Intralabyrinthine osteogenesis in Cogan's syndrome

Temporal bones from a 64-year-old man who had Cogan's syndrome were examined by light microscopy. Although tissues of the middle ear appeared unremarkable, extensive ectopic bone formation was observed bilaterally in the inner ear membranous labyrinth. Such osteogenesis completely obliterated the cochlear compartments apically. Only the scala tympani compartment of the basal cochlear turn remained patent. Ectopic bone tissue was observed also in the vestibular semicircular canals. Marked degeneration of eighth nerve fibers and associated ganglion cells also was observed. To our knowledge, this report represents only the fourth temporal bone findings of Cogan's syndrome and demonstrates a more advanced pathologic state of inner ear pathology than those reported previously.     

Otologic manifestations of the hydantoin syndrome

The temporal bones of a newborn infant with hydantoin syndrome showed multiple middle ear and inner ear anomalies. There was a constellation of bony and membranous defects involving the oval and round windows, cochlear ducts, cochlear aqueducts, endolymphatic ducts and sacs, and vestibular labyrinths. To the authors' knowledge, supernumerary vestibular sensory epithelial structures and an inner ear epidermoid cyst have not been previously reported. Wide communications between the subarachnoid space and inner ear were of surgical relevance.     

Inner ear anomalies in Waardenburg's syndrome associated with Hirschsprung's disease

Congenital inner ear anomalies are reported in temporal bones of a 22-month-old boy with Waardenburg's syndrome and Hirschsprung's disease. Although no changes in the central auditory pathway were identified, peripheral lesions of the cochlear and vestibular membranous labyrinth were observed. Bilateral atrophy of the organ of Corti and stria, and a sparsity of spiral ganglion cells were observed in the cochlea. Degeneration of the vestibular end organs, including a loss of Scarpa's ganglion cells, was also seen. This is the first report of temporal bone histopathology associated with Waardenburg's syndrome and Hirschsprung's disease. The pathoembryology of these inner ear anomalies associated with aganglionosis of the colon supports the hypothesis that Waardenburg's syndrome and Hirschsprung's disease are hereditary defects of neural crest cells.     

Tympanic membrane/middle ear pathologic correlates in chronic otitis media.

OBJECTIVE: To correlate pathologic findings of the tympanic membrane with pathologic changes in the middle ear cleft in chronic otitis media. STUDY DESIGN: Retrospective. MATERIAL AND METHODS: One hundred-fifty temporal bones from 97 subjects with chronic otitis media (defined as middle ear pathologic changes including granulation tissue, fluid, cholesteatoma, cholesterol granuloma, tympanosclerosis, and ossicular changes) were selected to correlate the presence of these middle ear pathologies with histopathologic changes of the tympanic membrane. The tympanic membrane pathologies included perforation, myringosclerosis, retraction, hemorrhage, fluid-filled cystic spaces, or dilated vessels. Temporal bones were also assessed for atelectasis. Fifty-six normal temporal bones were taken as controls for measurements. RESULTS: Significant correlations between tympanic membrane and middle ear pathology included myringosclerosis and granulation tissue, myringosclerosis and ossicular pathology, retraction and cholesterol granuloma, retraction and cholesteatoma, retraction and ossicular pathology, perforation and ossicular pathology, and hemorrhage and granulation tissue. Additive effects of some pathologies were also observed. Almost half the bones with middle ear pathology had no associated tympanic membrane pathology, whereas multiple pathologic changes in the tympanic membrane generally showed underlying multiple pathologic changes in the middle ear. CONCLUSION: When tympanic membrane pathology is detected otoscopically, its presence, alone or in combination, can be a strong indicator of underlying middle ear pathology. However, a normal-appearing tympanic membrane does not exclude the possibility of middle ear pathology. These findings suggest the need for other diagnostic tools such as multifrequency tympanometry and otoacoustic emissions to complement otoscopy for diagnosis of middle ear pathology, especially in a tympanic membrane that appears "normal."     

Labyrinthine pathology of chronic renal failure patients treated with hemodialysis and kidney transplantation

An audiologic study of 290 hemodialysis and renal transplant patients revealed that in 43 of these patients significant hearing loss developed which could be directly attributed to the therapy of the kidney problem. The clinicopathologic findings in 16 temporal bones of eight chronic hemodialysis and renal transplant patients were presented. The five patients treated with 59 or less hemodialyses had no subjective hearing loss; on the other hand, the three patients receiving 264 or more hemodialyses and multiple transplants, complained of hearing and vestibular difficulties. The pathologic findings common to all 14 temporal bones of the seven patients who underwent transplantation were blue stained concretions in the stria vascularis and/or vestibular receptors. The cochlear changes noted ranged from mild loss of outer hair cells and spiral ganglion cells in patients with few hemodialyses and transplants to complete absence of the organ of Corti in patients receiving more than 264 hemodialyses and multiple transplants; thus, the severity of the clinical and histopathological temporal bone findings was directly proportional to the number of hemodialyses and transplants to which the patient had been subjected. This seems to suggest that numerous hemodialyses or recurrent kidney transplants can induce electrolytic, osmotic, biochemical, vascular and/or immunological changes in the inner ear which can lead to severe audioves-tibular symptoms and pathology.     

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.     

Light and electron microscopic studies of a case with simultaneous Mondini and Michel deformities of the inner ears

Summary We performed light and electron microscopic studies on the temporal bones of a patient with genetic aplastic deafness, in which the right ear had a Mondini-type defect and the left ear a Michel-type anomaly. The round window of each ear was absent. The stapedial foot plate of the right ear was depressed at its central part and was covered by thin membrane. The course of the internal auditory meatus of the left ear was deviated ventrally. There was total aplasia of the cochlea in the left ear, while that of the right ear showed only three-quarters of a turn. In this ear, three sites of spherical bulging were found and were believed to represent outer hair cells. Other structures of the normal cochlear duct were not present. The modiolus of the left ear was round and dome-shaped, contained sparse nerve fibers and a primitive spiral ganglion. The saccule, utricle and semicircular canals of each ear were missing, so that a common cavity of the vestibular system was present. Several otoliths could be seen under the surface of the membrane covering the common cavity. No sensory cells or their related tissues could be found in either ear.     

Light and electron microscopic studies of a case with simultaneous Mondini and Michel deformities of the inner ears

We performed light and electron microscopic studies on the temporal bones of a patient with genetic aplastic deafness, in which the right ear had a Mondini-type defect and the left ear a Michel-type anomaly. The round window of each ear was absent. The stapedial foot plate of the right ear was depressed at its central part and was covered by thin membrane. The course of the internal auditory meatus of the left ear was deviated ventrally. There was total aplasia of the cochlea in the left ear, while that of the right ear showed only three-quarters of a turn. In this ear, three sites of spherical bulging were found and were believed to represent outer hair cells. Other structures of the normal cochlear duct were not present. The modiolus of the left ear was round and dome-shaped, contained sparse nerve fibers and a primitive spiral ganglion. The saccule, utricle and semicircular canals of each ear were missing, so that a common cavity of the vestibular system was present. Several otoliths could be seen under the surface of the membrane covering the common cavity. No sensory cells or their related tissues could be found in either ear.     

Cytomegalovirus-induced pathology in human temporal bones with congenital and acquired infection

ObjectivePublications on histopathology of human temporal bones with cytomegalovirus (CMV) infection are limited. We aim to determine histopathology of the inner ears and the middle ears in human temporal bones with congenital and acquired CMV infections.MethodsTemporal bones from 2 infants with congenital and 2 adults with acquired CMV infection were evaluated by light microscopy.ResultsTwo infants with congenital CMV infection showed striking pathological changes in the inner ear. There was a hypervascularization of the stria vascularis in the cochlea of the first infant, but no obvious loss of outer and inner hair cells was seen in the organ of Corti. However, cytomegalic cells and a loss of outer hair cells were found in the cochlea of the second infant. The vestibular organs of both infants showed cytomegalic cells, mostly located on dark cells. There was a loss of type I and type II hair cells in the macula of the saccule and utricle. Loss of hair cells and degeneration of nerve fibers was also seen in the semicircular canals. Both infants with congenital infection showed abundant inflammatory cells and fibrous structures in the middle ear cavity. No evidence of cytomegalic cells and hair cell loss was found in the cochlea or vestibular labyrinth in acquired CMV infection.ConclusionsIn two infants with congenital CMV infection, the cochlea, vestibule, and middle ear were highly affected. Temporal bones of adult donors with acquired viral infection showed histological findings similar to donors of the same age without ear disease.     

Otitis media (silent): A potential cause of childhood meningitis

Sixteen temporal bones from 8 infants with otitis media, who died of meningitis, and 6 controls from infants with only otitis media, were studied histologically. All bones contained middle ear effusion and residual mesenchyme, but, unlike the controls, the meningitic cases showed considerable histopathological tissue changes of chronic and acute otitis media and chronic inflammatory cells in the round window membrane and within the perilymph, the modiolus, and the cochlear aqueduct, suggesting the latter as likely portals from the inner ear to the meninges. Since all tympanic membranes were intact and 3 were histologically normal, this silent route of infection warrants medical vigilance.     

Extralabyrinthine Manifestations of DFNA9

DFNA9 is an autosomal dominant cause of non-syndromic adult-onset sensorineural hearing loss with associated variable vestibular dysfunction caused by mutations in the COCH gene. DFNA9 has previously been characterized by the presence of unique histopathologic features limited to the cochlear and vestibular labyrinth. This report describes newly discovered extralabyrinthine findings within the middle ear in DFNA9 and discusses their implications. The histopathologic anatomy of extralabyrinthine structures was reviewed in 12 temporal bones from seven individuals with DFNA9 and compared with age-matched controls. All temporal bones with DFNA9 had abnormal deposits within the tympanic membrane, incudomalleal joint, and incudostapedial joint. Hematoxylin and eosin stain and Movat’s pentachrome stain both revealed different staining patterns of the extralabyrinthine deposits compared with the intralabyrinthine deposits suggesting that the composition of the deposits varies with location. The deposits within the tympanic membrane resembled cartilage morphologically and stained positively for aggrecan, an extracellular matrix protein found in cartilage. However, the cellular component of the tympanic membrane deposits did not stain with immunomarkers for chondrocytes (s100 and connective tissue growth factor). These novel findings in DFNA9 have implications for the phenotypic expression of the disorder and the clinical workup of adult-onset sensorineural hearing loss.     

Temporal bone histopathology in chronically infected ears with intact and perforated tympanic membranes.

Chronic suppurative otitis media has been clinically defined as a chronic discharge from the middle ear in the presence of a perforation of the tympanic membrane. However, irreversible tissue pathology in the middle ear or mastoid can occur behind an intact tympanic membrane. One hundred forty-four human temporal bones with chronic otitis media were divided into two groups: those with perforated (28) and those with nonperforated (116) tympanic membranes. The histopathological findings of their middle ears were compared. Granulation tissue in various degrees was the most prominent pathological feature. It was observed in 96% of temporal bones with perforation of the tympanic membrane, and in 97% of those without perforation. Also found were ossicular bony changes (96% with perforation; 90.5% without), middle ear effusion (93% with perforation; 89% without), cholesterol granuloma (21% with perforation; 12% without), cholesteatoma (36% with perforation; 4% without), and tympanosclerosis (43% with perforation; 20% without). This study shows that the histopathological changes of the middle ear are similar in temporal bones with and without perforation of the tympanic membrane. The clinician should, therefore, be aware that an intact tympanic membrane does not necessarily preclude the presence of gross pathological changes of the middle ear cleft.     

Cochlear aqueduct

The morphology and structure within the cochlear aqueduct have been investigated on the basis of a histological analysis of 234 temporal bones ranging from 12-week old to adult. Barrier membrane was found in 28.6% of the temporal bones examined, and fibrous reticular tissues of the inner opening of the cochlear aqueduct in 38.1% of the temporal bones. No any tissue in the inner opening of the cochlear aqueduct was seen in 33.3%. Periotic duct was filled with loose reticular tissue. The clinical significance of contents in the cochlear aqueduct was discussed.     

Obliteration of vestibular and cochlear aqueducts in the guinea pig

The study involved 42 healthy adult guinea pigs placed into two major groups. In group A, only histologic studies were performed after obliteration of the right vestibular aqueduct, the cochlear aqueduct, or both the vestibular and cochlear aqueducts. In group B, hearing changes were monitored by repeated determination of averaged temporal response thresholds after the same obliterative operations as in group A. At the conclusion of the experiments, histologic studies of temporal bone were performed and hearing changes were correlated with the severity of disease found in the inner ear. Histologic examination of 32 temporal bones from animals in which the vestibular aqueduct or both the vestibular and cochlear aqueducts were obliterated consistently demonstrated endolymphatic hydrops. The endolymphatic hydrops was usually progressive, and its severity was proportional to the length of time from the operation. Serial determination of averaged temporal response thresholds in 18 guinea pigs after these operations demonstrated a progressive deterioration in thresholds. The hearing loss was greater at the lower frequencies. The audiometric changes correlated positively with the severity of endolymphatic hydrops. No significant audiometric or temporal bone histologic changes were found after obliteration of the cochlear aqueduct.     

Computed tomography of the temporal bone in diagnosis of chronic exudative otitis media

Computed tomography (CT) of the temporal bone was made in 37 patients aged 2 to 55 years with chronic exudative otitis media (CEOM). In 21 of them the pathology was bilateral. The analysis of 58 CT images has identified CT signs of chronic exudative otitis media. They include partial (17 temporary bones) or complete (38 temporal bones) block of the bone opening of the auditory tube, pneumatic defects of the tympanic cavity (58 temporal bones), pneumatic defects of the mastoid process and antrum (47 temporal bones), pathologic retraction of the tympanic membrane. The examination of the temporal bone detected both CT-signs of CEOM and other causes of hearing disorders in 14 patients (26 temporal bones) with CEOM symptoms and inadequately high hypoacusis. Among these causes were malformation of the auditory ossicula (n=5), malformation of the labynthine window (n=2), malformation of the middle and internal ear (n=4), a wide aqueduct of the vestibule, labyrinthine anomaly of Mondini's type (n=1), cochlear hypoplasia (n=4), stenosis of the internal acoustic meatuses (n=2). Sclerotic fibrous dysplasia was suggested in 2 temporal bones (by CT data). CT was repeated after surgical treatment of 10 patients (14 temporal bones) and visual assessment of tympanostomy results was made.     

Inner ear pathology associated with Reye's syndrome

Severe pathological changes were observed in the inner ear tissues of a 2-month-old patient who died of Reye's syndrome after 5 days of hospitalization. In the organ of Corti, the inner hair cells appeared to be more severely damaged than the outer hair cells. Various degrees of degeneration were observed in all non-sensory epithelial cells lining the cochlear duct. In most turns of the cochlear duct, Reissner's membrane was ruptured and/or collapsed onto the organ of Corti. Likewise, both sensory and non-sensory cells of the vestibular end organs were markedly degenerated. These observations suggest that the inner ear tissues are acutely affected in patients with Reye's syndrome, and that the changes may cause impairment of hearing and/or equilibrium in patients who recover.     

Application of multiplanar reconstruction of spiral CT in the diagnosis and treatment of enlarged vestibular aqueducts

Background: Multiplanar reconstruction (MPR) of High Resolution Computed Tomography (HRCT) makes it possible to achieve a clear view of inner ear structures. However, no agreement was reached on the standard measurement of inner ear.

Objectives: To establish standard inner ear measurements for building criteria for cochlear structure evaluation.

Material and Methods: HRCT scanning of the temporal bones of 82 cases with normal inner ear structures and 104 cases with an EVA and bilaterally sensorineural hearing loss (SNHL) was performed. Three standard cochlear plane and one vestibular plane were reconstructed by MPR.

Results: Normative data of inner ear was measured and formulated. The most common malformation found in cases with EVA was incomplete partition type II (IP-II; 90.4%). The IP-II group had significantly greater modiolar height, cochlear aperture width, vestibular area, and vestibule width than did the control group. Different degrees of IP-II modiolar defects were observed using MPR.

Conclusions and Significance: Standard cochlear plane can help us to evaluate the cochlear structure. The MPR standard measurement of inner ear is clinically valuable for the diagnosis and cochlear implant of EVA.     

An experimental study of inner ear pathology due to NaCl on the round window

An animal experiment was performed to evaluate structural and ultrastructural changes to the inner ear as a result of placing 3-4 crystals of reagent grade sodium chloride (NaCl) on the round window membrane. Chinchillas were sacrificed at 8 and 24 hours after treatment and the cochlear and vestibular tissues were examined by light microscopy and scanning electron microscopy. Inner ear pathology consisted of destruction of both sensory and supporting cells in the basal turn of the organ of Corti, atrophy of the stria vascularis and alterations to the otoconia and the maculae and ampullae of the vestibular system. This study demonstrates that NaCl in the middle ear does not provide a model for Meniere's disease as previously suggested by Arslan. It may, however, be utilized in the destructive treatment of selected inner ear disorders.     

Paget's disease and the temporal bone--a clinical and histopathological review of six temporal bones

Paget's disease of bone occurs more commonly in the elderly and has been reported to involve the temporal bone in 30% of those afflicted. The clinical and histopathological features of six temporal bones from three patients with this disease are reported and the relevant literature reviewed. The effect of Paget's disease on the middle ear structures was more variable than its extension into the otic capsule. Pagetic involvement of the otic capsule was observed in five temporal bones. One patient had bilateral asymptomatic neurofibromas in the eighth cranial nerve. the potential mechanisms responsible for the conductive deafness, the sensorineural deafness and vestibular dysfunction associated with Paget's disease are discussed.     

The pathology of silent otitis media: A predecessor to tympanogenic meningitis in infants

Objective

To determine the association of bacteria embedded within a fibrous matrix in the middle and inner ear in infants with tympanogenic meningitis.

Methods

Thirty-one cases with meningitis from the human temporal bone collection at the University of Minnesota were screened to select those with tympanogenic meningitis. Inclusion criteria for tympanogenic meningitis were acute meningitis with histopathological evidence of chronic otitis media, and no other source of infection. The presence of labyrinthitis and pathologic changes such as granulation tissue, fibrosis, cholesterol granuloma, cholesteatoma, tympanic membrane perforation, tympanosclerosis, and the type of effusion were noted. The extent and location of bacteria embedded in a fibrous matrix were also explored.

Results

Seventeen temporal bones, from nine cases that included two females and seven males, ranging in age from five to twenty-three months, met our criteria of tympanogenic meningitis. Eighty two percent of these temporal bones had bacteria within the fibrous matrices (BFM). BFM were located in one anatomical region in one temporal bone and multiple anatomic regions in sixteen temporal bones. The most common locations were the areas near the oval and round windows. They were also commonly seen in the epitympanum, facial recess, and supratubal recess. BFM within the inner ear were observed in the scala tympani and modiolus in the middle and basal turns of the cochleae of nine temporal bones. In one of these temporal bones, BFM were seen in the internal auditory canal. Labyrinthitis was seen in all ears. The tympanic membrane was intact in all cases. BFM were not seen in three temporal bones from two patients. In one case only one side was available for study.

Conclusions

Our findings show an association between the presence of BFM in the ear with chronic pathologic changes and tympanogenic meningitis. Potential pathways of bacteria from the middle ear include hematogeous spread and/or direct spread to dura through the tympanic tegmen, and/or to the inner ear through the oval and round windows, and from there to the modiolus and the meninges. Chronic pathologic changes in the middle ear behind an intact tympanic membrane and the lack of ear symptoms may result in potentially serious sequelae and complications in infant age groups. There should be a heightened awareness of this condition.