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Maximum preimplantation monosyllabic score as predictor of cochlear implant outcome.

ABSTRACT: OBJECTIVE:This study investigated the speech perception of cochlear implant (CI) recipients with measurable preoperative ipsilateral speech perception. These data should support improved individual counselling of CI candidates. MATERIALS AND METHODS:Pre- and postoperative speech audiometric parameters were analyzed, including maximum score for phonemically balanced words (PBmax) and monosyllabic score at a normal conversational level of 65 dBSPL, with hearing aids one hand and CI on the other. Data of 284 experienced adult CI wearers were grouped and evaluated in terms of preoperative PBmax. RESULTS:The preoperative PBmax was exceeded by the postoperative monosyllabic score in 96% of cases. The overall median postoperative score was 72.5%. The groups with preoperative PBmax > 0% showed significantly better speech perception scores with CI than the group with PBmax = 0%. Median improvement compared to the preoperative monosyllabic score with hearing aids was 65 percentage points, independent of preoperative PBmax. CONCLUSION:The preoperatively measured PBmax may be used as a predictor for the minimum speech perception obtained with CI. This is of high clinical relevance for CI candidates with a PBmax above zero.

SUBMITTER: Hoppe U

PROVIDER: S-EPMC6565665 | biostudies-other | 2019 Jun

REPOSITORIES: biostudies-other

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Maximum preimplantation monosyllabic score as predictor of cochlear implant outcome.

Hoppe Ulrich U Hocke Thomas T Hast Anne A Iro Heinrich H

HNO 20190601 Suppl 2

<h4>Objective</h4>This study investigated the speech perception of cochlear implant (CI) recipients with measurable preoperative ipsilateral speech perception. These data should support improved individual counselling of CI candidates.<h4>Materials and methods</h4>Pre- and postoperative speech audiometric parameters were analyzed, including maximum score for phonemically balanced words (PB<sub>max</sub>) and monosyllabic score at a normal conversational level of 65 dB<sub>SPL</sub>, with hearing ...[more]

PMID: 30944946

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Project description:Wolfram syndrome type 1 gene (WFS1), which encodes a transmembrane structural protein (wolframin), is essential for several biological processes, including proper inner ear function. Unlike the recessively inherited Wolfram syndrome, WFS1 heterozygous variants cause DFNA6/14/38 and wolfram-like syndrome, characterized by autosomal dominant nonsyndromic hearing loss, optic atrophy, and diabetes mellitus. Here, we identified two WFS1 heterozygous variants in three DFNA6/14/38 families using exome sequencing. We reveal the pathogenicity of the WFS1 variants based on three-dimensional (3D) modeling and structural analysis. Furthermore, we present cochlear implantation (CI) outcomes in WFS1-associated DFNA6/14/38 and suggest a genotype-phenotype correlation based on our results and a systematic review. We performed molecular genetic test and evaluated clinical phenotypes of three WFS1-associated DFNA6/14/38 families. A putative WFS1-NCS1 interaction model was generated, and the impacts of WFS1 variants on stability were predicted by comparing intramolecular interactions. A total of 62 WFS1 variants associated with DFNA6/14/38 were included in a systematic review. One variant is a known mutational hotspot variant in the endoplasmic reticulum (ER)-luminal domain WFS1(NM_006005.3) (c.2051 C > T:p.Ala684Val), and the other is a novel frameshift variant in transmembrane domain 6 (c.1544_1545insA:p.Phe515LeufsTer28). The two variants were pathogenic, based on the ACMG/AMP guidelines. Three-dimensional modeling and structural analysis show that non-polar, hydrophobic substitution of Ala684 (p.Ala684Val) destabilizes the alpha helix and contributes to the loss of WFS1-NCS1 interaction. Also, the p.Phe515LeufsTer28 variant truncates transmembrane domain 7-9 and the ER-luminal domain, possibly impairing membrane localization and C-terminal signal transduction. The systematic review demonstrates favorable outcomes of CI. Remarkably, p.Ala684Val in WFS1 is associated with early-onset severe-to-profound deafness, revealing a strong candidate variant for CI. We expanded the genotypic spectrum of WFS1 heterozygous variants underlying DFNA6/14/38 and revealed the pathogenicity of mutant WFS1, providing a theoretical basis for WFS1-NCS1 interactions. We presented a range of phenotypic traits for WFS1 heterozygous variants and demonstrated favorable functional CI outcomes, proposing p.Ala684Val a strong potential marker for CI candidates.

Project description:Currently, it is not possible to accurately predict how well a deaf individual will be able to understand speech when hearing is (re)introduced via a cochlear implant. Differences in brain organisation following deafness are thought to contribute to variability in speech understanding with a cochlear implant and may offer unique insights that could help to more reliably predict outcomes. An emerging optical neuroimaging technique, functional near-infrared spectroscopy (fNIRS), was used to determine whether a pre-operative measure of brain activation could explain variability in cochlear implant (CI) outcomes and offer additional prognostic value above that provided by known clinical characteristics. Cross-modal activation to visual speech was measured in bilateral superior temporal cortex of pre- and post-lingually deaf adults before cochlear implantation. Behavioural measures of auditory speech understanding were obtained in the same individuals following 6 months of cochlear implant use. The results showed that stronger pre-operative cross-modal activation of auditory brain regions by visual speech was predictive of poorer auditory speech understanding after implantation. Further investigation suggested that this relationship may have been driven primarily by the inclusion of, and group differences between, pre- and post-lingually deaf individuals. Nonetheless, pre-operative cortical imaging provided additional prognostic value above that of influential clinical characteristics, including the age-at-onset and duration of auditory deprivation, suggesting that objectively assessing the physiological status of the brain using fNIRS imaging pre-operatively may support more accurate prediction of individual CI outcomes. Whilst activation of auditory brain regions by visual speech prior to implantation was related to the CI user's clinical history of deafness, activation to visual speech did not relate to the future ability of these brain regions to respond to auditory speech stimulation with a CI. Greater pre-operative activation of left superior temporal cortex by visual speech was associated with enhanced speechreading abilities, suggesting that visual speech processing may help to maintain left temporal lobe specialisation for language processing during periods of profound deafness.

Dataset Information

Maximum preimplantation monosyllabic score as predictor of cochlear implant outcome.

Publications

Maximum preimplantation monosyllabic score as predictor of cochlear implant outcome.

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