Management of bilateral head and neck paragangliomas at a single-institution across four decades.

BACKGROUND: Bilateral head and neck paragangliomas (HNPGLs) require nuanced management to balance tumor control with functional preservation. METHODS: All patients seen at a single-institution for bilateral paraganglioma between 1983 and 2023 were retrospectively reviewed. Demographics, genetic testing results, and tumor characteristics were analyzed and compared to treatment modality and cranial nerve outcomes. RESULTS: There were 49 patients with 116 tumors (90 carotid body tumors [CBTs], 15 vagal paragangliomas [VPs], and 11 jugular paragangliomas [JPs]). Twenty-six patients had SDH pathologic variants (PV). Surgical management was more commonly utilized in younger patients (OR: 0.97, 95% CI: 0.950-0.992) and for JPs (OR: 9, 95% CI: 1.386-58.443). In surgical cases, CBTs had a lower risk of postoperative cranial nerve deficits compared to JPs and VPs (OR: 0.095, 95% CI: 0.013-0.692). CONCLUSIONS: Younger patients with bilateral HNPGLs, especially those with JP and CBT, are more often treated with surgery. CBTs have lowest risk of cranial nerve deficits after surgery.

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.

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.

Understanding Patient Utilization Patterns of Cochlear Implant Processors.

OBJECTIVE: To evaluate the extent of benefit the second processor provides and to better understand utilization patterns regarding cochlear implant (CI) sound processors. BACKGROUND: Institutional contracts determine the external CI sound processor hardware that a patient is eligible for. Despite the high prevalence of CI worldwide, there is a paucity in the literature regarding patient preferences and how patients utilize provided external hardware. METHODS: A close-ended, multiple-choice survey was mailed to all patients over the age of 18 years who underwent CI between 2016 to 2020 at a tertiary academic medical center. Patients who received their CI hardware prior to 2018 were provided 2 processors, whereas those who received their hardware in 2018 or later were provided 1 processor. RESULTS: A total of 100/263 surveys were returned for a response rate of 38.0%. Of the cohort with 1 processor, 31.3% experienced a period without a functioning processor and access to sound compared to 5.6% of the cohort with 2 processors (P = -.003). Of the cohort with 2 processors, 24.3% noted that they often or always utilize their second processor. When asked how important having a second processor was, 62.9% of the 2-processor group responded that it was very important (P = .001). The most common reason for utilizing the second processor was a damaged primary processor. Patients who received 2 processors had a significantly lower number of postoperative audiology clinic visits for device troubleshooting (P < .001). CONCLUSION: Patients who have 2 CI external processors identify this as being very important to them and experience significantly less time without access to sound due to lack of a functioning processor. As institutional contracts often dictate whether a patient will receive 1 or 2 sound processors with their CI hardware, it is important to understand patient preferences and utilization patterns in order to guide patient-centric policies.

Salvage Microsurgery Following Failed Primary Radiosurgery in Sporadic Vestibular Schwannoma.

Importance: Management of sporadic vestibular schwannoma with radiosurgery is becoming increasingly common globally; however, limited data currently characterize patient outcomes in the setting of microsurgical salvage for radiosurgical failure. Objective: To describe the clinical outcomes of salvage microsurgery following failed primary stereotactic radiosurgery (SRS) or fractionated stereotactic radiotherapy (FSRT) among patients with sporadic vestibular schwannoma. Design, Setting, and Participants: This was a cohort study of adults (≥18 years old) with sporadic vestibular schwannoma who underwent salvage microsurgery following failed primary SRS/FSRT in 7 vestibular schwannoma treatment centers across the US and Norway. Data collection was performed between July 2022 and January 2023, with data analysis performed between January and July 2023. Exposure: Salvage microsurgical tumor resection. Main Outcomes and Measures: Composite outcome of undergoing less than gross total resection (GTR) or experiencing long-term facial paresis. Results: Among 126 patients, the median (IQR) age at time of salvage microsurgery was 62 (53-70) years, 69 (55%) were female, and 113 of 117 (97%) had tumors that extended into the cerebellopontine angle at time of salvage. Of 125 patients, 96 (76%) underwent primary gamma knife SRS, while 24 (19%) underwent linear accelerator-based SRS; the remaining patients underwent FSRT using other modalities. Postoperative cerebrospinal fluid leak was seen in 15 of 126 patients (12%), hydrocephalus in 8 (6%), symptomatic stroke in 7 (6%), and meningitis in 2 (2%). Each 1-mm increase in cerebellopontine angle tumor size was associated with a 13% increased likelihood of foregoing GTR (64 of 102 patients [63%]) or long-term postoperative House-Brackmann grade higher than I (48 of 102 patients [47%]) (odds ratio, 1.13; 95% CI, 1.04-1.23). Following salvage microsurgery, tumor growth-free survival rates at 1, 3, and 5 years were 97% (95% CI, 94%-100%), 93% (95% CI, 87%-99%), and 91% (95% CI, 84%-98%), respectively. Conclusions: In this cohort study, more than half of patients who received salvage microsurgery following primary SRS/FSRT underwent less than GTR or experienced some degree of facial paresis long term. These data suggest that the cumulative risk of developing facial paresis following primary SRS/FSRT by the end of the patient's journey with treatment approximates 2.5% to 7.5% when using published primary SRS/FSRT long-term tumor control rates.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

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.

Epidemiology and Risk Factors for Development of Sporadic Vestibular Schwannoma.

Vestibular schwannomas (VSs) are benign, slow-growing tumors of the eighth cranial nerve. Sporadic unilateral VSs constitute approximately 95% of all newly diagnosed tumors. There is little known about risk factors for developing sporadic unilateral VS. Potential risk factors that have been reported are familial or genetic risk, noise exposure, cell phone use, and ionizing radiation, whereas protective factors may include smoking and aspirin use. More research is needed to elucidate the risk factors for development of these rare tumors.

Approach to Cochlear Implantation in Patients With Ventriculoperitoneal Shunts.

OBJECTIVE: To evaluate the safety and outcomes of cochlear implantation (CI) in patients with ventriculoperitoneal (VP) shunts to inform clinical practice. STUDY DESIGN: Historical cohort study. SETTING: Tertiary referral centers. METHODS: A multi-institutional historical cohort of patients with VP shunts and CI was identified and analyzed. RESULTS: A total of 46 patients (median age 8 years [interquratile range, IQR: 2-46]) with VP shunts and CI were identified. Of these, 41 (89%) patients had a VP shunt prior to CI. Based on institutional preference and individual patient factors, CI was performed contralateral to a pre-existing VP shunt in 24 of these 41 cases (59%) and ipsilateral in 17 (41%). Furthermore, pre-CI relocation of the VP shunt was performed in 3 cases (7%), and 2 patients (5%) underwent planned revision of their VP shunt concurrent with CI. In total, 2 of 27 pediatric patients (7%) required unanticipated revision shunt surgery, both contralateral to CI device placement, given VP shunt malfunction. One of 19 adult patients (5%) required shunt revision during CI due to shunt damage noted intraoperatively. Among 43 patients with available follow-up, 38 (88%) are regular CI users, with a median consonant-nucleus vowel-consonant word: score of 58% (IQR: 28-72). CONCLUSION: CI can be performed at low risk, either contralateral or ipsilateral, to a VP shunt, and does not mandate shunt revision in most cases. Additional considerations regarding CI receiver-stimulator placement are necessary with programmable shunts to mitigate device interaction. Preoperative planning, including coordination of care with neurosurgery, is important to achieving optimal outcomes.

Disease recidivism after subtotal petrosectomy and ear canal closure.

PURPOSE: To describe the presentation, diagnosis, and management of chronic otitis media recidivism after subtotal petrosectomy and ear canal closure (STP). MATERIALS AND METHODS: Patients with temporal bone pathology detected during follow-up after STP were identified in the electronic medical record. Pertinent clinical details regarding surveillance plan, presentation, imaging findings, and revision surgery were collected and analyzed. RESULTS: A total of 10 patients were identified with recurrent or persistent pathology after STP. The median time to detection was 46 months (IQR 24-84). Five patients (50 %) had non-specific symptomatology, 4 patients (40 %) were completely asymptomatic, and 1 patient (10 %) was asymptomatic outside of two instances of mastoiditis with cochlear implant device infection treated with incision and drainage. One patient (10 %) was noted to have proptosis on examination, but no other patients had objective signs of disease at detection of disease recidivism. Nine (90 %) patients had pathology identified on preoperative imaging. All patients underwent revision surgery, with identification of cholesteatoma in 8 cases (80 %) and cholesterol granuloma in 2 cases (20 %). Extensive disease was noted in 6 patients (60 %), all of whom followed-up greater than 3 years from surgery. All patients tolerated revision surgery without complication. CONCLUSIONS: Recidivistic disease often remains clinically silent for extended periods of time after STP. Planned follow-up - with imaging or second look surgery - to facilitate early detection should be considered. Undetected disease recurrence or development may result in morbidity in a proportion of patients if surveillance is not performed.

Expanding Understanding of Electrocochleography in Cochlear Implantation: Auditory Neuropathy Spectrum Disorder With Normal Pure Tone Average.

Objective: Describe the preoperative decision-making, intraoperative electrocochleographic (ECoG) findings, and outcome of cochlear implantation (CI) in a patient with auditory neuropathy spectrum disorder (ANSD) and normal pure-tone thresholds. Patients: A 19-year-old with a history of hypoxic ischemic encephalopathy and seizures was referred for hearing rehabilitation in the setting of typical hearing by pure tone audiometry but poor speech understanding. A diagnosis of ANSD was made based on acoustic brainstem response (ABR), distortion product otoacoustic emission, and acoustic reflex testing. Imaging revealed no central cause of hearing impairment. Interventions: Right-sided CI. Main Outcome Measures: Preoperative and postoperative audiometric data. Intraoperative ECoG. Results: Preoperatively the patient underwent comprehensive audiologic testing with behavioral audiometry, ABR testing, and CI candidacy evaluation. In the right ear, the pure tone average (PTA) was 15 dB and word recognition score was 36%. ABR confirmed ANSD. Preoperative CNC and AzBio in quiet were 8% and 0%, respectively. Intraoperative ECoG amplitudes and audiometry showed responses in the 100 uV range and estimated PTA of 42 dB HL. Postoperative testing at 1-month post-initial activation revealed PTA of 45 dB HL and unchanged word and sentence scores. However, the patient cites an improved ability to communicate and increased confidence and averages over 14 hours of device use daily. Conclusions: To our knowledge, this is the first reported case of CI in an ear with normal PTA. Given that nearly all presently available ECoG data comes from patients with greater degrees of hearing loss, this unique case adds to our understanding of hearing preservation in CI.

Cochlear promontory anatomy relevant to development of subendosteal and transpromontory electrodes using 192-section ultra-high resolution temporal bone CT imaging.

Objective: To characterize normative adult ranges for cochlear promontory thickness relevant to the development of subendosteal and transpromontory electrodes to rehabilitate various neurotologic disorders. Patients: Adults (≥18 years). Intervention: In vivo radiologic assessment using a 192-slice CT scanner (Force-192; Siemens Healthcare) with ultrahigh-resolution scan mode combined and iterative reconstruction. Main Outcome Measure: Cochlear promontory thickness. Results: Among 48 included patients (96 ears), the mean (SD) age was 56 (18) years (range 25-94) and included 25 (52%) women. Of that 12 patients (25%) had osteopenia (n = 6) or osteoporosis (n = 6). The mean (SD) body mass index was 28 (5) kg/m2. The mean (SD) promontory thickness for the 96 temporal bones under study was 1.22 (0.24) mm (range 0.55-1.85). There was not a statistically significant association between age and promontory thickness (correlation coefficient .08; p = .44). Promontory thickness was significantly greater for men than women (mean 1.28 vs. 1.17 mm; p = .03) and increased with increasing body mass index (correlation coefficient .30; p = .004). Last, promontory thickness was significantly less for patients with osteopenia or osteoporosis compared with those without these conditions (mean 1.09 vs. 1.27 mm; p = .002). Conclusions: Cochlear promontory thickness can vary by almost 1.5 mm across patients and is significantly associated with patient sex, body mass index, and comorbid osteopenia/osteoporosis. Subendosteal and transpromontory electrode placement techniques must account for this degree of variability. Level of Evidence: IV.

Factors associated with hearing outcomes after a middle fossa approach in 131 consecutive patients with vestibular schwannomas.

OBJECTIVE: The middle fossa approach is an effective option for the treatment of small (Koos grade I and II) vestibular schwannomas (VSs) when the goal is hearing preservation. The authors evaluated the rates of hearing preservation and examined the factors associated with improved hearing outcomes after the middle fossa approach for VSs. METHODS: In this retrospective, single-center cohort study evaluating the clinical outcomes after resection of small VSs using the middle fossa approach, consecutive adult patients (> 18 years) who underwent surgery between January 2000 and December 2021 were included. Clinical and imaging characteristics were analyzed, including baseline hearing status, duration of surgery, anesthetic parameters, and imaging characteristics of the surgically treated tumors. RESULTS: Among the 131 included patients, 102 had valid and discoverable pre- and postoperative audiology assessments. The mean follow-up was 26 months (range 1-180 months). There were 85 patients with serviceable hearing preoperatively, defined as American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B, of whom 78% retained class A or B hearing at the last follow-up. Binary logistic regression demonstrated that preoperative hearing AAO-HNS class (OR 0.19, 95% CI 0.05-0.77; p = 0.02), overlap between fundus and cochlea (OR 0.32, 95% CI 0.11-0.96; p = 0.04), and duration of anesthesia (OR 0.98, 95% CI 0.97-0.99; p = 0.03) were independent predictors of hearing outcomes. Additionally, 75% of patients with high diffusion-weighted imaging signal in the tumor (p = 0.009) and 67% of patients with the tumor originating at the modiolus of the cochlea (p = 0.004) had poor hearing outcomes. CONCLUSIONS: The hearing preservation rates after microsurgical resection of small VSs using the middle fossa approach are high, with 78% of patients maintaining AAO-HNS class A or B hearing. Poor hearing status at baseline, longer duration of anesthesia, and large overlap between the fundus of the internal auditory canal and the cochlea were independently associated with unfavorable hearing outcomes. Imaging characteristics can be used to stratify patients' risk of hearing loss.

Cochlear Implantation in Patients With Known Cognitive Impairment: What Are the Benefits?

OBJECTIVE: To evaluate the outcomes of cochlear implantation (CI) in adults with preoperatively diagnosed cognitive impairment. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary-care academic center. PATIENTS: Adults undergoing CI with preexisting cognitive impairment. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: 1) Hearing improvement after CI; 2) morbidity and mortality associated with surgery. RESULTS: Eight patients met inclusion criteria with mean age 77.8 years (SD, 9.6 y) at time of implantation; 7 were included in subsequent analysis as one did not have speech recognition scores. Average preoperative MoCA cognitive score of 22.6 (SD, 3.9, ≤25 demonstrates cognitive impairment). Average follow-up was 29.0 months (SD, 33.3 mo). Two patients passed away at an average 58.0 months (SD, 31.1 mo) after surgery. Median preoperative pure tone average was 86.3 dB HL ( interquartile range 31.3 dB HL) compared with 33.8 dB HL (IQR 5.0 dB HL) postoperatively ( p = <0.001). Median preoperative speech testing score (AzBio/HINT) was 21% (IQR, 24%) compared with 44% (IQR, 21%) postoperatively ( p = <0.001). There were no observed surgical complications during the follow-up period. CONCLUSIONS: This study demonstrates that patients with cognitive impairment before CI can experience improved hearing, no increased risk of complications, and good longevity after CI. Further prospective studies are needed to further define the utility of CI in patients with impaired cognition.

Is There an Inherited Contribution to Risk for Sporadic Unilateral Vestibular Schwannoma? Evidence of Familial Clustering.

OBJECT: Unlike the autosomal dominant inheritance of neurofibromatosis 2, there are no known inherited risk factors for sporadic, unilateral vestibular schwannoma (VS), which comprise most VS cases. The authors tested a hypothesis positing a genetic contribution to predisposition to these lesions by analyzing familial clustering of cases. METHODS: Familial clustering of individuals with unilateral VS was analyzed in two independent genealogical resources with linked diagnosis data: the Veterans Health Administration Genealogy Resource and the Utah Population Database. Tests for excess relatedness, estimation of relative risks (RRs) in close and distant relatives, and identification of pedigrees with a significant excess of unilateral VS among descendants were performed. RESULTS: The average pairwise relatedness of the Veterans Health Administration Genealogy Resource VS cases significantly exceeded the expected relatedness ( p = 0.016), even when close relationships were ignored ( p = 0.002). RR for third- and fifth-degree relatives developing VS were significantly elevated (RR, 60.83; p = 0.0005; 95% confidence interval [CI], 7.37-219.73) and (RR, 11.88; p = 0.013; 95% CI, 1.44-42.90), respectively. No VS-affected first-, second-, or fourth-degree relatives were observed. In the Utah Population Database population, no first- or second-degree relatives with VS were observed. RR for fifth-degree relatives developing VS was significantly elevated (RR, 2.23; p = 0.009; 95% CI, 1.15-3.90). CONCLUSION: These results provide strong evidence for an inherited predisposition to sporadic, unilateral VS. This study exhibits the value of genealogical resources with linked medical data for examining hypotheses regarding inherited predisposition. The high-risk unilateral VS pedigrees identified in two independent resources provide a powerful means of pursuing predisposition gene identification.

A Comprehensive Review of Thrombocytopenia With a Spotlight on Intensive Care Patients.

Thrombocytopenia is a common entity seen in ICU patients and is associated with increased morbidity such as bleeding and transfusions, and mortality in ICU patients. Various mechanisms such as decreased platelet production, sequestration, destruction, consumption, and sometimes a combination of these factors contribute to thrombocytopenia. An understanding of the mechanism is essential to diagnose the cause of thrombocytopenia and to help provide appropriate management. The management strategies are aimed at treating the underlying disorder, such as platelet transfusion to treat complications like bleeding. Several studies have aimed to provide the threshold for platelet transfusions in various clinical settings and recommend a conservative approach in the appropriate scenario. In this review, we discuss various pathophysiological mechanisms of thrombocytopenia and the diverse scenarios of thrombocytopenia encountered in the ICU setting to shed light on the varied thresholds for platelet transfusion, alternative agents to platelet transfusion, and future directions for the implementation of thromboelastography (TEG) in multiple clinical scenarios to assist in the administration of appropriate blood products to correct coagulopathy.

Caregiver Quality of Life After Cochlear Implantation in Older Adults.

OBJECTIVE: To evaluate factors affecting quality of life (QOL) in caregivers of older cochlear implant (CI) recipients. STUDY DESIGN: Cross-sectional survey. SETTING: Academic medical center. PATIENTS: Adults over age 65 receiving CI between July 13, 2000 and April 3, 2019. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Linear regression models for caregiver QOL measured by Significant Other Scale for Hearing Disability (SOS-HEAR), with independent variables: caregiver role, patient gender, 11 factor modified frailty index (mFI), duration of hearing loss, hearing aid use, age at surgery, time since surgery, change in pure tone average (PTA), processor input type and Nijmegen Cochlear Implant Questionnaire (NCIQ). Correlations between SOS-HEAR and patient speech recognition scores. RESULTS: Questionnaires were mailed to all 294 living CI recipients. Seventy-one caregivers completed the questionnaire. Only patient gender and mFi were significant predictors of caregiver QOL on both univariate (p ≤ 0.001, ß= -20.26 [95% confidence interval -30.21, -10.3]; 0.005, -0.72 [-1.20, -0.23], respectively) and multivariate (p = 0.005, ß = -20.09, -33.05 to -7.13; 0.003, -0.93 [-1.50, -0.37]) analysis, where caregivers of female patients with lower mFI (better health) had better QOL scores. Caregiver QOL was significantly associated with patient's change in PTA and self-reported QOL scores on univariate (p = 0.041, ß = 0.52 [0.08, 0.96]; 0.024, -0.27 [-0.52, -0.02]) but not multivariate analysis. Time since CI was significant only on multivariate analysis (0.041, -0.17 [-0.33, -0.01]). Caregiver QOL did not correlate with patient speech recognition scores. CONCLUSIONS: Higher QOL scores were found among caregivers of healthier, female CI recipients. Patient hearing measurements did not correspond with better caregiver QOL.

Auricular Cartilage Resection for Treatment-Refractory Idiopathic Chronic Chondritis: A Case Series.

OBJECTIVE: Describe a series of cases of idiopathic chronic auricular chondritis refractory to antibiotics and steroids treated successfully with surgery. STUDY DESIGN: Case series. SETTING: Two tertiary academic medical centers. PATIENTS: We analyzed four patients diagnosed with chronic auricular deformity, pain, and drainage for a period of 1 to 5 years who had failed prolonged treatment consisting of antibiotics, corticosteroids, and incision and drainage. All four patients were smokers, three were diabetic, and two had a history of bariatric surgery. INTERVENTIONS: Operative subcutaneous partial auriculectomy (removal of diseased cartilage and excess skin) was performed. MAIN OUTCOME MEASURES: Resolution of pain and drainage, need for additional procedures, and reduction in narcotics required for pain control were analyzed. RESULTS: Two of the four patients were given an immediate postoperative course of doxycycline and ciprofloxacin. With a minimum of 6 weeks' follow-up, all four patients had complete resolution of pain and recurrent drainage postoperatively. One patient requiring daily narcotic medication for pain and benzodiazepine for sleep preoperatively no longer required prescription medication. All specimens revealed chronic dermal and cartilage inflammation. Three of four cases had polymicrobial infection. One case had only skin contaminant growth on culture following multiple oral and parental antibiotic regimens. CONCLUSIONS: Surgical excision of diseased cartilage as a result of idiopathic chronic chondritis is an effective treatment in those cases refractory to antibiotics and incision and drainage, and should be considered in the treatment algorithm for similar patients, potentially offering definitive cure.

Frailty and Quality of Life After Cochlear Implantation in Older Adults.

OBJECTIVE: To examine the relationship among frailty index, hearing measures, and hearing-related quality of life (QOL) in older recipients of cochlear implants. STUDY DESIGN: Cross-sectional survey. SETTING: Academic medical center. METHODS: Adults aged ≥65 years at the time of receiving cochlear implants between July 13, 2000, and April 3, 2019, were asked to complete a questionnaire on hearing-related QOL. Chart review was performed to identify patients' characteristics. Correlations were calculated between frailty index and audiologic outcome measures as well as between speech recognition scores and QOL scores. Linear regression models were developed to examine the impact of clinical characteristics, frailty index, and hearing measures on hearing-related QOL. RESULTS: Data for 143 respondents were included. The mean age was 80.7 years (SD, 7.1), with a mean 27.8 years of hearing loss (SD, 17.4) before implantation. The mean frailty index was 11.1 (SD, 10.6), indicating that patients had 1 or 2 of the measured comorbidities on average. No correlation was found between lower frailty index (better health) and hearing scores, including pure tone averages (PTAs) and speech recognition scores. Lower frailty index and larger improvement in PTA after cochlear implantation predicted better QOL scores on univariate analysis (respectively, P = .002, ß = -0.42 [95% CI, -0.68 to -0.16]; P = .008, ß = -0.15 [95% CI, -0.26 to -0.04]) and multivariate analysis (P = .047, ß = -0.28 [95% CI, -0.55 to -0.01]; P = .006, ß = -0.16 [95% CI, -0.28 to -0.05]). No speech recognition scores correlated with QOL after cochlear implantation. CONCLUSIONS: Frailty index does not correlate with hearing scores after cochlear implantation in older adults. Lower frailty index and more improvement in PTA predict better QOL scores after cochlear implantation in older adults.