Bilateral External Auditory Canal Research Articles

A New Case of Nager Syndrome as a Rare Cause of Acrofacial Dysostosis

Introduction: Nager acrofacial dysostosis (#154400) is a rare and mostly sporadic malformation syndrome characterized by craniofacial and extremity findings. In the study, a new case diagnosed in the neonatal period will be presented. Case presentation: The neonatal intensive care unit consulted to our pediatrics genetic clinic for a 2-week-old male patient, who was being followed up in their unit, due to his dysmorphic findings and extremity defects. In the physical examination, downward palpebral fissures, zygomatic bone hypoplasia, mandibular hypoplasia, retromicrognathia, bilateral microtia, bilateral external auditory canal atresia, and bilateral thumb agenesis were observed. Direct radiographs showed left radius hypoplasia and bilateral thumb agenesis. Nager syndrome was considered in the presence of typical craniofacial findings, preaxial extremity deformities, and radiological findings. In the SF3B4 sequence analysis, c.737dupC p.(V247Sfs*239) heterozygous variant was detected. Conclusion: As a result of the segregation analysis, it was demonstrated that the variant was de novo. Nager acrofacial dysostosis should be considered in the patients with craniofacial malformations and radial ray findings.

Three-Dimensional Computed Tomography Scanning Study of the Superior Labial Artery in Chinese Individuals for Assessing Filler Injection Safety.

Lip filler injection is one of the most common minimally invasive cosmetic procedures involving the face; however, vascular complications are not uncommon. The aim of this study was to investigate the anatomy of the superior labial artery (SLA) and provide precise topographic information for dermal filler injection into the lips. Computed tomography (CT) scans of 52 cadaveric heads injected with lead oxide were obtained. We then used Mimics software to construct 3D images of the SLA described by a coordinate system based on the bilateral external auditory canal and the left orbit. This study aimed to classify the SLA in the Han Chinese population, measure its diameter at specific points, and determine the thickness of the lip at those points. Ultimately, we utilized a thermal imaging technique to illustrate the course and depth of the SLA within the lip. The objective of this study was to provide safe guidance for clinical injections. In this study, the SLA was successfully identified in all cadavers. The mean overall diameter of the superior labial arteries was 1.36±0.28 mm. The superior labial artery showed a general course from deep to shallow with an average depth of 5.68±1.68 mm from the oral commissure to the midline. There are anatomical differences in the superior labial arteries among Chinese people. Furthermore, 3D CT images can digitally elucidate the exact positions of the superior labial artery via a coordinate system, improving the safety of upper lip filler injections in clinical settings. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Management of a Case of Bilateral Severe External Auditory Canal Stenosis, Bilateral Cholesteatoma Causing Right-sided Inner Ear Erosion, and Bilateral Profound Sensorineural Hearing Loss

Bilateral simultaneous external auditory canal (EAC) stenosis with bilateral cholesteatoma is a rare presentation. We present a case report of surgical and audiological management of an elderly male who presented with bilateral EAC stenosis, bilateral cholesteatoma, right-sided inner ear erosion, and bilateral profound sensorineural hearing loss.

800 A Rare Cause of Unilateral Facial Palsy in an Adult Presenting to a Tertiary ENT Centre

Abstract Introduction Sinonasal inverted papilloma are benign tumors usually appearing in 5th decade of life. They are characterised by their recurrent nature with extensive local destruction and malignant potential. Case Report A 54-year-old male was referred to our tertiary centre for further management of recurring Inverted papillomas. On review in clinic, he was found to have right facial palsy- House Brackman (HB) grade V with extensive disease in nasal passages and external auditory canal (EAC) bilaterally. This was associated with reduced visual acuity (VA)- 6/12 and proptosis of the right eye. CT Head was suggestive of a lesion involving the right mastoid, external and middle ear, extending into the right middle cranial fossa is likely to account for the right facial nerve palsy. The MRI scan showed multiple enhancing massess in the nasal cavity, the right mastoid and tympanic cavity, the left tympanic cavity with further possible disease in the oropharynx and nasopharynx. He was admitted to hospital from clinic for surgical management and underwent Endoscopic debulking of nasal tumour + Biopsy of bilateral external auditory canals + biopsy of pharynx. The patient was discharged a day after undergoing the procedure and remained well on ENT follow-up visit. He was further referred to Oncology team once histology results were available which showed Nonkeratinising sinonasal carcinoma and is due to receive his first dose of chemotherapy shortly. Conclusion The study showcases the need for awareness in such conditions and potential complications that can arise due to disease progression.

Toward automatic reformation at the orbitomeatal line in head computed tomography using object detection algorithm.

Consistent cross-sectional imaging is desirable to accurately detect lesions and facilitate follow-up in head computed tomography (CT). However, manual reformation causes image variations among technologists and requires additional time. We therefore developed a system that reformats head CT images at the orbitomeatal (OM) line and evaluated the system performance using real-world clinical data. Retrospective data were obtained for 681 consecutive patients who underwent non-contrast head CT. The datasets were randomly divided into one of three sets for training, validation, or testing. Four landmarks (bilateral eyes and external auditory canal) were detected with the trained You Look Only Once (YOLO)v5 model, and the head CT images were reformatted at the OM line. The precision, recall, and mean average precision at the intersection over union threshold of 0.5 were computed in the validation sets. The reformation quality in testing sets was evaluated by three radiological technologists on a qualitative 4-point scale. The precision, recall, and mean average precision of the trained YOLOv5 model for all categories were 0.688, 0.949, and 0.827, respectively. In our environment, the mean implementation time was 23.5 ± 2.4s for each case. The qualitative evaluation in the testing sets showed that post-processed images of automatic reformation had clinically useful quality with scores 3 and 4 in 86.8%, 91.2%, and 94.1% for observers 1, 2, and 3, respectively. Our system demonstrated acceptable quality in reformatting the head CT images at the OM line using an object detection algorithm and was highly time efficient.

Hearing rehabilitation with osseointegrated hearing implant in bilateral congenital external auditory canal atresia

Introduction and objectivesCongenital atresia of the external auditory canal (EAC) is a congenital defect present in one in every 10,000–20,000 births. It causes conductive hearing loss, with an air-bone gap of 50–60dB. Early amplification is essential in bilateral cases to ensure normal language development. The aim of this study is to present the osseointegrated hearing implant as a treatment for bilateral EAC atresia, reviewing the audiometric results and the rate of complications. Material and methodsRetrospective analysis of patients diagnosed with bilateral congenital EAC atresia under follow-up in the pediatric ENT clinic of the ENT and Head and Neck Surgery department of a Portuguese Tertiary Hospital, between 2003 and 2019. We reviewed the medical records and collected information on the assessment of the initial audiometric status. In the cases submitted for implantation with an osseointegrated hearing implant, we analyzed the details of follow-up, including immediate and long-term post-operative complications, as well as the audiometric results. ResultsWe present 8 pediatric patients, 6 girls and 2 boys, with a diagnosis of bilateral congenital EAC atresia. The audiometric assessment revealed moderate to severe bilateral conductive hearing loss with a mean speech recognition threshold (SRT) of 51dB. Six patients underwent osseointegrated hearing implantation. All 6 patients showed good audiometric results, with an average SRT of 20dB and closure of the air-bone gap. ConclusionsThe osseointegrated hearing implant was an effective treatment option in these patients, without significant morbidity or complications. Osseointegrated hearing implantation should be considered first line treatment for children with bilateral congenital EAC atresia, as it presents good functional results and a high level of patient satisfaction.

Hearing rehabilitation with osseointegrated hearing implant in bilateral congenital external auditory canal atresia

Introduction and objectivesCongenital atresia of the external auditory canal (EAC) is a congenital defect present in one in every 10,000–20,000 births. It causes conductive hearing loss, with an air-bone gap of 50–60dB. Early amplification is essential in bilateral cases to ensure normal language development. The aim of this study is to present the osseointegrated hearing implant as a treatment for bilateral EAC atresia, reviewing the audiometric results and the rate of complications. Material and methodsRetrospective analysis of patients diagnosed with bilateral congenital EAC atresia under follow-up in the pediatric ENT clinic of the ENT and Head and Neck Surgery department of a Portuguese Tertiary Hospital, between 2003 and 2019. We reviewed the medical records and collected information on the assessment of the initial audiometric status. In the cases submitted for implantation with an osseointegrated hearing implant, we analyzed the details of follow-up, including immediate and long-term post-operative complications, as well as the audiometric results. ResultsWe present 8 pediatric patients, 6 girls and 2 boys, with a diagnosis of bilateral congenital EAC atresia. The audiometric assessment revealed moderate to severe bilateral conductive hearing loss with a mean speech recognition threshold (SRT) of 51dB. Six patients underwent osseointegrated hearing implantation. All 6 patients showed good audiometric results, with an average SRT of 20dB and closure of the air-bone gap. ConclusionsThe osseointegrated hearing implant was an effective treatment option in these patients, without significant morbidity or complications. Osseointegrated hearing implantation should be considered first line treatment for children with bilateral congenital EAC atresia, as it presents good functional results and a high level of patient satisfaction.

Light Chain Amyloidosis Presenting as Bilateral External Auditory Canal Obstructing Masses.

: A morbidly-obese 57-year-old diabetic and hypertensive man with chronic kidney disease, diastolic heart failure, and bilateral hearing loss was found to have soft tissue masses/densities in the lateral aspect of both auditory canals on local examination and on imaging. He underwent biopsies of both ear canal masses and histologic examination revealed amyloid deposits in the dermis. These deposits were confirmed as AL (amyloid light chain) kappa-type amyloid by laser mass spectrometry. A systemic work-up showed plasma cell dyscrasia with 9% kappa light chain restricted plasma cells in the bone marrow as well as amyloid deposits on a kidney biopsy.Involvement of the external auditory canals is a rare manifestation of systemic amyloidosis with only 13 cases reported so far, of which four cases had bilateral external auditory canal involvement, associated with multiple myeloma. Interpretation of small biopsies can be challenging especially with early, scant amyloid deposits, but a sufficient biopsy to type amyloid by immunohistochemistry or laser mass spectrometry is imperative for further management. Early diagnosis and treatment of systemic light chain amyloidosis translates to better patient outcomes, while delay in management could lead to dismal prognosis.

A Case of Squamous Cell Carcinoma of the Bilateral External Auditory Canals

A Case of Squamous Cell Carcinoma of the Bilateral External Auditory Canals

Peripheral T-Cell Lymphoma Presenting as an Auricle Mass

Peripheral T-Cell Lymphoma Presenting as an Auricle Mass

Metachronous squamous cell carcinoma of bilateral external auditory canals: A rare case report

Squamous cell carcinoma of the external auditory canal (EAC) is a rare and unusual entity with an incidence of 0.2% cases of all head and neck carcinomas has been reported in the literature, and even fewer describe bilateral tumors. Its clinical presentation is similar to chronic otitis media or otitis externa. Hence, the diagnosis often delayed. Early diagnosis is directly related to the prognosis of the patient. The surgical treatment is a challenging task for the experienced skull base surgeon. Here, we report a case of metachronous bilateral EAC carcinoma in a 33-year-old male. The gap between the development of left and right side carcinoma is 3 years.

Trauma patterns, symptoms, and complications associated with external auditory canal fractures.

External auditory canal (EAC) trauma, although rare, can have significant long-term adverse outcomes. This study aims to investigate the frequency, treatment, and complications of external ear canal injury in association with mandibular and temporal bone trauma. Retrospective chart review. Computed tomography images with mandibular or temporal bone trauma were reviewed for EAC fractures. Patient data were collected from initial presentation and subsequent follow-up clinic visits. Thirty-nine percent of temporal bone fractures and 3.3% of mandible trauma involved the EAC. In particular, 10% of condylar or subcondylar trauma included an EAC fracture (P = 0.0006). One patient sustained bilateral EAC fractures despite an isolated, unilateral condylar fracture. The most common presenting sign was blood in the external auditory canal. Two patients underwent exam under anesthesia and removal of debris and stenting as treatment, whereas 42% of the patients were placed on otic drops and 5% received packing or a stent. Follow-up data were only available for 16% of the patients. Hearing loss from otic capsule involvement or ossicular chain disruption were follow-up complaints, and one patient had persistent canal stenosis. External auditory canal trauma is present in a significant proportion of mandibular and temporal bone trauma, including both condylar and noncondylar fractures with a higher incidence of condylar fractures. One case was seen with bilateral EAC fractures despite a unilateral mandibular fracture. Complications of these fractures can include hearing loss and canal stenosis; however, additional outpatient follow-up is needed to further elucidate long-term complications and shape treatment recommendations.

Osseointegrated Hearing Device Placement in Congenital Lamellar Ichthyosis

To present a case of osseointegrated hearing device placement in a child with conductive hearing loss related to manifestations of congenital lamellar ichthyosis. A 5-year-old female patient with congenital lamellar ichthyosis resulting in conductive hearing loss because of bilateral external auditory canal stenosis and tympanic membrane blunting. Unilateral osseointegrated hearing device placement using a traditional skin flap technique. Frequency and severity of adverse skin reactions, device usage, and audiometric testing. After 51 months of follow-up postoperatively, the patient has only required 2 treatments for minor skin reactions (Holgers Grade I). Aided speech reception threshold was 15 dB hearing level (HL) compared to 35 dB HL unaided. The subject has used the device continuously with parental report of improvement in school performance. Osseointegrated hearing device placement may be a viable option in patients with congenital lamellar ichthyosis despite the skin-related comorbidities known to be associated with this disease condition.

Keratinic amyloidosis of the external auditory canal

We present a rare case of keratinic amyloidosis of the external auditory canal. This is only the seventh case reported of localized cutaneous amyloidosis of the external auditory canal with no systemic symptoms. A 62-year-old man, who had complained of an itchy external auditory canal and left-side hearing loss, was referred to our hospital because of a bilateral external auditory canal mass. Biopsy of the external auditory canal mass suggested a diagnosis of amyloidosis. However, total systemic examination failed to identify any disease due to systemic amyloidosis. This led us to diagnose him with localized cutaneous amyloidosis of the external auditory canal. We follow up periodically with systemic examination and local observation. Thirty months after the initial diagnosis, he remains in follow-up and has not shown any significant aggravation of the disease. In previous cases, the chief complaints were itching sensations and pain in the external auditory canal as well as a sense of discomfort when wearing a hearing aid. This suggests that chronic stimulation and inflammation of the skin lining the external auditory canal induced amyloidosis.

Infantile Donovanosis Presenting as External Auditory Canal Polyps

Two infants, 6 months and 4 months of age, presented with bilateral or unilateral external auditory canal polyps and otorrhea, respectively. Additional findings on examination included otitis media and mastoiditis. Tympanic membrane perforation was noted in one patient and a postauricular abscess in the other. Incisional biopsies of the polyps and abscess were reported as nonspecific mixed inflammation and abscess wall, respectively. There was a limited response to an empirical 5-day course of trimethoprim sulfamethoxazole. The children were referred to the academic hospital, and excision of the polyps and biopsies of the middle ear, mastoid, and postauricular abscess was undertaken. All the biopsies demonstrated donovanosis. Reappraisal of the initial incisional biopsies also confirmed donovanosis. Trimethoprim sulfamethoxazole was administered to both patients for 3 weeks, with resolution of the lesions. Subsequent investigations confirmed genital tract donovanosis, human immunodeficiency virus seropositivity, acquired immunodeficiency syndrome, and pulmonary tuberculosis in both mothers. Heightened awareness of the occurrence of donovanosis at unusual sites and improved recognition of the histomorphological features of the disease, especially in small and superficial biopsies, are pivotal not only for its correct diagnosis in extragenital cutaneous and extracutaneous locations but also for timely and adequate therapy and an improved infant and maternal outcome.

Middle ear implant in conductive and mixed congenital hearing loss in children

PurposeActive middle ear implant can be used in children and adolescents with congenital hearing loss. The authors report their experience with the semi implantable Medel Vibrant Soundbridge® (VSB) in the audiologic rehabilitation of such patients. MethodsIn this retrospective study, audiological and surgical data of 10 children (10.5±4 years) implanted with 12 VSB in 2 tertiary cares ENT Departments were analysed. ResultsTwo children with bilateral external auditory canal (EAC) atresia and mixed hearing loss (mean air conduction (AC) thresholds=65dB HL) were bilaterally implanted. Eight children presented with microtia associated with EAC atresia bilaterally (n=3) and unilaterally (n=5). All of them had a conductive hearing loss in the implanted ear (mean (AC) thresholds were 58.75dB HL preoperatively). The Floating Mass Transducer was crimped on the long process of the incus (n=8) or on the suprastructure of the stapes (n=4). There were no intra- or postoperative surgical complications. All the children wore their implants after 5 weeks. Postoperative mean bone conduction (BC) thresholds were unchanged. The mean aided thresholds with VSB (four frequencies warble tones at 0.5, 1, 2 and 4kHz) were 28dB HL (±10). Word discrimination threshold in quiet conditions in free field with the VSB unilaterally activated was 50% at 38dB SPL (±9). ConclusionThe results indicate that satisfaction of the children and their parents is very encouraging but surgeons should be cautious with this new approach in relation to the pinna reconstruction and to possible risks to inner ear and facial nerve.

Exostoses of the external auditory canal

Auditory exostoses are bone masses located in the external auditory canal. Currently, most researchers agree that the environment (especially water temperature, but also atmospheric temperature and wind action) plays a pivotal role in the development of exostosis. Exostoses are a condition in which the external auditory canal becomes stenotic secondary to hyperplastic bone formation. Also known as ‘‘surfer’s ear,’’ exostoses are thought to be a reactive process from repeated stimulation by cold water because it occurs commonly in populations such as beach lifeguards and surfers. Exostoses are usually asymptomatic and discovered on routine otoscopy. Rarely, exostoses become sufficiently obstructive to impair hearing or cause recurrent otitis externa. Exostoses are almost always bilateral, symmetric, and occurring in the medial portion of the external auditory canal. Tthe authors report a case of bilateral external auditory canal exostoses in an adult male treated with surgical excision. DOI: http://dx.doi.org/10.3329/bjo.v18i1.10428 Bangladesh J Otorhinolaryngol 2012; 18(1): 91-95

耳内異物を認めた被虐待児症候群例

A case is presented of an infant who presented with foreign bodies in bilateral external auditory canals. Battered child syndrome was defined in 1962 by Kempe et al. as nonaccidental attack or injury repeatedly inflicted upon children by persons caring for them. Otolaryngologists are rarely in a position to diagnose and initiate management of child abuse presenting as injury to the head and neck area. The number of reported cases of child abuse has increased dramatically over the past several years and it is likely that physicians will examine such cases and be unaware of the fact that the child is being abused.

Primary lymphoma of bilateral external auditory canals

We report a rare case of primary lymphoma originating in the bilateral external auditory canals. A 49-year-old man presented with cauliflower-like tumors on both of his external auditory canals. Immunohistopathologic examination and immunoglobulin heavy chain gene rearrangement studies led to a diagnosis of B-cell lymphoma. He had no other systemic involvement. After chemotherapy, total gross tumor removal by surgery was achieved, and no recurrence has been detected during 1 year of follow-up. To our knowledge, this is the third case of malignant lymphoma originating in the external auditory canal to be reported in the literature, and this is the first report that includes immunohistochemical findings of fresh tissue. (Am J Otolaryngol 2002;23:49-52. Copyright © 2002 by W.B. Saunders Company)

Otolaryngologic manifestations of Rosai–Dorfman disease

Purpose: To describe an unusual head and neck occurrence of Rosai–Dorfman Disease (RDD) and to review the otolaryngologic manifestations of this rare entity. Methods: A case presentation with review of the literature describing Rosai–Dorfman Disease and its head and neck involvement. Setting: A tertiary care, urban children's hospital. Results: This is the first description, to the best of our knowledge, of RDD [Sinus Histiocytosis with Massive Lymphadenopathy (SHML)] involving bilateral external auditory canals and middle ear in a 12 year old patient previously diagnosed with ‘asthma’ and hearing loss. This patient also had extensive involvement of the tracheobronchial tree. Representative clinical, radiographic and histological findings are presented. Its etiology, diagnosis and management are also reviewed. Conclusion: This is the first reported case of middle ear and external auditory canal involvement of RDD in a patient with hearing loss and associated tracheobronchial lesions. RDD, although rare, may be considered in the differential diagnosis of unusual histiocytic lesions of the external auditory canal, especially with findings of similar or comparable lesions involving the respiratory tract. Confirmation is with identification of emperipolesis and appropriate immunohistochemical staining (S-100 positive, CD-68 positive and CD-1a negative). Intervention is recommended in cases where there is increased risk of mortality, as in severe obstruction of the tracheobronchial tree. Otherwise, since these lesions are self-limiting, the patients can be observed closely.