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.

Imaging of Pathologies of the Temporal Bone and Middle Ear: Inflammatory Diseases, Their Mimics and Potential Complications-Pictorial Review.

Imaging of the temporal bone and middle ear is challenging for radiologists due to the abundance of distinct anatomical structures and the plethora of possible pathologies. The basis for a precise diagnosis is knowledge of the underlying anatomy as well as the clinical presentation and the individual patient's otological status. In this article, we aimed to summarize the most common inflammatory lesions of the temporal bone and middle ear, describe their specific imaging characteristics, and highlight their differential diagnoses. First, we introduce anatomical and imaging fundamentals. Additionally, a point-to-point comparison of the radiological and histological features of the wide spectrum of inflammatory diseases of the temporal bone and middle ear in context with a review of the current literature and current trends is given.

External auditory canal sebaceous carcinoma.

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.

A homozygous missense variant in the PLCB4 gene causes severe phenotype of auriculocondylar syndrome type 2.

Auriculocondylar syndrome (ARCND) is a rare craniofacial birth defect characterized by malformations in the mandible and external ear (Question Mark Ear). Genetically, three distinct subtypes of ARCND (ARCND1, ARCND2, and ARCND3) have been identified. ARCND2 is linked to pathogenic variants in the PLCB4 gene (phospholipase C ß4). PLCB4 is a key effector of the EDN1-EDNRA pathway involved in craniofacial development via the induction, migration, and maintenance of neural crest cells. ARCND2 is typically inherited in an autosomal dominant pattern, with recessive inheritance pattern being rare. In this study, we report the first homozygous missense variant (NM_000933.4: c.2050G>A: p.(Gly684Arg)) in the PLCB4 gene causing ARCND in a 3-year-old patient with a severe clinical phenotype of the syndrome. The patient presented with typical craniofacial ARCND features, in addition to intestinal transit defect, macropenis, and hearing loss. These findings further delineate the phenotypic spectrum of ARCND associated with autosomal recessive PLCB4 loss of function variants. Notably, our results provide further evidence that these variants can result in a more severe and diverse manifestations of the syndrome. Clinicians should consider the rare features of this condition for better management of patients.

The Rate of Eustachian Tube Dysfunction in Adult Patients With Chronic Inflammatory Middle Ear Disease Is Low.

OBJECTIVE: To assess the rate of dilatory (chronic obstructive) eustachian tube dysfunction (ETD) in adult patients scheduled for surgery for chronic inflammatory middle ear disease. PATIENTS: We included adult patients with chronic inflammatory middle ear disease (chronic suppurative otitis media, adhesive otitis media [middle ear atelectasis], cholesteatoma). We excluded patients with pathologies that do not stem from ETD (e.g., noninflammatory chronic middle ear disease [e.g., otosclerosis], tumors, solely external auditory canal conditions), patients scheduled for implantable electronic hearing devices independent of disease, patients with otitis media with effusion and scheduled paracentesis or ventilation tubes only, patients with previous radiotherapy or previous balloon eustachian tuboplasty, and children and patients unable to complete questionnaires. INTERVENTIONS: We evaluated ETD with Valsalva maneuver and in case of negative or unclear Valsalva with the eustachian tube score (ETS). A negative Valsalva maneuver and an ETS score of 5 or lower were used to define dilatory (chronic obstructive) ETD. MAIN OUTCOME MEASURES: Rate of ETD in the included patients. RESULTS: From a total of 482 consecutive patients, 350 patients had positive Valsalva maneuver or ETS score higher than 5. From the 193 patients with negative or unclear Valsalva maneuver, 77 patients had an ETS score of 5 or lower, and 55 rejected further diagnostics with ETS. CONCLUSIONS: Based on a large cohort of adult patients with chronic inflammatory middle ear disease scheduled for middle ear surgery, the majority (82%) seems to have no dilatory (chronic obstructive) ETD that can be detected with current routine clinical methods and tubomanometry. Common pathophysiological explanations should be questioned critically, especially in the context of communication with patients.

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

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

Taste Alteration in External Auditory Canal Cholesteatoma: Indicator of Impending N VII Affection.

INTRODUCTION: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients. METHODS/STUDY DESIGN: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients. RESULTS: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results. DISCUSSION/CONCLUSION: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve.

Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis.

Langerhans cell histiocytosis is a rare condition affecting the temporal bone in up to 60% of cases. Symptoms are non-specific and the differential diagnosis includes infection, benign lesions such as cholesteatoma, and malignant lesions of the skull base. Here, we report the case of a 14-yearold child referred with chronic ear discharge, and background of multifocal Langerhans cell histiocytosis 9 years prior. Recurrence of Langerhans cell histiocytosis was initially suspected and systemic treatment was considered. Further imaging workup and surgical exploration of the mastoid showed a secondary acquired cholesteatoma arising from a dehiscent posterior ear canal wall. Surgical removal of the cholesteatoma was performed with a canal wall down procedure. We review the presentation and management of temporal bone Langerhans cell histiocytosis. We recommend that cholesteatoma should be considered in case of recurrence of otological symptoms in patients with a background of Langerhans cell histiocytosis.

Mast Cells Are Activated in the Giant Earlobe Keloids: A Case Series.

Mast cells and inflammatory cells are abundant in keloid and hypertrophic scar tissues. Even if the cause of physical injury is similar, such as piercing or scratching with hands, clinical findings show differences in the size of keloids in the same area. Hence, we performed histological studies on giant keloids larger than the earlobe, and other smaller keloids. We also examined the risk factors associated with the formation of giant lesions. No statistically significant differences in the association of the risk factors were observed. However, histological observations clearly showed a high number of degranulated or active mast cells with a trend towards a greater number of degranulated mast cells in the giant keloid tissues. Collagen production also tended to increase. Two patients with giant keloids were severely obese, suggesting that the persistent inflammatory state of obesity may also be involved in the growth of keloid lesions.

Quiste epidermoide en conducto auditivo externo: reporte de caso / Epidermoid cysts of the external auditory canal: case report

Resumen El quiste epidérmico es una lesión benigna y común de la piel. Se desarrolla por un bloqueo de la unidad pilosebácea, con la consecuente proliferación de células epiteliales y secuestro de queratina. El 7% se desarrollan en cabeza y cuello, sin embargo, son infrecuentes en canal auditivo externo. Su patrón de crecimiento es lento y progresivo durante años, siendo asintomáticos. Al aumentar de tamaño causan sintomatología variable, acorde a su localización; en el canal auditivo tienen un comportamiento obstructivo que genera síntomas como otalgia e hipoacusia. Se presenta caso de una paciente de 69 años, con acúfeno e hipoacusia progresiva derecha. Durante la otoscopia se observó una neoformación obstructiva del 100% de la luz del conducto. Se realizaron estudios de imagen que reportaron tumoración de características quísticas de conducto auditivo derecho, bien circunscrita, sin erosión ósea. Para el diagnóstico definitivo, se realizó resección quirúrgica y biopsia reportando quiste epidérmico. Durante el seguimiento posoperatorio sin hallazgos de recidiva.

Abstract The epidermal cyst is a common and benign lesion of the skin. It develops due to a blockage of the pilosebaceous unit, with the consequent proliferation of epithelial cells and keratin sequestration. Seven percent develop in the head and neck; however, they are infrequent in the external auditory canal. Its growth pattern is slow and progressive over the years, being asymptomatic. As they increase in size, they cause variable symptoms, according to their location. In the ear canal they have an obstructive behavior that generates symptoms such as earache and hearing loss. A case of a 69-year-old female with tinnitus and progressive right hearing loss is presented. At otoscopy, a 100% obstructive neoformation of the canal lumen was observed. Imaging studies showed a well circumscribed, cystic tumor of the right ear canal, without bone erosion. For the definitive diagnosis, a resection and biopsy were performed, reporting an epidermal cyst. During follow up there was no recurrence of tumor.

Patulous eustachian tube after balloon eustachian tuboplasty in a post-radiation patient.

BACKGROUND: Balloon eustachian tuboplasty (BET) is a minimally invasive surgical treatment that is effective and safe for obstructive eustachian tube dysfunction. However, BET complications include excessive widening of the eustachian tube, causing a patulous eustachian tube (PET). Herein, we report a case of PET following BET in a patient who underwent radiation therapy and reviewed the literature on considerations for reducing complications after BET. CASE PRESENTATION: A 63-year-old woman complained of bilateral ear fullness after concurrent chemoradiation therapy for nasopharyngeal lymphoma. BET was performed on the left side because the left-sided serous otitis media persisted. A left-sided PET was performed two weeks after the BET, along with eustachian tube silicone plug insertion on the left side. The patient became asymptomatic immediately after the surgery, with no recurrence reported after a 12-month follow-up period. CONCLUSIONS: To our knowledge, there has been no report of PET following BET in a post-radiation patient, and it was successfully treated via ET silicone plug insertion.

Patulous Eustachian Tube Patients With Oculopharyngeal Muscular Dystrophy.

OBJECTIVES: To describe cases of patulous Eustachian tube (PET) or patent ET conditions in oculopharyngeal muscular dystrophy (OPMD). PATIENTS: Four cases of PET or patent ET conditions with OPMD. MAIN OUTCOME MEASURES: Clinical case records, objective ET function tests (tubo-tympano-aerodynamic graphy and sonotubometry), and swallowing function (videoendoscopic examination and Food Intake Level Scale) were analyzed. RESULTS: Two cases of definite PET, one case of possible PET, and one case lacking aural symptoms with findings of patent ET. All patients have ptosis, and three cases have dysphagia. Body mass index indicated that three cases were underweight. Magnetic resonance imaging in case 4 showed atrophy and fat replacement of palatine and masticatory muscles. CONCLUSIONS: It is important to consider PET or patent ET conditions when OPMD patients describe aural symptoms.

Fibroepithelial Polyp of the External Auditory Canal in a 2-Year-Old Child.

Here, we report a unique case in which a fibroepithelial polyp was found in the cartilaginous part of the external auditory canal of a 2-year-old child. The polyp was successfully treated by excision using an endaural approach and healed without complications. This is the very first report of a fibroepithelial polyp in the external auditory canal in the pediatric population. Although fibroepithelial polyp is an extremely rare diagnosis, it should be considered in the differential diagnosis of a child's external auditory canal polyp.

Endoscopic Transcanal and Transmastoid Laser-Assisted Resection of Middle Ear Capillary Hemangioma.

OBJECTIVE: To present a rare case of a middle ear capillary hemangioma in an adult. PATIENT: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss. INTERVENTION: Endoscopic laser-assisted resection. MAIN OUTCOME MEASURES: Clinical, radiographic, and histopathological findings of a capillary hemangioma. RESULTS: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively. CONCLUSIONS: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization.

Merocel compression dressing: a novel modified surgical technique for treating auricular pseudocyst.

PURPOSE: Auricular pseudocyst (AP) is a benign, noninflammatory swelling to the ear, located on either the front or side surface. Deroofing surgery with variable compression methods is considered the most effective method. However, post-operative wound pain is the main drawback following compression. We are introducing a novel painless surgical procedure which involves deroofing technique followed by Merocel® compression dressing. METHODS: From 2015 to 2020, thirty-one patients with AP received this new surgical compression method in our university-affiliated tertiary hospital. Retrospective chart review and the analysis of the results were conducted. RESULTS: All patients had unilateral lesions, with left side lesions (58.1%) predominant. The concha cymba (38.7%) and concha cavum (35.8%) were the most common sites. Previous aspiration or drainage had been performed for the cysts in eight patients (25.8%). Only one patient had the recurrence after post-operative 3 months and received the revised surgery without following recurrence and discomfort. Among all patients, 29 (93.5%) patients claimed minimal pain around 0 or 1 in numeric rating scale of pain score. Three patients had mild ecchymosis but recovered after conservative treatment. One patient had mild auricular deformity after surgery due to pre-treatment partial cartilage necrosis. All patients had follow-up for at least 6 months. CONCLUSION: This novel Merocel® compression dressing technique to treat AP after deroofing is an effective procedure. Minimal pain, simple to do and easily acquired materials were the advantages of this novel procedure.

Chondrodermatitis Nodularis Nasi: A Case Report of a Rare Variant of Chondrodermatitis Nodularis Helicis.

ABSTRACT: Chondrodermatitis nodularis helicis is an inflammatory condition affecting the helix or antihelix of the ear. It is commonly described as a solitary, painful, ulcerated nodule affecting the cartilage or skin because of continuous pressure, trauma, sun exposure, or ischemic changes. In this case report, we present a rare variant of chondrodermatitis, named chondrodermatitis nodularis nasi, which affects the skin and cartilage of the nose. Clinical resemblance to neoplasms of the skin such as squamous cell carcinoma and basal cell carcinoma requires that a biopsy be performed for definitive diagnosis. On histopathological examination, chondrodermatitis presents as a central ulcer bordered by epidermal hyperplasia, hypergranulosis, hyperkeratosis, and parakeratosis with fibrin deposits in the papillary dermis admixed with sparse inflammatory cells. Included in this case report is a discussion of the clinical and histopathology of chondrodermatitis nodularis nasi. With increased frequency of diagnosing this rare variant, better management and treatments can be explored.

New locus underlying auriculocondylar syndrome (ARCND): 430 kb duplication involving TWIST1 regulatory elements.

BACKGROUND: Auriculocondylar syndrome (ARCND) is a rare genetic disease that affects structures derived from the first and second pharyngeal arches, mainly resulting in micrognathia and auricular malformations. To date, pathogenic variants have been identified in three genes involved in the EDN1-DLX5/6 pathway (PLCB4, GNAI3 and EDN1) and some cases remain unsolved. Here we studied a large unsolved four-generation family. METHODS: We performed linkage analysis, resequencing and Capture-C to investigate the causative variant of this family. To test the pathogenicity of the CNV found, we modelled the disease in patient craniofacial progenitor cells, including induced pluripotent cell (iPSC)-derived neural crest and mesenchymal cells. RESULTS: This study highlights a fourth locus causative of ARCND, represented by a tandem duplication of 430 kb in a candidate region on chromosome 7 defined by linkage analysis. This duplication segregates with the disease in the family (LOD score=2.88) and includes HDAC9, which is located over 200 kb telomeric to the top candidate gene TWIST1. Notably, Capture-C analysis revealed multiple cis interactions between the TWIST1 promoter and possible regulatory elements within the duplicated region. Modelling of the disease revealed an increased expression of HDAC9 and its neighbouring gene, TWIST1, in neural crest cells. We also identified decreased migration of iPSC-derived neural crest cells together with dysregulation of osteogenic differentiation in iPSC-affected mesenchymal stem cells. CONCLUSION: Our findings support the hypothesis that the 430 kb duplication is causative of the ARCND phenotype in this family and that deregulation of TWIST1 expression during craniofacial development can contribute to the phenotype.

Screening Anti-Inflammatory Effects of Flavanones Solutions.

There are a large number of remedies in traditional medicine focused on relieving pain and inflammation. Flavanones have been a potential source in the search for leading compounds and biologically active components, and they have been the focus of much research and development in recent years. Eysenhardtia platycarpa is used in traditional medicine for the treatment of kidney diseases, bladder infections, and diabetes mellitus. Many compounds have been isolated from this plant, such as flavones, flavanones, phenolic compounds, triterpenoid acids, chalcones, sugars, and fatty acids, among others. In this paper, natural flavanone 1 (extracted from Eysenhardtia platycarpa) as lead compound and flavanones 1a-1d as its structural analogues were screened for anti-inflammatory activity using Molinspiration® and PASS Online in a computational study. The hydro alcoholic solutions (FS) of flavanones 1, 1a-1d (FS1, FS1a-FS1d) were also assayed to investigate their in vivo anti-inflammatory cutaneous effect using two experimental models, a rat ear edema induced by arachidonic acid (AA) and a mouse ear edema induced by 12-O-tetradecanoylphorbol acetate (TPA). Histological studies and analysis of pro-inflammatory cytokines TNF-α, IL-1ß, and IL-6 were also assessed in AA-inflamed rat ear tissue. The results showed that the flavanone hydro alcoholic solutions (FS) caused edema inhibition in both evaluated models. This study suggests that the evaluated flavanones will be effective when used in the future in skin pathologies with inflammation, with the results showing 1b and 1d to be the best.