Comparative Impacts of Scala Vestibuli Versus Scala Tympani Cochlear Implantation on Auditory Performances and Programming Parameters in Partially Ossified Cochleae.

OBJECTIVE: To compare scala vestibuli versus scala tympani cochlear implantation in terms of postoperative auditory performances and programming parameters in patients with severe scala tympani ossification. STUDY DESIGN: Retrospective case-control study. SETTING: Tertiary referral center. PATIENTS: One hundred three pediatric and adult patients who underwent cochlear implant surgery between 2000 and 2016. Three groups were formed: a scala vestibuli group, a scala tympani with ossification group, and a scala tympani without ossification group. Patients were matched based on their age, sex, duration of deafness, and side of implantation (ratio of 1:2:2). INTERVENTIONS: Postoperative evaluation of auditory performances and programming parameters following intensive functional rehabilitation program completion. MAIN OUTCOME MEASURES: Multimedia adaptive test (MAT), hearing in noise test (HINT SNR +10 dB, HINT SNR +5 dB, and HINT SNR +0 dB), impedances, neural response telemetry thresholds (NRT), neural response imaging thresholds (NRI), comfortable levels (C-levels), and threshold levels (T-levels) were compared between groups. RESULTS: Twenty-one patients underwent scala vestibuli cochlear implantation: 19 adults and two children. Auditory performances were similar between groups, although sentence recognition in a noisy environment was slightly higher in the scala vestibuli group. Impedance values were also higher in the scala vestibuli group, but all other programming parameters were similar between groups. CONCLUSIONS: We present the largest series of patients with scala vestibuli cochlear implantation. This approach provides at least comparable auditory performances without having any deleterious effects on programming parameters. This viable and useful insertion route might be the primary surgical alternative when facing partial cochlear ossification.

A surgical navigation system for guiding exact cochleostomy using auditory feedback: a clinical feasibility study.

In cochlear implantation (CI), the insertion of the electrode array into the appropriate compartment of the cochlea, the scala tympani, is important for an optimal hearing outcome. The current surgical technique for CI depends primarily on the surgeon's skills and experience level to achieve the correct placement of the electrode array, and the surgeon needs to confirm that the exact placement is achieved prior to completing the procedure. Thus, a surgical navigation system can help the surgeon to access the scala tympani without injuring important organs in the complex structure of the temporal bone. However, the use of a surgical microscope has restricted the effectiveness of the surgical navigation because it has been difficult to deliver the navigational information to the surgeon from outside of the surgeon's visual attention. We herein present a clinical feasibility study of an auditory feedback function developed as a computer-surgeon interface that can guide the surgeon to the preset cochleostomy location. As a result, the surgeon could confirm that the drilling point was correct, while keeping his or her eyes focused on the microscope. The proposed interface reduced the common frustration that surgeons experience when using surgical navigation during otologic surgeries.

Reliability of cone beam computed tomography in scalar localization of the electrode array: a radio histological study.

Postoperative imaging plays a growing role in clinical studies concerning prognostic factors in cochlear implantation. Indeed, intracochlear position of the cochlear implant has recently been identified as a contributor in functional outcomes and radiological tools must be accurate enough to determine the final placement of the electrode array. The aim of our study was to validate cone beam computed tomography as a reliable technique for scalar localization of the electrode array. We performed therefore a temporal bone study on ten specimens that were implanted with a perimodiolar implant prototype. Cone beam reconstructions were performed and images were analyzed by two physicians both experienced in cochlear implant imaging, who determined the scalar localization of the implant. Temporal bones then underwent histological control to document this scalar localization and hypothetical intracochlear lesions. In four cases, a dislocation from scala tympani to scala vestibuli was suspected on cone beam reconstructions of the ascending part of the basal turn. In three of these four specimens, dislocation in pars ascendens was confirmed histologically. In the remaining temporal bone, histological analysis revealed an elevation with rupture of the basilar membrane. Histological assessment revealed spiral ligament tearing in another bone. We conclude that cone beam is a reliable tool to assess scalar localization of the selectrode array and may be used in future clinical studies.

An experimental study of inner ear injury in an animal model of eosinophilic otitis media.

CONCLUSION: As the periods of intratympanic injection of ovalbumin (OVA) to the middle ear became longer, marked eosinophil infiltration in the perilymphatic space was observed. Moreover severe morphological damage of the organ of Corti was observed in the 28-day antigen-stimulation side. These results indicate that eosinophilic inflammation occurred in the inner ear and caused profound hearing loss. OBJECTIVE: The purpose of the present study was to elucidate the inner ear damage in a new animal model of eosinophilic otitis media (EOM) which we recently constructed. METHODS: We constructed the animal model of EOM by intraperitoneal and intratympanic injection of OVA. Infiltrating cells and the inner ear damage were examined by histological study. RESULTS: In the inner ear, a few eosinophils were seen in the scala tympani of the organ of Corti and the dilation of capillaries of the stria vascularis was observed in the 7-day stimulation side. In the 14-day antigen stimulation side, some eosinophils and macrophages were seen in not only the scala tympani but also the scala vestibule. In the 28-day antigen-stimulation side, severe morphological damage of the organ of Corti and many eosinophils, red blood cells, and plasma cells infiltrating the perilymph were observed.

Hearing loss in Paget's disease: a temporal bone histopathology study.

BACKGROUND: Hearing loss in Paget's disease of bone (PDB) is typically mixed and bilateral. Although different mechanisms have been proposed, the pathophysiology of hearing impairment remains unclear. OBJECTIVE: The purpose of this study is to describe the histopathologic findings of temporal bones in patients with PDB and elucidate possible pathologic mechanisms related to hearing impairment. METHODS: This is an archival human temporal bone study of 8 subjects diagnosed with Paget's disease from the temporal bone collection of the UCL Ear Institute. RESULTS: A fractured stapes footplate was observed in 1 temporal bone and stapes footplate fixation in 2 other specimens. Obliteration of Cotugno's canal by extensive bone remodeling was observed in 78% of temporal bones. An intracochlear vestibular schwannoma was observed in 1 specimen (previously reported in the literature). Other findings include microfissures and microfractures of the otic capsule, bleeding in the scalae, strial atrophy, and cystic lesions in the spiral ligament. CONCLUSION: This study is the first to report a fractured stapes footplate, as a causative lesion of conductive hearing loss in PDB. Extensive bone remodeling around Cotugno canal also was a frequent finding, not reported before in the literature. We hypothesize that sensorineural hearing loss in patients with PDB of the temporal bone may, in some cases, be attributed to obliteration of Cotugno's canal by remodeling pagetoid bone, thus obstructing the venous drainage of the cochlea, with a subsequent effect on the function of stria vascularis and spiral ligament. This seems to be consistent with experimental studies in animals.

Local delivery of reporter gene to the cochlea does not spread to brain tissue in an animal model.

CONCLUSION: The results suggest that local injection of 1 microl of lentiviral-green fluorescent protein (LV-GFP) into rat scala tympani as a lentiviral (LV) vector in the cochlea does not disseminate into the surrounding brain tissue. OBJECTIVE: To investigate whether the LV vector will spread into the cerebrospinal fluid (CSF) and affect brain tissue after local cochlear injection in an animal model. MATERIALS AND METHODS: Sixteen animals were sacrificed after cochleostomy and injection of 1 microl LV-GFP vectors with different promoters such as CAG (consisting of the cytomegalovirus immediate early enhancer and the chicken beta-actin promoter), EF-1alpha (human elongation factor 1alpha), PGK (human phosphoglycerate kinase 1) and CPPT (central polypurine tract). Eleven brain tissues were fixed in 4% paraformaldehyde at 4 degrees C, processed for cryosectioning and examined under fluorescence microscope. RESULTS: The patterns of the fluorescent signals with red and green filters were compared to identify the GFP signals in the brain tissue. GFP reporter gene expression was not detected in any examined brain region in any of the animals.

Macrophage inflammatory proteins in cytomegalovirus-related inner ear injury.

OBJECTIVE: Inner ear inflammation triggered by CMV infection may play a role in CMV-related auditory pathogenesis. The purpose of the study was to determine if a virally encoded macrophage inflammatory protein played a role in CMV-related hearing loss. DESIGN: Mutagenesis was performed with deletion of a guinea pig CMV macrophage inflammatory protein. Intracochlear inoculations were performed on three groups of animals (n = 18). Group 1 received sterile viral media, Group 2 received wild-type CMV virus, and Group 3 received "knockout" (KO) virus with a deleted immunomodulation gene. Baseline and postinoculation ABRs were obtained. ELISA and PCR were performed and temporal bones examined. SUBJECTS: Eighteen guinea pigs. RESULTS: The KO group had significantly better hearing than the WT group. There were no significant differences between the KO and sham groups. The WT group had significant hearing loss at all frequencies. Inflammation and fibrosis were noted in the WT temporal bones only. CONCLUSIONS: Virally encoded macrophage inflammatory proteins appear to play a significant role in CMV-related hearing loss.

Impact of electrode insertion depth on intracochlear trauma.

OBJECTIVE: To assess the effect of cochlear implant (CI) insertion depth and surgical technique on intracochlear trauma. STUDY DESIGN AND SETTING: Twenty-one fresh human temporal bones were implanted with CI electrodes and underwent histologic processing and evaluation. Specimens were grouped into 3 categories: 1) soft implantation technique and standard electrode; 2) soft implantation technique and flexible prototype array; 3) forceful implantations and standard electrode. Based on the grading system (1 to 4), 2 numeric values were calculated indicating the overall severity of cochlear damage (trauma indices). RESULTS: Mean trauma index values were 13.8, 36.3, and 59.2 for group 1, 2, and 3, respectively. Differences in cochlear trauma (trauma index) were nonsignificant between specimens in groups 1 and 2 but were significant between groups 1 and 3. CONCLUSION: This study gives evidence that intracochlear trauma increases with deep insertions. Thus, in cases where cochlear integrity might be important, limited insertions should be achieved.

High resolution micro-CT scanning as an innovative tool for evaluation of the surgical positioning of cochlear implant electrodes.

X-ray microtomography (micro-CT) is a new technique allowing for visualization of the internal structure of opaque specimens with a quasi-histological quality. Among multiple potential applications, the use of this technique in otology is very promising. Micro-CT appears to be ideally suited for in vitro visualization of the inner ear tissues as well as for evaluation of the electrode damage and/or surgical insertion trauma during implantation of the cochlear implant electrodes. This technique can greatly aid in design and development of new cochlear implant electrodes and is applicable for temporal bone studies. The main advantage of micro-CT is the practically artefact-free preparation of the samples and the possibility of evaluation of the interesting parameters along the whole insertion depth of the electrode. This paper presents the results of the first application of micro-CT for visualization of the inner ear structures in human temporal bones and for evaluation of the surgical positioning of the cochlear implant electrodes relative to the intracochlear soft tissues.

Clinical performance of children following revision surgery for a cochlear implant.

OBJECTIVES: The purpose of this study was to describe the causes for revision surgery and to compare clinical performance before and after surgery in children who required electrode reinsertion. STUDY DESIGN AND SETTINGS: Soundfield thresholds and speech recognition scores were compared before device failure and following electrode reinsertion. Temporal bones from a deceased adult implant user who underwent bilateral revision implantation were analyzed. RESULTS: Histopathology in the adult temporal bones revealed new bone formation in the scala tympani and substantially reduced spiral ganglion counts, with open-set speech recognition realized. Of 28 children undergoing revision surgery, 18 required electrode reinsertion. With the exception of 1 patient with severe cochlear malformation, new electrode arrays were fully inserted without difficulty. Clinical outcomes between pre-device failure and post-electrode reinsertion did not differ statistically. CONCLUSION AND SIGNIFICANCE: Electrode reinsertion is technically feasible in the pediatric population. The majority of children recover their prerevision clinical performance. EBM RATING: C-4.

Perimodiolar electrodes in cochlear implant surgery.

Perimodiolar-positioned cochlear implant electrodes have been developed in order to bring the electrode contacts as close as possible to the spiral ganglion cells, which are the target of electrostimulation. This results in lower electrical thresholds, higher dynamic ranges and less channel interaction when compared with normal implant electrodes which are usually located peripherally within the scala tympani. In this study we evaluated 4 different types of perimodiolar electrode: the Clarion Preformed electrode, the Clarion Preformed electrode with positioner, the Nucleus Contour electrode and the Med-El Perimodiolar Combi 40 electrode. These devices require different approaches to achieve a perimodiolar electrode position. The electrodes were inserted in fresh human temporal bones. After processing these bones with the electrodes in situ by employing a sawing, grinding and polishing technique, the inner ear structures as well as the electrode positions could be evaluated in detail. All electrode types studied had a more or less perimodiolar position; however, each type produced a certain amount of trauma to cochlear structures which is discussed in relation to mechanical properties. Further human temporal bone studies with improved perimodiolar cochlear implant electrodes are necessary in order to find an optimized type of electrode.

Characterization of an experimentally induced inner ear immune response.

OBJECTIVE: To determine the effects of a sterile immune response on the structure and function of the cochlea. METHODS: An immune response was created in guinea pigs by systemically sensitizing the animals to keyhole limpet hemocyanin and subsequently challenging the inner ear with the protein. Animals were allowed to survive for 1 to 5 weeks, after which the cochlea was evaluated histologically. Hearing was measured by auditory brainstem response before the inner ear challenge, during the survival period, and prior to sacrifice. RESULTS: Inflammatory cells infiltrated the cochlea from the circulation. Surface preparations and plastic sections of the organ of Corti 1 and 2 weeks after the initiation of the inflammation demonstrated degeneration of the sensory and supporting cells in cochlear turns containing inflammatory cells. Good preservation of structures was seen in the more apical cochlear turns with little or no inflammatory cells. In cochleas from animals that survived 5 weeks, most of the infiltrated cells were cleared after undergoing apoptosis and the inflammatory matrix in the scala tympani began to calcify. Hearing loss was moderate to severe depending on the amount of inflammation. CONCLUSION: Although in general the immune response serves to protect an organism from infection, these results demonstrate that bystander injury associated with local immune responses in the cochlea, an organ incapable of regeneration, causes permanent cochlear destruction and hearing loss.

Comparison of three-dimensional visualization techniques for depicting the scala vestibuli and scala tympani of the cochlea by using high-resolution MR imaging.

BACKGROUND AND PURPOSE: Cochlear implantation requires introduction of a stimulating electrode array into the scala vestibuli or scala tympani. Although these structures can be separately identified on many high-resolution scans, it is often difficult to ascertain whether these channels are patent throughout their length. The aim of this study was to determine whether an optimized combination of an imaging protocol and a visualization technique allows routine 3D rendering of the scala vestibuli and scala tympani. METHODS: A submillimeter T2 fast spin-echo imaging sequence was designed to optimize the performance of 3D visualization methods. The spatial resolution was determined experimentally using primary images and 3D surface and volume renderings from eight healthy subjects. These data were used to develop the imaging sequence and to compare the quality and signal-to-noise dependency of four data visualization algorithms: maximum intensity projection, ray casting with transparent voxels, ray casting with opaque voxels, and isosurface rendering. The ability of these methods to produce 3D renderings of the scala tympani and scala vestibuli was also examined. The imaging technique was used in five patients with sensorineural deafness. RESULTS: Visualization techniques produced optimal results in combination with an isotropic volume imaging sequence. Clinicians preferred the isosurface-rendered images to other 3D visualizations. Both isosurface and ray casting displayed the scala vestibuli and scala tympani throughout their length. Abnormalities were shown in three patients, and in one of these, a focal occlusion of the scala tympani was confirmed at surgery. CONCLUSION: Three-dimensional images of the scala vestibuli and scala tympani can be routinely produced. The combination of an MR sequence optimized for use with isosurface rendering or ray-casting algorithms can produce 3D images with greater spatial resolution and anatomic detail than has been possible previously.

Changes in immunostaining of inner ears after antigen challenge into the scala tympani.

To study the mechanisms of immune responses and immune injuries in inner ears, labyrinthitis was induced by inoculation of keyhole limpet hemocyanin (KLH) into the scala tympani of systemically sensitized guinea pigs. Inner ears were then immunostained for KLH, immunoglobulin G (IgG), albumin, connexin26 (Cx26), and sodium-potassium adenosine triphosphate (Na,K-ATPase). Inflammatory cells containing KLH were observed in the scala tympani and in the collecting venule of the spiral modiolar vein (SMV). Spiral ligament, spiral limbus, and blood vessels including the SMV were diffusely positive for IgG and albumin. Immunoreactivity for Cx26 and Na,K-ATPase was decreased compared with the normal ears in the fibrocytes of the spiral ligament. These results suggest that inflammatory cells and blood constituents could extravasate into the cochlea from blood vessels and that fibrocyte damage in the spiral ligament could cause cochlear dysfunction.

Mesothelial cell proliferation in the scala tympani: a reaction to the rupture of the round window membrane.

The inner layer of the round window membrane is composed of mesothelial cells and this mesothelial cell layer extends to the scala tympani. This study describes the histopathologic findings of temporal bone analysis from a patient with bilateral perilymphatic fistula of the round window membrane. The left ear showed proliferation of mesothelial cells in the scala tympani of the basal turn adjoining the round window membrane. This cell proliferation is thought to be a reaction to the rupture of the round window membrane.

Cochlear implants in children: what constitutes a complication?

The surgeon must ultimately accept the responsibility for any complications that occur as the result of a cochlear implant. Listening to the cochlear implant team members and responding to their needs may enhance the child's progress. Surgical complications, (i.e., skinflap problems, infection, and facial paralysis) are indeed infrequent, but nonsurgical problems are not. Surgical and nonsurgical experiences were reviewed in 55 children. Ages ranged from 23 months to 18 years at the time of cochlear implantation, which occurred from 1984 to 1995. There were no surgical complications. However, the most common surgical obstacle was ossification, which was present in 40% and undetected by computed tomographic scanning in 16.3% of children. Ossification occurred at the round window and scala tympani in 32.7% and involved the cochlea more extensively in 7.3% of children. In only one child was the cochlea entirely ossified. There were, however, many nonsurgical problems that were viewed as complications in patient management. The single most important complication was device failure. This occurred in 10.9% (5/46) of children with the Cochlear Corporation multichannel implant. Head banging and other temper tantrums, parental interference with rehabilitation, socioeconomic factors, poor compliance by the family unit, equipment problems, educational deficiencies, and impatience with habilitative training were some of the other problems.

The cochlear aqueduct in pediatric temporal bones.

The cochlear aqueduct is a bony channel which contains the fibrous periotic duct and connects the perilymphatic space of the basal turn of the cochlea with the subarachnoid space of the posterior cranial cavity. Previous histological studies suggested that patency depended on age, whereas a more recent study showed no statistical correlation between age and patency. To clarify patency in pediatric cochlear aqueducts, we selected 21 temporal bones from 12 infants and children, varying in age from birth to 9 years, in which the cochlear aqueduct was fully visible on one histological section. Photographs were taken for documentation and the length and width of the orifice of the external aperture of the aqueduct at the scala tympani were measured and followed to the internal aperture at the subarachnoid space. The lumen of the duct was examined for mononucleated cells, blood cells and fibrous tissue. Measurements revealed that the mean length of the cochlear aqueduct was 4.6 mm (range, 2.4-10.7 mm), mean width of the external aperture was 484 microm (range, 225-869 microm), and mean width of the internal aperture was 1293 microm (range, 699-2344 microm). The mean diameter of the narrowest part (isthmus) was 151 microm (range, 75-244 microm). In all temporal bones the cochlear aqueduct was patent, with one exception. This latter temporal bone was from a 2-month-old girl with multiple intralabyrinthine anomalies, with the missing cochlear aqueduct believed to be due to an aplasia. Our results support prior measurements of the cochlear aqueduct and demonstrate a short and patent cochlear aqueduct in newborns. With growth, a significant increasing length of the duct was found.

Temporal bone findings in central nervous system leukemia.

Central nervous system (CNS) leukemia is a pathologic condition whereby leukemic cells enter the cerebrospinal fluid (CSF) causing meningeal irritation syndrome. Temporal bone findings in the case of a 32-year-old man who died of CNS leukemia are presented. He complained of fullness of the ear 4 months prior to death and of dizziness 1 month prior to death. Leukemic cells were seen to have infiltrated the scala tympani of the basal turn in the cochlea, the saccule, and posterior semicircular canal along the vestibulocochlear nerve.

Labyrinthitis ossificans: histopathologic consideration for cochlear implantation.

Labyrinthitis ossificans may be a hindrance to cochlear implantation by making electrode insertion difficult. We performed a histopathologic study of 24 temporal bones with labyrinthitis ossificans from multiple causes. The organ of Corti was graphically reconstructed and the degree of obstruction was estimated for each millimeter of the cochlea. Correlations were calculated between the degree of new bone formation and the cause, patient's age and sex, and time from the original temporal bone insult. Our results demonstrate that complete cochlear ossification is rare. The scala tympani in the basal turn of the cochlea is the most frequent area of ossification, regardless of the cause of the labyrinthitis ossificans. Meningogenic labyrinthitis, usually a childhood disease, was associated with the greatest amount of ossification. When ossification resulted from tympanogenic labyrinthitis, the scala tympani was completely ossified near the round window niche in all temporal bones. Neo-ossification of the basal turn associated with otosclerosis was limited to the proximal 6 mm of the scala tympani in all cases. Three temporal bones had a patent round window niche and basal turn, but significant apical and middle-turn ossification. Peripheral sensorineural elements were severely degenerated in the region of the ossification in all specimens, and spiral ganglion cell counts were decreased.