Temporal Bone Lesions Research Articles

A rare clinical case of a labyrinthine lesion: an endolymphatic sac tumor

Endolymphatic sac tumors are rare neoplasms originating from the endolymphatic epithelium of the intraosseous part of the endolymphatic duct and sac, typically characterized by slow growth and local destructive changes. We present a case of an endolymphatic sac tumor in a 28-year-old man who consulted an otoneurologist with complaints of progressive hearing loss and dizziness attacks and was referred for a CT scan of the temporal bones and MRI of the inner ear with delayed contrast enhancement. The data of otoneurological and radiological examinations before and after surgical treatment and the results of surgical treatment with histological verification of the tumor are presented. The objective of this case presentation is to demonstrate the usefulness of CT and MRI methods in detecting an endolymphatic sac tumor, to compare the results of otoneurological and radiological examinations and intraoperative and histological data followed by surgery. Radiological examination revealed a neoplasm of the right temporal bone with typical imaging characteristics of endolymphatic sac tumor. Surgery was performed, the mass was removed completely, and the endolymphatic sac tumor was histologically verified. Radiologic presentation includes a typically located area of bone destruction in the petrous part of the temporal bone in the area of the vestibular aqueduct, possibly with the spread of the tumor into the cerebellopontine angle, with a heterogeneous structure, with uneven contrast enhancement. Due to the rarity of this pathology, diagnosis in the early stages is often difficult, and for the correct interpretation of radiologic findings in patients with hearing loss and dizziness, knowledge of the normal anatomy of the temporal bone and awareness of rare variants of its lesions are of key importance. Timely examination of patients with suspected temporal bone lesions and correct diagnosis are the key to successful treatment and maintaining the quality of life in this group of patients.

Deep learning reconstruction improves the image quality of low-dose temporal bone CT with otitis media and mastoiditis patients

ObjectiveTo evaluate the image quality of low-dose temporal bone computed tomography (CT) in otitis media and mastoiditis patients by using deep learning reconstruction (DLR). Materials and methodsA total of ninety-seven temporal bones from 53 consecutive adult patients who had suspected otitis media and mastoiditis and underwent temporal bone CT were prospectively enrolled. All patients underwent high resolution CT protocol (group A) and an additional low-dose protocol (group B). In group A, high resolution data were reconstructed by filter back projection (FBP). In group B, low-dose data were reconstructed by DLR mild (B1), DLR standard (B2) and DLR strong (B3). The objective image quality was analyzed by measuring the CT value and image noise on the transverse image and calculating the signal-to-noise ratio (SNR) on incudomallear joint, retroauricular muscle, vestibule and subcutaneous fat. Subjective image quality was analyzed by using a five-point scale to evaluate nine anatomical structures of middle and inner ear. The number of temporal bone lesions which involved in five structures of middle ear were assessed in group A, B1, B2 and B3 images. ResultsThere were no significant differences in the CT values of the four reconstruction methods at four structures (all p > 0.05). The DLR group B1, B2 and B3 had significantly less image noise and a significantly higher SNR than group A at four structures (all p < 0.001). The group B1 had comparable subjective image quality as group A in nine structures (all p > 0.05), however, the group B3 had lower subjective image quality than group A in modiolus, spiral osseous lamina and stapes (all p < 0.001), the group B2 had lower subjective image quality than group A in modiolus and spiral osseous lamina (both p < 0.05). The number of temporal bone lesions which involved in five structures for group A, B1 and B2 images were no significant difference (all p > 0.05), however, the number of temporal bone lesions which involved in mastoid for group B3 images were significantly more than group A (p < 0.05). The radiation dose of high resolution CT protocol and low-dose protocol were 0.55 mSv and 0.11 mSv, respectively. ConclusionCompared with high resolution CT protocol, in the low-dose protocol of temporal bone CT, DLR mild and standard could improve the objective image quality, maintain good subjective image quality and satisfy clinical diagnosis of otitis media and mastoiditis patients.

Imaging of Temporal Bone Mass Lesions: A Pictorial Review.

Tumoral lesions of the temporal bone include benign or malignant tumors and congenital or inflammatory lesions. Temporal bone lesions are difficult to approach. Therefore, making a preoperative diagnosis and considering whether the lesions require treatment are necessary; if they require treatment, then the type of treatment requires consideration. These tumors cannot be observed directly and must be diagnosed based on symptoms and imaging findings. However, the differentiation of temporal bone lesions is difficult because they are rare and large in variety. In this pictorial review, we divided temporal bone lesions by location such as along the facial nerve, along the internal jugular vein, around the endolymphatic sac, in the internal auditory canal/cerebellopontine angle, petrous apex, middle ear, and mastoid, focusing on the imaging findings of temporal bone lesions. Then, we created a diagnostic flowchart that suggested that the systematic separation of imaging findings is useful for differentiation. Although it is necessary to make comprehensive judgments based on the clinical symptoms, patient background, and imaging findings to diagnose temporal bone mass lesions, capturing imaging features can be a useful differentiation method.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Menière's Syndrome Associated With Fibrous Dysplasia of the Temporal Bone.

Massive jugular foramen schwanoma revealed by the hypogloss paralysis

Jugular foramen schwannomas are a rare event with an estimated 200 cases reported in the literature. These tumors represent only 2.9% to 4% of all intracranial schwannomas and less than 1% of all temporal bone lesions [1,2].

Langerhans Cell Histiocytosis of the Head and Neck: Experience at a Rural Tertiary Referral Center.

Retrospectively analyze head and neck Langerhans Cell Histiocytosis at a rural tertiary referral center and compare results with previously published data. Electronic health record review was performed from 2003 to 2019. Patients with biopsy proven LCH with primary head and neck involvement were included. Demographics, presentation, imaging characteristics, treatment modality, delay in diagnosis (DD, ≥60 days), and outcomes were analyzed and reported. Twenty-four patients were included. The most common presenting symptoms were otorrhea (n = 6) and scalp pain or swelling (n = 6). All patients had bony involvement. The most common site was facial or skull lesions (n = 20). Most skull lesions (75%) demonstrated CNS risk. Six patients were treated with primary surgery, 15 with primary chemotherapy, and 3 with surgery plus adjuvant chemotherapy. Nine patients experienced relapse of disease with median time to documented relapse of 11.4 months; all were treated with salvage chemotherapy to achieve complete remission (median follow-up: 72 months). Patients most likely to relapse were those with multisystem disease (5/7, 71.4%), temporal bone lesions (4/7, 57.1%), and DD (7/12, 58.3%). Of the 9 total patients who experienced relapse, 78% had a delay in diagnosis. LCH is a complex disease process in which diagnosis can be delayed if not considered in the differential. Within the head and neck, the skull, including isolated temporal bone involvement, is the most common site of involvement. Treatment modality does not appear to have an influence on relapse rates. Relapse was more likely to occur in the first year after treatment and close monitoring is required.

Temporal Bone Rhabdomyosarcoma Mimicking Chronic Suppurative Otitis Media

Rhabdomyosarcoma (RMS) is one of the most commonly encountered soft-tissue sarcomas in childhood. The most common location is in the head-and-neck region, followed by the urogenital system, extremities, and torso. Head and neck is a common site though the temporal bone is an extremely rare site with only a few reported cases in the literature till now. Herein, we report a case of a sclerosing type of temporal bone RMS in an 8-year-old young girl. We discuss clinical, pathological, radiological findings and management of temporal bone RMS. Misdiagnosis is common because of this aggressive temporal bone lesion's similarity to more common benign diseases. Clinicians should maintain suspicion for RMS in patients with chronic otitis media not responsive to medical therapy and should refer a specialist multidisciplinary team in a timely manner as an early diagnosis will optimize the chance of survival. Clinical features, radiologic findings, and histopathological examination using immunohistochemistry are all important for the correct diagnosis of temporal bone RMS. It is important to be aware of and to know how to deal with such rare cases.

Surgical management of 27 cases with temporal bone cerebrospinal fluid leakage

Objective:To study the clinical manifestations and surgical treatment of temporal bone cerebrospinal fluid leakage. Methods:The clinical data of twenty seven cases with temporal bone cerebrospinal fluid leakage were analyzed retrospectively. Different surgical procedures were adopted according to the location of the leak and the hearing status of the affected ear.If the leakage location was clear before surgery, direct repair was performed via mastoid path or middle cranial fossa path with or without mastoid abdominal fat packing.For patients with unclear leak, large lesion or intractable CEREBROspinal fluid leakage of temporal bone, subtotal petrosal resection and abdominal fat packing were performed.Subtotal temporal bone resection and abdominal fat packing were performed for patients with cochlear involvement. Results:In this study, cases of temporal bone CSF leakage including 13 cases of inner ear malformation; 5 cases secondary to head trauma or previous surgery,5 cases of idiopathic intracranial hypertension or meningocele, 2 cases of langerhans histiocytosis in the temporal bone and 2 cases of inner ear inflammation. High resolution CT （HRCT） of temporal bone showed bone defect of inner auditory canal with vestibular communication in 13 patients with inner ear malformation.The temporal bone HRCT of the remaining 14 patients showed bone defects in the middle cranial fossa or posterior cranial fossa, while MRI of the temporal bone showed meningeal continuity interruption with cerebrospinal fluid inflow into the temporal bone or meningoencephalocele in 12 patients. The 26 patients were followed up from 6 months to 6 years. 1 patient lost follow-up.Meningitis recurred in only 1 patient with inner ear malformation, and subtotal resection of rock bone plus abdominal fat packing was performed.Postoperative hearing was preserved or improved in 7 patients.None of the patients had serious complications, and only 1 patient developed HB Grade Ⅱ facial paralysis after vestibular obliteration, and the facial paralysis recovered within one week. Conclusion:Temporal bone cerebrospinal fluid leakage is relatively rare. Surgical intervention should be taken as early as possible when conservative treatment is failure. Preoperative HRCT and MRI examination are necessary for the localization of the leakage, and individualized surgical approaches can be adopted according to the location of the leakage and the features of the lesion.

Protuberant fibro-osseous lesion of the temporal bone: report of four cases and review of the literature

‘Bullough lesions’, also referred to as protuberant fibro-osseous lesions (PFOL), are rare temporal bone lesions initially described in 1999. Since only 12 cases have been reported, several key issues, such as their origin and recommended management strategies, remain unresolved. This article reports the largest cohort included in the literature to date, comprising four patients with PFOL. PFOL appears to be characterized by female and right-side predominance. These lesions were consistently located regarding the mastoid, generally diagnosed in early adulthood, without functional symptoms, and were always fibro-osseous. Invasive/malignant features were not found on imaging or histology. The main differential diagnosis was malignant low-grade parosteal osteosarcoma. Clinical examination and computed tomography images provided strong elements supporting the diagnosis of PFOL. Biopsy allowed molecular biology investigations (MDM2 and CDK4 amplification), in order to rule out low-grade parosteal osteosarcoma.

Symptoms: Clogged Ears and Muffled Hearing

Symptoms: Clogged Ears and Muffled Hearing

Haemangioma of the temporal bone

IntroductionHaemangiomas of the temporal bone are rare tumours and haemangiomas involving the middle ear are even rarer. The exceptional nature of these lesions makes their management particularly complicated. Case reportThe authors report the case of a 16-year-old girl, who presented with an osteolytic lesion of the left petrous temporal bone that proved to be a haemangioma with extension to the middle ear, causing conductive hearing loss. DiscussionSurgical biopsy is essential to establish the diagnosis of haemangioma because imaging alone only rarely provides a definitive diagnosis. Surgery is the reference treatment to prevent recurrence. Arteriography is an essential part of the preoperative assessment in order to limit the risk of bleeding.

Symptoms: Dizziness and Sudden Hearing Loss

Symptoms: Dizziness and Sudden Hearing Loss

A Hospital based study of High Resolution Computed Tomography and Conventional Radiography for Evaluation of Pathologies of Temporal Bone

Background: Many imaging modalities have been in use for the evaluation of temporal bone lesions. Plain X-rays are cost-effective however; they provide limited details and hence sometimes may result in an inaccurate diagnosis. With the advent of HRCT, the method of imaging of temporal bone has evolved with special algorithms and multiplanar formats. In the present study, we tried to evaluate the different pathologies of the temporal bone with X-ray and HRCT. Methods: The study was conducted in the Departments of Otorhinolaryngology and Radiology, Prathima Institute of Medical Sciences, Naganoor, Karimnagar. A total of n=40 patients were identified and selected based on their symptoms and clinical findings suggestive of a lesion involving the temporal bone such as Otalgia, Otorrhoea, And Sensorineuronal deafness, pulsatile tinnitus, vertigo, and giddiness. These patients were subjected to high resolution computed tomography of the temporal bone on the 128 Slice CT Scanner (PHILIPS INGENUITY). The patients also underwent plain Radiography of temporal bone (CARESTREAM DRX-1 System). Results: Of the total n=40 patients studied the most common temporal bone disease was due to inflammation 85% and tumors were found in 15% of the patients. The common site of involvement of middle ear and mastoid air cells in chronic otitis media was epitympanum in n=30(90%) of cases, Mesotympanum in n=20(60%) of cases. In diseased ears, radiographs of the mastoids revealed pneumatised mastoid in 6(15%), diploic in 6(15%), and sclerosed mastoid in 28(70%) cases. HRCT temporal bone revealed pneumatised mastoid in 9(22.5%), diploic in 5(12.5%) and sclerosed mastoid in 26(65%) cases. Conclusion: The role of plain radiography is found to be limited to know the type of mastoid pneumatisation. It can also as Original Article detect bony erosion in few cases. Because of the ability to see temporal bone structures with great clarity, HRCT can be recommended not only in cases suspected with potential complications but also in all cases of temporal bone pathologies to know the extent of disease, inter-relationships of the tympanomastoid compartment with adjacent neurovascular structures, varied pneumatisation and the presence of anatomical variations, which should alert the clinician and guide in surgical approach and treatment plan.

Supratentorial and cervical extensions of a giant middle ear cholesteatoma

Introduction Cholesteatomas are slowly progressive and well demarcated lesions but if not treated, they can spread to the inner ear and brain and lead to serious neurological complications. Case report A 59-year old male was referred to our practice. His initial imaging showed that a huge intracranial mass has spread to the neck. Beyond surgical exploration, a sizable temporal bone lesion with extensive keratinisation was observed which later on histopathological exam turned out to be a cholesteatoma. Conclusions Cholesteatomas may be aggressive lesions by rapidly growing and gross destructive features. In such cases, more attempts should be given to ruling out any malignant neoplastic tissues.

A Case of Extramedullary Relapse of APL on the Tympanic Membrane

Temporal bone lesions are rarely seen in patients with acute promyelocytic leukemia (APL) during the remission phase. We report a case of APL who showed extramedullary relapse on the tympanic membrane while in the complete remission phase. A 48-year-old male patient with APL in complete remission after the initial therapy presented with left ear fullness. Endoscopic examination revealed a mass on the left tympanic membrane, and temporal bone CT showed a mass localized on the left tympanic membrane. He was diagnosed as having extramedullary relapse of APL based on the histopathological findings of the lesion on the tympanic membrane. There were no APL cells in the blood or bone marrow. However, PCR analysis revealed the presence of PML-RARα in the bone marrow. Based on these findings, we diagnosed the patient as having molecular recurrence of APL, and administered chemotherapy. During the treatment, the ear lesion decreased steadily in size, and finally disappeared. Absence of PML-RARα in the bone marrow specimen after the treatment indicated that the patient had entered the second remission phase. Subsequently, hematopoietic stem-cell transplantation was performed, and the patient remains in remission. In a patient with a history of leukemia presenting with temporal bone lesions, extramedullary relapse should be considered in the differential diagnosis even if the patient is in the remission phase.

Detectability of minute temporal bone structures with ultra-high resolution CT

ObjectiveComputed tomography (CT) is the imaging tool of choice in the diagnosis of temporal bone lesions. With the recent progress in imaging technology, CT with higher spatial resolution (Ultra-high resolution CT) has become available in the clinical setting. The purpose of this study is to evaluate the visibility of small temporal bone structures using ultra-high resolution CT. Material and methodsThe visibility of 27 minute temporal bone structures on ultra-high resolution CT images was evaluated. Non-helical axial scans were performed in 18 normal hearing ears without previous otologic diseases. Visibility was scored by an experienced radiologist and otologist. ResultsMinute temporal bone structures including the ossicular chain, the crus of the stapes, the greater superficial petrosal nerve, and the anterior malleolar ligament were clearly visualized on ultra-high resolution CT. The stapedius muscle tendon and the chorda tympani exiting the posterior canaliculus and coursing medial to the malleus could be visualized. ConclusionUltra-high resolution CT provides good visualization of small temporal bone structures in normal subjects.

Facial nerve paralysis as first sign of metastatic breast cancer to the temporal bone

Introduction. Facial nerve paralysis originates from various factors, although in most cases etiology is idiopathic. Temporal bone metastases are quite rare, but should still be suspected in cases when congenital disorders, inflammatory disease, infection or trauma are excluded as cause of facial palsy. We present an unusual case of facial nerve paralysis as the initial sign of temporal bone metastasis of breast carcinoma and discuss diagnostic pitfalls. Case outline. A 70-year-old patient presented with facial nerve palsy, severe otalgia, hearing loss and vertigo. Patient underwent steroid treatment 6 months earlier due to peripheral facial palsy with complete neurological resolution. CT scan revealed osteolytic lesion of the right temporal bone with extension into the parietal bone and soft-tissue. Additional examination confirmed ductal breast carcinoma and osteolysis of the ribs and vertebrae. After four months, the patient with metastatic breast carcinoma to the temporal bone died despite chemotherapy. Conclusion. Temporal bone metastasis of breast cancer is very rare condition with poor prognosis. Late diagnosis and inadequate management of breast cancer are factors that contribute to the temporal bone metastasis formation. Temporal bone metastasis should be excluded in elderly patients, both with and without any history of malignancy, especially in cases of peripheral facial palsy refractory to treatment.

Pediatric Langerhans cell histiocytosis of the lateral skull base

ObjectiveDescribe the presentation, imaging characteristics, management, and outcomes of pediatric patients with Langerhans cell histiocytosis (LCH) of the temporal bone. MethodsA retrospective chart review was performed between 2000 and 2014 at a single tertiary care children's hospital. Fourteen patients were identified with a diagnosis of LCH and involvement of the temporal bone. ResultsTen patients were female and ten were Caucasian. Mean age at diagnosis was 3 years (range 0.3–9.6 years). The most common presenting symptoms were scalp lesions, postauricular lesions, otalgia, and persistent ear infections. Three patients had documented hearing loss. Four cases had otic capsule invasion. Computed tomography demonstrated a lytic temporal bone lesion within the following subsites: mastoid, squamous temporal bone, external auditory canal, middle ear, and petrous apex. Four patients had intracranial disease. Extent and location of disease prompted all patients to be initially managed with chemotherapy. Surgical excision was limited to one case of localized recurrence. After a mean follow-up of 85.2 months (SD 42.4 months) there were no deaths but eight patients had a recurrence. Two patients developed long-term otologic sequelae requiring surgery. One patient developed labyrinthitis ossificans. ConclusionLCH has a varied presentation, age distribution, and treatment algorithm. This series of 14 pediatric patients with temporal bone involvement supports a limited role for surgical resection. Long-term follow-up is critical for detection of local and disseminated disease, and for monitoring of otologic complications.

6 cases of temporal bone carcinoid clinical analysis

Objective: This study is aimed at reporting 6 cases of temporal bone carcinoid and analyzing clinical and prognostic features of temporal bone carcinoid.Method: Clinical data from 6 cases of temporal bone carcinoid treated from July 2008 to July 2015 at the Chinese PLA General Hospital were reviewed. Based on temporal bone lesions row subtotal temporal bone resection or temporal bone extensive excision.Result: Six patients imaging data shows a different range of temporal bone lesions. Pathological both temporal bone carcinoid. The final diagnosis is unilateral primary temporal bone carcinoid. All of them received surgical partial resection of the temporal bone in our hospital,and there were no serious complications.Follow-up of 5 cases had no recurrence,and 1 case died spread to the liver.Conclusion:The diagnosis of temporal bone carcinoid relies mainly on pathology and immunohistochemistry. Temporal bone carcinoid could be treated by surgical.

Pediatric temporal bone rhabdomyosarcoma.

Rhabdomyosarcoma is one of the most common soft-tissue sarcomas in children. Prompt diagnosis and treatment significantly improve survival; however, misdiagnosis is common because of this aggressive temporal bone lesion's similarity to more common benign diseases. Clinicians should maintain a high index of suspicion for rhabdomyosarcoma in patients with a presumed otologic infection not responsive to medical therapy.