488 Anatomical and Audiological Considerations in Branchio-otic/Branchio-oto-renal Syndrome: A Systematic Review and Narrative Synthesis

2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
K Biggs ◽  
G Crundwell ◽  
C Metcalfe ◽  
J Muzaffar ◽  
P Monksfield ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Middle Ear Surgery ◽  
Narrative Synthesis ◽  
Renal Anomalies ◽  
Anatomical Variants ◽  
Long Term Follow Up ◽  
Ear Anomalies

Abstract Introduction Branchio-otic/ Branchio-oto-renal syndrome (BO/BOR) is a rare autosomal dominant condition characterised by hearing loss, branchiogenic and renal anomalies. Anatomical variants, audiological outcomes and optimal management are considered. Method Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on year of publication. Results Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 503 patients from at least 104 families. In 308 patients hearing loss was categorised as sensorineural (29%), conductive (20%) and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity, ranging from mild to profound deafness. 43% patients presented with inner ear anomalies, 35% had middle and 36% had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however successful cochlear implantation was described in all five cases. Conclusions The anatomical and audiological profiles of patients with BO/BOR are variable. Cochlear implantation outcomes were good however the studies lacked long-term follow-up. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment.

scholarly journals Cochlear Implantation Outcomes in Patients with Mitochondrial Hearing Loss: A Systematic Review and Narrative Synthesis

2021 ◽  
Vol 17 (1) ◽  
pp. 72-80
Author(s):  
Nawal Zia ◽  
◽  
Yasmin Nikookam ◽  
Jameel Muzaffar ◽  
Peter Kullar ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Cochlear Implantation ◽  
Narrative Synthesis

Systematic review of cochlear implantation in adults with asymmetrical hearing loss

2021 ◽  
pp. 1-19
Author(s):  
Raghunandhan Sampathkumar ◽  
Axel Kaehne ◽  
Nirmal Kumar ◽  
Mohan Kameswaran ◽  
Richard Irving
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Cochlear Implantation

scholarly journals Hearing Loss Treatment in Older Adults With Cognitive Impairment: A Systematic Review

2018 ◽  
Vol 61 (10) ◽  
pp. 2589-2603 ◽  
Author(s):  
Sara K. Mamo ◽  
Nicholas S. Reed ◽  
Carrie Price ◽  
Dona Occhipinti ◽  
Alexandra Pletnikova ◽  
...  
Keyword(s):  
Systematic Review ◽  
Older Adults ◽  
Hearing Loss ◽  
Cognitive Impairment ◽  
Hearing Aids ◽  
Controlled Trial ◽  
Controlled Vocabulary ◽  
Assessment Tools ◽  
Cochrane Library ◽  
Full Text Review

Purpose The purpose of this systematic review was to assess studies of treating hearing loss in older adults with cognitive impairment. Of interest to this review is identifying clinical adaptations that may be used to tailor hearing loss treatment to older adults with cognitive impairment in order to better serve this vulnerable population. Method A systematic search with controlled vocabulary and key word terms was applied to PubMed, the Cochrane Library, Embase, CINAHL, and PsycINFO. Search concepts included terms related to hearing loss and cognitive impairment. The overall search resulted in 4,945 unique references, 50 of which were eligible for full-text review and 13 of which were included in the final review. Included manuscripts were categorized according to the American Speech-Language-Hearing Association's levels of evidence and the National Institutes of Health Quality Assessment Tools. Results Only 1 study implemented a randomized controlled trial design to assess cognitive function and behavioral symptoms after treatment with hearing aids. Other quasiexperimental studies evaluated dementia-related symptoms and/or auditory function after treating hearing loss in pre/post research designs. Finally, evidence from case studies suggested that hearing loss treatment is feasible, reduces stressful communication for caregivers, and improves dementia-related behavior problems. Conclusion Based on the systematic review, evidence suggests that treating hearing loss in persons with cognitive impairment can have benefits to communication and quality of life. Because of the quasi- and nonexperimental nature of most of the evidence found in this review, further studies are necessary to understand the effect of treatment in the context of a variable and progressive disease.

Cochlear Implantation in Children with Postlingual Progressive Steeply Sloping High-Frequency Hearing Loss

2017 ◽  
Vol 28 (10) ◽  
pp. 913-919 ◽  
Author(s):  
Margaret A. Meredith ◽  
Jay T. Rubinstein ◽  
Kathleen C. Y. Sie ◽  
Susan J. Norton
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
High Frequency ◽  
Case Series ◽  
Hearing Preservation ◽  
Rapid Improvement ◽  
Retrospective Case ◽  
Monosyllabic Word

Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). Research Design: Retrospective case series. Study Sample: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children’s Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. Data Collection and Analysis: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. Results: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. Conclusions: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.

scholarly journals Hearing Aid Treatment for Patients with Mixed Hearing Loss. Part II: Speech Recognition in Comparison to Direct Acoustic Cochlear Stimulation

2020 ◽  
Vol 25 (3) ◽  
pp. 133-142
Author(s):  
Nina Wardenga ◽  
Ad F.M. Snik ◽  
Eugen Kludt ◽  
Bernd Waldmann ◽  
Thomas Lenarz ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Middle Ear ◽  
Hearing Aids ◽  
Dynamic Range ◽  
Hearing Aid ◽  
Middle Ear Surgery ◽  
Promising Alternative ◽  
Mixed Hearing Loss ◽  
Technical Specifications

Background: The conventional therapy for severe mixed hearing loss is middle ear surgery combined with a power hearing aid. However, a substantial group of patients with severe mixed hearing loss cannot be treated adequately with today’s state-of-the-art (SOTA) power hearing aids, as predicted by the accompanying part I of this publication, where we compared the available maximum power output (MPO) and gain from technical specifications to requirements for optimum benefit using a common fitting rule. Here, we intended to validate the theoretical assumptions from part I experimentally in a mixed hearing loss cohort fitted with SOTA power hearing aids. Additionally, we compared the results with an implantable hearing device that circumvents the impaired middle ear, directly stimulating the cochlea, as this might be a better option. Objectives: Speech recognition outcomes obtained from patients with severe mixed hearing loss supplied acutely with a SOTA hearing aid were studied to validate the outcome predictions as described in part I. Further, the results obtained with hearing aids were compared to those in direct acoustic cochlear implant (DACI) users. Materials and Methods: Twenty patients (37 ears with mixed hearing loss) were provided and fitted with a SOTA power hearing aid. Before and after an acclimatization period of at least 4 weeks, word recognition scores (WRS) in quiet and in noise were studied, as well as the speech reception threshold in noise (SRT). The outcomes were compared retrospectively to a second group of 45 patients (47 ears) using the DACI device. Based on the severity of the mixed hearing loss and the available gain and MPO of the SOTA hearing aid, the hearing aid and DACI users were subdivided into groups with prediction of sufficient, partially insufficient, or very insufficient hearing aid performance. Results: The patients with predicted adequate SOTA hearing aid performance indeed showed the best WRS in quiet and in noise when compared to patients with predicted inferior outcomes. Insufficient hearing aid performance at one or more frequencies led to a gradual decrease in hearing aid benefit, validating the criteria used here and in the accompanying paper. All DACI patients showed outcomes at the same level as the adequate hearing aid performance group, being significantly better than those of the groups with inadequate hearing aid performance. Whereas WRS in quiet and noise were sensitive to insufficient gain or output, showing significant differences between the SOTA hearing aid and DACI groups, the SRT in noise was less sensitive. Conclusions: Limitations of outcomes in mixed hearing loss individuals due to insufficient hearing aid performance can be accurately predicted by applying a commonly used fitting rule and the 35-dB dynamic range rule on the hearing aid specifications. Evidently, when outcomes in patients with mixed hearing loss using the most powerful hearing aids are insufficient, bypassing the middle ear with a powerful active middle ear implant or direct acoustic implant can be a promising alternative treatment.

Are head bandages really required after middle-ear surgery? A systematic review

2015 ◽  
Vol 129 (8) ◽  
pp. 740-743 ◽  
Author(s):  
I Khan ◽  
S Mohamad ◽  
S Ansari ◽  
A Iyer
Keyword(s):  
Systematic Review ◽  
Middle Ear ◽  
Cochlear Implantation ◽  
Randomised Controlled Trials ◽  
Case Series ◽  
Middle Ear Surgery ◽  
Controlled Trials ◽  
Retrospective Case ◽  
Ear Surgery ◽  
Randomised Controlled

AbstractObjective:A systematic review was performed to evaluate the role and effectiveness of head bandages after routine elective middle-ear surgery.Methods:Studies that compared the effectiveness of head bandage use after elective middle-ear surgery (e.g. myringoplasty, mastoidectomy and cochlear implantation) were identified using the following databases: Ovid Medline and Embase, the Ebsco collections, the Cochrane Library, PubMed, and Google Scholar. An initial search identified 71 articles. All titles and abstracts were reviewed. Thirteen relevant articles were inspected in more detail; of these, only five met the inclusion criteria. These included three randomised, controlled trials, one retrospective case series and one literature review.Results:The three randomised, controlled trials (level of evidence 1b) showed no statistically significant differences in post-operative outcomes (in terms of complications) associated with head bandage use in middle-ear surgery. This finding was supported by the retrospective case series involving patients undergoing cochlear implantation.Conclusion:Current available evidence shows no advantage of head bandage use after middle-ear surgery. Head bandages may not be required after routine, uncomplicated middle-ear surgery.

scholarly journals Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Birgul Gumus ◽  
Armagan Incesulu ◽  
Mehmet Ozgur Pinarbasli
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
External Ear ◽  
Ear Canal ◽  
External Ear Canal ◽  
Brainstem Response ◽  
Visual Disturbances

Background.Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature.Case Report.This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry.Conclusion.Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

scholarly journals The Influence of Hearing Aids on Balance Control: A Systematic Review

2020 ◽  
pp. 1-9
Author(s):  
Daniele Borsetto ◽  
Virginia Corazzi ◽  
Sebastiano Franchella ◽  
Chiara Bianchini ◽  
Stefano Pelucchi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Hearing Loss ◽  
Hearing Aids ◽  
Balance Control ◽  
Dynamic Balance ◽  
Subjective Perception ◽  
Cross Sectional ◽  
Self Confidence ◽  
Computerized Posturography ◽  
Romberg Test

<b><i>Aim:</i></b> To assess the current opinion on the effects of hearing loss treatment by hearing aids (HAs) and the benefits of HA use on imbalance. <b><i>Methods:</i></b> PRISMA-compliant systematic review was done, including observational studies in patients affected by mild to severe sensorineural hearing loss with HAs, investigating the benefits of HAs on balance. Electronic searches were performed through Medline, Cochrane, Embase, Web of Science, and Scopus. <b><i>Results:</i></b> A total of 200 patients in 8 studies were included in this systematic review. Four studies were cross-sectional, 3 cross-sectional controlled and 1 prospective nonrandomized study. Static and dynamic balance in the aided condition improved in patients assessed using clinical investigations including Romberg test and Functional Ambulation Performance/mini-BESTest, respectively. Variable outcomes were found measuring static and dynamic balance during the aided condition with objective tests (computerized posturography, Mobility Lab device). Improved quality of life outcomes and self-confidence were noted, while subjective measurements of balance had conflicting results. <b><i>Conclusion:</i></b> Although an improvement in balance in patients with HAs has been shown in certain conditions, the overall benefit is still unclear and it is only possible to speculate that HAs may also improve static, dynamic, or subjective perception of balance function in adults affected by hearing loss.

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