Do people with cognitive impairment benefit from cochlear implants? A scoping review.

PURPOSE: To identify and evaluate the evidence for the benefits of cochlear implants for people with cognitive impairment or dementia in terms of speech recognition, quality of life, behavioural and psychological symptoms of dementia, cognition, function in daily life, mental well-being, and caregiver burden. METHODS: Ten electronic databases were searched systematically from inception to December 2023 for studies reporting on outcomes for cochlear implants that included adults identified with cognitive impairment, mild cognitive impairment, or dementia. RESULTS: Thirteen studies were included in this review with a combined total of 222 cochlear implant patients with cognitive impairment, mild cognitive impairment. Two studies were non-randomised controlled design, the remainder were single group studies, case series or single case studies. Evidence suggested that people with cognitive impairment benefit in terms of improved speech recognition from cochlear implants, although they may benefit less than those with healthy cognition and the degree of benefit depends on the level of cognitive impairment. There was no evidence for increased adverse events among those with cognitive impairment. There was limited or no evidence for any other outcome. CONCLUSION: People with cognitive impairment or dementia do benefit from cochlear implants. To inform policy and clinical practice, further data are needed about the broader benefits of cochlear implants for people with cognitive impairment or dementia, and referral, eligibility, and cochlear implant support needs for people with cognitive impairment and their caregivers.

Electrically evoked compound action potentials are associated with the site of intracochlear stimulation.

OBJECTIVES: Objective measurements to predict the position of a cochlear electrode during cochlear implantation surgery may serve to improve the surgical technique and postoperative speech outcome. There is evidence that electrically evoked compound action potentials (ECAP) are a suitable approach to provide information about the site of stimulation. This study aims to contribute to the knowledge about the association between the intraoperative intracochlear ECAP characteristics and the site of stimulation. METHODS: In a retrospective cohort study, patients undergoing cochlear implant surgery with flexible lateral wall electrode arrays (12 stimulating channels) between 2020 and 2022 were analyzed. The CDL was measured using a CT-based clinical planning software. ECAP were measured for all electrode contacts and associated to the CDL as well as to the site of stimulation in degree. RESULTS: Significant differences among the amplitudes and slopes for the individual stimulated electrode contacts at the stimulation sites of 90°, 180°, 270°, 360°, 450° and 540° were found. The values showed a trend for linearity among the single electrodes. CONCLUSIONS: ECAP characteristics correlate with the electrode's position inside the cochlea. In the future, ECAP may be applied to assess the intracochlear position inside the cochlea and support anatomy-based fitting.

Comparing linear and non-linear models to estimate the appropriate cochlear implant electrode array length-are current methods precise enough?

PURPOSE: In cochlear implantation with flexible lateral wall electrode arrays, a cochlear coverage (CC) range between 70% and 80% is considered ideal for optimal speech perception. To achieve this CC, the cochlear implant (CI) electrode array has to be chosen according to the individual cochlear duct length (CDL). Here, we mathematically analyzed the suitability of different flexible lateral wall electrode array lengths covering between 70% and 80% of the CDL. METHODS: In a retrospective cross-sectional study preoperative high-resolution computed tomography (HRCT) from patients undergoing cochlear implantation was investigated. The CDL was estimated using an otosurgical planning software and the CI electrode array lengths covering 70-80% of the CDL was calculated using (i) linear and (ii) non-linear models. RESULTS: The analysis of 120 HRCT data sets showed significantly different model-dependent CDL. Significant differences between the CC of 70% assessed from linear and non-linear models (mean difference: 2.5 mm, p < 0.001) and the CC of 80% assessed from linear and non-linear models (mean difference: 1.5 mm, p < 0.001) were found. In up to 25% of the patients none of the existing flexible lateral wall electrode arrays fit into this range. In 59 cases (49,2%) the models did not agree on the suitable electrode arrays. CONCLUSIONS: The CC varies depending on the underlying CDL approximation, which critically influences electrode array choice. Based on the literature, we hypothesize that the non-linear method systematically overestimates the CC and may lead to rather too short electrode array choices. Future studies need to assess the accuracy of the individual mathematical models.

Third-party disability in cochlear implant users.

OBJECTIVE: To date, auditory rehabilitation mainly focuses on the person with hearing impairment (PHI). This study aimed to analyse the burden of hearing loss on significant others (SOs), and to explore the impact of contextual and mediating psychosocial co-factors and auditory rehabilitation by cochlear implantation (CI). DESIGN AND STUDY SAMPLE: Third-party disability (SOS-HEAR) and quality of life (Nijmegen Cochlear Implant Questionnaire) were evaluated in 41 PHI scheduled for CI surgery and their close partners pre- and 6-month post-implantation. Further, age, hearing status, educational level, depressive symptoms (GDS-15), coping strategies (Brief-COPE), resilience (RS-13), stress (PSQ) of SOs and PHI were studied. RESULTS: Hearing loss imposes a burden on SOs, particularly in relation to changes in communication and socialisation. Third-party disability was higher in SOs of PHI with lower educational background (p = 0.04) and of advanced age (p = 0.008). Hearing status of SOs negatively correlated with SOS-HEAR (p = 0.04). After CI, quality of life of PHI and third-party disability of SOs improved (p < 0.001), except in relationship changes. SOs with higher pre-operative burden also experienced more third-party disability afterwards (p ≤ 0.003). CONCLUSION: Audiological rehabilitation should expand to include SOs in the rehabilitation process, as the burden experienced by SOs might persist even after CI.

Effect of Cochlear Implant Electrode Insertion Depth on Speech Perception Outcomes: A Systematic Review.

Objective: The suitable electrode array choice is broadly discussed in cochlear implantation surgery. Whether to use a shorter electrode length under the aim of structure preservation versus choosing a longer array to achieve a greater cochlear coverage is a matter of debate. The aim of this review is to identify the impact of the insertion depth of a cochlear implant (CI) electrode array on CI users' speech perception outcomes. Databases Reviewed: PubMed was searched for English-language articles that were published in a peer-reviewed journal from 1997 to 2022. Methods: A systematic electronic search of the literature was carried out using PubMed to find relevant literature on the impact of insertion depth on speech perception. The review was conducted according to the preferred reporting items for systematic reviews and meta-analyses guidelines of reporting. Studies in both, children and adults with pre- or postlingual hearing loss, implanted with a CI were included in this study. Articles written in languages other than English, literature reviews, meta-analyses, animal studies, histopathological studies, or studies pertaining exclusively to imaging modalities without reporting correlations between insertion depth and speech outcomes were excluded. The risk of bias was determined using the "Risk of Bias in Nonrandomized Studies of Interventions" tool. Articles were extracted by 2 authors independently using predefined search terms. The titles and abstracts were screened manually to identify studies that potentially meet the inclusion criteria. The extracted information included: the study population, type of hearing loss, outcomes reported, devices used, speech perception outcomes, insertion depth (linear insertion depth and/or the angular insertion depth), and correlation between insertion depth and the speech perception outcomes. Results: A total of 215 relevant studies were assessed for eligibility. Twenty-three studies met the inclusion criteria and were analyzed further. Seven studies found no significant correlation between insertion depth and speech perception outcomes. Fifteen found either a significant positive correlation or a positive effect between insertion depth and speech perception. Only 1 study found a significant negative correlation between insertion depth and speech perception outcomes. Conclusion: Although most studies reported a positive effect of insertion depth on speech perception outcomes, one-third of the identified studies reported no correlation. Thus, the insertion depth must be considered as a contributing factor to speech perception rather than as a major decisive criterion. Registration: This review has been registered in PROSPERO, the international prospective register of systematic reviews (CRD42021257547), available at https://www.crd.york.ac.uk/PROSPERO/.

Hearing Impairment in Old Age.

BACKGROUND: Hearing impairment associated with old age (presbycusis) is becoming more common because the population is aging. METHODS: This review is based on publications retrieved by a selective search in Medline and Google Scholar, including individual studies, meta-analyses, guidelines, Cochrane reviews, and other reviews. RESULTS: The cardinal symptom of presbycusis is impaired communication due to bilateral hearing impairment. Patients may be unaware of the problem for a long time because of its insidious progression. Evidence suggests that untreated hearing impair- ment in old age can have extensive adverse effects on the patient's mental, physical, and social well-being. Early detection is possible with the aid of simple diagnostic tests or suitable questionnaires. In most cases, bilateral hearing aids are an effective treatment. Surgery is rarely indicated. For patients with uni- or bilateral deafness, a cochlear implant is the treatment of choice. These treatments can improve many patients' quality of life. CONCLUSION: The small amount of evidence that is currently available suggests that presbycusis is underdiagnosed and under- treated in Germany. Early detection by physicians of all specialties, followed in each case by a specialized differential diagnostic evaluation, is a desirable goal.

Can cochlear implantation improve neurocognition in the aging population?

INTRODUCTION: The relationship between cognition and the ability to hear is well known. Due to changes in demographics, the number of people with sensorineural hearing loss and cognitive impairment is increasing. The aim of this study was to identify the impact of hearing rehabilitation via cochlear implantation on cognitive decline among the aging population. PATIENTS AND METHODS: This prospective study included 60 subjects aged between 50 and 84 years (mean 65.8 years, SD=8.9) with a severe to profound bilateral hearing impairment. A computer-based evaluation of short- and long-term memory, processing speed, attention, working memory and inhibition was performed prior to surgery as well as 6 and 12 months after cochlear implantation. Additionally, speech perception at 65 and 80 dB (Freiburger monosyllabic speech test) as well as disease-related (Nijmegen Cochlear Implant Questionnaire) and general (WHOQOL-OLD) quality of life were assessed. RESULTS: Six months postimplantation, speech perception, quality of life and also neurocognitive abilities significantly increased. The most remarkable improvement after 6 months was detected in executive functions such as attention (p<0.001), inhibition (p=0.025) and working memory (n-back: p=0.002; operation span task: p=0.008), followed by delayed recall (p=0.03). In contrast, long-term memory showed a significant change of performance only after 12 months (p=0.021). After 6 months, most cognitive domains remained stable, except working memory assessed by the operation span task, which significantly improved between 6 and 12 months (p<0.001). No correlation was found between cognitive results and duration of deafness, speech perception or quality of life. CONCLUSION: Cochlear implantation does not only lead to better speech perception and quality of life, but has also been shown to improve cognitive skills in hearing impaired adults aged 50 years or more. These effects seem to be independent of each other.

CHARGE and Kabuki syndromes: a phenotypic and molecular link.

CHARGE syndrome is a complex developmental disorder caused by mutations in the chromodomain helicase DNA-binding gene CHD7. Kabuki syndrome, another developmental disorder, is characterized by typical facial features in combination with developmental delay, short stature, prominent digit pads and visceral abnormalities. Mutations in the KMT2D gene, which encodes a H3K4 histone methyltransferase, are the major cause of Kabuki syndrome. Here, we report a patient, who was initially diagnosed with CHARGE syndrome based on the spectrum of inner organ malformations like choanal hypoplasia, heart defect, anal atresia, vision problems and conductive hearing impairment. While sequencing and MLPA analysis of all coding exons of CHD7 revealed no pathogenic mutation, sequence analysis of the KMT2D gene identified the heterozygous de novo nonsense mutation c.5263C > T (p.Gln1755*). Thus, our patient was diagnosed with Kabuki syndrome. By using co-immunoprecipitation, immunohistochemistry and direct yeast two hybrid assays, we could show that, like KMT2D, CHD7 interacts with members of the WAR complex, namely WDR5, ASH2L and RbBP5. We therefore propose that CHD7 and KMT2D function in the same chromatin modification machinery, thus pointing out a mechanistic connection, and presenting a probable explanation for the phenotypic overlap between Kabuki and CHARGE syndromes.

Aarskog-Scott syndrome: a novel mutation in the FGD1 gene associated with severe craniofacial dysplasia.

UNLABELLED: Aarskog syndrome (AAS) is an X-linked human disease that affects the skeletal formation and embryonic morphogenesis and is caused by mutations in the FGD1 gene. Patients typically show distinctive skeletal and genital developmental abnormalities, but a broad spectrum of clinical phenotypes has been observed. We report here on the clinical and molecular analysis of a family that reveals a novel FGD1 mutation in a 9-year-old boy displaying extreme craniofacial dysplasia associated with attention deficit hyperactivity disorder. Sequencing of FGD1 revealed a novel mutation in exon 7 at position c.1468 C > T in the index patient, leading to a stop codon in the highly conserved RhoGEF gene domain. His mother and maternal grandmother were also found to be heterozygous for this FGD1 mutation. CONCLUSION: Our results identify a novel mutation of FDG1 in a family with Aarskog syndrome and underscore the phenotypical variability of this condition.

Intraoral pressure patterns during swallowing.

Swallowing disturbances are common after neurological disease and oropharyngeal tumor resection. In this case the oral stage is often affected. So far the clinical evaluation of the oral phase is limited. Recently the role of pressure changes during oropharyngeal swallowing has been pointed out, but until now there are not enough data. Thereby 52 healthy adults aged between 20 and 45 years were examined using an oral shield (Silencos(®), Bredent, Senden, Germany) connected to a digital manometer (GDUSB 1000(®), Greisinger electronics, Regenstauf, Germany) able to record pressures in a range of 2,000 to -1,000 mbar at a frequency of 1 kHz. Three swallowing conditions were measured: an active bolus intake (ABI) of water, a passive bolus application of a water-bolus (PWA) and a passive application of a gel-bolus (PGA). We found negative pressures with a median value of -278.9 mbar during ABI, of -24.2 mbar during PWA and of -29.4 mbar during PGA. Significant differences in pressure amplitudes and the pressure pattern were observed depending on the kind of bolus application and its consistency. The used test presents a simple and easy to handle method to assess the oral phase of swallowing.

Lymphangiosis carcinomatosa in squamous cell carcinomas of larynx and hypopharynx--value of conventional evaluation and additional immunohistochemical staining of D2-40.

BACKGROUND: Recent studies revealed a predictive value of lymphatic vessel invasion (L1) for the nodal metastasizing and poor prognosis in malignant tumors at different sites. The monoclonal antibody D2-40 (podoplanin) stains specifically endothelial cells of lymphatic vessels and improves the search for L1. However, the importance of this immunohistochemical staining was not investigated in squamous cell carcinomas (SCC) of larynx and hypopharynx. AIM: This study was performed to compare the diagnostic potential of conventional and immunohistochemical determination of L1 in SCC of larynx and hypopharynx with special respect to the predictive value for nodal metastasizing and prognosis. METHODS: 119 SCCs of the larynx (n = 70) respectively hypopharynx (n = 49) were investigated. The lymphatic vessel invasion was assessed by conventional method (HE stain) and immunohistochemical staining with an antibody against D2-40 (DAKO, Germany). Immunohistochemistry was performed in accordance with manufacturer's protocol. L1 was searched microscopically in a standardized magnification (x200) in serial sections of tumor samples (1 section per cm tumor diameter). RESULTS: The immunohistochemical investigation did not show significant advantages for the prediction of regional nodal metastases. Despite a low sensitivity (< 50%) in both methods, the specificity can reach 80%. The negative predictive value in both methods seems acceptable (up to 80%), whereas the positive predictive value is not higher than 64%. Cases with L1 detected either conventionally or immunohistochemically did not show a significant shorter survival than cases with L0. However, a non-significant shorter survival was found. Only in SCC of hypopharynx, a combination of both methods revealed patients with a significant worse prognosis. CONCLUSION: The status of lymphatic vessel invasion should be documented in standardized tumor reports. A benefit of an additional immunohistochemical investigation was not found, for the daily routine HE-stain seems sufficient.

[Cylindrocarcinoma in a patient with Brooke-Spiegler syndrome]. / Malignes dermales Zylindrom bei der Brooke-Spiegler-Phakomatose.

BACKGROUND: Cutaneous cylindromas are benign adnexal tumors that may occur as a solitary lesion or in a multiple familiar pattern. Malignant change is rare. The association of multiple trichoepitheliomas and cylindromas, the so called Brooke-Spiegler Syndrome, is supposed to be inherited in an autosomal dominant manner. PATIENTS: A 55 year old woman presented with multiple skin tumors on the scalp, as well as the nasolabial and periauricular area since the age of 20 years. Her daughter and her nephew were also affected. Tumors were surgically removed for cosmetic reasons and showed histological signs of benign cylindromas and trichoepitheliomas. 35 years after diagnosis patient presented with a large exulceration on the right occipital area with intracranial invasion. Staging revealed multiple metastases in both lungs. Patient died 1 month later. RESULTS: Histological examination showed beside zones of massive necrosis multiple mitosis. The jigsaw puzzle pattern and the thick PAS positive basal layer, the striking characteristics of benign cylindromas, were lost. These histological findings and the clinical presentation led to the diagnosis of cylindrocarcinoma. CONCLUSIONS: Cylindrocarcinoma is an aggressive tumor with tendency to a local destructive growth and metastases. As malignant transformation of a benign cylindroma occurs more often in the multiple form a close follow-up of patients with multiple cylindromas is necessary. A family study is indicated.