumor resection were reviewed. Intervention Temporal bone defects were repaired with a dural substitute, layered fat graft, and a resorbable mesh plate secured with screws. Main outcome measures Primary outcome measures included the incidence of postoperative CSF wound leak or rhinorrhea, pseudomeningocele formation, and surgical site infection. Results Fifty-three cases (average age, 54.0 yr; range, 19.3-75.1 yr) were analyzed. The average body mass index was 30.8 kg/m2 (range, 17.9-48.3 kg/m2), and the average tumor size was 18.8 mm (range, 8-38 mm). One patient (1.9%) experienced CSF rhinorrhea on postoperative Day 16, which resolved after transmastoid middle ear and eustachian tube packing. One patient (1.9%) experienced a surgical site infection requiring surgical debridement and mesh removal 4 months after surgery. Compared with 1,441 prior translabyrinthine surgeries analyzed from our institution using a traditional fat graft closure without mesh, the rate of postoperative CSF leak was significantly less using the resorbable mesh cranioplasty technique (p = 0.0483). Conclusion Resorbable mesh cranioplasty is a safe and effective method to reduce postoperative CSF leak and pseudomeningocele formation after translabyrinthine craniotomy for tumor excision.

Temporal lobe encephaloceles are characterized by protrusion of brain parenchyma through a structural defect in the floor of the middle fossa. They have been reported to cause cerebrospinal fluid (CSF) leaks, conductive hearing loss, meningitis, and seizures. The association between temporal encephaloceles and epileptiform activity is particularly rare.All patients who presented to a single tertiary referral center between 2011 and 2014 with intractable seizures and radiographic evidence of a middle cranial fossa encephalocele were evaluated. Five patients from this subset who underwent surgical repair of their encephalocele are presented.Middle cranial fossa approach for encephalocele repair.Postoperative epileptiform activity.Five patients underwent a craniotomy for resection of a temporal lobe encephalocele with repair of a middle fossa floor defect. After surgery, CSF rhinorrhea resolved, when present, and all patients remained seizure-free through their last available follow-up. Range of follow-up time was 3.5 months to 4 years. Average follow-up time was 19.7 months.Temporal lobe encephaloceles are an infrequent cause of seizures. Given that these lesions can be missed with standard imaging modalities, they are likely underdiagnosed upon initial medical evaluation. This diagnosis should be considered in patients with intractable seizures. If an encephalocele is found, focused resection of epileptogenic tissue associated with herniation and repair of the temporal floor defect can provide definitive treatment.

Objective To describe the incidence and management of patients with facial nerve stimulation (FNS) associated with cochlear implant (CI) use in the setting of a prior temporal bone fracture. Patients One adult CI recipient is reported who experienced implant associated FNS with a history of a temporal bone fracture. Additionally, a literature search was performed to identify similar patients from previous descriptions of CI related FNS. Main outcome measures Presence of FNS after implantation and ability to modify implant programming to avoid FNS. Results The patient in the present report experienced FNS for middle and basal electrodes during intraoperative neural response telemetry (NRT) in the absence of any surgical exposure or manipulation of the facial nerve. FNS was absent during device activation, but it recurred during follow-up programming sessions. However, additional programming has prevented further FNS during regular implant use. Four other patients with FNS after temporal bone fracture were identified from the literature, and the present case represents the one of two cases in which reprogramming allowed for implant use without FNS. Conclusions CI associated FNS is uncommon in patients with a history of a temporal bone fracture, but it is likely that fracture lines provide a lower impedance pathway to the adjacent facial nerve and thus reduce the threshold for FNS. The present report suggests that, in the setting of a prior temporal bone fracture, NRT is not always a reliable predictor of FNS during implant use, and programming changes can help to mitigate FNS when it occurs.

Petroclival meningiomas are most commonly found in women around 50 years of age and have the general tendency to grow and affect several cranial nerves. At presentation, many patients complain of headaches, gait disturbances and cranial neuropathies. Treatment options include surgery, with a variety of surgical approaches, and or radiotherapy. Current trends support subtotal resection with postoperative radiotherapy. This review summarizes the literature of petroclival meningiomas, discussing topics including definitions, genetics, common presenting signs and symptoms, imaging characteristics, natural history, common surgical approaches, surgical outcomes, complications, and radiotherapy.

Cholesterol granuloma is the most common primary lesion of the petrous apex. Management of petrous apex cholesterol granuloma has evolved in recent years from primary surgical treatment to conservative observation. In this article, a case of spontaneous resolution of a petrous apex cholesterol granuloma is described. To the authors' knowledge, this is the first known report of spontaneous involution of a petrous apex cholesterol granuloma. Radiographic differential diagnosis of petrous apex lesions and the natural history of cholesterol granulomas are discussed.

Temporal bone paragangliomas (TBPs) are slow-growing, highly vascular primary neoplasms of the middle ear and lateral skull base that are composed of paraganglion cells derived from the neural crest. Paraganglioma can be found throughout the body, though in the temporal bone, two forms predominate: tympanic paraganglioma (TP) that originate in the middle ear cleft along the tympanic plexus associated with Jacobson’s (IX) and Arnold’s (X) nerves and jugular paraganglioma (JP) derived from chief cells located in the adventitia of the jugular bulb (Moore et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 54(4):597–605, 2016; Carlson et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 152(1):98–105, 2015; Wanna et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 151(6):991–995, 2014; Langerman et al, Arch Otolaryngol – Head Neck Surg 138(4):341–345, 2012; Jackson et al, Arch Otolaryngol;108(7):401–410, 1982). Most TBP are solitary, non-biochemically secreting and benign, while less than 5% are associated with catecholamine release or malignant character; up to 17% of patients display multiple paragangliomas (Jackson et al, Am J Surg 59(4):389–393, 1990). TBP exhibit a female predominance and most commonly manifest during the fourth to fifth decades of life with symptoms of unilateral pulsatile tinnitus, conductive hearing loss, and less commonly lower cranial neuropathy. Owing to the rare prevalence and typical insidious growth, many patients endorse a long duration of symptoms at diagnosis (Moore et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 54(4):597–605, 2016; Carlson et al, Otolaryngol – Head Neck Surg: Off J Am Acad Otolaryngol-Head Neck Surg 152(1):98–105, 2015).

(1) To investigate the outcomes of cochlear implant receiver-stimulator (RS) placement using a tight subperiosteal pocket technique without device fixation and (2) to compare the efficiency of this approach with the traditional bony well and trough technique.Case series with planned chart review.Single tertiary academic referral center.All cochlear implant surgeries utilizing a tight subperiosteal pocket without additional fixation or use of a bone well were identified retrospectively. Revision cases were only included if the tight subperiosteal pocket technique was used during the initial surgery. Patients with less than 6 months of postoperative follow-up were excluded. Primary outcome measures included RS migration, flap complications, device failure, and percentage reduction in operative time.Two hundred twenty-eight cases (average age 45.3 years) met inclusion criterion and were analyzed. At a mean follow-up of 18.1 months, no patient experienced RS migration. One patient experienced a postoperative hematoma that was managed with observation. One patient developed a surgical site infection that resolved following exploration and intravenous antibiotics. The subperiosteal pocket technique resulted in an 18.9% reduction in total operative time compared to a more conventional RS placement method (P.01).The tight subperiosteal pocket without fixation is a safe, durable, and time-saving technique for RS placement during cochlear implantation. Notably, device migration and flap complications are very uncommon.

OBJECTIVE To investigate whether the use of aspirin or other non-steroidal anti-inflammatory drugs (NSAIDs) impact the growth of vestibular schwannoma (VS). STUDY DESIGN Retrospective case series. SETTING Single academic, tertiary care center. PATIENTS Patients with VS who underwent at least two magnetic resonance imaging (MRI) studies before intervention. INTERVENTION(S) Serial MRI studies. MAIN OUTCOME MEASURE(S) VS tumor growth, defined as more than or equal to 2 mm increase in the maximum tumor diameter between consecutive MRI studies, or between the first and last available study. Mean growth rate was also calculated, defined as the change in tumor size divided by length of follow-up. RESULTS A total of 564 VS patients met inclusion criteria, with 234 (41.2%) taking some type of NSAID. Aspirin use was not associated with VS tumor growth, presenting tumor diameter, or mean VS growth rate. Further, aspirin dosage did not impact growth outcomes or presenting tumor diameter. A total of 96 (17.0%) patients took an NSAID other than aspirin. Neither non-aspirin NSAID use nor degree of cyclooxygenase-2 (COX-2) selectivity, including aspirin, was significantly associated with VS tumor growth, presenting tumor diameter, or mean VS growth rate. CONCLUSIONS While previous studies have suggested a relationship between aspirin usage and VS growth, we found no significant association in our series of 564 observed VS. Furthermore, there was no apparent relationship between aspirin dosage, non-aspirin NSAID use, and COX-2 selectivity with VS growth, presenting tumor diameter at presentation, or mean VS growth rate.

Paragangliomas are rare vascular tumors of neuroectodermal tissue arising from neural crest cells. They can be found at various locations of the paraganglion system throughout the body. Tumors originating from chromaffin cells of the adrenal medulla are referred to as pheochromocytomas. Most paragangliomas are benign, but morbidity can result from either growth and compression of nearby structures or physiologic derangements secondary to abnormal secretion of catecholamines. The vast majority of head and neck paragangliomas are nonsecretory [1, 2].

Historically, the mainstay of treatment for jugular paraganglioma has been gross total resection. While this strategy has led to relatively low recurrence rates [1–5], it has often been pursued at the expense of cranial nerve function. This trade-off was especially true for larger tumors extending through the pars nervosa of the jugular foramen and those with intracranial extension. Bacciu et al. [6] recently reviewed 122 patients with Fisch class C and D jugular paraganglioma and found that 54% of patients developed one or more new cranial nerve deficits after surgery with gross total resection being achieved in 86% of cases. Similarly, the Vanderbilt Otology Group’s review of 202 patients with jugular paragangliomas found a 60% rate of new cranial nerve injuries after performing gross total resection in 90% of cases [2, 7].

Jugular paragangliomas (JP) are uncommon, slow growing, highly vascular primary neoplasms of the temporal bone that develop from chief cells located in the adventitia of the jugular bulb [1–5]. The great majority of extra-adrenal paraganglioma reside in the abdomen, while only 10% are located in the head and neck—carotid body tumors (CBT) being most common, followed by JP and vagal paraganglioma (VP). Most JP are solitary, nonsecreting, and benign, while less than 5% are associated with catecholamine release or malignant character, and up to 17% of patients display multiple paragangliomas [6]. JP exhibit a female predominance and most commonly manifest during the fourth to fifth decades of life with symptoms of unilateral pulsatile tinnitus, conductive hearing loss, and less commonly lower cranial neuropathy. Owing to the rare prevalence and insidious growth, many patients endorse a long duration of preceding symptoms and are found to have advanced disease at diagnosis [1, 2].

Purpose The purpose of the present investigation was to determine whether there are significant differences in patient/healthcare provider perceptions of patient's dizziness severity, dizziness disability/handicap, anxiety, and signs of autonomic system activation. Materials and methods This was a prospective investigation of 30 patient–provider dyads drawn as a sample of convenience from an otology clinic in a large, tertiary care, medical center. Patients completed both the Dizziness Handicap Inventory (DHI) and the Vestibular Symptom Scale (VSS) prior to vestibular function testing. Providers were instructed to complete the same measures following the patient's clinic visit from what they estimated was the patient's point of view. The two measures were analyzed for concordance and discordance. Results Patient/provider differences in DHI and VSS vertigo subscale scores were not significantly different. However, difference scores on the VSS anxiety/autonomic subscale indicated that providers significantly under-estimated patient anxiety and symptoms of autonomic system activation. Conclusions The results suggest that providers may be missing information pertinent to the role anxiety and autonomic system activation may play in patient visits for complaints of dizziness. We suggest that this problem can be mitigated by administrating to patients prior to their clinic visit a standardized measure that quantifies patient self-report dizziness, vertigo, anxiety and autonomic system arousal. Patterns of response by patients on these measures can enable providers to diagnose correctly dizziness disorders that are rooted in clinically significant anxiety either related to, or unrelated to, a history of vestibular system impairment.

Objective The majority of patients with vestibular schwannoma (VS) initially experience audiovestibular dysfunction prompting medical evaluation. Over the past several years, the authors have noticed a significant disparity in clinical presentation among patients with preexisting cognitive impairment compared with the general population. The objective of the current study is to review the clinical presentation of 5 consecutive patients with baseline cognitive delay who were ultimately diagnosed with VS. Design Retrospective review. Setting Two tertiary academic referral centers. Patients Consecutive subjects with baseline cognitive impairment who were evaluated for VS between 2010 and 2013. Main outcome measures Presenting symptoms and tumor characteristics. Results Five adult subjects were identified. The most common reasons for seeking medical care included worsening ataxia and headaches, despite the fact that all patients had profound ipsilateral hearing loss. Additionally, 2 patients endorsed facial numbness, and 2 had partial facial paresis. MRI revealed a median preoperative tumor size of 4.7 cm (4.3-5.7 cm), and all patients had varying degrees of hydrocephalus and peritumoral vasogenic edema. Conclusion Preexisting cognitive impairment is a risk factor for late presentation of VS. Patients with normal cognitive function most commonly seek medical care after unilateral hearing loss and tinnitus. In contrast, individuals with poor cognitive capacity may be unable to acknowledge such symptoms or communicate their presence. In such patients, the diagnosis of VS is elusive, and clinicians should maintain a low threshold for imaging to avoid further delays in diagnosis and treatment.

To evaluate tumor control following subtotal resection of advanced jugular paragangliomas in patients with functional lower cranial nerves and to investigate the utility of salvage radiotherapy for residual progressive disease.Case series with planned chart review.Tertiary academic referral center.Patients who presented with advanced jugular paragangliomas and functional lower cranial nerves were analyzed. Primary outcome measures included extent of resection, long-term tumor control, need for additional treatment, and postoperative lower cranial nerve function.Twelve patients (mean age, 46.2 years; 7 women, 58.3%) who met inclusion criteria were evaluated between 1999 and 2013. The mean postoperative residual tumor volume was 27.7% (range, 3.5%-75.0%) of the preoperative volume. When the residual tumor volume was less than 20% of the preoperative volume, no tumor growth occurred over an average of 44.6 months of follow-up (P.01). Four tumors (33.3%) demonstrated serial growth at a mean of 23.5 months following resection, 2 of which were treated with salvage stereotactic radiotherapy providing control through the last recorded follow-up. No patient experienced permanent postoperative lower cranial neuropathy as a result of surgery.Subtotal resection of jugular paragangliomas with preservation of the lower cranial nerves is a viable management strategy. If more than 80% of the preoperative tumor volume is resected, the residual tumor seems less likely to grow.

Objective: This study aimed to better identify factors associated with recurrence of squamous cell carcinoma (SCC) involving the temporal bone. Methods: A retrospective study was conducted at a tertiary hospital. Sixty patients who were diagnosed over a 10-year period with SCC involving the temporal bone and underwent surgical resection were analyzed. All patients were staged based on the University of Pittsburgh staging system. Demographic, intraoperative, and pathologic data were analyzed with respect to recurrence. Results: Thirteen (21.7%) patients were T1, 8 (13.3%) T2, 7 (11.7%) T3, and 32 (53.3%) T4. Eighteen patients (30.0%) recurred in the study period. The mean time to recurrence was 5.8 months. Tumors originating in the skin overlying the parotid gland and the external auditory canal had higher recurrence rates than those from the auricle/postauricular skin and temporal bone ( P = .05). Direct parotid and perineural spread accounted for 15.0% of all routes of temporal invasion but resulted in 22.2% of all recurrences ( P = .04). Increased N stage was statistically associated with increased risk of recurrence ( P = .01). Cervical, as compared to perifacial and parotid, lymph node involvement was associated with increased risk of recurrence (odds ratio = 6.91; 95% confidence interval, 1.11–42.87). Conclusion: We have identified multiple factors that are associated with increased recurrence of SCC involving the temporal bone.

To determine the risk factors for unanticipated readmission, prolonged index admission, and discharge to a facility after vestibular schwannoma surgery.Retrospective cohort study of those undergoing surgery for vestibular schwannoma in the Nationwide Readmissions Database (2013-2014). Main outcome measures included readmission rate, length of stay, discharge destination.There were 4585 cases identified. The overall unanticipated readmission rate was 8.1%, and 9.1% had a prolonged length of stay (PLOS) of ≥7 days. Mean and median LOS were 4.63 and 4.00 days, respectively, and90% of patients were discharged after 7 days. Disposition to a facility occurred in 6.7% of cases. Teaching hospitals were protective against unintended readmission (odds ratio [OR] 0.44, p .001). Major functional loss was associated with PLOS (OR 12.55, p .001). High volume centers were associated with decreased risk of PLOS (OR 0.46, p .001) and facility discharge (OR 0.68, p .001). The most common readmission diagnoses included "other nervous system complications" (n = 128), cerebrospinal fluid leak (n = 71), "other postoperative infection" (n = 61), and meningitis (n = 59).Unanticipated readmission and prolonged LOS following vestibular schwannoma surgery are common, with varied sociodemographic, hospital, and patient factors independently associated with each. Further studies are needed to investigate targeted interventions aimed at minimizing readmission and prolonged LOS using the factors outlined above.

Objective To describe a contemporary, pragmatic approach to managing cholesteatoma in the only hearing ear. Study design Retrospective case series. Setting Single tertiary referral center. Patients All patients that underwent cholesteatoma surgery, having profound hearing loss in the contralateral ear. Intervention(s) Cholesteatoma surgery. Main outcome measure(s) Surgical strategy, preoperative and postoperative audiometric outcomes, short- and long-term complications, recidivism. Results Twenty-eight patients met criteria, representing 0.25% of all chronic ear surgeries performed between 1970 and 2012. Patients undergoing surgery in the latter half of the study underwent intact canal wall procedures and ossicular chain reconstruction more frequently despite having similar severities of disease. All patients with inner ear fistula underwent an open-cavity operation. In the early postoperative period, 86% of ears had stable or improved hearing levels, and all patients maintained preoperative bone conduction thresholds. At a mean follow-up of 48 months, 79% of patients maintained stable or improved pure tone thresholds, whereas 2 subjects experienced delayed sensorineural hearing loss and 2 experienced isolated declining speech discrimination. Notably, 3 of the latter 4 patients were diagnosed with labyrinthine fistula and had undergone radical mastoidectomy. None of the patients who received an intact canal wall tympanomastoidectomy experienced worsening bone conduction thresholds, whereas 1 subject demonstrated a delayed decline in speech discrimination and another recurred. Conclusion It is commonly held that the radical or classic modified radical mastoidectomy is the procedure of choice when managing cholesteatoma in the only hearing ear while intact canal wall techniques are contraindicated. Over the last 20 years, we have adopted a less-rigid, functional approach favoring intact canal wall procedures in the absence of inner ear fistula rather than unequivocally committing to an open cavity. This strategy has been influenced by advancements in preoperative evaluation, increasing familiarity and refinement of closed-cavity techniques, postoperative imaging surveillance options, and the potential for cochlear implant "salvage" in the rare case of profound hearing loss. Based on the current series, this approach appears safe when performed by an experienced surgeon, and reliable long-term patient follow-up is maintained.

Object The goals of this study were to report the clinical presentation, radiographic findings, operative strategy, and outcomes among patients with temporal bone encephaloceles and cerebrospinal fluid fistulas (CSFFs) and to identify clinical variables associated with surgical outcome. Methods A retrospective case series including all patients who underwent a middle fossa craniotomy or combined mastoid–middle cranial fossa repair of encephalocele and/or CSFF between 2000 and 2012 was accrued from 2 tertiary academic referral centers. Results Eighty-nine consecutive surgeries (86 patients, 59.3% women) were included. The mean age at time of surgery was 52.3 years, and the left side was affected in 53.9% of cases. The mean delay between symptom onset and diagnosis was 35.4 months, and the most common presenting symptoms were hearing loss (92.1%) and persistent ipsilateral otorrhea (73.0%). Few reported a history of intracranial infection (6.7%) or seizures (2.2%). Thirteen (14.6%) of 89 cases had a history of major head trauma, 23 (25.8%) were associated with chronic ear disease without prior operation, 17 (19.1%) occurred following tympanomastoidectomy, and 1 (1.1%) developed in a patient with a cerebral aqueduct cyst resulting in obstructive hydrocephalus. The remaining 35 cases (39.3%) were considered spontaneous. Among all patients, the mean body mass index (BMI) was 35.3 kg/m2, and 46.4% exhibited empty sella syndrome. Patients with spontaneous lesions were statistically significantly older (p = 0.007) and were more commonly female (p = 0.048) compared with those with nonspontaneous pathology. Additionally, those with spontaneous lesions had a greater BMI than those with nonspontaneous disease (p = 0.102), although this difference did not achieve statistical significance. Thirty-two surgeries (36.0%) involved a middle fossa craniotomy alone, whereas 57 (64.0%) involved a combined mastoid–middle fossa repair. There were 7 recurrences (7.9%); 2 patients with recurrence developed meningitis. The use of artificial titanium mesh was statistically associated with the development of recurrent CSFF (p = 0.004), postoperative wound infection (p = 0.039), and meningitis (p = 0.014). Also notable, 6 of the 7 cases with recurrence had evidence of intracranial hypertension. When the 11 cases that involved using titanium mesh were excluded, 96.2% of patients whose lesions were reconstructed with an autologous multilayer repair had neither recurrent CSFF nor meningitis at the last follow-up. Conclusions Patients with temporal bone encephalocele and CSFF commonly present with persistent otorrhea and conductive hearing loss mimicking chronic middle ear disease, which likely contributes to a delay in diagnosis. There is a high prevalence of obesity among this patient population, which may play a role in the pathogenesis of primary and recurrent disease. A middle fossa craniotomy or a combined mastoid–middle fossa approach incorporating a multilayer autologous tissue technique is a safe and reliable method of repair that may be particularly useful for large or multifocal defects. Defect reconstruction using artificial titanium mesh should generally be avoided given increased risks of recurrence and postoperative meningitis.

OBJECTIVE To report the clinical presentation, management strategies, and outcomes of 14 endolymphatic sac tumors (EST). STUDY DESIGN Retrospective case series. SETTING Collective experience accrued from 2 tertiary referral centers. PATIENTS All patients with ESTs. INTERVENTION Microsurgery, stereotactic radiosurgery (SRS). MAIN OUTCOME MEASURE(S) Treatment-related morbidity, recurrence. RESULTS Fourteen ESTs (13 patients) met inclusion criteria. Eight tumors were sporadic, and 6 were associated with von Hippel-Lindau disease (VHL). Twelve lesions were managed primarily, whereas 2 were referred for treatment of sizable recurrences after subtotal resection. Including all patients, the median age at diagnosis was 36 years, and the median duration of posttreatment follow-up was 78 months. Among primary tumors, the median delay between symptom onset and diagnosis was 36 months, and the median tumor diameter at presentation was 23 mm with most lesions demonstrating intracranial involvement. Subjects with VHL frequently presented with smaller, less extensive tumors and were more commonly female compared with patients with sporadic disease. Of the 9 ESTs presenting with normal facial nerve function, 8 maintained good (HB 1-2) posttreatment capacity. Two of 5 ears with useful preoperative hearing maintained pretreatment hearing levels after surgery. One of 12 tumors managed primarily recurred after microsurgical resection. Primary SRS was used in 1 medically infirm patient providing durable tumor control (94 mo) at last follow-up. Among recurrent tumors, 2 were managed successfully with reexcision, whereas 1 underwent salvage surgery followed by 2 sequential treatments of SRS for 2 separate intracranial recurrences; no in-field recurrence has been detected to date. CONCLUSION ESTs are rare primary neoplasms of the temporal bone that may occur sporadically or in association with VHL. Outcomes after treatment of small less-extensive tumors are favorable compared with patients with advanced primary or recurrent disease. Furthermore, subtotal resection carries a high risk of bulky or multifocal recurrence. As such, early gross total resection remains the management strategy of choice for primary and recurrent ESTs; however, SRS should be considered in poor surgical candidates or in cases of focal intracranial recurrence when the morbidity of salvage surgery is high.