Objective:To analyze the diagnosis, treatment and prognosis of patients with rare malignant tumors of the temporal bone. Methods:Four cases of rare temporal bone malignant tumors in our hospital between March 2014 and December 2020 were reviewed, including two cases of chondrosarcoma, one case of fibrosarcoma and one case of endolymphatic cystic papillary adenocarcinoma. There were three males and one female, ages between 28 and 56 years at the time of surgery. Common symptoms included hearing loss, facioplegia, tinnitus, and headache. All patients underwent imaging examinations to evaluate the extent of the lesions. Tumors were removed by subtotal temporal bone resection or infratemporal fossa approach, and postoperative adjuvant radiotherapy was applied if necessary. Results:One of the two chondrosarcoma patients was cured by complete resection of the tumor for 75 months, the other one recurred after the first excision of the tumor and underwent infratemporal fossa approach resection of skull base mass again with no recurrence found yet for 112 months. One patient with fibrosarcoma survived for 28 months after surgery with a positive margin and post-operative radiotherapy. One patient with endolymphatic cystic papillary adenocarcinoma recurred 12 months after subtotal lithotomy, and underwent subtotal temporal bone resection again, combined with radiotherapy. No recurrence was found for 63 months. Conclusion:The incidence of rare temporal bone malignant tumors is extremely low, the location is hidden, and the symptoms are atypical. Attention should be paid for early detection and early treatment. Surgical resection is the main treatment, and radiotherapy can be supplemented in the advanced stage or with a positive margin.
Adult ; Female ; Humans ; Male ; Middle Aged ; Chondrosarcoma/surgery* ; Fibrosarcoma ; Neoplasm Recurrence, Local ; Retrospective Studies ; Skull Base/surgery* ; Skull Base Neoplasms/surgery* ; Temporal Bone/pathology* ; Treatment Outcome

A cholesterol granuloma is the most common primary lesion of the petrous apex, and accounts for 40% of the pathologies that arise in this region. The primary treatment for symptomatic lesions is by surgery to decompress and drain or to completely remove the lesion. Here we describe the use of infralabyrinthine approach to access a lesion through the temporal bone and completely remove it with the assistance of a 0-degree endoscope. A 43-year-old man visited our clinic for diplopia. Magnetic resonance imaging revealed a 2.3-cm cholesterol granuloma located in the left petrous apex that caused deviation of the left abducens nerve. The tumor was completely removed using the endoscopic-assisted infralabyrinthine approach; the patient is currently being followed up, and there is no evidence of disease recurrence. This case report describes the successful completion of a petrous apex cholesterol granuloma that preserved the cochlear and vestibular systems.
Abducens Nerve ; Adult ; Cholesterol* ; Diplopia ; Endoscopes ; Granuloma* ; Humans ; Magnetic Resonance Imaging ; Pathology ; Recurrence ; Temporal Bone

There are a number of reports on the pathologies of vestibular disorders. However, these studies included only a few examples, which were not quantitative but merely anecdotal or descriptive. However, a single tissue section may be relevant to a specific disease in multiple ways. The histopathological characteristics of common peripheral vestibulopathies, including benign paroxysmal positional vertigo, Ménière's syndrome, labyrinthitis, vestibular neuritis, and ototoxicity, have been described. A recent study validated a new quantitative method for determining vestibular otopathology. Detailed quantitative analyses of vestibular pathology are required to obtain a deeper understanding of the vestibular system. Such studies will likely reveal the pathophysiological causes of specific diseases by elucidating the correlations between structural and functional features. Therefore, histopathological studies of vestibular disorders should be performed.
Benign Paroxysmal Positional Vertigo ; Ear, Inner ; Labyrinthitis ; Methods ; Pathology ; Temporal Bone ; Vestibular Neuronitis

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.
Aged ; Diagnosis, Differential* ; Female ; Gadolinium ; Glomus Jugulare Tumor ; Hearing Loss ; Humans ; Meningioma* ; Neurilemmoma ; Otolaryngology ; Paraganglioma ; Pathology ; Tail ; Temporal Bone ; Tinnitus

OBJECTIVE: To explore. HRCT and MRI three-dimensional fast imaging employing steady state ac-quisition(3D-FIESTA) imaging features and clinical characteristics of bilateral large vestibular aqueduct syndrome(LVAS). METHOD: The imaging and clinical features of 14 cases of bilateral LVAS identified over a 5-year periodwere retrospectively analyzed. All patients underwent HRCT and MRI 3D-FIESTA scanning of head and neck;MRI three dimensional reconstructions of internal acoustical meatus were also completed at the same time. RESULT: Audiogram showed mild to moderate hearing loss and was progressive. The cut-off values for the coronal midpointand operculum planes on the HRCT scan to diagnose an EVA were 1. 5 mm and 4. 3 mm respectively; the averagevalue was 2. 4 mm. VA expansion degree were not linked to the degree of hearing loss. MRI showed VA andlymph sac abnormalities. Concomitant image finding was cochlear hypoplasia. CONCLUSION HRCT and MRI 3D-FI-ESTA are important examinations for accurate diagnosis of LVAS. HRCT can acquire the specific size of reamedVA. MRI and 3D reconstructions of internal acoustical meatus can noninasive show more intuitive display ofLVAS and other inner ear malformations than HRCT.
Ear, Inner ; Head ; Hearing Loss ; Hearing Tests ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging ; Retrospective Studies ; Temporal Bone ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; pathology ; Vestibular Diseases ; diagnosis

Cholesterol granulomas are inflammatory deposits commonly found in the mastoid antrum and air cells of temporal bone. They rarely occur in the nose. Here, we report an extremely rare case of cholesterol granuloma in the nasal septum, and include a short literature review. The clinical characteristics, pathology, and surgical treatment are also discussed.
Cholesterol* ; Granuloma* ; Mastoid ; Nasal Septum* ; Nose ; Pathology ; Temporal Bone

OBJECTIVE: To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone in children. METHOD: Seven children with LCH of the temporal bone n our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations, imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed. RESULT: Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external auditory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were. received different therapies. Followed up for 1-5 years, 4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly different from the single system group (P < 0.05). CONCLUSION The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.
Child ; Combined Modality Therapy ; Deafness ; etiology ; Ear Canal ; pathology ; Ear Diseases ; etiology ; Female ; Histiocytosis, Langerhans-Cell ; diagnosis ; pathology ; therapy ; Humans ; Male ; Prognosis ; Retrospective Studies ; Steroids ; therapeutic use ; Temporal Bone ; pathology

OBJECTIVE: To analyze the diagnosis, therapy and prognosis of the rare cases of malignant tumors in external auditory canal. METHOD: Eight cases of rare malignant tumors in external auditory canal were collected except squamous cell carcinoma and adenoid cystic carcinoma. The clinical manifestation, staging, surgical method, secondary treatment and prognosis were analysed. RESULT: There were 5 cases of basal cell carcinoma and 2 cases of mucoepidermoid carcinoma and 1 case of myoepithelial carcinoma. Three cases of the 5 basal cell carcinoma in external auditory canal have been misdiagnosed for a long time. After admission, 4 of the 5 basal cell carcinoma were T1 stage and cured only by a complete resection of tumor. One case of T2 stage basal cell carcinoma was found recurrence 2 years later after the first excision of tumor, and was treated with radiotherapy. Tumor was controlled. Two cases of mucoepidermoid carcinoma had been misdiagnosed as a benign tumor and received a resection. One case was found metastasis to the parapharyngeal space and nasopharynx and was treated with concurrent chemotherapy. Tumor was also controlled after 5-years follow-up. Another case was found metastasis to parotid gland and received an expanding tumor resection. No recurrence was detected after a 1-year followed up. One case of T4 stage myoepithelial carcinoma in external auditory canal was a metastasis of parotid and received a partial temporal bone resection. No-recurrence was found 1 year later. CONCLUSION Because the tumors above have a very low incidence in external ear canal and the location of tumors are hidden, they are often misdiagnosed and delayed in treatment. Therefore, otologists should pay more attention to avoid the misdiagnosis. To the treatment, complete surgical resection of early-stage tumor is important and for late-stage tumor, a supplemented by chemoradiotherapy may be needed.
Carcinoma, Adenoid Cystic ; diagnosis ; surgery ; Carcinoma, Basal Cell ; diagnosis ; surgery ; Carcinoma, Squamous Cell ; diagnosis ; surgery ; Ear Canal ; pathology ; Ear Neoplasms ; diagnosis ; surgery ; Humans ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Parotid Gland ; pathology ; Prognosis ; Retrospective Studies ; Temporal Bone ; surgery

One case with metal crochet through external auditory meatus embedded stylomastoid foramen, preoperative found no paralysis, underwent temporal bone CT examination to assess the illness after emergency surgery to remove the foreign body, postoperative patients of peripheral facial paralysis, explore and summarize the clinical experience.
Ear Canal ; pathology ; Facial Paralysis ; surgery ; Foreign Bodies ; surgery ; Humans ; Metals ; Otorhinolaryngologic Surgical Procedures ; methods ; Temporal Bone

OBJECTIVE: To investigate imaging and audiology features of temporal bone and analyze the classification and prevalence of inner ear abnormalities in children with sensorineural hearing loss. METHOD: Children who were diagnosed with sensorineural hearing loss were examined by high resolution CT and the inner ear fluid of MRI. And each chart was retrospectively reviewed to determine the imaging and audiology features. RESULT: There were 125 patients(232 ears) found with inner ear malformation in 590 children with SNHL. About 21.71% of the inner ear malformation occurred in severe and profound hearing loss ears, and 12.85% occurred in r moderate hearing loss ears. The inner ear malformation rate in normal hearing ears were 13.59%. CONCLUSION CT and MRI examinations of temporal bone are important diagnostic tools to indentify inner ear malformations. Inner ear malformations are almost bilateral and hearing loss are profoud. Cochleo-vestibular malformations and large vestibular aqueduct are the 2 most frequent deformities. Among the children with SNHL, deformity rate in the severe and profound hearing loss ears is higher than that in moderate hearing loss ear. Inner ear malformations can exist in people with normal hearing.
Audiology ; Child ; Ear, Inner ; abnormalities ; Hearing Loss, Sensorineural ; congenital ; pathology ; Humans ; Magnetic Resonance Imaging ; Prevalence ; Retrospective Studies ; Temporal Bone ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; abnormalities