Patient and clinician experiences with the multidisciplinary single-day cochlear implant selection (MSCS) protocol.

PURPOSE: This study assessed the MSCS (Multidisciplinary Single-day Cochlear Implant Selection) protocol with a primary focus on sustaining or enhancing patient satisfaction throughout the cochlear implant selection process. MATERIALS AND METHODS: Following the implementation of the new selection protocol, where all selection appointments take place on the same day, we surveyed 37 individuals who underwent the process. Twenty adhered to the standard procedure, while 17 followed the MSCS protocol. We also gathered feedback from seven out of eight involved healthcare providers. This method enabled us to evaluate the protocol's effectiveness in maintaining patient satisfaction and ensuring staff contentment with care delivery within a condensed timeframe. RESULTS: Patient responses showed slight variations in average scores without statistical significant differences, indicating comparable satisfaction between the MSCS pathway and the standard protocol. The majority of patients preferred the MSCS protocol, with none of the MSCS participants opting for appointments spread over multiple days. Healthcare practitioners of the CI center also displayed similar or increased satisfaction levels with the MSCS protocol. CONCLUSION: The adoption of the MSCS in daily clinical care has led to a decrease in patient appointment times without sacrificing patient satisfaction. Additionally, the majority of individuals actively choose the MSCS protocol. Among those who have directly experienced it, there is unanimous preference for the consolidated appointments over spreading them across multiple days. Professionals within the CI team express equal satisfaction with both the new and old protocols, indicating that the reduction in patient time does not diminish overall satisfaction.

Pilot study of a multidisciplinary single-day cochlear implant selection protocol.

PURPOSE: This study aimed to explore and introduce the potential of a MSCS (Multidisciplinary Single-day Cochlear Implant Selection) protocol. The primary objectives of this pilot were to reduce the duration between referral and surgery, minimize hospital visits and decrease the time healthcare professionals dedicate to the cochlear implant (CI) selection process. MATERIALS AND METHODS: We established a pilot program at the CI center of the Erasmus MC, a tertiary referral center in the Netherlands, with the goal of improving and shorten the selection process. We evaluated our pilot, including 15 CI candidates, and conducted a retrospective analysis for time and cost savings. RESULTS: The results showed that the pilot of the MSCS protocol significantly reduced the length of the CI selection phase (84 days vs 1; standard intake vs MSCS protocol) and the number of hospital visits (6 vs 2 visits; standard vs MSCS protocol), resulting in less travel time and lower costs for the CI candidates. The total time of professionals spend on patients was also reduced with 27 %. CONCLUSION: This study highlights the potential benefits of the MSCS protocol in terms of reducing the burden on patients and healthcare providers and improving the efficiency of the CI selection process.

Analysis of Rotterdam Study cohorts confirms a previously identified RIPOR2 in-frame deletion as a prevalent genetic factor in phenotypically variable adult-onset hearing loss (DFNA21) in the Netherlands.

BACKGROUND: A 12-nucleotide RIPOR2 in-frame deletion was recently identified as a relatively common and highly penetrant cause of autosomal dominant non-syndromic sensorineural hearing loss, type DFNA21, in the Netherlands. The associated hearing phenotype is variable. The allele frequency (AF) of 0.039% of this variant was determined in a local cohort, and the reported phenotype may be biased because studied families were identified based on index patients with hearing loss (HL). In this study, we determine the AF in a cohort from a different geographical region of the Netherlands. Additionally, we examine the hearing phenotype in individuals with the variant but not selected for HL. METHODS: The AF was determined in participants of the Rotterdam Study (RS), a large cohort study. The phenotype was characterised using individual clinical hearing data, including audiograms. RESULTS: The observed AF in the RS cohort was 0.072% and not statistically significantly different from the previously observed 0.039%. The AF in the two cohorts combined was 0.052%. Consistent with previous findings, we found a highly variable audiometric phenotype with non-penetrance of HL in 40% of subjects aged 55-81, which is higher than the 10% at age 50 previously observed. CONCLUSION: We found an overall higher AF and lower penetrance than previously reported, confirming that DFNA21 is relatively common in the Netherlands. This supports its potential suitability as a target for therapeutic development. Studying possible modifying factors is essential to explain the phenotypical variability and to identify patients eligible for such a therapy.

Risk Factors For Hearing Decline From Childhood To Early Adolescence.

OBJECTIVE: To identify risk factors of hearing decline between 9 and 13 years of age. The risk factors examined included sociodemographic, health, and lifestyle-related factors. METHODS: This study was embedded within a population-based prospective cohort study from fetal life onwards in the Netherlands. Pure-tone audiometry and tympanometry were performed at the age of 9 and 13 years. The hearing decline was defined as an increase in low-frequency or high-frequency pure-tone average of at least 5 dB in one of both ears. Multivariable logistic regression was performed to examine the association of possible risk factors with hearing decline. The study was conducted from April 2012 to October 2015, and from April 2016 to September 2019. RESULTS: Of the 3,508 participants included, 7.8% demonstrated a hearing decline in the low frequencies, and 11.3% in the high frequencies. Participants who reported alcohol consumption were more likely to have a hearing decline in the low frequencies (OR 1.5, 95% CI 1.1; 2.0). Moreover, a lower educational level was associated with an increased odds of having a hearing decline in the high frequencies (OR 1.4, 95% CI 1.0; 1.8). Age, sex, household income, personal music player use, and body mass index were not associated with hearing decline. CONCLUSION: Educational level and risky behavior were significantly associated with hearing decline from childhood to early adolescence. The findings of the present study can help in the design of public health interventions to prevent hearing loss at a young age. LEVEL OF EVIDENCE: 2 (prospective cohort study) Laryngoscope, 133:389-395, 2023.

The impact of face masks on the communication of adults with hearing loss during COVID-19 in a clinical setting.

OBJECTIVE: The aim of the study was to investigate the effect of surgical masks and face shields on speech intelligibility of adults with moderate to severe hearing loss. DESIGN: This study measured speech tracking scores in quiet for life speech in three different conditions: without a mask, with a surgical mask and with a face shield. Acoustic effects of the masks and face shields on the speech signal were also investigated. Study sample: The study sample consists of 42 patients with moderate to severe hearing loss, 23 cochlear implant users and 19 hearing aid users. RESULTS: A significant average difference in speech perception scores was found for the use of a surgical mask compared to the listening situation "without mask". The worse the speech understanding in quiet, the larger the impact of the surgical mask. For the worse performers even the face shield had a negative impact on speech perception. The sound distortion for the face shield compared to the surgical mask was greater. CONCLUSION: This study shows that even for speech perception in quiet, surgical face masks, and face shields to a lesser extent, have a negative effect for patients with moderate to severe hearing loss.

Impact of face masks in public spaces during COVID-19 pandemic on daily life communication of cochlear implant users.

OBJECTIVE/HYPOTHESIS: Investigate potential problems in the daily life communication of cochlear implant (CI) patients due to the widespread use of face masks in public places during the COVID-19 pandemic. STUDY DESIGN: Prospective survey study. METHODS: This study used an online questionnaire about the effects of face masks on daily life communication of adult CI users. The questionnaire consists of three parts: (a) A face mask questionnaire, (b) loneliness question (c) three subdomains of the Nijmegen Cochlear Implant Questionnaire to assess quality of life. The questionnaires were send out on October 20, 2020. Four hundred and seven adult CI users were invited to participate in the study. The survey inclusion was closed on November 5, 2020. The study setting was the Rotterdam Cochlear Implant Center, Erasmus MC, a tertiary referral center in The Netherlands. The loneliness question and Nijmegen Cochlear Implant Questionnaire were analyzed for possible differences between the current situation with masks and the situation before, when masks were not commonly used. RESULTS: Two hundred and twenty one adult CI users (54% female, mean age 62 years) participated in the study. The face mask questionnaire showed that face masks cause considerable problems in daily life communication of 80% of the participants. Also, CI users tend to feel more lonely and all used subdomains of the Nijmegen Cochlear Implant Questionnaire worsened due to the use of face masks. CONCLUSION: The widespread use of face masks greatly complicates the daily life communication of CI users and reduces quality of life. LEVEL OF EVIDENCE: 3.

Tinnitus Affects Speech in Noise Comprehension in Individuals With Hearing Loss.

BACKGROUND: Tinnitus is a prevalent auditory disorder that frequently co-occurs with hearing loss. It is suggested that tinnitus might have negative impact on speech perception. However, studies thus far have not been able to disentangle tinnitus, hearing loss, and speech in noise intelligibility. We therefore investigated whether there is an association between tinnitus and speech understanding in noise, independent of hearing loss. METHODS: Of 4,211 participants from the population-based Rotterdam Study (mean age 67.8 [SD 8.9], 57.3% female) data was available on tinnitus, pure-tone audiometry, and digits in noise test. We compared the speech reception threshold in noise (SRTn) in participants with and without tinnitus for the whole population as well as for subgroups stratified for average hearing threshold in 10-dB strata. Additionally, we regressed tinnitus on SRTn with a multivariable regression model, adjusting for sex, age, highest achieved education, and cognitive function. RESULTS: Participants with tinnitus (20.8%) had a higher SRTn (-3.6 dB [SD 3.7] versus -4.6 dB [SD 3.1]). This difference remained only in the subgroups of participants with hearing loss, between 0.6 and 0.8 dB difference in the SRTn for the different subgroups. In the fully adjusted model tinnitus was associated with 0.2 dB (95% CI 0.00, 0.39) SRTn increase. CONCLUSION: We have shown that tinnitus is associated with speech intelligibility in noise, but it is a small effect, only found in people with co-occurring hearing loss.

Assessing hearing loss in older adults with a single question and person characteristics; Comparison with pure tone audiometry in the Rotterdam Study.

INTRODUCTION: Hearing loss (HL) is a frequent problem among the elderly and has been studied in many cohort studies. However, pure tone audiometry-the gold standard-is rather time-consuming and costly for large population-based studies. We have investigated if self-reported hearing loss, using a multiple choice question, can be used to assess HL in absence of pure tone audiometry. METHODS: This study was performed within 4,906 participants of the Rotterdam Study. The question (in Dutch) that was investigated was: 'Do you have any difficulty with your hearing (without hearing aids)?'. The answer options were: 'never', 'sometimes', 'often' and 'daily'. Mild hearing loss or worse was defined as PTA0.5-4(Pure Tone Average 0.5, 1, 2 & 4 kHz) ≥20dBHL and moderate HL or worse as ≥35dBHL. A univariable linear regression model was fitted with the PTA0.5-4 and the answer to the question. Subsequently, sex, age and education were added in a multivariable linear regression model. The ability of the question to classify HL, accounting for sex, age and education, was explored through logistic regression models creating prediction estimates, which were plotted in ROC curves. RESULTS: The variance explained (R2) by the univariable regression was 0.37, which increased substantially after adding age (R2 = 0.60). The addition of sex and educational level, however, did not alter the R2 (0.61). The ability of the question to classify hearing loss, reflected in the area under the curve (AUC), was 0.70 (95% CI 0.68, 0.71) for mild hearing loss or worse and 0.86 (95% CI 0.85, 0.87) for moderate hearing loss or worse. The AUC increased substantially when sex, education and age were taken into account (AUC mild HL: 0.73 (95%CI 0.71, 0.75); moderate HL 0.90 (95%CI 0.89, 0.91)). CONCLUSION: Self-reported hearing loss using a single question has a good ability to detect hearing loss in older adults, especially when age is accounted for. A single question cannot substitute audiometry, but it can assess hearing loss on a population level with reasonable accuracy.

Comparing Two Hearing Aid Fitting Algorithms for Bimodal Cochlear Implant Users.

OBJECTIVES: To investigate the possible advantage of the use of a dedicated bimodal hearing aid fitting formula, the Adaptive Phonak Digital Bimodal (APDB), compared with a frequently used standard hearing aid fitting formula, the NAL-NL2. We evaluated the effects of bimodal hearing aid fitting on provided hearing aid gain and on bimodal auditory functioning in a group of experienced bimodal cochlear implant (CI) users. A second aim of our study was to determine the effect of broadband loudness balancing on the prescribed gain of those two fitting formulas. DESIGN: This prospective study used a crossover design in which two fitting methods were compared varying in basic prescription formula (NAL-NL2 or APDB fitting formula). The study consisted of a three-visit crossover design with 3 weeks between sessions. Nineteen postlingually deafened experienced bimodal CI users participated in this study. Auditory functioning was evaluated by a speech in quiet test, a speech in noise test, and a questionnaire on auditory performance. RESULTS: Significant differences between the two fitting formulas were found for frequencies of 2000 Hz and above. For these frequencies, less gain was provided by the APDB fitting formula compared with NAL-NL2. For the APDB fitting formula, a higher compression ratio for frequencies of 1000 Hz and above was found compared with the NAL-NL2 fitting formula. Loudness balancing did not result in large deviations from the prescribed gain by the initial fitting formula. Bimodal benefit was found for speech perception in quiet and for speech perception in noise. No differences in auditory performance were found between the two fitting formulas for any of the auditory performance tests. CONCLUSIONS: The results of this study show that CI users with residual hearing at the contralateral ear can benefit from bimodal stimulation, regardless of the fitting method that was applied. Although significant differences between the output and compression ratio of the NAL-NL2 and the APDB fitting formula existed, no differences in bimodal auditory performance were observed. Therefore, NAL-NL2 or the APDB fitting prescription both seem suited for bimodal fitting purposes. Additional loudness balancing has a marginal effect on the provided hearing aid output.

A directional remote-microphone for bimodal cochlear implant recipients.

To evaluate whether speech recognition in noise differs according to whether a wireless remote microphone is connected to just the cochlear implant (CI) or to both the CI and to the hearing aid (HA) in bimodal CI users. The second aim was to evaluate the additional benefit of the directional microphone mode compared with the omnidirectional microphone mode of the wireless microphone. This prospective study measured Speech Recognition Thresholds (SRT) in babble noise in a 'within-subjects repeated measures design' for different listening conditions. Eighteen postlingually deafened adult bimodal CI users. No difference in speech recognition in noise in the bimodal listening condition was found between the wireless microphone connected to the CI only and to both the CI and the HA. An improvement of 4.1 dB was found for switching from the omnidirectional microphone mode to the directional mode in the CI only condition. The use of a wireless microphone improved speech recognition in noise for bimodal CI users. The use of the directional microphone mode led to a substantial additional improvement of speech perception in noise for situations with one target signal.

The Effect of Binaural Beamforming Technology on Speech Intelligibility in Bimodal Cochlear Implant Recipients.

Although the benefit of bimodal listening in cochlear implant users has been agreed on, speech comprehension remains a challenge in acoustically complex real-life environments due to reverberation and disturbing background noises. One way to additionally improve bimodal auditory performance is the use of directional microphones. The objective of this study was to investigate the effect of a binaural beamformer for bimodal cochlear implant (CI) users. This prospective study measured speech reception thresholds (SRT) in noise in a repeated-measures design that varied in listening modality for static and dynamic listening conditions. A significant improvement in SRT of 4.7 dB was found with the binaural beamformer switched on in the bimodal static listening condition. No significant improvement was found in the dynamic listening condition. We conclude that there is a clear additional advantage of the binaural beamformer in bimodal CI users for predictable/static listening conditions with frontal target speech and spatially separated noise sources.

Evaluation of a wireless remote microphone in bimodal cochlear implant recipients.

OBJECTIVE: To evaluate the benefit of a wireless remote microphone (MM) for speech recognition in noise in bimodal adult cochlear implant (CI) users both in a test setting and in daily life. DESIGN: This prospective study measured speech reception thresholds in noise in a repeated measures design with factors including bimodal hearing and MM use. The participants also had a 3-week trial period at home with the MM. STUDY SAMPLE: Thirteen post-lingually deafened adult bimodal CI users. RESULTS: A significant improvement in SRT of 5.4 dB was found between the use of the CI with the MM and the use of the CI without the MM. By also pairing the MM to the hearing aid (HA) another improvement in SRT of 2.2 dB was found compared to the situation with the MM paired to the CI alone. In daily life, participants reported better speech perception for various challenging listening situations, when using the MM in the bimodal condition. CONCLUSION: There is a clear advantage of bimodal listening (CI and HA) compared to CI alone when applying advanced wireless remote microphone techniques to improve speech understanding in adult bimodal CI users.

Prevalence of age-related hearing loss, including sex differences, in older adults in a large cohort study.

OBJECTIVES/HYPOTHESIS: To obtain actual status of age-related hearing loss in a general unscreened population of older Dutch adults and to investigate whether the prevalence or degree has changed over time. STUDY DESIGN: To investigate the prevalence and degree of hearing loss, we conducted a large prospective cohort study of older adults between February 2011 and July 2015. METHODS: Pure-tone air- and bone-conduction thresholds were measured for 4,743 participants. Results were compared to previous cohort studies. RESULTS: As expected, hearing loss increased with age. We found a correlation of R2 = 0.317 for men and R2 = 0.354 for women (right ears). A prevalence of hearing loss greater than 35 dB hearing level the average of 0.5/1/2/4 kHz in the better ear, was found in 33% of the male and almost 29% of the female participants aged 65 years and older. Compared with previous studies, men had less hearing loss at the frequencies of 2 kHz and above. Hearing thresholds in women were significantly higher at 4 and 8 kHz. The difference in hearing loss between men and women is significantly less than in earlier studies. CONCLUSIONS: Our study confirms that hearing loss is highly prevalent in the general unscreened population of older adults. However, the difference in hearing between sexes was considerably less than previously reported. This is probably due to changing lifestyle and environmental circumstances, LEVEL OF EVIDENCE: 2b Laryngoscope, 127:725-730, 2017.

Using the Digits-In-Noise Test to Estimate Age-Related Hearing Loss.

OBJECTIVE: Age-related hearing loss is common in the elderly population. Timely detection and targeted counseling can lead to adequate treatment with hearing aids. The Digits-In-Noise (DIN) test was developed as a relatively simple test to assess hearing acuity. It is a potentially powerful test for the screening of large populations, including the elderly. However, until to date, no sensitivity or specificity rates for detecting hearing loss were reported in a general elderly population. The purpose of this study was to evaluate the ability of the DIN test to screen for mild and moderate hearing loss in the elderly. DESIGN: Data of pure-tone audiometry and the DIN test were collected from 3327 adults ages above 50 (mean: 65), as part of the Rotterdam Study, a large population-based cohort study. Sensitivity and specificity of the DIN test for detecting hearing loss were calculated by comparing speech reception threshold (SRT) with pure-tone average threshold at 0.5, 1, 2, and 4 kHz (PTA0.5,1,2,4). Receiver operating characteristics were calculated for detecting >20 and >35 dB HL average hearing loss at the best ear. RESULTS: Hearing loss varied greatly between subjects and, as expected, increased with age. High frequencies and men were more severely affected. A strong correlation (R = 0.80, p < 0.001) was found between SRTs and PTA0.5,1,2,4. Moreover, 65% of variance in SRT could be explained by pure-tone thresholds. For detecting mild or moderate hearing loss, receiver operating characteristics showed areas under the curve of 0.86 and 0.98, respectively. CONCLUSIONS: This study demonstrates that the DIN test has excellent test characteristics when screening for moderate hearing loss (or more) in an elderly population. It is less suited to screen for mild hearing loss. The test is easy to complete and should be suitable for implementation as an automated self-test in hearing screening programs. Ultimately, when combined with active counseling, hearing screening could lead to higher hearing aid coverage in the hearing impaired elderly.

Hearing loss in Pompe disease revisited: results from a study of 24 children.

Little information is available regarding the auditory function in Pompe patients. Hearing loss has been reported in classic infantile patients, but it is still unknown whether central nervous system involvement interferes with auditory function and whether enzyme replacement therapy can improve hearing. Auditory function has not been studied in children with milder forms of the disease. We analyzed repetitive auditory brainstem response measurements and pure tone audiometry in 24 children with Pompe disease. Only 1 of 13 patients with milder phenotypes showed recurrent conductive hearing loss, while 10 out of 11 classic infantile patients had sensorineural hearing defects. These patients also had a high prevalence of conductive hearing loss. Five patients showed evidence of mild retrocochlear pathology, suggestive of glycogen accumulation in the central nervous system. Hearing loss persisted during therapy in all patients. The results emphasize the need for careful monitoring of auditory function in classic infantile Pompe patients, and for early implementation of hearing aids to protect speech and language development.