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 Electric and Acoustic Stimulation in Cochlear Implant Recipients with Hearing Preservation

2018 ◽  
Vol 39 (04) ◽  
pp. 414-427 ◽  
Author(s):  
Christopher Welch ◽  
Margaret Dillon ◽  
Harold Pillsbury
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Cochlear Implant ◽  
Hearing Aids ◽  
High Frequency ◽  
Low Frequency ◽  
Hearing Preservation ◽  
The United States ◽  
Acoustic Stimulation ◽  
Electric Hearing

AbstractHearing loss affects 30 million people in the United States, and a subset of these patients have normal low-frequency hearing and ski-sloped high-frequency hearing loss. For these patients, hearing aids alone may not provide adequate benefit. Cochlear implantation alone has been utilized to improve speech perception. The addition of high-frequency electric hearing to low-frequency acoustic hearing in these patients is beneficial. Technical improvements have allowed preservation of low-frequency hearing in cochlear implant recipients, allowing for electric and acoustic stimulation in the same ear with significant improvements in speech perception, sound localization, music appreciation, and quality of life.

scholarly journals Listener Performance with a Novel Hearing Aid Frequency Lowering Technique

2017 ◽  
Vol 28 (09) ◽  
pp. 810-822 ◽  
Author(s):  
Benjamin J. Kirby ◽  
Judy G. Kopun ◽  
Meredith Spratford ◽  
Clairissa M. Mollak ◽  
Marc A. Brennan ◽  
...  
Keyword(s):  
Signal Processing ◽  
Hearing Loss ◽  
Speech Perception ◽  
Hearing Aids ◽  
High Frequency ◽  
Hearing Aid ◽  
Sound Quality ◽  
Processing Strategy ◽  
Listening Effort ◽  
At Home

AbstractSloping hearing loss imposes limits on audibility for high-frequency sounds in many hearing aid users. Signal processing algorithms that shift high-frequency sounds to lower frequencies have been introduced in hearing aids to address this challenge by improving audibility of high-frequency sounds.This study examined speech perception performance, listening effort, and subjective sound quality ratings with conventional hearing aid processing and a new frequency-lowering signal processing strategy called frequency composition (FC) in adults and children.Participants wore the study hearing aids in two signal processing conditions (conventional processing versus FC) at an initial laboratory visit and subsequently at home during two approximately six-week long trials, with the order of conditions counterbalanced across individuals in a double-blind paradigm.Children (N = 12, 7 females, mean age in years = 12.0, SD = 3.0) and adults (N = 12, 6 females, mean age in years = 56.2, SD = 17.6) with bilateral sensorineural hearing loss who were full-time hearing aid users.Individual performance with each type of processing was assessed using speech perception tasks, a measure of listening effort, and subjective sound quality surveys at an initial visit. At the conclusion of each subsequent at-home trial, participants were retested in the laboratory. Linear mixed effects analyses were completed for each outcome measure with signal processing condition, age group, visit (prehome versus posthome trial), and measures of aided audibility as predictors.Overall, there were few significant differences in speech perception, listening effort, or subjective sound quality between FC and conventional processing, effects of listener age, or longitudinal changes in performance. Listeners preferred FC to conventional processing on one of six subjective sound quality metrics. Better speech perception performance was consistently related to higher aided audibility.These results indicate that when high-frequency speech sounds are made audible with conventional processing, speech recognition ability and listening effort are similar between conventional processing and FC. Despite the lack of benefit to speech perception, some listeners still preferred FC, suggesting that qualitative measures should be considered when evaluating candidacy for this signal processing strategy.

scholarly journals Speech Perception and Information-Carrying Capacity for Hearing Aid Users of Different Ages

2016 ◽  
Vol 21 (Suppl. 1) ◽  
pp. 16-20 ◽  
Author(s):  
Ulrich Hoppe ◽  
Thomas Hocke ◽  
Alexander Müller ◽  
Anne Hast
Keyword(s):  
Hearing Loss ◽  
Speech Recognition ◽  
Speech Perception ◽  
Hearing Aids ◽  
Carrying Capacity ◽  
Pure Tone ◽  
Hearing Aid ◽  
The Elderly ◽  
Monosyllabic Word ◽  
Older Subjects

Hearing impairment in the elderly is usually treated with conventional hearing aids; however, a large number of older people do not achieve sufficient speech recognition with hearing aids. The aim of the study was to describe speech perception with hearing aids in comparison to pure-tone hearing loss and maximum speech recognition scores for phonemically balanced words. Data from 392 hearing aid users with different degrees of hearing loss were evaluated retrospectively. In particular, pure-tone thresholds, the maximum monosyllabic word score, and the monosyllabic word score in quiet at conversational level with a hearing aid were analysed. The results showed that speech perception scores decline with increasing age. Even when corrected for pure-tone hearing loss, a significant decline in speech recognition scores after the age of 80 years was observed. Regarding the maximum monosyllabic word score, the effect is smaller but still observable; thus, speech recognition with hearing aids is significantly lower for older subjects. This can be attributed partially to the reduction of the information-carrying capacity in this group.

scholarly journals Cochlear Implantation for Single-Sided Deafness: Observations in Poor Performers

2018 ◽  
Vol 01 (01) ◽  
pp. 018-022 ◽  
Author(s):  
Philipp Mittmann ◽  
A. Ernst ◽  
S. Scholz ◽  
R. Battmer ◽  
I. Todt
Keyword(s):  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Case Series ◽  
Poor Performance ◽  
Resonance Imaging ◽  
Retrospective Case ◽  
Monosyllabic Word ◽  
Cerebral Involvement ◽  
Magnetic Resonance Imaging Mri ◽  
Single Sided Deafness

Abstract Objectives Patients with single-sided deafness can nowadays receive a cochlear implant. A majority of these patients are well adapted and benefit from the implant. The aim of this study was to evaluate the reasons for poor performance in a group of patients with single-sided deafness, who received a cochlear implant. Design A total of 65 patients were enrolled into a retrospective case series. Seven poor performers were present in the group. Freiburger monosyllabics, localization testing, and radiologic images were evaluated. Results Localization testing showed the absence of lateralization ability in three patients, whereas the Freiburger monosyllabic word scores improved in three patients. One patient had no speech perception after 1 year of rehabilitation. Findings of magnetic resonance imaging (MRI) revealed cerebral involvement in five patients. Conclusion Various factors influence the outcome in unilateral cochlear implantation in patients with single-sided deafness. These reasons may be preoperative, operative, or postoperative. Nevertheless, the majority of patients benefit from these implants.

scholarly journals Hearing Preservation after Cochlear Implantation: UNICAMP Outcomes

2013 ◽  
Vol 2013 ◽  
pp. 1-8 ◽  
Author(s):  
Guilherme Machado de Carvalho ◽  
Alexandre C. Guimaraes ◽  
Alexandre S. M. Duarte ◽  
Eder B. Muranaka ◽  
Marcelo N. Soki ◽  
...  
Keyword(s):  
Speech Perception ◽  
Surgical Technique ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Hearing Preservation ◽  
Acoustic Stimulation ◽  
Residual Hearing ◽  
Low Frequencies ◽  
Great Satisfaction ◽  
New Device

Background. Electric-acoustic stimulation (EAS) is an excellent choice for people with residual hearing in low frequencies but not high frequencies and who derive insufficient benefit from hearing aids. For EAS to be effective, subjects' residual hearing must be preserved during cochlear implant (CI) surgery.Methods. We implanted 6 subjects with a CI. We used a special surgical technique and an electrode designed to be atraumatic. Subjects' rates of residual hearing preservation were measured 3 times postoperatively, lastly after at least a year of implant experience. Subjects' aided speech perception was tested pre- and postoperatively with a sentence test in quiet. Subjects' subjective responses assessed after a year of EAS or CI experience.Results. 4 subjects had total or partial residual hearing preservation; 2 subjects had total residual hearing loss. All subjects' hearing and speech perception benefited from cochlear implantation. CI diminished or eliminated tinnitus in all 4 subjects who had it preoperatively. 5 subjects reported great satisfaction with their new device.Conclusions. When we have more experience with our surgical technique we are confident we will be able to report increased rates of residual hearing preservation. Hopefully, our study will raise the profile of EAS in Brazil and Latin/South America.

Metachronous Sudden Sensorineural Hearing Loss: Patient Characteristics and Treatment Outcomes

2020 ◽  
Vol 162 (3) ◽  
pp. 337-342
Author(s):  
Timothy Cooper ◽  
Ronak Dixit ◽  
Candace E. Hobson ◽  
Barry E. Hirsch ◽  
Andrew A. McCall
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Case Series ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Retrospective Case ◽  
Rare Presentation ◽  
Profound Hearing Loss ◽  
The Mean

Objectives Idiopathic sudden sensorineural hearing loss (ISSNHL) is a distressing condition that can significantly affect quality of life. Unilateral ISSNHL, occurring first in 1 ear and then the contralateral ear at a separate and discrete time, is a rare presentation that we refer to as metachronous ISSNHL. Our objective was to characterize the presentation of metachronous ISSNHL and report on management and hearing outcomes. Study Design Retrospective case series. Setting Otology clinic at an academic tertiary referral center. Subjects and Methods Patients ≥18 years old presenting with metachronous ISSNHL between April 2008 to November 2017 were identified through review of the clinic electronic medical record. Metachronous ISSNHL was defined as unilateral ISSNHL occurring in temporally discrete episodes (>6 months apart) affecting both ears. Patients with identifiable causes for sudden hearing loss were excluded. Patient demographics, comorbidities, management, and audiologic outcomes were recorded. Results Eleven patients with metachronous ISSNHL were identified out of 558 patients with ISSNHL. In patients with metachronous ISSNHL, the mean ± standard deviation age at the time of ISSNHL in the second ear was 58.6 ± 15.2 years (range, 31-77 years). The mean interval between episodes was 9.6 ± 7.5 years (range, 1-22 years). Patients were treated with systemic and intratympanic steroids with variable hearing recovery; 5 patients with resultant bilateral severe to profound hearing loss underwent successful cochlear implantation. Conclusion Metachronous ISSNHL is uncommon. Treatment is similar to ISSNHL, and cochlear implantation can successfully restore hearing in individuals who do not experience recovery.

Modiolar ossification in paediatric patients with auditory neuropathy

2017 ◽  
Vol 131 (7) ◽  
pp. 598-601 ◽  
Author(s):  
L Wang ◽  
Z Wang ◽  
F Gao ◽  
K A Peng
Keyword(s):  
Computed Tomography ◽  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Comparison Group ◽  
Case Series ◽  
Auditory Neuropathy ◽  
Sensorineural Hearing ◽  
Retrospective Case ◽  
Paediatric Patients

AbstractObjective:To describe our finding of increased ossification of the modiolus in paediatric patients with auditory neuropathy who met criteria for cochlear implantation.Methods:A retrospective case series with a comparison group at a tertiary referral centre is described. Seven paediatric patients with auditory neuropathy who met criteria for and underwent cochlear implantation were identified. Fifteen paediatric implantees with bilateral profound sensorineural hearing loss were included as the comparison group. All patients underwent pre-operative computed tomography. Attenuation at the modiolus was measured in all subjects by a neuroradiologist blinded to clinical information.Results:Attenuation values in the modiolus in the auditory neuropathy patients (mean ± standard deviation = 796.2 ± 53.0 HU) was statistically significantly higher than in the comparison sensorineural hearing loss patients (267.1 ± 45.6 HU; p < 0.05, t-test).Conclusion:Patients with auditory neuropathy who meet criteria for cochlear implantation demonstrate significantly higher modiolar attenuation on computed tomography imaging, consistent with increased ossification at the modiolus.

Modified Earpieces and CROS for High Frequency Hearing Losses

1968 ◽  
Vol 11 (1) ◽  
pp. 204-218 ◽  
Author(s):  
Elizabeth Dodds ◽  
Earl Harford
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
High Frequency ◽  
Hearing Aid ◽  
Sound Field ◽  
Intensity Level ◽  
Test Results ◽  
High Frequency Hearing Loss ◽  
Increased Sensitivity ◽  
Difficult Cases

Persons with a high frequency hearing loss are difficult cases for whom to find suitable amplification. We have experienced some success with this problem in our Hearing Clinics using a specially designed earmold with a hearing aid. Thirty-five cases with high frequency hearing losses were selected from our clinical files for analysis of test results using standard, vented, and open earpieces. A statistical analysis of test results revealed that PB scores in sound field, using an average conversational intensity level (70 dB SPL), were enhanced when utilizing any one of the three earmolds. This result was due undoubtedly to increased sensitivity provided by the hearing aid. Only the open earmold used with a CROS hearing aid resulted in a significant improvement in discrimination when compared with the group’s unaided PB score under earphones or when comparing inter-earmold scores. These findings suggest that the inclusion of the open earmold with a CROS aid in the audiologist’s armamentarium should increase his flexibility in selecting hearing aids for persons with a high frequency hearing loss.

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.

Cochlear Implant Patients with Superficial Siderosis

2009 ◽  
Vol 20 (06) ◽  
pp. 348-352 ◽  
Author(s):  
Sarah A. Sydlowski ◽  
Michael J. Cevette ◽  
Jon Shallop ◽  
David M. Barrs
Keyword(s):  
Central Nervous System ◽  
Hearing Loss ◽  
Cochlear Implant ◽  
Cochlear Implantation ◽  
Case Reports ◽  
Superficial Siderosis ◽  
Viable Option ◽  
Retrospective Case ◽  
Fatal Disease ◽  
The Central Nervous System

Background: Considered a rare disorder, superficial siderosis of the central nervous system (SSCN) has become more frequently diagnosed in recent years. As it is characterized by progressive sensorineural hearing loss, patients' needs may surpass the capability of hearing aid technology. Despite the retrocochlear nature of the disorder, patients have undergone cochlear implantation (CI) with varying success. Purpose: To summarize the issues surrounding cochlear implant candidates with SSCN as well as highlight trends in performance postimplantation. Research Design: Retrospective case reports of seven cochlear implant candidates detail the symptoms, typical audiologic presentation, and array of clinical issues for patients with this progressive and potentially fatal disease. Results: Despite the retrocochlear component of a hearing loss caused by SSCN, cochlear implantation may be a viable option. Conclusions: It is essential that the CI audiologist not only be aware of the disorder but also be well versed in the resulting implications for the cochlear implant process. A more thorough case history, an expanded candidacy test battery, and knowledge of the typical presentation of SSCN are critical. The diagnosis of SSCN will impact expectations for success with the cochlear implant, and counseling should be adjusted accordingly.

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