Hearing outcomes following reimplantation of functional legacy cochlear implants.

OBJECTIVES: Recently, patients with certain legacy cochlear implants (CIs) have sought out reimplantation to enjoy the benefits offered by newer processor technology. This decision can be difficult, especially when the individual relies exclusively on the device for communication and scores at the ceiling of performance metrics. To date, most outcome data is derived from reimplantation of a non-functioning CI-a relatively easy decision. The aim of this study is to report hearing outcomes following reimplantation of legacy implants to guide surgeons and patients approaching this high-stakes clinical situation. PATIENTS AND INTERVENTION: Four patients implanted with Advanced Bionics Clarion C1 devices over 20 years ago underwent reimplantation. RESULTS: Three reimplanted patients demonstrated a maintenance or improvement in their audiometric performance with one patient experiencing only a 5 % decrease in AzBioQ score. Each patient expressed satisfaction with the expansion of technological capabilities including improved battery life, and device connectivity. There were no failed reimplantations or other adverse effects. CONCLUSIONS: Reimplantation of a functioning legacy CI result in stability or improvement in auditory performance. All individuals in this series report that they enjoy the new connectivity and programming technologies. As the rate advancement in CI technology continues to increase and newer device architectures emerge, these data will help to inform the decision to reimplant functioning devices.

Staged implantation of active transcutaneous bone conduction hearing devices (BCHD) after explantation of older generation bone anchored hearing aids (BAHAs): Surgical outcomes and approach to management.

PURPOSE: To evaluate outcomes following explantation of percutaneous or transcutaneous bone conduction implants (pBCIs or tBCIs) and subsequent implantation of transcutaneous active bone conduction hearing devices (BCHDs); to provide guidance regarding staging of surgery and adjunctive procedures. MATERIALS AND METHODS: Retrospective chart review of eight adult subjects (ten ears) with pBCIs or tBCIs who underwent explantation of their device and subsequent implantation with a BCHD [MED-EL BONEBRIDGE™ (n = 7, 70 %) or Cochlear™ Osia® (n = 3, 30 %)]. RESULTS: Reasons for pBCI or tBCI explantation were pain (60 %, 6/10), infection (60 %, 6/10), skin overgrowth (50 %, 5/10), and inability to obtain new processors (20 %, 2/10). Median time between pBCI or tBCI removal and BCHD staged implant was 4.7 (IQR 2.2-8.1) months. Two subjects developed complications following BCHD implantation. One had a persistent wound overlying the osseointegrated screw after removal of the pBCI abutment, requiring removal and temporalis rotational flap. Staged Osia® implantation was performed, but ultimately wound dehiscence developed over the device. The second subject experienced an infection after BONEBRIDGE™ implantation (32 days after pBCI explant), necessitating washout and treatment with intravenous antibiotics. There was subsequent device failure. CONCLUSION: The transition from a pBCI or tBCI to a novel transcutaneous device is nuanced. Staged pBCI or tBCI explantation and novel BCHD implantation with sufficient time for wound healing is vital. Adjunctive procedures to augment soft tissue in cases of prior attenuation may be required to avoid complications with larger internal devices.

Comparing Approaches for Repair of Superior Semicircular Canal Dehiscence.

OBJECTIVE: Compare outcomes for subjects who underwent middle cranial fossa (MCF) or transmastoid (TM) repair of superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective cohort study. SETTING: Quaternary-care, academic neurotology practice. METHODS: Subjects who underwent MCF or TM repair of SSCD between December 1999 and April 2023 were identified. Main outcome measures included demographic data, length of surgery and hospital stay, clinical presentation, and audiometric testing. RESULTS: Ninety-three subjects (97 ears) who underwent surgery for SSCD met inclusion criteria: 58.8% (57) via MCF, 39.2% (38) via TM, and 2.0% (2) via TM + MCF. Median operative time was shorter for the TM (35) compared to the MCF (29) approach (118 vs 151 minutes, P < .001). Additionally, median hospital stays were shorter for TM (36) compared to the MCF (56) approach (15.3 vs 67.7 hours, P < .001). Overall, 92% (49/53) of MCF and 92% (33/36) of TM surgeries resulted in an improvement or resolution of one or more symptoms (P = .84). There was no significant preoperative to postoperative change in the median air conduction pure-tone average (PTA), air-bone gap, or word recognition score in both the MCF and TM groups (P > .05). Improvements of >10 dB in the pre- to postoperative absolute change in bone conduction PTA were noted in 3 subjects in the MCF group and 4 subjects in the TM group (P = .49). CONCLUSION: The TM approach for SSCD demonstrates shorter operative times and length of hospital stay. The TM and MCF approaches have comparable audiometric and clinical outcomes.

The Natural History of Observed SDHx -Related Head and Neck Paragangliomas Using Three-Dimensional Volumetric Tumor Analysis.

OBJECTIVE: Characterize the natural history and clinical behavior of head and neck paragangliomas (HNPGLs) in subjects with succinate dehydrogenase ( SDHx ) pathogenic variants using volumetric tumor measurements. STUDY DESIGN: Cohort study. SETTING: Tertiary academic referral center. PATIENTS: Subjects with SDHx HNPGLs under observation for at least 6 months with 2 or more magnetic resonance imaging or computed tomography scans. INTERVENTIONS: Diagnostic interventions include next-generation sequencing, magnetic resonance imaging, and computed tomography. Therapeutic interventions include microsurgical resection or stereotactic radiosurgery. MAIN OUTCOME MEASURES: Radiographic progression was defined as a 20% or greater increase in volume. Cranial nerve (CN) functional outcomes were assessed using clinical documentation. RESULTS: A total of 19 subjects with 32 tumors met the inclusion criteria. Median radiographic follow-up was 2.2 years, and the median volumetric growth rate was 0.47 cm 3 /yr. Kaplan-Meier estimated rates of survival free of radiographic progression for all SDHx tumors at 1, 2, and 3 years were 69, 50, and 22%, respectively. No tumors developed new CN palsies during the period of observation. CONCLUSIONS: Over intermediate-term follow-up, observation of treatment-naive SDHx -related HNPGLs did not result in new cranial neuropathy. Although indefinite observation is only appropriate for select cases, these data support an interval of observation to characterize growth rate in asymptomatic to minimally symptomatic patients, who are at high risk of treatment-related morbidity. Given the early age at diagnosis and high risk of bilateral multifocal phenotypes in SDHx HNPGL mutation carriers, these data may aid in optimizing patient tumor control and CN functional preservation. Further studies are necessary to determine whether pretreatment growth rate is correlated with clinical outcomes.

Clinical Presentation of Pediatric Recurrent Croup: Implications for Diagnosis.

Recurrent croup is a common clinical entity afflicting the pediatric population, but is not well-characterized in the literature. We describe the largest series of recurrent croup in North America from a single tertiary care academic center, and differentiate subpopulations based on demographic characteristics and clinical presentation. We identified 114 patients for inclusion. Common symptoms included barky cough (86.0%) and inspiratory stridor (60.5%). Many (26.3%) experienced full symptom resolution within a day, and 41.2% reported onset of symptoms at any time of the day. Male patients had a higher number of prior croup episodes at presentation compared with female patients (mean of 9.8 ± 8.0 in males vs 6.6 ± 4.8 in females, P = .03). On multivariate regression, racial/ethnic minority patients were at higher risk for dyspnea (odds ratio [OR]: 58.6; 95% confidence interval [CI]: 7.2-475.4) and upper respiratory infection prodrome (OR: 7.6; 95% CI: 1.6-35.3) compared with non-Hispanic white patients.

The impact of a fast-track questionnaire in pediatric paradoxical vocal fold motion disorder (PVFMD).

OBJECTIVE: PVFMD is a frequent cause of dyspnea in the healthy adolescent. When PVFMD is suspected, the current standard of care includes referral to an otolaryngologist (ENT) prior to beginning laryngeal control therapy (LCT) with a speech language pathologist (SLP). We hypothesize that a "fast-track" screening questionnaire will improve time to treatment and decrease patient charges. METHODS: Patients (n = 258, group 1) who received traditional referral and were evaluated in pediatric voice clinic with a diagnosis of PVFMD between 11/2013 and 11/2017 were identified and compared with 66 patients (group 2) from 10/2018 to 11/2019 who were prospectively studied and placed into a fast-tracked subgroup for LCT without preceding ENT evaluation if they scored 8/10 or higher on a designed screening questionnaire. RESULTS: Female gender (group 1: 81%, group 2: 83%, p = 0.73) and median age (group 1:14 years IQR 4; group 2:14 years IQR 3, p = 0.83) were similar. The median duration from symptom onset to LCT was shorter for group 2 (group 1: 12 months, IQR 18; group 2: 8.5 months, IQR 8)(p = 0.02). Time from referral to LCT was shorter for group 2 at 3 weeks (IQR 3) compared to group 1 at 4 weeks (IQR 3.5, p < 0.01). The minimum single patient charge for group 1 was estimated at $5123 and $1649 for group 2, yielding a potential reduction of charges of over $3000. CONCLUSION: Using a fast-track screening questionnaire for pediatric PVFMD patients significantly decreases the time to treatment without altering the response rate of LCT.

Association of Self-Reported Coping Strategies With Speech Recognition Outcomes in Adult Cochlear Implant Users.

OBJECTIVE: To characterize the degree to which individual coping strategies may influence speech perception after cochlear implantation. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Adult, postlingually deaf cochlear implant recipients. INTERVENTIONS: The Coping Orientation to Problems Experience inventory, a validated, multidimensional self-reported coping scale, was administered preoperatively. MAIN OUTCOME MEASURES: Speech perception was measured using consonant-nucleus-consonant (CNC) phoneme and word scores, AzBio sentence accuracy in quiet and noise, and Hearing in Noise Test sentences in quiet preoperatively and at 1, 3, and 6 months postoperatively. Quality of life was measured with the Hearing Implant Sound Quality Index and the Nijmegen Cochlear Implant Questionnaire. RESULTS: Thirty-six patients were included in this study. Mean age at surgery was 70.7 ± 11.4 years. Acceptance was associated with a decreased AzBio in noise score in the 6 months after CI (regression coefficient b = -0.05; 95% confidence interval [CI], -0.07 to -0.03; p < 0.01). Denial was associated with a decreased AzBio in quiet score (b = -0.05; 95% CI, -0.09 to -0.01; p < 0.05), whereas humor was associated with an increased AzBio in quiet score (b = 0.02; 95% CI, 0.01 to 0.04; p < 0.05). Humor was also associated with an increased Hearing in Noise Test score (b = 0.05; 95% CI, 0.02 to 0.07; p < 0.05). Denial was associated with decreased CNC word (b = -0.04; 95% CI, -0.06 to -0.02; p < 0.01) and phoneme (b = -0.04; 95% CI, -0.07 to -0.02; p < 0.01) scores, whereas substance use was associated with increased CNC word (b = 0.03; 95% CI, 0.01 to 0.05, p < 0.01) and phoneme (b = 0.04; 95% CI, 0.02 to 0.06; p < 0.01) scores. Scores on self-reported quality of life measures were not significantly correlated with coping strategies. CONCLUSION: A variety of adaptive and maladaptive coping strategies are used by postlingually deaf adult cochlear implant users. Denial and acceptance may be more predictive of poor speech performance, whereas humor and substance use may be more predictive of improved speech performance.

Association of Pepsin With Inflammatory Signaling and Effusion Viscosity in Pediatric Otitis Media.

OBJECTIVE: Otitis media (OM) is a common inflammatory disease spectrum. Cytokine signaling, neutrophil activity, and mucin hypersecretion during recurrent and chronic OM contribute to persistent, viscous middle ear (ME) effusions, hearing loss, and potential for developmental delay. Extraesophageal reflux (EER), specifically pepsin, triggers inflammatory signaling in respiratory mucosa and is associated with OM. The objective of this study was to investigate the association of pepsin with ME inflammatory signaling and the outcomes and examine causality in vitro. STUDY DESIGN: Cross-sectional study. METHODS: ME fluid (MEF) and preoperative audiometric data were collected from 30 pediatric subjects undergoing tympanostomy tube placement for recurrent OM or OM with effusion. MEF viscosity was characterized by the surgeon. Pepsin, inflammatory molecules, and mucin were assayed by enzyme-linked immunosorbent assay (ELISA). ME epithelial primary culture was exposed to 0.1 to 1 mg/ml pepsin at pH 5, 6, and 7 for 30 minutes, and cytokine expression was assayed via qPCR. RESULTS: Pepsin was observed in the MEF of 77% of patients (range 71-2,734 ng/ml). Pepsin correlated with effusion viscosity, interleukins -6 and -8, neutrophil elastase, and mucin 5B (P < .05). Pepsin-negative MEF was more frequently absent of interleukin 8 or mucin 5B (P < .05). Weak acid was generally insufficient to elicit cytokine expression in ME cells in vitro, however, pepsin induced IL6, IL8, and TNF at pH 7 (P < .05) and weak acid (pH 6) facilitated a response at lower pepsin concentration. CONCLUSIONS: Pepsin may contribute to inflammatory signaling, persistent viscous effusion, and poorer OM outcomes. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:470-477, 2022.

Analysis of Inflammatory Signaling in Human Middle Ear Cell Culture Models of Pediatric Otitis Media.

OBJECTIVES/HYPOTHESIS: Cell culture models are valuable tools for investigation of the molecular pathogenesis of diseases including otitis media (OM). Previous study indicates that age-, sex-, and race-associated differences in molecular signaling may impact disease pathophysiology. Currently, a singular immortalized middle ear epithelial (MEE) cell line exists, HMEEC-1, derived from an adult without known middle ear disease. In this study, HMEEC-1 and primary MEE cultures from pediatric patients with and without OM were stimulated with inflammatory cytokines or OM-pathogenic bacterial lysates to examine differences in the response of molecules associated with OM pathogenesis. STUDY DESIGN: Case-control series. METHODS: MEE cultures were established from patients aged <6 years: two with recurrent OM (ROM), two with OM with effusion (OME), and one patient without OM who was undergoing cochlear implant surgery control undergoing cochlear implantation (Peds CI). Primary MEE cultures and HMEEC-1 cells were stimulated with tumor necrosis factor-α, interleukin (IL)-1ß, or nontypeable Haemophilus influenzae lysate. TNFA, IL1B, IL6, IL8, IL10, and MUC5B were assayed via quantitative polymerase chain reaction. IL-8 was assayed by enzyme-linked immunosorbent assay. RESULTS: Gene/protein target expressions were frequently higher in pediatric OM lines than in HMEEC-1 and Peds CI. HMEEC-1 cells were frequently less responsive to stimuli than all pediatric lines. OME lines were often more responsive than ROM lines. CONCLUSIONS: OM may be associated with specific molecular phenotypes that are retained in primary cell culture. Adult-derived HMEEC-1 cells differ significantly in baseline expression and response of OM-associated molecules relative to pediatric MEE cells. Work is underway to immortalize pediatric OM MEE cultures as improved tools for the OM research community. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:410-416, 2021.

The direct laryngoscopy and rigid bronchoscopy findings and the subsequent management of infants with failed extubations.

INTRODUCTION: There is limited data regarding the role of direct laryngoscopy and rigid bronchoscopy (DLRB) in infants with failed extubations. Pediatric otolaryngologists are frequently consulted to perform DLRB in infants with failed extubations. OBJECTIVE: To determine the DLRB findings in infants with failed extubations and the interventions performed based on these findings. METHODS: A retrospective chart review was performed on infants less than 12 months old undergoing DLRB for failed extubations from January 2013-June 2017 at a tertiary care children's hospital. Data was collected on age, birth weight, perinatal complications, comorbid conditions, number of failed extubations, length of most recent intubation, operative findings, and subsequent interventions, including tracheostomy. Descriptive and comparative analyses were performed. RESULTS: Of the 62 subjects who met study criteria, median age at DLRB was 3.0 months, corrected age was 1.0 months, gestational age was 27.1 weeks, birth weight was 0.97 kg, and number of failed extubations was 2.0. About 80% had respiratory distress at birth requiring intubation, and 76% carried a diagnosis of bronchopulmonary dysplasia (BPD). The median number of days intubated prior to DLRB was 27. Twenty-seven percent of subjects had no significant abnormal findings on DLRB, and 26% had subglottic stenosis. The majority (74%) underwent tracheostomy. Eighteen percent of subjects had an initial intervention for abnormal DLRB finding(s) other than tracheostomy and were able to avoid tracheostomy as a future intervention. Tracheostomy placement was associated with a diagnosis of BPD (RR 1.78, 95% CI 1.02, 3.10), having a birth weight less than 0.71 kg (RR 1.45, CI 1.01, 2.10), and being intubated for 48 or more days prior to DLRB (RR 1.57, 95% CI 1.05, 2.36); it was not associated with the number of failed extubations prior to DLRB. CONCLUSIONS: Infants with failed extubations commonly had abnormal findings on airway evaluation by DLRB. Most children in this population still required tracheostomy placement, but about 20% were able to have an alternate intervention and avoid tracheostomy.

Significance of positive tracheal cultures in the 30 days following tracheostomy.

INTRODUCTION: Positive tracheal cultures obtained after tracheostomy are often considered organ/space surgical site infections by the National Surgical Quality Improvement Project. However, the definition of bacterial tracheitis after tracheostomy is not well described. OBJECTIVE: To determine the relationship of positive tracheal cultures in the 30 days following pediatric tracheostomy, antibiotic treatment of these cultures, and signs/symptoms of respiratory infection. METHODS: A retrospective chart review was performed on subjects who underwent tracheostomy from November 2012-September 2017 at a tertiary care pediatric center. The following data was studied: positive tracheal cultures, antibiotic treatment for positive cultures, and other signs/symptoms of infection including fever and elevated white blood cell count. Descriptive data analysis was performed, and relative risk and 95% confidence intervals were calculated. Multivariate logistic regression model was used to assess independent association when applicable. RESULTS: There were 173 subjects who met study criteria. Median age at tracheostomy was 4.6 months. Fifty-one percent (89/173) of subjects had at least one positive tracheal culture in the 30 days following tracheostomy. Of those subjects, 38% (34/89) had fever, 71% (32/45) had an elevated white blood cell count, 31% (22/72) had consolidation on chest imaging, 61% (54/89) had increased tracheal secretions, 70% (62/89) had increased ventilation requirements, and 60% (53/89) were treated with antibiotics for a diagnosis of tracheitis. There was no meaningful difference when comparing fever, increased white blood cell count, lung consolidation, increased tracheal secretions, or increased ventilation requirements in those with and without a positive tracheal culture or in those with and without antibiotic treatment for a positive culture. Multivariate logistic regression analysis showed that increased age at time of tracheostomy, more days on the ventilator after tracheostomy, and an increased number of positive cultures in the year after tracheostomy were related to having a positive tracheal culture within 30 days of tracheostomy. CONCLUSION: For post-operative pediatric tracheostomy subjects, there were no meaningful differences when comparing signs/symptoms of infection between those with and without a positive tracheal culture and between those with and without antibiotic treatment for a positive culture.

Primary tracheocutaneous fistula closure with immediate transition to nocturnal noninvasive positive pressure ventilation in two children with Congenital Central Hypoventilation Syndrome.

Transitioning children with Congenital Central Hypoventilation Syndrome (CCHS) from nocturnal invasive ventilation via tracheostomy to noninvasive positive pressure ventilation (NIPPV) is challenging due to the leak caused by the tracheocutaneous fistula (TCF), resulting in insufficient ventilation. Decannulation and primary closure of the TCF with immediate transition to nocturnal NIPPV was performed in two children with CCHS at a tertiary care children's hospital. Neither child developed significant adverse effects such as pneumomediastinum or pneumothorax. This technique is a novel approach that may improve decannulation outcomes and aid transition to NIPPV in this patient population.

Management trends of infantile hemangioma: A national perspective.

INTRODUCTION: The primary management of infantile hemangioma (IH) has changed since 2008, with the initiation of propranolol. The change that propranolol has affected on resource utilization is unknown. MATERIALS AND METHODS: The Kids' Inpatient Database (KID) in 2003, 2006, 2009, and 2012 was queried for ICD-9 codes for IH in children under age three. The number of patients undergoing the following procedures of interest: tracheostomy, tracheoscopy and laryngoscopy with biopsy, and excision of skin lesion were evaluated. Data was analyzed for demographics and details on the admission. Trends were identified. Weighted statistical analyses were performed with SAS 9.4. RESULTS: The number of qualified admissions significantly increased over the years (9271 in 2003-12029 in 2012, OR 1.042 per year increase, p < 0.001). The mean age at admission ranged from 26 to 28 days but did not vary over time (p = 0.54). The percentage undergoing tracheostomy significantly decreased from 1.05% in 2003 to 0.27% in 2012 (p = 0.0055), and the percentage undergoing tracheoscopy and laryngoscopy with biopsy significantly decreased from 7.29% in 2003 to 4.20% in 2012 (p = 0.011) among those with IH of unspecified or other sites. The percentage undergoing skin lesion excision also significantly decreased from 1.87% in 2003 to 1.03%, in 2012 (p = 0.0038) among those with IH of skin and subcutaneous tissue. These findings suggest a potential impact of propranolol. After adjusting for inflation, the total hospital charges increased from a mean of $17,838 in 2003 to an adjusted mean of $41,306 in 2012 (p < 0.0001). CONCLUSIONS: Total admissions and hospital charges in children with IH has increased from 2003 to 2012. The percentage of patients undergoing tracheostomy, tracheoscopy and laryngoscopy with biopsy, and skin lesion excision significantly decreased in 2012 compared to 2003, suggesting a potential impact of propranolol. Further studies are needed to examine these changes more closely.

NIH Funding within Otolaryngology: 2005-2014.

Objective Analyze grants awarded between 2005 and 2014 to otolaryngology departments that appear in the National Institutes of Health (NIH) RePORTER database, summarize characteristics of grant recipients associated with otolaryngology departments as listed in the RePORTER between 2005 and 2014, and identify trends in otolaryngology NIH funding between 2005 and 2014 by topic. Study Design Case series. Setting NIH database inquiry. Subjects Grant recipients. Methods The RePORTER was queried for all grants awarded to otolaryngology departments between 2005 and 2014. All grants classified as new, renewal, or revision were included while duplicates were excluded. Results In total, 475 grants to 51 institutions were categorized by topic and subtopic. Internet searches were conducted for characteristics of 352 principal investigators. Sixty-seven percent of awardees had a PhD, 22% had an MD, and 11% had an MD/PhD. Sex ratios varied by degrees held. Although 31% of all grant recipients were women, this ratio was not seen when recipients were classified by degree type, with 78% of women holding a PhD compared with 55% of men ( P = .0013). Of the award types, 39% were R01s, 15% were R21s, and 10% were R03s. The top 3 represented topics were otology/neurotology (52%), audiology (25%), and head and neck surgery (14%). The mean annual award amount, after adjusting for inflation to 2014 dollars, was $226,495.76, with 72.8% awarded by the National Institute of Deafness and Communication Disorders. Twenty percent of awardees received multiple grants. Conclusion NIH funding in otolaryngology tends to be awarded to those with PhDs studying the hearing sciences, with 1 in 5 having multiple awards. As in other areas of NIH funding, women are underrepresented overall.

Implementation of a Sustainable Ventilator-Associated Pneumonia Prevention Protocol in a Pediatric Intensive Care Unit in Managua, Nicaragua.

BACKGROUND: Ventilator-associated pneumonia (VAP) is a common nosocomial infection in pediatric intensive care units (ICUs). Ventilator-associated pneumonia protocols decrease the incidence of VAP; however, many components of these protocols are not feasible in all settings. This study was done in a large pediatric hospital in Nicaragua. OBJECTIVE: The aim of this study is to implement a sustainable evidence-based VAP protocol, in a different culture, for the purpose of decreasing VAP rates. METHODS: This quality improvement study used a bidirectional cohort design with the retrospective group as the control and the prospective group as the experimental population. A daily checklist monitored compliance to the implemented protocol in the prospective group. A 2-sided Fisher exact test compared the differences in VAP rates between the 2 populations. RESULTS: During the 90-day implementation period, 123 ventilated patients in 3 separate ICU wings were evaluated, with 99 included in the final analysis. These data for 2014 were compared with the VAP rates recorded for the same time period in 2013. The highest adherence to the protocol was demonstrated by ICU wing 1, with a 90% decrease in VAP rates. No statistical difference in VAP rates was demonstrated by ICU 2, and ICU 3 demonstrated an increase in both patient acuity and VAP rates. DISCUSSION: Implementation of a sustainable VAP protocol in a pediatric ICU in Nicaragua can reduce the incidence of VAP. Multiple barriers and challenges associated with implementation in a resource-constrained environment are discussed.

Facial nerve stimulation associated with cochlear implant use following temporal bone fractures.

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.

Cranial base manifestations of neurosarcoidosis: a review of 305 patients.

OBJECTIVE: Neurosarcoidosis is a rare granulomatous disease that can result in cranial neuropathy, chronic meningitis, and intracranial granuloma formation. Meningeal involvement may cause focal nodular enhancement that can simulate common cranial base tumors. The objective of the current study is to further define the clinical features of neurosarcoidosis in a large cohort of patients, focusing on characteristics relevant to the skull base surgeon. STUDY DESIGN: Retrospective series. SETTING: Two tertiary academic referral centers. PATIENTS: Consecutive patients diagnosed with neurosarcoidosis. INTERVENTION(S): Review of clinical presentation, physical examination, radiologic findings, biopsy results, and laboratory testing. MAIN OUTCOME MEASURES: Prevalence and distribution of cranial neuropathy, radiologic features of meningeal enhancement, and patterns of simulated tumors. RESULTS: A total of 305 patients met study criteria. The mean age at diagnosis was 47 years and 53% were female. The optic nerve was the most commonly involved cranial nerve, followed by the trigeminal and the facial nerve. Meningeal enhancement was present in 67% of cases with 17% demonstrating focal or multicentric nodular enhancement simulating tumor. The most common locations of inflammatory tumor development included the cavernous sinus, petrous temporal bone, and sphenoid wing; six patients had bilateral internal auditory canal lesions, several mimicking neurofibromatosis type II. CONCLUSION: Establishing the diagnosis of neurosarcoidosis remains challenging. Meningeal involvement and cranial neuropathy often mimic other more common conditions. Careful review of patient history and clinical imaging can reveal important clues toward the diagnosis of neurosarcoidosis. The clinician must maintain a high index of suspicion in patients with atypical presentation to avoid misdiagnosis and facilitate early medical treatment.