[SLEEP HABIT MODIFICATION AS A POSSIBLE PREVENTION OF RECURRENT BENIGN PAROXYSMAL POSITIONAL VERTIGO].

BACKGROUND: Benign paroxysmal positional vertigo (BPPV) is effectively treated with a variety of repositioning maneuvers but one-third to one-half of patients experience recurrence, usually within 2 years after the first attack. OBJECTIVES: The aim of this study was to investigate possible prevention of recurrent BPPV by sleep habit modification. METHODS: Patients diagnosed with posterior semicircular canal BPPV (p-BPPV) were asked their preferred lying side during nocturnal sleep. Following Epley maneuver they were recommended to change their head lying side at least every 2 hours during nocturnal sleep and to come back in case of recurrence. RESULTS: A total of 266 patients were diagnosed with p-BPPV. The mean patient's age was 57 years (range 14-87 years). There were 167 patients with right p-BPPV and 99 patients with left p-BPPV; 134 (50%) patients habitually slept on the right side. Of those, 112 (84%) were diagnosed with right p-BPPV (P= 0.0006); 87 patients (33%) habitually slept on the left side; 56 of them (64%) were diagnosed with left p-BPPV (P <0.0001). Among the 45 patients (17%) who expressed no preference concerning their sleeping positions, the right versus left p-BPPV was nearly even. During the follow-up period (1-80 months, mean 41) 11 patients (4%) were diagnosed with recurrent p-BPPV. Of those, 9 had a recurrence in the same posterior semicircular canal and 2 in the contralateral one. All of them reported that they had not modified their sleep habits. CONCLUSIONS: The results of our study can shed some light on the etiology of BPPV and may be helpful in preventing recurrent BPPV by changing sleep-position habits.

Protective effect of soluble Klotho in pediatric patients undergoing cardiac surgery with cardiopulmonary bypass support-A pilot study.

OBJECTIVE: The Klotho protein family plays important roles in several metabolic pathways. Soluble Klotho has been recently put forward as an antiaging protein, demonstrating renal and cardiovascular protective traits. Cardiopulmonary bypass (CPB) support during cardiac surgery has been implicated in several adverse outcomes in pediatric and adult patients. Our goal was to assess whether serum Klotho levels can be used to predict outcomes in children undergoing cardiac surgery with CPB due to congenital heart defects (CHDs). METHODS: This prospective study was conducted on pediatric patients admitted to two Pediatric Cardiac Intensive Care Units, between 2012 and 2018. All patients were born with CHD and underwent corrective surgery with CPB. Sequential blood samples were analyzed by enzyme-linked immunosorbent assay for soluble Klotho levels at baseline, 2, 6, and 24 h after surgery. The association between Klotho levels and several demographic, intraoperative, and postoperative clinical and laboratory parameters was studied. RESULTS: Twenty-nine children undergoing cardiac surgery with CPB support were included. Serum Klotho levels were shown to significantly decrease 2 h after surgery and increase to baseline levels after 6 h (p < .001 and p < .05, respectively). Patients with low Klotho levels 2 h after surgery were at a 32-fold higher risk for developing postoperative complications (p = .015, odds ratio < 0.03). Moreover, Klotho levels at each of the four time points were lower in patients who developed postoperative complications. CONCLUSIONS: Cardiac surgery with CPB results in a significant decrease of serum Klotho levels 2 h after surgery in pediatric patients with CHDs, which can be used to predict development of postoperative complications in this patient population.

Imaging of the age-related anatomical relationship between the innominate artery and the trachea.

PURPOSE: To measure the age-dependent changes of anatomical positions and relations between the trachea, cricoid cartilage (CC), and innominate artery (IA) in adults by computed tomography (CT). METHODS: A retrospective cohort reviewing images of 127 consecutive adult patients who underwent CT angiography (CTA) of the neck. The trachea-to-IA (T-IA) distance was measured as the minimal horizontal distance between them. The vertical distance between the CC and the IA was measured between the axial section, demonstrating the CC's inferior border to the axial section at the level of the T-IA measurement. RESULTS: Images of 125 patients (median age 53 years, range 18-89; 74 males) were reviewed. The mean T-IA distance was 2.3 ± 1.1 mm for males and 1.7 ± 0.9 mm for females (P = 0.002). The vertical C-IA distance was 44.2 ± 11.4 mm and 49.5 ± 12.5 mm for males and females, respectively (P = 0.01). Age correlated negatively with the vertical C-IA distance (P < 0.0001) and positively with the T-IA distance (P < 0.0001). The rate of IA variants was 23.2%, with no significant difference between the measurements of distances among patients with or without IA variants. CONCLUSIONS: This is the first description of the relationships between the trachea, CC, and IA distances in adults as depicted on CTA. The T-IA distance becomes larger while the T-CC distance becomes shorter with age.

Trans-canal endoscopic ear surgery and canal wall-up tympano-mastoidectomy for pediatric middle ear cholesteatoma.

PURPOSE: To evaluate clinical parameters, outcomes and complications of transcanal endoscopic ear surgeries (EES) and canal wall-up tympano-mastoidectomy (CWU) for middle ear cholesteatoma in children and to compare between the two surgical approaches. METHODS: A retrospective chart review of all children (< 16 years) who underwent surgery for cholesteatoma involving the middle ear only with a minimal follow-up period of 12 months. Demographic features, site and extent of disease, outcome and complications were reviewed and compared between the groups. RESULTS: Thirty EES and 19 CWU were included. The overall disease relapse rates in the EES and CWU groups were 20% (n = 6, residual rate = 10%, recurrence rate = 10%) and 47% (n = 9, residual rate = 11%, recurrence rate = 37%), respectively (p = 0.04), with mean duration of follow-up of 32.6 and 37.2 months, respectively. In the EES and CWU groups, the most common site of residual disease was the mastoid cavity/antrum (n = 2, 66% and n = 2, 100%, respectively). Most recurrences involved the epitympanum and extended into the tympanic cavity (n = 2, 66%) in the EES group and into the tympanic cavity, posterior mesotympanum and mastoid cavity/antrum (n = 3, 43%, each) in the CWU group. The overall complication rates in the EES and CWU groups were 10% (n = 3) and 11% (n = 2), respectively (p = 0.61). CONCLUSIONS: Endoscopic ear surgeries in children were found to be an acceptable and safe technique for the treatment of cholesteatoma limited to the middle ear cavity. A better overall success rate and a similar complication rate were found in the EES group when compared to CWU.

Remote optical sensing in otolaryngology: middle ear effusion detection.

Otitis Media (OM) is related to a group of inflammatory diseases of the middle ear (ME) commonly encountered, worldwide. A method based on a simple device, which can be used by medical staff and non-experts to detect OM is presented. The method is based on detection of tympanic membrane (TM) vibrations. A laser beam is pointed on an infra-sonic stimulated TM with fast camera capturing the back scattered secondary speckle patterns. A camera enables inspection of the frequency and amplitude of the changes in TM characteristics obtained by analysis of the spatial-temporal statistics of the speckle patterns. The results may provide information that express ME effusion.

Awake Tracheostomy: Indications, Complications and Outcome.

BACKGROUND: Awake tracheostomy (AT) is aimed at securing the airway of patients with upper airway obstruction when other means are not feasible or have failed. Reports on AT in the literature are scarce. The goal of this study was to review our experience with the indications, complications and outcome of AT. METHODS: A retrospective chart review was conducted on all ATs performed between 2010 and 2016 in two university-affiliated, tertiary medical centers. Data on demographics, indications, techniques, urgency and postoperative complications were retrieved from the medical charts. RESULTS: The 37 of the 1023 recorded tracheostomies (3.62%) that were ATs comprised the study group (mean age of the patients 60.3 years, 32 [86.5%] males). The most common indication was head and neck (HN) malignancy (oncologic group, 70.3%), with the larynx (53.8%) being the most commonly involved site. Patients in the non-oncologic group (n = 11) were significantly younger (P = 0.048) and had a significantly higher prevalence of urgent surgery compared to the oncologic group (P = 0.0009). Major postoperative complications included tube dislodgement (n = 2) and pneumothorax (n = 1) that were managed successfully. One of the two patients with severe hypoxia and arrhythmia that necessitated cardiopulmonary resuscitation died. CONCLUSION: Whether the etiology of the AT was related to HN oncological disease or not was the most important clinical factor in our cohort. The non-oncologic group was significantly younger, suffered from more urgent events and tended to have more complications (nonsignificant). ATs had a 97.3% rate of immediate survival, a 5.4% risk of major irreversible complications and a 2.7% risk of mortality.

Assessing the Different Oncologic Disease Distribution and Postoperative Complications of Octogenarian and Nonagenarian Head and Neck Oncology Patients.

PURPOSE: The admission rate of patients aged 80 years or older (oldest-old) with head and neck (HN) oncologic disease is on the rise. Our goal was to study the demographic characteristics, reasons for admission, types of surgical procedures, and postoperative complications of the oldest-old patients with HN malignancy. MATERIALS AND METHODS: We conducted a retrospective cohort study including all inpatients aged 80 years or older who were admitted to the department of otolaryngology-head and neck surgery or department of oral and maxillofacial surgery because of HN oncologic disease between 2009 and 2013. The control group was composed of a matched number of randomly selected patients aged 60 to 79 years. We compared the demographic characteristics, diagnoses, comorbidities, surgical interventions, and postoperative complications of the 2 age groups to characterize the oldest-old patients' admissions and determine whether age alone increases the risk of postoperative complications. RESULTS: The study included 109 oldest-old patients (median age, 83 years) and 107 patients in the control group (median age, 68 years). Although the oldest-old patients had significantly more underlying diseases (4.41 vs 2.86) and drugs prescribed (4.76 vs 3.21), similar rates of postoperative complications occurred in both groups. An important finding was that ischemic heart disease and chronic lung disease were the only significant risk factors for postoperative complications among the oldest-old patients (odds ratio on multivariate analysis of 5.5 and 4.5, respectively). CONCLUSIONS: Although comorbidities and prescribed drugs are more prevalent in the oldest-old patients, the rate of postoperative complications did not differ between the age groups, suggesting that age alone should not be a factor in the surgical treatment of HN malignancies.

Traumatic Perforation of the Tympanic Membrane: A Review of 80 Cases.

BACKGROUND: Traumatic perforation of the tympanic membrane (TPTM) is often encountered in primary care or in the emergency department (ED). Several therapeutic interventions have been described, but conservative follow-up until spontaneous complete recovery is the most common choice. OBJECTIVE: Our goal was to analyze the trauma mechanism, perforation characteristics, and outcome of patients with TPTM. METHODS: The study included patients examined in the ED of a tertiary, university-affiliated medical center because of TPTM between 2012 and 2016. Their medical records were retrospectively reviewed for demographics, trauma mechanism, clinical characteristics, and outcome. A phone survey was performed to obtain the missing information of all the patients who did not continue their follow-up in our outpatient clinic. RESULTS: We reviewed the histories of 80 patients with a mean age of 26.7 ± 14.6 years (20 children; 25%). TPTM was caused by blunt trauma in 45 patients (56%) and penetrating trauma in 35 patients (44%). Thirty-five patients (44%) completed their follow-up in the hospital outpatient clinic, with a mean duration of 6.2 weeks. Twenty-five patients (38%) completed their follow-up in a community-based otolaryngology clinic, 6 patients (9%) chose not to complete their follow-up, and 14 patients were lost to follow-up. Of the 60 patients who completed follow-up, 56 patients recovered spontaneously, 3 patients underwent successful tympanoplasty, and 1 patient was referred to surgery but was lost to follow-up. All children healed spontaneously. CONCLUSION: TPTM was more common in young males with main mechanisms of blunt trauma (an assault) or cleaning the ear canal. All children demonstrated complete spontaneous recovery.

Warthin tumor within the superficial lobe of the parotid gland: a suggested criterion for diagnosis.

The location of Warthin tumor (WT) in the parotid gland impacts the surgical approach and may be indicative of the elusive origin of this intriguing entity. Location in the deep versus superficial lobe of the gland is not directly addressed when defining WT characteristics. Our observation, of rare occurrence of deep lobe WT, if at all, led to the current investigation. The study design is cohort study. This is a retrospective chart review of all patients undergoing parotidectomy for WT in two tertiary academic referral centers: the Sheba Medical Center (SMC), Israel, and the Christiana Care (CC), Newark, Delaware, USA. 122 consecutive adult patients underwent parotidectomy for WT (72 from SMC and 50 from CC). Seventy percent were males, with a mean age of 60.6 years. Bilateral WT or multi-centric WT were found in 9.8 and 17.2% of the cases, respectively. In one case, the tumor was described as originating in the deep lobe. In all other cases, the tumor originated and was limited to the superficial lobe. 99.2% of WT originated in the superficial lobe, corresponding with the few reports directly addressing its location in the gland. The reason for the tumor to be limited almost uniformly to the superficial lobe is unknown, and could be related to the etiopathogenesis of this elusive entity. We suggest adding tumor location within the superficial lobe to the common characteristics of WT (male, smoking, and lower pole) that serve as "common criterion" while evaluating a parotid lesion.

The admission patterns of octogenerians nonagenerians and centenarians to the Department of Otoloaryngology.

Life expectancy in Israel has risen by almost 6 years during the last 25 years, and the proportion of people 65 years of age or older is expected to reach 12 % of the total population by 2020. A substantial increase in the workload for Otolaryngologists and Head and Neck surgeons is anticipated. Our goal was to characterize the admissions of patients 80 years of age and older to the Department of Otolaryngology, Head and Neck Surgery in a tertiary medical center. The study group included all patients 80 years of age and older who were admitted to the Department of OTOHNS in our institute between 2009 and 2013. There were two control groups for comparison divided by age; one group 40-59 years old and the other group 60-79 years old. There were 385 admissions of 317 patients aged 80-103 years (4.2 % of overall admissions). Over the study period, admissions of patients over 80 years increased on average by 3 % per annum (p = 0.4), and those patients over 90 years old by 52 % per annum (p < 0.001). The most common indication was HN malignancy (28.8 %) followed by otologic disorders (22.0 %). Of the overall 158 operations conducted, 131 patients (82.9 %) underwent elective procedures (mainly oncology) and 27 patients (17.1 %) underwent emergent procedures. The distribution of the reasons for admission of the patients older than 80 years is surprisingly different from that of the "younger" patients. With life expectancy rising, our study predicts a workload increase mainly in the HN oncologic and otologic services.

Cochlear Implantation in Charcot-Marie-Tooth Patients: Speech Perception and Quality of Life.

OBJECTIVES: There is a limited understanding of the impact of cochlear implantation (CI) in patients with Charcot-Marie-Tooth disease (CMT), given the scarcity of reported cases. We aim to evaluate the audiological outcomes and quality of life (QoL) after CI in CMT. METHODS: Multi-institutional, university-affiliated, tertiary-referral centers, retrospective chart review.Our cohort includes 5 patients with CMT. Patients' charts were reviewed for demographic characteristics, operation notes, and pre- and post-implantation audiology evaluation. Patients completed the Cochlear Implant Quality of Life-10 (CIQOL-10) Global questionnaire. RESULTS: Pre-implantation, the mean pure tone average was 84.1 ± 7.2 dB, and the mean word recognition score was 2.4% in the implanted ear. AzBio sentence test was performed in quiet, revealing a mean of 4 ± 1.4% in the implanted ear. Post-implantation, PTA results were all within the mild hearing loss range (mean 33.0 ± 5.9 dB). Post-CI, AZ-Bio test results were 5%, 65%, and 74% (for 3 patients), and HINT scores were 55% and 58% (for 2 patients). The mean score of the CIQOL-10 questionnaire was 42.7 ± 10.47 (range 1-100). Patients were most satisfied with their ability to listen to the television or radio, have conversations in a quiet environment, and feel comfortable being themselves. CONCLUSION: To the best of our knowledge, this is the most extensive series of CI in CMT-associated sensorineural hearing loss and auditory neuropathy. Our cohort suggests that CI is a safe and reliable method for hearing rehabilitation that can achieve good speech performance and improve QoL in CMT patients.

Revision Cochlear Implantation With Device Manufacturer Conversion: Surgical Outcomes and Speech Perception Performance.

OBJECTIVE: Describe the clinical profile of revision cochlear implantation (RCI) cases involving device manufacturer conversion (RCImc+), compare them to cases without manufacturer conversion (RCImc-), and classify the reasons for manufacturer conversion (MC). STUDY DESIGN: Retrospective case review. SETTING: Tertiary academic center. METHODS: Data on demographics, RCI indications, medical background, surgical details, and the reasons for MC were collected for all RCIs from 1989 to 2020. Post-RCI speech perception performance was categorized as unchanged, improved, or declined, according to clinically based criteria. RESULTS: Of 185 RCIs, 39 (21%) involved MC, mostly in pediatric patients (67%). The leading RCImc+ indications were device-related (59%) and medical (31%) failures. Initial implant manufacturers were Advanced Bionics (49%), Cochlear (25.5%), or Medel (25.5%). Most MC reasons were patient-driven (64%) versus CI team recommendations (36%). The RCImc+ group demonstrated a 3-fold higher rate of medical indications than RCImc- (31% vs 11.5%, P = .007). The time interval from symptom onset to RCI was longer in RCImc+ (43 vs 20.3 months, P = .001), and the rate of multiple revisions in the same ear was higher (25.6% vs 8.2%, P = .009). Complete reinsertion rates were high in both RCImc+ and RCImc- (94.8% vs 94.5%, P = 1) without any complications. Speech perception improved or remained unchanged in most (84%) cases, with no significant difference between the groups (P = .183). CONCLUSION: This retrospective study showed that RCI involving MC is safe and beneficial. Although RCImc+ patients exhibited distinct clinical characteristics, MC did not impact surgical or speech perception outcomes. This provides evidence-based data to support informed decision-making by CI teams and patients.

Virtual Reality Helps Describe the Progression of Thyroid Cartilage Calcification.

OBJECTIVES: Thyroid cartilage (TC) calcifications may impact surgical planning and clinical management. However, few studies to date have implemented virtual reality (VR) to evaluate these calcifications. This study assessed the feasibility of evaluating TC calcifications in various regions and measuring their volumes through VR models generated from computed tomography scans. We also investigated age and gender-related differences in calcification patterns. METHODS: Ninety-two participants were categorized into younger, middle-aged, and older age groups. Calcification patterns (degree in Hounsfield units and volume of calcification in cm3) in different TC regions were identified by VR analysis, which enabled comparisons between age groups and genders. RESULTS: Significant differences in calcification patterns were observed between males and females, particularly in the middle right, middle left, bottom left, and vertex regions. Age-related differences in the vertex region showed increased calcification in the older age group. CONCLUSION: This study points to the contribution of VR in the evaluation of complex anatomical structures. The findings revealed significant gender and age patterns in TC calcification. These insights can inform surgical planning and highlight the potential of using VR to gain a better understanding of TC calcification clinically.

Rates, Indications, and Speech Perception Outcomes of Revision Cochlear Implantations.

Revision cochlear implant (RCI) is a growing burden on cochlear implant programs. While reports on RCI rate are frequent, outcome measures are limited. The objectives of the current study were to: (1) evaluate RCI rate, (2) classify indications, (3) delineate the pre-RCI clinical course, and (4) measure surgical and speech perception outcomes, in a large cohort of patients implanted in a tertiary referral center between 1989-2018. Retrospective data review was performed and included patient demographics, medical records, and audiologic outcomes. Results indicated that RCI rate was 11.7% (172/1465), with a trend of increased RCI load over the years. The main indications for RCI were device-related failures (soft-45.4%, hard-23.8%), medical failure (14%), trauma (8.1%), and surgical failure (6.4%). Success rate was 98.8%. Children comprised 78% (134) of the cohort and were more likely than adults to undergo RCI. Most (70%) of the RCIs were performed within 10 years from primary implantation. Speech perception outcome analysis revealed unchanged or improved performance in 85% of the cases and declined performance in 15%. Current findings confirm that RCI is a safe with high clinical efficacy; however, the non-negligible percentage of patients that exhibited declined performance post-RCI should be considered in decision-making processes regarding RCI. Routine follow-up during their first years post-implantation is warranted.

State of the art regeneration of the tympanic membrane.

PURPOSE OF REVIEW: One of the most common diseases of the tympanic membrane is a perforation, and tympanoplasty is one of the more common procedures in otolaryngology. Tympanic membrane regeneration and bioengineering aim to improve the success rate of the procedure, increase the availability of different scaffolds and provide innovative tools that will simplify the surgical technique and make it accessible for surgeons with varying expertise level. This review aims to raise awareness of current tissue engineering developments in tympanic membrane regeneration and how they may augment current clinical practices. We focus here on achievements in tympanic membrane cell cultures and on innovations in development of new scaffolds and growth factors that enhance regeneration of patient's native tympanic membranes. RECENT FINDINGS: In recent years, great achievements were reached in the field of tympanic membrane regeneration in the three hallmarks of bioengineering: cells, scaffolds and bioactive molecules. New techniques for modeling normal tympanic membrane proliferation were developed, as well as for isolation and expansion of normal tympanic membrane keratinocytes from miniature samples of scarred tissue. Ongoing clinical trials aim to seal the perforation by applying different scaffolds infiltrated by growth factors on the tympanic membrane. SUMMARY: Research efforts in tympanic membrane regeneration continue to seek the ideal single tissue-engineered substitute. Recent advances in tympanic membrane bioengineering include new types of scaffolds that may augment and provide a safe and effective alternative to the current gold-standard autograft. New bioactive molecules may simplify the surgical procedure and reduce surgical time by augmenting the native tympanic membrane regeneration. Several groups of bioengineering scientists and neurotologists are continuing to move forward and develop new strategies, seeking to create a fully functional tissue-engineered tympanic membrane.

The Feasibility to Isolate and Expand Tympanic Membrane Squamous Epithelium Stem Cells From Scarred Perforation Margins.

HYPOTHESIS: The scarred rim of chronic tympanic membrane (TM) perforation contains keratinocytes with potential for regeneration while maintaining their morphological and genetic characteristics. BACKGROUND: The squamous epithelium of the TM has a good regeneration capacity. Successful isolation and expansion of human TM keratinocytes (hTMKR) was reported from a full, en-bloc, healthy TM. METHODS: Trimmed margins of the TM perforation (harvested during tympanoplasty) underwent enzymatic digestion (collagenase or trypsin) and were seeded either with serum-containing medium (SCM) or keratinocyte serum-free medium (KSFM) and progenitor cell growth medium (PR) (KSFM:PR, 1:1). Gene expression analysis by real-time qRT-PCR was used to compare between human TM cells derived from scarred perforation margins (hTMKR), normal human skin keratinocytes (NhSKR), and human fibroblasts. RESULTS: Twelve patients were included in the study. In 9 of 12 cases (75%) single-cell isolation with fibroblastic or epithelial cell morphology (or both) was achieved. Cells seeded with KSFM:PR yielded epithelial morphology (hTMKR) while SCM culturing resulted in a fibroblastic morphology (hTMFib). Gene expression analysis revealed significant higher expression of VCAN (p = 0.002) and FOXC2 (p = 0.015) at the mRNA levels (normal hTMKR markers) in hTMKR compared to NhSKR. In addition, a comparison of gene expression between hTMKR and hTMFib revealed significantly higher levels of both VCAN (p = 0.045) and SLC6A14 (p = 0.036) among hTMKR. CONCLUSION: For the first time, we developed a protocol to isolate hTMKR from scarred TM perforation margins. Furthermore, we succeeded in achieving tissue expansion that preserved the characteristic of healthy TM cells. This study bridges "regenerative medicine" approach with clinical and surgical objectives.

Epistaxis in the setting of antithrombotic therapy: A comparison between factor Xa inhibitors, warfarin, and antiplatelet agents.

OBJECTIVES/HYPOTHESIS: To describe the characteristics and severity of epistaxis in patients taking factor Xa inhibitors novel anticoagulants. STUDY DESIGN: Retrospective cohort study. METHODS: A study of adult patients hospitalized due to spontaneous epistaxis under the treatment of warfarin, rivaroxaban, or apixaban between the years 2011 and 2017 was performed. A control group of patients under antiplatelet therapy (acetylsalicylic acid, clopidogrel) was included. The mean follow-up periods in the warfarin, rivaroxaban, apixaban, and antiplatelet groups were 18, 14.5, 13.5, and 18.2 months, respectively. We compared demographics, location and severity of bleeding, treatment methods, and outcome between the groups. RESULTS: The study included 109 patients (35 under factor Xa inhibitors), the majority of whom presented with anterior epistaxis (68%). The antiplatelet group had more episodes of epistaxis prior to admission, and required endoscopic surgical control of bleeding more often, in comparison with anticoagulants (2.23 vs. 1.44, P < .05 and 23% vs. 6%, respectively, P < .05). Among anticoagulants, combined therapy (cauterization and packing) was required more frequently in the apixaban group compared to the rivaroxaban and warfarin groups (64% vs. 25% and 33%, respectively, P < .05). The rate of readmissions due to epistaxis, within 1 year of follow-up was lower in the factor Xa inhibitor groups compared with the warfarin and antiplatelet groups (16% vs. 9% and 4%, respectively, P < .05). Cessation of factor Xa inhibitor therapy was effective and uneventful with no further epistaxis events. CONCLUSIONS: Epistaxis under factor Xa inhibitors was effectively treated with no worse and perhaps even a better outcome when compared to other anticlotting medications. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:119-123, 2019.

Recurrent pleomorphic adenoma: is treatment considerably delayed thus affecting surgical morbidity?

OBJECTIVE: To examine the incidence of late presentation of patients with recurrent pleomorphic adenoma (RPA) of the parotid gland. METHODS: This is a retrospective analysis of patients treated in our center. We examined patients demographics, disease characteristics, treatment, and outcome and as well as the time period length from the first discovery of a recurrent mass until seeking treatment at our clinic and its effect on morbidity. RESULTS: A total of 30 patients were included. 26% underwent initial enucleation in other institutions. In eight patients (26%), the recurrence of the mass was second or higher. The patients average time period length until seeking treatment was 2.48 years, with 33% of patients showing a time period length of over three years. Multifocal tumor, tumor diameter larger than 2 cm and facial nerve involvement were found in 15 (50%),16 (53.3%) and seven (25%) patients respectively. Patient's delay of presentation by ≥3 years was associated with a tumor size of ≥2 cm (Relative Risk [RR] = 2, p = .02). Patient's delay of presentation by ≥2 years was also associated with a trend towards a higher rate of post-operative facial nerve palsy (RR = 3.37, p = .07, CI = 0.88-12.85). CONCLUSION: Most patients with RPA were presented late, thus affecting disease extent and surgical morbidity.

Mutations in AIFM1 cause an X-linked childhood cerebellar ataxia partially responsive to riboflavin.

BACKGROUND: AIFM1 encodes a mitochondrial flavoprotein with a dual role (NADH oxidoreductase and regulator of apoptosis), which uses riboflavin as a cofactor. Mutations in the X-linked AIFM1 were reported in relation to two main phenotypes: a severe infantile mitochondrial encephalomyopathy and an early-onset axonal sensorimotor neuropathy with hearing loss. In this paper we report two unrelated males harboring AIFM1 mutations (one of which is novel) who display distinct phenotypes including progressive ataxia which partially improved with riboflavin treatment. METHODS: For both patients trio whole exome sequencing was performed. Validation and segregation were performed with Sanger sequencing. Following the diagnosis, patients were treated with up to 200 mg riboflavin/day for 12 months. Ataxia was assessed by the ICARS scale at baseline, and 6 and 12 months following treatment. RESULTS: Patient 1 presented at the age of 5 years with auditory neuropathy, followed by progressive ataxia, vermian atrophy and axonal neuropathy. Patient 2 presented at the age of 4.5 years with severe limb and palatal myoclonus, followed by ataxia, cerebellar atrophy, ophthalmoplegia, sensorineural hearing loss, hyporeflexia and cardiomyopathy. Two deleterious missense mutations were found in the AIFM1 gene: p. Met340Thr mutation located in the FAD dependent oxidoreductase domain and the novel p. Thr141Ile mutation located in a highly conserved DNA binding motif. Ataxia score, decreased by 39% in patient 1 and 20% in patient 2 following 12 months of treatment. CONCLUSION: AIFM1 mutations cause childhood cerebellar ataxia, which may be partially treatable in some patients with high dose riboflavin.