Patient-reported measures of tinnitus for individuals with neurofibromatosis type 2-related schwannomatosis: Recommendations for clinical trials.

BACKGROUND: Neurofibromatosis type 2-related schwannomatosis is a genetic disease characterized by the development of bilateral vestibular schwannomas, ependymomas, meningiomas, and cataracts. Mild to profound hearing loss and tinnitus are common symptoms reported by individuals with neurofibromatosis type 2. While tinnitus is known to have a significant and negative impact on the quality of life of individuals from the general population, the impact on individuals with neurofibromatosis type 2 is unknown. Consensus regarding the selection of suitable patient-reported outcome measures for assessment could advance further research into tinnitus in neurofibromatosis type 2 patients. The purpose of this work is to achieve a consensus recommendation by the Response Evaluation in Neurofibromatosis and Schwannomatosis International Collaboration for patient-reported outcome measures used to evaluate quality of life in the domain of tinnitus for neurofibromatosis type 2 clinical trials. METHODS: The Response Evaluation in Neurofibromatosis and Schwannomatosis Patient-Reported Outcomes Communication Subgroup systematically evaluated patient-reported outcome measures of quality of life in the domain of tinnitus for individuals with neurofibromatosis type 2 using previously published Response Evaluation in Neurofibromatosis and Schwannomatosis rating procedures. Of the 19 identified patient-reported outcome measures, 3 measures were excluded because they were not validated as an outcome measure or could not have been used as a single outcome measure for a clinical trial. Sixteen published patient-reported outcome measures for the domain of tinnitus were scored and compared on their participant characteristics, item content, psychometric properties, and feasibility for use in clinical trials. RESULTS: The Tinnitus Functional Index was identified as the most highly rated measure for the assessment of tinnitus in populations with neurofibromatosis type 2, due to strengths in the areas of item content, psychometric properties, feasibility, and available scores. DISCUSSION: Response Evaluation in Neurofibromatosis and Schwannomatosis currently recommends the Tinnitus Functional Index for the assessment of tinnitus in neurofibromatosis type 2 clinical trials.

A risk model for occupational noise-induced hearing loss in workers.

BACKGROUND: Occupational hearing loss is one of the most common work-related diseases with various risk factors and considerable negative impacts on both physical and mental well-being of affected workers. Occupational noise-induced hearing loss (ONIHL) has a complex interaction with personal, environmental and occupational factors. OBJECTIVE: This study aimed to develop a risk model for ONIHL in workers by identifying risk factors and their interactions. METHODS: The subjects were 605 males in an industrial factory in Arak, Iran. The study took place between 2022 and 2023. The sociodemographic and occupational characteristics were collected by a health technician using questionnaires and medical records. Hearing status was assessed using audiometry by a qualified audiologist. Hearing loss was analyzed by univariate logistic analysis including age, smoking, medical history, type of occupation, and some workplace hazards. The risk model was generated by logistic regression. RESULTS: Hearing loss in the participants was 44.13% (n = 267). In univariate logistic analysis, age (OR: 2.93,95% CI: 1.848-4.656), smoking (OR: 1.80, 95% CI: 1.224-2.655), work experience (OR: 1.06, 95% CI: 1.016-1.107), previous exposure to noise (OR: 1.60, 95% CI: 1.112-2.312) or vibration (OR: 1.68, 95% CI: 1.150-2.475) and type of occupation (OR: 2.126, 95% CI: 1.055-4.285) were associated with an increased risk of ONIHL (P <  0.05). CONCLUSION: It was found that vibration exposure, work experience, previous noise exposure, type of occupation as well as age and smoking significantly affected the likelihood of developing ONIHL. This risk model could help management to prevent ONIHL and enhance application-oriented research on the condition.

[Newborn hearing screening in Germany-results of the 2011/2012 and 2017/2018 evaluations]. / Neugeborenen-Hörscreening in Deutschland ­ Ergebnisse der Evaluationen 2011/2012 und 2017/2018.

BACKGROUND: Newborn hearing screening (NHS) was introduced nationwide by the Federal Joint Committee (Gemeinsamer Bundesausschuss, G­BA) in 2009. In this process, quality targets were also set in the pediatrics directive. In order to review the quality NHS in Germany, the G­BA commissioned a consortium to conduct an initial evaluation for the years 2011 and 2012 and a follow-up evaluation for 2017 and 2018. METHODS: The evaluations were based on NHS screening parameters (Sammelstatistiken) that must be documented by all obstetrics and neonatology departments as NHS providers and can also be compiled through cooperation with hearing screening centers (HSCs). Additional data were collected through questionnaires and interviews and routine data were used to evaluate the screening process. RESULTS: In 13 federal states, a total of 15 HSCs are involved in the screening process. Across Germany, an NHS screening rate of 86.1% was documented in 2018 (82.4% in 2012), but this differed significantly between the federal states. The specified quality targets could not yet be implemented everywhere. For example, only less than half of the obstetric departments achieved the specified screening rate of over 95%. A comparison of data from the follow-up evaluation and the first evaluation showed that the structural quality of NHS had improved, while the process quality remained the same or had deteriorated. The refer rate (children who were discharged without passing the screening) increased from 5.3% to 6.0%. DISCUSSION: To improve the quality of NHS, HSCs should be established nationwide and a second screening should be carried out more consistently before discharge in the case of a refer result in the initial screening.

Hearing Screening Alternative Using a Website-Based Application.

BACKGROUND AND OBJECTIVES: Indonesians encounter several barriers to regular functional hearing assessment. Hearing loss screening is only provided by tertiary-care hospitals that are not reachable by people in remote regions. This study aimed to develop a website-based hearing screening application that is accessible and inexpensive. Subjects and. METHODS: This comparative study was conducted between July and August 2022 in the Otolaryngology Clinic of Muhammadiyah Bandung Hospital with noise levels below 50 dB. The hearing screening was conducted using a website-based application (www.Screenout.id) and audiogram as the gold standard method. On ScreenOut, patients heard sounds with frequencies at 500, 1,000, 2,000, 4,000, and 8,000 Hz and sound intensity of each frequency at 35, 55, and 75 dB using earphones. RESULTS: A total of 133 participants were enrolled in our study. ScreenOut showed high sensitivity, specificity, accuracy, positive predictive value, and negative predictive value (90.9%, 98.9%, 93.6%, 99.4%, and 84.8%, respectively). Regarding hearing threshold, a very strong correlation was found between ScreenOut and audiogram, ranging between r=0.843 and r=0.899. Aside from that, there was no significant difference in hearing threshold values between ScreenOut and audiogram. CONCLUSIONS: Many advantages of the ScreenOut were found, including low-cost, accessibility, and easy-to-use interface, making it favorably used in low-middle-income countries such as Indonesia.

Surveillance for ototoxicity in platinum-based chemotherapy using mobile health audiometry with extended high frequencies.

OBJECTIVE: This study investigated mobile health enabled surveillance in ototoxicity. METHOD: This was a longitudinal study of 32 participants receiving chemotherapy. Baseline and exit audiograms that included conventional and extended high frequency audiometry were recorded within the patient's treatment venue using a validated mobile health audiometer. RESULTS: Average hearing thresholds at baseline were within the normal range (81.2 per cent left; 93.8 per cent right), reducing at exit testing (71.9 per cent left; 78.1 per cent right). Half of participants presented with a threshold shift according to ototoxicity monitoring criteria. The frequencies affected the most were between 4000 and 16 000 Hz, with left ears significantly more affected than right ears. Noise levels exceeded the maximum permissible ambient noise levels in up to 43.8 per cent of low frequencies (250-1000 Hz). CONCLUSION: Mobile health supported audiometry proved to be an efficacious tool for ototoxicity monitoring at the treatment venue. Changes in hearing ability over time could be tracked, improving surveillance in patients with full treatment schedules.

Hearing disorder following COVID-19 vaccination: A pharmacovigilance analysis using the Vaccine Adverse Event Reporting System.

WHAT IS KNOWN AND OBJECTIVE: Evidence on whether the coronavirus disease 2019 (COVID-19) vaccination could cause hearing-related adverse events is still conflicting. This study aims to access the association between COVID-19 vaccine and hearing disorder. METHODS: The Vaccine Adverse Event Reporting System (VAERS) was queried between January 2020 to November 2021. The disproportionality pattern for hearing impairment of COVID-19 vaccine was accessed by calculating the reporting odds ratio (ROR) and proportional reporting ratio (PRR). A further subgroup analysis based on the type of COVID-19 vaccine and the doses administered was performed. In addition, the disproportionalities for hearing dysfunction between COVID-19 and influenza vaccines were compared. RESULTS AND DISCUSSION: A total of 14,956 reports of hearing-related adverse events were identified with COVID-19 vaccination and 151 with influenza vaccine during the analytic period in VAERS. The incidence of hearing disorder following COVID-19 vaccination was 6.66 per 100,000. The results of disproportionality analysis revealed that the adverse events of hearing impairment, after administration of COVID-19 vaccine, was significantly highly reported (ROR 2.38, 95% confidence interval [CI] 2.20-2.56; PRR: 2.35, χ2 537.58), for both mRNA (ROR 2.37, 95% CI 2.20-2.55; PRR 2.34, χ2 529.75) and virus vector vaccines (ROR 2.50, 95% CI 2.28-2.73; PRR 2.56, χ2 418.57). While the disproportional level for hearing dysfunction was quite lower in influenza vaccine (ROR 0.36, 95% CI 0.30-0.42; PRR 0.36, χ2 172.24). WHAT IS NEW AND CONCLUSION: This study identified increased risk for hearing disorder following administration of both mRNA and virus vector COVID-19 vaccines compared to influenza vaccination in real-world settings.

Genetic research progress in branchio-oto syndrome/ branchio-oto-renal syndrome. / 鳃耳综合征/é³ƒè€³è‚¾ç»¼åˆå¾çš„é—ä¼ å­¦ç ”ç©¶è¿›å±•.

Branchio-oto syndrome (BOS)/branchio-oto-renal syndrome (BORS) is a kind of autosomal dominant heterogeneous disorder. These diseases are mainly characterized by hearing impairment and abnormal phenotype of ears, accompanied by renal malformation and branchial cleft anomalies including cyst or fistula, with an incidence of 1/40 000 in human population. Otic anormalies are one of the most obvious clinical manifestations of BOS/BORS, including deformities of external, middle, inner ears and hearing loss with conductive, sensorineural or mix, ranging from mild to profound loss. Temporal bone imaging could assist in the diagnosis of middle ear and inner ear malformations for clinicians. Multiple methods including direct sequencing combined with next generation sequencing (NGS), multiplex ligation-dependent probe amplification (MLPA), or array-based comparative genomic hybridization (aCGH) can effectively screen and identify pathogenic genes and/or variation types of BOS/BORS. About 40% of patients with BOS/BORS carry aberrations of EYA1 gene which is the most important cause of BOS/BORS. A total of 240 kinds of pathogenic variations of EYA1 have been reported in different populations so far, including frameshift, nonsense, missense, aberrant splicing, deletion and complex rearrangements. Human Endogenous Retroviral sequences (HERVs) may play an important role in mediating EYA1 chromosomal fragment deletion mutations caused by non-allelic homologous recombination. EYA1 encodes a phosphatase-transactivator cooperated with transcription factors of SIX1, participates in cranial sensory neurogenesis and development of branchial arch-derived organs, then regulates the morphological and functional differentiation of the outer ear, middle ear and inner ear toward normal tissues. In addition, pathogenic mutations of SIX1 and SIX5 genes can also cause BOS/BORS. Variations of these genes mentioned above may cause disease by destroying the bindings between SIX1-EYA1, SIX5-EYA1 or SIX1-DNA. However, the role of SIX5 gene in the pathogenesis of BORS needs further verification.

Is Auditory Steady-State Response Testing the Key for Diagnosing Non-Organic Hearing Disorders? Implications for Current Audiological Practice.

BACKGROUND AND OBJECTIVES: To describe all possible facets of non-organic hearing disorders (NOHD) and emphasize the superiority of auditory steady-state response (ASSR) over previously employed hearing assessment tools. SUBJECTS AND METHODS: A series of seven patients consisting of three males and four females with NOHD were assessed at Attikon University Hospital (age range: 17-59 years). Three patients had Munchausen syndrome, three intentionally feigned hearing loss, and one intentionally feigned normal hearing. The audiological evaluation consisted of tympanometry, pure-tone audiometry, and ASSR testing. RESULTS: The hearing of all patients was accurately determined using ASSR. The results were confirmed by auditory brainstem responses (ABR) and otoacoustic emissions. CONCLUSIONS: NOHD is a multi-faceted condition encompassing various etiologies. ASSR testing represents an objective and reliable method of hearing assessment, which can serve as a gold standard method for distinguishing NOHD from actual hearing loss. It can reliably indicate the hearing levels at the four main frequencies (500, 1,000, 2,000, and 4,000 Hz) by obtaining a valid estimated audiogram through statistical measures. Compared to ABR testing, ASSR thresholds are closer to the actual audiometric thresholds in the presence of hearing impairment and are superior when the corresponding pure-tone audiogram is widely ranging between the adjacent frequencies or when the obtained ABR curves are not easily distinguished. A non-confrontational approach should be adopted by ENT doctors towards cases of suspected NOHD as the use of ASSR could reliably assess hearing even when medical or medico-legal implications are involved.

Prevalence, Pattern, and Factors Associated With Self-reported Disability Among the Bhutanese Population: A Secondary Data Analysis of Population and Housing Census.

We analyzed the Population and Health Census of Bhutan (PHCB) 2017 to assess the prevalence and pattern of self-reported disability among people aged ≥15 years and the associated factors. The PHCB 2017 used the Washington Group Short Set on Functioning questionnaire to assess the disability ("lot of difficulty" or "cannot do at all") in seeing, hearing, mobility, cognition, self-care, and communication. Of the 536 443 persons included in the analysis, 384 101(71.6%) were aged <45 years, 283 453(52.8%) were men, and 206 103(38.4%) were from the rural area. The prevalence of any self-reported disability was 2.8%, among whom 34.2% reported multiple disabilities. The disability prevalence (any) was significantly higher among people aged ≥65 years, illiterate, economically inactive, permanent residents, residing in a rural area, and from central and eastern regions of the country compared with their respective counterparts. Further research on access to rehabilitation and linking with social protection schemes for the disabled is required in this country.

Genetic research progress in branchio-oto syndrome/ branchio-oto-renal syndrome / 中南大学学报(医学版)

Branchio-oto syndrome (BOS)/branchio-oto-renal syndrome (BORS) is a kind of autosomal dominant heterogeneous disorder. These diseases are mainly characterized by hearing impairment and abnormal phenotype of ears, accompanied by renal malformation and branchial cleft anomalies including cyst or fistula, with an incidence of 1/40 000 in human population. Otic anormalies are one of the most obvious clinical manifestations of BOS/BORS, including deformities of external, middle, inner ears and hearing loss with conductive, sensorineural or mix, ranging from mild to profound loss. Temporal bone imaging could assist in the diagnosis of middle ear and inner ear malformations for clinicians. Multiple methods including direct sequencing combined with next generation sequencing (NGS), multiplex ligation-dependent probe amplification (MLPA), or array-based comparative genomic hybridization (aCGH) can effectively screen and identify pathogenic genes and/or variation types of BOS/BORS. About 40% of patients with BOS/BORS carry aberrations of EYA1 gene which is the most important cause of BOS/BORS. A total of 240 kinds of pathogenic variations of EYA1 have been reported in different populations so far, including frameshift, nonsense, missense, aberrant splicing, deletion and complex rearrangements. Human Endogenous Retroviral sequences (HERVs) may play an important role in mediating EYA1 chromosomal fragment deletion mutations caused by non-allelic homologous recombination. EYA1 encodes a phosphatase-transactivator cooperated with transcription factors of SIX1, participates in cranial sensory neurogenesis and development of branchial arch-derived organs, then regulates the morphological and functional differentiation of the outer ear, middle ear and inner ear toward normal tissues. In addition, pathogenic mutations of SIX1 and SIX5 genes can also cause BOS/BORS. Variations of these genes mentioned above may cause disease by destroying the bindings between SIX1-EYA1, SIX5-EYA1 or SIX1-DNA. However, the role of SIX5 gene in the pathogenesis of BORS needs further verification.

Serotonin Transporter Defect Disturbs Structure and Function of the Auditory Cortex in Mice.

Serotonin transporter (SERT) modulates the level of 5-HT and significantly affects the activity of serotonergic neurons in the central nervous system. The manipulation of SERT has lasting neurobiological and behavioral consequences, including developmental dysfunction, depression, and anxiety. Auditory disorders have been widely reported as the adverse events of these mental diseases. It is unclear how SERT impacts neuronal connections/interactions and what mechanism(s) may elicit the disruption of normal neural network functions in auditory cortex. In the present study, we report on the neuronal morphology and function of auditory cortex in SERT knockout (KO) mice. We show that the dendritic length of the fourth layer (L-IV) pyramidal neurons and the second-to-third layer (L-II/III) interneurons were reduced in the auditory cortex of the SERT KO mice. The number and density of dendritic spines of these neurons were significantly less than those of wild-type neurons. Also, the frequency-tonotopic organization of primary auditory cortex was disrupted in SERT KO mice. The auditory neurons of SERT KO mice exhibited border frequency tuning with high-intensity thresholds. These findings indicate that SERT plays a key role in development and functional maintenance of auditory cortical neurons. Auditory function should be examined when SERT is selected as a target in the treatment for psychiatric disorders.

Amoxicillin and Risk of Hearing Loss: Analysis of Reported Cases Submitted to the WHO Global Database.

BACKGROUND: Penicillins have never been associated with any form of hearing disorders. The Eritrean Pharmacovigilance Centre recently received two cases of hearing disorder (including one case of deafness) following the intake of amoxicillin capsules. This study was conducted to assess the causal link between amoxicillin and deafness. METHODS: Data mining was carried out in the WHO global database of individual case safety reports, VigiBase, and the Austin Bradford-Hill criteria were used to assess causality. RESULTS: A total of 94 cases of 'hearing disorders' related to amoxicillin were retrieved from VigiBase. Of these, 49 (52.1%) were reported as "deafness," and amoxicillin was marked as the sole suspected drug in 18 and the only drug administered in 13 cases. In the rest of the deafness cases (n=31), amoxicillin was concurrently administered with other drugs and reported as co-suspected. The median time to onset was three days and the outcome was marked as recovered (14), recovering (2), not recovered (16) and unknown (17). In five cases, deafness resolved following the withdrawal of amoxicillin. CONCLUSION: There appears a causal association between amoxicillin and hearing loss that requires further substantiation with better epidemiologic studies.

Hearing-impaired population performance and the effect of hearing interventions on Montreal Cognitive Assessment (MoCA): Systematic review and meta-analysis.

BACKGROUND: Older adults are at high risk of developing age-related hearing loss (HL) and/or cognitive impairment. However, cognitive screening tools rely on oral administration of instructions and stimuli that may be impacted by HL. This systematic review aims to investigate (a) whether people with HL perform worse than those without HL on the Montreal Cognitive Assessment (MoCA), a widely used screening tool for cognitive impairment, and what the effect size of that difference is (b) whether HL treatment mitigates the impact of HL. METHOD: We conducted a systematic review and meta-analysis including studies that reported mean MoCA scores and SDs for individuals with HL. RESULTS: People with HL performed significantly worse on the MoCA (4 studies, N = 533) with a pooled mean difference of -1.66 points (95% confidence interval CI -2.74 to -0.58). There was no significant difference in MoCA score between the pre- vs post-hearing intervention (3 studies, N = 75). However, sensitivity analysis in the cochlear implant studies (2 studies, N = 33) showed improvement of the MoCA score by 1.73 (95% CI 0.18 to 3.28). CONCLUSION: People with HL score significantly lower than individuals with normal hearing on the standard orally administered MoCA. Clinicians should consider listening conditions when administering the MoCA and report the hearing status of the tested individuals, if known, taking this into account in interpretation or make note of any hearing difficulty during consultations which may warrant onward referral. Cochlear implants may improve the MoCA score of individuals with HL, and more evidence is required on other treatments. J Am Geriatr Soc 68:-, 2020.

4q27 deletion and 7q36.1 microduplication in a patient with multiple malformations and hearing loss: a case report.

BACKGROUND: Chromosome deletions of the long arm of chromosome 4 in 4q syndrome are characterized by mild facial and digital dysmorphism, developmental delay, growth retardation, and skeletal and cardiac anomalies, which is regarded as an autism spectrum disorder. Moreover, some scarce reports indicate that patients with 4q interstitial deletion and 7p duplication may present symptoms associated with hearing loss. CASE PRESENTATION: A boy with a severe developmental delay not only post-natal but also intrauterine and several dysmorphic features including microcephaly, ocular hypertelorism, exophthalmos, low-set ears, single palmar flexion crease, and overlapping toes presented discontinued cyanosis and recurrent respiratory infections. MRI, BAEP, echocardiogram and bronchoscopy revealed that he had persistent falcine sinus with a thin corpus callosum, left auditory pathway disorder, patent foramen ovale (2 mm), and tracheobronchomalacia with the right superior bronchus arising from the lateral posterior wall of the right main bronchus. Finally, the patient died with severe pneumonia at 10 months. Array CGH revealed a 23.62 Mb deletion at chromosome 4q27, arr [hg19] 4q27-q31.21 (121, 148, 089-144, 769, 263) × 1, and a 0.85 Mb duplication at chromosome 7q36.1, arr [hg19] 7q36.1-q36.2 (152, 510, 685-153, 363,5 98) × 3. It is rare for 4q syndrome cases or 7q duplications previously reported to have a hearing disorder, pulmonary dysplasia, and pulmonary arterial hypertension. CONCLUSIONS: The phenotype of our patient mainly reflects the effects of haploinsufficiency of FGF2, SPATA5, NAA15, SMAD1, HHIP genes combined with a microduplication of 7q36.1.

Time-compressed speech test in adults with and without central auditory processing disorders / Teste de Fala Comprimida em adultos com e sem transtorno do processamento auditivo central

ABSTRACT Purpose: to analyze and compare the performance in the time-compressed speech test and the auditory behavior of adults with and without central auditory processing disorders. Methods: an observational, analytical, cross-sectional study with a total of 40 people of both genders aged 18 to 35 years participating in the study. They were submitted to anamnesis, basic audiological assessment, and a core battery of tests for central auditory processing - including the dichotic digits test (binaural integration), frequency pattern test, and time-compressed speech test (TCST). Based on the results of the dichotic digits and frequency pattern tests, the subjects were divided into two groups, with and without central auditory processing disorders. The auditory behavior was assessed with the Scale of Auditory Behavior (SAB) questionnaire. The Mann-Whitney and Fisher's exact tests were used for the statistical analysis, setting the significance level at p < 0.05. Results: no difference in performance was found between the groups regarding the ears. There was a difference between the groups only in the time-compressed speech test with monosyllable stimuli in the left ear (p = 0.026). Monosyllables were the words that resulted in most errors. Conclusion: it was verified that only the list of stimuli influenced the performance, differing the individuals with and without central auditory processing disorders. There was an association of auditory behavior, analyzed with the SAB questionnaire, with the performance in the TCST with the list of monosyllables. It is suggested that this list be used when assessing adults by the time-compressed speech test.

RESUMO Objetivo: analisar e comparar o desempenho no Teste de Fala Comprimida e o comportamento auditivo de adultos com e sem alteração do Processamento Auditivo Central. Métodos: estudo observacional, transversal e analítico. Participaram do estudo 40 indivíduos, com idade de 18 a 35 anos, de ambos os sexos. Os participantes foram submetidos à anamnese, avaliação audiológica básica, e bateria mínima do Processamento Auditivo Central, incluindo o Teste Dicótico de Dígitos (integração binaural), Teste Padrão de Frequência e o Teste de Fala Comprimida. A partir do resultado dos Testes Dicótico de Dígitos e Padrão de Frequência foram distribuídos em grupos de indivíduos sem e com alteração de Processamento Auditivo Central. O comportamento auditivo foi avaliado por meio do questionário Scale of Auditory Behaviors(SAB). Para análise estatística foram utilizados os testes Mann-Whitney e Fisher's Exact test, considerando o nível de significância p<0,05. Resultados: em relação à variável orelha não foi encontrada diferença de desempenho entre os grupos. Houve diferença entre os grupos apenas no Teste Fala Comprimida monossílabos da orelha esquerda (p=0,026). As palavras com maior ocorrência de erros foram as monossílabas. Conclusão: verificou-se que apenas a lista de estímulos influenciou no desempenho entre os indivíduos com e sem alteração de Processamento Auditivo Central. Houve associação do comportamento auditivo analisado pelo questionário SAB com o desempenho do TFC na lista de monossílabos. Sugere-se que esta lista seja utilizada na avaliação do teste fala comprimida em adultos.

Hydrochlorothiazide and risk of hearing disorder: a case series.

BACKGROUND: Hydrochlorothiazide is not known to cause hearing disorder. The Eritrean Pharmacovigilance Centre, however, has received cases of hearing disorder, including irreversible deafness, associated with hydrochlorothiazide. The aim of this study is, therefore, to assess the causal relationship between hydrochlorothiazide and hearing disorder. METHODS: Data was retrieved from the WHO global adverse drug reaction database, VigiBase™. A search on VigiBase™ was made on August 6, 2017 using "hydrochlorothiazide" as drug substance, and "ototoxicity", "decreased hearing", and "vestibular disorder" as reaction terms. Cases were retrieved using VigiLyze™ and exported to an Excel spreadsheet for descriptive analysis. Causality was assessed using Austin Bradford-Hill criteria and labeledness of the reaction was evaluated through a thorough literature review including the summary of product characteristics. RESULTS: From 1972 to August 2017, 94 cases of hearing disorder associated with hydrochlorothiazide were submitted from 18 countries to VigiBase™. The median age was 64 years with almost equal male to female ratio. In 53.2% of the cases, hydrochlorothiazide was reported as the only suspected drug. Of these, 26 cases encountered hearing disorder following the sole intake of hydrochlorothiazide. Reaction was marked as "serious" in 36% of the cases and median time to reaction onset was 3 days. Outcome was reported as reversible in 66.7% of the cases. Reaction resolved in 17 cases following withdrawal of hydrochlorothiazide and recurred in one case after subsequent rechallenge with the product. Consistency of cases and a dose-response relationship was also observed in this case series. CONCLUSIONS: This case series assessment found a suggestive causal relationship between hydrochlorothiazide and hearing disorder. Taking the inherent limitations of this study into account, results should be interpreted with caution and further studies are required to validate the safety signal.

Hearing disorder from music; a neglected dysfunction.

CONCLUSION: Music-induced acute acoustic trauma is not inevitably linked to hearing dysfunction as validated by conventional pure tone audiometry. Tinnitus is often in combination with hyperacusis. Our results point at 'silent hearing loss' as the underlying pathology, having afferent nerve terminal damage rather than hair cell loss as the structural correlate. OBJECTIVES: Exposure to loud music is one of the most common causes of acute acoustic trauma, which adolescents and teenagers experience by voluntary exposure to loud music of sound levels up to 110 dB(A). METHODS: The clinical and psychophysical data of 104 consecutive patients with music-induced hearing disorder (MIHD) were analyzed to construct individual hearing and tinnitus profiles. In all cases, tinnitus was the presenting symptom. RESULTS: Hearing abilities were normal in about two-thirds of the tinnitus patients. Tinnitus was experienced most often as a high-frequency tone (83%). The Tinnitus Handicap Inventory (THI) scores ranged from 0 to 94 with an average score of 43.1. Visual analog scales (VAS) were used to assess tinnitus loudness (average 42.4) and annoyance (average 54.2), and tinnitus awareness was estimated (average 60.3). All VAS values correlated strongly with the THI. Hyperacusis was present in 65% and 71% of the patients reported sleeping disorders.

Newborn Hearing Screening in Bavaria-Is It Possible to Reach the Quality Parameters?

Since the 1 January, 2009, newborn hearing screening (NHS) has been obligatory for every child in Germany. NHS is part of the Pediatrics Directive of the Federal Joint Committee. In this directive, details of the procedures and screening quality to be achieved are given. We evaluate if these quality criteria were met in Bavaria in 2016. The NHS data of children born in 2016 in Bavaria were evaluated for quality criteria, such as screening coverage in screening facilities, screening methods, referral rate (rate of failed tests at discharge) and a child's age at the diagnosis of a hearing disorder. NHS was documented for 116,776 children born in Bavaria in 2016. In the first step, 78,904 newborns were screened with transient evoked otoacoustic emissions and 37,865 with automated auditory brainstem response. Of these, 9182 (7.8%) failed the first test in one or both ears. A second screening before discharge was performed on 53.3% of the newborns with a refer result in the first test, out of which 58.7% received a pass result. After the screening process, 4.6% of the newborns were discharged with a refer result. Only 18% of the first controls after discharge were performed by a pediatric audiologist. In 37.9% of the newborns, the screening center intervened to assure the control of any failed screening test. The median age of diagnosis for bilateral hearing loss was 5.3 months. In Bavaria, NHS was implemented successfully. A tracking system for all children who failed the hearing screening test is pivotal for early diagnosis and therapy of children with hearing deficiency.

Screening for hearing loss and deafness-related genes in newborns / 中华全科医师杂志

To screen the hearing loss and deafness-related genes in newborns,the screenings for hearing loss and the mutations of common deafness-related genes were performed among 8 187 infants born in Shaoxing Maternal and Child Health Care Hospital from August 2013 to November 2014.Twenty mutation spots in deafness-related genes GJB2,GJB3,12SrRNA and SLC26A4 were detected with matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS).Of 8 187 newborns,hearing loss was confirmed in 10 cases,and mutations in deafness-related genes were detected in 441 cases with a detection rate of 5.39％.Among 441 cases with positive gene mutations,there were 243 cases with GJB2 mutations (2.97％).147 cases with SCL26A4 mutations (1.80％),43 cases with GJB3 mutations (0.53％) and 15 cases with mutations of mitochondrial gene 12SrRNA (0.18％).The spot of highest detection frequency was GJB2 235delC (2.31％),followed by SCL26A4 IVS72A ＞ G (1.31％).The deafness gene detection rate for newborns who did not pass the hearing tests (8.16％,79/968) was higher than these who passed (5.01％,362/7 219;x2 =10.978,P ＜0.05).Five of 10 newborns with hearing loss were detected carrying deafness genes.The detection rate of the common deafness genes among the newborns is relatively high in this region.Screening for hearing loss and deafness gene may contribute to early diagnosis and intervention,and also to long-term precaution for those carrying heterozygosity deafness genes.

Implantable hearing devices.

Combined hearing loss is an essential indication for implantable hearing systems. Depending on the bone conduction threshold, various options are available. Patients with mild sensorineural deafness usually benefit from transcutaneous bone conduction implants (BCI), while percutaneous BCI systems are recommended also for moderate hearing loss. For combined hearing losses with moderate and high-grade cochlear hearing loss, active middle ear implants are recommended. For patients with incompatibilities or middle ear surgery, implants are a valuable and proven addition to the therapeutic options.