Cochlear Implants in Alström Syndrome

2020 ◽  
Vol 129 (8) ◽  
pp. 833-837 ◽  
Author(s):  
Flavia Gheller ◽  
Samanta Gallo ◽  
Patrizia Trevisi ◽  
Ezio Caserta ◽  
Francesca Dassie ◽  
...  
Keyword(s):  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Surgical Intervention ◽  
Absolute Contraindication ◽  
Present Case Report ◽  
Alström Syndrome ◽  
Before And After ◽  
Excellent Functional Outcome ◽  
Alstrom Syndrome

Objectives: Too little is known about hearing loss rehabilitation in patients with Alström syndrome (AS). Benefits of hearing aids (HA) have not been fully documented and only one case treated with a Cochlear Implant (CI) has been described in the proceedings of a conference. Furthermore, comorbidities and risk of complications following surgical intervention may contraindicate Cochlear Implant procedures in these patients. The present case report concerns the first AS patient with CI in the literature. Methods: After reporting a concise description of the audiological profile of patients with AS described in the literature, the case of a 22-year-old woman with genetically confirmed Alström syndrome who underwent a sequential bilateral CI (Bi-CI) rehabilitation is reported. Audiological results before and after cochlear implantation are described. Results: The patient showed an excellent functional outcome with CIs, which enabled her to achieve communicative, social and academic results comparable with her peers, and no complications occurred. Conclusions: AS is not necessarily an absolute contraindication to CI. For many AS patients, a good cognitive function and adequate life expectancy represent a clear indication to prompt and adequate hearing rehabilitation with CIs. The description of this type of clinical cases could in the future also generate indications for a tailored audiological treatment of patients with very specific needs, such as patients with Alström Syndrome.

Cochlear Implants 2016: Advances in Candidacy, Technology, and Outcomes, Factors That Drive the Expansion of Pediatric Cochlear Implant Candidacy

2016 ◽  
Vol 1 (9) ◽  
pp. 21-28 ◽  
Author(s):  
Teresa A. Zwolan ◽  
Donna L. Sorkin
Keyword(s):  
Cochlear Implant ◽  
Cochlear Implants ◽  
Early Identification ◽  
Surgical Intervention ◽  
Auditory Brainstem ◽  
Follow Up Care ◽  
Auditory Brainstem Implants

Cochlear implant candidacy and outcomes have advanced reflecting technology improvements, early identification, and better linkage between the surgical intervention and follow-up care. Children and adults with a range of hearing losses and other issues are now benefitting importantly from traditional cochlear implants as well as hybrid and auditory brainstem implants.

Effects of Universal Newborn Hearing Screening on an Early Intervention Program for Children with Hearing Loss, Birth to 3 Yr of Age

2010 ◽  
Vol 21 (03) ◽  
pp. 169-175 ◽  
Author(s):  
Kathy S. Halpin ◽  
Kay Y. Smith ◽  
Judith E. Widen ◽  
Mark E. Chertoff
Keyword(s):  
Hearing Loss ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Late Onset ◽  
Hearing Aid ◽  
Profound Hearing Loss ◽  
Universal Newborn Hearing Screening ◽  
Before And After ◽  
Age Of Diagnosis ◽  
Diagnosis Age

Background: Universal Newborn Hearing Screening (UNHS) was introduced in Kansas in 1999. Prior to UNHS a small percentage of newborns were screened for and identified with hearing loss. Purpose: The purpose of this study was to determine the effects of UNHS on a local early intervention (EI) program for young children with hearing loss. Research Design: This was a retrospective study based on the chart review of children enrolled in the EI program during target years before and after the establishment of UNHS. Study Sample: Charts for 145 children were reviewed. Data Collection and Analysis: The chart review targeted the following aspects of the EI program: caseload size, percentage of caseload identified by UNHS, age of diagnosis, age of enrollment in EI, degree of hearing loss, etiology of hearing loss, late onset of hearing loss, age of hearing aid fit, percentage of children fit with hearing aids by 6 mo, percentage of children with profound hearing loss with cochlear implants, and percentage of children with additional disabilities. Results: Changes in the EI program that occurred after UNHS were increases in caseload size, percentage of caseload identified by UNHS, percentage of children fit with hearing aids by 6 mo of age, and percentage of children with profound hearing loss with cochlear implants. There were decreases in age of diagnosis, age of enrollment in EI, and age of hearing aid fit. Before UNHS, the majority of children had severe and profound hearing loss; after UNHS there were more children with mild and moderate hearing loss. The percentage of known etiology and late-onset hearing loss was approximately the same before and after UNHS, as was the percentage of children with additional disabilities. Conclusion: UNHS had a positive impact on caseload size, age of diagnosis, age of enrollment in EI, and age of hearing aid fit. The percentage of the caseload identified in the newborn period was about 25% before UNHS and over 80% after its implementation. After UNHS, the EI caseload included as many children with mild and moderate hearing loss as with severe and profound loss. By the last reporting year in the study (academic year 2005–2006) all children with profound hearing losses had cochlear implants.

scholarly journals Current and Planned Cochlear Implant Research at New York University Laboratory for Translational Auditory Research

2012 ◽  
Vol 23 (06) ◽  
pp. 422-437 ◽  
Author(s):  
Mario A. Svirsky ◽  
Matthew B. Fitzgerald ◽  
Arlene Neuman ◽  
Elad Sagi ◽  
Chin-Tuan Tan ◽  
...  
Keyword(s):  
New York ◽  
Speech Perception ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
New York University ◽  
School Of Medicine ◽  
Basic Studies ◽  
University Laboratory ◽  
Auditory Research

The Laboratory of Translational Auditory Research (LTAR/NYUSM) is part of the Department of Otolaryngology at the New York University School of Medicine and has close ties to the New York University Cochlear Implant Center. LTAR investigators have expertise in multiple related disciplines including speech and hearing science, audiology, engineering, and physiology. The lines of research in the laboratory deal mostly with speech perception by hearing impaired listeners, and particularly those who use cochlear implants (CIs) or hearing aids (HAs). Although the laboratory’s research interests are diverse, there are common threads that permeate and tie all of its work. In particular, a strong interest in translational research underlies even the most basic studies carried out in the laboratory. Another important element is the development of engineering and computational tools, which range from mathematical models of speech perception to software and hardware that bypass clinical speech processors and stimulate cochlear implants directly, to novel ways of analyzing clinical outcomes data. If the appropriate tool to conduct an important experiment does not exist, we may work to develop it, either in house or in collaboration with academic or industrial partners. Another notable characteristic of the laboratory is its interdisciplinary nature where, for example, an audiologist and an engineer might work closely to develop an approach that would not have been feasible if each had worked singly on the project. Similarly, investigators with expertise in hearing aids and cochlear implants might join forces to study how human listeners integrate information provided by a CI and a HA. The following pages provide a flavor of the diversity and the commonalities of our research interests.

scholarly journals The Function of Cochlear Implant After Cardioversion in a Patient With Atrial Flutter: A Case Report

2021 ◽  
pp. 014556132110019
Author(s):  
Hong Chan Kim ◽  
Chung Man Sung ◽  
Hyung Chae Yang ◽  
Hyong-Ho Cho
Keyword(s):  
Hearing Loss ◽  
Older People ◽  
Cochlear Implant ◽  
Hearing Aids ◽  
Heart Rhythm ◽  
Electrical Shock ◽  
Implant Surgery ◽  
Increased Risk ◽  
Cochlear Implant Surgery ◽  
Before And After

Hearing loss in older people can cause communication impairments, decreased quality of life, social isolation, depression, and dementia. Cochlear implant surgery is an effective treatment for older patients with hearing loss who cannot achieve satisfactory audiologic outcomes with hearing aids. However, older people have an increased risk of heart disease and often take medications that affect heart rhythm. Herein, we report a case of an 80-year-old woman who underwent cardioversion at 50J after cochlear implant surgery. Electrical impedance before and after cardioversion showed only minor changes without abnormality, and the cochlear implant functioned well. We believe that the electronic circuits of the cochlear implant may have been relatively tolerant to the electrical shock from the external defibrillator. Typically, cardioversion should be avoided in cochlear implant recipients because it may damage the implant. If cardioversion cannot be avoided, we strongly recommend starting cardioversion at the lowest energy level (50 J) and removing the sound processor of the implant during the procedure.

Examining the Relationship Between Speech Recognition and a Spectral–Temporal Test With a Mixed Group of Hearing Aid and Cochlear Implant Users

2021 ◽  
Vol 64 (3) ◽  
pp. 1073-1080
Author(s):  
Justin M. Aronoff ◽  
Leah Duitsman ◽  
Deanna K. Matusik ◽  
Senad Hussain ◽  
Elise Lippmann
Keyword(s):  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Significant Relationship ◽  
Signal To Noise Ratio ◽  
Hearing Aid ◽  
Mixed Group ◽  
Signal To Noise ◽  
Hearing Devices ◽  
The Relationship

Purpose Audiology clinics have a need for a nonlinguistic test for assessing speech scores for patients using hearing aids or cochlear implants. One such test, the Spectral-Temporally Modulated Ripple Test Lite for computeRless Measurement (SLRM), has been developed for use in clinics, but it, as well as the related Spectral-Temporally Modulated Ripple Test, has primarily been assessed with cochlear implant users. The main goal of this study was to examine the relationship between SLRM and the Arizona Biomedical Institute Sentence Test (AzBio) for a mixed group of hearing aid and cochlear implant users. Method Adult hearing aid users and cochlear implant users were tested with SLRM, AzBio in quiet, and AzBio in multitalker babble with a +8 dB signal-to-noise ratio. Results SLRM scores correlated with both AzBio recognition scores in quiet and in noise. Conclusions The results indicated that there is a significant relationship between SLRM and AzBio scores when testing a mixed group of cochlear implant and hearing aid users. This suggests that SLRM may be a useful nonlinguistic test for use with individuals with a variety of hearing devices.

scholarly journals Comparison of Relative Loudness Judgment in Children using Listening Devices with Typically Developing Children

2020 ◽  
Author(s):  
Shubha Tak ◽  
Asha Yathiraj
Keyword(s):  
Cochlear Implant ◽  
Hearing Impairment ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Hearing Aid ◽  
Normal Hearing ◽  
Standard Group ◽  
Typically Developing ◽  
Typically Developing Children ◽  
Loudness Judgment

Abstract Introduction Loudness perception is considered important for the perception of emotions, relative distance and stress patterns. However, certain digital hearing devices worn by those with hearing impairment may affect their loudness perception. This could happen in devices that have compression circuits to make loud sounds soft and soft sounds loud. These devices could hamper children from gaining knowledge about loudness of acoustical signals. Objective To compare relative loudness judgment of children using listening devices with age-matched typically developing children. Methods The relative loudness judgment of sounds created by day-to-day objects were evaluated on 60 children (20 normal-hearing, 20 hearing aid users, & 20 cochlear implant users), utilizing a standard group comparison design. Using a two-alternate forced-choice technique, the children were required to select picturized sound sources that were louder. Results The majority of the participants obtained good scores and poorer scores were mainly obtained by children using cochlear implants. The cochlear implant users obtained significantly lower scores than the normal-hearing participants. However, the scores were not significantly different between the normal-hearing children and the hearing aid users as well as between the two groups with hearing impairment. Conclusion Thus, despite loudness being altered by listening devices, children using non-linear hearing aids or cochlear implants are able to develop relative loudness judgment for acoustic stimuli. However, loudness growth for electrical stimuli needs to be studied.

Verification of a Proposed Clinical Electroacoustic Test Protocol for Personal Digital Modulation Receivers Coupled to Cochlear Implant Sound Processors

2017 ◽  
Vol 28 (07) ◽  
pp. 625-635
Author(s):  
Erika L. Nair ◽  
Rhonda Sousa ◽  
Shannon Wannagot
Keyword(s):  
American Academy ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Clinical Setting ◽  
Cochlear Nucleus ◽  
Behavioral Testing ◽  
Test Protocol ◽  
Digital Modulation ◽  
Sound Processor

AbstractGuidelines established by the AAA currently recommend behavioral testing when fitting frequency modulated (FM) systems to individuals with cochlear implants (CIs). A protocol for completing electroacoustic measures has not yet been validated for personal FM systems or digital modulation (DM) systems coupled to CI sound processors. In response, some professionals have used or altered the AAA electroacoustic verification steps for fitting FM systems to hearing aids when fitting FM systems to CI sound processors. More recently steps were outlined in a proposed protocol.The purpose of this research is to review and compare the electroacoustic test measures outlined in a 2013 article by Schafer and colleagues in the Journal of the American Academy of Audiology titled “A Proposed Electroacoustic Test Protocol for Personal FM Receivers Coupled to Cochlear Implant Sound Processors” to the AAA electroacoustic verification steps for fitting FM systems to hearing aids when fitting DM systems to CI users.Electroacoustic measures were conducted on 71 CI sound processors and Phonak Roger DM systems using a proposed protocol and an adapted AAA protocol. Phonak’s recommended default receiver gain setting was used for each CI sound processor manufacturer and adjusted if necessary to achieve transparency.Electroacoustic measures were conducted on Cochlear and Advanced Bionics (AB) sound processors. In this study, 28 Cochlear Nucleus 5/CP810 sound processors, 26 Cochlear Nucleus 6/CP910 sound processors, and 17 AB Naida CI Q70 sound processors were coupled in various combinations to Phonak Roger DM dedicated receivers (25 Phonak Roger 14 receivers—Cochlear dedicated receiver—and 9 Phonak Roger 17 receivers—AB dedicated receiver) and 20 Phonak Roger Inspiro transmitters.Employing both the AAA and the Schafer et al protocols, electroacoustic measurements were conducted with the Audioscan Verifit in a clinical setting on 71 CI sound processors and Phonak Roger DM systems to determine transparency and verify FM advantage, comparing speech inputs (65 dB SPL) in an effort to achieve equal outputs. If transparency was not achieved at Phonak’s recommended default receiver gain, adjustments were made to the receiver gain. The integrity of the signal was monitored with the appropriate manufacturer’s monitor earphones.Using the AAA hearing aid protocol, 50 of the 71 CI sound processors achieved transparency, and 59 of the 71 CI sound processors achieved transparency when using the proposed protocol at Phonak’s recommended default receiver gain. After the receiver gain was adjusted, 3 of 21 CI sound processors still did not meet transparency using the AAA protocol, and 2 of 12 CI sound processors still did not meet transparency using the Schafer et al proposed protocol.Both protocols were shown to be effective in taking reliable electroacoustic measurements and demonstrate transparency. Both protocols are felt to be clinically feasible and to address the needs of populations that are unable to reliably report regarding the integrity of their personal DM systems.

Efficacy of Multiple-Talker Phonetic Identification Training in Postlingually Deafened Cochlear Implant Listeners

2016 ◽  
Vol 59 (1) ◽  
pp. 90-98 ◽  
Author(s):  
Sharon E. Miller ◽  
Yang Zhang ◽  
Peggy B. Nelson
Keyword(s):  
Cochlear Implant ◽  
Cochlear Implants ◽  
Time Course ◽  
Procedural Learning ◽  
Control Group ◽  
Learning Effects ◽  
Phonetic Perception ◽  
Training Package ◽  
Before And After ◽  
Talker Identification

Purpose This study implemented a pretest-intervention-posttest design to examine whether multiple-talker identification training enhanced phonetic perception of the /ba/-/da/ and /wa/-/ja/ contrasts in adult listeners who were deafened postlingually and have cochlear implants (CIs). Method Nine CI recipients completed 8 hours of identification training using a custom-designed training package. Perception of speech produced by familiar talkers (talkers used during training) and unfamiliar talkers (talkers not used during training) was measured before and after training. Five additional untrained CI recipients completed identical pre- and posttests over the same time course as the trainees to control for procedural learning effects. Results Perception of the speech contrasts produced by the familiar talkers significantly improved for the trained CI listeners, and effects of perceptual learning transferred to unfamiliar talkers. Such training-induced significant changes were not observed in the control group. Conclusion The data provide initial evidence of the efficacy of the multiple-talker identification training paradigm for CI users who were deafened postlingually. This pattern of results is consistent with enhanced phonemic categorization of the trained speech sounds.

Microphone Directionality, Pre-Emphasis Filter, and Wind Noise in Cochlear Implants

2011 ◽  
Vol 22 (09) ◽  
pp. 586-600 ◽  
Author(s):  
King Chung ◽  
Nicholas McKibben
Keyword(s):  
Speech Recognition ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aid ◽  
Low Frequency ◽  
Correlation Coefficients ◽  
Wind Noise ◽  
Before And After ◽  
Wind Noise Reduction ◽  
Omnidirectional Microphones

Background: Wind noise can be a nuisance or a debilitating masker for cochlear implant users in outdoor environments. Previous studies indicated that wind noise at the microphone/hearing aid output had high levels of low-frequency energy and the amount of noise generated is related to the microphone directionality. Currently, cochlear implants only offer either directional microphones or omnidirectional microphones for users at-large. As all cochlear implants utilize pre-emphasis filters to reduce low-frequency energy before the signal is encoded, effective wind noise reduction algorithms for hearing aids might not be applicable for cochlear implants. Purpose: The purposes of this study were to investigate the effect of microphone directionality on speech recognition and perceived sound quality of cochlear implant users in wind noise and to derive effective wind noise reduction strategies for cochlear implants. Research Design: A repeated-measure design was used to examine the effects of spectral and temporal masking created by wind noise recorded through directional and omnidirectional microphones and the effects of pre-emphasis filters on cochlear implant performance. A digital hearing aid was programmed to have linear amplification and relatively flat in-situ frequency responses for the directional and omnidirectional modes. The hearing aid output was then recorded from 0 to 360° at flow velocities of 4.5 and 13.5 m/sec in a quiet wind tunnel. Study Sample: Sixteen postlingually deafened adult cochlear implant listeners who reported to be able to communicate on the phone with friends and family without text messages participated in the study. Intervention: Cochlear implant users listened to speech in wind noise recorded at locations that the directional and omnidirectional microphones yielded the lowest noise levels. Data Collection and Analysis: Cochlear implant listeners repeated the sentences and rated the sound quality of the testing materials. Spectral and temporal characteristics of flow noise, as well as speech and/or noise characteristics before and after the pre-emphasis filter, were analyzed. Correlation coefficients between speech recognition scores and crest factors of wind noise before and after pre-emphasis filtering were also calculated. Results: Listeners obtained higher scores using the omnidirectional than the directional microphone mode at 13.5 m/sec, but they obtained similar speech recognition scores for the two microphone modes at 4.5 m/sec. Higher correlation coefficients were obtained between speech recognition scores and crest factors of wind noise after pre-emphasis filtering rather than before filtering. Conclusion: Cochlear implant users would benefit from both directional and omnidirectional microphones to reduce far-field background noise and near-field wind noise. Automatic microphone switching algorithms can be more effective if the incoming signal were analyzed after pre-emphasis filters for microphone switching decisions.

Comparing Speech Perception of Children With Cochlear Implants or Hearing Aids

2010 ◽  
Vol 20 (2) ◽  
pp. 70-75 ◽  
Author(s):  
Lisa S. Davidson
Keyword(s):  
Speech Perception ◽  
Cochlear Implant ◽  
Cochlear Implants ◽  
Hearing Aids ◽  
Hearing Aid ◽  
Empirical Studies ◽  
Current Device ◽  
Device Technology

Cochlear implant (CI) candidacy guidelines continue to evolve as a result of advances in both cochlear implant and hearing aid technology. Empirical studies comparing the speech perception abilities of children using cochlear implants or hearing aids will be reviewed in the context of current device technology and CI candidacy evaluations.

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