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In search of pathologic correlates for hearing loss and vertigo in Paget's disease. A clinical and histopathologic study of 26 temporal bones.

Mixed sensorineural and conductive hearing loss is a common clinical manifestation of Paget's disease of the temporal bone, and while there are numerous clinical and pathologic reports on the condition, none have identified a consistent pathologic explanation for the hearing loss. We performed histologic studies on 26 temporal bones exhibiting Paget's disease from 16 persons, of whom 7 had audiometric testing performed. Contrary to common opinion, the conductive hearing loss is not caused by ossicular fixation; in fact, no cause could be found in the seven ears with documented conductive hearing losses. While the sensorineural hearing losses were greater than normal for age, we could not identify cochlear disorders that could be attributed to Paget's disease. It is concluded that the hearing losses in Paget's disease are caused by changes in bone density, mass, and form that serve to dampen the finely tuned motion mechanics of the middle and inner ears.

Adult↗

[Diagnosis and treatment on osteoradionecrosis of temporal bone in cases with nasopharyngeal carcinoma after radiotherapy].

OBJECTIVE: To discuss the diagnosis and treatment on osteoradionecrosis of temporal bone in case with nasopharyngeal carcinoma (NPC) after radiotherapy. METHOD: The clinical data of 21 cases (22 ears) diagnosed as osteoradionecrosis of temporal bone with NPC were studied, including 17 ears of localized type and 5 ears of diffuse type. Minimally surgical debridement with otoscope was performed on 17 ears of localized type and 1 ear of diffuse type. Four ears of diffuse type underwent the modified radical mastoidectomy and obliteration with vascularized fascia flaps. RESULT: The common clinical features were foul-smelling otorrhea and otalgia. Otoscopy revealed the exposed bone and bony sequestration in the external auditory canal with impacted keratotic debris, granulation tissue and cholesteatoma accumulation. It was with erosion or/and orifice to the mastoid. CT scan demonstrated the presentations: osteomyelitis, bone destruction as the islet-appearance and soft tissue mass. Among the 17 ears of localized type, 12 ears (70.6%) showed complete epithelization of the raw surface, 4 ears (23.5%) not fully epithelized were followed up without sequestration, and 1 ear (5.9%) was developing progressively. As for the 5 ears of diffuse type, 1 ear received the minimally surgical debridement and another 3 ears treated with mastoidectomy and obliteration were healed. Only 1 ear needed secondary surgery because of re-sequestration. CONCLUSION: Both otoscopy and CT scan can offer the diagnostic value with different characteristics. The minimally surgical debridement of the sequestration by otoscopy can obtain good effects on localized type. It should be careful to select the surgery recipient on the diffuse type, and mastoid obliteration with vascularized flaps from regional tissue may be warranted after mastoidectomy. Regular follow up is needed because of possibility of the progressive development of the disease.

Adult↗

Epidermoids involving the temporal bone: clinical, radiological and pathological aspects.

Epidermoids or congenital cholesteatomas arise from aberrant epithelial remnants and are, therefore, considerd blastomatous malformations. Their predilective sites are the intracranial cavity, the diploe of the skull and the spinal canal. In the base of the skull the temporal bone is the most frequent site. Epidermoids account for about 0.2-1.5 percent of all intracranial tumors. The majority originate in the cerebello-pontine angle where they account for 6-7 percent of all tumors. Their age incidence reveals a great scatter from birth to 80 years. The majority are recognized during the third and fourth decades with the onset of clinical symptoms occurring much earlier. They affect males more frequently than females. Their delicate capsule with a whitish mother-of-pearl sheen lends them a typical appearance. Epidermoids are generally slow growing lesions which may remain asymptomatic for years. The irritative effect of their content, however, can produce symptoms of dysfunction and intense inflammation. Malignant changes occur infrequently. Diploic epidermoids are easily recognized, whereas, intradural epidermoids are more difficult to identify. Epidermoids may arise in the vicinity, on the outer aspect or within the temporal bone. Epidermoids originating in any of these locations have certain characteristic features which may arouse suspicion of their presence. Examples of an epidermoid with origin in the typical locations within the temporal bone and cerebello-pontine angle are discussed to portray their individual characteristics.

Adolescent↗

Temporal bone studies of the human peripheral vestibular system. Normative Scarpa's ganglion cell data.

Scarpa's ganglion cell counts were performed in 106 serially sectioned, normal human temporal bones from 75 individuals. Of these 106 bones, 15 were from neonates less than 30 days old, 14 were from infants between the ages of 1 and 12 months, and the remainder were distributed throughout each decade of life, with sample sizes ranging from 4 to 10 per decade. All temporal bones had to meet 2 criteria: no symptoms or signs of inner ear disease except for presbycusis in the medical case history and no abnormality in the inner ear on light microscopy. The total ganglion cell counts declined significantly with age at an average rate of 57 cells per year. The age-related decline was significantly greater in the superior division than in the inferior division. There was also a significant sex effect, independent of age: for any age, the count in men averaged 1,526 cells higher than in women. There was no significant interaural difference. Mathematical models were developed to compute the mean and 95% prediction intervals for Scarpa's ganglion cell counts in terms of age and sex parameters. The counts and models will serve as a normative database against which to compare counts made in temporal bones from subjects with known vestibular disorders.

Adolescent↗

Tumours of the mastoid temporal bone: with interesting cases in the paediatric age group.

Tumours of the mastoid temporal bone are interesting clinical entities owing to their rarity. A wide variety of benign and malignant tumours occur in and around the temporal bone reflecting the complicated embryological origin and resultant diversity of tumours found in the area. Friedmann (1974), in a classification of tumours of the ear, has grouped some tumours as intermediate in variety, e.g. glomus jugulare tumour, in addition to various benign and malignant tumours. Metastatic deposits from various distant organs such as the lung, breast and kidney can occur in the mastoid bone (Schucknecht, 1968). Invasive tumours can spread to the mastoid bone from adjacent areas such as neoplasms of the parotid and cranium.

Child↗

Effects of acute hypertension on the extravasation of macromolecule in the temporal bone--the possible involvement of the blood-inner ear barrier.

In guinea pigs the radioactivity in the vascular beds was washed out by a cardiac perfusion of saline 15 min after the injection of 50 microCi of radioiodinated human serum albumin (RISA). The brain and temporal bones were dissected and a blood sample was obtained. To examine the degree of extravasation of RISA, the extravascular radioactivity in the tissues was measured and expressed as a ratio to that of the blood. Intravenous injection of amphetamine sulfate increased the extravasation in the brain and the temporal bone of the rat but did not increase it in either tissue of the guinea pig. It was proposed that this was because amphetamine did not cause the blood pressure of the guinea pig to reach the "critical level" which would cause the opening of the blood-tissue barrier. In the rat the degrees of extravasation in both the brain and the temporal bone paralleled the maximal mean arterial pressure caused by amphetamine. It is proposed on the basis of these data that there may exist a blood-inner ear barrier and that this barrier has the same characteristics as the blood-brain barrier.

Acute Disease↗

Primary chondroid chordoma of the base of the petrous temporal bone.

The authors describe an unusual case of a primary chondroid chordoma arising from the base of petrous temporal bone. The patient, a young male, presented with a left sided jugular foramen syndrome. The CT findings of a tumour arising from the base of the left petrous temporal bone were confirmed at infratemporal exploration. The clinical and histopathological and immunohistochemical differences between classical (typical) chordoma and its chondroid variant are highlighted. The rarity of primary chordomas at this site is stressed.

Adult↗

Preparation of human temporal bone for immunohistochemical investigation.

Successful treatment of progressive sensorineural hearing loss with corticosteroids and cyclophosphamide has promoted the notion of a new entity 'autoimmune hearing loss'. In this context, we have examined sera of patients suffering from idiopathic sensorineural hearing loss, in order to study the binding of humoral antibodies to structures of the normal human inner ear. Investigations were carried out with indirect fluorescence techniques on temporal bones obtained at autopsy. To minimize the possibility of artifacts, we suggested that indirect immunofluorescence with the patients' sera should yield a reproducible positivity on sections of at least three normal temporal bones. In this study, we describe a method for removal and preparation of human temporal bone which ensures reproducible immunohistochemical results.

Adolescent↗

Alkaline phosphatase activity in the ganglion cells in and near the temporal bone.

The distribution pattern of alkaline phosphatase was studied in the ganglion cells in and near the temporal bone of some mammals. No or extremely weak activity of alkaline phosphatase was observed in or near the spiral and vestibular ganglion cells of the bat, rat, cat, and monkey. However, intense to moderate enzyme activity was noted exclusively in the peripheral areas of the spiral and vestibular ganglion cells of the guinea pig. The enzyme activity was also demonstrated in the nerve fibers at some distance from both axonal and dendritic processes of the spiral and vestibular ganglion cells of the guinea pig. Trigeminal and geniculate ganglion cells of all mammals examined showed intense to moderate enzyme activity in their capsular cells. There is a marked difference in the phosphate metabolism in the capsular tissues of the ganglion cells in or near the temporal bone of various mammals.

Alkaline Phosphatase↗

[Malignant melanoma of the temporal bone].

The present article describes a case of malignant melanoma with the rare localisation in the right temporal bone. The 79-year old female patient presented a polypous tumour with obturation of the outer ear canal and sanguineous otorrhoea (Fig. 1). The inner ear function was impaired showing a combined defective hearing and complete peripheral palsy of the facial nerve as well as an abducens nerve palsy (Fig. 1) with diplopia. CT and MR imaging showed a tumour destroying the temporal bone up to the apex of the pyramid and the clivus reaching the middle cranial fossa (Fig. 2-3). After palliative radiotherapy with 40 Gy the general condition and clinical symptoms improved. After a period of 8 months with no signs of further tumour progression the patient was admitted again in reduced general condition showing pulmonary and pleural metastases. The patient died 14 days later.

Aged↗

The structure of the fibrous tissue on the articular surface of the temporal bone in the monkey (Macaca mulatta).

The articulating surface of bones which ossify in mesenchyme, like the mandible, is covered by a layer of dense, fibrous tissue. The purpose of the present study was to examine the structure of the fibrous tissue on the surface of the articular surface of the temporal bone in the monkey. Young Rhesus monkeys (Macaca mulatta) were perfused with glutaraldehyde-paraformaldehyde. The specimens were demineralized in 0.5M EDTA. Small pieces of fibrous tissue and underlying bone were dissected out and processed for light and electron microscopy. The mandibular fossa is shallower and the articular eminence flatter in the monkeys as compared to humans. The articular part of the temporal bone is covered by a layer of avascular, soft tissue extending from the surface to the underlying bone. The tissue can be divided into three zones which gradually merge into one another. The zone facing the articular cavity consists of dense, fibrous tissue with layers of collagen fibers, oriented parallel to the articular surface, but at angles to each other. Fibers thought to be elastic fibers oriented parallel with the collagen fibers are also observed, particularly close to the surface, and their function is probably to impart resilience to the fibrous articular tissue. Between the fibers scattered cells with an ample rough endoplasmic reticulum are present. A thin layer of granular appearance is often observed on the surface. This layer may be of importance in joint lubrication. The second zone is more cell rich and the cells have long slender cellular processes and are surrounded by a dense collagenous matrix with an irregular orientation. These cells are probably precursor for the underlying cartilage but, not for the cells in the outer articular layer. In the third zone next to the bone the fibrous tissue gradually turns into cartilage. The cartilagenous zone is narrow, sometimes absent and is replaced by bone tissue. In some areas chondroclasts are observed, with forming osteons with osteoid seams. These observations indicate that remodeling is taking place and that cartilage is replaced by bone. The three zones observed correspond to findings in the mandibular condyle, but the zones are not as constant and distinct as in the condyle, and this reflects the adaptive role of the temporal bone in the growth of the temporomandibular joint.

Animals↗

[Temporal bone histopathology in a patient suspected of inner ear extension of otitis media].

We report the histopathological findings in the temporal bone of a 30-year-old female who died of cervical esophageal carcinoma. The temporal bone sections revealed severe bilateral suppurative labyrinthitis and otitis media that presumably occurred immediately before her death. Many inflammatory cells were present in the middle ear, particularly around the stapes and the round window niche. They had also infiltrated the inner ear via the annular ligament of the stapes and the round window membrane. Inflammatory cell accumulation was also observed in the peri- and endolymphatic spaces, and it was most severe in the basal turn. Most of the inner and outer hair cells were preserved, but some had degenerated or were missing. Numerous round cells were observed in the modiolus, and some of the spiral ganglion cells had degenerated. On the basis of these findings, we concluded that bacterial otitis media had extended in to the inner ear via the oval window and round window membrane and had resulted in suppurative labyrinthitis. These findings are consistent with those of stage II suppurative labyrinthitis according to the classification of Schuknecht.

Adult↗

[Preoperative computed tomography of temporal bones and its use in selecting patients with sensorineural deafness for cochlear implantation].

Cochlear implantation (CI) involves the insertion of electrode systems in the inner ear in order to restore hearing in patients with sensorineural deafness. Positive CI results are closely related with careful selection of candidates. Preoperative computed tomography (CT) and its results are decisive in this selection. Temporal bone CT was made in 40 patients aged 1 to 57 years who had sensorineural deafness. The following changes were detected: malformations of the inner ear, the inner auditory canal, meningititis-induced cochlear obliteration, post-traumatic temporal bone changes, local auditory ossicle malformation, inflammatory middle ear changes, the high position of the jugular vein bulb, and its diverticula. Temporal bone CT plays an important role in selecting candidates for CI and circumvents potential difficulties and complications at CI.

Adolescent↗

Temporal bones for dissection. A diminishing asset?

In this prospective study, the author studied four local ENT departments to discover how many temporal bones were being harvested. The results show that only eight per cent of routine post mortems have temporal bones removed.

Autopsy↗

Temporal bone findings in keratitis, ichthyosis, and deafness syndrome. Case report.

In 1981, the term KID syndrome was suggested for patients with congenital ichthyosis associated with deafness and keratitis. We had a chance to examine the temporal bone of an infant with this syndrome. This patient showed no auditory brain stem response in either ear. Temporal bone studies revealed cochleosaccular abnormality. These findings are offered as a possible explanation for the patient's deafness. The pathologic inner ear findings of congenital deafness syndromes associated with ichthyosis have been heretofore reported in Refsum's syndrome and in a case with universal alopecia. In these cases, the temporal bone pathologic findings were a result of cochleosaccular abnormality. From our case and previous reports, it is suggested that the deafness associated with congenital ichthyosis might be the result of cochleosaccular abnormality.

Cochlea↗

Role of 3D CT in the evaluation of the temporal bone.

In recent years, three-dimensional (3D) multiplanar reformatted images from conventional cross-sectional computed tomographic (CT) data have been increasingly used to better demonstrate the anatomy and pathologic conditions of various organ systems. Three-dimensional volume-rendered (VR) CT images can aid in understanding the temporal bone, a region of complex anatomy containing multiple small structures within a relatively compact area, which makes evaluation of this region difficult. These images can be rotated in space and dissected in any plane, allowing assessment of the morphologic features of individual structures, including the small ossicles of the middle ear and the intricate components of the inner ear. The use of submillimeter two-dimensional reconstruction from CT data in addition to 3D reformation allows depiction of microanatomic structures such as the osseous spiral lamina and hamulus. Furthermore, 3D VR CT images can be used to evaluate various conditions of the temporal bone, including congenital malformations, vascular anomalies, inflammatory or neoplastic conditions, and trauma. The additional information provided by 3D reformatted images allows a better understanding of temporal bone anatomy and improves the ability to evaluate related disease, thereby helping to optimize surgical planning.

Bone Diseases↗