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Malleus Teflon piston prosthesis.

The malleus Teflon piston prosthesis is very useful to reconstruct the sound conducting mechanism of the middle ear in absent or inadequate incus, congenital malformation of the middle ear, and epitympanic fixation of the incus and/or malleus. It has a thin-walled loop at its upper end to lessen the tendency for ulceration of the overlying drum with an inside diameter of 1 mm to fit around the mid portion of the handle of the malleus at an angle of 30 degrees to the shaft of the prosthesis. There is a cut in the distal part of the loop through which it is snapped onto the handle of the malleus after it is separated from the drum. Examples of the various situations in which this prosthesis has been used will be shown together with the results regarding hearing gain, complications, and other problems. Those situations in which the prosthesis was used in 1972 will be reviewed to give a 10-year follow-up, plus some notable examples of other uses of the prosthesis from other times.

Ear Ossicles↗

Hydroxylapatite ossicular replacement prostheses: results in 157 consecutive cases.

The use of hydroxylapatite for ossicular chain reconstruction is increasing. In this study, hearing results and extrusion rate for 157 consecutive patients receiving hydroxylapatite prostheses were evaluated. Results were compared to those of a control group of patients who had received either homograft bone or Plasti-Pore prostheses. Four (2.6%) cases of extrusion have occurred. Hearing success was defined as a postoperative puretone average air-bone gap of < or = 15 dB for incus prostheses and partial ossicular replacement prostheses (PORPs) or < or = 25 dB for incus-stapes prostheses and total ossicular reconstruction prostheses (TORPs). Overall success rate in the hydroxylapatite group was 51.4% for the 140 patients with postoperative data (mean follow-up, 11.5 months) and 46.7% for 90 patients who were followed for more than 6 months (mean follow-up, 16 months). PORP results were significantly poorer than those of the other prostheses. The success rate for the control group was 58.6% for 58 patients. The difference between hydroxylapatite and control group success rates appears to be due to the poorer results of the hydroxylapatite PORP.

Adult↗

Autologous fibrin tissue adhesive for ossicular reconstruction in cats.

Autologous fibrin tissue adhesive is currently the most promising adhesive for otologic use with respect to strength and biocompatibility without the risk of transmissable disease that is of concern with the commercially prepared fibrin adhesive. We set out to evaluate the practicality of preparing autologous fibrin adhesive in cats and to see if the adhesive's duration and strength of bonding was sufficient to allow natural tissue union to occur with various grafting materials. Autologous fibrin adhesive was prepared preoperatively from ten cats using the ammonium sulfate precipitation technique. Twenty otologic procedures were performed in which the incus long process was resected and the defect bridged with one of four grafting materials: autograft ossicular bone, bone pate-fibrin glue, porous hydroxylapatite, and Plastipore-bone pate. All grafts were secured with the autologous adhesive. The cats were sacrificed at 6 and 12 weeks. We found the the autologous adhesive provided adequate duration and strength of support to enable a firm tissue union between all the grafting materials and the adjoining incus and stapes.

Animals↗

[The morphometry of the ear ossicles in humans during development].

The dimensions and the mass of the auditive ossicles was determined bilaterally in 100 human fetuses, of each sex aged from 21 to 40 weeks and 20 individual adults aged 18 to 40 years. It was found that the development of auditive ossicles in human is not completed during fetal life. The analyzed parameters of malleus (a, b, c, d1, d2, e) in the over fetal life period increased adequately by 14.02%, 11.22%, 16.70%, 12.80%, 12.01%, 21.98%, incus by (a, b, c1, c2, d, e) - 7.61%, 11.48%, 11.40%, 23.59%, 12.14%, 14.94%, stapes by (a, b, c, d, e, f) - 6.28%, 7.66%, 8.40%, 4.54%, 4.54%, 4.16%. In the over fetal life period increase of weight of malleus by 22.05%, incus by 26.49%, stapes by 11.57% was also observed. Described parameters of ossicles system are very important with respect to classification to the operations improving hearing in the system convecting of vibration of the tympanic membrane in children.

Adult↗

Reconstruction of the ossicular chain with hydroxyapatite prostheses.

To achieve a lasting result in reconstruction of the ossicular chain, strict material and surgical criteria must be combined. The best biomaterial, apart from bone, is hydroxyapatite, the mineral matrix of living bone tissue. For the reconstruction, the assembly technique is used instead of a columella. Direct contact with the drum membrane is not reliable if a columella is used, and extrusion or lateralization may influence postoperative results. In the case of a missing incus, an assembly prosthesis of dense hydroxyapatite is placed on top of the stapes head and the handle of the malleus, in a pocket between drum membrane and the neck of the malleus. Long postoperative follow-up of 120 patients showed an air-bone gap closure within 20 dB for 83.3% of the patients, no extrusion was observed, and the hearing gain stayed constant. Reconstruction of a tympanic membrane perforation and posterior canal wall was performed in the same stage in sixty patients. In the case of a missing incus and stapes superstructure, an incus/stapes prosthesis of dense hydroxyapatite, as assembly between footplate and handle of the malleus, was used. Long postoperative follow-up of twenty-five patients showed an air-bone gap closure within 20 dB for 68% of the patients. Reconstruction of a tympanic membrane perforation and posterior canal wall was performed in sixteen patients. No extrusion was observed and the hearing gain stayed constant. Hydroxyapatite prostheses have proven to be a good alternative for autologous and homologous ossicles.

Adolescent↗

[Postnatal development of the sound-transporting apparatus in the middle ear of rabbits].

The middle ear apparatus realized a multiplicatory function during the transfer of the vibrational energy, depending on the area ratio of pars tensa of tympanic membrane and of stapes footplate as well as the lever ratio of long arms of manubrium and of incus. These structures exhibit a size increase during the postnatal development, but the sequel on the multiplicatory function in rabbits is unknown. The middle ear structures of 46 rabbits, 1 to 30 d old, were prepared and measured. The area of pars tensa and the levers of malleus and incus increase with age. After the 10th d of life, no statistical significant growth were measurable. But the calculated multiplicatory factor of single animals indicate the end of the development at the 15th postnatal d. In contrast, the cochlear function attains the adult values not till the 26th d of life. It is 10 d longer than the middle ear growth.

Aging↗

The loose wire syndrome.

The loose wire syndrome occurs in patients who have had stapedectomy surgery and insertion of a prosthesis which attaches to the long process of the incus by means of a crimped wire. The syndrome consists of a triad of one or more symptoms which improve temporarily with middle ear inflation. These include improvements in auditory acuity, in distortion of sound, and in speech discrimination. Forty-three patients were studied for age, sex, and symptom distribution, and for results of corrective surgery. In all cases, a loose wire at its attachment to the incus was found and corrective surgery (tightening of the wire) was performed. Objectively and subjectively all but two patients were relieved of their symptoms.

Adult↗

Osteoma of the middle ear. Report of a case.

Osteomas of the middle ear are rare. We report a case of a 7-year-old boy with osteoma originating from the pyramidal eminence, combined with congenital cholesteatoma. The osteoma and cholesteatoma were successfully removed by tympanomastoidectomy. The long process of the incus and the superstructure of the stapes disappeared. The body of the incus was sculpted and used as a columella. Histopathologically, the osteoma was much the same as an external auditory canal osteoma. The possibility of a primary congenital origin of this neoplasm is suggested.

Child↗

Pathogenesis of retinoic acid-induced ear malformations in primate model.

13-cis retinoic acid (RA) is a causative agent for human/monkey retinoic acid embryopathy (RAE), in which the most common type of malformation is microtia or anotia. In the present study, malformed ears of monkey fetuses exposed to RA during early embryogenesis were analyzed and revealed a subtype of defects., i.e., apparent duplication of the external/middle ear. A part of the posterior auricle appeared to be ectopically formed in the anterior auricular region or in the region posterior to the auricle. Additionally, there was duplication of the zygomatic arch, malleus, and incus. In order to characterize possible pathogenetic events underlying these malformations, embryos at selected stages were collected after dosing dams with RA at 5 mg/kg/day during gestational days 12-27. Cellular retinoic acid binding protein I whole-mount immunostaining showed that RA induced specific alterations in the migration of cranial neural crest cells (NCC). NCC en route to the second pharyngeal arch were bifurcated, and some of these NCC migrated abnormally into the first and/or third arches, which may underlie external ear duplication. Scanning electron microscopy and neurofilament immunostaining provided evidence that there was partial duplication of trigeminal nerve/ganglion following RA insult. The duplication of NCC neuronal derivatives in the first pharyngeal arch is consistent with duplication of NCC mesenchymal components (zygomatic arch, malleus, and incus). Therefore, RA-induced alterations in cranial NCC migration patterns are likely to be a pathogenetic event underlying ear malformations (including duplication) of RAE in monkeys.

Animals↗

New evidence from Le Moustier 1: computer-assisted reconstruction and morphometry of the skull.

In this study, we present a new computerized reconstruction of the Le Moustier 1 Neanderthal skull and discuss its significance for Neanderthal growth and variability. Because of the precarious state of preservation of the original material, we applied entirely noninvasive methods of fossil reconstruction and morphometry, using a combination of computed tomography, computer graphics, and stereolithography. After electronic restoration, the isolated original pieces were recomposed on the computer screen using external and internal anatomical clues to position the bone fragments and mirror images to complete missing parts. The inferred effects of general compressive deformation that occurred during fossilization were corrected by virtual decompression of the skull. The resulting new reconstruction of the Le Moustier 1 skull shows morphologic features close to the typical Neanderthal adult state. Residual asymmetry of skeletal parts can be traced to in vivo skeletal modification: the left mandibular joint shows signs of a healed condylar fracture, and the anatomy of the occipital region suggests mild plagiocephaly. Using micro-CT analysis, the left incus could be recovered from the matrix filling of the middle ear cavity. Its morphometric dimensions are similar to those of the La Ferrassie III incus. The morphometric characteristics of the inner ear deviate substantially from the condition reported as typical for Neanderthals and fall within the range of modern human variability.

Animals↗

Rotation of middle ear ossicles during cetacean development.

Cetacean middle ears are unique among mammals in that they have an elongated tympanic membrane, a greatly reduced manubrium mallei, and an incudal crus longum that is shorter than the crus breve. Elongation of the tympanic membrane and reduction of the manubrium is thought to be related to an evolutionary rotation of the incus and malleus out of the plane of the tympanic membrane. We examined if rotation also occurs during ontogeny by comparing the middle ears of two species of dolphins (Delphinus delphis, Stenella attenuata) at different stages of development. We observed that: the incus has the body and crural proportions as in terrestrial mammals early in development; the incudomallear complex rotates approximately 90 degrees following ossification; the tympanic membrane is not elongated until relatively late in development. Therefore, some of the unique characteristics of the cetacean middle ear develop as modifications of an initially terrestrial-like morphology.

Animals↗

Ontogenetic and phylogenetic transformations of the ear ossicles in marsupial mammals.

This study is based on the examination of histological sections of specimens of different ages and of adult ossicles from macerated skulls representing a wide range of taxa and aims at addressing several issues concerning the evolution of the ear ossicles in marsupials. Three-dimensional reconstructions of the ear ossicles based on histological series were done for one or more stages of Monodelphis domestica, Caluromys philander, Sminthopsis virginiae, Trichosurus vulpecula, and Macropus rufogriseus. Several common trends were found. Portions of the ossicles that are phylogenetically older develop earlier than portions representing more recent evolutionary inventions (manubrium of the malleus, crus longum of the incus). The onset of endochondral ossification in the taxa in which this was examined followed the sequence; first malleus, then incus, and finally stapes. In M. domestica and C. philander at birth the yet precartilaginous ossicles form a supportive strut between the lower jaw and the braincase. The cartilage of Paauw develops relatively late in comparison with the ear ossicles and in close association to the tendon of the stapedial muscle. A feeble artery traverses the stapedial foramen of the stapes in the youngest stages of M. domestica, C. philander, and Sminthopsis virginiae examined. Presence of a large stapedial foramen is reconstructed in the groundplan of the Didelphidae and of Marsupialia. The stapedial foramen is absent in all adult caenolestids, dasyurids, Myrmecobius, Notoryctes, peramelids, vombatids, and phascolarctids. Pouch young of Perameles sp. and Dasyurus viverrinus show a bicrurate stapes with a sizeable stapedial foramen. Some didelphids examined to date show a double insertion of the Tensor tympani muscle. Some differences exist between M. domestica and C. philander in adult ossicle form, including the relative length of the incudal crus breve and of the stapes. Several differences exist between the malleus of didelphids and that of some phalangeriforms, the latter showing a short neck, absence of the lamina, and a ventrally directed manubrium. Hearing starts in M. domestica at an age in which the external auditory meatus has not yet fully developed, the ossicles are not fully ossified, and the middle ear space is partially filled with loose mesenchyme. The ontogenetic changes in hearing abilities in M. domestica between postnatal days 30 and 40 may be at least partially related to changes in middle ear structures.

Animals↗

Modulated expression of type X collagen in Meckel's cartilage with different developmental fates.

Mammalian Meckel's cartilage undergoes regionally diverse histodifferentiation: the caudal end of Meckel's cartilage extends to the developing ear and gives rise to malleus and incus through endochondral ossification while its major distal region differentiates into sphenomandibular ligament and the anterior ligament of the malleus tympanic plate through fibrous transformation. Since the entire Meckel's cartilage develops up to chondrocyte hypertrophy, the regional extracellular matrix components in the hypertrophic Meckel's cartilage may differ in association with the diverse developmental fates. In this project, the expressions of cartilage collagens were investigated in developing rat Meckel's cartilage and particular interest was given to type X collagen. A cDNA, HP114, encoding the NC1 domain of rat alpha 1(X) collagen was cloned, and a synthetic peptide based on the sequence deduced from HP114 was used to generate a monospecific antibody. In situ hybridization of newborn rat condylar and angular cartilages undergoing endochondral ossification showed restricted labeling with the alpha 1(X) collagen probe in the hypertrophic chondrocyte layer. In contrast, the alpha 1(X) collagen probe totally failed to label the major distal portion of Meckel's cartilage even in the hypertrophic cartilage zone. Immunohistochemistry using the anti-type X collagen monospecific antibody consistently failed to recognize the epitope in the corresponding portion of Meckel's cartilage throughout the experimental periods of gestational Day 17, newborn, and Postnatal Day 7, while the strictly localized positive staining was found in the posterior part of Meckel's cartilage which gave rise to malleus and incus. Since major cartilage collagens type II and type IX were found to be present throughout Meckel's cartilage, we postulate that the regulatory molecular mechanism of type X collagen expression may be closely associated with the developmental fates of fibrous transformation and endochondral ossification in mammalian Meckel's cartilage.

Amino Acid Sequence↗

[In vivo studies of a piezoelectric implantable hearing aid transducer in the cat].

Recently, we presented an implantable piezoelectrical hearing aid transducer. Its characteristics make it suitable for implantation in patients with sensorineural hearing loss. The transducer transmits micromechanical vibrations instead of sound into the hearing organ. Efficiency of the transducer implant was investigated in ten cat ears. After determining preoperative (acoustical) BERA threshold, the middle ear was opened and the piezoelectrical transducer coupled to various ossicles or the perilymph. BERA responses were recorded following stimulation of umbo, long incus process, stapes head, stapes foot plate, and vestibulum. By comparing the acoustical and mechanical threshold, a correlation was found between the stimulus level of acoustical and mechanical stimulation. An electrical transducer voltage of 1 Vrms was equivalent to sound-pressure levels between 100 and 128 dB SPL at the tympanic membrane. To judge hearing impression, stimulus-dependent latencies of the early acoustically and mechanically evoked potentials (waves P1 to P5) and their thresholds were analyzed. After coupling the piezoelectrical transducer to the long incus process, latencies corresponded well to stimulation. They were almost completely similar when the equivalent sound-pressure level of 100 dB SPL was achieved by the transducer voltage level.

Animals↗

[Cold deformation elements for attaching an implantable hearing aid transducer to ear ossicles or perilymph].

Development and short-term implantation results of the Tübingen implantable hearing aid (TI = Tübingen implant) have been presented. The TI is designed for patients with sensorineural hearing loss due to a malfunction of the cochlear amplifier. This can be identified by the presence of positive recruitment and the absence of TEOAE (transitory evoked otoacoustic emissions). The Tübingen implant functions in two ways: it allows electronic amplification of the auditory signal and electromechanical signal transduction into a micromechanical vibratory stimulus. There are two paths by which vibratory stimulus reaches the cochlea: (1) directly through a perforation in the stapes foot plate into the perilymph or (2) via the ossicular chain. Made of pure titanium, the casing of the helium-tight welded transducer includes the piezoelectric actuator. An implantable manipulator device is designed for transducer positioning and anchoring in the mastoid cavity. Usually, the transducer probe tip is directly coupled to the body of the incus. This functions without a special coupling device by utilization of an Erbium-YAG laser. Special anatomical situations or the loss of incus and/or stapes suprastructure, however, requires coupling of the vibratory signal to other points of the ossicular chain or to the perilymph. A major problem, however, was an intraoperative, irreversible link between the titanium probe tip and coupling elements. To overcome this problem, the coupling elements were made of gold. A crimp technique was developed, allowing the surgeon to induce cold deformation of the gold. The cold deformation technique (crimp) results in an irreversible coupling between the titanium probe tip and the golden coupling element.

Cochlear Implantation↗

[An osseointegrated micromanipulator as anchor for implantable hearing aid transducers. 1: Fitting to the surgical anatomy of the temporal bone and surgical technical properties].

The first electronic implantable hearing aids for patients with hearing loss are coupled to the ossicular chain or perilymph during implantation and are now available. Our new Tübingen implant designed for sensorineural hearing loss (SNHL) is the combination of an implantable microphone and piezotransducer. To avoid hearing losses during implantation, the Tübingen piezotransducer will be (1) fixed to the mastoid cavity and (2) positioned to one of the ossicular target points. This can be done with a micromanipulator which will be implanted together with transducer and microphone in the mastoid cavity. The manipulator weights 0.7 g. With four degrees of freedom, it allows highly secure and safe positioning of the transducer's probe tip to the ossicular chain under close to stereotactic conditions. The main advantages of the present micromanipulator are (1) easy handling during surgery, (2) the transducer's precise positioning to the ossicular target point with sufficient degrees of freedom, and (3) the transducer's stable fixation in the mastoid cavity in the final position. Following integrated safety as the leading principle, ossicular or inner ear injuries caused, e.g., by the patient's head movement or unintentional manual contact by the surgeon, are minimized. The micromanipulator is, as it were, the surgeon's vibration-free "artificial hand". The manipulator's development and its optimization to the mastoid cavity by test implantation in 50 human temporal bones are shown in detail. While coupling the transducer to the body of the incus, transducer, microphone, and micromanipulator can be implanted into 76% of all mastoid cavities without protrusion. In the case of transducers coupling to the long process of the incus, the protrusion-free implantation rate of the above-mentioned three implant modules is 78%.

Bone Plates↗

In vivo experiments in the cat with an implantable piezoelectric hearing aid transducer.

We have recently developed an implantable piezoelectric hearing aid transducer that is suitable for implantation in patients with sensorineural hearing loss. The transducer does not transmit sound but conducts micromechanical vibrations to the cochlea. In ten cat ears we investigated the efficiency of the implantable transducer with respect to the direct transfer of vibrations within the audible frequency range via the ossicles to the cochlea or directly into the vestibule. The acoustically evoked brainstem potential (ABR) threshold was determined prior to implantation, and the middle ear was then opened and the piezoelectric transducer coupled to the ossicles or to the perilymph. Acoustically evoked brainstem potentials were recorded following stimulation at the umbo, long process of the incus, stapes head, stapes foot plate, and in the vestibulum. Comparisons of the acoustically and mechanically evoked thresholds revealed a good correlation of the two stimulation levels. An electrical transducer voltage of 1 V(RMS) produced equivalent sound pressure levels (SPL) of 100-128 dB at the tympanic membrane. To assess the hearing we compared stimulus-dependent latencies of the early potentials (peaks P1-P5) and thresholds. This evaluation was based on four ears with normal hearing in which the piezoelectric transducer was coupled to the long process of the incus. The mean values of the latencies and their scattering range correlated extremely well in the two stimulation modes. They were nearly identical when the equivalent SPL of 100 dB was assigned to the maximally applied electrical level of 0 dB. These in vitro and in vivo findings demonstrate that the characteristics of the transducer warrant its development further from the prototype stage to become a component of an implantable hearing device for patients with sensorineural hearing loss.

Acoustic Stimulation↗

The impact of a syndromal diagnosis on surgery for congenital minor ear anomalies.

Between 1964 and 1986, 104 ears of 86 patients with a minor congenital ear anomaly underwent an exploratory tympanotomy at the Institute of Otorhinolaryngology of the University Hospital Nijmegen. A classification of these anomalies is proposed based on the surgical findings and results. The 4 groups in this classification are: isolated stapes ankylosis, stapes ankylosis associated with an anomaly of the malleus and incus, an isolated anomaly of the malleus and incus with a mobile stapes footplate and finally, aplasia of the oval and/or round window. In a total of 29 ears (22 patients) out of these 104 ears, the anomaly formed part of a syndrome. The various syndromes and the anomalies encountered are discussed. The impact of a syndromal diagnosis on the outcome of reconstructive ear surgery is discussed per syndrome.

Abnormalities, Multiple↗