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Temporal bone histopathologic abnormalities associated with mitochondrial mutation T7511C.

OBJECTIVES: We previously reported a mitochondrial T7511C mutation in the tRNA gene in a Japanese family with nonsyndromic hearing loss (HL). However, the temporal bone histopathology associated with T7511C has not been reported. The aim of the present study is to report histopathologic findings of a temporal bone from a patient in the Japanese family with this mutation. STUDY DESIGN: Single case study. METHODS: A temporal bone was obtained from the right ear of a male subject with progressive HL from 5 years of age and who died at 60 years of age from cerebral infarction. The bone was embedded, sectioned, and stained with hematoxylin-eosin for light microscopic study. Graphic reconstruction of the cochlea was performed using the method described by Schuknecht to determine loss of the stria vascularis and neurosensory elements including hair cells and spiral ganglion neurons. RESULTS: The most significant histopathologic finding was severe loss of spiral ganglion cells in all turns of the cochlea. Severe loss of neuronal filaments in Rosenthal's canal was also observed. The organ of Corti showed scattered loss of inner and outer hair cells in the basal turn. Partial atrophy of the stria vascularis was observed in all turns of the cochlea. CONCLUSION: Our results suggest that severe loss of spiral ganglion cells was the main cause of sensorineural HL associated with the T7511C mutation.

Atrophy↗

Temporal bone fractures in children: a review with emphasis on long-term sequelae.

A 10-year retrospective study of pediatric basilar skull fractures identified 120 patients with temporal bone fractures, of which 92 had otolaryngologic and audiologic evaluations. The otologic data provide documentation of the nature, common physical findings, complications, and management of these fractures. The prognosis of hearing impairment in children caused by temporal bone fractures is elucidated, as 76 of the 92 patients were available for long-term follow-up. Close interaction between involved services evaluating and treating pediatric trauma cases is mandatory for optimal patient outcome. A literature review of the presentation, complications, and management of pediatric temporal bone fractures is presented.

Adolescent↗

Malakoplakia of the temporal bone in a nine-month-old infant.

A case of malakoplakia, of the temporal bone in a nine-month-old male child is reported. The lesion presented as an aural polyp, associated with a lower motor neuron facial palsy. On exploration, the granuloma was noted to involve the temporal bone, eroding the bony labyrinth. It was successfully treated with surgical debridement, and antibiotics. A review of the relevant literature is presented.

Bone Diseases↗

[Monostotic fibrous dysplasia of the temporal bone coinciding with cholesteatoma].

Fibrous dysplasia is a rare disease which occasionally affects the temporal bone. In its monostotic form, only 27 cases have been reported until today. We present a case of monostotic fibrous dysplasia of the temporal bone coincident with a cholesteatoma of the EAC, operated on by mean of a modified radical mastoidectomy, with good results.

Adolescent↗

Results of radiotherapy in chemodectoma of the temporal bone.

Between 1958 and 1986, twenty-four patients with chemodectoma of the temporal bone received radiation therapy at the Center of Oncology in Cracow. In 22 patients incomplete excision of the tumor had been performed at other hospitals before admission for radiotherapy. Two patients had biopsy only. Three patients had early stage tumors without bone destruction or cranial nerve palsy and 21 patients had advanced tumors at presentation. Three patients treated between 1958 and 1967 received orthovoltage 250 V irradiation, 20 patients received 60Co teletherapy, and one patient received a combination of photon and electron therapy. Tumor doses ranged from 30 Gy to 60 Gy, given in 2-2.5 Gy fractions, 5 times weekly. There were no serious acute or late complications. Long-term local control was achieved in 23 patients with follow-up ranging from 16 months to 21.5 years (median 87 months). Only one patient had locally uncontrolled tumor along with lung and cervical node metastases. Irradiation appeared to be an effective and well tolerated method of treatment in chemodectoma of the temporal bone.

Adult↗

Spiral CT of the temporal bone in unsedated pediatric patients.

PURPOSE: To determine the feasibility of performing rapid, high-resolution, low-radiation-dose spiral CT scans of the temporal bone in unsedated pediatric patients. METHODS: Axial spiral CT examinations with an effective section thickness of 1.25 mm were performed in 41 infants and children. Multiplanar reconstructions were generated using 360 degrees and 180 degrees linear interpolation algorithms. Three-dimensional reconstructions of the middle ear were also performed. RESULTS: Complete axial spiral scans were performed without sedation in all patients. Three hundred sixty-degree linear interpolation images had comparable resolution to conventional CT; 180 degrees linear interpolation images with an effective section thickness of 1.0 mm at 0.2-mm intervals showed good detail of middle and inner ear anatomy, and multiplanar reconstructions from this algorithm were comparable to direct scans. Three-dimensional reconstructions clearly displayed the oval window, malleus, and incus. CONCLUSION: Spiral CT is a clinically feasible method for rapidly evaluating the temporal bone in pediatric patients, eliminating the need for sedation. The images obtained from this technique are comparable to conventional CT. High-quality multiplanar reconstructions can be generated from the spiral data set, providing an alternative to direct scans in multiple planes. Additionally, three-dimensional images of internal temporal bone anatomy can be generated from the spiral data.

Child↗

Insufficient and absent acoustic temporal bone window: potential and limitations of transcranial contrast-enhanced color-coded sonography and contrast-enhanced power-based sonography.

The aim of this study was to investigate the diagnostic potential of contrast-enhanced transcranial color-coded sonography (CE-TCCS) and contrast-enhanced transcranial power-based sonography (CE-TPS) in patients with insufficient or absent acoustic bone windows (IABW). Due to temporal bone thickness, the basal cerebral circulation could not be insonated in 21 of 172 patients using unenhanced transcranial color-coded real-time sonography (TCCS) and transcranial power-based sonography. Additional CE-TCCS and CE-TPS were performed after application of 400 mg/ml galactose microbubble suspension. In both modalities, the use of echo-contrast agents allowed visualisation of the first segment of the middle cerebral artery (MCA) in all patients. The A1 segment of the anterior cerebral artery (67% in CE-TCCS; 81% in CE-TPS), P1 segment of the posterior cerebral artery (71% in CE-TCCS; 76% in CE-TPS) and the basilar artery (48% in CE-TCCS; 67% in CE-TPS) were depictable in the majority of the examinations. The M3 (5% in CE-TCCS; 33% in CE-TPS; p < 0.05), P2 (24% in CE-TCCS; 71% in CE-TPS; p < 0.005), P3 segments (0% in CE-TCCS; 43% in CE-TPS; p < 0.005) and the posterior communicating artery (5% in CE-TCCS; 33% in CE-TPS; p < 0.05) were detected in a significantly greater proportion of subjects using power Doppler. In conclusion, CE-TCCS and CE-TPS appear to be sensitive ultrasonic tools that provide reliable data regarding the basal cerebral circulation in patients with IABW. Furthermore, CE-TPS offers advantages over CE-TSSC in the identification of small-caliber arteries and vessels that run at unfavorable angels to the ultrasound beam. Both methods can overcome hyperostosis of the skull that is a major hindrance in transcranial ultrasonography, and may be helpful in the diagnosis of occlusive diseases of intracranial vessels.

Adult↗

Massive acquired cholesteatoma of the temporal bone.

Nine patients with massive acquired cholesteatoma of the temporal bone were treated by radical mastoidectomy, facial nerve decompression, and excision of the inner ear. The commonest presenting symptoms were otorrhea and deafness, but otalgia, vertigo, external swelling and facial nerve palsies were also seen. Polytomography was helpful in assessing the degree of bone erosion preoperatively. The large cavity present following cholesteatoma excision was partially obliterated by a superiorly based temporalis muscle pedicle which helped to seal the internal auditory meatus and prevented the development of a cerebral hernia. Those patients with preoperative facial nerve palsies showed evidence of nerve degeneration and had incomplete recovery at a one year follow-up.

Adolescent↗

Sclerosteosis involving the temporal bone: histopathologic aspects.

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

Adult↗

Heat radiation during caloric vestibular test: thermographic demonstration in temporal bone experiments.

Since Barany's descriptions in 1906, cold and warm irrigations of the external ear canal have been used for unilaterally testing vestibular excitability. The fluid kinetics within the endolymph have been studied thoroughly, whereas relatively few published articles deal with the mechanisms of heat transfer from the external to the internal ear. Even though heat conduction via the bone seemed to be well established in the textbooks, Feldmann and co-workers found heat radiation to be a very important factor. Using a rather uncommon method, we tried to make this heat radiation more apparent: in temporal bone experiments, temperature distribution was followed by thermography. Temporal bone specimens were prepared in such a way that heat distributions became visible after experimental caloric irrigations of the external ear canal. Temperature changes could well be verified by changes in coloration of the 2-dimensional thermograms. Conclusions were drawn from registrations performed in 2 projections, 90 degrees to each other, allowing cautious statements about 3-dimensional distribution. The velocity of heat transfer at the very onset of the reaction can only be explained by radiation, whereas later, other mechanisms, such as conduction via the bone or convection via the middle ear gas, may have some influence. Our results support Feldmann's findings and underline the significance of radiation in the heat transfer mechanism.

Body Temperature↗

Temporal bone pathology in patients without caloric response.

Pathologies of serial sections of 9 temporal bones from 6 patients without caloric response were studied. The patients died of epipharyngeal cancer, middle ear cancer, chronic renal failure with DIC, pancreatic cancer with DIC, multiple brain metastasis of pancreatic cancer, and leukemia. Under light microscopy, 8 ears showed pathologies in the vestibular end organs and the nerves. Possible causes of the lack of caloric responses in the 8 ears were endolymphatic hemorrhage, labyrinthitis, leukemic infiltration in the labyrinth, degeneration or disappearance of the vestibular sensory cells, reduction in the number of vestibular nerve fibers, and/or brainstem lesions. The remaining one ear did not show abnormal findings in the vestibular end organs or the nerves, but the patient had brainstem lesions. Four types of temporal bone pathologies were observed according to the lesion site responsible for the lack of caloric response; i) sensory type, ii) neural type, iii) mixed type and iv) central type.

Adult↗

Evaluation of the advance off-stylet insertion technique and the cochlear insertion tool in temporal bones.

OBJECTIVE: The concept and design of new cochlear implant electrodes is a challenging process. To evaluate new electrode designs, we present a study that uses a microgrinding procedure to evaluate damage to the microstructures of the cochlea resulting from the insertion procedure. In this study, we compared different insertion techniques with the Contour electrode with Softip for placement inside the cochlea and any resulting damage. METHODS: Twenty-five fresh frozen human temporal bones were used to compare electrode insertion characteristics with three insertion techniques (i.e., conventional insertion, Advance Off-Stylet performed manually, and Advance Off-Stylet performed with insertion tool) and two prototype variants of the Contour electrode with Softip (referred as Softip I and Softip II in this article). Five temporal bones were used for each arm of the study: Softip I electrode and conventional insertion; Softip I electrode and manual Advance Off-Stylet insertion; Softip I electrode and Advance Off-Stylet insertion with an early experimental insertion tool; Softip II prototype electrode and manual Advance Off-Stylet insertion; and Softip II prototype and Advance Off-Stylet insertion with a prototype insertion tool. The temporal bones were dehydrated and embedded in epoxy and used for the microgrinding procedure. Resulting images were documented and compared with conventional radiographic images. RESULTS: Our results showed that, especially when using the conventional insertion technique with Softip I electrode arrays, basilar membrane perforations were observable. Using the prototype insertion tool, good placement of the electrode array but also two basilar membrane perforations (one with each type of electrode) were observed. In contrast, the Advance Off-Stylet insertion technique did not show basilar membrane perforation with Softip I and II electrodes and resulted in reliable perimodiolar placement of the arrays. CONCLUSION: Using microgrinding of temporal bones, the Advance Off-Stylet insertion technique was proven to enable more atraumatic insertions of Contour electrodes with Softip and to provide very reliable perimodiolar placements.

Basilar Membrane↗

Histopathology of human temporal bone after cis-platinum, radiation, or both.

Preserving organs by use of multiple modalities has become protocol in treating squamous cell carcinomas of the head and neck, but cis-platinum and radiation can impair hearing. To determine the effect of cis-platinum, radiation, or a combination of these treatments on the temporal bone, we studied histopathologic slides of 15 human temporal bones: four after cis-platinum, five after radiation, two after combined treatment, and four from normal controls. Hair cells and cells in spiral ganglia were counted in reconstructed organs of Corti. Lumen-to-diameter indexes in arterioles near facial nerves were quantified for four normal controls and seven irradiated patients. Available audiograms were compared. Decreased spiral ganglion cells, loss of inner and outer hair cells, and atrophy of stria vascularis were demonstrated in groups receiving cis-platinum, radiation, and combinations, compared with age-matched controls. Arterioles around facial nerves demonstrated fibrinous clots within the intima, endothelial proliferation, and hypertrophy and fibrosis of vascular walls in smooth muscle. Fibrosis in connective tissue was clearly progressive after radiation. Cis-platinum and radiation can contribute to otologic sequelae, including sensorineural hearing losses, vascular changes, serous effusion, or fibrosis. Prophylactic treatments and techniques to deliver them should be considered for protection of temporal bones and preservation of hearing after oncologic modalities.

Adult↗

Malignant fibrous histiocytoma of the temporal bone with intracranial extension.

An 18-year-old male presented with a right temporal bone tumour; histology revealed malignant fibrous histiocytoma. Following surgical excision and radiation the tumour recurred. The patient was treated with Vindesine (desacetyl vinblastine amide sulphate) with marked response. Malignant fibrous histiocytomas of the temporal bone are extremely rare; this report suggests that the occasional dramatic response may be obtained by combined modality treatment.

Adolescent↗

Registration and fusion of CT and MRI of the temporal bone.

OBJECTIVE: To present and evaluate a registration method to fuse complementary information of CT and MRI of the temporal bone. METHODS: CT and MRI of the temporal bone of 26 patients were independently registered 4 times. A manual, iterative, intrinsic, rigid, and retrospective registration method was used. Mean CREm (consistency registration error) was calculated as a reproducibility measurement. RESULTS: CREm was 0.6 mm (95% CI = 0.52-0.68 mm). T-test revealed no difference between pathologic and normal cases (t[102] = -1.71; P = 0.09). Time needed: 13 minutes. In the registered and fused datasets, important bony surgical landmarks (eg, facial nerve canal, inner ear) could be assessed in 3 dimensions relatively to tumor tissue (eg, acoustic schwannoma). Fluid distribution within partially obliterated cochleae could be assigned to either scalae. CONCLUSION: An accurate, reproducible registration and fusion method that improves tumor surgery and cochlea implantation planning with only minor changes to the clinical workflow was presented and described. We suggest this method in selected cases.

Adolescent↗

A planimetric study of temporal bone pneumatization.

The present study is based on the results of planimetric measurements of temporal bone pneumatization in 60 children. Radiological and clinical follow-up studies show that a myringotomy is sufficient therapy for cases of serous effusions. In contrast, mucous effusion require long-term middle ear ventilation with tympanostomy tubes to get approximately the same increase in temporal bone pneumatization.

Child↗

[Osteoma of the temporal bone as a rare differential diagnosis of a retroauricular tumor].

Extracanalicular osteomas of the temporal bone represent a rare differential diagnosis of osseous tumours in the ear region in contrast to the common finding of exostoses of the external auditory canal. Basing on a report on a retroauricular osteoma of the squama of the temporal bone treated in the authors' ENT clinic, the morphological and clinical characteristics of this tumour are discussed.

Adult↗

Fixation of the stapes footplate in children: a clinical and temporal bone histopathologic study.

OBJECTIVE: To clarify the anatomic characteristics, cause, and surgical outcomes relating to fixation of the stapes footplate in children. STUDY DESIGN: Retrospective case review and four-center histopathologic study of temporal bones. SETTING: Tertiary referral center. PATIENTS: We reviewed charts and histologic specimens from 12 children, aged 7 to 13 years, who underwent surgery for footplate fixation. We also studied stapes footplates in 288 temporal bones from 181 children ranging from newborn (20-44 weeks of gestation) to 13 years of age. MAIN OUTCOME MEASURE: Anomalies of the stapes footplate in children. RESULTS: The average age of diagnosis of hearing loss was 6.6 years. Criteria for a diagnosis of otosclerosis were progression of a conductive hearing loss and an intraoperative finding of fixation of the anterior stapediovestibular joint in five patients. In contrast, a nonhomogeneous, thickened, fixed footplate and the absence of an annular ligament were indicators of congenital fixation in six children. In one child, there was neither new bone from the otic capsule nor any obvious otosclerotic foci. In the temporal bone study, 17 of 181 (9.4%) children had anomalies of the stapes footplates, with ankylosis in 4 (2.2%). In two subjects (1.1%), there was an otosclerotic focus not in contact with the stapes footplate. CONCLUSION: Children younger than 6 years with various congenital anomalies are more likely to have congenital footplate fixation, which will present intraoperatively as a thickened footplate with a partial or absent annular ligament. Children older than 6 years with progressive conductive hearing loss are more likely to have otosclerosis, which presents as fixation of the anterior stapediovestibular joint. The difference in surgical outcomes is probably related to different degrees of footplate abnormality.

Adolescent↗