Paget disease of the temporal bone.
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Aged, 80 and over↗
Biomedical subjects
Saumil N Merchant
Publications and source records attributed to Saumil N Merchant.
A downloadable three-dimensional virtual model of the visible ear.
PURPOSE: To develop a three-dimensional (3-D) virtual model of a human temporal bone and surrounding structures. METHODS: A fresh-frozen human temporal bone was serially sectioned and digital images of the surface of the tissue block were recorded (the 'Visible Ear'). The image stack was resampled at a final resolution of 50 x 50 x 50/100 micro m/voxel, registered in custom software and segmented in PhotoShop 7.0. The segmented image layers were imported into Amira 3.1 to generate smooth polygonal surface models. RESULTS: The 3-D virtual model presents the structures of the middle, inner and outer ears in their surgically relevant surroundings. It is packaged within a cross-platform freeware, which allows for full rotation, visibility and transparency control, as well as the ability to slice the 3-D model open at any section. The appropriate raw image can be superimposed on the cleavage plane. The model can be downloaded at: (https://research.meei.harvard.edu/Otopathology/3dmodels/).
Aged, 80 and over↗
The effect of methodological differences in the measurement of stapes motion in live and cadaver ears.
Methodological differences in sound-induced stapes velocity (Vs) measurements in live and cadaveric ears were examined using fresh cadaveric temporal bones. On average, differences in preparation (sectioning the stapedius tendon, removing the facial nerve, and widely opening the facial recess) had statistically insignificant effects on measured Vs. Differences in the achievable measurement angle (with respect to the axis of piston-like stapes motion) had a significant effect on measured Vs below 2 kHz. These results suggest that much if not all of the differences in Vs measurements between live and cadaveric ears can be explained by the differences in measurement angle between the two preparations. Measurement angle was found to have minimal effect on measured Vs above 2 kHz. This demonstrates that the commonly used method of estimating stapes translational velocity by dividing the measured velocity by the cosine of the measurement angle is not valid above 2 kHz.
Aged↗
Cochlin immunostaining of inner ear pathologic deposits and proteomic analysis in DFNA9 deafness and vestibular dysfunction.
Seven missense mutations and one in-frame deletion mutation have been reported in the coagulation factor C homology (COCH) gene, causing the adult-onset, progressive sensorineural hearing loss and vestibular disorder at the DFNA9 locus. Prevalence of COCH mutations worldwide is unknown, as there is no systematic screening effort for late-onset hearing disorders; however, to date, COCH mutations have been found on four continents and the possibility of COCH playing an important role in presbycusis and disorders of imbalance has been considered. Cochlin (encoded by COCH) has also been shown as a major target antigen for autoimmune sensorineural hearing loss. In this report, we present histopathology, immunohistochemistry and proteomic analyses of inner ear tissues from post-mortem DFNA9 temporal bone samples of an individual from a large Dutch kindred segregating the P51S mutation and adult human unaffected controls, and wild-type (+/+) and Coch null (-/-) knock-out mice. DFNA9 is an inner ear disorder with a unique histopathology showing loss of cellularity and aggregation of abundant homogeneous acellular eosinophilic deposits in the cochlear and vestibular labyrinths, similar to protein aggregation in well-known neurodegenerative disorders. By immunohistochemistry on the DFNA9 temporal bone sections, we have shown cochlin staining of the characteristic cochlear and vestibular deposits, indicating aggregation of cochlin in the same structures in which it is normally expressed. Proteomic analysis identified cochlin as the most abundant protein in mouse and human cochleae. The high-level expression and stability of cochlin in the inner ear, even in the absence and severe atrophy of the fibrocytes that normally express COCH, are shown through these studies and further elucidate the pathobiologic events occurring in DFNA9 leading to hearing loss and vestibular dysfunction.
Adult↗
Determinants of hearing loss in perforations of the tympanic membrane.
BACKGROUND: Although tympanic membrane perforations are common, there have been few systematic studies of the structural features determining the magnitude of the resulting conductive hearing loss. Our recent experimental and modeling studies predicted that the conductive hearing loss will increase with increasing perforation size, be independent of perforation location (contrary to popular otologic belief), and increase with decreasing size of the middle-ear and mastoid air space (an idea new to otology). OBJECTIVE: To test our predictions regarding determinants of conductive hearing loss in tympanic membrane perforations against clinical data gathered from patients. STUDY DESIGN: Prospective clinical study. SETTING: Tertiary referral center. INCLUSION CRITERIA: Patients with tympanic membrane perforations without other middle-ear disease. MAIN OUTCOME MEASURES: Size and location of perforation; air-bone gap at 250, 500, 1,000, 2,000, and 4,000 Hz; and tympanometric estimate of volume of the middle-ear air spaces. RESULTS: Isolated tympanic membrane perforations in 62 ears from 56 patients met inclusion criteria. Air-bone gaps were largest at the lower frequencies and decreased as frequency increased. Air-bone gaps increased with perforation size at each frequency. Ears with small middle-ear volumes, < or = 4.3 ml (n = 23), had significantly larger air-bone gaps than ears with large middle-ear volumes, > 4.3 ml (n = 39), except at 2,000 Hz. The mean air-bone gaps in ears with small volumes were 10 to 20 dB larger than in ears with large volumes. Perforations in anterior versus posterior quadrants showed no significant differences in air-bone gaps at any frequency, although anterior perforations had, on average, air-bone gaps that were smaller by 1 to 8 dB at lower frequencies. CONCLUSION: The conductive hearing loss resulting from a tympanic membrane perforation is frequency-dependent, with the largest losses occurring at the lowest sound frequencies; increases as size of the perforation increases; varies inversely with volume of the middle-ear and mastoid air space (losses are larger in ears with small volumes); and does not vary appreciably with location of the perforation. Effects of location, if any, are small.
Acoustic Impedance Tests↗
Three-dimensional virtual model of the human temporal bone: a stand-alone, downloadable teaching tool.
OBJECTIVE: To develop a three-dimensional virtual model of a human temporal bone based on serial histologic sections. BACKGROUND: The three-dimensional anatomy of the human temporal bone is complex, and learning it is a challenge for students in basic science and in clinical medicine. METHODS: Every fifth histologic section from a normal 14-year-old male was digitized and imported into a general purpose three-dimensional rendering and analysis software package called Amira (version 3.1). The sections were aligned, and anatomic structures of interest were segmented. RESULTS: The three-dimensional model is a surface rendering of these structures of interest, which currently includes the bone and air spaces of the temporal bone; the perilymph and endolymph spaces; the sensory epithelia of the cochlear and vestibular labyrinths; the ossicles and tympanic membrane; the middle ear muscles; the carotid artery; and the cochlear, vestibular, and facial nerves. For each structure, the surface transparency can be individually controlled, thereby revealing the three-dimensional relations between surface landmarks and underlying structures. The three-dimensional surface model can also be "sliced open" at any section and the appropriate raw histologic image superimposed on the cleavage plane. The image stack can also be resectioned in any arbitrary plane. CONCLUSION: This model is a powerful teaching tool for learning the complex anatomy of the human temporal bone and for relating the two-dimensional morphology seen in a histologic section to the three-dimensional anatomy. The model can be downloaded from the Eaton-Peabody Laboratory web site, packaged within a cross-platform freeware three-dimensional viewer, which allows full rotation and transparency control.
Adolescent↗
Osteoprotegrin knockout mice demonstrate abnormal remodeling of the otic capsule and progressive hearing loss.
OBJECTIVES: The otic capsule, when compared with other bones in the body, is unique in that it undergoes no significant remodeling of bone after development. We previously demonstrated that osteoprotegerin (OPG), which inhibits formation and function of osteoclasts, is produced at high levels in the inner ear of normal mice and secreted into the perilymph from where it diffuses into the surrounding otic capsule bone through a lacunocanalicular system. To test our hypothesis that the high level of OPG may be important in the inhibition of otic capsule remodeling, we studied the light microscopic histology of the otic capsule in OPG knockout mice for evidence of abnormal remodeling of bone. We also tested the hearing in OPG knockout mice to determine whether OPG and its influence on surrounding bone is important for auditory function. METHODS: Temporal bone histopathology and pathophysiology were compared in homozygous OPG knockout mice and C57BL/6 (B6) mice, the background strain for the knockouts. Auditory function in age-matched animals from each group was evaluated at approximately 4-week intervals from 8 to 21 weeks using frequency-specific auditory brainstem responses (ABR) and distortion product otoacoustic emissions (DPOAE). After each of the last three evaluations, the cochleae from one mouse of each group were harvested, processed, and examined by light microscopy. RESULTS: Osteoprotegerin knockout mice demonstrated abnormal remodeling of bone within the otic capsule with multiple foci showing osteoclastic bone resorption and formation of new bone. Such changes were not seen in the age-matched B6 controls. The active bone remodeling process in the knockout animals showed many similarities to otosclerosis seen in human temporal bones. Over the time period that we monitored, auditory function was significantly and progressively compromised in the knockout animals relative to B6 controls. At the earliest age of test (8 wk), the loss was apparent as a mild, high-frequency reduction in sensitivity by ABR. In contrast, DPOAE losses in the knockouts were substantial even at 8 weeks, and by 21 weeks, these losses exceeded our equipment limits. Results of ABR testing showed hearing sensitivity changes in the animals of the background strain were confined largely to the high frequencies, whereas OPG knockouts demonstrated substantial low-frequency shifts in addition to those at high frequencies. CONCLUSIONS: The histopathological and pathophysiological findings in OPG knockout mice support the hypothesis that OPG is important in the inhibition of bone remodeling within the otic capsule and the maintenance of normal auditory function. This mouse may provide a valuable animal model of human otosclerosis.
Acoustic Stimulation↗
Polyester wax: a new embedding medium for the histopathologic study of human temporal bones.
BACKGROUND: Celloidin and paraffin are the two common embedding mediums used for histopathologic study of the human temporal bone by light microscopy. Although celloidin embedding permits excellent morphologic assessment, celloidin is difficult to remove, and there are significant restrictions on success with immunostaining. Embedding in paraffin allows immunostaining to be performed, but preservation of cellular detail within the membranous labyrinth is relatively poor. OBJECTIVES/HYPOTHESIS: Polyester wax is an embedding medium that has a low melting point (37 degrees C), is soluble in most organic solvents, is water tolerant, and sections easily. We hypothesized that embedding in polyester wax would permit good preservation of the morphology of the membranous labyrinth and, at the same time, allow the study of proteins by immunostaining. METHODS: Nine temporal bones from individuals aged 1 to 94 years removed 2 to 31 hours postmortem, from subjects who had no history of otologic disease, were used. The bones were fixed using 10% formalin, decalcified using EDTA, embedded in polyester wax, and serially sectioned at a thickness of 8 to 12 mum on a rotary microtome. The block and knife were cooled with frozen CO2 (dry ice) held in a funnel above the block. Sections were placed on glass slides coated with a solution of 1% fish gelatin and 1% bovine albumin, followed by staining of selected sections with hematoxylin and eosin (H&E). Immunostaining was also performed on selected sections using antibodies to 200 kD neurofilament and Na-K-ATPase. RESULTS: Polyester wax-embedded sections demonstrated good preservation of cellular detail of the organ of Corti and other structures of the membranous labyrinth, as well as the surrounding otic capsule. The protocol described in this paper was reliable and consistently yielded sections of good quality. Immunostaining was successful with both antibodies. CONCLUSION: The use of polyester wax as an embedding medium for human temporal bones offers the advantage of good preservation of morphology and ease of immunostaining. We anticipate that in the future, polyester wax embedding will also permit other molecular biologic assays on temporal bone sections such as the retrieval of nucleic acids and the study of proteins using mass spectrometry-based proteomic analysis.
Adolescent↗
Vestibular evoked myogenic potentials (VEMP) can detect asymptomatic saccular hydrops.
OBJECTIVE: The objective of this study was to explore the useful of vestibular evoked myogenic potential (VEMP) testing for detecting endolymphatic hydrops, especially in the second ear of patients with unilateral Ménière disease (MD). METHODS: This study was performed at a tertiary care academic medical center. Part I consisted of postmortem temporal bone specimens from the temporal bone collection of the Massachusetts Eye & Ear Infirmary; part II consisted of consecutive consenting adult patients (n = 82) with unilateral MD by American Academy of Otolaryngology-Head and Neck Surgery criteria case histories. Outcome measures consisted of VEMP thresholds in patients and histologic saccular endolymphatic hydrops in postmortem temporal bones. RESULTS: Saccular hydrops was observed in the asymptomatic ear in six of 17 (35%) of temporal bones from donors with unilateral MD. Clinic patients with unilateral MD showed elevated mean VEMP thresholds and altered VEMP tuning in their symptomatic ears and, to a lesser degree, in their asymptomatic ears. Specific VEMP frequency and tuning criteria were used to define a "Ménière-like" response. This "Ménière-like" response was seen in 27% of asymptomatic ears of our patients with unilateral MD. CONCLUSIONS: Bilateral involvement is seen in approximately one third of MD cases. Saccular hydrops appears to precede symptoms in bilateral MD. Changes in VEMP threshold and tuning appear to be sensitive to these structural changes in the saccule. If so, then VEMP may be useful as a detector of asymptomatic saccular hydrops and as a predictor of evolving bilateral MD.
Acoustic Stimulation↗
Cochleosaccular dysplasia associated with a connexin 26 mutation in keratitis-ichthyosis-deafness syndrome.
OBJECTIVE: The objective of this study was to characterize the temporal bone phenotype associated with a mutation of GJB2 (encoding connexin 26). STUDY DESIGN: The authors conducted correlative clinical, molecular genetic, and postmortem histopathologic analysis. METHODS: The study subject was a male infant with keratitis-ichthyosis-deafness (KID) syndrome. We performed a nucleotide sequence analysis of GJB2 and a histopathologic analysis of the temporal bones. RESULTS: The subject was heterozygous for G45E, a previously reported KID syndrome mutation of GJB2. The primary inner ear abnormality was dysplasia of the cochlear and saccular neuroepithelium. CONCLUSIONS: GJB2 mutations can cause deafness in KID syndrome, and possibly in other GJB2 mutant phenotypes, by disrupting cochlear differentiation.
Cochlea↗
Results after revision stapedectomy with malleus grip prosthesis.
Revision stapedectomy with a malleus grip prosthesis is a technically challenging otologic procedure. The prosthesis is usually longer and extends deeper into the vestibule than a conventional stapes prosthesis, creating the potential to affect the vestibular sense organs. The prosthesis also bypasses the ossicular joints, which are thought to play a role in protecting the inner ear from large changes in static pressure within the middle ear. The prosthesis is in close proximity to the tympanic membrane, thus increasing the risk for its extrusion. We reviewed our experience with revision stapedectomy with the Schuknecht Teflon-wire malleus grip prosthesis in 36 ears with a mean follow-up of 23 months. The air-bone gap was closed to within 10 dB in 16 ears (44%) and to within 20 dB in 26 ears (72%). The incidence of postoperative sensorineural hearing loss was 8% (3 ears). There were no dead ears. Extrusion of the prosthesis occurred in 1 case (3%). Nearly 50% of patients reported various degrees of vertigo or disequilibrium during the first 3 weeks after surgery. These vestibular symptoms resolved by 6 weeks in all but 1 case. We did not find evidence of damage to the inner ear due to the length of the prosthesis or due to the potential for direct transmission of changes in static pressures within the middle ear to the labyrinth. Our results are similar to those published in the literature for malleus attachment stapedectomy and conventional revision incus stapedectomy.
Adult↗
Cockayne syndrome in adults: review with clinical and pathologic study of a new case.
Cockayne syndrome and xeroderma pigmentosum-Cockayne syndrome complex are rare autosomal recessive disorders with poorly understood biology. They are characterized by profound postnatal brain and somatic growth failure and by degeneration of multiple tissues resulting in cachexia, dementia, and premature aging. They result in premature death, usually in childhood, exceptionally in adults. This study compares the clinical course and pathology of a man with Cockayne syndrome group A who died at age 31(1/2) years with 15 adequately documented other adults with Cockayne syndrome and 5 with xeroderma pigmentosum-Cockayne syndrome complex. Slowing of head and somatic growth was apparent before age 2 years, mental retardation and slowly progressive spasticity at 4 years, ataxia and hearing loss at 9 years, visual impairment at 14 years, typical Cockayne facies at 17 years, and cachexia and dementia in his twenties, with a retained outgoing personality. He experienced several transient right and left hemipareses and two episodes of status epilepticus following falls. Neuropathology disclosed profound microencephaly, bilateral old subdural hematomas, white-matter atrophy, tigroid leukodystrophy with string vessels, oligodendrocyte proliferation, bizarre reactive astrocytes, multifocal dystrophic calcification that was most marked in the basal ganglia, advanced atherosclerosis, mixed demyelinating and axonal neuropathy, and neurogenic muscular atrophy. Cellular degeneration of the organ of Corti, spiral and vestibular ganglia, and all chambers of the eye was severe. Rarely, and for unexplained reasons, in some patients with Cockayne syndrome the course is slower than usual, resulting in survival into adulthood. The profound dwarfing, failure of brain growth, cachexia, selectivity of tissue degeneration, and poor correlation between genotypes and phenotypes are not understood. Deficient repair of DNA can increase vulnerability to oxidative stress and play a role in the premature aging, but why patients with mutations in xeroderma pigmentosum genes present with the Cockayne syndrome phenotype is still not known.
Adult↗
Distribution of type IV collagen in the cochlea in Alport syndrome.
OBJECTIVE: To determine the distribution of alpha1, alpha3, and alpha5 chains of type IV collagen in the cochlea in Alport syndrome. DESIGN: Case-control study. PATIENTS: Two patients with sensorineural hearing loss due to Alport syndrome. Both patients had known mutations in the COL4A5 gene. MAIN OUTCOME MEASURES: Immunostaining was used to study the distribution of type IV collagen (alpha1, alpha3, and alpha5 chains) within the cochlea. Immunostaining was also performed in the cochlear tissues of an unaffected individual used as a control. RESULTS: In the control ear, alpha1 staining was observed in the basement membrane overlying the basilar membrane, in the basement membrane of cochlear blood vessels and Schwann cells, and within the spiral limbus. In the control ear, we also observed strong staining for alpha3 and alpha5 chains in the basement membrane overlying the basilar membrane and within the spiral ligament. In both cases with Alport syndrome, no immunostaining was observed for alpha3 or alpha5 chains within the cochlea, whereas alpha1 staining was present in locations similar to that seen in the control ear. CONCLUSIONS: The results indicate that isotype switching does not occur within the cochlea in Alport syndrome. The results are also consistent with the hypothesis that the sensorineural hearing loss in Alport syndrome may be due to alterations in cochlear micromechanics and/or dysfunction of the spiral ligament.
Adult↗
Experimental ossicular fixations and the middle ear's response to sound: evidence for a flexible ossicular chain.
A human temporal-bone preparation was used to determine the effects of various degrees of artificial ossicular fixation on the sound-induced velocity at the input-side (the umbo of the malleus) and the output-side (the stapes) of the ossicular chain. Construction of various degrees of attachment between an ossicle and the surrounding temporal bone provided a range of reduction in ossicular mobility or "fixations". The results demonstrate different effects of the fixations on the umbo and stapes velocity: fixations of the stapes or incus produce larger reductions in sound-induced stapes velocity (as much as 40-50 dB with extensive stapes fixation), than reductions in umbo velocity (typically less than 10 dB). Fixations of the malleus produce similar-sized changes in both umbo and stapes velocity. These differential effects are consistent with significant flexibility in the ossicular joints (the incudo-malleolar joint and the incudo-stapedial joint) that permits relative motion between the coupled ossicles. The existence of flexibility in the ossicular joints indicates that joints in the ossicular chain can effect a loss of sound-induced mechanical energy between the umbo and the stapes, with a concomitant reduction in the sound-induced motion of the stapes. The introduction of such losses in sound transmission by the joints raises questions concerning the utility of three ossicles in the mammalian ear. The consequences of ossicular flexibility to ossicular-chain reconstruction is discussed. Also, as examined in a more clinically directed paper [Laryngoscope 115 (2005) 147], the different effects of the various ossicular fixations on the motion of the umbo and malleus may be useful in the diagnosis of the site of fixations in humans with conductive hearing losses caused by such pathologies.
Aged↗
Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic hydrops?
BACKGROUND: The association of Meniere's syndrome with endolymphatic hydrops has led to the formation of a central hypothesis: many possible etiologic factors lead to hydrops, and hydrops in turn generates the symptoms. However, this hypothesis of hydrops as being the final common pathway has not been proven conclusively. SPECIFIC AIM: To examine human temporal bones with respect to the role of hydrops in causing symptoms in Meniere's syndrome. If the central hypothesis were true, every case of Meniere's syndrome should have hydrops and every case of hydrops should show the typical symptoms. METHODS: Review of archival temporal bone cases with a clinical diagnosis of Meniere's syndrome (28 cases) or a histopathologic diagnosis of hydrops (79 cases). RESULTS: All 28 cases with classical symptoms of Meniere's syndrome showed hydrops in at least one ear. However, the reverse was not true. There were 9 cases with idiopathic hydrops and 10 cases with secondary hydrops, but the patients did not exhibit the classic symptoms of Meniere's syndrome. A review of the literature revealed cases with asymptomatic hydrops (similar to the current study), as well as cases where symptoms of Meniere's syndrome existed during life but no hydrops was observed on histology. We also review recent experimental data where obstruction of the endolymphatic duct in guinea pigs resulted in cytochemical abnormalities within fibrocytes of the spiral ligament before development of hydrops. This result is consistent with the hypothesis that hydrops resulted from disordered fluid homeostasis caused by disruption of regulatory elements within the spiral ligament. CONCLUSION: Endolymphatic hydrops should be considered as a histologic marker for Meniere's syndrome rather than being directly responsible for its symptoms.
Adult↗
Pathology and pathophysiology of idiopathic sudden sensorineural hearing loss.
BACKGROUND: The cause and pathogenesis of idiopathic sudden sensorineural hearing loss remain unknown. Proposed theories include vascular occlusion, membrane breaks, and viral cochleitis. AIMS: To describe the temporal bone histopathology in 17 ears (aged 45-94 yr) with idiopathic sudden sensorineural hearing loss in our temporal bone collection and to discuss the implications of the histopathologic findings with respect to the pathophysiology of idiopathic sudden sensorineural hearing loss. METHODS: Standard light microscopy using hematoxylin and eosin-stained sections was used to assess the otologic abnormalities. RESULTS: Hearing had recovered in two ears and no histologic correlates were found for the hearing loss in both ears. In the remaining 15 ears, the predominant abnormalities were as follows: 1) loss of hair cells and supporting cells of the organ of Corti (with or without atrophy of the tectorial membrane, stria vascularis, spiral limbus, and cochlear neurons) (13 ears); 2) loss of the tectorial membrane, supporting cells, and stria vascularis (1 ear); and 3) loss of cochlear neurons only (1 ear). Evidence of a possible vascular cause for the idiopathic sudden sensorineural hearing loss was observed in only one ear. No membrane breaks were observed in any ear. Only 1 of the 17 temporal bones was acquired acutely during idiopathic sudden sensorineural hearing loss, and this ear did not demonstrate any leukocytic invasion, hypervascularity, or hemorrhage within the labyrinth, as might be expected with a viral cochleitis. DISCUSSION: The temporal bone findings do not support the concept of membrane breaks, perilymphatic fistulae, or vascular occlusion as common causes for idiopathic sudden sensorineural hearing loss. The finding in our one case acquired acutely during idiopathic sudden sensorineural hearing loss as well as other clinical and experimental observations do not strongly support the theory of viral cochleitis. CONCLUSION: We put forth the hypothesis that idiopathic sudden sensorineural hearing loss may be the result of pathologic activation of cellular stress pathways involving nuclear factor-kappaB within the cochlea.
Adult↗
Experimental and clinical studies of malleus fixation.
OBJECTIVES/HYPOTHESIS: Preoperative clinical diagnosis of malleus fixation can be difficult. "Fixation" of the malleus can be caused by various disorders or diseases: fibrous tissue, bony spurs, and neo-osteogenesis around the malleus head or stiffening of the anterior malleal ligament. The conductive hearing loss produced by these disorders or diseases has not been well characterized. The study goals were 1) to determine the effects of various types of malleus fixation using a cadaveric temporal bone preparation and 2) to assess the clinical utility of umbo velocity measurements in preoperative differential diagnosis of malleus fixation and stapes fixation. METHODS: Umbo and stapes velocity were measured in 18 fresh cadaveric human temporal bones with laser vibrometry before and after controlled application of adhesives to the malleus, stapes, or both ossicles. RESULTS: Each simulated pathological condition produced a specific degree of loss in stapes velocity: stiffening of anterior malleal ligament, 0 to 8 dB; fibrous tissue around malleus head, less than 10 dB; bony bar to malleus head, 10 to 30 dB; and extensive neo-osteogenesis around malleus head, greater than 35 dB. Simulated malleus fixations generally produced similar reductions in both umbo and stapes velocity. Stapes fixation reduced stapes velocity with little change in umbo velocity. Because the change in stapes velocity would be similar to conductive hearing loss, experimental results were directly compared with clinical measurements of umbo velocity in surgically confirmed cases of malleus or stapes fixation. The effects of malleus and stapes fixations between the clinical and experimental data were similar. CONCLUSION: The study showed that measurements of umbo velocity and air-bone gap can enable one to diagnose malleus fixation and specifies how to differentiate malleus from stapes fixation.
Aged↗
Osteoprotegerin in the inner ear may inhibit bone remodeling in the otic capsule.
OBJECTIVES: To elucidate factors that may be responsible for the inhibition of remodeling of bone within the otic capsule. METHODS: Expression of osteoprotegerin (OPG), receptor activator of nuclear factor kappa B (RANK), and RANK ligand (RANKL) were assayed in samples of bone obtained from the otic capsule, calvarium, and femur, and from the soft tissue within the cochlea using semiquantitative reverse transcriptase polymerase chain reaction (RT-PCR) in mice. Immunostaining was used for histologic localization of the gene products. An enzyme-linked immunosorbent assay (ELISA) was used to quantify the amount of OPG within perilymph, serum, and cerebrospinal fluid. The micro-anatomy of the interface between the otic capsule and the fluid spaces of the cochlea was investigated by brightfield and phase-contrast microscopy and by three-dimensional reconstruction in the mouse and human. RESULTS: OPG, a powerful inhibitor of bone remodeling, was expressed at extremely high levels within the soft tissue of the cochlea and was present in the perilymph at very high concentrations. The OPG produced within the inner ear may diffuse into the surrounding otic capsule, where it may be responsible for inhibition of bone turnover. Our anatomic studies revealed an extensive system of interconnected canaliculi within the otic capsule that had direct openings into the fluid spaces of the inner ear, thus providing a possible anatomic route for the diffusion of OPG from the inner ear into the surrounding bone. CONCLUSION: OPG, a potent inhibitor of osteoclast formation and function, is expressed at high levels within the inner ear and is secreted into the perilymph and the surrounding bone and may serve to inhibit active bone remodeling within the otic capsule, especially immediately adjacent to the cochlea. By this means, the cochlear soft tissue may control the nature of the surrounding petrous bone.
Animals↗