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Cochleosaccular dysplasia: a morphometric and histopathologic study in a series of temporal bones.

OBJECTIVE: The objective of this study was to perform a morphometric analysis of a series of temporal bones with cochleosaccular dysplasia to clarify the extent of inner ear changes in this disease. STUDY DESIGN: This human temporal bone histopathologic study of a series of deaf-mute cases involves morphometric analysis, including stria vascularis and spiral ligament area measurements and spiral ganglion and hair cells counts. SUBJECTS: Thirteen temporal bones were selected from 35 with deaf mutism based on the histopathologic findings described by Scheibe. Twenty normal age-matched control subjects were used for comparisons. RESULTS: All temporal bones had the main histopathologic findings described by Scheibe, as well as severe affected stria vascularis. Seven temporal bones had cystic areas in the stria and three had concretions. Cross-sectional strial areas in temporal bones with cochleosaccular dysplasia were smaller than normal in all cochlear turns; however, no difference was found in spiral ligament cross-sectional areas. Reissner's membrane was hydropic in three temporal bones and the organ of Corti was absent in at least one cochlear turn in five. Concretions were present in the macula of seven temporal bones. Twelve temporal bones showed some level of spiral ganglion cell loss. No hair cells were observed in any temporal bone. A familial history of deafness was found in three cases. CONCLUSION: Pathologic findings were variable and limited to the saccule and scala media. The variation, perhaps, reflects the different etiologies involved in the origin of cochleosaccular dysplasia.

Adolescent↗

[Differences in temporal bone development in various ear diseases].

To investigate the differences in temporal bone development in various ear diseases--bilateral Meniere's disease (n = 13), unilateral Meniere's disease (n = 41), unilateral chronic otitis media (COM) (n = 25), temporal bone fracture (n = 9) and otosclerosis (n = 12)--the following 4 distances on a temporal bone CT slice encompassing the lateral semicircular canal (LSC) were measured by using the NIH Image program. These distances included the minimal distance between the posterior semicircular canal (PSC) and the posterior petrous surface (PPS) (P-P distance), the minimal distance between the PSC and the LSC (P-L distance), the minimal distance between the vestibule and the PPS (V-P distance) and the minimal distance between the PSC and the anterior margin of the sigmoid sinus (P-S distance). Both the P-P and V-P distances showed significant differences among the ear diseases, but the P-L and P-S distance did not. Meniere's disease showed significantly shorter P-P and V-P distance especially in bilateral Meniere's disease than those in COM and otosclerosis (p < 0.05). Affected ears in unilateral Meniere's disease showed a shorter P-P distance than non-affected ears (p < 0.01). In contrast, both ears with otosclerosis showed significantly longer P-P and V-P distances than those in Meniere's disease, COM (p < 0.01) and temporal bone fracture (p < 0.05). COM and temporal bone fracture showed intermediate P-P and V-P distances, without a difference between the affected and non-affected ears. In conclusion, the development of the posterior part of the temporal bone is reduced in Meniere's disease and greatly increased in otosclerosis. These findings by CT may be useful for diagnosing ear diseases.

Ear Diseases↗

Management of complications from 820 temporal bone fractures.

OBJECTIVE: To review the incidence of complications resulting from temporal bone fractures and analyze the outcomes from surgical and nonsurgical management. STUDY DESIGN: A retrospective review of 699 patients with 820 temporal bone fractures occurring over a 5-year period. SETTING: A single level 1 trauma center. PATIENTS: All patients with clinical or radiologic evidence of a temporal bone fracture. RESULTS: A total of 820 fractured temporal bones resulted in 58 facial nerve injuries, 122 CSF fistulae, and 15 cases of meningitis. The two most important prognostic factors in recovery of facial paralysis were severity and delay of onset. All patients with incomplete paralysis recovered. All but one of the delayed onset palsies had good recovery of function. A total of 40% of patients with immediate onset complete paralysis had poor recovery of function. Ninety-five of the 122 CSF fistulae closed spontaneously within 1 week. CSF fistulae persisting for > 7 days had a significantly increased risk of developing meningitis (23%) compared with patients whose fistulae closed within 7 days (3%) (p = 0.001). Another important risk factor for the development of meningitis was concurrent infection. CONCLUSIONS: Facial function following temporal bone fractures should be evaluated in the emergency room. If facial motion is noted at any time after the injury, surgical intervention is rarely indicated. Prophylactic antibiotics should be considered in temporal bone fractures when CSF fistulae are present. Surgical closure of a CSF fistula is indicated if it persists for > 7-10 days.

Adolescent↗

Metastatic tumours of the temporal bone. A histopathological report.

Metastatic tumours of the temporal bone seem to be more common than is recognized. Most of these tumours are microscopic and asymptomatic in nature. Microscopic examination of 22 temporal bones belonging to 13 cases of metastatic tumours is reported. The commonest site of involvement in the temporal bone was the petrous apex followed by the tegmen tympani, mastoid bone and internal auditory canal. Primary tumours were most commonly located in the breast. Other sites of primary tumours included the thyroid gland, brain, lungs, prostate and blood (leukaemia). Two cases had undetermined sites of origin. Full neurotologic evaluation is indicated in every case suspected of having a temporal bone metastasis. All three modalities (of surgery, radiotherapy and chemotherapy) are used in combination for the treatment of these tumours.

Adenocarcinoma↗

Otological manifestations of thalassaemia intermedia: evidence of temporal bone involvement and report of a unique cholesteatoma-like lesion.

Thalassaemia intermedia should be considered in any chronically anaemic patient presenting from the Middle East with hearing impairment. We report here three Saudi siblings with thalassaemia intermedia and features of severe bone marrow expansion, particularly invading the temporal bone. They were seen first for their otological problems before they had access to proper haematological evaluation. One member was admitted for surgical exploration of a cholesteatoma, which was then found to be marrow expansion of the temporal bone. Screening of the family revealed two more anaemic siblings with thalassaemia intermedia. Audiological examination of all the family members showed that only the two affected members had a high frequency sensori-neural hearing loss. Bone marrow expansion into the temporal bone is a rare feature of thalassaemia intermedia. Cholesteatoma-like lesion has not been previously described. It has to be considered in all cases of symptomatic thalassaemia intermedia manifesting with cavitation and lytic lesions in the mastoid system. The likelihood that sensorineural hearing loss may complicate the thalassaemias is raised and the possible mechanism for such involvement discussed. The proper management for different otological manifestations of the thalassaemias is suggested. These cases would suggest a more extensive involvement of the temporal bone in the thalassaemias than has been previously recognized. Further large scale studies are required to illuminate the subject.

Adolescent↗

Three-dimensional reconstruction of the temporal bone.

Study of the complex anatomy and pathology of the temporal bone has traditionally used microscopy which permits analysis in only two dimensions. Recent advances in bioimaging technology have permitted visualization and reconstruction of computed tomography images in three dimensions. We have developed a technique that applies this technology in the imaging and reconstruction of human temporal bones. Data taken from serial histologic sections of the temporal bone are entered into a computer. The sections are edited and, through the use of specially developed software, a realistic three-dimensional reconstruction is produced. The reconstructed image can be rotated along any of three axes, and structures within the temporal bone can be isolated for more detailed analysis. Applications for the study of pathologic conditions of the temporal bone will be discussed.

Adult↗

Guided tissue generation in the temporal bone.

Ten patients with malformations of the temporal bones were submitted to installation of implants for anchorage of bone-anchored auricular epistheses or bone-anchored hearing aids. Due to lack of bone in the temporal region, expanded polytetrafluoroethylene membranes were used to aid bone generation, each patient serving as his or her own control. It was found that bone formation around the flange fixtures covered with membranes increased significantly compared to the uncovered fixtures. It is concluded that the use of barrier membranes is one way to support bone generation in temporal bones lacking sufficient bone for implant-related surgery.

Bone Regeneration↗

The pathology of the temporal bones of a child with acquired cytomegalovirus infection: studies by light microscopy, immunohistochemistry and polymerase-chain reaction.

STUDY DESIGN: The first case of an acquired cytomegalovirus (CMV) infection of the inner ear is reported in a 3-year-old girl in remission from acute lymphocytic leukemia. METHODS: Horizontal sections of the temporal bones were studied by light microscopy and immunohistological staining by avidin-biotin-complex-technique was performed on selected archival sections. Three sections were processed for detection of the virus genome by the polymerase chain reaction (PCR). RESULTS: By light microscopy the epithelium of the endolymphatic sac, the utricle and the semicircular canals showed deeply stained acidophilic inclusions and the stria vascularis had a loose structure especially in the intermediate layer. The changes were limited to the non-sensory parts of the labyrinth and no CMV type cells were observed in the organ of Corti. There was a loss of inner and outer hair cells and loss of cochlear ganglion cells caused by either the virus or treatment with gentamicin. Standard immunohistochemistry failed to demonstrate staining with CMV antibodies, but PCR, demonstrated CMV-DNA in one section. CONCLUSION: Molecular techniques may be able to detect acquired CMV infections in archival pediatric bones temporal bones. The histologic findings in the labyrinth were milder, however showed some similarity to children with congenital CMV labyrinthitis.

Autopsy↗

Radiographic classification of temporal bone fractures: clinical predictability using a new system.

OBJECTIVE: To compare the traditional system of radiographic classification of temporal bone fractures (transverse vs longitudinal vs oblique) with a newer system (otic capsule violating vs otic capsule sparing) with respect to their ability to predict sequelae of temporal bone trauma. DESIGN: Retrospective chart and radiology review. SETTING: University trauma center and Department of Otolaryngology-Head and Neck Surgery. PATIENTS: Patients with temporal bone fractures. INTERVENTIONS: Clinic records and computed tomographic scans were reviewed to evaluate the clinical predictability of complications of temporal bone fractures. MAIN OUTCOME MEASURES: Complications of temporal bone fractures (ie, sensorineural hearing loss, conductive hearing loss, cerebrospinal fluid leakage, and facial nerve weakness) were recorded. Two classification schemes for temporal bone fractures were statistically analyzed and compared as to their ability to predict each complication. RESULTS: A total of 234 temporal bone fractures were identified; 30 cases met our strict criteria for inclusion. The traditional classification system of temporal bone fractures did not significantly predict temporal bone complications (P = .71). On the other hand, the otic capsule-based system did demonstrate statistically significant predictive ability (P < .001). Patients with otic capsule-violating fractures were 5 times more likely to have facial nerve injury, 25 times more likely to have sensorineural hearing loss, and 8 times more likely to have cerebrospinal fluid otorrhea than those with otic capsule-sparing fractures. CONCLUSIONS: The traditional radiographic classification system failed to demonstrate clinical predictability in our series. Furthermore, the newer system of classification (otic capsule sparing vs otic capsule violating) demonstrated statistically significant predictive ability for serious clinical outcomes associated with temporal bone fractures.

Accidental Falls↗

Cranial base morphology and temporal bone pneumatization in Asian Homo erectus.

The external morphological features of the temporal bone are used frequently to determine taxonomic affinities of fossils of the genus Homo. Temporal bone pneumatization has been widely studied in great apes and in early hominids. However, this feature is rarely examined in the later hominids, particularly in Asian Homo erectus. We provide a comparative morphological and quantitative analysis of Asian Homo erectus from the sites of Ngandong, Sambungmacan, and Zhoukoudian, and of Neandertals and anatomically modern Homo sapiens in order to discuss causes and modalities of temporal bone pneumatization during hominid evolution. The evolution of temporal bone pneumatization in the genus Homo is more complex than previously described. Indeed, the Zhoukoudian fossils have a unique pattern of temporal bone pneumatization, whereas Ngandong and Sambungmacan fossils, as well as the Neandertals, more closely resemble the modern human pattern. Moreover, these Chinese fossils are characterized by a wide midvault and a relatively narrow occipital bone. Our results support the point of view that cell development does not play an active role in determining cranial base morphology. Instead, pneumatization is related to available space and to temporal bone morphology, and its development is related to correlated morphology and the relative disposition of the bones and cerebral lobes. Because variation in pneumatization is extensive within the same species, the phyletic implications of pneumatization are limited in the taxa considered here.

Animals↗

Eosinophilic granuloma of the temporal bone.

Involvement of the temporal bone by eosinophilic granuloma is rare. Two cases are presented and their clinical manifestations, histopathological findings and treatment procedures discussed.

Adult↗

Auricular carcinoma with temporal bone invasion: outcome analysis.

Invasion of the temporal bone by cutaneous carcinoma of the auricle and periauricular skin is an ominous prognostic sign. Management includes aggressive resection of cutaneous disease as well as resection of temporal bone to obtain a medial margin. Analysis of data from 21 patients with temporal bone invasion caused by cutaneous malignancy is presented. Overall survival is approximately 63%. Cumulative survival is significantly decreased in patients with squamous cell carcinoma when compared with other invasive malignancies. Univariate and covariate analyses demonstrate that nodal status, positive microscopic soft tissue margins, and persistent perineural disease at the skull base did not significantly affect survival in this series. There is a trend toward increased survival in patients receiving postoperative radiation in this series.

Adult↗

Temporal bone fractures.

This report has illustrated the variety of temporal bone fractures that involve the zygomatic, squamous, and petromastoid components of the temporal bone. Complications as they relate to each type of fracture have been emphasized. Although longitudinal and transverse fractures can be identified, temporal bone trauma usually creates unique fractures constellations that must be individually described. In each case, a careful inspection of the ossides, otic capsule (vestibule/cochlea), internal auditory, and facial nerve canal must be undertaken. When a patient presents with temporal bone fracture and facial nerve paralysis, identification of the site of facial nerve injury may now lead to surgical decompression of this area of the facial nerve canal and possible nerve grafting.

Humans↗

The Lacrimo-Auriculo-Dento-Digital (LADD) syndrome: temporal bone CT findings.

The temporal bone CT examination of a 16-year-old female patient with the LADD syndrome or Levy-Hollister syndrome showed multiple bilateral middle as well as inner ear malformations. Ossicular chain anomalies were seen, especially of the incus and stapes. The oval window was very narrow to absent. Both cochleas were hypoplastic and showed modiolar deficiency. A common cavity between the vestibule and lateral semicircular canal was bilaterally present.

Abnormalities, Multiple↗

Amplification of RNA from archival human temporal bone sections.

BACKGROUND: The amplification of DNA from celloidin-embedded human temporal bone sections by polymerase chain reaction (PCR) has been applied to some auditory diseases, such as herpes zoster oticus and hearing loss caused by the mutations of mitochondrial DNA. However, few studies have reported detection of RNA from temporal bone sections. OBJECTIVES: Because RNA analysis from temporal bone sections may elucidate the development of the diseases in the auditory, vestibular, and facial nerves, the authors investigated whether RNA in these sections can be amplified by reverse transcription (RT)-PCR. METHODS: Sections that were formalin-fixed, decalcified, and embedded between 1972 and 1986 were used. Nucleic acid was extracted from the celloidin-embedded temporal bone sections and subjected to RT-PCR. Human alpha-tubulin RNA was reverse transcribed to cDNA and amplified by nested PCR using two sets of primers that were designed to distinguish cDNA from genomic DNA based on the presence of an intron between the primers. RESULTS: Human alpha-tubulin RNA was detected in 11 of 14 temporal bone sections (79%) by RT-PCR. RNA was detected in even the oldest sections, which were processed in 1972. CONCLUSIONS: These results indicate that RNA can be analyzed from archival celloidin-embedded human temporal bone sections.

Archives↗

[Oscillometry on various stape protheses. Experimental examination of human temporal bone preparations].

In human temporal bone specimens the vibrations of the labyrinthine fluid were measured by a piezoelectric system after application of various techniques of otosclerosis surgery. Compared with the amplitude in the case of a normal stapes, the surgical techniques according to Zangemeister, Shea and Schuknect resulted in considerable transmission losses in the high frequency range, while transmission of low frequencies to the inner ear was almost equivalent to normal transmission via the stapes. For frequencies of more than 3,000 Hertz the damping influence of the fibrous tissue implanted into the oval window was verified. The results of surgery according to Zangemeister, Shea and Schuknechtdid not differ significantly in the low and medium frequency range. Only a Robinson prosthesis implanted like a Teflon piston caused an impairment of transmission, while the method of fitting this prosthesis onto fibrous tissue was equilvalent to the other techniques.

Ear, Middle↗

Effects of opening and resealing the cochlea on the mechanical response in the isolated temporal bone preparation.

The isolated temporal bone preparation has been used previously for studying the micromechanical behaviour of the cochlea. Mechanical tuning curves have been obtained from several cells and structures within the hearing organ. In order to obtain access to the apical turns the bony shell of the cochlea has to be opened. To study how the opening affects the mechanical response of the cochlea, experiments were performed in which the cochlea was opened and then sealed with a glass window. Responses were measured from the same identified cells in the opened and in the sealed cochlea. The opening of the cochlea reduced the vibration amplitude mainly at frequencies below 300 Hz. Below the mechanical resonance frequency the slope of the tuning curve became steeper. The shape was not affected appreciably above the resonance frequency. The relative vibration amplitude of different cells remained unchanged by opening and closing the cochlea.

Acoustic Stimulation↗

Meniere's disease and endolymphatic hydrops without Meniere's symptoms: temporal bone histopathology.

We studied temporal bone histopathology in 21 ears with Meniere's disease and 24 ears with endolymphatic hydrops without Meniere's symptoms and compared the findings to those in 10 ears with presbycusis and 11 ears with normal hearing. Normal hearing ears showed less degeneration of cochlear structures than the other ears. In ears with endolymphatic hydrops without Meniere's symptoms, the degeneration of spiral ligament, hair cells, dendrites (peripheral processes) and apical spiral ganglion cells was more severe than in the other three groups. In ears with Meniere's disease and endolymphatic hydrops without Meniere's symptoms, the hair cells and dendrites were more affected than ganglion cells and there was no correlation between hair cell and ganglion cell degeneration. These findings suggest that a permanent threshold shift in late stage endolymphatic hydrops is not related to ganglion cell loss but rather to degeneration of sensory elements.

Aged↗