Epithelioid Hemangioendothelioma of the Mastoid Bone with Extension of Middle Cranial Fossa: A Case Report.

Epithelioid hemangioendothelioma (EHE) is a rare sarcoma of vascular origin. It is frequently seen in the liver, lungs and bones. Cases with mastoid bone and intracranial extension are rarer. A 40 year old male, presented with a history of progressive headaches and a mass in the postauricular region for a duration of three months. Computer tomography of the temporal bone showed an approximately 40 × 30 mm soft tissue with osteolytic bony changes located in the mastoid bone. Epithelioid hemangioendothelioma is a rare sarcoma of vascular origin. Surgery is the first line of therapy. Prognosis is generally good, and may be better for primary intracranial disease than that for EHE originating elsewhere.

Anatomical and Clinical Evaluation of Tympanic Tegmen and Mastoid Bone with Multidetector Computed Tomography / Evaluación Anatómica y Clínica del Tegmen Timpánico y Hueso Mastoideo con Tomografía Computarizada Multidetector

SUMMARY: Tegmen level and mastoid bone thickness are important parameters of surgical risk in middle ear and mastoid region surgeries. This retrospective cohort study was conducted to provide a risk classification for the mastoid and middle ear regions. The study population comprised of 300 patients who underwent multidetector computed tomography (MDCT) for various indications. Patients with no pathology that disrupted the structure of the temporal region were included in the study. A risk classification was generated by analyzing the data obtained from mastoid and tympanic tegmen depths and the mastoid bone thickness by MDCT. The mastoid and tympanic tegmen were lower on the right side than on the left. In women, the right-sided mastoid bone thickness and mastoid tegmen were lower, and low-level tympanic and mastoid tegmen on the left and thin right mastoid bones were more common. According to the risk classifications for mastoid and middle ear region surgeries, women demonstrated a higher risk than men. In addition, as the thickness of the mastoid bone increased, the levels of the mastoid and tympanic tegmen increased. The present study provides a proper risk classification that may be helpful for preoperative risk assessment prior to middle ear and mastoid region surgery.

El nivel del tegmen y el grosor del hueso mastoideo son parámetros importantes del riesgo quirúrgico en las cirugías del oído medio y la región mastoidea. Este estudio de cohorte retrospectivo se llevó a cabo para proporcionar una clasificación del riesgo en las regiones mastoidea y del oído medio. La población de estudio estuvo compuesta por 300 pacientes que se sometieron a una tomografía computarizada multidetector (MDCT) por diversas indicaciones. Se incluyeron en el estudio pacientes sin patología que alterase la estructura de la región temporal. Se generó una clasificación de riesgo analizando los datos obtenidos de las profundidades del tegmen mastoideo y timpánico y el grosor del hueso mastoideo por TCMD. El tegmen mastoideo y timpánico estaban más bajos en el lado derecho que en el izquierdo. En las mujeres, el grosor del hueso mastoideo del lado derecho y el tegmen mastoideo eran más bajos, y eran más frecuente la presencia de tegmen timpánico y mastoideo de bajo nivel en los huesos mastoideos izquierdo y delgados en el lado derecho. Según las clasificaciones de riesgo de las cirugías de la región mastoidea y del oído medio, las mujeres presentaban un mayor riesgo que los hombres. Además, a medida que aumentaba el grosor del hueso mastoides, aumentaban los niveles del tegmen mastoideo y timpánico. El presente estudio proporciona una clasificación de adecuada de riesgo que puede ser útil para la evaluación preoperatoria del riesgo antes de la cirugía del oído medio y la región mastoidea.

Mastoid Growth and the Configuration of Cochlear Implant Electrode Lead.

OBJECTIVES: To study the changes in the coiled configuration of electrode excess lead in the mastoid cavity in the cochlear implant recipients over time. METHODS: Post-operative CT scans at two different appointments of fourteen patients with cochlear implants (CI) were retrospectively analyzed using a DICOM viewer software (3D-slicer). Mastoid thickness (MT) was measured in the oblique coronal plane from the round window (RW) entrance to the mastoid edge and inter-cochlear distance (ICD) was measured in the axial plane at the fundus level between two ears. 3D segmentation of the entire inner ear of both sides and coiled electrode excess lead was performed to visually compare the changes in coiled configuration between the two CT scan time points. RESULT: MT and ICD increased logarithmically with the patient's age, as has been measured from both the 1st and the 2nd CT scans and a weak linear correlation between MT and ICD was observed. Growth in MT and ICT measured between the time of 1st and 2nd CT scans showed a strong linear correlation. In eight cases, changes in the electrode excess lead have been observed in the 2nd CT scan, either a change in the coiling configuration of electrode excess lead or shifted laterally toward the mastoid edge. The ICD growth between the 1st and the 2nd CT scans was >2 mm in only seven cases and all of them were children. All other six cases had no observed changes in the coiled electrode lead. In addition, the mastoid growth between the 1st and the 2nd CT scan was >2.5 mm in only 4 cases. CONCLUSION: Coiled configuration of electrode excess lead could change when the MT and ICD increased over time.

Pseudomonas Aeruginosa Sepsis in a Previously Healthy Infant with Subcutaneous Nodules and Mastoid Bone Destruction.

Pseudomonas aeruginosa septicemia is rare in previously healthy children. Skin lesions such as subcutaneous nodules and ecthyma gangrenosum may be the first manifestation of Pseudomonas infection that have rarely been reported. Herein we reported a previously healthy 6-month-old boy patient who presented with suppurative otitis media, multiple nodules, septic shock, and P. aeruginosa was identified in cultures of the blood, skin lesions, and purulent material of his ears.

Invasive, aggressive mastoid bone eumycetoma: a treatment challenge.

Mycetoma is a chronic granulomatous disease that significant affects the subcutaneous tissue and deep structures. Mycetoma is caused by certain fungi (eumycetoma) or higher bacteria (actinomycetoma). The clinical presentation is variable and depends on the causative agent. For proper treatment and patient management, an accurate diagnosis of the species is mandatory. The disease mainly involves the extremities and it is rarely seen in the head and neck or other sites. In this communication, we present an interesting case of both invasive and aggressive mastoid bone eumycetoma caused by Madurella mycetomatis. Such cases are defied by complex challenges in finding effective surgical and medical treatments when the patient does not respond to both prolonged and different antifungal therapies.

Important landmarks and distances for posterior fossa surgery measured by temporal MDCT.

In this retrospective study, we aimed to present important anatomical structures and distances for posterior fossa surgery by temporal multidetector computed tomography (MDCT). The temporal MDCT images of 317 adult patients (158 males and 159 females) were retrieved from the hospital's picture archiving and communication system (PACS). In the coronal temporal MDCT views, the cochlea-carotid canal and jugular bulb-mastoid bone outer surface were measured. In the axial MDCT views, the carotid canal-jugular bulb and carotid canal-posterior fossa distances were measured; the carotid canal and jugular bulb anterior-posterior (AP) and transverse dimensions were also measured. The bilateral cochlea-carotid canal, jugular bulb-mastoid bone outer surface, and right carotid canal-jugular bulb distances were significantly greater in the males than those in the females (p < 0.05). The carotid canal-posterior fossa distance was not different in both genders (p > 0.05). The carotid canal-jugular bulb and the carotid canal-posterior fossa distances were greater on the left side than those on the right side in both genders (p < 0.05). In males, the outer surface distance was greater on the left jugular bulb-mastoid bone than that on the right side of that bone (p < 0.05). The difference between the carotid canal AP dimensions was not significant between males and females (p > 0.05). However, the carotid canal transverse dimension, jugular bulb AP, and transverse dimensions were significantly greater in the males than those in the females, bilaterally (p < 0.05). In each gender separately, the carotid canal AP and transverse dimensions were greater on the left side and the jugular bulb AP and transverse dimensions were greater on the right side than those on the left side (p < 0.05). Positive correlations were found between the cochlea-carotid canal, the jugular bulb-mastoid bone outer surface, and the carotid canal-jugular bulb distances as well as between the jugular bulb-mastoid bone outer surface and the carotid canal-posterior fossa distances (p < 0.05). In older patients, the carotid canal-posterior fossa distances were shorter on the left side (p < 0.05). Vascular and neural localizations should be well understood in the operative area before applying the surgical approach in the posterior fossa. Computed tomography (CT) has a greater role in the evaluation of bone structures and vascular canals in this area.

An activating germline IDH1 variant associated with a tumor entity characterized by unilateral and bilateral chondrosarcoma of the mastoid.

Chondrogenic tumors involving the temporal bone are rare and typically arise spontaneously with unilateral presentation. Somatic IDH mutations are common in these tumors, but germline inheritance has not been documented to our knowledge. We describe familial chondrosarcoma, grade 1, of the mastoid with unilateral presentation in the mother and bilateral presentation in each of her two children. Each individual presented with headaches, facial paresis, and conductive hearing loss between the ages of 9-12. Exome sequencing of all three affected family members identified a shared germline heterozygous c.299G>A (p.Arg100Gln) missense variant in IDH1. The p.Arg100Gln variant has only rarely been observed as a somatic mutation in glial tumors, and previous in vitro experiments have shown that p.Arg100Gln produces small amounts of the oncometabolite D-2-hydroxyglutarate (D2HG). Biochemical testing in all three affected family members on urine and plasma was unable to detect increases in D2HG in these sample types. Due to insufficient tumor for methylation studies, we performed genome-wide methylation analysis of an IDH1 p.Arg100Gln mutant brain tumor from an unrelated individual to functionally evaluate this variant. These studies demonstrated a global hypermethylation phenotype consistent with other known isocitrate dehydrogenase (IDH) mutant brain tumors, suggesting that this variant has neomorphic activity despite low-level production of D2HG. The bones of the facial skeleton are formed by membranous ossification and we hypothesize that abnormal embryonic cartilage that rests within the suture lines may be involved in this tumor entity. Testing of additional individuals with similar presentations is needed to confirm this finding and clarify the associated phenotypes.

Osteoma of Mastoid Bone; A Rare Presentation: Case Report.

Mastoid osteomas are benign, slow growing tumours arising in temporal bone. These are rare; few cases reported till date. Normally are asymptomatic, may present with cosmetic deformity, pain and hearing loss. These are readily excised. Recurrence is rare.

Differential Diagnosis of Post Auricular Swelling with Mastoid Bone Involvement.

Post auricular swelling with mastoid bone involvement most commonly occurs as a cranial complication of chronic or acute suppurative otitis media. Aim of this study, to detect etiology of postauricular swelling with mastoid bone involvement. This is a retrospective study which was done on all patients whom underwent mastoidectomy for post auricular swelling with mastoid bone involved in El Minia University Hospital from January 2014 to June 2017. A total of 49 patients were identified in this study, 23 males and 26 females, their ages ranged 5-28 years old with a mean of 25.8 years old. Group A: these were 45 patients with a history of otorrhea (chronic suppurative otitis media), of 3 months to 4 years duration. Group B: these were four patients without any history of otorrhea. Two patients diagnosed as acute otitis media, one patient diagnosed as Langerhans cell histiocytosis and one patient diagnosed as fungal mastoiditis. Chronic suppurative otitis media is the most common causes of postauricular swelling. Langerhans cell histiocytosis of the temporal bone and fungal mastoiditis are considered a rare presentation of postauricular swelling.

Primary epidermoid cysts of the mastoid: clinical and treatment implications.

Epidermoid cysts of the temporal bone are extremely rare and such lesions arising in isolation within the mastoid bone have never been reported in literature. We report and describe the first two unique cases of primary epidermoid cysts arising in the mastoid bone. Of the two cases, one presented with progressive headache and imbalance and the other with unilateral hearing loss and tinnitus. Both cases needed CT and MRI scans and needed surgical management. We review the clinical presentations, histology, pathogenesis, radiological findings and management of these challenging cases. The diagnosis of an epidermoid cyst is based on clinical presentation, physical examination and especially the radiological, histological and intraoperative findings. Total removal of the lesion along with its capsule is recommended to prevent recurrence and to allow for a good long-term prognosis.

Development of a low grade lymphoma in the mastoid bone in a patient with atypical Cogan's syndrome: A case report.

Cogan's syndrome is a rare disorder characterized by ocular and audiovestibular manifestations in its typical form and caries a wide variety of atypical manifestations. It is considered as an autoimmune disease. We present the first case in the literature of a 67 year old woman with the development of low grade non-Hodgkin lymphoma (NHL) in the mastoid bone in a pre-existing history of atypical Cogan's syndrome. The anatomical development of NHL was to a "target" organ of Cogan's syndrome, which is the inner ear.

Intramastoid solitary neurofibroma mimicking middle ear carcinoma.

We report an extremely rare case of intramastoid neurofibroma. A mass with destruction of the mastoid bone of a 51-year-old woman was examined with computed tomography. Subsequent magnetic resonance imaging demonstrated an ill-defined soft tissue mass with the opacification of mastoid air cells that had a mass effect in the same area. The patient underwent left subtotal temporal bone resection, and histological and immunohistochemical findings confirmed the lesion to be a neurofibroma. Given that similar imaging features of neurofibroma have been reported previously elsewhere in the head/neck and extremities, we suggest that it may be possible to include this tumor in the preoperative differential diagnosis.

Trautmann's triangle anatomy with application to posterior transpetrosal and other related skull base procedures.

Trautmann's triangle (TT) faces the cerebellopontine angle and is exposed during posterior transpetrosal approaches. However, reports on the morphometric analysis of this structure are lacking in the literature. The goal was to better understand this important operative corridor. TT was exposed from an external approach (transmastoid) in ten cadavers (20 sides) and from an internal approach on 20 dry adult temporal bones. Measurements included calculation of the area of TT and the distance of the endolymphatic sac from the anterior border of the sigmoid sinus. The area range of TT was 45-210 mm(2) (mean 151 mm(2); SD 37 mm(2)). Three types of triangles were identified based on area. Type I triangles had areas less than 75 mm(2), Type II areas were 75-149 mm(2), and Type III areas were 150 mm(2) and greater. These types were observed in 37.5%, 35%, and 27.5% of sides, respectively. The distance from the jugular bulb's anterior border to the posterior border of the posterior semicircular canal ranged from 6 to 11 mm (mean 8.5 mm). The endolymphatic sac was located in the inferior portion of TT and traveled anterior to the sigmoid sinus. The horizontal distance from the anterior edge of the sigmoid sinus to the posterior edge of the endolymphatic sac ranged from 0 to 13.5 mm (mean 9 mm). Additional anatomic knowledge regarding TT may improve neurosurgical procedures in this region by avoiding intrusion into the endolymphatic sac and sigmoid sinus.

Mastoid cavity obliteration and Vibrant Soundbridge implantation for patients with mixed hearing loss.

OBJECTIVES/HYPOTHESIS: To review the results of obliteration of a preexisting mastoid cavity with abdominal fat and Vibrant Soundbridge implantation in patients with mixed hearing loss (MHL) and to compare the data with results of Vibrant Soundbridge implantation in patients with MHL without mastoid cavity and with pure sensorineural hearing loss (SNHL). STUDY DESIGN: Retrospective chart analysis of 10 patients (10 ears) with MHL and preexisting mastoid cavity, 18 patients (19 ears) with MHL alone and nine patients (10 ears) with SNHL treated in one tertiary referral center. METHODS: Vibrant Soundbridge implantation and obliteration in case a mastoid cavity existed previously. Pure tone audiometry (average air-bone gap, average functional gain), speech audiometry (Freiburg Monosyllabic Test) and complication rate were main outcome measures. RESULTS: Postoperative average air-bone gap was -15.1 ± 21.2 dB in patients with MHL with mastoid cavity obliteration, -7.2 ± 11.4 dB in patients with MHL without mastoid cavity, and -5.7 ± 11.2 dB in patients with SNHL. Average functional gain was 40.0 ± 23.5 dB, 39.7 ± 12.1 dB, and 9.5 ± 10.6 dB. Postoperative speech discrimination rate was 77.9 ± 20.8%, 83.3 ± 13.6%, and 83.6 ± 6.3%. No severe intraoperative or postoperative complications were noted. CONCLUSIONS: Mastoid cavity obliteration during Vibrant Soundbridge implantation in patients with MHL and preexisting mastoid cavity is a safe procedure. The audiometric results are satisfying and comparable to those of other patient groups implanted with the same device. LEVEL OF EVIDENCE: 4.

A Case of Large Intradiploic Epidermoid Cyst of the Temporal Bone / 대한이비인후과학회지

Intradiploic epidermoid cysts of the skull are benign lesions that are derived from the ectodermal cells of the cranium. They are rare tumors and they represent less than 1% of all intracranial tumors. The frontal and parietal bones are the most common sites for the cysts, but the temporal bone is rarely involved. They are slowly growing tumors, and may often reach an enormous size without producing neurological symptoms. For treatment, it is important to completely remove the capsule of the cysts to avoid recurrence. Malignant transformations of the cysts, which are very rare, are generally found in cases which have repeated recurrences or infections. We present a patient with an intradiploic epidermoid cyst of the temporal bone, which is located behind the left mastoid cavity. We removed the cyst successfully through transmastoid approach.

The recovering of the radical surgery of mastoid bone by the temporal facio-musscle

95% cases of otitis mastoid were sucessfully treated by the reservation and surgery and the rest 5% cases with this were treated by many different methods aiming to management of the hollows of the radical surgery of mastoid bone according to the cause or individuals. The recovering of the partly poor epithelization in the surgical hollow reported effectively. 13 patients received a surgery in the Central Military Hospital 108 during 1987-1996 were studied. The indicators of age, gender, symptoms before receiving operation and status of the surgical hollow were used to classify the result