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

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

Pilomatricoma: A Case Report.

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

Hematohidrosis: A Rare Case of Bleeding Ears.

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

Results of Modified Stenströms Technique Otoplasty and Patients' Satisfaction at King Fahad Armed Forces Hospital (KFAFH).

Background Globally, the prevalence of protruding ears is relatively frequent. Ear deformities manifest due to underdevelopment of the antihelical fold, conchal hypertrophy, and/or an obtuse conchoscaphal angle. The availability of multiple approaches proves that there isn't a single optimal accepted procedure. The Modified Stenström otoplasty technique supports the surgeon in the management of underdeveloped antihelix fold, conchal hypertrophy, and obtuse conchoscaphal angle among other deformities. We are the first to evaluate the clinical effects and measure the satisfaction rate post-otoplasty using the modified Stenström technique with a case series study. Methods Six patients were included in the study with a total of 12 ears operated on between February 2021 and July 2022. Utilizing the modified Stenström technique for bilateral protruding ears. All patients had six postoperative follow-up visits with fixed intervals; one week, three weeks, six weeks, three months, six months, and one year. During their one-year postoperative follow-up appointment, all patients completed the satisfaction survey questions. Results Six individuals were studied, three males and three females with a mean age of 23.1 (range, 7-53 years old). There were no complications or recurrences observed. Based on the responses we collected, all patients reported a high satisfaction rate at one-year postoperative follow-up. Conclusion The modified Stenström technique yields good naturally appearing ears. It is an easy and safe technique to apply. It has a short recovery period, and no hospital stay is required. All contribute to a high satisfaction rate among studied patients.

Perception of cauliflower ear amongst combat sport athletes.

OBJECTIVE: Cauliflower ear, or "hematoma auris," is a permanent condition that is typically viewed as a deformity. Despite this, it has anecdotally been observed that combat sport athletes view cauliflower ear as a respected aesthetic trait. This study characterizes and quantifies the differences in initial impressions of subjects with cauliflower ear between combat sport athletes and the general population. METHODS: In a cross-sectional survey, participants were shown frontal and profile views of four subjects with cauliflower ear and five control subjects. Respondents rated the subjects on a scale of 0-100 in perceived success, attraction, approachability, and affect display. Numeric scores were compared between combat sport athletes and the general population. Additionally, first impressions were categorized into positive, neutral, or negative classes via latent class analysis (LCA). RESULTS: 678 combat sport athletes and 129 general casual observers participated in the survey. Combat sport athletes rated subjects with cauliflower ear significantly more favorably than respondents in the general population in all personal attributes: perceived success (+4.03, 95 % CI:1.8-6.2, p = 0.0003), attractiveness (+4.11, 95 % CI:1.8-6.4, p = 0.0005), approachability (+11.57, 95 % CI: 8.4-14.7, p < 0.0001), and affect display (+4.14, 95 % CI: 1.9-6.3, p = 0.0002). They also had approximately seven times greater odds (95 % CI:4.0-12.6, p < 0.001) of reporting a positive first impression of a person with cauliflower ear than the general population. CONCLUSION: Confirming anecdotal observation, the combat sports group had a more positive perception of cauliflower ear than the general population. Conditions that are typically viewed as deformities can be looked upon favorably in specific subpopulations.

Identification of a novel phenotype of external ear deformity related to Coffin-Siris syndrome-9 and literature review.

De novo germline variants of the SRY-related HMG-box 11 gene (SOX11) have been reported to cause Coffin-Siris syndrome-9 (CSS-9), a rare congenital disorder associated with multiple organ malformations, including ear anomalies. Previous clinical and animal studies have found that intragenic pathogenic variant or haploinsufficiency in the SOX11 gene could cause inner ear malformation, but no studies to date have documented the external ear malformation caused by SOX11 deficiency. Here, we reported a Chinese male with unilateral microtia and bilateral sensorineural deafness who showed CSS-like manifestations, including dysmorphic facial features, impaired neurodevelopment, and fingers/toes malformations. Using trio-based whole-exome sequencing, a de novo missense variant in SOX11 (NM_003108.4: c.347A>G, p.Y116C) was identified and classified as pathogenic variant as per American College of Medical Genetics guidelines. Moreover, a systematic search of the literature yielded 12 publications that provided data of 55 SOX11 intragenic variants affecting various protein-coding regions of SOX11 protein. By quantitatively analyzing phenotypic spectrum information related to these 56 SOX11 variants (including our case), we found variants affecting different regions of SOX11 protein (high-mobility group [HMG] domain and non-HMG regions) appear to influence the phenotypic spectrum of organ malformations in CSS-9; variants altering the HMG domain were more likely to cause the widest range of organ anomalies. In summary, this is the first report of CSS with external ear malformation caused by pathogenic variant in SOX11, indicating that the SOX11 gene may be not only essential for the development of the inner ear but also critical for the morphogenesis of the external ear. In addition, thorough clinical examination is recommended for patients who carry pathogenic SOX11 variants that affect the HMG domain, as these variants may cause the widest range of organ anomalies underlying this condition.

A Case of Squamoid Eccrine Ductal Carcinoma of the Auricle Mimicking Perichondritis.

The subtype of eccrine carcinoma known as squamoid eccrine ductal carcinoma (SEDC) is rare; only 38 cases, including only 6 cases in the ear, have been documented in the literature. This may be the first case to focus on the fact that SEDC, located within the dermal and subcutaneous layers, spares the epidermis histopathologically, which can cause clinicians to confuse SEDC with acute perichondritis.

"Mesmerizing and Terrifying": Senusret III's Unique Macrotia.

The ancient Egyptian pharaoh Senusret III was a legend to both his contemporaries and his descendants: an ideal of kingly power whose legacy of control and intimidation was remembered for centuries. Of particular note is the unique macrotia that the king's statues display. In this paper, we discuss possible etiologies of Senusret's unique presentation and ultimately conclude that Senusret's immortalized features were likely rooted in propaganda rather than a medical cause.

An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024.

ETV6::NTRK3-associated papillary adenocarcinoma: let us play it by ear.

Ceruminous glands are modified apocrine glands, situated in the external auditory canal (EAC) that, together with sebaceous glands, produce cerumen. The neoplastic transformation of these structures is exceedingly rare. We encounter two cases of EAC adenocarcinoma with ETV6::NTRK3 fusion. Despite this genetic overlap, the morphology and immunophenotype delineate its clear separation from secretory carcinoma. These cases demonstrate novel primary EAC adenocarcinoma with papillary morphology, which expands the ever-increasing list of ETV6::NTRK3-positive malignancies and which we would like to term ETV6::NTRK3-translocation associated papillary adenocarcinoma. We also advocate the use of molecular techniques in rare tumors of uncertain type or differentiation, to increase understanding and possibilities of reproducible classification of these rare neoplasms. Pathologists and oncologists should recognize this entity, which leads to a direct approach for detecting NTRK fusion for appropriate treatment.

Malignant myoepithelioma of the external auditory canal - a rare case report with literature review and clinical importance of foramen of Huschke.

BACKGROUND: A malignant myoepithelioma is a rare tumor, mostly arising from the salivary glands. Myoepitheliomas of the ear have rarely been reported. The manuscript reports myoepithelial carcinoma of the external auditory canal (EAC) spreading to the infratemporal fossa. A clinician must be aware of anatomical variation of the bony EAC wall, such as the foramen of Huschke. This rare defect may be a pathway for spreading pathologies between these two anatomical regions. CASE REPORT: We present a case of osteoma-like stenosis of the EAC, which turned out to be an extremely rare malignant tumor. The preoperative MRI and PET/CT revealed that two parts of the tumor communicated through a defect in the antero-inferior portion of the bony ear canal. No distant metastases were detected. Subsequently, the tumor was resected from the ear canal and the infratemporal fossa en bloc. Perioperatively the defect in the EAC wall was suspected of the foramen of Huschke. After the surgery, the older scans of the patient from the past showed no presence of a congenital EAC wall defect. Therefore, the authors concluded that the tumor aggressively grew through the bone due to its biological nature. CONCLUSION: Malignant myoepithelioma of the external auditory canal is an extremely rare condition and could be misdiagnosed as other benign lesions. In cases of suspicious lesions, it is advisable to do a probatory biopsy from the EAC. Surgery is the treatment of choice in malignant myoepitheliomas, and regular follow-ups are essential to monitor for recurrence or metastatic disease. Any mass located at the antero-inferior portion of the EAC wall warrants close evaluation due to its potential for expansion from the EAC.

Incidence of audiologic or otolaryngologic evaluation in patients with external ear anomalies.

PURPOSE: The relationship between specific external ear anomalies (EEA) and hearing loss has been previously described. However, there is no literature regarding the appropriate evaluation of patients with EEA by audiology or otolaryngology. The objective of this study was to determine the incidence of audiologic or otolaryngologic evaluation of patients with EEA. MATERIALS AND METHODS: A retrospective review of charts was conducted following approval from the institutional review board at Boston Medical Center. Charts of patients younger than 18 years old with EEA, identified using International Classification of Diseases (ICD)-9 codes 380-380.99, 744, and 744.4 and ICD-10 codes H61.90-92, Q16.0-16.9, Q17.0-17.9, Q18.0-18.2, from January 2012 to January 2019 were reviewed. Primary variables included incidence of audiologic and otolaryngologic evaluation, newborn hearing screen and audiometry results, and completion of surgical intervention. Binary logistic regressions were conducted for each group for diagnostic, procedural, and demographic characteristics. RESULTS: A total of 723 patients were diagnosed with EEA from January 2012 to January 2019. Of these patients, 327 (45.2 %) were evaluated by audiology and 327 (45.2 %) were evaluated by otolaryngology. Of the 364 patients who obtained audiograms, 63 (17.3 %) demonstrated hearing loss. Surgical procedures were performed on 119 (16.5 %) patients, with the most common procedure being excision of the EEA (n = 79, 66.4 %). A total of 468 patients had a documented newborn hearing screen. Failure of newborn hearing screen and presence of microtia were associated with increased otolaryngologic and audiologic evaluation. CONCLUSIONS: A majority of patients with EEAs do not obtain audiologic or otolaryngologic evaluation.

Head-related transfer functions of rabbits within the front horizontal plane.

The head-related transfer function (HRTF) describes the direction-dependent acoustic filtering by the head that occurs between a source signal in free-field space and the signal at the tympanic membrane. HRTFs contain information on sound source location via interaural differences of their magnitude or phase spectra and via the shapes of their magnitude spectra. The present study characterized HRTFs for source locations in the front horizontal plane for nine rabbits, which are a species commonly used in studies of the central auditory system. HRTF magnitude spectra shared several features across individuals, including a broad spectral peak at 2.6kHz that increased gain by 12 to 23dB depending on source azimuth; and a notch at 7.6kHz and peak at 9.8kHz visible for most azimuths. Overall, frequencies above 4kHz were amplified for sources ipsilateral to the ear and progressively attenuated for frontal and contralateral azimuths. The slope of the magnitude spectrum between 3 and 5kHz was found to be an unambiguous monaural cue for source azimuths ipsilateral to the ear. Average interaural level difference (ILD) between 5 and 16kHz varied monotonically with azimuth over ±31dB despite a relatively small head size. Interaural time differences (ITDs) at 0.5kHz and 1.5kHz also varied monotonically with azimuth over ±358 µs and ±260 µs, respectively. Remeasurement of HRTFs after pinna removal revealed that the large pinnae of rabbits were responsible for all spectral peaks and notches in magnitude spectra and were the main contribution to high-frequency ILDs (5-16kHz), whereas the rest of the head was the main contribution to ITDs and low-frequency ILDs (0.2-1.5kHz). Lastly, inter-individual differences in magnitude spectra were found to be small enough that deviations of individual HRTFs from an average HRTF were comparable in size to measurement error. Therefore, the average HRTF may be acceptable for use in neural or behavioral studies of rabbits implementing virtual acoustic space when measurement of individualized HRTFs is not possible.

Measurements of bone-conducted sound in the chinchilla external ear.

We measure bone-conduction (BC) induced skull velocity, sound pressure at the tympanic membrane (TM) and inner-ear compound-action potentials (CAP) before and after manipulating the ear canal, ossicles, and the jaw to investigate the generation of BC induced ear-canal sound pressures and their contribution to inner-ear BC response in the ears of chinchillas. These measurements suggest that in chinchilla: i.) Vibrations of the bony ear canal walls contribute significantly to BC-induced ear canal sound pressures, as occluding the ear canal at the bone-cartilaginous border causes a 10 dB increase in sound pressure at the TM (PTM) at frequencies below 2 kHz. ii.) The contributions to PTM of ossicular and TM motions when driven in reverse by BC-induced inner-ear sound pressures are small. iii.) The contribution of relative motions of the jaw and ear canal to PTM is small. iv.) Comparison of the effect of canal occlusion on PTM and CAP thresholds point out that BC-induced ear canal sound pressures contribute significantly to bone-conduction stimulation of the inner ear when the ear canal is occluded.

Surgical outcomes for cutaneous squamous cell carcinoma of the auricle.

Background: Cutaneous squamous cell carcinoma (CSCC) on the auricle is believed to carry a higher risk of metastatic spread. The rates of lymphatic metastasis reported in the literature have varied widely. There are no established prognostic criteria to determine which of these tumors are higher risk and warrant prophylactic treatment of the associated lymphatic basins. Objective: To retrospectively evaluate outcomes after surgical treatment of auricular CSCC in our department, examining excision completeness, tumor recurrence, and lymphatic metastasis. Secondarily, to identify factors associated with lymphatic metastasis. Methods: One hundred and thirty-eight consecutive cases of auricular SCC were excised from 126 patients in our department over a 7-year period (January 2012-December 2018). Data were retrospectively collected on patient characteristics, tumor histology, surgical procedures, and follow-up. Results: Incomplete initial excision occurred in 17 cases (12.32%). Six patients (4.76%) had a local recurrence. Lymphatic metastasis occurred in eight patients (6.35%), on average within 10.25 months after primary excision. Six patients with metastasis died during follow-up, with a mean survival of 10.2 months. Older age was associated with lymphatic metastasis (P = 0.0267). Other factors, including tumor recurrence, size, grade, cartilage invasion, and positive margins, were evaluated and not significantly associated with metastasis. Conclusion: In this study, the metastatic rate of auricular SCC was 6.35%, which is within the previously reported ranges. No histological prognostic factors were identified in this study, which may be due to our limited sample size. In the absence of established prognostic criteria, decisions regarding prophylactic treatment should be made on an individual basis with multidisciplinary support.

Glomangioma of the External Ear: A Case Report.

Glomangiomas of the external ear are exceptionally rare. These tumors are a type of glomus tumor, which are soft tissue neoplasms of mesenchymal origin that result from undifferentiated smooth muscle. In this report, we describe a case of an ear lobule glomangioma that was treated with surgical excision. Laryngoscope, 2023.

Noise-Induced Hearing Threshold Shift Correlated with Body Weight and External-Ear Amplification in Chinchilla: a Preliminary Analysis.

BACKGROUND: External-ear amplification (EEA) has been shown to vary from 5-19 dB-A in large datasets of pediatric, adolescent, and adult human participants. However, variable EEA is an overlooked characteristic that likely plays a role in individual noise-induced hearing loss (NIHL) susceptibility. A noise exposure varying 5-19 dB-A translates to high-EEA individuals theoretically experiencing 3-4 times greater NIHL risk than low-EEA individuals. OBJECTIVE: The purpose of this preliminary analysis was to test the hypothesis that higher EEA is correlated with increased noise-induced threshold shift susceptibility. DESIGN: Nine chinchillas were exposed to 4-kHz octave-band noise at 89 dB-SPL for 24 h. Auditory brainstem response thresholds were obtained pre-exposure, 24-h post-exposure, and 4-week post-exposure. Relationships between EEA and threshold shift were analyzed. RESULTS: Open-ear EEA ranged 11-19 dB-SPL, and occluded-ear EEA ranged 10-21 dB-SPL. Higher occluded-ear EEA was correlated with increased NIHL susceptibility (p = 0.04), as was lower body weight (p = 0.01). Male animals exhibited more threshold shift than female animals (p = 0.02), lower body weight than female animals (p = 0.02), and higher occluded-ear EEA (male mean = 18 dB; female mean = 15 dB). CONCLUSIONS: Taken together, increased threshold shift susceptibility was observed in the smallest animals, animals with the highest occluded-ear EEA, and in male animals (which tended to have higher occluded-ear EEA). Given the established relationship between smaller body size and higher occluded-ear EEA, these preliminary results suggest that body size (and occluded-ear EEA; a function of body size) could be a potential, underlying driver of NIHL susceptibility differences, rather than true sex differences.

Nodular fasciitis of the external auditory canal: Clinical case report and review of the literature.

Nodular fasciitis is a benign reactive soft tissue tumor arising from fibroblasts and myofibroblasts. Its incidence is low and misdiagnosis is frequent especially for malignant lesions. This can lead to inappropriate and unnecessary invasive treatment. Nodular fasciitis of the external auditory canal is extremely rare. So far, around fifteen cases have been reported. We present here the case of a 90-year-old patient with nodular fasciitis of the right external auditory canal. The lesion extends anteriorly for 6.5 cm and reaches the posterior wall of the maxillary sinus. To our knowledge, this is the first case in the literature of an external auditory canal nodular fasciitis presenting as an inflammatory ear polyp with such a wide extension.