An Uncommon Culprit: Dural Arteriovenous Fistula Presenting as Otodynia and Dizziness.

Dural arteriovenous fistulas (DAVFs) are rare vascular abnormalities that can present with diverse neurological symptoms. We report a case of a woman in her early 60s who presented with pain in the left ear and dizziness. Neurological evaluation and imaging studies revealed a DAVF in the left cerebellopontine angle. This case underscores the importance of considering DAVF as a potential etiology in patients presenting with atypical otological symptoms.

Pilomatricoma: A Case Report.

Pilomatricoma is one of the rare skin benign neoplasms arising from the matrix cells of hair follicles. We report the case of a 17-year-old female who had a swelling over the medial surface of the right ear lobule, which was determined to be an epidermal inclusion cyst by radiological and clinical examination. Over the period of eight days, it grew to 2 × 2 cm. A well-defined encapsulated lesion with thick cellular debris was found on an ultrasonography of the local region. Under local anesthesia, the patient had a cyst excision. Early detection by ultrasonography is beneficial in skin lesions to confirm if they are benign or malignant. Complete excision of the cyst is the treatment in order to limit the morbidity and lower the aggressive behavior. In this case, we came to a diagnosis after a histopathological examination, confirming it as a pilomatricoma. Due to its rarity, it is often misdiagnosed.

Taste Disorders After Middle Ear Surgery: Chorda Tympani Nerve Injury and Quality of Life.

OBJECTIVE: To evaluate taste disorders after middle ear surgery, their modifying factors especially chorda tympani nerve injury or underlying otologic disease. We investigated consequences of taste disorders on quality of life. STUDY DESIGN: Prospective cohort study. SETTING: Monocentric study in tertiary care center. METHODS: A total of 214 patients who underwent middle ear surgery were included. Data regarding taste disorders were collected by questionnaires over a 1-year follow-up period. RESULTS: Taste disorders were reported in 42.7% at 10 days, in 23.3% at 4 months, and in 9.2% 1 year postoperatively. When the chorda tympani nerve was initially healthy, taste disorders were more frequent after its transection throughout the follow-up period. When it was involved in a cholesteatoma or inflammatory process, postoperative taste disorders were more frequent after nerve stretching. Postoperative discomfort in daily life was rated on the Likert scale at 3.5 out of 10. Dietary modifications were reported by 25.8% of patients, and mood alterations by 15% of patients. CONCLUSION: Taste disorders are frequent after middle ear surgery although they mostly improve in the first months. When the CTN is healthy, cutting it leads to more taste disorders than stretching it, thus advocating its preservation to prevent these symptoms. However, in cases of pathological CTN, cutting this nerve, which is sometimes necessary to control the disease, is less likely to cause taste disorders than stretching it. These taste disorders are a source of discomfort and may present risks of dietary modifications and emotional impact.

Development of Magnetic Nanosystems with Potential for the Treatment of Inner Ear Pathologies.

Hearing loss (HL) affects more than 5% of the global population, with projections indicating an impact of up to 50% on young individuals in the next years. HL treatments remain limited due to the inner ear's hermeticism. HL often involves inflammatory processes, underscoring the need for enhanced delivery of antiinflammatory agents to the inner ear. Our research focuses on the development of a directed therapy based on magnetic nanoparticles (MNPs). We previously synthesized biocompatible folic acid-coated iron oxide-core nanoparticles (MNPs@FA) as potential carriers for the anti-inflammatory Diclofenac (Dfc). This study aims to incorporate Dfc onto MNPs@FA to facilitate targeted drug delivery to the inner ear. Through optimizing the loading procedure, we achieved optimal loading capacity. Dfc release was studied in the simulated target fluid and the administration vehicle. Complete characterization is also shown. In vitro biocompatibility testing ensured the biosafety of the resulting formulation. Subsequent ex vivo targeting assays on murine cochleae validated the nanosystems' ability to penetrate the round window membrane, one of the main HL therapy barriers. These findings serve as validation before continuing to more complex in vivo studies. Together, the data here presented represent an advancement in addressing unmet medical needs in HL therapy.

Ear Dyskinesia in the Absence of Neuroleptics: A Case Report.

Ear dyskinesia, also known as "moving ear syndrome," is a rare movement disorder characterized by involuntary, rhythmic, or semi-rhythmic contractions of the external ear muscles. The condition is not well-documented in the medical literature, with only a few case reports available. We present the case of a 37-year-old teacher from Saudi Arabia who developed a history of sudden, progressive involuntary movement of the posterior head region, provoking movement of the external ears, over the course of one year. The movements were non-rhythmical, more prominent on the right side, and associated with occasional involvement of the face and anterior neck muscles. The patient had no history of neuroleptic use or other relevant medical conditions. Examination confirmed the presence of palpable muscle contractions originating mainly from the posterior region, with the movements not synchronized across the two sides. Investigations, including blood tests and brain MRI, did not reveal any underlying pathology. A diagnosis of ear dyskinesia was made, and botulinum toxin treatment was recommended; however, the treatment showed no results, and then the patient was subsequently lost to follow-up. This case adds to the limited literature on the rare phenomenon of ear dyskinesia, highlighting the clinical presentation and the challenges in the management of this unusual movement disorder. Further research is needed to better understand the underlying mechanisms and optimal treatment approaches for this condition.

3D 4K Exo-Endoscopic Temporal Bone Dissection: A Novel Approach for Sharing the Anatomy.

BACKGROUND: Temporal bone dissection is overwide recognized as an ideal training method for otologic surgeons. The knowledge of temporal bone anatomy and especially of the course of infratemporal facial nerve is pivotal in practice. The 3D exoscope is an innovative and promising tool, that was recently introduced in ear surgery. METHODS: A high-definition 3D exoscope (3D VITOM®) mounted on the VERSACRANETM holding system (Karl Storz) was used to perform two temporal bone dissection, with the aim to study the anatomy of infratemporal facial nerve. The 3D endoscope (TIPCAM®1 S 3D ORL, Karl Storz) was used in combination to provide a close-up high-quality view and to provide a different angle of view on fine anatomical relationships. RESULTS: The high-definition 3D exoscope allowed to conduct the dissection with high quality visualization and to share the same surgical field with trainees. Moreover, it showed a high interchangeability with the 3D endoscope. CONCLUSIONS: 3D 4 K Exo-endoscopic temporal bone dissection seems to have benefits in terms of educational purpose, especially concerning anatomy understanding. The superiority in teaching value of this tool should be further investigated in cohort studies.

Applicability of ChOLE staging to preoperative HRCT temporal bone in chronic otitis media with cholesteatoma.

PURPOSE: Chronic otitis media with cholesteatoma is a frequent disease entity in otology, requiring surgery in overwhelming majority of cases. Despite the huge burden there is no established grading system available to assess the severity and extent of disease preoperatively until date. Aim of our study is to assess the applicability of ChOLE staging to preoperative HRCT temporal bone in Chronic otitis media with cholesteatoma. METHODOLOGY: Patients clinically diagnosed as COM with cholesteatoma, who underwent preoperative HRCT temporal bone imaging and mastoid exploration at our tertiary care centre were included. Preoperative radiology was assessed and a radiological ChOLE (r-ChOLE) was given by radiologist. This was then compared with the postop ChOLE. RESULTS: 21 patients were included in the study. Data was linear and normally distributed (Shapiro wilk test). Pearson's product-moment correlation used to see relationship between radiological and postop Total score showed strong statistically significant positive correlation with correlation coefficient (r) of 0.977. Paired t test showed p value was 0.329 (p > 0.05) suggesting no significant difference between radiological and postop Total scores. Cohen kappa test of agreement was applied. It revealed an overall strong agreement (p < 0.001). CONCLUSION: ChOLE staging may be extended to preoperative HRCT of temporal bone in COM with cholesteatoma (rCHOLE). A preoperative radiological staging will help in better prioritizing, planning and execution of tympanomastoid surgeries.

Resolution of a chronic occipital wound with exposed skull bone with a fish skin graft: a successful treatment approach.

Chronic skin defects in the head, face and neck pose challenges for closure, especially after multiple surgeries or radiation therapy. We report the case of a woman in her 70s with a chronic occipital wound following squamous cell carcinoma resections, resulting in exposed skull bone. Despite various options, we successfully treated the 4 cm x 5 cm wound with a Kerecis fish skin graft (FSG), observing significant improvement within a week. The FSG promoted granulation tissue formation, enabling subsequent full-thickness skin grafting from the patient's groin. Complete wound closure was achieved within 2 weeks, indicating FSG's efficacy in complex wound management. Our experience highlights FSG's potential as a valuable tool in wound healing and reconstruction, particularly in challenging cases involving the head and neck.

A Clinicopathological Study of Aural Polyps: A Retrospective Analysis in a Tertiary Care Hospital.

Introduction Aural polyps are a misnomer. Any lesion can present as a mass in the external auditory canal. Aural polyps are proliferation of the granulation tissue due to long standing inflammatory process with associated otalgia and otorrhea. Objectives To document the clinicoradiological presentations, intraoperative findings, and histopathological diagnosis of aural polyp, correlating them. Methods In our study 81 patients underwent treatment for aural polyps in the department of Ear, Nose, and Throat (ENT) from April 1997 to April 2022. Results were tabulated, a simple descriptive analysis was done using the Statistical Package Social Sciences software, and the results obtained were represented as percentages and presented in tables. Results The majority (38) of the patients presenting with aural polyps were diagnosed with mucosal and squamous type of CSOM, and 22 with simple granulation polyps. There were also 5 patients with malignant otitis externa, 3 patients had glomus tumors, 2 patients with retained foreign bodies, and 3 patients with brain herniation. We also identified aberrant internal carotid artery, high jugular bulb, one patient had facial nerve neuroma, one patient had polyp from the tragus diagnosed with tuberculosis, one patient with keratosis obturans, and one with exostosis. Conclusion A thorough detailed examination and mastoid exploration with radiological and histopathological evaluation is mandatory for better defining the definitive treatment. Utmost care and meticulousness are advised for the surgeons while dealing with aural polyps to avoid any complications.

Pediatric Myringoplasty using the Periosteum: An Institutional Overview.

Introduction Myringoplasty is a common otologic procedure to restore the integrity of the tympanic membrane in cases of traumatic or pathologic perforations. Many grafting materials have been used with different techniques. Objective In the present work, we evaluate the surgical and audiological outcomes of periosteal graft overlying the mastoid cortex through a retroauricular incision in a pediatric cohort. Methods A retrospective study was carried out involving all children aged ≤ 16 years who underwent periosteal graft myringoplasty for the treatment of chronic suppurative otitis media with dry central perforation in our hospital from April 2019 to April 2021. All patients were followed up for one year to assess the anatomical success and functional outcomes by comparing the preoperative and postoperative (after six months) results of pure tone audiometry (PTA). Results The sample was composed of 36 patients; 20 of them were female (55.6%) and 16 were male (44.4%) subjects, with ages ranging from 7 to 16 (mean: 12.7) years. Four patients underwent surgery in both ears (with an interval of 6 to 9 months). Out of 40 surgeries performed, 38 ears have shown anatomical success (95%). A highly significant improvement in hearing was obtained (the mean difference between the pre- and postoperative results of the PTA was of 14.6 ± 3.45 dB ( p < 0.001). Conclusion We advocate the use of periosteal graft in the pediatric population as a good alternative for other types of grafts, with comparable and even better functional and anatomical outcomes.

A Technique of Autologous Costal Cartilage Graft Combined With Auricular Cartilage Folding to Correct Question Mark Ear in a Single Procedure.

Background: Question mark ear is a rare congenital deformity, mainly characterized the interruption of the natural continuity between the lower border of the helix and the earlobe. In severe cases, the earlobe may be absent. In addition, there may be protrusion and outward expansion of the upper part of the auricle, with partial or complete disappearance of the antihelix. This article aims to introduce a technique that combines autologous costal cartilage carving with auricular cartilage folding to achieve a stable and aesthetic auricle. Method: This study included 26 patients with sporadic question mark ear deformity who were treated at our clinical center from January 2020 to December 2022. Based on the different appearances of the lower part of the auricle, they were divided into 2 categories:11 cases showed a natural continuity interruption between the helix and the earlobe, while 15 cases showed the absence of the earlobe. All patients underwent corrective surgery using costal cartilage transplantation combined with the upper part of the auricular cartilage folding, performed by senior surgeons. Results: Question mark ear was effectively improved and with no significant rebound. The average follow-up period was 8.4 months (ranging from 6 to 12 months). A satisfaction survey showed that 23 patients (88%) were satisfied, 3 patients (12%) were partially satisfied, and no patients were dissatisfied. Most patients experienced temporary swelling after surgery, which resolved within 3 months to half a year. Conclusion: Autologous costal cartilage transplantation combined with folding of the auricular cartilage is an ideal surgical method to correct question mark ear.

Lymphatic Vessels in the Inner Ear of Patients With Meniere Disease: A Novel Pathological Finding.

Background: Meniere disease, characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure, is a common cause of vertigo in humans. The pathogenesis of Meniere disease remains unknown. The current study aimed to describe a novel pathological change discovered in the inner ears of patients with Meniere disease who underwent labyrinthectomy. Methods: This retrospective case-control study was conducted with 21 patients with MD who underwent labyrinthectomy. A total of 15 patients diagnosed with acoustic neuroma or glomus jugular tumor were review over the same period of time as control. The clinical information of the patients and the pathological features of the membrane are described. Results: The new pathological tissue was a morbid membrane structure sealing the round window, characterized by the formation of lymphatic capillaries. Histochemical and immunofluorescent staining was positive for D2-40, LYVE-1, podoplanin, and PROX1, which are the classical markers of the lymphatic vessels. Transmission electron microscopy revealed that the lymph capillaries lacked a typical basement membrane and that their ends were blind, composed of a single layer of endothelial cells with valval connection structures between adjacent capillary epithelial cells. Conclusion: This is the first report of lymphatic vessels in the human inner ear, and this pathological structure is a completely new discovery. The lymphatic vessels may develop due to inflammation or decompensation of pressure in the inner ear, suggesting that the inner ear can reactively form lymphatic vessels in some inflammation and fluid flow-dependent pathological conditions. The current findings help in improving our understanding of the pathogenesis of Meniere disease.

Recent Progress in Generation of Inner Ear Organoid.

Inner ear organoids play a crucial role in hearing research. In comparison to other animal models and 2D cell culture systems, inner ear organoids offer significant advantages for studying the mechanisms of inner ear development and exploring novel approaches to disease treatment. Inner ear organoids derived from human cells are more closely resemble normal human organs in development and function. The 3D culture system of the inner ear organoid enhances cell-cell interactions and mimics the internal environment. In this review, the progress and limitations of organoid culture methods derived from tissue-specific progenitors and pluripotent stem cells (PSCs) are summarized, which may offer new insights into generating organoids that closely resemble the inner ear in terms of morphology and function.

Hematohidrosis: A Rare Case of Bleeding Ears.

Hematohidrosis is a rare disease and should be treated as a diagnosis of exclusion. Its pathogenesis remains unclear, though several theories have been proposed. It is often associated with psychological stress and anxiety, with the most commonly affected areas being the ears, nose, and oral mucosa. There is no specific management modality for hematohidrosis yet, as it is generally self-limiting. Enhanced monitoring and surveillance are required to avoid complications and determine the severity of the condition.

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.

Efficacy and safety of a self-expandable retainer in endoscopic ear surgery.

PURPOSE: During endoscopic ear surgery (EES), it is important to maintain a clear view of the endoscopic camera to achieve a clean field. In this study, a self-expandable external auditory canal (EAC) retainer was developed to enable a more efficient and safer EES. This study aimed to evaluate the retainer's efficacy and safety in EES. METHODS: Among adult patients scheduled for endoscopic tympanoplasty, 50 participants were recruited. They were assigned to either the retainer or control group in a 1:1 ratio. The anatomical characteristics, number of endoscopic cleanings during surgery, surgeon's satisfaction, and other factors were evaluated. RESULTS: No differences were observed in the surgical direction, EAC size measured on preoperative temporal bone computed tomography scans, location and size of tympanic membrane perforation, or bleeding degree between the two groups. When comparing the surgical time, including retainer insertion and removal, the time was similar between groups (retainer group, 35 min; control group, 33.2 min). The frequency of endoscopic cleaning per minute was statistically significantly lower in the retainer group than in the control group (0.18 times per minute, p = 0.048). No side effects, including sensory abnormalities or allergic reactions, were reported in any patient who used the retainer. CONCLUSION: A reduction in unnecessary endoscopic cleaning during EES was observed while using the self-expandable retainer, leading to increased surgeon satisfaction and efficiency. Furthermore, as a safe method without side effects, the retainer could be widely used to various indications for EES beyond tympanoplasty.

Insidious Cases of Enlarged Vestibular Aqueduct (EVA) Syndrome Resembling Otosclerosis: Clinical Features for Differential Diagnosis and the Role of High-Resolution Computed Tomography in the Pre-Operative Setting.

BACKGROUND: Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air-bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors' studies and seems to be related to a third mobile window (TMW) phenomenon. This can lead to misdiagnosis and inappropriate treatment. Given that it would be inappropriate and harmful to perform CT scans in all patients with a clinical diagnosis of otosclerosis, this study aims to highlight some clinical features useful for the differential diagnosis between otosclerosis and these rare cases of EVA presenting with an ABG, thus enabling the identification of suspected cases to be tested with CT scans. METHODS: Between April and May 2024, a narrative review was conducted focusing on the differential diagnosis between some rare cases of EVA and otosclerosis. Clinical, audiological, and radiologic features of both conditions were investigated. RESULTS: This review demonstrates the diagnostic challenge in differentiating atypical cases of EVA from otosclerosis in a subset of patients. Clinical and audiological features are important for differential diagnosis, but may not always be sufficient. Therefore, high-resolution computed tomography (HRCT) of the temporal bone plays a pivotal role in definitive diagnosis. CONCLUSIONS: In some specific cases, pre-operative imaging assessment using HRCT emerges as an essential tool for differentiating these two conditions and avoiding unnecessary stapes surgery.

Combined underwater endoscopic and microscopic surgery for tympanic paraganglioma: A case report.

The resection of middle ear paragangliomas can be challenging given their vascular nature and the small volume of the tympanic cavity, particularly when the tumor in the hypotympanum is close or attached to the internal carotid artery (ICA). We performed combined underwater endoscopic and microscopic surgery for a Class B1 middle ear paraganglioma according to the modified Fisch classification. The suspicious bone in the hypotympanum and around the petrous ICA was drilled with underwater endoscopy. The feeding arteries, the caroticotympanic and inferior tympanic arteries, were suctioned and cauterized under microscopy. To the best of our knowledge, no case of middle ear paraganglioma treated with underwater endoscopy has been reported. Underwater endoscopy, providing a clear operative field with blood and bone dust irrigation, is a good indication for middle ear paragangliomas. In contrast, microscopic preparation for unexpected bleeding is important, particularly when the tumor closely extends to vital structures, such as the ICA or the jugular bulb.

Poly(Ethylene Glycols) to Facilitate Celloidin Removal for Immunohistochemical Studies on Archival Human Brain and Temporal Bone Sections.

Pathology repositories worldwide store millions of celloidin-processed human brain and temporal bone (TB) sections vital for studying central nervous system diseases and sensory organs. However, accessing these sections for modern molecular-pathological research, like immunohistochemistry, is hindered by the challenge of removing celloidin without damaging tissue. In this study, we explored the use of polyethylene glycols (PEGs), a class of non-hazardous, ethylene glycol oligomers, combined with an improved section mounting technique, to gently and effectively dissolve celloidin from sections archived for up to 40 years. Optimizing our protocol involved exploring celloidin dissolution kinetics in PEGs of varying molecular weights and terminations, as well as different temperatures. Low molecular weight PEGs, particularly PEG 200, were the most efficient celloidin solvent. Nuclear magnetic resonance (NMR) spectroscopy of celloidin-PEG 200 dissolution products revealed no chemical alterations, suggesting pure solvation without chemical modification. Because the solvation of celloidin in PEG was inhibited by proteins, we further developed a protein-free mounting protocol allowing complete celloidin removal in 30 to 60 minutes by immersing in PEG 200. In summary, our approach overcomes major methodological hurdles, rendering decades-old archival celloidin sections viable for immunohistochemical and other molecular biological techniques, while enhancing safety and workflow efficiency.

N6-methyladenosine methylation analysis of circRNAs in acquired middle ear cholesteatoma.

Introduction: Middle ear cholesteatoma is a chronic middle ear disease characterized by severe hearing loss and adjacent bone erosion, resulting in numerous complications. This study sought to identify pathways involved in N6-methyladenosine (m6A) modification of circRNA in middle ear cholesteatoma. Methods: A m6A circRNA epitranscriptomic microarray analysis was performed in middle ear cholesteatoma tissues (n = 5) and normal post-auricular skin samples (n = 5). Bioinformatics analyses subsequently explored the biological functions (Gene Ontology, GO) and signaling pathways (Kyoto Encyclopedia of Genes and Genomes, KEGG) underlying middle ear cholesteatoma pathogenesis. Methylated RNA immunoprecipitation qPCR (MeRIP-qPCR) was performed to verify the presence of circRNAs with m6A modifications in middle ear cholesteatoma and normal skin samples. Results: Microarray analysis identified 3,755 circRNAs as significantly differentially modified by m6A methylation in middle ear cholesteatoma compared with the normal post-auricular skin. Among these, 3,742 were hypermethylated (FC ≥ 2, FDR < 0.05) and 13 were hypomethylated (FC ≤ 1/2, FDR < 0.05). GO analysis terms with the highest enrichment score were localization, cytoplasm, and ATP-dependent activity for biological processes, cellular components, and molecular functions respectively. Of the eight hypermethylated circRNA pathways, RNA degradation pathway has the highest enrichment score. Peroxisome Proliferator-Activated Receptor (PPAR) signaling pathway was hypomethylated. To validate the microarray analysis, we conducted MeRIP-qPCR to assess the methylation levels of five specific m6A-modified circRNAs: hsa_circRNA_061554, hsa_circRNA_001454, hsa_circRNA_031526, hsa_circRNA_100833, and hsa_circRNA_022382. The validation was highly consistent with the findings from the microarray analysis. Conclusion: Our study firstly presents m6A modification patterns of circRNAs in middle ear cholesteatoma. This finding suggests a direction for circRNA m6A modification research in the etiology of cholesteatoma and provides potential therapeutic targets for the treatment of middle ear cholesteatoma.