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Temporal bone findings in a case of otopalatodigital syndrome.

The principal features of the otopalatodigital syndrome are hearing loss, cleft palate, and skeletal dysplasia of the hands and feet. The right temporal bone was acquired from a boy with this syndrome who died at the age of 2 1/2 years. Behavioral audiometry had indicated a conductive hearing loss, with probable near-normal sensorineural function; brainstem evoked response audiometry indicated a mild sensorineural hearing loss. Histologic studies of the temporal bone revealed dysmorphic features in both the middle ear and the bony labyrinth. The ossicles were deformed, the stapes was fixed, and no round window was present. A defect of the modiolus resulted in a wide communication between the subarachnoid space of the internal auditory canal and the scala vestibuli. These anomalies would clearly have frustrated any attempt to improve the patient's hearing through reconstructive middle ear surgery.

Abnormalities, Multiple↗

Obliteration of mastoid and middle ear for severe trauma to the temporal bone.

A review of four cases of severe trauma to the temporal bone involving fracture of the external auditory canal indicates that adipose obliteration of the mastoid and middle-ear cleft with closure of the external auditory canal is the optimal method of repair. In two of four patients, one-stage repair was successful using this technique, and in the other two patients, obliteration was required as a revision operation after cholesteatoma developed from previous procedures.

Adolescent↗

Temporal bone fractures in children: a review of 34 cases.

Head injuries are commonly seen in accident and emergency (A&E) departments and within this group a small proportion will have a temporal bone fracture. Thirty-four such cases were identified from a 7-year period and their case notes were reviewed. The mechanisms of injury included:falls outdoors (15 cases), falls in the home (eight cases), road traffic accidents (RTAs; seven cases), missiles (three cases) and non-accidental injury (one case). In 20 cases the fracture involved more than one cranial bone, and the implications of this with regard to non-accidental injury are discussed. CT scans were carried out in 14 cases and 11 of these showed intracranial haematoma. The criteria for CT scan following head injury in general, and temporal bone fracture in particular are discussed. Outcome measures indicated that those injured as a result of RTAs had the poorest outcome, followed by those who fell outdoors and then those who fell in the home.

Accidental Falls↗

Chondromyxoid fibroma of the temporal bone.

BACKGROUND: Chondromyxoid fibromas are benign neoplasms comprising approximately 0.5% of primary bone tumors. The occurrence of this tumor in the skull is extremely rare, with only four previously reported cases involving the temporal bone and a total of 17 cases with intracranial involvement. CASE DESCRIPTION: We describe the case of a 22-year-old man who experienced a 1-year history of headaches with progressive ear pain and mild hearing loss. Computed tomography and magnetic resonance imaging of the head demonstrated an extraaxial tumor arising from the left temporal mastoid region. A craniotomy was performed and the tumor resection required extensive drilling of the temporal bone. Grossly, the tumor consisted of a firm semigelatinous myxomatous tissue containing multiple areas of calcification and was histologically consistent with chondromyxoid fibroma. CONCLUSIONS: Complete resection is the goal of surgery. Recurrence rates of 7% to 27% have been reported, dependent on the degree of initial resection. Radiation is not recommended because of the potential for sarcomatous conversion. Reoperation may be indicated for symptomatic recurrence.

Adult↗

Monostotic fibrous dysplasia of temporal bone: report of two cases and review of its characteristics.

Fibrous dysplasia is a bone disorder of unknown origin characterized by slow, progressive replacement of bone by abnormal proliferative isomorphic fibrous tissue. The disease was first described by McCune and Bruch in 1937. Craniofacial involvement is found in only 10% of cases of the monostotic variety, while temporal bone involvement is rare. We report herein two cases of monostotic fibrous dysplasia involving temporal bone and briefly review the clinical implications and management of the disease.

Adult↗

[Chondroblastoma of the temporal bone--report of a case and a review of the literature of 54 cases].

Chondroblastoma of the skull is a rare tumor and only 54 cases have been reported to date. A case of chondroblastoma arising from the squamous part of the left temporal bone is reported. A 34-year-old woman had 6-month history of left conductive hearing disturbance and tenderness in the left temporal region. Plain skull X-ray showed a well demarcated osteolytic lesion in the temporal bone. CT demonstrated a heterogeneously high density mass, with enhancement. T1-weighted MRI showed a low intensity mass while T2-weighted images showed no signal area. The left external carotid angiograms showed a marked staining supplied by the left middle meningeal artery. This tumor grew destroying the left temporal squama and pyramidal bone, and extended to the external auditory canal and the middle ear cavity. The tumor was subtotally resected. Histologically, this tumor consisted of clusters of round or polygonal chondroblasts with oval or grooved nuclei and well-defined cell border. Multinucleated giant cells were also observed. Chondroid matrix was found in some areas. Immunohistochemically, the tumor cells were positive for S-100 protein. These findings lead us to the diagnosis of chondroblastoma. The diagnosis, histology, therapy, and prognosis of chondroblastoma are discussed including the review of 54 cases in the literature.

Adult↗

Temporal bone: three-dimensional CT. Part II. Pathologic alterations.

Three-dimensional (3D) surface renderings were obtained from routine axial computed tomographic (CT) images in 15 patients with a variety of complex temporal bone abnormalities. The 3D CT reformations served as an adjunct to conventional sectional CT examination. While no diagnosis was substantially changed because of the 3D CT images, they did provide a more global perspective in cases of large tumors and fractures and at the postmastoidectomy site. Three-dimensional CT surface reformations are now practical and may be potentially useful for visualizing temporal bone lesions characterized by complex destructive change.

Adolescent↗

Inverted papilloma involving the temporal bone and its association with squamous cell carcinoma: critical analysis of the literature.

Inverted papilloma is a rare, benign neoplasm, which usually originates from the lateral nasal wall and can be locally aggressive, extending into surrounding structures. Unusually, this disease involves temporal bone. There have been only 18 reported cases in the literature. The available data regarding inverted papilloma of the temporal bone are reviewed, and its etiopathogenesis, recurrence rate and association with squamous cell carcinoma are discussed.

Bone Neoplasms↗

Systemic vasculitis: a temporal bone histopathologic study.

Systemic vasculitis includes a broad spectrum of disorders that may involve blood vessels of any size in any organ system. Systemic vasculitis is associated with immunopathogenic mechanisms. Sixteen temporal bones from eight persons were studied to determine histopathologic changes that occur in systemic vasculitis. Three persons had Wegener's granulomatosis, two had polyarteritis nodosa, and three had systemic lupus erythematosus. Otitis media was seen in 15 ears, with ten ears showing chronic middle ear changes and two showing fibrotic inner ear changes. In Wegener's granulomatosis, granulation tissue was observed around the eustachian tube and protympanum, and in polyarteritis nodosa, inflammatory cell infiltrate and thickened blood vessels were observed around the facial nerve. Although sensorineural hearing loss has been described clinically in systemic lupus erythematosus, the present report describes findings in temporal bones, including severe fibrosis and new bone formation throughout the inner ear.

Adolescent↗

Quantitative analysis of extracellular matrix proteins in hypertrophic layers of the mandibular condyle and temporal bone during human fetal development.

A computer image analysis of immunostained extracellular matrix (ECM) proteins (collagen types I, II, III and V, fibronectin and tenascin) in hypertrophic layers in the mandibular condyle and temporal bone of human fetuses, which ranged in gestational age from 12 to 32 weeks, was performed. The percentage of cells positive for proliferating cell nuclear antigen (PCNA) increased in almost the same manner in each region. The level of PCNA was markedly elevated at 16 weeks. The percentage of PCNA-positive cells was low in temporal bone and in the bone-forming layer of the mandibular condyle at 24 weeks. Specific concentration patterns of proteins in the ECM were found at each stage of development. The extent of accumulation of fibrillar collagen and of fibronectin differed, while those of other proteins in the ECM, such as tenascin, osteocalcin and osteonectin, were similar at the two sites.

Bone Density↗

Unbiased stereological estimation of the spiral ligament and stria vascularis volumes in aging and Ménière's disease using archival human temporal bones.

The present study applies the unbiased stereological technique-Cavalieri principle to measure the volumes of the stria vascularis (SV) and the spiral ligament (SL) using postmortem archival human temporal bones from normal young and older subjects and subjects with Ménière's disease. Normative data was obtained from subjects without ages ranging from 15 to 84 years old who had no history of audiovestibular disease (N=25). For comparison purposes, the normative specimens were divided into three groups: group 1 (n=8) had ages ranging from 15 to 38 years old, average age=23.9; group 2 (n=8) had ages ranging from 51 to 59 years old, average age=55.1; group 3 (n=9) had ages ranging from 64 to 84 years old, average age=74.3. The average SV volume of group 3 (0.479 mm3) was significantly lower than that of group 1 (0.705 mm3) (p<0.0005) and was significantly lower than that of group 2 (0.603 mm3) (p=0.01). The average SL volume of group 3 (8.42 mm3) was significantly lower than that of group 1 (9.54 mm3) (p<0.05), but was not significantly lower than that of group 2 (8.58 mm3). Five subjects with Ménière's disease, confirmed by histopathological examination (ages ranging from 63 to 91 years old, average age=73.4), were studied. The average SV volume in Ménière's subjects (0.378 mm3) was significantly lower than age-matched controls (p<0.05). The average SL volume in Ménière's subjects (7.01 mm3) was also significantly lower than age-matched controls (p<0.05). The SV and SL volumes were unaffected by gender. The present study demonstrates for the first time the use of the unbiased stereological technique-Cavalieri principle-as a reliable and efficient method to obtain volumetric estimates of the SV and the SL by using archival human temporal bone specimens.

Adolescent↗

Idiopathic sudden sensorineural hearing loss and postnatal viral labyrinthitis: a statistical comparison of temporal bone findings.

Although the cause of idiopathic sudden sensorineural hearing loss remains uncertain, a viral origin has been suggested in many cases on the basis of anamnestic microbiologic and pathologic data. Twenty-two temporal bone specimens from 18 patients who during life suffered a sudden partial or complete sensorineural hearing loss were studied. On the basis of clinical data, these cases were assigned to one of three diagnostic categories, and the temporal bones were studied by light microscopy and serial section analysis. The implications of the histopathologic findings for the pathogenesis of idiopathic sudden sensorineural hearing loss are discussed.

Adult↗

[Paget's disease with temporal bone involvement, hypoacusis and vertigo. Apropos a case].

The Paget disease is a chronic osteodystrophy of unknown etiology, very frequent in 50 years of age and above. It's an anatomo-radiologic syndrome that sometimes, is accompanied by clinical symptoms. May be monostotic or polyostotic but never generalized. Occasionally it affects the temporal bone producing deafness, tinnitus and vertigo. We report a case with important temporal bone demineralization, deafness and vertigo.

Audiometry, Pure-Tone↗

Early facial reanimation following radical parotid and temporal bone tumor resections.

A retrospective study of 35 patients who underwent early facial reanimation following extirpative parotid and temporal bone surgery requiring facial nerve sacrifice was performed. Regional facial reanimation performed immediately or within several days included 16 patients who underwent temporalis muscle transposition and 27 who underwent gold weight or eyespring lid reanimation with lower lid tightening. Simultaneous nerve grafts or nerve crossover procedures were performed in 22 patients. The authors' favored approaches to facial reanimation are discussed, with an emphasis on the value of early reanimation using properly selected techniques.

Adult↗

Idiopathic perilymphatic fistulas. A temporal bone histopathologic study with clinical, surgical, and histopathologic correlations.

OBJECTIVE: To report a case of idiopathic perilymphatic fistulas diagnosed and successfully treated during life and confirmed histopathologically post mortem. METHODS: Perilymphatic fistulas were diagnosed clinically and repaired surgically. Light microscopic histopathologic studies were made of both temporal bones after death. RESULTS: Vestibular symptoms had improved postoperatively. Postmortem histopathologic examination of the temporal bones demonstrated patencies of the labyrinth capsule that had been predicted clinically during life, the qualities of the membranous labyrinth in both the operated-on and the unoperated-on ears, and the elements of the surgical repair. Notably, there was no evidence of endolymphatic hydrops. CONCLUSIONS: The histopathologic findings document the relationships of the fistula ante fenestram and the fissure connecting the round window niche and the posterior semicircular canal to idiopathic perilymphatic fistulas. Also, the diagnostic features reported herein allowed this patient with fluctuating hearing loss, episodic vertigo, and tinnitus to be distinguished from a patient with Meniere's syndrome. These findings have implications regarding past and future studies of vestibular and cochlear disorders.

Aged↗

Gadolinium-enhanced magnetic resonance imaging in temporal bone lesions.

A 2-year experience with an enhancement agent in magnetic resonance imaging (MRI) is examined through case presentations. Characteristics of the agent Gadolinium DTPA/Dimeglumin are reviewed. Radiographic capabilities in enhanced versus unenhanced MRI are compared. Temporal bone lesions, including acoustic neuromas and facial nerve neuromas, are presented. Recent experience with facial nerve enhancement in Bell's palsy is also presented. Greater accuracy in defining small temporal bone lesions and increased delineation of facial nerve involvement appear to be significant benefits with enhanced MRI.

Adult↗