Variability in Cochlear Implantation Outcomes in a Large German Cohort With a Genetic Etiology of Hearing Loss.
Anke Tropitzsch, Thore Schade-Mann, Philipp Gamerdinger and 18 others
PMID 37438890WHAT IT FOUND
Cochlear implant recipients whose hearing loss came from genes affecting nerve-related tissue in the inner ear scored lower on a word recognition test than recipients with other genetic diagnoses.
Their median score was 45%, while the cohort median was 70%.
Key findings
01Ears with genes expressed in neural cochlear tissue had a median word recognition score of 45%, significantly lower than the overall cohort median of 70%.
02Ears with TMPRSS3 variants had a median word recognition score of 30%, below the cohort's lower quartile of 45%.
03GJB2, the most frequently affected gene, had a median word recognition score of 80%.
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
Many single-gene medians were based on very few ears; for two-thirds of the genes, the median came from three or fewer implanted ears, so single-gene predictions are unstable. The statistical unit mattered. When the analysis used 76 subjects instead of 123 ears, the overall median WRS 65 changed from 70% to 65%, and the neural cluster result became a trend rather than significant. The study did not include CI processor use time, which is an important factor for speech recognition outcomes. It was retrospective, drew on 25 years of implantations, and included all implant types without a phenotype restriction, so results may not apply to a specific modern implant program. The cohort was limited to patients with a definitive genetic diagnosis and German language proficiency, excluding non-genetic etiologies and patients who could not use the German speech test, so generalizability is limited. The analysis examined 30 parameters, increasing the chance that some findings are unstable or driven by small groups.
Declared interests
The authors have no conflicts of interest to disclose.
The easy way to misread this
Do not read the neural genetic median of 45% as a prediction that every patient with a neural diagnosis will perform poorly. The neural cluster included ears with 0% performance and ears with 90% to 95% performance, and the study was retrospective with small gene subgroups.