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

A scoping review

Funding Information: LA is funded by King Abdul-Aziz University Hospital (KAU), Jeddah, Kingdom of Saudi Arabia. DH and MS are funded through the National Institute for Health and Care Research (NIHR) Biomedical Research Centre programme. The views expressed are those of the authors and not necessarily those of the NIHR, the NHS, the Department of Health, and Social Care. CC is funded by UNICAMP University of Campinas, Brazil, HH is funded by ENT Department, Hospital Cuf Tejo—Nova Medical School, Lisbon, Portugal and finally RD is funded by Sir Peter Mansfield Imaging Centre, School of Physics and Astronomy, University of Nottingham, United Kingdom. The funders had no role in preparation of the manuscript. Publisher Copyright: Copyright © 2022 Alzahrani, Sereda, Chamouton, Haider, Dewey and Hoare.Background: Tinnitus is defined as the subjective perception of sound in the absence of an external stimulus, and tinnitus disorder becomes relevant when it is associated with emotional distress, cognitive dysfunction, and/or autonomic arousal. Hearing loss is recognized as the main risk factor for the pathogenesis of tinnitus. However, clinical guidelines for tinnitus disorder provide little direction for those who have severe-to-profound hearing loss including those who are pre-lingually Deaf. The aim of this scoping review was to catalogue what is known from the existing literature regarding the experience and management of tinnitus in adults who have a severe-to-profound hearing loss. Summary: A scoping review was conducted following the Preferred Reporting Item for Systematic Reviews and Meta-analysis extension for Scoping Reviews. Records were included if they reported an evaluation of tinnitus in adults who had severe-to-profound hearing loss. The online databases Ovid (MEDLINE, EMBASE and PsycINFO), CINAHL, ProQuest, Scopus, and Google Scholar were searched using the search terms ‘tinnitus’ (as a MESH term) and ‘deaf’ OR ‘profound hearing loss. Thirty-five records met the inclusion criteria for this review and were cataloged according to three major themes: Impact of tinnitus in deaf adults; Primary treatment of tinnitus in deaf adults; and Cochlear implant studies where tinnitus was a secondary outcome. Tinnitus symptom severity was assessed before and after intervention using tinnitus validated questionnaires in 29 records, with six further records using other assessment tools to measure tinnitus severity. Participants using cochlear implants were included in 30 studies. Medication, repetitive transcranial magnetic stimulation (rTMS), electrical promontory stimulation, and behavioral self-control therapy were each reported in single records. Key messages: This scoping review cataloged the experience, assessment, and treatment of tinnitus in adults who have severe-to-profound hearing loss. It is shown that there is very limited research reported in this field. Although this review included many records, most focused on the provision of cochlear implants for severe-to-profound hearing loss, with assessment and measurement of tinnitus as a baseline or secondary outcome. Largely missing in the literature are empirical studies that seek firstly to understand the nature of the experience of tinnitus by people with no or little residual access to external sound.publishersversionpublishe

Technical challenges and safety of magnetic resonance imaging with in situ neuromodulation from spine to brain

PURPOSE: This review summarises the need for MRI with in situ neuromodulation, the key safety challenges and how they may be mitigated, and surveys the current status of MRI safety for the main categories of neuro-stimulation device, including deep brain stimulation, vagus nerve stimulation, sacral neuromodulation, spinal cord stimulation systems, and cochlear implants. REVIEW SUMMARY: When neuro-stimulator systems are introduced into the MRI environment a number of hazards arise with potential for patient harm, in particular the risk of thermal injury due to MRI-induced heating. For many devices however, safe MRI conditions can be determined, and MRI safely performed, albeit with possible compromise in anatomical coverage, image quality or extended acquisition time. CONCLUSIONS: The increasing availability of devices conditional for 3 T MRI, whole-body transmit imaging, and imaging in the on-stimulation condition, will be of significant benefit to the growing population of patients benefitting from neuromodulation therapy, and open up new opportunities for functional imaging research

Doctor of Philosophy

dissertationFor many with severe-to-profound hearing loss, a condition in which the cochlea is unable to convert sound vibration into neural information to the brain, the cochlear implant has become the standard treatment. The goal of a cochlear-implant system is to bypass the malfunctioned cochlea and directly stimulate the nerves responsible for hearing through an array of electrodes on a silicone-elastomer carrier. However, the insertion of the electrode arrays can often cause intracochlear damage and eliminate residual hearing. With increased focus on hearing preservation in cochlear implantation, methods to minimize intracochlear damage have become a priority in electrode-array insertions. This dissertation explores the application of magnetic manipulation toward improved cochlear-implant electrode-array insertions. We start with initial 3-to-1 proof-of-concept experiments to demonstrate the feasibility of this approach. Then, to achieve relevancy at clinical scale, lateral-wall-type electrode-array models, used in the clinic, are slightly modified at the tip to include a tiny magnet. Next, a scala-tympani phantom is designed with both simulated cochleostomy and round-window openings to mimic both classes of insertions typically conducted. In particular, this is the first phantom to model a round-window opening and can be used reliably to simulate insertion forces in cadaver cochleae. Electrode arrays are then magnetically guided through these phantoms with a statistically significant (p < 0.05) reduction in insertion forces, and by as much as 50% for some electrode-array models. In particular, guiding the electrode-array tip through the cochlear hook and the basal turn, in the same insertion, was demonstrated for the first time using this technology. All existing methods to guide the electrode array can only be accomplished for the basal turn. Analysis is conducted to determine the optimal size and placement of a magnetic dipole-field source for use in the clinic. Its placement is determined to be consistently lateral to and anterior to the patient’s cochlea. Its size depends on numerous factors including the patient, torque requirements, and registration error. Sensitivity curves summarizing these factors are provided. The volume of the magnetic dipole-field source can be reduced by a factor of 5, on average, by moving it from the modiolar configuration originally proposed to this optimal configuration. We verify that magnetic forces do not pose any appreciable risk to the basilar membrane at the optimal configuration. Although patient-specific optimal configurations are characterized, a one-size-fits-all version is described that may be more practical and carries the benefit of substantial robustness to registration error

American Cochlear Implant Alliance Task Force guidelines for clinical assessment and management of adult cochlear implantation for single-sided deafness

The indications for cochlear implantation have expanded to include individuals with profound sensorineural hearing loss in the impaired ear and normal hearing (NH) in the contralateral ear, known as single-sided deafness (SSD). There are additional considerations for the clinical assessment and management of adult cochlear implant candidates and recipients with SSD as compared to conventional cochlear implant candidates with bilateral moderate to profound sensorineural hearing loss. The present report reviews the current evidence relevant to the assessment and management of adults with SSD. A systematic review was also conducted on published studies that investigated outcomes of cochlear implant use on measures of speech recognition in quiet and noise, sound source localization, tinnitus perception, and quality of life for this patient population. Expert consensus and systematic review of the current literature were combined to provide guidance for the clinical assessment and management of adults with SSD

Neurosurgical interventions at the cochlear nerve &amp; nucleus for treatment of tinnitus

Tinnitus is the perception of sound or noise in the absence of a physical sound source. For some patients, tinnitus has a significant impact on their quality of life and conventional treatment options can be insufficient. In this thesis, we evaluated and investigated several neurosurgical treatment options for patients with severe, intractable tinnitus. Tinnitus can be the result of compression of the cochlear nerve with a blood vessel (neurovascular conflict). In this thesis, we showed that a decompression surgery for this conflict has a low success percentage and a relatively high surgical complication rate. However, when patients experience both tinnitus and vertigo, the success percentage is considerably higher. A neurovascular conflict may be diagnosed with MR-imaging, however they do not always relate to ipsilateral symptoms. Our research showed that the type of compression, e.g. causing a dimple in the nerve, is not a good indicator for a symptomatic neurovascular conflict.In order to reduce tinnitus, neurostimulation can be performed. The effect of direct stimulation of the cochlear nerve with a ring electrode was evaluated, but appeared to be not a viable treatment option as damage of the nerve lead to increase in hearing loss in the majority of patients. Another novel option is neurostimulation of the cochlear nucleus in the brainstem with an auditory brainstem implant (ABI). We described a pilot study studying the effect of the ABI on tinnitus. Preliminary results (one year follow-up) in two patients show a stable tinnitus reduction. Also, hearing ability was undamaged after implantation

An investigation of the biological effects of electromagnetic fields and risk assessment of magnetic resonance imaging systems

Thesis (Nucl. E.)--Massachusetts Institute of Technology, Dept. of Nuclear Engineering, 1994.Includes bibliographical references (leaves 22-24).by Mahmood A. Cheema.Nucl.E

MRI safety and devices: An update and expert consensus

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/154533/1/jmri26909_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/154533/2/jmri26909.pd

Electrosurgery and Implantable Electronic Devices: Review and Implications for Office‐Based Procedures

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/86867/1/j.1524-4725.2011.02006.x.pd