scholarly journals A Case of Intralabyrinthine Schwannoma Removal and Sequential BonebridgeTM Implantation

2021 ◽  
Vol 64 (12) ◽  
pp. 949-953
Author(s):  
Jeong Hae Park ◽  
Jae Sang Han ◽  
Yeonji Kim ◽  
Shi Nae Park
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Benign Tumor ◽  
Bone Conduction ◽  
Tumor Removal ◽  
Hearing Rehabilitation ◽  
Bone Conduction Implant ◽  
Intralabyrinthine Schwannoma ◽  
Rare Benign Tumor ◽  
Cochlear Ossification

Intralabyrinthine schwannoma (ILS) is a rare benign tumor that arises within the membranous labyrinthine. Since hearing loss is inevitable after tumor removal in most cases, an appropriate rehabilitation method should be considered. A 41-year-old male, who was diagnosed with ILS underwent tumor removal via translabyrinthine approach, has subsequently experienced right-side deafness as a result. Seventeen months after the tumor removal, a surgery for hearing rehabilitation was performed. Since cochlear implantation was not doable due to cochlear ossification, an active transcutaneous bone conduction implant (BonebridgeTM , MEDEL) was placed at the sinodural angle. To the best of our knowledge, this is the first report describing hearing rehabilitation with BonebridgeTM implantation after ILS removal.

scholarly journals Challenges of Cochlear Implantation in Intralabyrinthine Schwannoma Patients: Surgical Procedures and Auditory Outcome

2021 ◽  
Vol 10 (17) ◽  
pp. 3899
Author(s):  
Sophia Marie Häussler ◽  
Agnieszka J. Szczepek ◽  
Stefan Gräbel ◽  
Heidi Olze
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Cochlear Implantation ◽  
Benign Tumor ◽  
Tumor Location ◽  
Tumor Removal ◽  
Intralabyrinthine Schwannoma ◽  
Rare Benign Tumor ◽  
Lost To Follow Up

Intralabyrinthine schwannoma (ILS) is a rare benign tumor of the inner ear potentially causing unilateral sensorineural hearing loss and vertigo. This study evaluated the outcome of one surgical session comprising microsurgical ILS resection and cochlear implantation in terms of surgical feasibility, complications, and auditory outcome. Ten clinically and histologically confirmed ILS patients included in this study (three women and seven men; mean age 56.4 ± 8.6) underwent surgery between July 2015 and February 2020. Eight patients had intracochlear tumor location; the remaining two had vestibulocochlear and intravestibular ILS. One of the three following methods was used for tumor removal: an extended cochleostomy, subtotal cochleoectomy, or a translabyrinthine approach. Although negligible improvement was observed in two of the patients, two patients were lost to follow-up, and one opted out from using CI, the speech perception of the five remaining ILS patients improved as per the Freiburg Monosyllable Test (FMT) from 0% before surgery to 45– 50% after the implantation. Our study supports the presented surgical approach’s feasibility and safety, enabling tumor removal and hearing restoration shortly after surgery.

scholarly journals Halláscsökkenést okozó etiológiai tényezők cochlearis implantáción átesett gyermekekben

2019 ◽  
Vol 160 (21) ◽  
pp. 822-828
Author(s):  
Nóra Kecskeméti ◽  
Anita Gáborján ◽  
Magdolna Szőnyi ◽  
Marianna Küstel ◽  
Ildikó Baranyi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Speech Development ◽  
Sensorineural Hearing ◽  
Diagnostic Process ◽  
Sufficient Information ◽  
Hearing Rehabilitation ◽  
Junction Protein ◽  
Newborn Hearing

Abstract: Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1–3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development’s period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822–828.

Sound Localization in Patients With Congenital Unilateral Conductive Hearing Loss With a Transcutaneous Bone Conduction Implant

2017 ◽  
Vol 38 (3) ◽  
pp. 318-324 ◽  
Author(s):  
Erich Vyskocil ◽  
Rudolfs Liepins ◽  
Alexandra Kaider ◽  
Michaela Blineder ◽  
Sasan Hamzavi
Keyword(s):  
Hearing Loss ◽  
Sound Localization ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Bone Conduction Implant ◽  
Conductive Hearing

scholarly journals Audiological Performance of ADHEAR Systems in Simulated Conductive Hearing Loss: A Case Series with a Review of the Existing Literature

2021 ◽  
Vol 11 (4) ◽  
pp. 537-546
Author(s):  
Enrico Muzzi ◽  
Valeria Gambacorta ◽  
Ruggero Lapenna ◽  
Giulia Pizzamiglio ◽  
Sara Ghiselli ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Bone Conduction ◽  
Free Field ◽  
Hearing Threshold ◽  
Case Series ◽  
Binaural Hearing ◽  
Bone Conduction Implant ◽  
Conductive Hearing ◽  
Loudness Summation

A new non-invasive adhesive bone conduction hearing device (ABCD) has been proposed as an alternative solution for reversible bilateral conductive hearing loss in recurrent or long-lasting forms of otitis media with effusion (OME) in children that cannot undergo surgical treatment. Our aim was to assess the effectiveness of ABCD in children with OME. Twelve normal-hearing Italian-speaking volunteers, in whom a conductive hearing loss was simulated, participated in the study. The free-field average hearing threshold was determined and, to evaluate binaural hearing skills, loudness summation and the squelch effect were assessed. Five conditions were tested: (1) unaided without earplugs, (2) unaided with bilateral earplugs, (3) aided right ear with bilateral earplugs, (4) aided left ear with bilateral earplugs, and (5) bilateral aid with bilateral earplugs. Post-hoc analysis showed a significant statistical difference between plugged, unplugged, and each aided condition. The main results were a better loudness summation and a substantial improvement of the squelch effect in the bilaterally aided. Our results suggest that ABCD is a valid treatment for patients with conductive hearing loss that cannot undergo bone conduction implant surgery. It is also important to consider bilateral aids in order to deal with situations in which binaural hearing is fundamental.

scholarly journals The Bonebridge BCI 602 Active Transcutaneous Bone Conduction Implant in Children: Objective and Subjective Benefits

2021 ◽  
Vol 10 (24) ◽  
pp. 5916
Author(s):  
Katarzyna B. Cywka ◽  
Henryk Skarżyński ◽  
Bartłomiej Król ◽  
Piotr H. Skarżyński
Keyword(s):  
Hearing Loss ◽  
Word Recognition ◽  
Middle Ear ◽  
Bone Conduction ◽  
Free Field ◽  
Subjective Assessment ◽  
Congenital Defects ◽  
Mixed Hearing Loss ◽  
Speech Reception ◽  
Bone Conduction Implant

Background: the Bonebridge hearing implant is an active transcutaneous bone conduction implant suitable for various types of hearing loss. It was first launched in 2012 as the BCI 601, with a newer internal part (BCI 602) released in 2019. With the new size and shape, the BCI 602 can be used in patients previously excluded due to insufficient anatomical conditions, especially in patients with congenital defects of the outer and middle ear. Objectives: the purpose of this study is to evaluate the objective and subjective benefits of the new Bonebridge BCI 602 in children who have hearing impairment due to conductive or mixed hearing loss. Safety and effectiveness of the device was assessed. Methods: the study group included 22 children aged 8–18 years (mean age 14.7 years) who had either conductive or mixed hearing loss. All patients were implanted unilaterally with the new Bonebridge BCI 602 implant. Pure tone audiometry, speech recognition tests (in quiet and noise), and free-field audiometry were performed before and after implantation. Word recognition scores were evaluated using the Demenko and Pruszewicz Polish Monosyllabic Word Test, and speech reception thresholds in noise were assessed using the Polish Sentence Matrix Test. The subjective assessment of benefits was carried outusing the APHAB (Abbreviated Profile of Hearing Aid Benefit) questionnaire. Results: after implantation of the Bonebridge BCI 602 all patients showed a statistically significant improvement in hearing and speech understanding. The mean word recognition score (WRS) changed from 12.1% before implantation to 87.3% after 6 months. Mean speech reception threshold (SRT) before implantation was +4.79 dB SNR and improved to −1.29 dB SNR after 6 months. All patients showed stable postoperative results. The APHAB questionnaire showed that difficulties in hearing decreased after implantation, with a statistically significant improvement in global score. Pre-operative scores (M = 35.7) were significantly worse than post-operative scores at 6 months (M = 25.7). Conclusions: the present study confirms that the Bonebridge BCI 602 is an innovative and effective solution, especially for patients with conductive and mixed hearing loss due to anatomical ear defects. The Bonebridge BCI 602 system provides valuable and stable audiological and surgical benefits. Subjective assessment also confirms the effectiveness of the BCI 602. The BCI 602 offers the same amplification as the BCI601, but with a smaller size. The smaller dimensions make it an effective treatment option for a wider group of patients, especially children with congenital defects of the outer and middle ear.

Auditory Outcomes with Hearing Rehabilitation in Children with Unilateral Hearing Loss: A Systematic Review

2017 ◽  
Vol 157 (4) ◽  
pp. 565-571 ◽  
Author(s):  
Swathi Appachi ◽  
Jessica. L. Specht ◽  
Nikhila Raol ◽  
Judith E. C. Lieu ◽  
Michael S. Cohen ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Sound Localization ◽  
Hearing Aids ◽  
Bone Conduction ◽  
Unilateral Hearing Loss ◽  
Hearing Rehabilitation ◽  
Hearing Devices ◽  
Bone Conduction Hearing ◽  
Speech Recognition In Noise

Objective Options for management of unilateral hearing loss (UHL) in children include conventional hearing aids, bone-conduction hearing devices, contralateral routing of signal (CROS) aids, and frequency-modulating (FM) systems. The objective of this study was to systematically review the current literature to characterize auditory outcomes of hearing rehabilitation options in UHL. Data Sources PubMed, EMBASE, Medline, CINAHL, and Cochrane Library were searched from inception to January 2016. Manual searches of bibliographies were also performed. Review Methods Studies analyzing auditory outcomes of hearing amplification in children with UHL were included. Outcome measures included functional and objective auditory results. Two independent reviewers evaluated each abstract and article. Results Of the 249 articles identified, 12 met inclusion criteria. Seven articles solely focused on outcomes with bone-conduction hearing devices. Outcomes favored improved pure-tone averages, speech recognition thresholds, and sound localization in implanted patients. Five studies focused on FM systems, conventional hearing aids, or CROS hearing aids. Limited data are available but suggest a trend toward improvement in speech perception with hearing aids. FM systems were shown to have the most benefit for speech recognition in noise. Studies evaluating CROS hearing aids demonstrated variable outcomes. Conclusions Data evaluating functional and objective auditory measures following hearing amplification in children with UHL are limited. Most studies do suggest improvement in speech perception, speech recognition in noise, and sound localization with a hearing rehabilitation device.

scholarly journals Medical, Technical and Audiological Outcomes of Hearing Rehabilitation with the Bonebridge Transcutaneous Bone-Conduction Implant: A Single-Center Experience

2019 ◽  
Vol 8 (10) ◽  
pp. 1614 ◽  
Author(s):  
Brkic ◽  
Riss ◽  
Scheuba ◽  
Arnoldner ◽  
Gstöttner ◽  
...  
Keyword(s):  
Hearing Aids ◽  
Bone Conduction ◽  
Technical Failure ◽  
Single Center ◽  
Hearing Rehabilitation ◽  
Device Failure ◽  
Hearing Gain ◽  
Bone Conduction Implant ◽  
The Mean

Bone-conduction implants are a standard therapeutic option for patients with conductive, unilateral, or mixed hearing loss who either do not tolerate conventional hearing aids or can benefit from surgery. The aim of this study was to evaluate long-term medical and technical outcomes, and audiological results with the Bonebridge transcutaneous bone-conduction implant. This retrospective study included all patients implanted with a bone-conduction hearing implant at a tertiary medical referral center between March 2012 and October 2018. Medical and technical outcomes included the mean length of implant usage, medical and technical complications (skin and wound infection, lack of benefit, technical failure), explantations and revisions, coupling approaches, implant failure rate, implant survival and the implant loss for added follow-up years. Auditory results were measured by functional hearing gain and the Freiburger monosyllabic test at 65 dB sound pressure level. Sixty-four patients were included in the study; five of these were implanted bilaterally (69 devices). Five unilaterally implanted patients were lost to follow-up. The mean follow-up was 27.1 months (range: 0.2 months–6.3 years). The mean implant usage was 25.9 months (range: 0.2 months–6.3 years). Fifty-seven implants (89.1%) were in use at the end of the follow-up period. Complications occurred in six ears (9.4%). Five implants (7.8%) were explanted without reimplantation. Device failure occurred in one implant (1.6%), which was possibly caused by recurrent head trauma. The rate of implant loss due to technical device failure (damage to device) was 1 per 72 follow-up years. The mean improvement on the Freiburger monosyllabic test (52.1%, p = 0.0001), and in functional hearing gain across frequencies (26.5 dB, p = 0.0001) was significant. This single-center follow-up reveals the medical and technical reliability of a transcutaneous bone-conduction implant for hearing rehabilitation because complication and revision rates were low. The majority of patients still used the device at the end of the observation period. Implantation resulted in favorable hearing outcomes in comparison to that of unaided conditions. Cautious patient selection mainly regarding co-morbidities, the history of chronic otologic diseases and proper surgical technique seems to be crucial in reducing complications.

Cochlear implantation in superficial siderosis

1996 ◽  
Vol 110 (12) ◽  
pp. 1151-1153 ◽  
Author(s):  
Richard M. Irving ◽  
John M. Graham
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hearing Loss ◽  
Cochlear Implantation ◽  
Superficial Siderosis ◽  
Nervous System Disorder ◽  
Central Nervous System Disorder ◽  
Hearing Rehabilitation ◽  
System Disorder ◽  
Careful Assessment

AbstractSuperficial siderosis is a rare central nervous system disorder characterized by deafness, ataxia, and pyramidal signs. The hearing loss is believed to be predominantly neural and is usuallly progressive and bilateral. Careful assessment is therefore necessary to determine the best approach to hearing rehabilitation. A case is presented of usperficial siderosis in a young woman who has benegited significantly from cochlear implantation using the Nucleus dvice.

scholarly journals TMPRSS3 Gene Variants With Implications for Auditory Treatment and Counseling

2021 ◽  
Vol 12 ◽  
Author(s):  
In Seok Moon ◽  
Andrew R. Grant ◽  
Varun Sagi ◽  
Heidi L. Rehm ◽  
Konstantina M. Stankovic
Keyword(s):  
Hearing Loss ◽  
Genetic Testing ◽  
Cochlear Implantation ◽  
Clinical Manifestations ◽  
Clinical Information ◽  
Hearing Rehabilitation ◽  
Novel Variants ◽  
Novel Variant ◽  
Number Variation ◽  
Syndromic Hearing Loss

Objective: To identify and report novel variants in the TMPRSS3 gene and their clinical manifestations related to hearing loss as well as intervention outcomes. This information will be helpful for genetic counseling and treatment planning for these patients.Methods: Literature review of previously reported TMPRSS3 variants was conducted. Reported variants and associated clinical information was compiled. Additionally, cohort data from 18 patients, and their families, with a positive result for TMPRSS3-associated hearing loss were analyzed. Genetic testing included sequencing and copy number variation (CNV) analysis of TMPRSS3 and the Laboratory for Molecular Medicine’s OtoGenome-v1, -v2, or -v3 panels. Clinical data regarding patient hearing rehabilitation was interpreted along with their genetic testing results and in the context of previously reported cochlear implant outcomes in individuals with TMPRSS3 variants.Results: There have been 87 previously reported TMPRSS3 variants associated with non-syndromic hearing loss in more than 20 ancestral groups worldwide. Here we report occurrences of known variants as well as one novel variant: deletion of Exons 1–5 and 13 identified from our cohort of 18 patients. The hearing impairment in many of these families was consistent with that of previously reported patients with TMPRSS3 variants (i.e., typical down-sloping audiogram). Four patients from our cohort underwent cochlear implantation.Conclusion: Bi-allelic variants of TMPRSS3 are associated with down-sloping hearing loss regardless of ancestry. The outcome following cochlear implantation in patients with variants of TMPRSS3 is excellent. Therefore, cochlear implantation is strongly recommended for hearing rehabilitation in these patients.

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