Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent

Focal Spot, Spring 2002

https://digitalcommons.wustl.edu/focal_spot_archives/1090/thumbnail.jp

Postoperative Impedance-Based Estimation of Cochlear Implant Electrode Insertion Depth.

OBJECTIVES Reliable determination of cochlear implant electrode positions shows promise for clinical applications, including anatomy-based fitting of audio processors or monitoring of electrode migration during follow-up. Currently, electrode positioning is measured using radiography. The primary objective of this study is to extend and validate an impedance-based method for estimating electrode insertion depths, which could serve as a radiation-free and cost-effective alternative to radiography. The secondary objective is to evaluate the reliability of the estimation method in the postoperative follow-up over several months. DESIGN The ground truth insertion depths were measured from postoperative computed tomography scans obtained from the records of 56 cases with an identical lateral wall electrode array. For each of these cases, impedance telemetry records were retrieved starting from the day of implantation up to a maximum observation period of 60 mo. Based on these recordings, the linear and angular electrode insertion depths were estimated using a phenomenological model. The estimates obtained were compared with the ground truth values to calculate the accuracy of the model. RESULTS Analysis of the long-term recordings using a linear mixed-effects model showed that postoperative tissue resistances remained stable throughout the follow-up period, except for the two most basal electrodes, which increased significantly over time (electrode 11: ~10 Ω/year, electrode 12: ~30 Ω/year). Inferred phenomenological models from early and late impedance telemetry recordings were not different. The insertion depth of all electrodes was estimated with an absolute error of 0.9 mm ± 0.6 mm or 22° ± 18° angle (mean ± SD). CONCLUSIONS Insertion depth estimations of the model were reliable over time when comparing two postoperative computed tomography scans of the same ear. Our results confirm that the impedance-based position estimation method can be applied to postoperative impedance telemetry recordings. Future work needs to address extracochlear electrode detection to increase the performance of the method

Evaluating and Improving Cochlear Length Measurements on Clinical Computed Tomography Images

Cochlear implants provide the sensation of sound to deaf individuals. An accurate estimate of cochlear duct length (CDL) is required for pre-operative implant electrode selection and can be obtained from clinical computed tomography (CT) by measuring the “A-value”. The objectives of this work were to estimate the accuracy and variability in manual A-value measurements, and to automate measurements. Four specialists repeatedly measured the A-value on clinical CT images from which the inter- and intra-observer variability were calculated. Accuracy was assessed by comparison to measurements on higher resolution micro-CT images. Motivated by this study, software was developed to automate the A-value measurement by registering an annotated atlas to unlabelled images. There was significant variability in manual A-value measurements made using either standard clinical or multi-planar reformatted views with the latter exhibiting higher variability but better accuracy. The automated approach eliminated variability and improved accuracy, enabling the correct selection of electrode length

Magnetic Resonance Imaging Compatibility of the Polymer-based Cochlear Implant

ObjectivesIn this study, we compared the magnetic resonance (MR) image artifacts caused by a conventional metal-based cochlear implant and a newly developed liquid crystal polymer (LCP)-based device.MethodsThe metal-based cochlear implant system (Nurobiosys Co.) was attached to side of the head of a subject and the LCP-based device was attached to opposite side. In both devices, alignment magnets were removed for safety. Magnetic resonance imaging (MRI) was performed on a widely used 3.0 T and an ultra-high 7.0 T MRI machine. 3.0 and 7.0 T MR images were acquired using T1- and T2*-weighted gradient echo sequences, respectively.ResultsIn the 3.0 T images, the metal-based device on the left side generated the significant amount of artifacts. The MR images in the proximity of the metal package were obscured by the artifacts in both axial and sagittal views. On the other hand, the MR images near the LCP-based device were relatively free from the artifacts and clearly showed the brain structures. 7.0 T MR images showed the more severe distortion in the both sides but the metal-based cochlear implant system caused a much larger obscure area than the LCP-based system.ConclusionThe novel LCP-based cochlear implant provides a good MRI compatibility beyond present-day cochlear implants. Thus, MR images can be obtained from the subjects even with the implanted LCP-based neural prosthetic systems providing useful diagnostic information. Furthermore, it will be also useful for functional MRI studies of the auditory perception mechanism after cochlear implantations as well as for positron emission tomography-MRI hybrid imaging

Development of an auditory implant manipulator for minimally invasive surgical insertion of implantable hearing devices

Abstract Objective: To present the auditory implant manipulator, a navigation-controlled mechanical and electronic system which enables minimally invasive (‘keyhole') transmastoid access to the tympanic cavity. Materials and methods: The auditory implant manipulator is a miniaturised robotic system with five axes of movement and an integrated drill. It can be mounted on the operating table. We evaluated the surgical work field provided by the system, and the work sequence involved, using an anatomical whole head specimen. Results: The work field provided by the auditory implant manipulator is considerably greater than required for conventional mastoidectomy. The work sequence for a keyhole procedure included pre-operative planning, arrangement of equipment, the procedure itself and post-operative analysis. Conclusion: Although system improvements are necessary, our preliminary results indicate that the auditory implant manipulator has the potential to perform keyhole insertion of implantable hearing device

Variation of the cochlear anatomy and cochlea duct length: analysis with a new tablet-based software

PURPOSE In cochlear implantation, thorough preoperative planning together with measurement of the cochlear duct length (CDL) assists in choosing the correct electrode length. For measuring the CDL, different techniques have been introduced in the past century along with the then available technology. A tablet-based software offers an easy and intuitive way to visualize and analyze the anatomy of the temporal bone, its proportions and measure the CDL. Therefore, we investigated the calculation technique of the CDL via a tablet-based software on our own cohort retrospectively. METHODS One hundred and eight preoperative computed tomography scans of the temporal bone (slice thickness < 0.7~mm) of already implanted FLEX28™ and FLEXSOFT™ patients were found eligible for analysis with the OTOPLAN software. Measurements were performed by two trained investigators independently. CDL, angular insertion depth (AID), and cochlear coverage were calculated and compared between groups of electrode types, sex, sides, and age. RESULTS Mean CDL was 36.2 ± 1.8~mm with significant differences between sex (female: 35.8 ± 0.3~mm; male: 36.5 ± 0.2~mm; p = 0.037), but none concerning side or age. Differences in mean AID (FLEX28: 525.4 ± 46.4°; FLEXSOFT: 615.4 ± 47.6°), and cochlear coverage (FLEX28: 63.9 ± 5.6%; FLEXSOFT: 75.8 ± 4.3%) were significant (p < 0.001). CONCLUSION A broad range of CDL was observed with significant larger values in male, but no significant differences concerning side or age. Almost every cochlea was measured longer than 31.0~mm. Preoperative assessment aids in prevention of complications (incomplete insertion, kinking, tipfoldover), attempt of atraumatic insertion, and addressing individual necessities (hearing preservation, cochlear malformation). The preferred AID of 720° (two turns of the cochlea) was never reached, opening the discussion for the requirement of longer CI-electrodes versus a debatable audiological benefit for the patient in his/her everyday life

Flat panel angiography in the cross-sectional imaging of the temporal bone: Assessment of image quality and radiation dose compared with a 64-section multisection CT scanner

BACKGROUND AND PURPOSE: Cross-sectional imaging of the temporal bone is challenging because of the complexity and small dimensions of the anatomic structures. We evaluated the role of flat panel angiography in the cross-sectional imaging of the temporal bone by comparing its image quality and radiation dose with a 64-section multisection CT scanner. MATERIALS AND METHODS: We retrospectively collected 29 multisection CT and 29 flat panel angiography images of normal wholehead temporal bones. Image quality was assessed by 2 neuroradiologists, who rated the visualization of 30 anatomic structures with a 3-point ordinal scale. The radiation dose was assessed with an anthropomorphic phantom. RESULTS: Flat panel angiography showed better image quality than multisection CT in depicting the anterior and posterior crura of the stapes, the footplate of the stapes, the stapedius muscle, and the anterior ligament of the malleus (P &lt; .05). In contrast, multisection CT showed better image quality than flat panel angiography in assessing the tympanic membrane, the bone marrow of the malleus and incus, the tendon of the tensor tympani, the interscalar septum, and the modiolus of the cochlea (P &lt; .05). Flat panel angiography had a significantly higher overall image quality rating than multisection CT (P =.035). A reduction of the effective dose of approximately 40% was demonstrated for flat panel angiography compared with multisection CT. CONCLUSIONS: Flat panel angiography shows strengths and weaknesses compared with multisection CT. It is more susceptible to artifacts, but due to the higher spatial resolution, it shows equal or higher image quality in assessing some bony structures of diagnostic interest. The lower radiation dose is an additional advantage of flat panel angiography