scholarly journals Hearing Rehabilitation with Cochlear Implants after CyberKnife Radiosurgery of Vestibular Schwannoma: A Report Based on Four Clinical Cases

2021 ◽  
Vol 11 (12) ◽  
pp. 1646
Author(s):  
Sophia M. Häußler ◽  
Agnieszka J. Szczepek ◽  
Stefan Gräbel ◽  
Carolin Senger ◽  
Franziska Löbel ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Vestibular Schwannoma ◽  
Cochlear Implantation ◽  
Pure Tone Audiometry ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Profound Hearing Loss ◽  
Hearing Rehabilitation ◽  
Auditory Rehabilitation ◽  
Open Set

Severe sensorineural hearing loss can be a symptom of the benign tumor vestibular schwannoma (VS). The treatment of VS with non-invasive stereotactic radiosurgery (SRS) offers a high local tumor control rate and an innovative possibility of sequential hearing rehabilitation with cochlear implantation. This study evaluated the feasibility, complications, and auditory outcomes of such a therapeutic approach. Three males and one female (mean age 65.3 ± 9.4 years) scheduled for cochlear implantation and diagnosed with sporadic VS classified as T1 or T2 (according to Samii) were enrolled in this study. All patients had progressive hearing loss qualifying them for cochlear implantation. First, the tumor was treated using CyberKnife SRS. Next, sequential auditory rehabilitation with a cochlear implant (CI) was performed. Clinical outcomes and surgical feasibility were analyzed, and audiological results were evaluated using pure tone audiometry and speech recognition tests. All patients exhibited open-set speech understanding. The mean word recognition score (at 65 dB SPL, Freiburg Monosyllabic Test, FMT) improved after cochlear implantation in all four patients from 5.0 ± 10% (with hearing aid) preoperatively to 60.0 ± 22.7% six months postoperatively. Our results suggest that in patients with profound hearing loss caused by sporadic vestibular schwannoma, the tumor removal with SRS followed by cochlear implantation is an effective method of auditory rehabilitation.

Current indications for cochlear implantation in adults and children

2019 ◽  
Vol 73 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Witold Szyfter ◽  
Michał Karlik ◽  
Alicja Sekula ◽  
Simon Harris ◽  
Wojciech Gawęcki
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Unilateral Deafness ◽  
Bilateral Sensorineural Hearing Loss ◽  
Asymmetric Hearing Loss

Introduction: Surgical treatment of deafness by cochlear implants is used for more than 40 years, and during this period permanently, gradual and significant expansion of indications for this surgery has been observed. Material and methods: In our Department in the years 1994-2018 1480 cochlear implantations were performed, both in adults (647) and in children (883). In this study current indications and the rules for eligibility of patients based on 25 years of experience are presented. Results: Indications for cochlear implantation in adults are: 1) bilateral postlingual deafness, 2) bilateral sensorineural hearing loss - in pure tone audiometry > 70 dB HL (average 500-4000 Hz) and in speech audiometry in hearing aids understanding < 50% of words for the intensity of the stimulus 65 dB, in the absence of the benefits of hearing aids, 3) bilateral profound hearing loss for high frequency with good hearing for low frequency, in the absence of the benefits of hearing aids, 4) some cases of asymmetric hearing loss with intensive tinnitus in the deaf ear. An indication in children is bilateral sensorineural hearing loss > 80dB HL confirmed by hearing tests, after about 6 months of rehabilitation with the use of hearing aids. Discussion: Although cochlear implantation is used for more than 40 years, the indications for this treatment underlies constant modifications. They concern the age of eligible patients, implantation in patients with partially preserved hearing, as well as treatment for patients with difficult anatomical conditions. In many countries, bilateral implantations are commonly performed, and more and more centers recommend this treatment in the case of unilateral deafness or asymmetric hearing loss, especially with the accompanying tinnitus in the deaf ear.

scholarly journals Two Down Syndrome Patients with Bilateral Profound Hearing Loss: Case Report and Literature Review

2018 ◽  
Vol 1 (2) ◽  
pp. 8
Author(s):  
Yu Zheng ◽  
Juan-Mei Yang ◽  
Meng Zhao ◽  
Xiao-Qing Qian ◽  
Fang-Lu Chi
Keyword(s):  
Hearing Loss ◽  
Down Syndrome ◽  
Cochlear Implantation ◽  
Review Literature ◽  
Profound Hearing Loss ◽  
Rehabilitation Process ◽  
Hearing Rehabilitation ◽  
Suitability Assessment ◽  
First Case ◽  
Low Incidence

Hearing loss is not uncommon among patients with Down syndrome (DS). It has been reported in 38–78% of the Down syndrome population. However, profound hearing loss in DS patients is rarely noticed due to its low incidence. In this article, we reported two Down syndrome patients with bilateral profound hearing loss in two cases. The first case involved an eight-year-old DS child experiencing extremely severe defects in terms of language and severe defects in terms of gross motor function, adaptability, and sociability. The second case revolved around another DS child with bilateral cochlear nerve absence. We review literature on the DS patients with hearing loss and conclude that profound sensorineural hearing loss in those patients has not received enough attention so far. We also recommend that cochlear implantation (CI) suitability assessment and timely intervention via cochlear implantation are necessary in DS patients. Besides, benefits from CI would be limited and hearing rehabilitation process could be much slower when compared with children without additional inabilities.

scholarly journals Intralabyrinthine Schwannomas: Disease Presentation, Tumor Management, and Hearing Rehabilitation

2019 ◽  
Vol 80 (02) ◽  
pp. 196-202 ◽  
Author(s):  
Baishakhi Choudhury ◽  
Matthew Carlson ◽  
Daniel Jethanamest
Keyword(s):  
Magnetic Resonance Imaging ◽  
Hearing Loss ◽  
Magnetic Resonance ◽  
Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Resonance Imaging ◽  
Hearing Rehabilitation ◽  
Rare Tumors ◽  
Auditory Rehabilitation ◽  
Tumor Management

AbstractIntralabyrinthine schwannomas (ILS) are rare tumors that frequently cause sensorineural hearing loss. The development and increased use of magnetic resonance imaging in recent years have facilitated the diagnosis of these tumors that present with otherwise nondiscriminant symptoms such as tinnitus, vertigo, and hearing loss. The following is a review of the presentation, pathophysiology, imaging, and treatment with a focused discussion on auditory rehabilitation options of ILS.

Hearing Rehabilitation in a Patient with Sudden Sensorineural Hearing Loss in the Only Hearing Ear

2008 ◽  
Vol 19 (03) ◽  
pp. 267-274 ◽  
Author(s):  
David B. Hawkins
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
The Other ◽  
Profound Hearing Loss ◽  
Hearing Rehabilitation ◽  
Aural Rehabilitation ◽  
Product Manager ◽  
Software Product

A case report is presented of a 62-year-old software product manager who had normal hearing in one ear and a congenital profound hearing loss in the other ear and then sustained a sudden sensorineural hearing loss in the only hearing ear. The approach to amplification decisions, cochlear implant evaluation, and rehabilitation options are discussed. Providing aural rehabilitation and continually updating and providing new amplification options and accessories are described. Se presenta un reporte de caso de un gerente de productos de software de 62 años de edad quien tenía audición normal en un oído y un sordera congénita profunda en el otro, y quién súbitamente sufrió una sordera sensorineural súbita en el único oído con audición. Se discute el enfoque de decisiones de amplificación, la evaluación para implante coclear, y las opciones de rehabilitación. Se describen las pautas para proveer rehabilitación aural y para actualizar continuamente y aportar nuevas opciones de amplificación.

Cochlear Implant Outcomes in Patients with Neurosarcoidosis

2021 ◽  
pp. 1-7
Author(s):  
Dario Ebode ◽  
Fleur Cohen-Aubart ◽  
Stéphanie Trunet ◽  
Evelyne Ferrary ◽  
Ghizlène Lahlou ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Corticosteroid Treatment ◽  
Auditory Neuropathy ◽  
Disease Exacerbation ◽  
Profound Hearing Loss ◽  
Hearing Recovery ◽  
Speech Performance

<b><i>Introduction:</i></b> Audiovestibular symptoms are rare in sarcoidosis, but they may also be the first manifestation of the disease. Sudden or progressive bilateral hearing loss is usually associated with vestibular impairment. The mechanism of hearing loss remains unclear, but clinical presentation and magnetic resonance imaging suggest a retrocochlear site for the lesion in most patients. Several cases of hearing recovery after corticosteroid treatment have been reported. In patients with severe or profound hearing loss, the benefit of cochlear implantation is challenging to predict in the case of auditory neuropathy and is rarely described. We present a case series of cochlear implantation in patients with documented neurosarcoidosis. <b><i>Results:</i></b> Seven cases of cochlear implantation in 4 patients with neurosarcoidosis are reported. All of the patients showed a great improvement very quickly in both quiet and noise. Speech performance remained stable over time with a follow-up ranging from 4 to 11 years, even in patients who had disease exacerbation. <b><i>Conclusion:</i></b> Cochlear implantation is possible in deaf patients with neurosarcoidosis. The excellent benefit obtained in our patients suggests a particular type of neuropathy, but endocochlear involvement cannot be entirely ruled out.

Delayed Cochlear Implantation in Adults With Prelingual Severe-to-Profound Hearing Loss

2011 ◽  
Vol 32 (2) ◽  
pp. 223-228 ◽  
Author(s):  
Won Sun Yang ◽  
In Seok Moon ◽  
Hee Nam Kim ◽  
Won-Sang Lee ◽  
Sung Eun Lee ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Profound Hearing Loss

Outcome of Cochlear Implantation in Postmeningitis Profound Sensorineural Hearing Loss at Rajavithi Hospital

2021 ◽  
Vol 104 (2) ◽  
pp. 260-263
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Sensorineural Hearing ◽  
Preoperative Imaging ◽  
Profound Hearing Loss ◽  
Language Status ◽  
Auditory Performance ◽  
Electrode Insertion ◽  
One Year

Background: Bacterial meningitis is one of the major factors in the etiology of acquired sensorineural hearing loss in children and adults. Cochlear implantation in these patients is challenging because of inner ear ossification and fibrosis, and this procedure sometimes achieves poorer outcomes in this scenario than with other causes of sensorineural hearing loss. There has been little research into the factors affecting the outcomes of this procedure. Objective: To evaluate the outcomes of cochlear implantation in patients with postmeningitis profound sensorineural hearing loss and to evaluate the factors that affect the results. Materials and Methods: A retrospective review was conducted of thirty patients who were diagnosed with post meningitis profound hearing loss and underwent cochlear implantation at Rajavithi Hospital between 2001 and 2016. Preoperative language status, duration of deafness, preoperative imaging, and degree of electrode insertion were recorded. Categories of auditory performance-II test (CAP-II) was evaluated in all cases, one year postoperative. Results: Thirty postmeningitis deafness patients underwent cochlear implantation. The median age at diagnosis of meningitis and age at implantation were 41 years (range 1 to 75) and 49.50 years (range 3 to 75), respectively. The median duration of deafness was 12 months (range 4 to 300), and the overall mean CAP-II at one year after surgery was 5.47±2.21. The postlinguistic group had a significantly higher CAP-II score than the prelinguistic one (p=0.006). Electrodes were successfully totally inserted in 19 patients (63.3%) and partially inserted in 11 (36.7%). The average CAP-II score in the group with fully-inserted electrodes was significantly higher than in the group with partially-inserted electrodes (p=0.045). There was no correlation between CAP-II score and age at meningitis diagnosis (p=0.069), age at time of surgery (p=0.105), duration of deafness (p=0.506), or preoperative CT (p=0.228) or MRI abnormality (p=0.078). Conclusion: Cochlear implantation in patients with postmeningitis profound hearing loss had high success rates and favorable outcomes. Preoperative language status and degree of electrode insertion were factors that affected auditory performance results. Keywords: Cochlear implantation, Postmeningitis hearing loss, Sensorineural hearing loss, Meningitis, Rajavithi Hospital

Cochlear Implantation Outcomes in Children With CDH23 Mutations–Associated Hearing Loss

2021 ◽  
pp. 019459982110574
Author(s):  
Kaitian Chen ◽  
Bixue Huang ◽  
Jincangjian Sun ◽  
Yue Liang ◽  
Guanxia Xiong
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Outer Hair Cell ◽  
Cell Function ◽  
Hearing Level ◽  
Case Review ◽  
Retrospective Case ◽  
Profound Hearing Loss ◽  
Tertiary Referral Center ◽  
Cadherin 23

Objective Mutations in the cadherin 23 gene ( CDH23) have been reported to cause cochlear damage, but few studies have investigated the auditory and speech outcome of patients after cochlear implantation. Here, we describe the genetic, auditory, and postoperative outcomes of patients with CDH23 mutations who received cochlear implants. Study Design Retrospective case review. Setting Tertiary referral center. Methods Targeted deafness-related gene panels were sequenced in Chinese families with profound sensorineural hearing loss. The clinical features of subjects carrying potentially pathogenic CDH23 mutations were analyzed. Results Between 2017 and 2019, we identified 5 children with prelinguistically profound hearing loss at our center who harbored 6 variants of CDH23 that segregated with the disease. Of these, 4 variants were novel (c.2591G>T, c.4785G>C, c.5765A>G, and c.9280_9281insTT). All affected individuals had a loss of outer hair cell function, with an average residual hearing level of 3 to 10 dB SPL. Cochlear implantations were arranged for the patients at 11 to 36 months of age. All children made gains in their hearing, language, and speech performances 14 to 120 months after surgery. Their auditory outcomes improved during follow-up intervals. Conclusion This study revealed that children with congenital cochlear defects caused by CDH23 variants can acquire an acceptable auditory and speech outcome after cochlear implantation. Early genetic detection and prenatal counseling for rare deafness genes such as CDH23 remain a priority for the future.

Ipsilateral Cochlear Implantation in the Presence of Observed and Irradiated Vestibular Schwannomas

2020 ◽  
Vol 129 (12) ◽  
pp. 1229-1238
Author(s):  
Matthew J. Urban ◽  
Dennis M. Moore ◽  
Keri Kwarta ◽  
John Leonetti ◽  
Rebecca Rajasekhar ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cochlear Implantation ◽  
Neurofibromatosis Type ◽  
Auditory Brainstem ◽  
Observation Time ◽  
Vestibular Schwannomas ◽  
Open Set ◽  
Pubmed Database ◽  
Auditory Brainstem Implants ◽  
Hearing Outcomes

Objectives: Historically, eventual loss of cochlear nerve function has limited patients with neurofibromatosis type 2 (NF2) to auditory brainstem implants (ABI), which in general are less effective than modern cochlear implants (CI). Our objective is to evaluate hearing outcomes following ipsilateral cochlear implantation in patients with NF2 and irradiated vestibular schwannomas (VS), and sporadic VS that have been irradiated or observed. Methods: Multi-center retrospective analysis of ipsilateral cochlear implantation in the presence of observed and irradiated VS. MESH search in NCBI PubMed database between 1992 and 2019 for reported cases of cochlear implantation with unresected vestibular schwannoma. Results: Seven patients underwent ipsilateral cochlear implantation in the presence of observed or irradiated vestibular schwannomas. Four patients had sporadic tumors with severe-profound contralateral hearing loss caused by presbycusis/hereditary sensorineural hearing loss, and three patients with NF2 lost contralateral hearing after prior surgical resection. Prior to implantation, one VS was observed without growth for a period of 7 years and the others were treated with radiotherapy. Mean post-operative sentence score was 63.9% (range 48-91) at an average of 28 (range 2-84) months follow up. All patients in this cohort obtained open set speech perception. While analysis of the literature is limited by heterogenous data reporting, 85% of implants with observed schwannomas achieved some open set perception, and 67% of patients previously radiated schwannomas. Furthermore, blending literature outcomes for post implantation sentence testing in quiet without lip-reading show 59.0 ± 35% for patients with CI and observed tumors and 55.7 ± 35% for patients with radiated tumors, with both groups ranging 0 to 100%. Conclusion: This retrospective series and literature review highlight that hearing outcomes with CI for VS patients are superior to those achieved with ABI. However, important considerations including imaging, delayed hearing loss, and observation time cannot be ignored in this population.

Sign in / Sign up

Export Citation Format

Share Document