Management of a Unique Sinonasal Undifferentiated Carcinoma Subtype in the Era of SARS-CoV-2.

The novel coronavirus (SARS-CoV-2) pandemic has influenced the timeliness of care for patients with both common and rare conditions, particularly those affecting high-risk operative sites such as the upper aerodigestive tract. Sinonasal undifferentiated carcinoma (SNUC) represents a rare malignancy of the sinonasal tract, a unique subset of which has never been previously reported in the otolaryngology literature and is characterized by inactivation of the SMARCB (INI-1) tumor suppressor gene. This subtype exhibits a particularly poor prognosis and is characterized pathologically by its rhabdoid appearance. Here we present the case of an individual who was diagnosed with a sinonasal mass during the SARS-CoV-2 pandemic, which was ultimately found to be SMARCB (INI-1)-deficient sinonasal carcinoma. Advanced imaging was deferred in the interest of limiting the patient's exposure to the virus, and expedited operative management was performed which facilitated prompt referral for adjuvant chemoradiation. The SARS-CoV-2 pandemic presents unique challenges, but the work-up of high-risk lesions must be prioritized; this continues to be paramount as SARS-CoV-2 resurges in many cities across the USA.

Predictors of Nodal Metastasis in Mucoepidermoid Carcinoma of the Oral Cavity and Oropharynx.

INTRODUCTION: Mucoepidermoid carcinoma (MEC) of the upper aerodigestive tract (UADT) is an uncommon malignancy, with limited literature available on its clinical and pathologic characteristics. Here, we describe the behavior of MEC of the UADT including pathologic characteristics and predictors of nodal metastasis. METHODS: Retrospective cohort study of patients with MEC of the UADT treated at an academic medical center from January 2008 to May 2018. Data was collected about demographics and tumor characteristics including clinical and histological data. The two-tailed Student t test and χ2 analysis were performed to assess for predictors of nodal metastasis. RESULTS: We identified 44 patients with minor salivary gland MEC of the oral cavity (OC) and oropharynx (OP). All patients were treated with primary site surgery. The primary site was the OC in 25 patients (57%) and OP in 19 (43%). Low-grade histology was seen in 27 specimens (61.4%), intermediate histology in 9 specimens (20.5%), and high-grade histology in 8 specimens (18.2%). Perineural invasion was noted in 10 specimens (22.7%). Neck dissection was performed in 17 patients (39%), with pathologically positive nodes found in 9 (20.5%). Notably, 5 of the 9 positive nodal specimens were found in clinically node-negative necks. Pathologically positive cervical lymph nodes were significantly associated with the OP as the primary site (p = 0.0005), perineural invasion (p = 0.012), lymphovascular invasion (p < 0.001), and high-grade histology (p = 0.004) in the primary specimen. DISCUSSION: MEC of the UADT is an uncommon malignancy. Our findings suggest elective neck dissection should be considered with perineural and lymphovascular invasion, high-grade tumor, and the OP as the primary site.

Vestibulotoxicity in a patient without renal failure after inhaled tobramycin.

Aminoglycoside antibiotics have a long history of use in the control of gram-negative bacterial infections, but their systemic use has been complicated by known ototoxicity and nephrotoxicity. Because of the utility of these medications in patients with frequent pulmonary infections, there has been a move towards the use of inhaled agents, in particular tobramycin, due to a lower rate of systemic complications. Inhaled tobramycin is generally consider to be safe from otologic complications, with only two previous reports of ototoxicity, both in patients who had underlying chronic renal disease. Here we present the first case of a patient developing isolated vestibular toxicity, without associated hearing loss or evidence of renal insufficiency, in a patient receiving inhaled tobramycin. This is an extremely rare complication of an inhaled aminoglycoside and underscores the importance of careful monitoring despite perceived safety.

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

Conditional Deletion of Prnp Rescues Behavioral and Synaptic Deficits after Disease Onset in Transgenic Alzheimer's Disease.

Biochemical and genetic evidence implicate soluble oligomeric amyloid-ß (Aßo) in triggering Alzheimer's disease (AD) pathophysiology. Moreover, constitutive deletion of the Aßo-binding cellular prion protein (PrPC) prevents development of memory deficits in APPswe/PS1ΔE9 mice, a model of familial AD. Here, we define the role of PrPC to rescue or halt established AD endophenotypes in a therapeutic disease-modifying time window after symptom onset. Deletion of Prnp at either 12 or 16 months of age fully reverses hippocampal synapse loss and completely rescues preexisting behavioral deficits by 17 months. In contrast, but consistent with a neuronal function for Aßo/PrPC signaling, plaque density, microgliosis, and astrocytosis are not altered. Degeneration of catecholaminergic neurons remains unchanged by PrPC reduction after disease onset. These results define the potential of targeting PrPC as a disease-modifying therapy for certain AD-related phenotypes after disease onset.SIGNIFICANCE STATEMENT The study presented here further elucidates our understanding of the soluble oligomeric amyloid-ß-Aßo-binding cellular prion protein (PrPC) signaling pathway in a familial form of Alzheimer's disease (AD) by implicating PrPC as a potential therapeutic target for AD. In particular, genetic deletion of Prnp rescued several familial AD (FAD)-associated phenotypes after disease onset in a mouse model of FAD. This study underscores the therapeutic potential of PrPC deletion given that patients already present symptoms at the time of diagnosis.

Opposing effects of progranulin deficiency on amyloid and tau pathologies via microglial TYROBP network.

Progranulin (PGRN) is implicated in Alzheimer's disease (AD) as well as frontotemporal lobar degeneration. Genetic studies demonstrate an association of the common GRN rs5848 variant that results in reduced PGRN levels with increased risk for AD. However, the mechanisms by which PGRN reduction from the GRN AD risk variant or mutation exacerbates AD pathophysiology remain ill defined. Here, we show that the GRN AD risk variant has no significant effects on florbetapir positron emission tomographic amyloid imaging and cerebrospinal fluid (CSF) Aß levels, whereas it is associated with increased CSF tau levels in human subjects of the Alzheimer's disease neuroimaging initiative studies. Consistent with the human data, subsequent analyses using the APPswe/PS1ΔE9 (APP/PS1) mouse model of cerebral amyloidosis show that PGRN deficiency has no exacerbating effects on Aß pathology. In contrast and unexpectedly, PGRN deficiency significantly reduces diffuse Aß plaque growth in these APP/PS1 mice. This protective effect is due, at least in part, to enhanced microglial Aß phagocytosis caused by PGRN deficiency-induced expression of TYROBP network genes (TNG) including an AD risk factor Trem2. PGRN-deficient APP/PS1 mice also exhibit less severe axonal dystrophy and partially improved behavior phenotypes. While PGRN deficiency reduces these amyloidosis-related phenotypes, other neuronal injury mechanisms are increased by loss of PGRN, revealing a multidimensional interaction of GRN with AD. For example, C1q complement deposition at synapses is enhanced in APP/PS1 mice lacking PGRN. Moreover, PGRN deficiency increases tau AT8 and AT180 pathologies in human P301L tau-expressing mice. These human and rodent data suggest that global PGRN reduction induces microglial TNG expression and increases AD risk by exacerbating neuronal injury and tau pathology, rather than by accelerating Aß pathology.

Metabotropic glutamate receptor 5 couples cellular prion protein to intracellular signalling in Alzheimer's disease.

Alzheimer's disease-related phenotypes in mice can be rescued by blockade of either cellular prion protein or metabotropic glutamate receptor 5. We sought genetic and biochemical evidence that these proteins function cooperatively as an obligate complex in the brain. We show that cellular prion protein associates via transmembrane metabotropic glutamate receptor 5 with the intracellular protein mediators Homer1b/c, calcium/calmodulin-dependent protein kinase II, and the Alzheimer's disease risk gene product protein tyrosine kinase 2 beta. Coupling of cellular prion protein to these intracellular proteins is modified by soluble amyloid-ß oligomers, by mouse brain Alzheimer's disease transgenes or by human Alzheimer's disease pathology. Amyloid-ß oligomer-triggered phosphorylation of intracellular protein mediators and impairment of synaptic plasticity in vitro requires Prnp-Grm5 genetic interaction, being absent in transheterozygous loss-of-function, but present in either single heterozygote. Importantly, genetic coupling between Prnp and Grm5 is also responsible for signalling, for survival and for synapse loss in Alzheimer's disease transgenic model mice. Thus, the interaction between metabotropic glutamate receptor 5 and cellular prion protein has a central role in Alzheimer's disease pathogenesis, and the complex is a potential target for disease-modifying intervention.

Prion-Protein-interacting Amyloid-ß Oligomers of High Molecular Weight Are Tightly Correlated with Memory Impairment in Multiple Alzheimer Mouse Models.

Alzheimer disease (AD) is characterized by amyloid-ß accumulation, with soluble oligomers (Aßo) being the most synaptotoxic. However, the multivalent and unstable nature of Aßo limits molecular characterization and hinders research reproducibility. Here, we characterized multiple Aßo forms throughout the life span of various AD mice and in post-mortem human brain. Aßo exists in several populations, where prion protein (PrP(C))-interacting Aßo is a high molecular weight Aß assembly present in multiple mice and humans with AD. Levels of PrP(C)-interacting Aßo match closely with mouse memory and are equal or superior to other Aß measures in predicting behavioral impairment. However, Aßo metrics vary considerably between mouse strains. Deleting PrP(C) expression in mice with relatively low PrP(C)-interacting Aßo (Tg2576) results in partial rescue of cognitive performance as opposed to complete recovery in animals with a high percentage of PrP(C)-interacting Aßo (APP/PSEN1). These findings highlight the relative contributions and interplay of Aßo forms in AD.

Fyn inhibition rescues established memory and synapse loss in Alzheimer mice.

OBJECTIVE: Currently no effective disease-modifying agents exist for the treatment of Alzheimer disease (AD). The Fyn tyrosine kinase is implicated in AD pathology triggered by amyloid-ß oligomers (Aßo) and propagated by Tau. Thus, Fyn inhibition may prevent or delay disease progression. Here, we sought to repurpose the Src family kinase inhibitor oncology compound, AZD0530, for AD. METHODS: The pharmacokinetics and distribution of AZD0530 were evaluated in mice. Inhibition of Aßo signaling to Fyn, Pyk2, and Glu receptors by AZD0530 was tested by brain slice assays. After AZD0530 or vehicle treatment of wild-type and AD transgenic mice, memory was assessed by Morris water maze and novel object recognition. For these cohorts, amyloid precursor protein (APP) metabolism, synaptic markers (SV2 and PSD-95), and targets of Fyn (Pyk2 and Tau) were studied by immunohistochemistry and by immunoblotting. RESULTS: AZD0530 potently inhibits Fyn and prevents both Aßo-induced Fyn signaling and downstream phosphorylation of the AD risk gene product Pyk2, and of NR2B Glu receptors in brain slices. After 4 weeks of treatment, AZD0530 dosing of APP/PS1 transgenic mice fully rescues spatial memory deficits and synaptic depletion, without altering APP or Aß metabolism. AZD0530 treatment also reduces microglial activation in APP/PS1 mice, and rescues Tau phosphorylation and deposition abnormalities in APP/PS1/Tau transgenic mice. There is no evidence of AZD0530 chronic toxicity. INTERPRETATION: Targeting Fyn can reverse memory deficits found in AD mouse models, and rescue synapse density loss characteristic of the disease. Thus, AZD0530 is a promising candidate to test as a potential therapy for AD.

Matched Cohort Study of Radiographic Superior Semicircular Canal Dehiscence and Tegmen Dehiscence and Obstructive Sleep Apnea.

OBJECTIVE: To report the frequency of radiographic superior semicircular canal dehiscence (SSCD) and tegmen dehiscence in patients with and without obstructive sleep apnea (OSA). STUDY DESIGN: Retrospective matched cohort study. SETTING: Tertiary care center. PATIENTS: Adults with OSA and fine-cut computed tomographic scans including the temporal bone were matched to patients without OSA by age, sex, and type of computed tomography (protocol, scanner type, slice thickness). Ears with otologic surgery or temporal bone tumors were excluded. MAIN OUTCOME MEASURES: Prevalence of SSCD and tegmen dehiscence assessed by two independent reviewers. RESULTS: The average body mass index of the OSA patients was 29.2 kg/m 2 with an average apnea-hypopnea index of 36.8. The control group had an average body mass index of 26.2 kg/m 2 . Of the 352 temporal bones, 34 (9.7%) had SSCD in the OSA cohort versus 37 (10.5%) in the control group ( p > 0.05). Seven OSA patients (25.6% of those with SSCD) had bilateral SSCD versus 8 controls (27.6% of those with SSCD; p > 0.05). The majority (87.3%) of dehiscences involved the temporal lobe, with the remaining involving the superior petrosal sinus or both. Of the 352 OSA ears, 90 (25.6%) had a tegmen dehiscence versus 95 (27.0%) in the control group ( p > 0.05). Neither group had a laterality preference for SSCD or tegmen dehiscence. CONCLUSION: The prevalence of radiographic SSCD and tegmen dehiscences in OSA patients does not significantly differ from age- and sex-matched controls. This is in contrast to a previous case-control study finding patients with symptomatic SSCD to have higher rates of OSA. This may suggest that the effect size of OSA on SSCD prevalence may be limited despite OSA being a risk factor for elevated intracranial pressure.

Long-Term Health Utilization and Outcomes in Chronic Suppurative Otitis Media.

OBJECTIVE: To report health utilization patterns and outcomes of medical and surgical management in patients with chronic suppurative otitis media (CSOM). STUDY DESIGN: Retrospective cohort. SETTING: Academic otology clinic. METHODS: This study included 175 patients with CSOM with a first clinic visit at our institution between March 2011 and November 2016. All patients displayed a diagnosis of CSOM by International Classification of Diseases code, had at least 1 episode of active CSOM (defined as perforation with otorrhea), and had a documented history of chronic ear infections. The mean age was 49.5 ± 1.5 years, 53% were female, and mean follow-up time was 3.5 ± 0.3 years. RESULTS: Patients had an average of 9.5 ± 0.5 otology visits, 4.7 ± 0.4 prescriptions, and 1.7 ± 0.1 surgeries, with estimated per patient cost ranging from $3927 to $20,776. Under medical management, 69% of patients displayed recurrence of disease, with a median time to recurrence of 4 months. For tympanoplasty and tympanomastoidectomy, median time to recurrence was similar at 5 and 7 years, respectively (P = .73). At the most recent visit, the prevalence of all patients with CSOM displaying moderate or worse sensorineural hearing loss (SNHL) was 41%. CONCLUSIONS: CSOM represents a major public health issue with high health care utilization and associated costs. Surgery is superior to medical therapy for achieving short- to medium-term inactive disease. Patients with CSOM display a high SNHL burden.

Lateral Skull Base Chordoma Mimicking a Paraganglioma.

We present an unusual case of chordoma arising entirely from the lateral skull base with imaging features suggestive of a paraganglioma. Clinical history, management, histopathology, and imaging characteristics are described, including a review of gallium-dotate PET scanning somatostatinreceptor-positive tumors. We further provide a review of management options, including a summary of our approach with surgical biopsy via retrosigmoid and resection via transtemporal approaches. Based on radiologic characteristics and location, lateral skull base chordoma may arise with isolated lateral skull base involvement and has the potential to be misidentified as a glomus jugulare on initial workup.

Povidone-Iodine Fails to Eradicate Chronic Suppurative Otitis Media and Demonstrates Ototoxic Risk in Mice.

HYPOTHESIS: Commercially available povidone-iodine solution can eliminate biofilms and persister cells rapidly in in vivo achievable concentrations without inducing ototoxicity. BACKGROUND: Chronic suppurative otitis media (CSOM) is a substantial global problem. Current treatment options often induce a temporary remission without leading to a permanent cessation of symptoms secondary to the treatments' inability to eliminate persister cells. Povidone-iodine has been shown to be able to clear biofilm and planktonic cells in in vitro assays, but there are reports of ototoxic effects limiting its clinical utility. METHODS: Bacterial and biofilm growth with quantification by spectrophotomer, murine auditory brainstem response (ABR), and distortion product otoacoustic emissions, immunohistochemistry, in vivo povidone-iodine treatment of murine CSOM, persister cell assay. RESULTS: Commercially available 10% povidone-iodine solution is able to completely eradicate multiple clinical strains of Pseudomonas aeruginosa and Staphylococcus aureus in vitro with 10 minutes of exposure. Mice that have received a transtympanic injection of 1% povidone-iodine solution did not have significantly different auditory brainstem response or distortion product otoacoustic emission results compared with the control. Mice that received a povidone-iodine scrub or 10% povidone-iodine solution had significantly worsened hearing (25- and 13-dB increase in threshold, respectively; p < 0.05). In vivo CSOM infection recurred in all mice after the completion of treatment with 10% povidone-iodine solution, and there was no improvement in the bacterial load after treatment, indicating in vivo failure of therapy. CONCLUSION: Povidone-iodine solution is effective at eliminating biofilm and persister cells in vitro at in vivo achievable concentrations but fails in vivo most likely because of kinetics of distribution in vivo. Even if drug distribution could be improved, the therapeutic window is likely to be too small given that the diluted solution does not have ototoxic potential, whereas while the scrub variant, which contains detergents, and the undiluted solution are ototoxic after a single treatment.

Topical Therapy Failure in Chronic Suppurative Otitis Media is Due to Persister Cells in Biofilms.

OBJECTIVE: Chronic suppurative otitis media (CSOM) is characterized by a chronically draining middle ear. CSOM is typically treated with multiple courses of antibiotics or antiseptics which are successful in achieving quiescence; however, the disease is prone to relapse. Understanding why these treatment failures occur is essential. STUDY DESIGN: The minimum inhibitory concentration (MIC), minimal biofilm eradication concentration, and the inhibitory zone were determined for ototopicals and ofloxacin for the laboratory strains and CSOM-derived isolates. The percentage of persister cells and bacterial biofilm formation were measured. Disease eradication was tested in a validated in-vivo model of CSOM after treatment with ofloxacin. SETTING: Microbiology Laboratory. METHODS: Basic science experiments were performed to measure the effectiveness of a number of compounds against CSOM bacteria in a number of distinct settings. RESULTS: The minimal biofilm eradication concentration is higher than is physiologically achievable with commercial preparations, except for povo-iodine. Clincial isolates of CSOM have equivalent biofilm-forming ability but increased proportions of persister cells. Ofloxacin can convert to inactive disease temporarily but fails to eradicate disease in an in-vivo model. CONCLUSIONS: Higher percentages of persister cells in clinical CSOM isolates are associated with resistance to ototopicals. Current ototopicals, except povo-iodine, have limited clinical effectiveness; however, it is unknown what the maximum achievable concentration is and there are ototoxicity concerns. Fluoroquinolones, while successful in producing inactive disease in the short term, have the potential to encourage antimicrobial resistance and disease recalcitrance and do not achieve a permanent remission. Given these limitations, clinicians should consider surgery earlier or use of clinically safe concentrations of povo-iodine earlier into the treatment algorithm.

Predictors of Postoperative Electrode Deactivation Among Adult Cochlear Implantees.

OBJECTIVE: To characterize postoperative electrode functionality after adult cochlear implantation; to identify rationale and risk factors for electrode deactivation. STUDY DESIGN: Retrospective Chart Review. SETTING: Academic Cochlear Implant Center. SUBJECT POPULATION: Five hundred nineteen cochlear implants in 433 adult patients over 5 years. INTERVENTIONS: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Rate of electrode deactivation after adult cochlear implantation. RESULTS: One hundred twenty (27.7%) patients experienced electrode deactivation postoperatively, involving a total of 447 electrodes. The most common reasons for deactivation were bothersome nonauditory symptoms (n = 170, 38.0%), perceived benefit by patients (n = 64, 14.3%), and bothersome auditory symptoms (n = 60, 13.4%). Four hundred nineteen (93.7%) of involved electrodes remained deactivated at most recent follow-up, whereas 28 (6.3%) were able to be reactivated. Deactivation was most likely to occur within the first 4 weeks after activation (n = 90 patients,75.0%; p < 0.01). Among affected patients, the average number of electrodes deactivated was 3.44 (range 1-13; SD 2.50). Age was not associated with electrode deactivation. CONCLUSIONS: While 98% of cochlear implants had full insertions, more than a quarter of implantees may experience electrode deactivation postoperatively for a multitude of reasons, with bothersome nonauditory symptoms most prevalent. Deactivation of five or more electrodes and simultaneous deactivation of two or three electrodes seems to increase the odds of subsequent device failure. However, deactivation encompasses a wide range of issues that likely include patient factors, surgical technique, and device-specific issues. Prognosis varies greatly at the individual level and further evaluation is required to better identify the issues underlying deactivation and identify true predictors of failure.

Bitter Taste Receptors and Chronic Otitis Media.

OBJECTIVE: To evaluate the presence of bitter taste receptors (T2Rs) in the middle ear and to examine their relationship with chronic ear infections. STUDY DESIGN: Cross-sectional study. SETTING: Tertiary care hospital. METHODS: This study enrolled 84 patients being evaluated for otologic surgery: 40 for chronic otitis media (COM) and 44 for other surgical procedures (controls). We collected a small piece of mucosa from 14 patients for mRNA analysis and from 23 patients for immunohistochemistry. A total of 55 patients underwent a double-blind taste test to gauge sensitivity to phenylthiocarbamide, denatonium, quinine, sucrose, and sodium chloride; 47 patients gave a salivary sample for single-nucleotide polymorphism analysis of rs1376251 (TAS2R50) and rs1726866 (TAS2R38). RESULTS: Bitter taste receptors were found in all samples, but the repertoire varied among patients. T2R50 was the most consistently identified receptor by mRNA analysis. Its rs1376251 allele was related to susceptibility to COM but not the expression pattern of T2R50. Ratings of bitterness intensity of phenylthiocarbamide, a ligand for T2R38, differed significantly between the COM and control groups. CONCLUSION: T2Rs were found within the middle ear of every patient sampled; the rs1376251 allele of TAS2R50 appears to be related to chronic ear infections. These receptors are an intriguing target for future research and possible drug targeting.

Impact of Reconstruction With Hydroxyapatite Bone Cement on CSF Leak Rate in Retrosigmoid Approach to Vestibular Schwannoma Resection: A Review of 196 Cases.

OBJECTIVE: To assess the impact of reconstructive technique on the incidence of cerebrospinal fluid (CSF) leak following retrosigmoid approach to acoustic neuroma resection. STUDY DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: A total of 1,200 patients with acoustic neuromas presented to our institution from 2005 to 2018. Of these, 196 patients underwent surgical resection via a retrosigmoid approach. INTERVENTION: At our institution, internal auditory canal (IAC) reconstruction following a retrosigmoid approach was performed with bone wax and muscle plug or Norian hydroxyapatite bone cement from 2005 to 2013. Starting in 2014, a newer model of bone cement, Cranios hydroxyapatite, was used exclusively for reconstruction. MAIN OUTCOME MEASURES: Rates of CSF leak were evaluated across different methods of IAC reconstruction and types of bone cement. Patients whose leaks were attributable to the craniectomy site were excluded from analysis. RESULTS: The postoperative CSF leak rate among patients who did not receive bone cement for IAC reconstruction was 15.6% (n.5). The leak rate amongst patients who received Norian bone cement was 6.3% (n.4). After introduction of Cranios bone cement, the total leak rate decreased to 1% (n.1). Compared with all other types of closure, Cranios had a significantly reduced rate of postoperative CSF leak (p < 0.005). The leak rate following Cranios versus Norian was also significantly reduced (p < 0.05). Leak rate was not affected by tumor size (p.0.30) or age (p.0.43). CONCLUSION: CSF leak rate following acoustic neuroma resection was significantly reduced by introduction of Cranios hydroxyapatite bone cement.

Lateral Wall Electrodes Increase the Rate of Postactivation Nonauditory Percepts.

OBJECTIVE: To evaluate factors influencing the development of nonauditory percepts and facial nerve stimulation after cochlear implant (CI) activation. STUDY: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Over the course of 5 years, 433 consecutive patients were evaluated for CI and 518 ears were implanted. Of those, 497 ears had information regarding CI activation. INTERVENTIONS: Lateral wall electrodes (LWE) or perimodiolar/mid-scalar electrodes (PME) were used during implantation. PRIMARY OUTCOME MEASURE: Nonauditory percepts and facial nerve stimulation after activation of CI. RESULTS: Among the 497 devices, which were activated at our institution, 357 (72%) had LWE while 140 (28%) patients had a PME. Of the patients with LWE, 49 (13.7%) patients experienced some form of nonauditory percept. In comparison, 11 (9.2%) patients with a PME had some form of nonauditory percept (p < 0.05). Among the patients who had an LWE, 33 (9.2%) patients had facial nerve stimulation compared with 6 (4.3%) patients with PME (p < 0.05). Additionally, there were 11 (2.2%) patients with incomplete insertion of the electrode who had a significant increase (p < 0.05) in facial nerve stimulation. The mean number of electrodes requiring programming modification to control symptoms was 2.9. CONCLUSIONS: The use of LWE and incomplete insertions significantly increase the rate of nonauditory percepts and FNS after activation of CIs. Otic capsule anomalies are an independent risk factor for both.

Disorders Involving a Persistent Craniopharyngeal Canal: A Case Series.

Objectives A persistent craniopharyngeal canal (CPC) is a rare embryologic remnant that presents as a well-corticated defect of the midline sphenoid body extending from the sellar floor to the nasopharynx. Our case series aims to describe three unique presentations of this congenital anomaly and their subsequent management. Design Retrospective review. Setting Tertiary academic medical center. Participants Patients who underwent endoscopic transnasal surgical repair of a CPC lesion. Main Outcome Measures Resolution of symptoms and surgical outcomes. Results A total of three patients were identified. The clinical presentation varied, however, all cases prompted further imaging which demonstrated a persistent CPC and associated pathologic lesion. The presentation of a persistent CPC with nasal obstruction and subsequent iatrogenic cerebrospinal fluid leak as in Case 1 demonstrates the importance of imaging in this work-up. Cases 2 and 3 in the series were representative of the larger subset of patients in the literature who present with the defect incidentally but still warrant surgical management. Nonetheless, a standard approach to diagnosis with preoperative imaging and subsequent transnasal endoscopic repair of the skull base defect was undertaken. Conclusion The persistent CPC is a rare congenital anomaly associated with diverse pathology and careful review of preoperative radiology is critical to the management. When warranted, subsequent surgical repair and reconstruction is associated with excellent postoperative outcomes.

Hearing and Quality of Life Over Time in Vestibular Schwannoma Patients: Observation Compared to Stereotactic Radiosurgery.

OBJECTIVE: To examine quality of life changes for patients with vestibular schwannoma (VS) undergoing observation or stereotactic radiosurgery (SRS). STUDY DESIGN: Retrospective review. SETTING: Academic medical center. PATIENTS: Patients with VS who underwent observation or SRS and had at least two audiograms and Penn Acoustic Neuroma Quality of Life (PANQOL) surveys, a quality of life survey for patients with VS. INTERVENTIONS: SRS or observation. MAIN OUTCOME MEASURES: Pure-tone average (PTA), speech discrimination score (SDS), PANQOL score; controlling for tumor size, baseline hearing, and other factors. RESULTS: One hundred twenty-three patients met inclusion criteria: 89 underwent observation and 34 SRS. There was no significant difference in the rate of decline measured by PTA (PTA worsened at a rate of 0.25 dB/yr more in the observation group compared with the SRS group, p = 0.77) and SDS (SDS worsened at a rate of 2.1%/yr more in the SRS group compared with the observation group, p = 0.82). Kaplan-Meier analysis demonstrated the SRS group had a higher probability to progress to class D hearing over observation (hazard ratio 7.1, p = 0.005). The rate of change of the SRS PANQOL scores was significantly improved in the total (p = 0.005) and hearing (p = 0.04) domain score compared with observation. However, both groups regress to a similar PANQOL total and hearing domain score over time. CONCLUSION: PANQOL scores were higher at baseline in the observation group than in the SRS group. However, over time, PANQOL scores in the observation group decreased while PANQOL scores in the SRS group increased, resulting in PANQOL scores that were equivalent by the end of follow-up.