Barriers to and enablers of the use of the Otology Questionnaire Amsterdam in clinical practice-a qualitative post-implementation study.

BACKGROUND: The Otology Questionnaire Amsterdam (OQUA) is developed to evaluate multiple ear complaints and their impact on patients' daily lives. The current clinical use of this questionnaire is below the potential utilization. AIM: To identify the barriers and enablers of using the OQUA as perceived by ENT surgeons and patients and provide recommendations for an implementation strategy. METHODS: Prospective and qualitative analysis was performed using focus groups and interviews with ENT professionals (n = 15) and patients (n = 25) with ear complaints of one tertiary referral hospital and two regional hospitals. Barriers and enablers were identified and classified by using the Capability-Opportunity-Motivation-Behavior model and the Theoretical Domains Framework. Suggestions for an implementation strategy will be made accordingly. RESULTS: ENT professionals' barriers included lack of knowledge and skills to use the OQUA, inadequate technological support and perceived time constraints during consultation, uncertainty about the clinical relevance and lack of feedback on the outcomes of the OQUA. Enablers included beneficial consequences of the OQUA for the professional, organization and science. Patients' barriers included lack of knowledge about the objective and usefulness of the OQUA, perceived burden, difficulties in completing the questionnaire and insufficient feedback during consultation. Patient enablers included beliefs about beneficial consequences of the OQUA for the patient, health care and society. Suggested interventions involved education, training, environmental restructuring and incentivisation. CONCLUSION: Based on the findings, we propose an implementation strategy should focus on education and training about the objective, outcomes and relevance of the OQUA, environmental restructuring regarding the optimal use of the OQUA, and incentivisation with feedback on the valuable outcomes of the OQUA for the patient, professional and healthcare. Future research is needed to determine the feasibility of the implementation strategy.

Optical coherence tomography otoscope for imaging of tympanic membrane and middle ear pathology.

Significance: Pathologies within the tympanic membrane (TM) and middle ear (ME) can lead to hearing loss. Imaging tools available in the hearing clinic for diagnosis and management are limited to visual inspection using the classic otoscope. The otoscopic view is limited to the surface of the TM, especially in diseased ears where the TM is opaque. An integrated optical coherence tomography (OCT) otoscope can provide images of the interior of the TM and ME space as well as an otoscope image. This enables the clinicians to correlate the standard otoscopic view with OCT and then use the new information to improve the diagnostic accuracy and management. Aim: We aim to develop an OCT otoscope that can easily be used in the hearing clinic and demonstrate the system in the hearing clinic, identifying relevant image features of various pathologies not apparent in the standard otoscopic view. Approach: We developed a portable OCT otoscope device featuring an improved field of view and form-factor that can be operated solely by the clinician using an integrated foot pedal to control image acquisition. The device was used to image patients at a hearing clinic. Results: The field of view of the imaging system was improved to a 7.4 mm diameter, with lateral and axial resolutions of 38 µ m and 33.4 µ m , respectively. We developed algorithms to resample the images in Cartesian coordinates after collection in spherical polar coordinates and correct the image aberration. We imaged over 100 patients in the hearing clinic at USC Keck Hospital. Here, we identify some of the pathological features evident in the OCT images and highlight cases in which the OCT image provided clinically relevant information that was not available from traditional otoscopic imaging. Conclusions: The developed OCT otoscope can readily fit into the hearing clinic workflow and provide new relevant information for diagnosing and managing TM and ME disease.

[Auricular and periauricular pathologies in the ENT department of Sylvanus Olympio Teaching Hospital in Lome (Togo)]. / Pathologies auriculaires et périauriculaires au service d'ORL du Centre hospitalier universitaire Sylvanus Olympio de Lomé (Togo).

Objective: The aim of this study is to describe the epidemiological aspects, clinical features and principles of management of different types of auricular and peri-auricular pathologies. Patients and Methods: This is a 5-year retrospective descriptive study (from May 1, 2018 to April 30, 2023) of the records of patients who consulted for a functional or cosmetic complaint relating to the auricle or periauricular region in the ENT and cervico-facial surgery department of the Centre hospitalier universitaire Sylvanus Olympio. Results: A total of 159 cases over 5 years, i.e. an annual frequency of 31 cases, met the study criteria. The mean age of the patients was 22.2 years. Children and students accounted for 24.5% and 23.9% of cases respectively. Auricular pathologies accounted for 64.8% of cases and peri-auricular pathologies for 36.2%.Tumors and trauma accounted for 33.3% and 29.6% of cases respectively, and congenital pathologies were found in 29.9% of cases. Among traumatic lesions, intentional assault and battery was the cause in 21.3%, followed by road accidents in 17.2%. The right ear was affected in 48% and the lobule in 40.4%. Keloids accounted for 17.6% of all cases, and 53% of tumors and pseudotumors. The left ear was involved in 50% of cases. Piercing was the cause of keloids in 10.7% of cases. Conclusion: Auricular and peri-auricular pathologies were dominated by benign tumors, trauma and congenital pathologies, and involved young subjects. Management is based on the type of lesion, with functional and aesthetic considerations in mind.

Artificial Intelligence for Diagnosis in Otologic Patients: Is It Ready to Be Your Doctor?

OBJECTIVE: Investigate the precision of language-model artificial intelligence (AI) in diagnosing conditions by contrasting its predictions with diagnoses made by board-certified otologic/neurotologic surgeons using patient-described symptoms. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary care center. PATIENTS: One hundred adults participated in the study. These included new patients or established patients returning with new symptoms. Individuals were excluded if they could not provide a written description of their symptoms. INTERVENTIONS: Summaries of the patient's symptoms were supplied to three publicly available AI platforms: Chat GPT 4.0, Google Bard, and WebMD "Symptom Checker." MAIN OUTCOME MEASURES: This study evaluates the accuracy of three distinct AI platforms in diagnosing otologic conditions by comparing AI results with the diagnosis determined by a neurotologist with the same information provided to the AI platforms and again after a complete history and physical examination. RESULTS: The study includes 100 patients (52 men and 48 women; average age of 59.2 yr). Fleiss' kappa between AI and the physician is -0.103 (p < 0.01). The chi-squared test between AI and the physician is χ2 = 12.95 (df = 2; p < 0.001). Fleiss' kappa between AI models is 0.409. Diagnostic accuracies are 22.45, 12.24, and 5.10% for ChatGPT 4.0, Google Bard, and WebMD, respectively. CONCLUSIONS: Contemporary language-model AI platforms can generate extensive differential diagnoses with limited data input. However, doctors can refine these diagnoses through focused history-taking, physical examinations, and clinical experience-skills that current AI platforms lack.

External Auditory Canal Erosion at the 6 O'clock Spot.

OBJECTIVE: To report a common site of external ear canal erosion in multiple pathologies, located inferiorly at 6 o'clock. PATIENTS: Otology patients who came in 2023 for treatment of external auditory canal erosions. INTERVENTION: This clinical capsule is an observational report of the external canal's propensity to erosion at the 6 o'clock location. Patient treatments were canalplasty, mastoidectomy, and medical management. MAIN OUTCOME MEASURE: Documentation of the propensity to erosion at the 6 o'clock location in the external auditory canal. Locations of the niduses of prior series of external auditory canal pathologies are documented. RESULTS: Eight patients are presented with external auditory canal erosion in 10 ears originating at the 6 o'clock position medial to the bony-cartilaginous junction. No other patient with spontaneous canal erosion presented with their nidus of pathology in another canal location. (A review of 42 case series of 291 patients found that keratosis obturans and bisphosphonate-induced osteonecrosis tended to arise from the same 6 o'clock lateral bony canal location, while 26% of necrotizing otitis externa cases arose there.). CONCLUSIONS: The "6 o'clock spot" in the external canal is a common location of canal erosion for spontaneous wax and keratin collections and may be the precursor to keratosis obturans, bisphosphonate-induced osteonecrosis of the ear canal, and necrotizing otitis externa.

Red ear syndrome: a case series and review of the literature.

BACKGROUND: Red Ear Syndrome is a burning sensation and erythema of the ear, associated with a various number of disorders including migraine, trigeminal neuralgia, autoimmune disorders etc. Theories for RES pathophysiology have developed from current understandings of comorbid conditions. Characterizing the underlying mechanism of RES is crucial for defining effective treatments. CASE PRESENTATION: Three caucasian patients, ages 15, 47, and 67 years, with migraine, one with erythromelalgia are reported in this manuscript. RES pathophysiology is not fully understood due to its variable clinical presentation and numerous comorbid conditions, making it difficult to identify effective treatments. CONCLUSION: RES seems to be largely treatment-resistant, and most options involve treating the associated disorders and minimizing pain. Further investigation of future cases should lead to a more comprehensive understanding of the fundamental cause of RES and, hopefully, successful treatments.

Otology: Cerumen Impaction and Aural Foreign Bodies.

Cerumen lubricates and protects the external auditory canal, but excess accumulation can lead to ear fullness, itching, otalgia, discharge, hearing loss, and tinnitus. Cerumen should be treated whenever symptoms are present or if it limits diagnosis by preventing a needed otoscopic examination. Clinicians should evaluate for cerumen impaction in those using hearing aids and patients with intellectual disability. Cerumen impaction can be treated with cerumenolytics, ear irrigation, and manual removal with instrumentation. Aural foreign bodies can cause ear fullness, otalgia, discharge, and hearing loss. They are more common in children than adults. The most common type of aural foreign bodies in children is jewelry, followed by paper products, parts of pens or pencils, desk supplies (eg, erasers), BBs or pellets, and earplugs or earphones. In adults, the most common aural foreign bodies are cotton swabs or cotton, followed by hearing aid parts and jewelry or ear accessories. Patients should avoid using cotton tip applicators in the external auditory canal. Alligator forceps, small right angle hooks, and ear irrigation commonly are used to remove aural foreign bodies in an outpatient clinic setting, but the choice depends on the type of foreign body. Soft and irregularly shaped objects can be removed without referral to an otolaryngologist. Patients with hard, spherical, or cylindrical objects should be referred to an otolaryngologist if previous removal attempts have failed or if there is ear trauma to avoid worsening its position in the ear canal.

[The population morbidity with diseases of ear and mastoid and accessibility of otorhinolaryngological care].

The article analyses level and dynamics of morbidity of diseases of ear and mastoid in the Sakha Republic (Yakutia) in 2020-2021 and availability of otorhinolaryngological care. The methods of comparative statistics and mathematical analysis were applied to analyze official data provided by the Yakut Republic Medical Information and Analytical Center, the specialized Department of Otorhinolaryngology, the Republic Hospital № 2 - Center for Emergency Medical Care and the Federal State Statistics Service of Russia. The study established increasing trend of increasing morbidity of these diseases in both the adult and child population. In 2021, the growth rate of overall morbidity of adult population reached 17.7% and 8.8% in children, as compared to 2020. The primary morbidity of adults made up to 22.3%, in children - 15.7%. The comparative analysis demonstrated higher rates of general morbidity in the Republic: by 0.5% as compared with the Russian Federation and by 14.1% as compared with the Far Eastern Federal Okrug. The level of primary morbidity was lower than similar indicators of the compared territories by 17.1% and 3.0%, respectively. It is worth noting that analyzed morbidity of diseases of ear and mastoid reflects prevalence of ENT diseases in the region only indirectly, as the statistical data do not allow to estimate separately rate of upper respiratory tract lesions. Meanwhile, respiratory diseases rank first in the structure of population diseases in Yakutia. The growth of disability in children due to diseases of ear and mastoid requires attention. Among children of 0-17 years old, the indicator of primary disability increased from 0.38 to 0.8 per 10,000 of children population (increase of 110.5%); in children 0-3 years old - from 0.9 to 2.3 per 10,000 of the child population (an increase of 155.6%). The analysis of the number of beds in otorhinolaryngology wards established that that the bed capacity per 10,000 population was 0.6 that is significantly lower than the established standards. The article emphasizes need to adjust the Federal standards for hospital bed capacity, taking into account climatic and geographical conditions of the region, which contribute to spread and chronization of ENT diseases.

Novel GNAI3 mutation in a Chinese family with auriculocondylar syndrome and treatment of severe dentofacial deformities: a 5-year follow-up case report.

BACKGROUND: Auriculocondylar syndrome (ARCND) is an extremely rare autosomal dominant or recessive condition that typically manifests as question mark ears (QMEs), mandibular condyle hypoplasia, and micrognathia. Severe dental and maxillofacial malformations present considerable challenges in patients' lives and clinical treatment. Currently, only a few ARCND cases have been reported worldwide, but most of them are related to genetic mutations, clinical symptoms, and ear correction; there are few reports concerning the treatment of dentofacial deformities. CASE PRESENTATION: Here, we report a rare case of ARCND in a Chinese family. A novel insertional mutation in the guanine nucleotide-binding protein alpha-inhibiting activity polypeptide 3 (GNAI3) was identified in the patient and their brother using whole-exome sequencing. After a multidisciplinary consultation and examination, sequential orthodontic treatment and craniofacial surgery, including distraction osteogenesis and orthognathic surgery, were performed using three-dimensional (3D) digital technology to treat the patient's dentofacial deformity. A good prognosis was achieved at the 5-year follow-up, and the patient returned to normal life. CONCLUSIONS: ARCND is a monogenic and rare condition that can be diagnosed based on its clinical triad of core features. Molecular diagnosis plays a crucial role in the diagnosis of patients with inconspicuous clinical features. We present a novel insertion variation in GNAI3, which was identified in exon 2 of chromosome 110116384 in a Chinese family. Sequential therapy with preoperative orthodontic treatment combined with distraction osteogenesis and orthognathic surgery guided by 3D digital technology may be a practical and effective method for treating ARCND.

Efficiency of ear molding for treating constricted ears of different severity.

PURPOSE: To investigate the treatment time and efficiency of constricted ears of different severity after correction. MATERIALS AND METHODS: We included the patients with constricted ear presented to our hospital for treatment between December 2021 and December 2023 in this retrospective analysis. The patients were divided into class I, II and III groups based on the severity of the constriction. Then we collected the data on classification of severity from each patient, together with sex, family history, age at initial correction, being informed upon diagnosis after birth, as well as utilization of auricle correction system. Logistic regression analysis was performed to identify the factors associated with the treatment time and efficiency. RESULTS: The correction system yielded a high effective rate in the constricted ears. The treatment time in class II was significantly longer compared with those of class I after adjusting these parameters. Compared with the cases of class I, those with a class III showed significant attenuation in the symptoms and conditions (95 % CI: 0.034, 0.365; P < 0.001), after adjusting the age at initial correction, being informed upon diagnosis after birth, and utilization of auricle correction system. There were no statistical differences between class II and III in the treatment efficiency after correction. CONCLUSIONS: The Amazing Ear Correction System was effective in treating constricted ear, yielding satisfactory treatment efficiency. Patients with class II constriction required longer treatment time compared with those of class I. The treatment outcome in the class I constriction was better than that of class III.

Quality of life and level of physical activity of individuals with temporomandibular disorders with and without otological symptoms: Secondary analysis of a cross-sectional study.

BACKGROUND AND PURPOSE: Otological symptoms (OS) are highly prevalent in individuals with temporomandibular disorders (TMD). Individuals with TMD and OS have more neck disability and decreased deep neck muscles endurance when compared to individuals without OS. However, no studies have evaluated whether OS is associated with lower Quality of Life (QoL) and worse levels of physical activity. This study aimed to evaluate the QoL and level of physical activity of individuals with TMD with and without OS. METHODS: In this cross-sectional study, 62 individuals with TMD were allocated into 2 groups: TMD with OS (n = 36) or TMD without OS (n = 26). Self-reported complaints of dizziness, vertigo, tinnitus, earache, ear fullness, or hypoacusis were considered as OS. QoL was assessed with the WHOQOL-Bref and physical activity with the IPAQ-SF. Independent t-test and chi-squared test were used for analysis between-groups. Effect sizes were reported using Cohen's d. A Pearson correlation was used to compare the number of OS and QoL scores. A significance level of p < 0.05% and 95% confidence intervals were considered statistically significant. RESULTS: The total generic scores for QoL were not different between-groups (p = 0.076), but individuals with TMD with OS had lower satisfaction (p = 0.015; d: 0.63) and physical domain (p = 0.015; d: 0.64) scores with a moderate effect size. In TMD with OS, 69.4% of individuals were irregularly active and 50% for the TMD without OS, with no statistical significance (p > 0.05). The number of OSs was inversely and weakly associated with the QoL total score. CONCLUSION: Individuals with TMD and OS are associated with worsened QoL (physical domain and satisfaction) when compared to individuals with TMD without OS. The higher the number of OS, the worse the QoL score. Individuals with TMD with and without OS had similar levels of physical activity, but a high prevalence of irregularly active and sedentary individuals within TMD diagnosed population was found.

A New Community-to-Hospital Specialist Otology Pathway Using Telemedicine: A Pilot Study of 50 Patients.

INTRODUCTION: Pathway innovation using smartphone otoscopy and tablet-based audiometry technologies to deliver ear and hearing services via trained audiologists may improve efficiency of the service. An ENT-integrated-community-ear service (ENTICES-combining community audiology management, remote ENT review and novel technologies) was piloted. We aimed to assess the efficiency and safety of ENTICES. METHOD: ENTICES was a community-based and audiologist-led pathway. Patients with otological symptoms were self-referred to this service. Smartphone otoscopy and tablet-based audiograms were performed. Two otologists reviewed all decisions made in the community by audiologists based on video-otoscopy, hearing tests and chart reviews. Data on the first 50 consecutive new patients attending either consultant-led hospital otology clinics (HOC), audiologist-led hospital advanced audiology diagnostics (AAD) or ENTICES clinics were collected between 1 August 2021 and 31 December 2021. Data were collected through chart reviews and questionnaires to compare the three pathways with respect to efficiency, patient satisfaction, technology utility and safety. RESULTS: No audiology-led ENTICES decisions were amended by hospital otologists following remote review. Remote review of video-otoscopy with history was sufficient for a diagnosis in 80% of cases. Adding hearing tests and standardised history increased the diagnostic yield to 98%. Patient satisfaction scores showed 100% service recommendation. The cost per patient, per visit, was £83.36, £99.07 and £69.72 for AAD, HOC or ENTICES, respectively. CONCLUSION: ENTICES provides a safe ear and hearing service that patients rated highly. Thirty-two per cent of hospital otology patients were eligible for this service. For those patients, ENTICES is 20% more cost-effective and can reduce the number of clinic visits by up to 60% compared with HOC.

Clinical application of high-resolution spiral CT scanning in the diagnosis of auriculotemporal and ossicle.

Precision and intelligence in evaluating the complexities of middle ear structures are required to diagnose auriculotemporal and ossicle-related diseases within otolaryngology. Due to the complexity of the anatomical details and the varied etiologies of illnesses such as trauma, chronic otitis media, and congenital anomalies, traditional diagnostic procedures may not yield accurate diagnoses. This research intends to enhance the diagnosis of diseases of the auriculotemporal region and ossicles by combining High-Resolution Spiral Computed Tomography (HRSCT) scanning with Deep Learning Techniques (DLT). This study employs a deep learning method, Convolutional Neural Network-UNet (CNN-UNet), to extract sub-pixel information from medical photos. This method equips doctors and researchers with cutting-edge resources, leading to groundbreaking discoveries and better patient healthcare. The research effort is the interaction between the CNN-UNet model and high-resolution Computed Tomography (CT) scans, automating activities including ossicle segmentation, fracture detection, and disruption cause classification, accelerating the diagnostic process and increasing clinical decision-making. The suggested HRSCT-DLT model represents the integration of high-resolution spiral CT scans with the CNN-UNet model, which has been fine-tuned to address the nuances of auriculotemporal and ossicular diseases. This novel combination improves diagnostic efficiency and our overall understanding of these intricate diseases. The results of this study highlight the promise of combining high-resolution CT scanning with the CNN-UNet model in otolaryngology, paving the way for more accurate diagnosis and more individualized treatment plans for patients experiencing auriculotemporal and ossicle-related disruptions.

A Novel Method for Patients With Ear Keloid: Enucleation and Fat Grafting.

BACKGROUND: Keloid is a dermal fibroproliferative disease unique to humans. Due to the ambiguity in its pathophysiology and the frequent recurrence of keloid, there is no clear consensus on the treatment of keloid and there are many treatment methods defined. In order to benefit from the positive effects of fat grafting on pathological scars, we applied fat grafting to patients who underwent keloid enucleation. METHODS: Fifteen ear keloid patients included in the study. All patients underwent the same surgical procedure by the same surgeon. Routine follow-ups and examinations were performed to evaluate the results and in addition, the Patient and Observer Scar Assessment Scale (POSAS) survey was used. RESULTS: In the study, 15 patients were followed for a median (IQR) period of 21 (13-28) months. No recurrence was observed in any patient during follow-up, which occurred for a median of 21 (13-28) months. In the questionnaire filled out by the patients, the preoperative median value was found to be 48 (IQR: 12), whereas the postoperative median value was found to be 14 (IQR: 8). According to the patients, there was a statistically significant ( P < 0.05) positive improvement after surgery. CONCLUSIONS: Historically, surgical procedures were avoided because the surgical recurrence rate was very high, but today, recurrence rates are decreasing with combined treatments. These treatment combinations may require more than one intervention and require frequent clinical follow-ups. With our technique of fat grafting after enucleation, the treatment was completed with a single operation and no additional intervention was required.

Toward Automating Diagnosis of Middle- and Inner-ear Mechanical Pathologies With a Wideband Absorbance Regression Model.

OBJECTIVES: During an initial diagnostic assessment of an ear with normal otoscopic exam, it can be difficult to determine the specific pathology if there is a mechanical lesion. The audiogram can inform of a conductive hearing loss but not the underlying cause. For example, audiograms can be similar between the inner-ear condition superior canal dehiscence (SCD) and the middle-ear lesion stapes fixation (SF), despite differences in pathologies and sites of lesion. To gain mechanical information, wideband tympanometry (WBT) can be easily performed noninvasively. Absorbance , the most common WBT metric, is related to the absorbed sound energy and can provide information about specific mechanical pathologies. However, absorbance measurements are challenging to analyze and interpret. This study develops a prototype classification method to automate diagnostic estimates. Three predictive models are considered: one to identify ears with SCD versus SF, another to identify SCD versus normal, and finally, a three-way classification model to differentiate among SCD, SF, and normal ears. DESIGN: Absorbance was measured in ears with SCD and SF as well as normal ears at both tympanometric peak pressure (TPP) and 0 daPa. Characteristic impedance was estimated by two methods: the conventional method (based on a constant ear-canal area) and the surge method, which estimates ear-canal area acoustically.Classification models using multivariate logistic regression predicted the probability of each condition. To quantify expected performance, the condition with the highest probability was selected as the likely diagnosis. Model features included: absorbance-only, air-bone gap (ABG)-only, and absorbance+ABG. Absorbance was transformed into principal components of absorbance to reduce the dimensionality of the data and avoid collinearity. To minimize overfitting, regularization, controlled by a parameter lambda, was introduced into the regression. Average ABG across multiple frequencies was a single feature.Model performance was optimized by adjusting the number of principal components, the magnitude of lambda, and the frequencies included in the ABG average. Finally, model performances using absorbance at TPP versus 0 daPa, and using the surge method versus constant ear-canal area were compared. To estimate model performance on a population unknown by the model, the regression model was repeatedly trained on 70% of the data and validated on the remaining 30%. Cross-validation with randomized training/validation splits was repeated 1000 times. RESULTS: The model differentiating between SCD and SF based on absorbance-only feature resulted in sensitivities of 77% for SCD and 82% for SF. Combining absorbance+ABG improved sensitivities to 96% and 97%. Differentiating between SCD and normal using absorbance-only provided SCD sensitivity of 40%, which improved to 89% by absorbance+ABG. A three-way model using absorbance-only correctly classified 31% of SCD, 20% of SF and 81% of normal ears. Absorbance+ABG improved sensitivities to 82% for SCD, 97% for SF and 98% for normal. In general, classification performance was better using absorbance at TPP than at 0 daPa. CONCLUSION: The combination of wideband absorbance and ABG as features for a multivariate logistic regression model can provide good diagnostic estimates for mechanical ear pathologies at initial assessment. Such diagnostic automation can enable faster workup and increase efficiency of resources.

Auriculocondylar syndrome 2 caused by a novel PLCB4 variant in a male Chinese neonate: A case report and review of the literature.

BACKGROUND: Auriculocondylar syndrome (ARCND) is a rare congenital craniofacial developmental malformation syndrome of the first and second pharyngeal arches with external ear malformation at the junction between the lobe and helix, micromaxillary malformation, and mandibular condylar hypoplasia. Four subtypes of ARCND have been described so far, that is, ARCND1 (OMIM # 602483), ARCND2 (ARCND2A, OMIM # 614669; ARCND2B, OMIM # 620458), ARCND3 (OMIM # 615706), and ARCND4 (OMIM # 620457). METHODS: This study reports a case of ARCND2 resulting from a novel pathogenic variant in the PLCB4 gene, and summarizes PLCB4 gene mutation sites and phenotypes of ARCND2. RESULTS: The proband, a 5-day-old male neonate, was referred to our hospital for respiratory distress. Micrognathia, microstomia, distinctive question mark ears, as well as mandibular condyle hypoplasia were identified. Trio-based whole-exome sequencing identified a novel missense variant of NM_001377142.1:c.1928C>T (NP_001364071.1:p.Ser643Phe) in the PLCB4 gene, which was predicted to impair the local structural stability with a result that the protein function might be affected. From a review of the literature, only 36 patients with PLCB4 gene mutations were retrieved. CONCLUSION: As with other studies examining familial cases of ARCND2, incomplete penetrance and variable expressivity were observed within different families' heterozygous mutations in PLCB4 gene. Although, motor and intellectual development are in the normal range in the vast majority of patients with ARCND2, long-term follow-up and assessment are still required.

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.

Otologic disease trends in Japan post-COVID-19 outbreak: A retrospective time-series analysis.

OBJECTIVE: To evaluate the potential impact of coronavirus disease 2019 (COVID-19) and vaccinations on otologic diseases, including facial nerve paralysis (including Ramsay Hunt syndrome), vestibular neuritis, sudden sensorineural hearing loss, and Meniere's disease. METHODS: In this retrospective study, we conducted a time-series analysis employing a causal impact algorithm on a large-scale inpatient database in Japan. We compared the actual number of hospitalized patients with otologic diseases to two predictions: one without any covariates and another with a covariate accounting for the reduction in the number of hospitalized patients due to lockdown measures. Additionally, we performed Granger causality tests to ensure the robustness of our findings. RESULTS: No significant increase was noted in the number of hospitalized patients with otologic diseases following the onset of the COVID-19 pandemic in the causal impact analysis. Similarly, no notable surge was observed in hospitalizations for these diseases following the introduction of the COVID-19 vaccine. The Granger causality tests results aligned with the causal impact analysis findings. CONCLUSION: Our findings indicate that COVID-19 and vaccinations had minimal discernible effects on hospitalization of patients with otologic diseases, suggesting that otologic diseases may not be significantly impacted by COVID-19 and vaccinations, which could have implications for public health policies and the allocation of healthcare resources during a pandemic. Further research and monitoring of long-term effects are warranted to validate these findings and guide healthcare decision-making.