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.

Cuidado del oído y la audición en la atención primaria: manual de capacitación

El diagnóstico precoz de las enfermedades del oído y la pérdida auditiva es fundamental. Casi todas las personas que padecen enfermedades del oído o pérdida de audición se beneficiarían de adoptar medidas oportunas y adecuadas. Muchas de las causas comunes de enfermedades del oído y pérdida de audición, como por ejemplo infecciones de oído o sonidos y ruidos fuertes, se pueden prevenir. El tratamiento y la rehabilitación oportunos pueden beneficiar a todas las personas con enfermedades del oído y pérdida de audición. Los trabajadores de la salud, los médicos generales, los médicos de familia y los médicos que trabajan en atención primaria suelen ser el primer punto de contacto para que las personas reciban servicios de atención de salud. El cuidado del oído y la audición en la atención primaria: manual de capacitación es una guía práctica sobre cómo prevenir, identificar y controlar la pérdida auditiva y las enfermedades comunes del oído que conducen a la pérdida auditiva. Está destinado principalmente a trabajadores de la salud y médicos que trabajan en el nivel de atención primaria y brindan servicios a las personas, ya sea en establecimientos de salud o en comunidades. Está destinado a ser administrado por un formador/instructor familiarizado con los problemas del oído y la audición, su evaluación y tratamiento y va acompañado de un manual para el formador. El manual consta de una serie de módulos independientes. El manual fue desarrollado siguiendo un proceso de consulta basado en evidencia. Los procesos y pasos de diagnóstico y procesos de gestión/referencia descritos en el manual se basan en diversas fuentes de información.

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.

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.

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.

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.

Variant Types of Preauricular Sinuses: Classifications, Clinical Presentation and Management.

OBJECTIVE: To make surgeons aware of the differing types of preauricular sinuses (PAS), we summarize our experience with diagnosis and treatment of varying types of PAS. METHODS: We retrospectively reviewed clinical data from patients who had undergone preauricular fistulectomy between March 2015 and March 2020. These patients were categorized into two groups according to locations of congenital fistula pit. RESULTS: Twelve patients with variant PAS accounted for 6.8% (12/177) of all patients. The variant types of PAS could be classified into three types (from type 1 to type 3), based on the location of the fistula pit. Type 1 (seven patients; eight ears) patients had pits located on the ascending helix crus, whereas type 2 (four patients, four ears) and type 3 (one patient, one ear) patients had pits located on the external auditory canal (EAC) and lobule, respectively. Fistular tracts penetrated the cartilage of the helix crus in seven of the type 1 variant ears. Swelling and discharge were located at the ascending helix crus (in four ears), cavum concha (in two ears), and posterior to the auricle (in one ear). In four of the type 2 ears, the fistular tracts were located at the anterior margin of the ascending limb of the helix. CONCLUSION: Fistula tracts where fistula pit occurred on the ascending helix crus were more likely to penetrates through the cartilage, and fistula tracts with fistula pits that occurred on the EAC were adjacent to the cartilage of the ascending helix and tragus. Meticulous dissection and complete removal of fistula tissue are critical to avoid postoperative recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3839-3845, 2024.

Resolution of severe neurobehavioral difficulties in an individual with Primrose syndrome with sertraline.

Primrose syndrome (PS) is a rare genetic disease characterized by developmental delay, intellectual disability, sensorineural hearing loss, and dysmorphic features. PS is caused by de novo pathogenic variants in the ZBTB20 gene, which encodes a transcription factor modulating neurogenesis. We describe resolution with sertraline of neurobehavioral difficulties in a 17-year-old Hispanic male with PS with de novo heterozygous c.1916G > A (p.C639Y) variant of ZBTB20. Neurobehavioral difficulties included aggression towards self and others, irritability, tearfulness, and mood liability that did not respond to behavioral interventions or aripiprazole. Treatment with sertraline, a medication indicated for psychiatric disorders including anxiety and depression, led to the resolution of neurobehavioral difficulties after 2 weeks of initiation of medication. The treatment course suggests that selective serotonin reuptake inhibitors, such as sertraline, may be a useful tool for neurobehavioral difficulties in PS over antipsychotics that are accompanied by complex side effect profiles, and suggest that anxiety is the primary cause of the neurobehavioral difficulties in this patient.

Making Recommendations for an Evaluation and Treatment Algorithm for Patients with Ear Fullness and No Objective Abnormalities.

OBJECTIVE: To make recommendations for evaluation, approach to counseling and treatment for patients who present with ear fullness without abnormalities on otomicroscopic examination, standard audiometric studies, or imaging results. METHODS: Retrospective chart review of adult patients in a tertiary referral center presenting with ear fullness and/or otalgia without external, middle, and/or inner ear pathologies. Data collected include demographics (age and gender), laterality and duration of symptoms, co-morbid conditions and final diagnoses of temporomandibular joint (TMJ) dysfunction, intermittent Eustachian tube dysfunction (iETD), migraine disorder, and anxiety. RESULTS: In the span of 8 years of a single neurotologist's practice, 964 patients presented with ear fullness. After excluding all instances where external, middle, and inner ear disorders were identified and where audiometric and radiologic findings were abnormal, 263 patients had ear fullness and no objective causes. Women were more likely than men to complain of ear fullness and/or otalgia and were also more likely to present with no objective abnormalities ( p < 0.05). Patients who reported isolated ear fullness were more likely to be diagnosed with iETD, whereas patients who reported pain were more likely to be diagnosed with TMJ dysfunction (TMJD). Fourteen patients (5.3%) had completely unexplained sensation of ear fullness. CONCLUSIONS: There were 94.7% of the patients presenting with unexplained ear fullness were diagnosed as having a possible contribution of TMJ dysfunction, IETD, migraine disorder, anxiety, or a combination of these conditions to their symptomatology. Directing treatments toward these diagnoses may alleviate symptoms of ear fullness or, if unsuccessful, provide an avenue for counseling in the framework of functional neurologic disorders.

When Can Children Perform Valsalva and Toynbee Maneuvers? An Exploratory Study.

OBJECTIVES: Barochallenge-induced Eustachian tube dysfunction (ETD) is difficult to diagnose because the examination is often normal during clinical assessment. In adults, functional tympanometry testing, performed by asking the patient to Valsalva and Toynbee while measuring the pressure shift, can aid in the diagnosis of ETD. However, standardized values do not exist in children. We aim to determine the age at which children can perform these maneuvers and the normative values in this population. METHODS: Patients with a normal basic ear examination 4 years and older, presenting to the pediatric Otolaryngology clinic, were recruited. Otoscopy, baseline tympanometry, followed by Valsalva and Toynbee maneuvers were performed. Because there are no pediatric norms, we hypothesized that children would achieve the same minimum normal pressure shift as cited in the adult literature (+20 daPa or higher for Valsalva and -20 daPa or lower for Toynbee). The data were analyzed using receiver operating characteristic curves and logistic regression. RESULTS: One hundred sixty-eight children (276 ears) were assessed. Participants as young as 4 years old were able to perform a Valsalva and Toynbee. Age cut-offs at which children achieved adult norms were 12.5 years ( p = 0.016) and 8.5 years ( p = 0.071) for Valsalva and Toynbee maneuvers, respectively. Mean pressure shift ranged from +29 to -36 daPa, and males were 2.5 times more likely to achieve Toynbee compared with females ( p = 0.006). CONCLUSIONS: Functional tympanometry testing may be used to help diagnose barochallenge-induced ETD in older children.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

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.

Reconstruction of an obliterated Eustachian tube: transnasal lighted guidewire catheter stenting.

An obliterated Eustachian tube (ET) is a rare occurrence that can lead to chronic otitis media (OM) and aural fullness even with treatment. Our study presents a review of the literature on methods of stenting the ET. We additionally present a case of a man with mucoepidermoid carcinoma of the ET who underwent a radical nasal pharyngectomy with reconstruction and adjuvant radiation, and who had symptoms of intolerable otorrhea after tympanostomy tube placement to treat aural fullness and mucoid OM. We used a novel method of stenting the ET using a transnasal lighted guidewire catheter and steroid eluting stents placed along the entire medial ET. Previously described methods in the literature were unable to be used due to the complex middle ear anatomy filled with granulation tissue and the lack of a visible nasopharyngeal ET ostium available for straightforward placement of the stent. The procedure was successful, and postoperatively, the patient experienced decreased otorrhea.

Chronic otorrhea and osteomyelitis of the external auditory canal by Achromobacter xylosoxidans: an uncommon diagnosis.

PURPOSE: Achromobacter xylosoxidans is an emerging pathogen mainly associated with resistant nosocomial infections. This bacteria had been isolated in the ear together with other pathogens in cultures from patients with chronic otitis media, but it had never been reported as a cause of osteomyelitis of the external auditory canal. CASE PRESENTATION: We present a unique case of a healthy 81-year-old woman who presented with left chronic otorrhea refractory to topical and oral antibiotic treatment. Otomicroscopy revealed an erythematous and exudative external auditory canal (EAC) with scant otorrhea. The tympanic membrane was intact, but an area of bone remodeling with a small cavity anterior and inferior to the bony tympanic frame was observed. Otic culture isolated multi-drug-resistant A. xylosoxidans, only sensitive to meropenem and cotrimoxazole. Temporal bone computed tomography showed an excavation of the floor of the EAC compatible with osteomyelitis. Targeted antibiotherapy for 12 weeks was conducted, with subsequent resolution of symptoms and no progression of the bone erosion. CONCLUSIONS: Atypical pathogens such as A. xylosoxidans can be the cause of chronic otitis externa. Early diagnosis and specific antibiotherapy can prevent the development of further complications, such as osteomyelitis. In these cases, otic cultures play an essential role to identify the causal germ. This is the first case of EAC osteomyelitis due to A. xylosoxidans reported to date.

Cocaine-associated Eustachian tube stenosis causing chronic 'glue ear': a rare cocaine-induced destructive lesion.

BACKGROUND: Cocaine is one of the most used recreational drugs. Whilst medical uses exist, chronic recreational nasal use of cocaine is associated with progressive destruction of the osseocartilaginous structures of the nose, sinuses and palate - termed cocaine-induced midline destructive lesions. CASE REPORT: A 43-year-old male with a history of chronic cocaine use, presented with conductive hearing loss and unilateral middle-ear effusion. Examination under anaesthesia revealed a completely stenosed left Eustachian tube orifice with intra-nasal adhesions. The adhesions were divided and the hearing loss was treated conservatively with hearing aids. Whilst intra-nasal cocaine-induced midline destructive lesions are a well-described condition, this is the first known report of Eustachian tube stenosis associated with cocaine use. CONCLUSION: This unique report highlights the importance of thorough history-taking, rhinological and otological examination, and audiometric testing when assessing patients with a history of chronic cocaine use. This paper demonstrates the complexity of managing hearing loss in such cases, with multiple conservative and surgical options available.

Fungal Otitis Externa (Otomycosis) Associated with Aspergillus Flavus: A Case Image.

A 48-year-old man presented with a chief complaint of intermittent right ear otorrhea of several-month duration, occasional otalgia and progressive unilateral hearing impairment. He also reported frequent episodes of headache and pressure in the sinuses and maxilla. Previous systemic treatment with antibiotics failed to alleviate the symptoms. A head/neck CT showed completely normal mastoid, middle ear and external auditory canal regions without any evidence of opacification or bone erosion. Otoscopic examination of the right ear disclosed aggregates of dried, brown, fibrillar material and debris occluding the external auditory canal and obstructing the otherwise intact tympanic membrane. Dilation of the external auditory canal or thickening of the tympanic membrane were not appreciated. The canal was debrided and the fibrillar material was placed in formalin. Histopathologic examination revealed numerous branching, septated fungal hyphae organized in densely-packed clusters. In other areas, the fungal hyphae abutted or were attached to lamellated collections of orthokeratin. As highlighted by GMS staining, the fungi were morphologically compatible with Aspergillus species. The clinicopathologic findings supported a diagnosis of fungal otitis externa, while the numerous anucleate squamous cells were compatible with colonization of an underlying, probably developing, cholesteatoma. Culture of material isolated from the external auditory canal confirmed the presence of Aspergillus flavus. In this illustrative case, we present the main clinical and microscopic characteristics of Aspergillus-related otomycosis developing in the setting of a tautochronous cholesteatoma.

Safety, tolerability, and effect of a single aural dose of Dornase alfa at the time of ventilation tube surgery for otitis media: A Phase 1b double randomized control trial.

BACKGROUND: One third of children require repeat ventilation tube insertion (VTI) for otitis media. Disease recurrence is associated with persistent middle ear bacterial biofilms. With demonstration that Dornase alfa (a DNase) disrupts middle ear effusion biofilms ex vivo, we identified potential for this as an anti-biofilm therapy to prevent repeat VTI. First, safety and tolerability needed to be measured. METHODS: This was a phase 1B double-blinded randomized control trial conducted in Western Australia. Children between 6 months and 5 years undergoing VTI for bilateral middle ear effusion were recruited between 2012 and 2014 and followed for two years. Children's ears were randomized to receive either Dornase alfa (1 mg/mL) or 0.9 % sodium chloride (placebo) at time of surgery. Children were followed up at 2 weeks post-VTI and at 3-monthly intervals for 2 years. Outcomes assessed were: 1) safety and tolerability, 2) otorrhoea frequency, 3) blocked or extruded ventilation tube (VT) frequency, 4) time to blockage or extrusion, 5) time to infection recurrence and/or need for repeat VTI. RESULTS: Sixty children (mean age 2.3 years) were enrolled with 87 % reaching study endpoint. Treatment did not change otorrhoea frequency. Hearing improved in all children following VTI, with no indication of ototoxicity. Dornase alfa had some effect on increasing time until VT extrusion (p = 0.099); and blockage and/or extrusion (p = 0.122). Frequency of recurrence and time until recurrence were similar. Fourteen children required repeat VTI within the follow-up period. CONCLUSION: A single application of Dornase alfa into the middle ear at time of VTI was safe, non-ototoxic, and well-tolerated. TRIAL REGISTRATION: ACTRN12623000504617.