This report describes a case of adult temporal bone Langerhans cell histiocytosis（LCH） that presented as posterior canal dehiscence syndrome（PCDS）. The patient initially presented with vertigo, tinnitus, and hearing loss. Computed tomography（CT） revealed erosive changes in the mastoid and posterior semicircular canal. After the operation, the patient's hearing and vestibular symptoms disappeared and postoperative recovery was favorable. Adult LCH is relatively rare, and this case serves as an example to discuss the clinical features and treatment options of this disease, followed by a literature review.
Humans ; Histiocytosis, Langerhans-Cell/complications* ; Semicircular Canal Dehiscence/etiology* ; Temporal Bone/pathology*

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 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

OBJECTIVE: To investigate diagnostic value and clinical application of MRI in the children with sensorineural hearing loss (SNHL) before cochlear implantation. METHOD: MRI images of 80 children with the diagnosis of SHNL were retrospectively analyzed in combination with the latest classification of inner ear malformation. RESULT: There were 152 ears of inner ear malformation of 80 cases (160 ears), including 38 ears of cochlear malformation, 33 ears of vestibular malformation, 41 ears of semicircular canal malformation, 37 ears of vestibular aqueduct enlargement, 40 ears of internal auditory canal malformation, and 46 ears of cochlear nerve malformation. CONCLUSION MRI can provide detailed and reliable anatomical information for children with SNHL before cochlear implantation, and help to make the classification diagnosis. Therefore MRI is of great clinical significance for operation plan guidance and prognosis assessment.
Child ; Cochlear Implantation ; Cochlear Nerve ; pathology ; Hearing Loss, Sensorineural ; diagnosis ; pathology ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Semicircular Canals ; pathology ; Temporal Bone ; pathology ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; abnormalities ; pathology

A pregnant female taken realgar because of superstition, which caused the baby congenital deafness. Auditory test indicated that bilateral auditory brainstem response (ABR) hearing threshold level was greater than 90 dB nHL and auditory steady state response (ASSR) hearing level ranging from 0.5 kHz to 4 kHz was beyond 110 dB HL. Temporal bone CT showed that bilateral cochlear and semicircular canal malformations, with internal auditory canal broadened.
Arsenicals ; adverse effects ; Evoked Potentials, Auditory, Brain Stem ; Female ; Hearing Loss, Sensorineural ; chemically induced ; congenital ; Hearing Tests ; Humans ; Infant ; Maternal Exposure ; adverse effects ; Pregnancy ; Semicircular Canals ; pathology ; Sulfides ; adverse effects ; Temporal Bone ; pathology