ANIMAL EXPERIMENTS IN BONE CONDUCTION: CLINICAL CONCLUSIONS.
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CT of the normal suspensory ligaments of the ossicles in the middle ear.
PURPOSE: To establish the range of normal variation in the CT appearance of the middle ear ligaments and the stapedius tendon as an aid in detecting abnormal changes in these structures. METHODS: CT scans of the temporal bone in 75 normal middle ears, obtained with 1-mm-thick sections, were reviewed by two observers, who rated the visibility of the structures of interest on a scale of 1 to 5. RESULTS: The anterior, superior, and lateral malleal ligaments and the medial and lateral parts of the posterior incudal ligament were seen in 68%, 46%, 95%, 26%, and 34% of the ears, respectively. The stapedius tendon was seen in 27% of the cases. When visible, the ligaments were judged to be complete in 90% to 100% of the ears and the stapedius tendon was complete in 65% of cases. Their width varied considerably. Interobserver variability was high for most observations. CONCLUSION: CT scans are more likely to show the malleal than the incudal ligaments. Although the interobserver agreement was statistically significant for most study parameters, the percentage of agreement above that expected by chance was low. When seen, the ligaments usually appeared complete. Understanding the normal range of appearance may help identify abnormalities of the ligaments and tendons of the middle ear.
Adolescent↗
Hereditary bilateral conductive hearing loss caused by total loss of ossicles: a report of familial expansile osteolysis.
OBJECTIVE: The objective of this study was to report on three members of a family with familial expansile osteolysis; the important point about these patients was that none of them had middle-ear ossicles. STUDY DESIGN AND SUBJECTS: A retrospective case review including three cases with familial expansile osteolysis. SETTING: Department of Otolaryngology in a tertiary referral center. INTERVENTIONS: Each patient underwent computerized tomography of the temporal bone in the coronal view, audiometric and tympanometric evaluations, biochemical investigation, whole body isotope scans by Tc-99 mMDP and X-ray. Also the patients' pedigree was studied. Two of the patients had exploratory middle-ear surgery as well. RESULTS: The temporal-bone computed-tomography scan in the coronal view of all three patients and also exploratory middle-ear surgery, which was done on two of the patients, showed no ossicles in the middle ear of either ear in all three cases. This feature hadn't been reported in previous studies. Hearing loss was revealed in the medical histories since childhood. Audiometry indicated mild to moderate conductive and mixed hearing loss and also an AD-type tympanogram pattern along with an absence of acoustic reflexes in both ears of the cases. Both serum alkaline phosphatase and hydroxyproline levels were elevated. There was an increase in uptake and activity at multiple foci of the whole skeleton. No improvement in hearing thresholds was obtained after reconstruction of the middle ear. CONCLUSION: The total absence of middle-ear ossicles can probably be regarded as a new symptom in some patients with familial expansile osteolysis. Common ossiculoplasty for improving the hearing thresholds in this condition may be unsuccessful; therefore, both surgeons and patients must be completely aware of the contingent undesirable results.
Adolescent↗
Determination of the shape and inertia properties of the human auditory ossicles.
This paper describes a method to accurately determine the shape of the human middle ear ossicles. If the density and the shape of a body are known, the inertia properties can be calculated using commercially available software. The inertia properties have been calculated for the stapes and for the incudomalleal unit. Different rectangular views of the stapes and the incudomalleal unit are shown to depict the position of the center of gravity and the position of the principal axes of inertia.
Ear Ossicles↗
[Development of the auditory ossicles in the human embryo: correlations with data obtained in mice].
The comparison of human middle ear ossicles developmental features with morphological and experimental data obtained in the mouse embryo reveals some points of convergence. The stapes develops mainly form Reichert's cartilage, but the malleus and incus derived partially from the first arch cartilage (Meckel's) and from a Reichert's cartilage-derived blastema parallel to the tubo-tympanic recess. These results permit to validate the mouse model in teratological studies.
Animals↗
Use of plastipore for ossicular chain reconstruction: an evaluation.
With the exception of stapes prostheses, previous use of implantable alloplastic materials in reconstructing the sound conducting mechanism in middle ear surgery has been uniformly unsuccessful. A new material composed of ultra-high molecular weight polyethylene has been developed as a replacement for middle ear ossicles. This implant, called the Plastipore total ossicular replacement prosthesis (TORP), and the Plastipore drum to the stapes prosthesis were inserted in 87 patients, all of whom had a long history of extensive middle ear disease and/or surgery. One TORP patient was lost to follow-up. The overall average hearing gain was 14.8 db. The overall follow-up was 12.4 months with a range of from 3 to 25 months. Eleven of the 15 patients receiving the drum-to-stapes prosthesis had hearing gain; 55 of 71 patients receiving the TORP had hearing gain. Eight prostheses were extruded. Nine patients had no improvement in hearing. Eight patients had subsequent decrease in hearing after initial gains. All ears are now dry and aerated. Early evaluation appears to demonstrate that the Plastipore prosthesis shows promise for use in difficult reconstructive middle ear problems.
Ear Diseases↗
Malleus fixed (ossified) to the tegmen tympani in an ancient skeleton in Israel.
A right malleus fixed to the roof of the tympanic cavity in a 1,600-year-old skull is described. The possible etiolgy of this condition is examined and the literature concerning recent and paleopathology of the middle ear ossicles is reviewed.
Adult↗
An empirical bound on the compressibility of the cochlea.
Effects of a possible inner-ear compressibility on middle-ear transfer functions are explored and a small upper bound on the magnitude of that compressibility established. Consequently. the traditional two-port representation of middle-ear mechanics remains valid to within a few percent. If the compressibility of the cochlea is small but finite, a simple phenomenological model of that compressibility correctly predicts hearing thresholds in the "middleless" ear at low frequencies. Experiments to establish the value of cochlear compressibility and to explore further its possible contributions to residual hearing in patients with missing or disarticulated middle-ear ossicles are suggested.
Animals↗
Ossicular erosion by cholesteatoma: investigation by scanning electron microscopy utilizing a new preparation technique.
A new technique for removing the soft tissues from middle ear ossicles to prepare them for scanning electron microscopy is described. The technique involved use of a solution of hypochlorite. The bony surface of normal ossicles was studied as a control group after preparation by the technique and no morphological distortion was observed. Ossicles eroded by cholesteatoma were then studied, and we propose that the erosion occurs in three stages; pumicing, pitting and cavitating. The mucoperiosteum of normal ossicles and otosclerotic foci were also studied.
Cholesteatoma↗
[A micromanipulator for intraoperative vibratory hearing assessment with an implantable hearing aid transducer].
First concepts of implantable hearing aids to be coupled to the ossicular chain are available for patients with combined or sensorineural hearing loss (SNHL). To ensure that hearing can be improved intraoperative coupling of a test transducer to the ossicular chain is mandatory for allowing surgical anatomy to be checked and vibratory hearing tests to be performed. To achieve this, the test transducer has to be held and positioned securely in situ for some minutes, avoiding risks for middle or inner ear structures. This is not possible using conventional surgical instruments. Thus, a micromanipulator to hold the test transducer during intraoperative hearing tests was developed. This surgical device allows the surgeon safe, risk-free, and controlled coupling of the test transducer to the ossicular chain with one axial and three rotational degrees of freedom. With the aid of a conventional ear retractor (2x2 prongs), the manipulator is fixed at the patient's ear. In conjunction with a piezoelectric test transducer, the manipulator was used in nine patients during local anesthesia. The test transducer is part of an electronic hearing implant (Tübingen implant) specifically designed for SNHL that may be coupled to a middle ear ossicle or the perilymph of the cochlea. The micromanipulator was easy to handle. It allowed accurate positioning of the test transducer in the ear and the desired coupling of the transducer's probe tip to the ossicular chain during auditory tests. According to the principles of integrated safety, the intraoperative risk of ossicular or inner ear injuries caused, for instance, by the patient's head movement is minimized. The design of the manipulator system is universal, also allowing its use for other electronic hearing implants or minimal invasive surgery after minor modifications.
Ear Ossicles↗
Middle ear pathology in ancient and modern populations: incudal osteoma.
CONCLUSIONS: Osteomas of the incus were found in surprisingly high numbers in ancient and modern populations. The coexistence of osteoma formation and unique vascularization on the medial surface of the incus alludes to a relationship between the two findings. OBJECTIVE: Osteomas involving the middle ear ossicles are rare. The aim of this study was to compare the incudal pathology of ancient and recent periods using the recent literature. MATERIAL AND METHODS: Pathologies encountered in 1170 human incudes belonging to different populations from ancient and recent periods were studied. Most of the ossicles were found in the middle ear of dry skulls or during dissection. The observations were done with a binocular microscope and a Nikon Profile Projector. RESULTS: A total of 47 incudes (4.01%) presented with a variety of pathologies, osteomas being found in 19 cases (1.62%). The osteomas were noted only on the medial surface of the incus. Other findings, including prominent erosions, multiple nutrient foramina and pitting, were also found on the medial side of the incus. The incidence of osteoma was found to be more common in ancient populations (1.88%) and most prevalent among Bedouins (4.55%).
Ear Neoplasms↗
Unilateral fixation of the ossicular chain associated with G-group chromosome deletion.
Unilateral fixation of the middle ear ossicles and possible delayed pubescence were associated with a short-arm deletion of one of the G-group chromosomes in a 15-year-old Negro girl. A similar chromosomal abnormality was found in the mother and three of six siblings without any clinical evidence of middle ear disease. The association of G-group deletions with other hereditary disease of bone suggests, however, that a pathogenic relationship may exist between them.
Adolescent↗
Correlation between loss of middle ear bones and altered goosecoid gene expression in the branchial region following retinoic acid treatment of mouse embryos in vivo.
The homeobox gene goosecoid marks the Spemann organizer in vertebrate gastrula embryos, and is expressed in the craniofacial region, body wall and limbs during organogenesis. Mouse mutants of goosecoid displayed a variety of phenotypes related to the expression pattern at mid-embryogenesis. These defects included loss of the tympanic ring and malformation of the malleus, phenotypes which were reminiscent of the teratogenic effects of retinoic acid (RA). Here we investigated the correlation of goosecoid gene expression and RA-teratogenicity following treatment of mouse embryos in vivo at embryonic day (E) 8 + 5 h. We found that goosecoid was specifically affected at E10.5 in branchial arches I and II. Expression was either reduced to background levels or restricted to the branchial cleft region. This change in goosecoid gene expression correlated with a loss of middle ear ossicles and a partial or complete deletion of the tympanic ring, suggesting a role for goosecoid in executing the RA teratogenic effects.
Animals↗
Experimental viral infections of the temporal bone. I. Experimental otitis media due to vaccinia virus in hamsters.
Clinical and pathologic studies of patients with smallpox suggest that conductive hearing loss is a frequent complication of smallpox and that it is due to otitis media. A possible experimental model for this hearing loss has been developed by study of the pathogenesis of acute vaccinia virus infections of hamster middle ears. Infant hamsters developed severe necrotic otitis media, and vaccinia viral antigen was demonstrated by fluorescent antibody staining in large confluent areas of epithelial cells lining the middle ear. Juvenile hamsters developed a patchy, less severe infection of the middle ear, most of which present along the tympanic membrane and middle ear ossicles, Adult hamsters did not develop middle ear infection. These findings support the argument that variola (smallpox) virus can cause otitis media in humans and that viral infection of the middle ear may be the cause of hearing loss.
Acute Disease↗
Lateralization during the Weber test: animal experiments.
OBJECTIVES/HYPOTHESIS: The objective of this study were to present an assessment of a new theory to explain lateralization during the Weber test using an animal model. This theory is based on the discovery that a major pathway in bone conduction stimulation to the inner ear is through the skull contents (probably the cerebrospinal fluid [CSF]). The placement of a bone vibrator or tuning fork on the skull excites the inner ear by the classic osseous pathway and by the suggested CSF pathway. We assume that there is a phase difference between the stimulation mediated by the ossicular chain (inertial and occlusion mechanisms) and the one mediated by the CSF. The presence of a conductive pathology will decrease the magnitude of the sound energy mediated by the ossicular chain. Thus, the out-of-phase signal arriving through the bony pathways will be decreased, hence increasing the resultant sound intensity stimulating the cochlea. STUDY DESIGN: Prospective animal study. METHODS: The experiment was performed on 10 fat sand rats, which had undergone unilateral cochleostomy and a small craniotomy. The auditory nerve brainstem response (ABR) thresholds were measured to air-conducted stimulation, to stimulation with the bone vibrator applied to the skull, and to stimulation with the bone vibrator applied directly to the brain through the craniotomy. The ossicular chain of the second ear was then fixed to the middle ear walls with cyanoacrylate glue to induce a conductive hearing loss. The ABR thresholds to the same three stimuli were then measured again. RESULTS: After ossicular chain fixation, the ABR threshold to air-conducted stimulation increased, to bone vibrator stimulation on the bone decreased (hearing improvement), and to bone vibrator stimulation directly on the brain remained unchanged. CONCLUSIONS: This experiment confirms the proposed theory. During clinical bone conduction stimulation, there is a phase difference between sound energy reaching the inner ear through the middle ear ossicles and from the CSF. A middle ear conductive pathology removes one of these components, thus increasing the effective sound intensity in the affected ear. On the other hand, when the bone vibrator is applied on the brain, the inner ear is stimulated only through the CSF, so ossicular chain fixation does not change the ABR threshold. Moreover, this study proves that lateralization during the Weber phenomenon is the result, at least in part, of an intensity difference between sound energy reaching the two cochleae.
Acoustic Stimulation↗
Afferent influences on brain stem auditory nuclei of the chicken: effects of conductive and sensorineural hearing loss on n. magnocellularis.
Nucleus magnocellularis is the avian homologue of the spherical cell region of the mammalian anteroventral cochlear nucleus. Its primary excitatory synaptic input is from large end bulbs of Held from the eighth nerve ganglion cells. We have examined the effects of three peripheral manipulations--middle ear ossicle (columella) removal (monaural and binaural), columella removal and oval window puncture (monaural), and monaural earplug--on cross-sectional cell area ("cell size") of second-order auditory neurons in n. magnocellularis of the chicken. Manipulations were performed between embryonic day 19 and posthatch day 4. Survival time was varied from 2 to 60 days. Air conduction and bone conduction thresholds were determined to assess for conductive and sensorineural hearing loss associated with each of these manipulations. Hair cell counts were made from basilar papillae of each experimental group. We found that a columella removal alone, which produced a 50-55-dB purely conductive hearing loss, was not associated with changes in cell size of n. magnocellularis neurons. Similarly, chronic monaural earplugging did not affect the cross-sectional area of these second-order auditory neurons. Conversely, a combined columella removal and oval window puncture, which produced a mixed hearing loss with a 15-40-dB sensorineural component was associated with an 18-20% reduction in n. magnocellularis cell area. Hair cell counts for experimental ears were not significantly different from control ears. These results, in conjunction with measurements of multiunit activity recorded in n. magnocellularis, suggest that manipulations which markedly attenuate extrinsic auditory stimulation, but do not result in chronic change in the average activity levels, also do not influence the size of n. magnocellularis cell bodies. On the other hand, a manipulation which influences overall activity levels, but does not result in degeneration of receptor cells, resulted in marked changes in n. magnocellularis cell size.
Afferent Pathways↗
Synaptophysin immunoreactivity in the cochlear nucleus after unilateral cochlear or ossicular removal.
This study determined if unilateral cochlear removal in adult guinea pigs led to synaptic loss followed by synaptogenesis in the cochlear nucleus (CN) and if unilateral middle ear ossicle removal led to synaptic loss in the CN. Synaptic endings were identified immunohistochemically, using a monoclonal antibody to synaptophysin. Immunolabeling was quantified densitometrically in the CN 4-161 days after cochlear removal and 161 days after ossicle removal. Fiber degeneration was visualized with the Nauta-Rasmussen silver method. Tissue shrinkage was measured from drawings of CN sections. Compared to the contralateral side, immunolabeling density ipsilaterally was reduced by 4 days in the anterior division of the anteroventral CN (a-AVCN) and by 7 days in the anterior part of the posteroventral CN (a-PVCN). At 7 days, preterminal fiber degeneration was abundant in both areas. These findings were consistent with the loss of cochlear nerve endings and fibers. At later times, immunolabeling density recovered. In the a-AVCN, tissue shrinkage explained approximately half the recovery of staining density; the rest was attributed to synaptogenesis. In the a-PVCN, the entire recovery was attributed to tissue shrinkage. In the polymorphic layer of the dorsal CN, immunostaining density increased transiently at 4 days, while at 7 days preterminal fiber degeneration was abundant. A net loss of synaptic endings was not detected immunohistochemically. The increased immunostaining density may reflect a transient growth of immature processes or presynaptic endings. Ossicle removal produced a deficit in immunolabeling density only in the ipsilateral a-PVCN, without fiber degeneration, suggesting a loss of presynaptic endings or of synaptophysin expression.
Afferent Pathways↗
Hush puppy: a new mouse mutant with pinna, ossicle, and inner ear defects.
OBJECTIVES/HYPOTHESIS: Deafness can be associated with abnormalities of the pinna, ossicles, and cochlea. The authors studied a newly generated mouse mutant with pinna defects and asked whether these defects are associated with peripheral auditory or facial skeletal abnormalities, or both. Furthermore, the authors investigated where the mutation responsible for these defects was located in the mouse genome. METHODS: The hearing of hush puppy mutants was assessed by Preyer reflex and electrophysiological measurement. The morphological features of their middle and inner ears were investigated by microdissection, paint-filling of the labyrinth, and scanning electron microscopy. Skeletal staining of skulls was performed to assess the craniofacial dimensions. Genome scanning was performed using microsatellite markers to localize the mutation to a chromosomal region. RESULTS: Some hush puppy mutants showed early onset of hearing impairment. They had small, bat-like pinnae and normal malleus but abnormal incus and stapes. Some mutants had asymmetrical defects and showed reduced penetrance of the ear abnormalities. Paint-filling of newborns' inner ears revealed no morphological abnormality, although half of the mice studied were expected to carry the mutation. Reduced numbers of outer hair cells were demonstrated in mutants' cochlea on scanning electron microscopy. Skeletal staining showed that the mutants have significantly shorter snouts and mandibles. Genome scan revealed that the mutation lies on chromosome 8 between markers D8Mit58 and D8Mit289. CONCLUSION: The study results indicate developmental problems of the first and second branchial arches and otocyst as a result of a single gene mutation. Similar defects are found in humans, and hush puppy provides a mouse model for investigation of such defects.
Animals↗