scholarly journals Cochlear implantation in Van der Hoeve de Kleyn syndrome with severe hearing loss and deafness

2020 ◽  
Vol 19 (4) ◽  
pp. 94-101
Author(s):  
Kh. M. Diab ◽  
◽  
N. A. Daikhes ◽  
V. S. Korvyakov ◽  
A. A. Kaibov ◽  
...  

Van der Hoove syndrome is an inherited genetically determined generalized connective tissue disorder characterized by the formation of „pathologically immature type I collagen“. The literature describes the results of cochlear implantation in patients with van der Howe syndrome, after which there are often complications both intraoperative and postoperative. This article describes two clinical cases of van der Howe syndrome with severe hearing loss and deafness. In cases described: preoperative diagnosis and features of the surgical stage of cochlear implantation and postoperative results. Diagnostic features – thinning of bone tissue according to CT of temporal bones, expansion of cochlear duct, deafness according to tonal threshold audiometry. Cochlear implantation performed with the use of the endotracheal anesthesia under the control of the microscope, straight electrode grids were used, testing was normal. A distinctive feature of this syndrome is the softness of the cochlea’s bone tissue, the danger is that when the electrode array is inserted, it may fall outside the cochlea. Also, during the first connection or later, there may be pathological stimulation of the facial nerve.

2018 ◽  
Vol 22 (4) ◽  
pp. 25-30
Author(s):  
Sergey A. Ivanov ◽  
Sergey G. Zhuravskii

Paget’s disease of bone is a localized disorder of bone remodeling. These functional and structural alterations, interacting with the specific characteristics of the site of involvement, account for most of the complications of the disease. Bilateral progressive hearing loss is the most frequently encountered complication of Paget’s disease, because of the involvement of the temporal bones. There may be difficulty in distinguishing patients with presbycusis from those with Paget’s disease-related hearing loss. Pagetic hearing loss seems to be stabilized by effective medical treatment. For patients with severe hearing loss, cochlear implantation may be recommended.


2004 ◽  
Vol 132 (9-10) ◽  
pp. 302-305 ◽  
Author(s):  
Rade Kosanovic ◽  
Zoran Ivankovic ◽  
Sandra Stojanovic

During the last several decades, cochlear implant has been fully recognized in treatment of severe hearing loss. Development of modern technology enabled inconceivable possibilities of technical qualities of the device as well as development of usable coding strategies, which led to extraordinary results in patient rehabilitation. Although cochlear implantation has become one of the routine operative procedures throughout the world nowadays, it gives rise to certain complications. These complications, though rare, can sometimes be very serious, even with fatal outcome. If cochlear implantation is performed by experienced and well-educated team of experts, the possibility of complications is minimal and is certainly not the argument against cochlear implantation as a method of treatment of severe hearing impairments.


2019 ◽  
Vol 43 (4) ◽  
pp. 2002-2010 ◽  
Author(s):  
Bo Zheng ◽  
Caiyun Mao ◽  
Tianyi Gu ◽  
Haihua Pan ◽  
Changyu Shao ◽  
...  

This novel biomimetic mineralization technique provides an efficient method to produce an advanced mineralized matrix.


2021 ◽  
Vol 8 ◽  
Author(s):  
Huan Jia ◽  
Jinxi Pan ◽  
Wenxi Gu ◽  
Haoyue Tan ◽  
Ying Chen ◽  
...  

Background: As an advanced surgical technique to reduce trauma to the inner ear, robot-assisted electrode array (EA) insertion has been applied in adult cochlear implantation (CI) and was approved as a safe surgical procedure that could result in better outcomes. As the mastoid and temporal bones are generally smaller in children, which would increase the difficulty for robot-assisted manipulation, the clinical application of these systems for CI in children has not been reported. Given that the pediatric candidate is the main population, we aim to investigate the safety and reliability of robot-assisted techniques in pediatric cochlear implantation.Methods: Retrospective cohort study at a referral center in Shanghai including all patients of simultaneous bilateral CI with robotic assistance on one side (RobOtol® system, Collin ORL, Bagneux, France), and manual insertion on the other (same brand of EA and CI in both side), from December 2019 to June 2020. The surgical outcomes, radiological measurements (EA positioning, EA insertion depth, mastoidectomy size), and audiological outcomes (Behavior pure-tone audiometry) were evaluated.Results: Five infants (17.8 ± 13.5 months, ranging from 10 to 42 months) and an adult (39 years old) were enrolled in this study. Both perimodiolar and lateral wall EAs were included. The robot-assisted EA insertion was successfully performed in all cases, although the surgical zone in infants was about half the size in adults, and no difference was observed in mastoidectomy size between robot-assisted and manual insertion sides (p = 0.219). The insertion depths of EA with two techniques were similar (P = 0.583). The robot-assisted technique showed no scalar deviation, but scalar deviation occurred for one manually inserted pre-curved EA (16%). Early auditory performance was similar to both techniques.Conclusion: Robot-assisted technique for EA insertion is approved to be used safely and reliably in children, which is possible and potential for better scalar positioning and might improve long-term auditory outcome. Standard mastoidectomy size was enough for robot-assisted technique. This first study marks the arrival of the era of robotic CI for all ages.


Materials ◽  
2019 ◽  
Vol 12 (22) ◽  
pp. 3719 ◽  
Author(s):  
Giorgia Montalbano ◽  
Giorgia Borciani ◽  
Carlotta Pontremoli ◽  
Gabriela Ciapetti ◽  
Monica Mattioli-Belmonte ◽  
...  

In the last years bone tissue engineering has been increasingly indicated as a valid solution to meet the challenging requirements for a healthy bone regeneration in case of bone loss or fracture. In such a context, bioactive glasses have already proved their great potential in promoting the regeneration of new bone tissue due to their high bioactivity. In addition, their composition and structure enable us to incorporate and subsequently release therapeutic ions such as strontium, enhancing the osteogenic properties of the material. The incorporation of these inorganic systems in polymeric matrices enables the formulation of composite systems suitable for the design of bone scaffolds or delivery platforms. Among the natural polymers, type I collagen represents the main organic phase of bone and thus is a good candidate to develop biomimetic bioactive systems for bone tissue regeneration. However, alongside the specific composition and structure, the key factor in the design of new biosystems is creating a suitable interaction with cells and the host tissue. In this scenario, the presented study aimed at combining nano-sized mesoporous bioactive glasses produced by means of a sol–gel route with type I collagen in order to develop a bioactive hybrid formulation suitable for bone tissue engineering applications. The designed system has been fully characterized in terms of physico-chemical and morphological analyses and the ability to release Sr2+ ions has been studied observing a more sustained profile in presence of the collagenous matrix. With the aim to improve the mechanical and thermal stability of the resulting hybrid system, a chemical crosslinking approach using 4-star poly (ethylene glycol) ether tetrasuccinimidyl glutarate (4-StarPEG) has been explored. The biocompatibility of both non-crosslinked and 4-StarPEG crosslinked systems was evaluated by in vitro tests with human osteoblast-like MG-63 cells. Collected results confirmed the high biocompatibility of composites, showing a good viability and adhesion of cells when cultured onto the biomaterial samples.


2011 ◽  
Vol 493-494 ◽  
pp. 844-848 ◽  
Author(s):  
Marcia S. Sader ◽  
Gutemberg Alves ◽  
Racquel Z. LeGeros ◽  
Gloria Dulce de Almeida Soares

Natural bone constitutes of an inorganic phase (a biological nanoapatite) and an organic phase (mostly type I collagen). The challenge is to develop a material that can regenerate lost bone tissue with degradation and resorption kinetics compatible with the new bone formation. The aim of this study was to prepare self-organized magnesium and carbonate substituted apatite/collagen scaffolds, cross-linked with glutaraldehyde (GA). Bovine tendon was submitted to alkaline treatment resulting in a negatively charged collagen surface. The scaffolds were prepared by precipitation: simultaneous dropwise addition of solution containing calcium (Ca) and magnesium (Mg) ions and collagen into a buffered solution containing carbonate and phosphate ions in reaction vessel maintained at 37 °C, pH=8. The reaction products were cross-linked with 0.125 and 0.25% (v/v) glutaraldehyde (GA) solution and freeze-dried. The samples were characterized by Fourier-transformed infrared spectroscopy (FTIR). In vitro cytotoxicity (based on three parameters assays) and scaffolds degradation in culture medium and osteoblastic cells culture were performed in the cross-linked materials. No cytotoxic effects were observed. The cross-linked samples with the lower GA concentration showed a lower stability when placed in contact with culture medium. Human osteoblasts attached on the scaffolds surface cross-linked with 0.25% GA, forming a continuous layer after 14 days of incubation. These results showed potential application of the designed scaffolds for bone tissue engineering.


2001 ◽  
Vol 110 (9) ◽  
pp. 883-891 ◽  
Author(s):  
Joseph B. Nadol ◽  
Barbara J. Burgess ◽  
Bruce J. Gantz ◽  
Newton J. Coker ◽  
Darlene R. Ketten ◽  
...  

The insertion of an intrascalar electrode array during cochlear implantation causes immediate damage to the inner ear and may result in delayed onset of additional damage that may interfere with neuronal stimulation. To date, there have been reports on fewer than 50 temporal bone specimens from patients who had undergone implantation during life. The majority of these were single-channel implants, whereas the majority of implants inserted today are multichannel systems. This report presents the histopathologic findings in temporal bones from 8 individuals who in life had undergone multichannel cochlear implantation, with particular attention to the type and location of trauma and to long-term changes within the cochlea. The effect of these changes on spiral ganglion cell counts and the correlation between speech comprehension and spiral ganglion cell counts were calculated. In 4 of the 8 cases, the opposite, unimplanted ear was available for comparison. In 3 of the 4 cases, there was no significant difference between the spiral ganglion cell counts on the implanted and unimplanted sides. In addition, in this series of 8 cases, there was an apparent negative correlation between residual spiral ganglion cell count and hearing performance during life as measured by single-syllable word recognition. This finding suggests that abnormalities in the central auditory pathways are at least as important as spiral ganglion cell loss in limiting the performance of implant users.


2017 ◽  
Vol 131 (3) ◽  
pp. 209-214 ◽  
Author(s):  
S W Song ◽  
B C Jun ◽  
H Kim

AbstractObjective:To evaluate the clinical and radiological aspects of otic capsule sparing temporal bone fractures.Methods:Using medical records, 188 temporal bones of 173 patients with otic capsule sparing temporal bone fractures were evaluated. Otoscopic findings and symptoms, facial paralysis, and hearing loss were assessed.Results:Using regional analysis, 7 fractures were classified as type I, 85 as type II, 169 as type III and 114 as type IV. Fourteen of the 17 facial paralysis cases improved to House–Brackmann grade II or lower at an average of 57.6 days after the initial evaluation. Thirty-one patients underwent initial and follow-up pure tone audiometry examinations. The air–bone gap closed significantly from 27.2 dB at an average of 21.8 days post-trauma to 19.6 dB at an average of 79.9 days post-trauma, without the need for surgical intervention.Conclusion:Initial conservative treatment for facial paralysis or conductive hearing loss is possible in otic capsule sparing fracture cases after careful evaluation of the patient.


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