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We present an unusual case of bilateral temporal bone destruction due to eosinophilic granuloma in a young child. The patient initially presented with otitis media, with subsequent rapidly developing bilaterally symmetric masses in the mastoid region. Computed tomography demonstrated extensive bony destruction which, after a course of vinblastine chemotherapy, improved dramatically on follow-up scans.
The pathology of the temporal bone and the inner ear in particular has been a neglected area for histopathologist. This review sets out to answer a number of questions on the topic: why is it neglected; how do you learn its anatomy; how do you process it; why do it; and who should do it? The histopathological features of cholesteatoma, otosclerosis and presbyacusis are discussed in detail and future areas of research are identified.
CT and MRI have revolutionized the evaluation of the temporal bone and its diseases. Conventional polytomography, once the mainstay of clinical imaging, has now been completely replaced by CT for evaluation of the middle ear, ossicular chain, and otic capsule and by MRI for the study of the internal auditory canal, cerebellopontine angle, and brainstem. This article is an overview of these current methods and depicts normal and pathologic anatomy. The technical aspects and clinical indications for both modalities are discussed.
In a pair of temporal bones serial sectioning revealed widespread Paget's disease with otosclerosis on both sides and, at the right side, adenocarcinoma added. Stapedial fixation was produced on the right by otosclerosis, and on the left by Paget's disease. In the contest to reach the inner cochlear space, Paget was the winner on the left, where, at the tympanic scala, otosclerosis and Paget's disease almost met, while the immediately adjacent organ o Corti was uninvolved.
Modern immunologic techniques of immunostaining, immunoblotting, and creation of monoclonal antibodies are gaining wide application in studies of development, function, and pathology of the ear. These techniques require a source of inner ear tissue for production of antigen extract. Human tissue is not readily available, and other mammalian species common in auditory research are small in size. Bovine temporal bones are readily available, and the membranous portions of the inner ear are abundant and easily accessible. Herein we report our technique for acquisition and dissection of bovine temporal bones and preparation and preservation of inner ear antigen.
HYPOTHESIS: We attempted to determine if the common mitochondrial DNA aging deletion is also associated with presbycusis. BACKGROUND: Presbycusis is the most common cause of deafness in adults in the United States, affecting approximately 40% of the population older than 75 years of age. The ability to identify a gene(s) or a specific genetic deficit(s) associated with presbycusis has significant clinical importance. METHODS: The current study examined mitochondrial DNA (mtDNA) from cochlear sections of 34 human temporal bones: 17 with normal hearing and 17 with presbycusis. DNA was extracted from celloidin-embedded temporal bone sections; and specific oligonucleotide primers were designed to amplify the cytochrome b gene and a 4,977 base pair (bp) deletion of the mtDNA. Polymerase chain reaction (PCR) was used to amplify the base pair products that correspond to targeted gene regions, and sequencing was used to verify the products. RESULTS: Fourteen of the 17 patients with hearing loss showed the 4,977 bp deletion and this deletion was present in only eight of the 17 human specimens with normal audiograms. The cytochrome b gene was amplified from all specimens. CONCLUSIONS: The current study demonstrates the presence of a 4,977 bp deletion in human mitochondrial DNA genome that is associated with aging and with some forms of presbycusis. These results, coupled with previous animal studies, suggest that this 4,977 deletion may be associated with presbycusis.
Due to the fact that the temporal bone has broad segments of bone anterior to and behind the internal auditory canal which are empty of functionally important structures, a broad exposure of the cerebellopontine angle may be obtained from above. This technique is adequate for the management of acoustic neurinomas up to 3 cm in diameter. A low postoperative morbidity and a high percentage of hearing preservation with total tumor removal were obtained in a series of 190 cases. This report underlines the advantages of the procedure and points to various other indications.
We reviewed the CT examinations of the temporal bone, performed with 1-mm-thick contiguous sections, of seven patients with the CHARGE association. We found abnormalities of the incus and stapes, with ossicular chain fixation, absence of the stapedius muscle and oval window, hypoplasia or dysplasia of the vestibule and absence of the semicircular canals in all ears. The pyramidal eminence and tympanic sinus were absent and there were anomalies of the cochlea in 13 of 14 ears. Absence of the semicircular canals is the most specific change in patients with the CHARGE association.
Histological studies were performed on the temporal bones of 17 persons who died of intracranial abscess as a complication of chronic otitis media and mastoiditis. In all cases the route of intracranial infection was via the dura mater rather than through the labyrinth. During the acute stage of intracranial abscess formation, the site of intracranial invasion showed a destructive inflammatory reaction characterized by the presence of granulations, resorption and sequestration of bone, invasive cholesteatoma, collections of purulent exudate, and necrosis of dura mater. At this stage any attempt at definitive removal of diseased tissue would necessarily result in a larger dural defect at a time when local disease and systemic illness present unsuitable conditions for reparative procedures. For this reason it would seem prudent to limit early otologic surgery to ensuring adequate drainage (e.g., postauricular open-wound drainage) and to perform corrective surgery (e.g., tympanomastoidectomy with repair of the dural defect) after the intracranial abscess has been brought under control.
Eight patients with spontaneous cerebrospinal fluid leak of temporal bone origin are presented. Pertinent history and surgical findings are reviewed and contrasted with 33 previously reported patients. Unilateral ear fullness and mild hearing loss are the most common presenting symptoms. Profuse clear otorrhea following myringotomy is virtually pathognomonic. Diagnostic methods including high-resolution computed tomography, magnetic resonance imaging, and contrast cisternography are discussed. The indications for transmastoid and combined transmastoid/middle fossa surgical repairs are compared. Both surgical approaches were found to be equally effective. We favor the transmastoid as the initial approach because of simplicity, safety, and the ability to visualize both the middle fossa and posterior fossa plates as well as the middle ear.
We report the pathologic changes in the temporal bones of four children who died of bronchoppenumonia secondary to acute measles infection. All suffered from severe necrotizing otitis media characterized by round cell infiltration. In one case, multilobulated giant cells (Warthin-Finkelday) were found in the middle ear. These cells appear to be similar to those found elsewhere within the body during acute measles infection. Inner ear changes were seen in two cases. Severe loss of nerve fibers and ganglion cells with atrophy of the striae vascularis was present in one case. Adhesions were seen between the Reissner membrane and the tectorial membrane in one case and the Reissner membrane and the inner spiral limbus in a second case. Such findings are characteristic of those seen in the cochlear duct of persons suffering from prenatal rubella.
Microfissures in the human otic capsule have been observed since the start of the century, but it was Otto Mayer, in 1930, who first realized that some of them were of intravital origin and not just processing artifacts. Since then, a small number of publications, based on decalcified temporal bones, have mostly confirmed his findings. With the introduction by Frost in the late 1950s of the undecalcified bone technique and the bulk staining technique for peripheral bones, a method was developed and refined for identifying even very small intravital microfissures (microdamage). Bulk staining of undecalcified otic capsules has not yet been used to verify the findings from the previous decalcified specimens. The present report presents our experience with the pertinent techniques, and suggests modifications and shortcuts pertinent to temporal bone research. Both large and tiny microfissures of intravital genesis are demonstrable within bulk-stained undecalcified human otic capsules. The importance of microfissures in the petrous bone in the causation of otosclerosis and perilymphatic leakage has long been discussed, and the present techniques may advance our understanding of these pathological conditions
Aggressive papillary tumors of the temporal bone are neoplasms that are locally invasive and destructive. Previously classified on histologic study as middle ear adenomas or adenocarcinomas, observational evidence suggested that they arose from endolymphatic sac. To evaluate this hypothesis, we established a tissue culture from cells derived from such a papillary tumor and compared immunohistochemical stains of the original tumor with stains on endolymphatic epithelium. Similarities in expression of neuroectodermal antigens were observed. Similar staining antigens in cells derived from tumor and the endolymphatic sac provide evidence that epithelium from endolymphatic sac is the site of origin for these aggressive neoplasms. In tissue culture the cells remain contact inhibited and dependent on serum or growth factors with survival beyond the expected senescence at 30 to 50 generations. Therefore the cell culture technique provides a model for study of the disruption of growth control and invasive properties of this tumor.
Immunohistochemical analyses were conducted on archival celloidin-embedded human temporal bone sections from an 8-month-old boy with chronic otitis media and DiGeorge syndrome. We employed antigen retrieval methods with saturated sodium hydroxide-methanol solution, microwave incubation, and proteolytic treatment to demonstrate the distribution of T-lymphocytes, B-lymphocytes, macrophages, and intercellular adhesion molecule 1 (ICAM-1) expression in the middle ear. B-lymphocytes and macrophages were observed predominantly within the middle ear mucosa. T-lymphocytes were rare. Further, ICAM-1 was expressed in the vascular endothelium of the lamina propria, as well as infiltrating mononuclear cells. This suggests that the expression of ICAM-1 can be induced in the middle ear with otitis media, even if T-lymphocytes are depressed in a cell-mediated immunodeficiency disorder such as DiGeorge syndrome.
The temporal bone and the brain stem of an infant with Cornelia de Lange syndrome, aged 9 months, were studied histopathologically. Abnormalities in the inner ear included short cochlea, dilated vestibule, and mesenchyme-filled perilymphatic spaces of the vestibule and semicircular canals. The inner and outer hair cells were normal. Many spiral ganglion cells, which normally would be located in the canal of Rosenthal, were seen in the internal auditory meatus. Some of the vestibular ganglion cells intruded into the facial nerve. The facial nerve formed a loose curve and the geniculum was difficult to recognize. The middle ear was filled with mesenchyme, and there was marked dehiscence of the facial canal. The brain stem, particularly the pons, was smaller than normal, while the fourth ventricle was dilated. The microscopic observation of the pons revealed weak staining with Luxol Fast Blue and decreased number of oligodendroglia. These findings suggested the malformation of the myelin of transverse fibers.
This study aimed to demonstrate the temporal bone histopathology of two cases of Wegener's granulomatosis in which the initial symptoms were profound hearing loss and facial nerve palsy respectively. The first case, a woman of 44, suffered profound hearing loss which was remarkably improved by steroid and cyclophosphamide treatment for a time, and which seemed to be caused by invasion from granulation tissue filled in the tympanic cavity. The second case was a 61-year-old woman presenting with the facial nerve palsy. The bony canal of the horizontal portion of the facial nerve was destroyed due to granulation tissue which filled in the tympanic cavity, and granulomatous involvement was observed in the facial nerve. Wegener's granulomatosis can involve the middle ear and/or inner ear, causing hearing loss of conductive, mixed or sensorineural type. Pathogenesis of facial nerve palsy seems to be related to Wegener's granulomatous involvement of facial nerve, because the facial nerve palsy also resolved by using steroid and cyclophosphamide.
BACKGROUND: Cerebrospinal fluid (CSF) leaks of the temporal bone region require surgical treatment as they pose life-threatening risks such as meningitis. AIM: The aim of the study was to determine the surgical outcome depending on different operation techniques and grafts. METHOD: We performed a retrospective review of 28 cases of CSF leaks, operated in our department from 1983 to 2002. After a mean follow-up of 8 years, patients were interviewed concerning otorrhea or rhinorrhea and meningitis. In this context, our management of CSF leaks is presented. RESULTS: The CSF leak had arisen spontaneously (n = 3), traumatically (n = 6) or postoperatively (n = 19). The surgical CSF leak repairs were performed via a transmastoid (n = 13), a middle fossa (n = 11) or a combined (n = 4) approach. Surgical outcome was independent on the used graft. CSF leak could be sealed in 25 of 28 cases. Only 3 patients suffered from recurrences. Meningitis or other complications did not occur. CONCLUSIONS: Comparing different techniques and grafts, there were no differences in the surgical outcome.
Two infants who had multiple joint dislocations, unusual facies, and bony abnormalities, typical of the syndrome first reported by Larsen et al. (J. Pediatr., 37 (1950) 574-581) are described. This report expands Larsen's syndrome to include the following findings of temporal bone pathology. Case 1 (one year and 8 months old) showed dislocation of the malleus and incus, an abnormal foot plate, mesenchyme remaining in the attic and mesotympanum, poor development of the mastoid air cells, and poor development of the labyrinth. Case 2 (3 years and 6 months old) showed dislocation of the malleus and incus, mesenchyme remaining in the attic and mesotympanum, and an abnormal stapes. These histological findings suggest that a maldevelopment similar to that which occurs in the skeletal system occurs in the temporal bones as well as in the face and extremities including many joints.