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Benign dendritic melanocytic proliferation is usually observed in the skin. We report an extremely rare case of a melanocytic lesion in the middle ear mucosa. Only 3 cases of melanocytic lesions in the middle ear have been reported. Our report is the only one that describes an adult with a melanocytic lesion in the middle ear that extended to the inner ear and nasopharyngeal mucosa. A 23-year-old female presented with profound sensory neural hearing loss and recurrent discharge from the right ear. Examination revealed a blue right tympanic membrane with a small perforation. Computed tomography scans and magnetic resonance imaging were performed. After surgical exploration and histopathological examination, the patient was found to have a benign melanocytic lesion in the right middle ear. Melanocytic lesions are commonly found in the skin. There are no previous reports have described adult patients with melanocytic lesions in the middle ear that extend to the inner ear and nasopharynx, which cause profound hearing loss with recurrent ear discharge. These patients require regular follow-up to assess the progression of the lesion and to watch for any malignant behavior., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

To evaluate the genetics of chronic nonsuppurative otitis media (OM). We performed a genome-wide association study of 429,599 individuals included in the FinnGen study using three different case definitions: combined chronic nonsuppurative OM (7034 cases) (included serous and mucous chronic OM), mucous chronic OM (5953 cases), and secretory chronic OM (1689 cases). Individuals without otitis media were used as controls (417,745 controls). We used immunohistochemistry (IHC) of the murine middle ear to evaluate the expression of annexin A13. Four loci were significantly associated (p < 1.7 × 10 -8 ) with nonsuppurative OM. Three out of the four association signals included missense variants in genes that may play a role in otitis media pathobiology. According to our subtype-specific analyses, one novel locus, located near ANXA13, was associated with secretory OM. Three loci (near TNFRSF13B, GAS2L2, and TBX1) were associated with mucous OM. Immunohistochemistry of murine middle ear samples revealed annexin A13 expression at the apical pole of the Eustachian tube epithelium as well as variable intensity of the secretory cells of the glandular structure in proximity to the Eustachian tube. We demonstrated that secretory and mucous OM have distinct and shared genetic associations. The association of GAS2L2 with ciliary epithelium function and the pathogenesis of dysfunctional mucosa in mucous OM is suggested. The abundant expression of annexin A13 in the Eustachian tube epithelium, along with its role in apical transport for the binding and transfer of phospholipids, indicates the role of annexin A13 and phospholipids in Eustachian tube dysfunction., (© 2024. The Author(s).)

Middle-ear salivary gland choristoma (SGCh) is a rare, benign tumor that causes conductive hearing loss owing to middle-ear morphological abnormalities. Early diagnosis is challenging, and surgical resection is indispensable for a definitive diagnosis. We report the case of a 3-year-old boy diagnosed with middle-ear SGCh during the follow-up period for left-sided hearing loss discovered at newborn hearing screening (NHS). Long-term follow-up after the NHS result, subsequent computed tomography/magnetic resonance imaging, and surgical resection led to its relatively early diagnosis and treatment., Competing Interests: No potential conflict of interest relevant to this article was reported.

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., (© 2024 The Authors.)

Here we present the first case of sebaceous carcinoma of the middle ear. We discuss the treatment course and post treatment results after 11 years of follow up. We further summarize the available literature of sebaceous carcinoma of the temporal bone, which prior to this case was exclusively limited to the external auditory canal. Laryngoscope, 134:3769-3772, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

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., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Middle ear tumors are relatively rare, and among them, the diagnoses of middle ear lesions originating from cartilage-like tissue are even rarer. Use of transcanal endoscopic ear surgery (TEES) has increased in recent years because of its advantages, such as clear visual field and minimally invasive procedure. Here, we report a middle ear mass originating from cartilage-like tissue treated with TEES. A 62-year-old woman presented with progressive right-sided hearing loss. A white mass was revealed through the tympanic membrane, and pure-tone audiometry detected a mean 50.0 dB conductive hearing loss. Computed tomography showed a mass in the tympanic cavity. TEES was performed for diagnosis and treatment. A white translucent tumor was observed intraoperatively, and it was completely resected. Histopathological examination confirmed the diagnosis of a mass originating from degenerated cartilage-like tissue. To the best of our knowledge, this is the first study of a middle ear mass originating from cartilage-like tissue treated with TEES. TEES with its clear visual field and precise techniques was beneficial in treating the middle ear lesions circumscribed in the tympanic cavity., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: This study aims to investigate whether the middle ear resonance frequency (RF) is affected in acromegaly, which causes growth in the skull bone., Methods: Thirty acromegaly patients and 38 volunteers were included in the study. Pure tone average scores and middle ear RF values of the groups that underwent pure tone audiometry, tympanometry, and multifrequency tympanometry tests were compared., Results: The pure tone mean was 14.95 ± 12.13 in acromegaly patients and 5.70 ± 8.52 in the control group (p:0.18). Sensorineural hearing loss(SNHL) was observed in 16.6% of the patients. The average middle ear RF was calculated as 815 ± 179.05 Hz in patients with acromegaly and 773 ± 127.15 in the control group. (p = 0.0001)., Conclusion: This study is the first to evaluate middle-ear RF in acromegaly patients. Acromegaly-induced changes in soft tissues and bone structures impact middle ear functions. In this patient group, we found an increase in middle ear RF without conductive-type hearing loss and a 16.6% rate of SNHL., Competing Interests: Declaration of competing interest No conflict of interest was declared by the authors., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Significance Statement: Primary middle ear inverted papilloma is an exceedingly rare finding posing a diagnostic challenge, as symptomatology often mimics common clinical entities like serous otitis media. Clinical and radiological findings are not specific, whilst high recurrence rates, local destruction, and potential malignant transformation constitute an overall demanding surgical challenge, requiring aggressive procedures. We report the case of a primary inverted papilloma of the middle ear, discussing diagnostic and therapeutic issues., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Canine middle ear effusion (MEE) is usually asymptomatic, being an incidental finding when computed tomography or magnetic resonance imaging (MRI) of the head is performed for other reasons unrelated to otic disease. The clinical relevance of the presence of material in the tympanic bulla (TB) remains uncertain, and more detail about its prevalence and appearance in MRI are required., Objective: To assess the prevalence of presence of material within the TB of French bulldogs (FB) with no clinical signs suggestive of otitis (externa, media or interna) that underwent high-field MRI for other medical reasons., Animals: Two hundred fifty-two TB of 126 FB were included in this study., Materials and Methods: Nonexperimental retrospective study in which MRI images were evaluated by a board-certified veterinary radiologist., Results: Fifty-eight per cent of the dogs had material in the TB lumen (46% of the TB) and 59% were bilaterally affected. The signal intensity of this material related to the grey matter was variable on T1w and mainly hyperintense on T2w sequences., Conclusion and Clinical Relevance: FB are predisposed to MEE. This is important when assessing imaging studies of TB of FB with chronic otitis externa, as high percentage of cases may have concurrent MEE. MRI findings in FB with MEE are characterised by a hyperintense signal to the grey matter on T2w in most cases and variable on T1w sequences., (© 2024 ESVD and ACVD.)

Primary ectopic meningioma of the middle ear is relatively rare in clinical practice. It is often difficult to distinguish it from chronic otitis media or otitis media with effusion due to its similar and atypical clinical symptoms. We report a case of epithelial tympanic ectopic meningioma with the main complaints of otalgia, aural fullness, and hearing loss. It was accidentally discovered during tympanotomy due to the symptoms of recurring refractory secretory otitis media. This article briefly reviews the relevant literature in recent years, summarizes the characteristics of primary ectopic tympanic meningioma with intact tympanic membrane, and emphasizes the diagnosis and treatment strategy of the middle ear mass., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Inflammation and infection of the middle ear, known as otitis media (OM), is a leading cause of hearing loss and the most frequently diagnosed disease in children worldwide. Traditionally, mouse models for OM rely on inducing acute infection through inoculation of the middle ear, e.g. with the human otopathogen non-typeable Haemophilus influenzae (NTHi), and with very few genetic models with spontaneous or chronic OM. A2ML1 variants, including loss-of-function variants, were associated with susceptibility to OM in humans, but no animal model has been reported for A2ml1-related OM. Here, we report our middle ear findings in a mouse line with a CRISPR-induced knockout (KO) of A2ml1., Methods: Mice were X-rayed prior to harvest to determine if there are craniofacial or skeletal abnormalities. Tissue from mouse middle ears, as well as other upper respiratory mucosal tissues, were harvested. The harvested middle ear bullae were examined under microscope and submitted for histologic preparation to study phenotypic indications of OM. RNA samples isolated from middle ear tissue were assayed for expression of genes correlated with A2ML1 expression in humans., Results: Data from a total of 119 mice (35 wildtype, 40 heterozygous, 44 homozygous) are presented here, with each analyses being performed on subsets of these mice. There were no significant craniofacial differences by genotype (n = 22). Findings in mice with the A2ml1-KO indicated an increased incidence of OM (n=29; odds ratio = 11; CI: 1.1, 573.6; Fisher exact two-sided p = 0.02) with tympanic membrane perforations or thickening, as well as cases of middle ear effusion, inflammatory cells, or fluid from histologic sections. Dsp was upregulated in the middle ear tissues of homozygous mice (Wilcoxon test p = 0.001)., Conclusion: Thus far, our results in this A2ml1-KO mouse line indicate spontaneous occurrence of OM and dysregulation of Dsp in the middle ear as a potential disease mechanism for A2ml1-related OM., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Objectives: Although previous research has indicated inner ear changes in diabetes mellitus (DM) patients, no prior study has explored the middle ear, particularly the ossicles and their joints, in DM patients. This study aimed to investigate whether type 2 DM is associated with middle ear changes, specifically affecting the ossicular chain and joints., Methods: This study included 47 ears from 25 patients with DM (male = 13, female = 12, age: 51.0 ± 20.5) and age- and sex-matched controls (male = 10, female = 10, age: 54.8 ± 15.9) (sex; p = 1.000, Age; p = 0.991). Otopathological evaluations of the auditory ossicles and incudomalleolar joint (IMJ) were performed using light microscopy., Results: In the IMJ of DM cases, malleus hyalinized cartilage (Malleus hC) and incus hyalinized cartilage (Incus hC) were significantly increased compared with control cases (Malleus hC; DM, 34.17 ± 9.71 μm vs. control 21.96 ± 4.16 μm, p < 0.001) (Incus hC; DM 35.11 ± 10.12 μm vs. control 22.42 ± 4.368 μm, p < 0.001). In addition, bone-line distance was significantly longer than in DM cases than control cases (DM 266.72 ± 59.11 μm vs. control 239.81 ± 35.56 μm p = 0.040). On the other hand, joint discus distance was longer in the control group than in DM cases (DM 96.84 ± 36.80 μm vs. Control 113.63 ± 23.81 μm, p = 0.001)., Conclusions: This study reveals a notable increase in the hyalinized cartilage layer and bone-line distance accompanied by reducing joint discus distance within the IMJ in DM cases. These findings suggest that DM may influence microjoints, such as the IMJ, and potentially impact auditory function., Evidence Level: N/A Laryngoscope, 134:2871-2878, 2024., (© 2024 The Authors. The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Middle ear neuroendocrine tumors (MeNETs) are an exceptionally rare occurrence. These benign tumors stem from the tympanic mucosa and can easily be misinterpreted by the clinician and the pathologist. Clinical characteristics, otoscopic findings and medical imaging in these cases are non-specific. We present a case of a 60-year-old male patient with bilateral hearing loss following recent coronavirus disease 2019 disease. Diagnostic work-up revealed a soft tissue neoplasm of the left middle ear. Surgical resection of the tumor mass with implantation of a partial ossicular replacement prosthesis (PORP) was the main modality of treatment. Middle ear neuroendocrine tumors was confirmed through positive immunohistochemistry for neuroendocrine tumor markers. Follow-up magnetic resonance imaging 12 months after the surgery reported no tumor recurrence or significant residual disease with a stable PORP. Our report highlights challenges in diagnosing and treating these rare tumors, while emphasizing surgical resection pitfalls and resulting improvement of quality of life of the patient. We recommend a through followup of patients with unclear soft tissue masses in the middle ear to obtain a definitive diagnosis.

Objective: Tophaceous lesions of the middle ear from calcium pyrophosphate deposition disease (CPPD, or pseudogout) and gout are infrequently reported. Recognizing its characteristic findings will allow clinicians to accurately narrow the differential diagnosis of bony-appearing middle ear lesions and improve management., Patients: Two consecutive cases of tophaceous middle ear lesions presenting to a tertiary care center between January 2021 and December 2021. Neither with previous rheumatologic history., Interventions: Surgical excision of tophaceous middle ear lesions., Main Outcome Measure: Improvements in facial weakness and conductive hearing loss., Results: The first case was a 66-year-old gentleman with progressive conductive loss, ipsilateral progressive facial weakness over years, and an opaque, irregular-appearing tympanic membrane anterior to the malleus found to have CPPD on surgical pathology, with immediate postoperative improvement of facial function. The second was a 75-year-old gentleman with progressive conductive loss and similar appearing tympanic membrane as case 1, previously diagnosed with tympanosclerosis, found to have gout on surgical pathology. In both cases, the CT showed a heterogenous, bony-appearing lesion in the middle ear, and both tophaceous lesions were a of gritty, chalky consistency intraoperatively., Conclusion: Tophaceous lesions of the middle ear are rare but have similar findings. Notably, the tympanic membrane can appear opaque and irregular, and the CT demonstrates a radiopaque, heterogeneous appearance. Facial weakness is an unusual finding. Specimens of suspected tophi must be sent to pathology without formalin for accurate diagnosis., Competing Interests: Conflicts of interest: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Background: The management of glomus tympanicum tumours can be challenging. Blue laser coagulation may improve bleeding control thus facilitating an endoscopic transcanal excision. The objective of this presentation is to illustrate the authors' experience using this novel tool., Methods: Case report of a patient that underwent exclusive endoscopic transcanal blue laser surgery of a class A2 glomus tympanicum tumour in a tertiary referral center., Conclusion: The present study provides evidence of the safety and efficacy of endoscopic blue laser surgery, for the minimally invasive treatment of early-stage glomus tympanicum tumours., (© 2024. The Author(s).)

Opacification in the middle ear and mastoid region can stem from a wide range of factors. In terms of diagnostic imaging, CT is the primary tool due to its exceptional spatial resolution, particularly for examining the temporal bone and ossicles. MRI complements this by offering detailed soft tissue lesion characterization and assessing involvement in the inner ear and cranial nerves. This study focuses on inflammatory causes of opacification in the middle ear and mastoid, with an emphasis on the utility of CT and MRI. This comprehensive review aimed to provide a practical framework for considering potential differential diagnoses.

Objective: Rhabdomyosarcoma is a common soft tissue tumor, but isolated involvement of anterior portion of petrous bone is exceedingly rare. Here, we present a case of embryonal rhabdomyosarcoma involving the anterior petrous without involvement of the mastoid and middle ear., Patient: A 6-year-old boy presented with a progressive right side lower motor neuron facial paresis for 1-month duration along with headache and recurrent vomiting episodes. Radiology showed a contrast-enhancing lesion involving the right petrous apex. He underwent craniotomy and excision of the lesion. Based on the frozen section, a diagnosis of rhabdomyosarcoma was rendered, and gross total resection could be achieved. Postoperative course was uneventful., Conclusion: Isolated petrous bone involvement of embryonal rhabdomyosarcoma is a rare presentation. Intra-operative frozen section plays a key role in decision making regarding the extent of excision. Hence, a prompt and accurate diagnosis is essential in managing these cases.

Otitis media (OM) is one of the most globally pervasive pediatric conditions. Translocation of nasopharynx-resident opportunistic pathogens like nontypeable Haemophilus influenzae (NTHi) assimilates into polymicrobial middle ear biofilms, which promote OM pathogenesis and substantially diminish antibiotic efficacy. Oral or tympanostomy tube (TT)-delivered antibiotics remain the standard of care (SOC) despite consequences including secondary infection, dysbiosis, and antimicrobial resistance. Monoclonal antibodies (mAb) against two biofilm-associated structural proteins, NTHi-specific type IV pilus PilA (anti-rsPilA) and protective tip-region epitopes of NTHi integration host factor (anti-tip-chimer), were previously shown to disrupt biofilms and restore antibiotic sensitivity in vitro. However, the additional criterion for clinical relevance includes the absence of consequential microbiome alterations. Here, nine chinchilla cohorts (n = 3/cohort) without disease were established to evaluate whether TT delivery of mAbs disrupted nasopharyngeal or fecal microbiomes relative to SOC-OM antibiotics. Cohort treatments included a 7d regimen of oral amoxicillin-clavulanate (AC) or 2d regimen of TT-delivered mAb, AC, Trimethoprim-sulfamethoxazole (TS), ofloxacin, or saline. Fecal and nasopharyngeal lavage (NPL) samples were collected before and several days post treatment (DPT) for 16S sequencing. While antibiotic-treated cohorts displayed beta-diversity shifts (PERMANOVA, P < 0.05) and reductions in alpha diversity (q < 0.20) relative to baseline, mAb antibodies failed to affect diversity, indicating maintenance of a eubiotic state. Taxonomic and longitudinal analyses showed blooms in opportunistic pathogens (ANCOM) and greater magnitudes of compositional change (P < 0.05) following broad-spectrum antibiotic but not mAb treatments. Collectively, results showed broad-spectrum antibiotics induced significant fecal and nasopharyngeal microbiome disruption regardless of delivery route. Excitingly, biofilm-targeting antibodies had little effect on fecal and nasopharyngeal microbiomes., (© 2024. The Author(s).)

This article discusses otoscopic middle ear mastoid surgery from multiple perspectives. Firstly, it discusses the indications and contraindications for surgery from the nature of the lesion and the imaging manifestations; secondly, it recommends the applicable equipment and describes the surgical approach in detail; finally, it summarizes the principles of the management of the operative cavity of the mastoid process in the middle ear from the perspectives of function and reconstruction. The purpose of this article is to illustrate otoscopic middle ear mastoid surgery with the aim of providing reference or guidance for performing related surgeries., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Clinical Case: The 59-year-old patient complained of hearing loss on the left, ear murmur for a long time, periodic pain and discomfort in the left ear, dizziness for 6 months. She was found to have concurrent vestibular schwannoma in the internal auditory canal and temporal bone paraganglioma. Both tumors were removed in one operation. The schwannoma was removed by translabirinth access due to preoperative deafness, while the glomus tumor was removed during this access. Postoperative biopsy showed the presence of two unrelated diseases: paraganglioma (ICD-0 code 8690/3) and schwannoma (ICD-0 code 9560/0).

Purpose: Middle ear infection is the most prevalent inflammatory disease, especially among the pediatric population. Current diagnostic methods are subjective and depend on visual cues from an otoscope, which is limited for otologists to identify pathology. To address this shortcoming, endoscopic optical coherence tomography (OCT) provides both morphological and functional in vivo measurements of the middle ear. However, due to the shadow of prior structures, interpretation of OCT images is challenging and time-consuming. To facilitate fast diagnosis and measurement, improvement in the readability of OCT data is achieved by merging morphological knowledge from ex vivo middle ear models with OCT volumetric data, so that OCT applications can be further promoted in daily clinical settings., Methods: We propose C2P-Net: a two-staged non-rigid registration pipeline for complete to partial point clouds, which are sampled from ex vivo and in vivo OCT models, respectively. To overcome the lack of labeled training data, a fast and effective generation pipeline in Blender3D is designed to simulate middle ear shapes and extract in vivo noisy and partial point clouds., Results: We evaluate the performance of C2P-Net through experiments on both synthetic and real OCT datasets. The results demonstrate that C2P-Net is generalized to unseen middle ear point clouds and capable of handling realistic noise and incompleteness in synthetic and real OCT data., Conclusions: In this work, we aim to enable diagnosis of middle ear structures with the assistance of OCT images. We propose C2P-Net: a two-staged non-rigid registration pipeline for point clouds to support the interpretation of in vivo noisy and partial OCT images for the first time. Code is available at: https://gitlab.com/nct&#95;tso&#95;public/c2p-net., (© 2023. The Author(s).)

Objective: To discuss the management of sigmoid sinus thrombophlebitis secondary to middle ear cholesteatoma., Methods: We retrospectively analyzed all cases of sigmoid sinus thrombophlebitis caused by middle ear cholesteatoma over a period of 7 years. 7 male and 2 female patients, ranging in age from 9 to 66 years, were diagnosed with sigmoid sinus thrombophlebitis by clinical presentation and radiological examination. By executing a modified mastoidectomy and tympanoplasty (canal wall-down tympanoplasty) to entirely remove the cholesteatoma-like mastoid epithelium, all patients were effectively treated surgically without opening the sigmoid sinus. All patients were treated with broad-spectrum antibiotics, but no anticoagulants were used., Results: 9 patients had otogenic symptoms such as ear pus, tympanic membrane perforation, and hearing loss. In the initial stage of the surgery, modified mastoidectomy and tympanoplasty were performed on 8 of the 9 patients. 1 patient with a brain abscess underwent puncturing (drainage of the abscess) to relieve cranial pressure, and 4 months later, a modified mastoidectomy and tympanoplasty were carried out. Following surgery and medication, the clinical symptoms of every patient improved. After the follow-up of 6 months to 7 years, 3 patients were re-examined for MRV and showed partial sigmoid sinus recovery with recanalization. 4 months following middle ear surgery, the extent of a patient's brain abscess lesions was significantly reduced. 1 patient experienced facial paralysis after surgery and recovered in 3 months. None of the patients had a secondary illness, an infection, or an abscess in a distant organ., Conclusion: The key to a better prognosis is an adequate course of perioperative antibiotic medication coupled with surgical treatment. A stable sigmoid sinus thrombus can remain for a long time after middle ear lesions have been removed, and it is less likely to cause infection and abscesses in the distant organs. The restoration of middle ear ventilation is facilitated by tympanoplasty. It is important to work more closely with multidisciplinary teams such as neurology and neurosurgery when deciding whether to perform lateral sinusotomies to remove thrombus or whether to administer anticoagulation., (© 2023. The Author(s).)

Middle ear adenomatous neuroendocrine tumors (MEANT) are rare, mainly benign neoplasms and account for less than 2% of the middle and inner ear neoplasms. There are no specific radiological or clinical findings; therefore, the diagnosis is often difficult. Biopsy prior to surgery is often proposed. The definite treatment is the complete surgical resection. We present an uncommon clinical report of a MEANT, which was treated successfully with surgical excision., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Cholesteatoma is a rare middle ear pathology. It can be classified into acquired and congenital forms. Although benign, cholesteatomas can cause significant morbidity including hearing loss, infection, facial palsy and thrombosis. Congenital cholesteatomas are incredibly rare and bilateral disease has not commonly been published in the literature., Method: We describe the case of female identical (monozygotic, monochorionic, diamniotic) twins who both developed congenital cholesteatomas. In this report, we review the aetiology, treatment, embryology and pathology of cholesteatoma., Results: The patients have been followed up 15 years after their initial surgery with promising results - pure-tone audiometry and repeat scans have not illustrated any disease recurrence., Conclusion: This paper presents one of the only cases of female monozygotic twins presenting with unilateral and bilateral cholesteatomas., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Chronic inflammation causes bone destruction in middle ear cholesteatomas (MECs). However, the causes of their neoplastic features remain unknown. The present study demonstrated for the first time that neoplastic features of MEC are based on proto-oncogene mutations., Results: DNA was extracted from MEC and blood samples of five patients to detect somatic mutations using depth-depth exome sequencing. Exons with somatic variants were analyzed using an additional 17 MEC/blood test pairs. Variants detected in MECs but not in blood were considered pathogenic variant candidates. We analyzed the correlation between proto-oncogene (NOTCH1 and MYC) variants and the presence of bone destruction and granulation tissue formation. MYC and NOTCH1 variants were detected in two and five of the 22 samples, respectively. Two of the NOTCH1 variants were located in its specific functional domain, one was truncating and the other was a splice donor site variant. Mutations of the two genes in attic cholesteatomas (n = 14) were significantly related with bone destruction (p = 0.0148) but not with granulation tissue formation (p = 0.399)., Conclusions: This is the first study to demonstrate a relationship between neoplastic features of MEC and proto-oncogene mutations., (© 2023. The Author(s).)

Introduction: Middle ear lipomas are rare, accounting for less than 1% of all middle ear tumours. To our knowledge there have been only 16 reported patients with middle ear lipoma. Only one of these had bilateral congenital middle ear lipomas. The aim of this review is to comprehensive overview the diagnosis and management of congenital lipomas of the middle ear., Methods: A literature review was performed searching multiple data bases, using the key words "middle ear", AND "lipoma", AND "congenital". A total of 116 articles were identified. One hundred were excluded on various criteria, leaving 16 articles for detailed review. Additionally, we report the second case of bilateral congenital middle ear lipoma., Results: Based on this case report and literature review, the incidence of middle ear lipomas is greater in paediatric patients than in adult patients. Middle ear lipomas are distributed evenly between males and females. Three patients had recognised genetic syndromes, while five had prior ventilation tube placement. Clinically patients present with conductive hearing loss and middle ear effusions. In some cases, presence of a white or yellow mass behind the anterior aspect of the tympanic membrane is recognised. The most common location of the masses is in the anterosuperior quadrant of the middle ear, the site of predilection for small congenital cholesteatomas. An MRI scan is most useful in distinguishing between lipoma and cholesteatoma. All cases reported surgical excision of the middle ear lipoma. There was no evidence of recurrence noted up to 4 years follow up., Conclusion: Though rare, congenital lipomas of the middle ear should be a differential diagnosis of a middle ear masses. Early identification can allow surgical lipoma resection with minimal disruption to the ossicular chain. Though there is currently no consensus regarding management, surgical removal and appropriate reconstruction of the ossicular chain if needed, is appropriate., Competing Interests: Declaration of competing interest No conflict of interest., (Crown Copyright © 2023. Published by Elsevier B.V. All rights reserved.)

Cholesteatoma is a noncancerous cystic lesion caused by an abnormal growth of keratinizing squamous epithelium which is invasive and capable of destroying structures. A prospective study on the expression of membrane type1-matrix metalloproteinases (MMP-14) and its related influencing factors in middle ear cholesteatoma was conducted to fully understand the pathogenesis of cholesteatoma in the molecular level. We examined the expression of MMP-14 by immunohistochemical staining 39 middle ear cholesteatoma specimens and 10 external auditory meatus epithelial cell specimens. The cholesteatoma specimens were divided into 4 groups according to the degree of destruction of the ossicles during surgery. The associated factors affecting MMP-14 expression were analyzed using statistical methods; The positive expression of MMP-14 in the epithelium of the external auditory canal was significantly different between middle ear cholesteatoma and normal patients (P < .05); Gender, age, and the degree of hearing loss had no statistically significant effect on MMP-14 expression (P > .05); The expression of MMP-14 was positively correlated with the severity of bone destruction (R = 0.535, P < .05); MMP-14 plays an important role in the pathological development of the epithelium of cholesteatoma; MMP-14 expression in middle ear cholesteatoma tissue was not strongly correlated with the level of hearing loss, age or gender, but was positively correlated with the degree of middle ear bone destruction., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity., Methods: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier., Results: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases., Conclusion: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

The incidence of sebaceous carcinoma (SC) in the outer one-third of the external auditory canal is considered extremely rare, and only eight case reports have been published. We present a case of a male patient in his late 70s known case of hypertension on indapamide. His medical history included a postspinal tumour that had been treated with surgery and radiation more than 40 years ago and current complaints of right ear pain and purulent discharge. A right ear soft granular tissue mass was found. Complete debulking of the right ear mass was done in conjunction with middle ear exploration, moderately differentiated SC diagnosis was made based on the histopathological analysis. The patient was free of recurrence but then died of an unrelated pulmonary infection. SC should be suspected in elderly patients who present with long-term complaints of a mass with or without otalgia, and these patients should be examined for the presence of such tumours especially if they report a history of radiation. Additionally, more research is warranted to investigate the association of diuretics with SC., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and Objectives : The diagnosis of cholesteatoma is usually clinic, and the only efficient treatment is surgical. High-resolution computed tomography (HRCT) is not considered absolutely necessary for the management of an uncomplicated cholesteatoma, but unsuspected situations from a clinical point of view can be discovered using the scans, warning the surgeon. Our objective is to compare HRCT scan information with intraoperative findings in patients with cholesteatoma and analyze the usefulness of a preoperative HRCT scan from a surgical point of view. Materials and Methods : This is a prospective descriptive study conducted in the Department of Otolaryngology, Victor Babes University of Medicine and Pharmacy Timisoara, Romania, from May 2021 to April 2022. It was carried out on 46 patients with a clinical diagnosis of cholesteatoma who were consequently operated on in our department. All patients received full clinical and audiological examinations. In all cases, an HRCT scan was performed preoperatively as a mandatory investigation. Preoperative HRCT scans were analyzed, and their findings were compared to the intraoperative notes. The two sets of observations were analyzed using standard statistical methods. Results : Extensive cholesteatoma was the most common type of disease, involving 46% of the patients, followed by pars flaccida cholesteatoma (35%) and pars tensa cholesteatoma (19%). Eroded scutum was the most frequent lesion involving 70% of the patients, followed by incus erosion (67%). Comparison of the HRCT and intraoperative findings revealed a very good correlation for tegmen tympani erosion, sigmoid plate erosion, scutum and malleus erosion, and a moderate-to-good correlation for lateral semicircular canal erosion, incus and stapes erosion, and fallopian canal erosion. Conclusions : HRCT is a valuable tool in the preoperative assessment of cholesteatoma, helping in making surgical decisions. It can accurately predict the extent of disease and is helpful for detecting unapparent dangerous situations. However, it is not very accurate in detecting fallopian canal and stapes erosion.

Background: Otitis media (OM) is defined as middle ear (ME) inflammation that is usually due to infection. Globally, OM is a leading cause of hearing loss and is the most frequently diagnosed disease in young children. For OM, pediatric patients with Down syndrome (DS) demonstrate higher incidence rates, greater severity, and poorer outcomes. However, to date, no studies have investigated the bacterial profiles of children with DS and OM. Method: We aimed to determine if there are differences in composition of bacterial profiles or the relative abundance of individual taxa within the ME and nasopharyngeal (NP) microbiotas of pediatric OM patients with DS ( n  = 11) compared with those without DS ( n  = 84). We sequenced the 16S rRNA genes and analyzed the sequence data for diversity indices and relative abundance of individual taxa. Results: Individuals with DS demonstrated increased biodiversity in their ME and NP microbiotas. In children with OM, DS was associated with increased biodiversity and higher relative abundance of specific taxa in the ME. Conclusion: Our findings suggest that dysbioses in the NP of DS children contributes to their increased susceptibility to OM compared with controls. These findings suggest that DS influences regulation of the mucosal microbiota and contributes to OM pathology.

Significance: Cholesteatoma is an expansile destructive lesion of the middle ear and mastoid, which can result in significant complications by eroding adjacent bony structures. Currently, there is an inability to accurately distinguish cholesteatoma tissue margins from middle ear mucosa tissue, causing a high recidivism rate. Accurately differentiating cholesteatoma and mucosa will enable a more complete removal of the tissue., Aim: Develop an imaging system to enhance the visibility of cholesteatoma tissue and margins during surgery., Approach: Cholesteatoma and mucosa tissue samples were excised from the inner ear of patients and illuminated with 405, 450, and 520 nm narrowband lights. Measurements were made with a spectroradiometer equipped with a series of different longpass filters. Images were obtained using a red-green-blue (RGB) digital camera equipped with a long pass filter to block reflected light., Results: Cholesteatoma tissue fluoresced under 405 and 450 nm illumination. Middle ear mucosa tissue did not fluoresce under the same illumination and measurement conditions. All measurements were negligible under 520 nm illumination conditions. All spectroradiometric measurements of cholesteatoma tissue fluorescence can be predicted by a linear combination of emissions from keratin and flavin adenine dinucleotide. We built a prototype of a fluorescence imaging system using a 495 nm longpass filter in combination with an RGB camera. The system was used to capture calibrated digital camera images of cholesteatoma and mucosa tissue samples. The results confirm that cholesteatoma emits light when it is illuminated with 405 and 450 nm, whereas mucosa tissue does not., Conclusions: We prototyped an imaging system that is capable of measuring cholesteatoma tissue autofluorescence., (© 2023 The Authors.)

Background: Postoperative adhesion is an important complication after middle-ear surgeries. Although many materials have been tried to prevent this complication, the use of Poly (dl-lactide ε-caprolactone) as an anti-adhesive material after middle-ear surgery has not yet been reported. The aim of this study was to evaluate the anti-adhesive effect of poly (dl-lactide ε-caprolactone) on the ears of rats with middle-ear mucosa damage., Methods: In our study, 14 Wistar albino rats and 28 ears in total were used. The rats were randomly divided into 4 groups. Middle ear mucosa damage was performed in all groups with a transcanal approach under otomicroscopy in sterile conditions. The effects of poly (dl-lactide ε-caprolactone), silicone sheet, and absorbable gelatin sponge were compared histologically with the secondary healing group. In addition, hearing evaluation was performed before the procedure and on the 28th postoperative day., Results: No significant difference was observed in transient otoacoustic emission and distortion product otoacoustic emissions tests performed before and after the surgical procedure when the groups were compared. While adhesion was observed in the tympanic membrane in the absorbable gelatin sponge group, no adhesion was observed in the other groups. In the absorbable gelatin sponge group, increased fibroblastic activity, inflammation, and neovascularization were observed in the middle-ear mucosa. No significant difference was observed in silicone sheet, poly (dl-lactide ε-caprolactone), and control groups in terms of fibroblastic activity, inflammation, and neovascularization., Conclusion: It can be concluded that absorbable poly (dl-lactide ε-caprolactone) is nonototoxic and biocompatible with the rat's middle ear cavity by short-term evaluation.

Implantable tubes, shunts, and other medical conduits are crucial for treating a wide range of conditions from ears and eyes to brain and liver but often impose serious risks of device infection, obstruction, migration, unreliable function, and tissue damage. Efforts to alleviate these complications remain at an impasse because of fundamentally conflicting design requirements: Millimeter-scale size is required to minimize invasiveness but exacerbates occlusion and malfunction. Here, we present a rational design strategy that reconciles these trade-offs in an implantable tube that is even smaller than the current standard of care. Using tympanostomy tubes (ear tubes) as an exemplary case, we developed an iterative screening algorithm and show how unique curved lumen geometries of the liquid-infused conduit can be designed to co-optimize drug delivery, effusion drainage, water resistance, and biocontamination/ingrowth prevention in a single subcapillary-length-scale device. Through extensive in vitro studies, we demonstrate that the engineered tubes enabled selective uni- and bidirectional fluid transport; nearly eliminated adhesion and growth of common pathogenic bacteria, blood, and cells; and prevented tissue ingrowth. The engineered tubes also enabled complete eardrum healing and hearing preservation and exhibited more efficient and rapid antibiotic delivery to the middle ear in healthy chinchillas compared with current tympanostomy tubes, without resulting in ototoxicity at up to 24 weeks. The design principle and optimization algorithm presented here may enable tubes to be customized for a wide range of patient needs.

Hemangiomas in the pediatric middle ear are a rare occurrence. We present the case of a 3-year-old boy with unilateral hearing impairment and recurrent otitis media with effusion due to a hemangioma in the middle ear. This caused a venous sinus thrombosis, osteomyelitis, and mastoiditis. Interdisciplinary treatment was performed. This case shows that even benign lesions in the middle ear can cause severe, life-threatening complications due to the close proximity of important anatomic structures in the middle ear. Every case of asymmetric hearing loss should thus be examined in detail., (© 2023. The Author(s).)

Middle ear adenomas are rare, low grade glandular neoplasms with epithelial and neuroendocrine components and with varying patterns of differentiation. Due to the rarity of this tumor, there is a dearth of publications detailing the cytological features. We herein review our institution's pathological database for cytological material between 1992 and 2022 for MEA specimens and discuss possible differential diagnoses based on clinical, pathological, and cytologic data and material., (© 2023 Wiley Periodicals LLC.)

The wide frequency range of the human hearing could be narrowed by various pathologies in the middle ear and in the tympanic membrane that lead to conductive hearing loss. Diagnosing such hearing problems is challenging, however, often relying on subjective hearing tests supported by functional tympanometry. Here we present a method for in vivo 2D mapping of the impulse response of the tympanic membrane, and demonstrate its potential on a healthy human volunteer. The imaging technique is based on interferometric spectrally encoded endoscopy, with a handheld probe designed to scan the human tympanic membrane within less than a second. The system obtains high-resolution 2D maps of key functional parameters including peak response, rise and decay times, oscillation bandwidth and resonance frequency. We also show that the system can identify abnormal regions in the membrane by detecting differences in the local mechanical parameters of the tissue. We believe that by offering a full 2D mapping of broad-bandwidth dynamics of the tympanic membrane, the presented imaging modality would be useful for effective diagnosis of conductive hearing loss in patients., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Acute tympanic membrane perforations primarily occur due to injury or infection in humans. In acute cases, nearly 80-94 % of the perforations heal spontaneously. In chronic cases, non-surgical treatment becomes significantly limited, and the perforation can be restored only by myringoplasty. In addition to classical grafts such as the fascia or cartilage, promising results have been reported with various biological materials including silk or acellular collagen. However, despite of all the efforts, healing remains insufficient. Consequentially, a need for substances which actively promote tympanic cell migration and proliferation is deemed essential. In our study, we utilized Thymosin beta-4 (TB4), a 43aa peptide possessing many regenerative properties in various organ systems. Our aim was to reveal the impact of externally administered TB4 regarding impairments of the middle ear, particularly the tympanic membrane. We harvested tympanic membranes from adult mice and treated these with TB4 or PBS on both collagen gel matrixes and in the form of floating, ex vivo explants. Cell migration and proliferation was measured, while immunocytochemical analyses were performed to determine cell type and the nature of the targeted molecules. We discovered the peptide affects the behavior of epidermal and epithelial cells of the tympanic membrane in vitro. Moreover, as our initial results imply, it is not the differentiated, yet most likely the local epidermal progenitor cells which are the primary targets of the molecule. Our present results unveil a new, thus far undiscovered field regarding clinical utilization for TB4 in the future., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Background: The fusion of computed tomography images with non-echo planner diffusion-weighted magnetic resonance images may overcome the limitations of each individual modality., Objectives: This study aimed to assess the ability of the 'fusion' technique to predict the location of middle ear cholesteatoma by evaluating Its impact on preoperative surgical planning and postoperative results., Methodology: Eighty-three adults with cholesteatoma underwent preoperative CT scans and non-EPI-DW-MRI with or without the 'fusion' technique. We evaluated cholesteatoma localization in both groups, selecting the most appropriate surgical technique, and correlating it with the intraoperative findings. Both groups were compared in terms of residual/recurrent cholesteatoma at one, six and twelve months after surgery., Results: The 'fusion' technique's sensitivity, specificity, and accuracy in predicting the location of cholesteatoma were 97.5. 97.4, and 97.5%, respectively, versus 97.59, 57.69, and 73.21% of CT alone. There was a total operators agreement after the evaluation of the 'fusion' images with the adopted surgical technique. A statistically significant decrease in residual and recurrence cholesteatoma rates was found in group A., Conclusions: The 'fusion' technique provides the surgeon with the precise cholesteatoma location, guiding him in making the correct surgical decision, contributing to the decrease in postoperative residual and recurrence rates.

Schwannomas in the middle ear and external auditory canal are exceedingly rare. The facial nerve, chorda tympani nerve, and Jacobson's nerve have rarely been reported as the origins of primary schwannomas in the middle ear cavity. We experienced a case of carotid sympathetic plexus (CSP) schwannoma that arose from the carotid canal and extended into the middle ear and external auditory canal. The tumor presented bone erosion of the carotid canal, and it adhered tightly to the internal carotid artery. This report represents the first documented case of a CSP schwannoma, which involved the middle ear and external auditory canal.

We report on the metachronic appearance of an inverted papilloma in the ipsilateral middle ear after resection of an endonasal inverted papilloma and its malignant transformation. After multiple surgical interventions and adjuvant radiation and chemotherapy, there was recurrence with intracerebral growth, which ultimately led to the patient's death 3 years after the initial presentation., (© 2022. The Author(s).)

Tumor induced osteomalacia is a rare paraneoplastic syndrome caused by mesenchymal tumors that secrete fibroblast growth factor 23 (FGF23). Patients complain of progressive bone pain, muscle weakness and brittle fractures. Delayed diagnosis of osteomalacia caused by a tumor is often found in clinical practice. When verifying the exact localization of the neoplasm, radical removal within healthy tissues is recommended. The article considers a clinical example of FGF23 tumor induced osteomalacia with localization of neoplasm in the tympanic cavity.

Background: Teratoma is a true neoplasm composed of a number of different types of tissue derived from the three germinal layers but rarely occurs in the middle ear (ME). The features of middle ear teratomas (MET) have not been well described., Objective: The objective of this study is to explore the clinical and imaging features of MET, and report 2 rare cases of MET with ear malformation that have never been reported., Materials and Methods: The clinical, CT and MRI data of 8 patients with a pathological diagnosis of MET were collected and retrospectively mined, and 14 patients with MET reported in previous literature were also reviewed., Results: ① Female, left ear predominance in MET, and the most common symptoms were otorrhea and hearing loss. ② On CT and MRI, the MET presented as an irregular soft tissue mass that was heterogeneous, with fatty tissue and involved multiple sites, and the ET and tympanum were correspondingly expanded and locally destroyed. ③ Mictotia with MET in two patients was presented, which was the first report., Conclusion: MET has female sex and left ear predominance. CT and MRI can be used to diagnose MET and display its extent and its relationship to the carotid canal in detail. Complete surgical excision is the definitive treatment., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)