Speech Perception and Production in Cochlear Implant Recipients with Pendred Syndrome.
Ontology highlight
ABSTRACT: Aims
Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients.Methods
Ten patients with Pendred syndrome were analyzed for speech perception and production. The control group consisted of 41 non-syndromic implanted patients. All implantees were scored according to speech perception, speech production, and the sum of both. The data were statistically analyzed.Results
No statistical difference was found in language acquisition and production in implantees with Pendred syndrome when compared to non-syndromic patients with cochlear implants. Nor there was any difference in speech production and acquisition between the 2 compared groups regarding surgical age, time elapsed after surgery, or age during the testing.Conclusion
In this study evaluating language and speech production and acquisition, patients with Pendred syndrome who underwent cochlear implants show comparable results to their implanted peers with deafness of a different etiology.
SUBMITTER: Skrivan J
PROVIDER: S-EPMC8880977 | biostudies-literature | 2021 Jul
REPOSITORIES: biostudies-literature
Publications
Speech Perception and Production in Cochlear Implant Recipients with Pendred Syndrome.
Skrivan Jiri J Jurovcik Michal M Aksenovova Zdenka Z Astl Jaromír J Pourova Radka Kremlikova RK Dytrych Petra P Sieger Tomas T
Balkan medical journal 20210701 4
<h4>Aims</h4>Patients with the Pendred syndrome suffer very often from a hearing loss. They may be good candidates for a cochlear implantation, but unfortunately, due to the fluctuating character of the hearing loss, they may escape such indication. In the study, we compared speech production and speech acquisition in 2 groups of implanted patients: those with the Pendred syndrome, and standard non-syndromic patients.<h4>Methods</h4>Ten patients with Pendred syndrome were analyzed for speech per ...[more]
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Project description:Although significant progress has been made in understanding outcomes following cochlear implantation, predicting performance remains a challenge. Duration of hearing loss, age at implantation, and electrode positioning within the cochlea together explain ~ 25% of the variability in speech-perception scores in quiet using the cochlear implant (CI). Electrocochleography (ECochG) responses, prior to implantation, account for 47% of the variance in the same speech-perception measures. No study to date has explored CI performance in noise, a more realistic measure of natural listening. This study aimed to (1) validate ECochG total response (ECochG-TR) as a predictor of performance in quiet and (2) evaluate whether ECochG-TR explained variability in noise performance. Thirty-five adult CI recipients were enrolled with outcomes assessed at 3-months post-implantation. The results confirm previous studies showing a strong correlation of ECochG-TR with speech-perception in quiet (r = 0.77). ECochG-TR independently explained 34% of the variability in noise performance. Multivariate modeling using ECochG-TR and Montreal Cognitive Assessment (MoCA) scores explained 60% of the variability in speech-perception in noise. Thus, ECochG-TR, a measure of the cochlear substrate prior to implantation, is necessary but not sufficient for explaining performance in noise. Rather, a cognitive measure is also needed to improve prediction of noise performance.
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