Sirolimus-coated Eustachian tube balloon dilatation for treating Eustachian tube dysfunction in a rat model.

Eustachian tube balloon dilatation (ETBD) has shown promising results in the treatment of ET dysfunction (ETD); however, recurrent symptoms after ETBD frequently occur in patients with refractory ETD. The excessive pressure of balloon catheter during ETBD may induce the tissue hyperplasia and fibrotic changes around the injured mucosa. Sirolimus (SRL), an antiproliferative agent, inhibits tissue proliferation. An SRL-coated balloon catheter was fabricated using an ultrasonic spray coating technique with a coating solution composed of SRL, purified shellac, and vitamin E. This study aimed to investigate effectiveness of ETBD with a SRL-coated balloon catheter to prevent tissue proliferation in the rat ET after ETBD. In 21 Sprague-Dawley rats, the left ET was randomly divided into the control (drug-free ETBD; n = 9) and the SRL (n = 9) groups. All rats were sacrificed for histological examination immediately after and at 1 and 4 weeks after ETBD. Three rats were used to represent the normal ET. The SRL-coated ETBD significantly suppressed tissue proliferation caused by mechanical injuries compared with the control group. ETBD with SRL-coated balloon catheter was effective and safe to maintain ET luminal patency without tissue proliferation at the site of mechanical injuries for 4 weeks in a rat ET model.

[Contemporary diagnosis and management of congenital microtia and aural atresia : Part 2: Overview of therapeutic approaches]. / Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen : Teil 2: Übersicht der verschiedenen Therapieansätze.

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.

Temporomandibular treatments are significantly efficient in improving otologic symptoms.

Symptoms of temporomandibular disorders (TMD) could be present as otologic symptoms like earache and dizziness in some patients. In most cases, these symptoms are not recognized because otolaryngologists fail to diagnose TMD as a source of the problem. This investigation was conducted to evaluate the effect of TMD treatments on the otologic symptoms which after taking history and clinical examinations seemed to be related to TMD. In the present study, the patients who were complaining of otalgia, ear fullness, tinnitus, hearing loss, and dizziness were evaluated by an ear fellow. Forty patients who had no known otologic or other primary causes to explain their symptoms, were referred to the orofacial pain clinic with the possible diagnosis of TMD. If the diagnosis was confirmed by an orofacial pain specialist, a combination of TMD treatments was administered to each case and the patients were followed up. The results showed that following implementation of treatment protocols for TMD, more than 50% of the patients reported complete or partial recovery in the second follow-up (p < 0.05). The most common otologic symptom of the referred cases was earache, and the most common associated complaint was neck pain. All the patients had one or more parafunctional habits. This study showed that TMD treatments were significantly efficient in improving otologic symptoms partially or completely and the authors concluded that for the patients with otolaryngologic unexplained symptoms, an overhaul examination is needed to assess TMD as a possible cause of the patient complaint. It is recommended that in cases with unexplained otologic symptoms, otolaryngologists care more about the neck trigger points (TP) and ask about the patient's parafunctional habits. Otolaryngologists and dentists need to be aware of the risk of developing otologic symptoms caused by these habits or cervical TPs.

[Clinical analysis of 11 cases of otogenic intracranial complications treated by multidisciplinary collaboration].

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.

Results of Eustachian tube balloon dilation measured using the nine-step test.

Suggested several decades ago, the nine-step test is an intuitive test of Eustachian tube function. However, studies employing the nine-step test to assess the results of Eustachian tube balloon dilation (EBD) are limited. We aimed to objectively evaluate the efficacy of EBD in opening failure patients with decreased maximal peak pressure difference (MPD) using the nine-step test. Patients who had MPD values ≤ 13 daPa in the nine-step test were enrolled. The patients were categorized into two groups according to treatment decisions after discussion with a clinician: an EBD group (N = 26) and a medication group (N = 30). One month after treatment, the seven-item Eustachian Tube Dysfunction Questionnaire (ETDQ7) and the nine-step test were administered to all participants and subgroups of symptomatic participants (ETDQ7 > 15). MPD improved (increased) in both the EBD group and the medication group. ETDQ7 values improved (decreased) in the EBD group, but not in the medication group. In subgroup analysis, MPD and ETDQ7 values improved only in the symptomatic EBD group. According to the nine-step test, EBD can normalize 53.8% of decreased MPD. Posttreatment MPD and ETDQ7 scores were significantly better in the EBD group than in the medication group. However, EBD in patients with abnormal nine-step test results seemed less efficacious when the treatment results of the medication group were considered.

Dermatologic Conditions of the External Ear: Basics, Updates, and Pearls.

Cutaneous diseases of the ear encompass a wide range of symptoms, complaints, and factors that negatively impact patients' well-being. These observations are frequently encountered by otolaryngologists and other physicians who treat individuals with ear-related issues. In this document, we aim to offer up-to-date information on diagnosing, predicting outcomes, and treating commonly occurring ear diseases.

Research and application progress in deep learning in otology. / æ·±åº¦å­¦ä¹ åœ¨è€³ç§‘å­¦ä¸­çš„ç ”ç©¶ä¸Žåº”ç”¨è¿›å±•.

With the optimization of deep learning algorithms and the accumulation of medical big data, deep learning technology has been widely applied in research in various fields of otology in recent years. At present, research on deep learning in otology is combined with a variety of data such as endoscopy, temporal bone images, audiograms, and intraoperative images, which involves diagnosis of otologic diseases (including auricular malformations, external auditory canal diseases, middle ear diseases, and inner ear diseases), treatment (guiding medication and surgical planning), and prognosis prediction (involving hearing regression and speech learning). According to the type of data and the purpose of the study (disease diagnosis, treatment and prognosis), the different neural network models can be used to take advantage of their algorithms, and the deep learning can be a good aid in treating otologic diseases. The deep learning has a good applicable prospect in the clinical diagnosis and treatment of otologic diseases, which can play a certain role in promoting the development of deep learning combined with intelligent medicine.

Is there a role for Eustachian tube balloon dilation in pediatric patients with refractory Eustachian tube dysfunction?

BACKGROUND: Eustachian tube dysfunction (ETD) is common in children. Over the past decade, eustachian tube balloon dilation (ETBD) has become a more widespread treatment for this condition. Data has been encouraging in the adult population, but data among the pediatric population has been sparser. This comprehensive review aims to assess current evidence for ETBD in pediatric patients. REVIEW: Studies relevant to ETBD in the pediatric population were identified by utilizing the PubMed MEDLINE database. While multiple retrospective studies were found, this search yielded two systematic reviews focused on the pediatric population as the highest level of evidence assessing ETBD in children. There was overlap of included studies, but each review contained a unique set of studies. These reviews found that ETBD was safe in children. BEST PRACTICE SUMMARY: Overall, ETBD appears safe and efficacious in children with refractory ETD. Future prospective trials confirming this conclusion are warranted. At this time, there appears to be a role for ETBD with or without ventilation tube placement in children with refractory ETD.

Continuous positive airway pressure device-associated nonhealing ulcer on the nasal dorsum: chondrodermatitis nodularis nasi.

With increasing use of positive airway pressure devices for disordered breathing during sleep, appreciation for potential associated side effects is essential. Chondrodermatitis nodularis nasi is a rare variant of chondrodermatitis nodularis chronic helicis that presents as a nonhealing erosion or ulcer on the nose and can occur in association with chronic use of positive airway pressure devices. We present a case of symptomatic chondrodermatitis nodularis nasi associated with continuous positive airway pressure use, fully responsive to treatment, and propose that this condition is highly underrecognized. Dermatology referral can assist in diagnosis, management, and exclusion of cutaneous malignancy. CITATION: Reinhart JP, Isaq NA, Peters MS, Vidal NY. Continuous positive airway pressure device-associated nonhealing ulcer on the nasal dorsum: chondrodermatitis nodularis nasi. J Clin Sleep Med. 2023;19(6):1157-1159.

Clinical Predictors of Symptom Improvement Following Eustachian Tube Balloon Dilation.

OBJECTIVE: This study aims to identify clinical predictors of treatment response to Eustachian Tube Balloon Dilation (ETBD) as measured by changes in Eustachian Tube Dysfunction Questionnaire-7 (ETDQ-7) scores. METHODS: One hundred thirteen patients who underwent ETBD at an institution from 2017 to 2021 completed ETDQ-7 pre- and post-operatively. We conducted multivariable regression analyses with ETDQ-7 normalization (<2.1 post-op), minimum clinically important difference (MCID) (>0.5 pre-op - post-op), and quantitative improvement in ETDQ-7 score as outcome variables. Pre-operative ETDQ-7 score, tympanogram type, chronic otitis media, chronic rhinosinusitis (CRS), inferior turbinate hypertrophy, deviated septum, allergic rhinitis, and rhinorrhea were included as covariates. Models controlled for age, sex, ethnicity, prior ear or sinus surgery, and follow-up duration. RESULTS: The mean age was 49 years old. 51% were females, and all patients had pre-operative ETDQ-7 above 2.1. After a mean follow-up period of 13 months, 77% achieved MCID and 37% had normalized. Higher pre-operative ETDQ-7 score was associated with greater ETDQ-7 score improvement (B = 0.60, 95% CI = [0.37, 0.83]) and greater odds of achieving MCID (aOR = 1.65; 95% CI = [1.06, 2.59]). A history of CRS improved chances of achieving MCID (aOR = 4.53; 95% CI = [1.11, 18.55]) and a history of chronic otitis media predicted increased odds of ETDQ-7 normalization (aOR = 2.88; 95% CI = [1.09, 7.58]). CONCLUSIONS: Our findings suggest that ETBD was highly effective among patients with pre-operative ETDQ-7 above 2.1. Furthermore, higher pre-operative ETDQ-7 score, CRS, and chronic otitis media predicted more favorable symptomatic benefit from ETBD. These factors may be important to consider when counseling potential candidates for this procedure.

Balloon dilation for persistent unilateral chronic obstructive Eustachian tube dysfunction is effective: a prospective multicentre study.

PURPOSE: Balloon dilatation of the Eustachian tube (BDET) is an option for treating chronic obstructive Eustachian tube dysfunction (COETD). In this prospective multicentric study, the main objective was to evaluate the results of BDET in unilateral COETD refractory to medical treatment. METHODS: Adults with unilateral COETD whose Eustachian Tube Score (ETS) was less than 5 despite medical pressure therapy were included. The primary endpoint was the change in ETS measured at 2, 6, and 12 months after BDET. Secondary objectives were the evolution of clinical symptoms assessed by the Eustachian Tube Questionnaire (ETDQ-7), audiometry, tympanometry, and otoscopy changes after BDET. RESULTS: Twenty-eight patients were included between May 2014 and December 2017 and were treated with BDET without adverse effects in three different referral centers. Population's median age was 52 (Q25; Q75: 24, 82) years. The median follow-up time was 381 (Q25; Q75: 364; 418) days. The median ETS was 2 (Q25; Q75: 1; 4) before BDET. There was a significant improvement in ETS at 2 and 6 months and 1 year after BDET (score at 1 year: 6 (Q25; Q75: 2; 8) (p < 0.0001)). There was a significant, sustained improvement in the ETDQ-7 with a score of 4.21 (Q25; Q75-3.50; 4.79) before BDET and 3.43 (Q25; Q75-2.43; 4.14) (p = 0.0012) at 1 year. There was a suggestive improvement in tympanometry results at 1 year (p = 0.025). CONCLUSION: BDET provides an improvement in symptoms and objective measures assessed at 1 year in patients with COETD who have failed medical treatment. TRIAL REGISTRATION: NCT02123277 (April 25, 2014).

Clinical analysis of 11 cases of otogenic intracranial complications treated by multidisciplinary collaboration / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.

Balloon Dilation of the Eustachian Tube in Chronic Eustachian Tube Dysfunction: A Retrospective Study of 107 Patients.

BACKGROUND: Eustachian tube dysfunction affects from 1% to 5% of adults. This study evaluates the effectiveness of balloon dilation of eustachian tube for treating nonselected patients with eustachian tube dysfunction in a long term. METHODS: We evaluated all balloon dilation of eustachian tube operations at Turku University Hospital from 2013 to 2016. The data from 107 patients and 167 ears were collected retrospectively and a questionnaire was sent to these patients regarding their symptoms. RESULTS: An evident reduction of the symptoms was noticed. Aural fullness, otalgia, recurrent acute otitis media, or otitis media with effusion as well as symptoms from changes to ambient pressure and the ability to do the Valsalva maneuver were significantly improved. The mean followup time with the questionnaire was 33 months. Of patients, 80% (36/45) reported long-term reduction of symptoms. CONCLUSIONS: In our study, balloon dilation of eustachian tube seems to have reduced symptoms of eustachian tube dysfunction and the results appear to be long lasting.

Balloon Dilation of the Eustachian Tube: A Randomized Controlled Trial with 6 Months Follow-Up.

BACKGROUND: Obstructive Eustachian tube dysfunction in adults is common. The purpose of this study was to examine whether balloon dilation of the Eustachian tube can improve ventilation of the middle ear among adult patients with mild chronic Eustachian tube dysfunction. METHODS: This study included patients aged ≥18 years with unilateral chronic Eustachian tube dysfunction confirmed with an abnormal tympanometry and a retracted tympanic membrane. Patients were treated daily with nasal steroid spray and Valsalva maneuver for 2 months. If Eustachian tube dysfunction persisted, they were enrolled in the study and randomized to balloon dilation of the Eustachian tube or control. All patients underwent otomicroscopy, tympanometry, pure-tone audiometry and the Eustachian Tube Dysfunction Questionnaire-7. Follow-up visits were completed at 3 weeks, 3 months, and 6 months. RESULTS: In total, 24 patients completed the study (13 balloon dilation of the Eustachian tube, 11 control). The balloon dilation of the Eustachian tube group showed normalization from retraction or serous otitis media in 9 out of 13 patients (P = .0006) compared to 0 out of 11 patients in the control group. In the balloon dilation of the Eustachian tube group, 9 out of 13 patients showed an improvement in tympanometry from B to C/A or from C to A (P = .04) compared to 3 out of 11 patients in the control group. The audiometric data showed no difference (P = .38). There was no significant difference in mean Eustachian Tube Dysfunction Questionnaire-7 score between the two groups (P = .35). In the balloon dilation of the Eustachian tube group, 69% answered that they had benefitted from the treatment. CONCLUSION: The procedure is feasible and no complications were reported. The study indicates that balloon dilation of the Eustachian tube may be a beneficial treatment in a selected group of adult patients with mild chronic Eustachian tube dysfunction.

Updates in Eustachian Tube Dysfunction.

Eustachian tube dysfunction (ETD) is a common middle ear disorder in children that can have a significant impact on the quality of life. This review aims to provide an updated understanding of ETD and its clinical management. We will discuss the pathophysiology and diagnosis of ETD, as well as the medical and surgical treatment of ETD. We will also review studies of both adults and children with ETD, although special attention will be paid to children with ETD.

Impact of body mass index in patulous Eustachian tube: Does rapid weight loss influence symptom improvement?

OBJECTIVES: Weight loss has been proposed as risk factor for patulous Eustachian tube (PET), however, it has not been well-characterized how this subpopulation responds to standard treatments. This study aimed to evaluate PET symptom improvement in the setting of and absence of rapid weight loss. METHODS: This retrospective case series included patients diagnosed with PET at an academic institution. Demographic characteristics, medical comorbidities, presenting symptoms, treatment, and outcomes of symptom improvement were reviewed. Univariate analysis modeled the likelihood of symptom improvement between rapid weight loss and non-rapid weight loss patients. RESULTS: A total of 124 patients (median age 55 years, 61 % female) were included. At diagnosis, 7 (5.6 %) patients were underweight, 40 (32.3 %) were normal weight, 32 (25.8 %) were overweight, and 45 (36/3 %) were obese. There were 39 (31.5 %) patients who had history of weight loss prior to presentation; of these, 22 (17.7 %) noted rapid weight loss and 17 (13.7 %) had non-rapid weight loss. There were 62 (50.0 %) patients who were recommended conservative treatment, and 62 (50.0 %) who underwent medical and/or surgical treatment. Symptom resolution was achieved in 49 (39.5 %) patients. On univariate analysis, patients with rapid weight loss were significantly more likely to experience improvement (p = 0.006) than non-rapid weight loss. Rapid weight loss patients had a four-fold increased likelihood of symptom improvement compared to non-rapid weight loss patients (OR = 4.8, p = 0.053). CONCLUSIONS: While rapid weight loss and bariatric surgery are reported risk factors for the development of PET, our findings suggest that patients with rapid weight loss are significantly more likely to achieve symptom improvement than non-rapid weight loss.

Quality Improvement in Otolaryngology-Head and Neck Surgery: Developing Registry-Enabled Quality Measures From Guidelines for Cerumen Impaction and Allergic Rhinitis Through a Transparent and Systematic Process.

BACKGROUND AND SIGNIFICANCE: Quality measurement can drive improvement in clinical care and allow for easy reporting of quality care by clinicians, but creating quality measures is a time-consuming and costly process. ECRI (formerly Emergency Care Research Institute) has pioneered a process to support systematic translation of clinical practice guidelines into electronic quality measures using a transparent and reproducible pathway. This process could be used to augment or support the development of electronic quality measures of the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF) and others as the Centers for Medicare and Medicaid Services transitions from the Merit-Based Incentive Payment System (MIPS) to the MIPS Value Pathways for quality reporting. METHODS: We used a transparent and reproducible process to create electronic quality measures based on recommendations from 2 AAO-HNSF clinical practice guidelines (cerumen impaction and allergic rhinitis). Steps of this process include source material review, electronic content extraction, logic development, implementation barrier analysis, content encoding and structuring, and measure formalization. Proposed measures then go through the standard publication process for AAO-HNSF measures. RESULTS: The 2 guidelines contained 29 recommendation statements, of which 7 were translated into electronic quality measures and published. Intermediate products of the guideline conversion process facilitated development and were retained to support review, updating, and transparency. Of the 7 initially published quality measures, 6 were approved as 2018 MIPS measures, and 2 continued to demonstrate a gap in care after a year of data collection. CONCLUSION: Developing high-quality, registry-enabled measures from guidelines via a rigorous reproducible process is feasible. The streamlined process was effective in producing quality measures for publication in a timely fashion. Efforts to better identify gaps in care and more quickly recognize recommendations that would not translate well into quality measures could further streamline this process.

Otologic Manifestations of Langerhans Cell Histiocytosis: A Systematic Review.

OBJECTIVE: To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management. DATA SOURCES: PubMed/MEDLINE, Embase, and Cochrane Library. REVIEW METHODS: A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. A review of the references of all included articles was also conducted. RESULTS: Sixty-two articles encompassing 631 patients met inclusion criteria. Otologic symptoms at presentation were found in 246 (39%) patients in the reported studies with 48% reporting bilaterality. The mean age was 14.8 years with a male predominance (64%). The most common otologic presenting symptom was otorrhea (46%). A majority had the multisystem variant (52%). The most common treatment modalities were chemotherapy (52%), followed by surgery (50%), systemic steroids (45%), and radiotherapy (31%). Surgery was performed in 75.8% with unisystem involvement and in 50.6% with multisystem involvement. The most effective treatments included radiotherapy (56% success rate, 17% of treated patients), systemic steroids (44% success, 20% treated), chemotherapy (41% success, 21% treated), and surgical modalities (36% success, 19% treated). CONCLUSIONS: Otologic manifestations that occur with the multisystem variant or are at high risk for central nervous system involvement necessitate systemic treatment. For unifocal lesions, surgery is recommended. Lastly, radiotherapy should be reserved for extensive lesions involving vital structures or presenting in older patients.

Middle ear myoclonus: Systematic review of results and complications for various treatment approaches.

OBJECTIVE: To perform a systematic review of the diagnosis and treatment of patients with pulsatile tinnitus secondary to middle ear myoclonus. DATABASES REVIEWED: PubMed, EMBASE, and Scopus. METHODS: A systematic review was performed using standardized methodology. Computerized and manual searches were performed to identify studies of all ages (patients) who had middle ear myoclonus (intervention). All study designs were assessed. Extracted data included demographics, clinical features, duration of followup as well as the diagnosis and reversibility of symptoms with medical or surgical intervention. Studies were included if they included subjects with middle ear myoclonus. Exclusion criteria included letters/commentaries and reviews. RESULTS: Twenty articles representing 115 subjects with middle ear myoclonus were included. The mean age was 29.7 (range 6-67). The follow-up period ranged from 5 weeks to 36 months. Primary treatment consists of medical therapy utilising anxiolytics, antiepileptics, botulinum toxin and surgical treatment involving division of middle ear muscular tendon(s). In total, 60 patients underwent middle ear muscular tenotomies, with division of both stapedius and tensor tympani tendons being the most prevalent (88%). Limitations in the data arose from study design, related comorbidities such as palatal myoclonus, and concomitant drug administration. No study provided any objective criteria to diagnose this condition or evaluate post-treatment outcome. CONCLUSION: Middle ear myoclonus is an entity that is poorly assessed in the literature. There is a lack of consensus regarding the criteria and strategies for both diagnosing and treating this condition. Although level of evidence of current studies remains modest, it is felt that a stepwise approach is deemed best, with therapeutic decisions being made on an individual basis, evaluating each patient's specific circumstances and priorities.