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BACKGROUND: The incidence of tinnitus has been increasing together with its patient impact and societal costs. Much research has been conducted in the field of tinnitus, especially on treatment modalities because there still is no cure. This study aims to analyze the evolutions and developments in the scientific output relating to tinnitus. METHODS: We analyzed the Science Citation Index Expanded featured articles in the Web of Science Core Collection relating to tinnitus from 1980 to 2020. The publications were analyzed by characteristics such as the countries and institutions, journals, the most cited articles and references, and the most frequently used words in the abstracts and keywords. RESULTS: In total, 8282 articles relating to tinnitus were identified in the Web of Science. The number of publications has been significantly increasing after the 1990s. Of the 8282 articles, a major part originated from the American and European institutions. Most articles originated from high-impact journals, which consequently also covered the most cited papers. A major interest was seen in areas about treatment and pathogenic mechanisms. CONCLUSION: This bibliometric analysis here indicated an increasing trend in tinnitus research from 1980 to 2020, particularly with the increase in tinnitus burden and the societal costs by it. Specific interest has been seen in the specific tinnitus pathophysiological mechanisms and treatment. Individual researchers and institutions will gain a new perspective on their future studies based on the bibliometric data in our paper.

Background: Robot-assisted and image-guided surgery have become increasingly important because it outperforms human dexterity. For procedures on sub-millimet-ric level, fixing the patient’s head firmly is crucial. Although the neck is not the targetorgan of the operation, it may be at risk of postoperative complaints due to position-ing or fixation. The incidence of postoperative symptoms after head and neck surgery with fixation is hardly reported and probably underestimated, in regards to the life-threatening cranial pathologies for which the surgery was required. Methods: To identify papers on risk factors for postoperative complaints after headand neck surgery, we performed a systematic review. PubMed and Web of Sciencedatabases were searched using predefined in- and exclusion criteria. Meta-analysesand reviews were excluded. Postoperative complaints concerned pain, quality oflife, discomfort, neuropraxia, and musculoskeletal problems. This review is reportedaccording to PRISMA guidelines. Results: Seven eligible studies were identified, only 2 concerned surgery requiring head fixation. The significant risk factors resulting from our analysis were preopera- tive pain (odds ratio=2.19), expected pain (odds ratio=2.15), short-term fear (odds ratio = 1.42), age between 45 and 59 years old (odds ratio = 1.40), pain catastrophizing (odds ratio = 1.21), and female gender (odds ratio = 0.74). Conclusion: Six significant risk factors for iatrogenic postoperative complaints after head and neck surgery have been identified. These risk factors should be consideredas possible confounding factors in future research. Little literature could be found. Upcoming robotic surgeries in the head and neck area pose a clinical need for morespecific studies on postoperative iatrogenic complaints.

Fossa navicularis (FN) is defined as bony depression that is not always present and is located anterior to the foramen magnum and pharyngeal tubercle on the inferior aspect of the basilar part of the occipital bone. It has been reported that FN can create an infection spread path from the pharynx to the intracranial structures. Therefore, the diagnosis of this variation is important. Although cone beam computed tomography (CBCT) diagnostic accuracy has mostly been verified in detection and quantification particularly on human skulls, there is no study comparing morphometric measurements between direct measurement on the skull and CBCT measurement. The main object of this study is to evaluate the presence of FN on dry bones and CBCT images of the same dry skull and to examine the morphometric and morphological features of this formation. Thirty-two random craniums that were made available for this study that did not have any fractures or deformities of the cranial base were examined. The sagittal diameter, transverse diameter, and depth of the FN was measured both directly on dry skulls and radiologically on CBCT images of dry skull. In addition, the shape of FN (SFN) was determined. FN was detected in 10 (31.25%) of 32 craniums examined with both modalities. It was determined that sagittal diameter of the FN, transverse diameter of the FN, depth of the FN, and the shape of FN did not show a statistically significant difference between the 2 measurements. Unlike the literature, FN was investigated on dry bones both directly and in CBCT images in this study. In contrast to previously thought the FN may be smaller according to this findings, and this small variation can be detected with CBCT images. According to this findings, it can be said that morphometric evaluations on CBCT are accurate and reliable, and CBCT is a safe method for clinical diagnosis and treatment.

Dizziness is one of the most frequent complaints of patients and up to 20% consult physicians for it every year. Patients can suffer from different types of symptoms, including spontaneous vertigo spells, head-movement-induced vertigo spells and chronic dizziness. All of these symptoms are highly relevant while driving and may put the patient and their surroundings in significant danger. This paper highlights the impact of aspects relevant to the dizzy patient on driving ability (incl. the impact of medication and surgery).

Objective: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI).Design: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire).Results: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain “Attention” (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%.Conclusion: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation.

BACKGROUND: Research on hereditary hearing impairment has had several boosts to identify deafness-causing genes. The number of studies regarding the diagnosis and treatment modalities of hereditary hearing impairment is enormous and increasing; however, little or no research has been conducted for evaluating the development of scientific output and trends in the field. Here, we provide a comprehensive overview of centers that focus their research on hereditary hearing impairment and their scientific output. METHODS: Bibliometric analysis of the publications related to hereditary hearing impairment published between 1980 and 2019 were used in this study, which were also indexed in Web of Science database. RESULTS: The highest number of scientific articles on hereditary hearing impairment came from the United States, and it was also the most cited country. The University of Iowa is a leading center in the domain of hereditary hearing impairment in the world over the last 40 years. Fudan University, Central South University, and Harvard Medical School are also institutions that have had a focus on hereditary hearing impairment. CONCLUSIONS: There is a progressive increase in scientific papers on hereditary hearing impairment over the last 40 years that we have found in our bibliometric study. We identified key centers in the scientific research on hereditary hearing impairment in the world and also key journals that focus on hereditary hearing impairment. This information can facilitate new researchers in this field to seek collaboration with experienced partners, better synthesize the orientation and boundaries of the subject, and find target journals. Ultimately, we provided a certain benchmark value for key centers that perhaps should have a more prominent role in constructing experimental research or even clinical guidelines.

Objective: To compare the cognitive evolution of older adults with severe or profound hearing impairment after cochlear implantation with that of a matched group of older adults with severe hearing impairment who do not receive a cochlear implant (CI). Design: In this prospective, longitudinal, controlled, and multicenter study, 24 older CI users were included in the intervention group and 24 adults without a CI in the control group. The control group matched the intervention group in terms of gender, age, formal education, cognitive functioning, and residual hearing. Assessments were made at baseline and 14 months later. Primary outcome measurements included the change in the total score on the Repeatable Battery for the Assessment of Neuropsychological Status for Hearing impaired individuals score and on its subdomain score to assess cognitive evolution in both groups. Secondary outcome measurements included self-reported changes in sound quality (Hearing Implant Sound Quality Index), self-perceived hearing disability (Speech, Spatial, and Qualities of Hearing Scale), states of anxiety and depression (Hospital Anxiety and Depression Scale), and level of negative affectivity and social inhibition (Type D questionnaire). Results: Improvements of the overall cognitive functioning (p = 0.05) and the subdomain “Attention” (p = 0.02) were observed after cochlear implantation in the intervention group; their scores were compared to the corresponding scores in the control group. Significant positive effects of cochlear implantation on sound quality and self-perceived hearing outcomes were found in the intervention group. Notably, 20% fewer traits of Type D personalities were measured in the intervention group after cochlear implantation. In the control group, traits of Type D personalities increased by 13%. Conclusion: Intervention with a CI improved cognitive functioning (domain Attention in particular) in older adults with severe hearing impairment compared to that of the matched controls with hearing impairment without a CI. However, older CI users did not, in terms of cognition, bridge the performance gap with adults with normal hearing after 1 year of CI use. The fact that experienced, older CI users still present subnormal cognitive functioning may highlight the need for additional cognitive rehabilitation in the long term after implantation.

Deze brief aan de redactie werd ons bezorgd door prof. dr. Vincent Van Rompaey en is een actualisering van het artikel „Hoorimplantaten: wat kan voor welk gehoorverlies en wat brengt de toekomst?” (V. Van Rompaey, V. Topsakal, G. Mertens, P. Van de Heyning), gepubliceerd in nummer 2 van 2019 (Tijdschr Geneesk 2019; 75: 116-122).

DFNA9 is een ziektebeeld dat in de Lage Landen vooral wordt veroorzaakt door de p.P51S-mutatie in het COCH-gen, met een laattijdige aanvang (ongeveer het vierde levensdecennium) van het gehoorverlies én de evenwichtsstoornissen, die binnen een periode van enkele decennia evolueren naar beiderzijdse uitval. Een aantal patiënten kunnen in de beginfase een Ménière-achtig ziektebeeld vertonen onder de vorm van fluctuerend gehoorverlies, druksensaties en aanvallen van duizeligheid. Zowel het gehoor als het evenwicht takelen het snelst af tijdens het vijfde levensdecennium. De exacte aanvang van de evenwichtsstoornissen is minder goed omschreven omdat er weinig presymptomatische dragers werden onderzocht en omdat de vestibulaire symptomen in het begin discreter zijn of sterk kunnen verschillen per individu. Recente studies toonden aan dat bijna de helft van de gehoorgestoorde patiënten hun gehoorprobleem voor het eerst bespreekt met de huisarts, die daarom een belangrijke rol speelt in de aanpak ervan. Bij volwassenen met progressief gehoorverlies en/of een Ménière-achtig ziektebeeld én die een positieve familiale anamnese hebben voor gehoor- en/of evenwichtsproblemen, moet DFNA9 toegevoegd worden aan de differentiaaldiagnose. Dit artikel geeft een overzicht van de nieuwe inzichten in de verschillende mutaties in het COCH-gen, de pathogenese, de verschillende ziektebeelden tussen de mutaties die voorkomen in de Lage Landen, de recente nieuwe radiologische bevindingen bij DFNA9 en de toekomstperspectieven.

SPIRIT 2013 Checklist: Recommended items to address in a clinical trial protocol and related documents. (PDF 1226 kb)

Objectives/HypothesisTo determine the effect of cochlear implantation on tinnitus perception in patients with severe bilateral postlingual sensorineural hearing loss and to demonstrate possible differences between unilateral and bilateral cochlear implantation. Study DesignProspective study. MethodsThirty-eight adult patients were included in this prospective study, as part of a multicenter randomized controlled trial investigating the benefits of bilateral cochlear implantation versus unilateral cochlear implantation. Pre- and postoperative tinnitus perception scores were evaluated, before and 1 year after implantation on three tinnitus questionnaires; the Tinnitus Handicap Inventory (THI), the Tinnitus Questionnaire (TQ), and a visual analogue scale for tinnitus burden. ResultsBefore implantation, the tinnitus prevalence was 42.1% (16 of 38) in the whole study group. One year after implantation, the tinnitus questionnaire scores had decreased in 71.4% according to the TQ and 80.0% according to the THI. Tinnitus was induced after cochlear implantation in six patients, five in the bilateral and one in the unilateral group. ConclusionsOur study shows that cochlear implantation is effective in the reduction of tinnitus in patients with bilateral sensorineural hearing loss who suffered from preoperative tinnitus. Conversely, tinnitus may also increase or even be induced by the cochlear implantation itself. Cochlear implant candidates should be well informed about these possible consequences before undergoing surgery. Level of Evidence2b Laryngoscope, 126:956-961, 2016

OBJECTIVE: This review aimed to evaluate the additional benefit of pediatric cochlear implantation before 12 months of age considering improved speech and language development and auditory performance. MATERIALS AND METHODS: We conducted a search in PubMed, EMBASE and CINAHL databases and included studies comparing groups with different ages at implantation and assessing speech perception and speech production, receptive language and/or auditory performance. We included studies with a high directness of evidence (DoE). RESULTS: We retrieved 3,360 articles. Ten studies with a high DoE were included. Four articles with medium DoE were discussed in addition. Six studies compared infants implanted before 12 months with children implanted between 12 and 24 months. Follow-up ranged from 6 months to 9 years. Cochlear implantation before the age of 2 years is beneficial according to one speech perception score (phonetically balanced kindergarten combined with consonant-nucleus-consonant) but not on Glendonald auditory screening procedure scores. Implantation before 12 months resulted in better speech production (diagnostic evaluation of articulation and phonology and infant-toddler meaningful auditory integration scale), auditory performance (Categories of Auditory Performance-II score) and receptive language scores (2 out of 5; Preschool Language Scale combined with oral and written language skills and Peabody Picture Vocabulary Test). CONCLUSIONS: The current best evidence lacks level 1 evidence studies and consists mainly of cohort studies with a moderate to high risk of bias. Included studies showed consistent evidence that cochlear implantation should be performed early in life, but evidence is inconsistent on all speech and language outcome measures regarding the additional benefit of implantation before the age of 12 months. Long-term follow-up studies are necessary to provide insight on additional benefits of early pediatric cochlear implantation.

Item does not contain fulltext OBJECTIVES/HYPOTHESIS: To evaluate the influence of the introduction of newborn hearing screening programs on the age at cochlear implantation in children. STUDY DESIGN: Retrospective, multicenter cohort study. METHODS: All 1,299 pediatric cochlear implant users who received their implants before the age of 5 years between 1995 and 2011 in the Medical University Hannover, Germany and University Medical Center Utrecht, the Netherlands were enrolled in this study. Age at implantation and the number of children implanted within the first year of life was assessed for each center. RESULTS: Age at cochlear implantation gradually declined over the years in both centers. The introduction of the screening resulted in significant decline in the age at implantation in the Netherlands; simultaneously, the number of children implanted within their first year of life increased significantly. Comparing 4-year epochs immediately before and after introduction of the screening, the mean age decreased from 2.4 to 1.2 years, and the percentage of early implanted children increased from 9% to 37%. In the German population, a similar effect of the introduction of the hearing screening program was absent. CONCLUSIONS: The introduction of the national newborn hearing screening program has reduced the age at cochlear implantation in young children in the Netherlands but not in Germany. Correspondingly, it resulted in an increase in the number of children implanted early in life. The difference between the Dutch and German population might be due to differences in the follow-up and referral after the hearing screening. LEVEL OF EVIDENCE: 2b Laryngoscope, 125:985-990, 2015.

Objective: to investigate the familial correlations and intracall correlation of age-related hearing impairment (ARHI) in specific frequencies. In addition, heritability estimates were calculated. Study design: multicenter survey in 8 European centers. Subjects: one hundred ninety-eight families consisting of 952 family members, screened by otologic examination and structured interviews. Subjects with general conditions, known to affect hearing thresholds or known otologic cause were excluded from the sudy. Results: we detected familial correlation coefficients of 0.36, 0.37, 0.36, and 0.30 for 0.25, 0.5, 1 and 2 kHz respectively, and correlation coefficients of 0.20 and 0.18 for 4 and 8 kHz, respectively. Variance components analyses showed that the proportion of the total variance attributable to family differences was between 0.32 and 0.40 for 0.25, 0.5, 1, and 2 kHz and below 0.20 for 4 and 8 kHz. When testing for homogeneity between sib pair types, we observed a larger familial correlation between female than male subjects. Heritability estimates ranged between 0.79 a,d 0.36 across the frequencies. Discussion: our results indicate that there is a substantial shared familial effect in ARHI. We found that familial aggregation of ARHI is markedly highter in the low frequencies and that there is a trend toward higher familial aggregation in female compared with male subjects.

Contains fulltext : 52415.pdf (Publisher’s version ) (Closed access) HYPOTHESIS: The common GJB2 (Connexin 26) 35delG mutation might contribute to the development of age-related hearing impairment (ARHI) and noise-induced hearing loss (NIHL). BACKGROUND: GJB2, a gene encoding a gap junction protein expressed in the inner ear, has been suggested to be involved in the potassium recycling pathway in the cochlea. GJB2 mutations account for a large number of individuals with nonsyndromic recessive hearing loss, with 35delG being the most frequent mutation in populations of European origin. Other genes involved in potassium homeostasis have been suggested to be associated with ARHI and NIHL, and distortion product otoacoustic emission distortions indicative of hearing loss alterations have been found in 35delG carriers. METHOD: We genotyped 35delG in two distinct sample sets: an ARHI sample set, composed of 2,311 Caucasian samples from nine different centers originating from seven different countries with an age range between 53 and 67 years, and an NIHL sample set consisting of 702 samples from the two extremes of a noise-exposed Polish sample. RESULTS: After statistical analysis, we were unable to detect an association between 35delG and ARHI, nor between 35delG and NIHL. CONCLUSION: Our findings indicate that there is no increased susceptibility in 35delG carriers for the development of ARHI or NIHL.

Substantial evidence shows that cochlear implantation is the preferred treatment for infants presenting with severe to profound hearing loss. However, the sensitive period of neurolinguistic development varies between speech and language domains, and therefore, determining the ideal timing for cochlear implantation based on these time frames remains difficult and has not yet been strictly defined. Furthermore, differences between cochlear implant manufacturers’ and national cochlear implant guidelines exist regarding paediatric cochlear implant candidacy eligibility criteria. Since no uniform indication criteria currently exist, this dissertation formulates an evidence-based guideline for cochlear implantation in children. Definition of uniform indication criteria and a structured timeline of the selection process of pediatric cochlear implant cadidates can help parents understand both clinical processes and thus prevent delay in care. Through three literature studies and four retrospective studies, data were gathered to formulate evidence-based guidelines. Based on our findings, we recommend that children with prelingual hearing loss, without serious co-morbidity, undergo cochlear implantation if: 1. They are between 12 and 18 months old, based on four speech and language domains (speech perception and production, receptive language development and auditory performance) 2. They present with hearing loss of ≥ 80 decibels (2-frequency Pure Tone Average thresholds of ≥ 85 decibels of hearing loss or 4-frequency Pure Tone Average thresholds of ≥ 80 decibels of hearing loss) 3. The mastoidectomy with posterior tympanotomy technique is used peroperatively. In terms of anesthetic technique, both intravenous propofol and sevoflurane (inhalation) gas can be used. In our international assessment, we found that only 30% of the European pediatric population was implanted before the age of 24 months. This delay in care provision is remarkable and can be shortened in the future by: more optimal compliance with already implemented guidelines, improved alignment between international guidelines and more awareness among parents of the impact of hearing loss for their child. Through our evidence-based advice we hope to reduce variation from clinician to clinician. In the future, we expect that by providing information via telemedicine (e.g., the application of mobile applications), parents better understand the indication criteria and the timeline of the candidate selection and delay in auditory rehabilitation can be prevented.

Objectives: MYH9-related disease (MYH9-RD) is an autosomal-dominant disorder deriving from mutations in MYH9, the gene for the nonmuscle myosin heavy chain (NMMHC)-IIA. MYH9-RD has a complex phenotype including congenital features, such as thrombocytopenia, and noncongenital manifestations, namely sensorineural hearing loss (SNHL), nephropathy, cataract, and liver abnormalities. The disease is caused by a limited number of mutations affecting different regions of the NMMHC-IIA protein. SNHL is the most frequent noncongenital manifestation of MYH9-RD. However, only scarce and anecdotal information is currently available about the clinical and audiometric features of SNHL of MYH9-RD subjects. The objective of this study was to investigate the severity and propensity for progression of SNHL in a large series of MYH9-RD patients in relation to the causative NMMHC-IIA mutations. Design: This study included the consecutive patients diagnosed with MYH9-RD between July 2007 and March 2012 at four participating institutions. A total of 115 audiograms were analyzed from 63 patients belonging to 45 unrelated families with different NMMHCIIA mutations. Cross-sectional analyses of audiograms were performed. Regression analysis was performed, and age-related typical audiograms (ARTAs) were derived to characterize the type of SNHL associated with different mutations. Results: Severity of SNHL appeared to depend on the specific NMMHCIIA mutation. Patients carrying substitutions at the residue R702 located in the short functional SH1 helix had the most severe degree of SNHL, whereas patients with the p.E1841K substitution in the coiled-coil region or mutations at the nonhelical tailpiece presented a mild degree of SNHL even at advanced age. The authors also disclosed the effects of different amino acid changes at the same residue: for instance, individuals with the p.R702C mutation had more severe SNHL than those with the p.R702H mutation, and the p.R1165L substitution was associated with a higher degree of hearing loss than the p.R1165C. In general, mild SNHL was associated with a fairly flat audiogram configuration, whereas severe SNHL correlated with downsloping configurations. ARTA plots showed that the most progressive type of SNHL was associated with the p.R702C, the p.R702H, and the p.R1165L substitutions, whereas the p.R1165C mutation correlated with a milder, nonprogressive type of SNHL than the p.R1165L. ARTA for the p.E1841K mutation demonstrated a mild degree of SNHL with only mild progression, whereas the ARTA for the mutations at the nonhelical tailpiece did not show any substantial progression. Conclusions: These data provide useful tools to predict the progression and the expected degree of severity of SNHL in individual MYH9-RD patients, which is especially relevant in young patients. Consequences in clinical practice are important not only for appropriate patient counseling but also for development of customized, genotype-driven clinical management. The authors recently reported that cochlear implantation has a good outcome in MYH9-RD patients; thus, stricter follow-up and earlier intervention are recommended for patients with unfavorable genotypes. Fil: Verver, Eva J.J.. University of Utrecht; Países Bajos Fil: Topsakal, Vedat. University of Utrecht; Países Bajos Fil: Kunst, Henricus P. M.. Radboud University Medical Centre; Países Bajos Fil: Huygen, Patrick L. M.. Radboud University Medical Centre; Países Bajos Fil: Heller, Paula Graciela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Pujol Moix, Nuria. Universitat Autònoma de Barcelona; España Fil: Savoia, Anna. Università degli Studi di Trieste; Italia. Istituti di Ricovero e Cura a Carattere Scientifico; Italia Fil: Benazzo, Marco. Fondazione Policlinico San Matteo; Italia. Istituti di Ricovero e Cura a Carattere Scientifico; Italia Fil: Fierro, Tiziana. Università di Perugia; Italia Fil: Grolman, Wilko. University of Utrecht; Países Bajos Fil: Gresele, Paolo. Università di Perugia; Italia Fil: Pecci, Alessandro. Fondazione Policlinico San Matteo; Italia. Istituti di Ricovero e Cura a Carattere Scientifico; Italia. Università degli Studi di Pavia; Italia