Background The spiral ganglion hypothesis suggests that pathogenic variants in genes preferentially expressed in the spiral ganglion nerves (SGN), may lead to poor cochlear implant (CI) performance... Show moreBackground The spiral ganglion hypothesis suggests that pathogenic variants in genes preferentially expressed in the spiral ganglion nerves (SGN), may lead to poor cochlear implant (CI) performance. It was long thought that TMPRSS3 was particularly expressed in the SGNs. However, this is not in line with recent reviews evaluating CI performance in subjects with TMPRSS3-associated sensorineural hearing loss (SNHL) reporting overall beneficial outcomes. These outcomes are, however, based on variable follow-up times of, in general, 1 year or less. Therefore, we aimed to 1. evaluate long-term outcomes after CI implantation of speech recognition in quiet in subjects with TMPRSS3-associated SNHL, and 2. test the spiral ganglion hypothesis using the TMPRSS3-group. Methods This retrospective, multicentre study evaluated long-term CI performance in a Dutch population with TMPRSS3-associated SNHL. The phoneme scores at 70 dB with CI in the TMPRSS3-group were compared to a control group of fully genotyped cochlear implant users with post-lingual SNHL without genes affecting the SGN, or severe anatomical inner ear malformations. CI-recipients with a phoneme score <= 70% at least 1-year post-implantation were considered poor performers and were evaluated in more detail. Results The TMPRSS3 group consisted of 29 subjects (N = 33 ears), and the control group of 62 subjects (N = 67 ears). For the TMPRSS3-group, we found an average phoneme score of 89% after 5 years, which remained stable up to 10 years post-implantation. At both 5 and 10-year follow-up, no difference was found in speech recognition in quiet between both groups (p = 0.830 and p = 0.987, respectively). Despite these overall adequate CI outcomes, six CI recipients had a phoneme score of <= 70% and were considered poor performers. The latter was observed in subjects with residual hearing post-implantation or older age at implantation. Conclusion Subjects with TMPRSS3-associated SNHL have adequate and stable long-term outcomes after cochlear implantation, equal to the performance of genotyped patient with affected genes not expressed in the SGN. These findings are not in line with the spiral ganglion hypothesis. However, more recent studies showed that TMPRSS3 is mainly expressed in the hair cells with only limited SGN expression. Therefore, we cannot confirm nor refute the spiral ganglion hypothesis. Show less
Kleijbergen, W.J.; Sparreboom, M.; Mylanus, E.A.M.; Koning, G. de; Helleman, W.; Boermans, P.P.B.M.; ... ; Smit, A.L. 2022
Objective: To determine the benefit of sequential cochlear implantation after a long inter-implantation interval in children with bilateral deafness receiving their second implant between 5 and 18... Show moreObjective: To determine the benefit of sequential cochlear implantation after a long inter-implantation interval in children with bilateral deafness receiving their second implant between 5 and 18 years of age. Study design: Prospective cohort-study. Setting: Tertiary multicenter. Patients: 85 children with bilateral deafness and unilateral implantation receiving a contralateral cochlear implant at the age of 5 to 18 years. Method: The primary outcomes were speech recognition in quiet and noise (CVC) scores. The secondary outcomes were language outcomes and subjective hearing abilities, all measured before and 12 months after sequential bilateral cochlear implantation. Medians of the paired data were compared using the Wilcoxon signed-rank test. Univariable linear regression analyses was used to analyze associations between variables and performance outcomes. Results: A significant benefit was found for speech recognition in quiet (96% [89-98] vs 91% [85-96]; p < 0.01) and noise (65% [57-75] vs 54% [47-71]; p = 0.01) in the bilateral CI condition compared to unilateral (n = 75, excluded 10 non-users). No benefit was seen for language outcomes. The subjective sound quality score was statistically significant higher in bilateral compared to the unilateral CI condition. Pre-operative residual hearing level in the ear of the second implant, the inter-implant interval and age at time of second implantation was not significantly associated with performance scores. Conclusion: After 12 months of use, sequential bilateral cochlear implantation showed improved speech perception in quiet and noise and improved subjective sound quality outcomes in children despite a great inter-implantation interval (median of 8 years [range 1-16 years]). Show less
Background. Dynamic predictions on head and neck cancer survival could offer, besides improved patient counseling, insight into long-term effects of tumor- and patient-based characteristics on... Show moreBackground. Dynamic predictions on head and neck cancer survival could offer, besides improved patient counseling, insight into long-term effects of tumor- and patient-based characteristics on survival. Theoretically, there could be a certain time period after diagnosis after which the patient returns to a population risk on survival. Methods. In all, 7255 patients with a primary head and neck squamous cell carcinoma (HNSCC) aged 25 to 90 years, diagnosed between January 1980 and January 2004 in The Netherlands, were included. Conditional 5-year relative survival for every additional year survived was computed. Results. The overall conditional relative prognosis reached a plateau after approximately 4 years; a permanent 20% to 25% excess mortality for long-term HNSCC survivors remained. Conclusions. Conditional 5-year relative survival for patients with HNSCC remains poorer compared to age- and sex-matched counterparts in the general population, even when alive at 15 years after diagnosis. We assume that this is caused by an excess comorbidity in these patients, mainly due to smoking and alcohol abuse. (C) 2010 Wiley Periodicals, Inc. Head Neck 32: 1613-1618, 2010 Show less
Schroeff, M.P. van der; Schie, K. van; Langeveld, T.P.M.; Looman, C.; Jong, R.J.B. de 2010
Dedicated software packages incorporating prognostic models are meant to aid physicians in making accurate predictions of prognosis. This study concerns 742 predictions of 5-year survival on... Show moreDedicated software packages incorporating prognostic models are meant to aid physicians in making accurate predictions of prognosis. This study concerns 742 predictions of 5-year survival on consecutive newly diagnosed patients with head- and neck squamous cell carcinoma. The 5-year survival predictions made by the physicians are not compared with actual survival, but with a prediction made by OncologIQ, a dedicated software package. We used a linear regression and a linear mixed-effects model to look at absolute differences between both predictions and possible learning effects. Predictions made by the physicians were optimistic and inaccurate. Using the linear regression and linear mixed-effects models, the physicians' learning effect showed little improvement per successive prediction. We conclude that prognostic predictions in general are imprecise. When given feedback on the model's predicted survival, the accuracy increases, but only very modestly. Show less
