Combined Electric acoustic stimulation in a patient with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis.

There are few reports of the treatment for severe hearing loss due to otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) achieved by cochlear implantation (CI). Here, we have reported the case of a patient with severe bilateral sensorineural hearing loss with low-frequency residual hearing by OMAAV. CI was performed in her right ear based on the results of contrast-enhanced magnetic resonance imaging (CE-MRI) and promontory stimulation test (PST). The residual hearing in her right ear was preserved after CI and utilized for combined electric acoustic stimulation (EAS). The combined EAS was used for 3 years until the residual hearing became stabilized. However, the usable hearing in low frequency worsened gradually, and the fitting strategy of cochlear implant was changed from combined EAS to CI alone 4 years after CI. Even when the speech discrimination score with CI no longer exceeds 50 %, the patient continued using CI because of its advantages in maintaining the quality of life of the patient. The combined EAS was found to be a feasible option even in an OMAAV patient with residual hearing. CE-MRI and PST may thus be helpful in deciding the side of CI surgery in a patient with OMAAV.

Effective sound therapy using a hearing aid and educational counseling in patients with chronic tinnitus.

OBJECTIVE: This study aimed to assess the effectiveness of our sound therapy with appropriate hearing aid fitting and periodic hearing aid adjustment in patients with chronic tinnitus. METHODS: We conducted a retrospective study. The study included 490 individuals who received treatment with hearing aids for chronic tinnitus at least for 3 months. To determine the effects of tinnitus on patients' quality of life, the participants completed a series of questionnaires, including the Tinnitus Handicap Inventory (THI), Visual Analogue Scale (VAS) for loudness and annoyance, and questionnaires of subjective symptom improvement. Data were collected at entry and 3 months and 1 year after treatment initiation. RESULTS: All 490 participants completed the questionnaires at 3 months; however, only 312 completed them at 1 year. The mean ± standard deviation THI score before treatment decreased significantly at 3 months (490 participants: 53 ± 25 to 11 ± 16 and 312 participants: 55 ± 24 to 12 ± 16) and 1 year (55 ± 24 to 9 ± 14) (P < 0.01). The mean VAS score for tinnitus loudness before treatment decreased significantly at 3 months (490 participants: 70 ± 22 to 25 ± 27 and 312 participants: 71 ± 22 to 27 ± 26) and 1 year (71 ± 22 to 21 ± 28) (P < 0.01). In addition, the mean VAS score for tinnitus annoyance before treatment decreased significantly at 3 months (490 participants: 75 ± 26 to 20 ± 26 and 312 participants: 75 ± 25 to 23 ± 27) and 1 year (75 ± 25 to 17 ± 26) (P < 0.01). Approximately 80% of patients noticed improvements in their tinnitus annoyance and loudness, as determined by their responses to the questionnaires of subjective symptom improvement. CONCLUSION: The results of this study suggest that treatment with sound therapy may ameliorate the symptoms of chronic tinnitus associated with hearing loss.

Efficacy of Intratympanic Steroid on Idiopathic Sudden Sensorineural Hearing Loss: An Analysis of Cases With Negative Prognostic Factors.

Purpose We retrospectively studied the efficacy of intratympanic steroid administration in comparison with hyperbaric oxygen (HBO) therapy for idiopathic sudden sensorineural hearing loss (ISSNHL) with negative prognostic factors. Method We enrolled 301 patients (302 ears) with ISSNHL (average hearing level at 250-4000 Hz ≥ 40 dB; time from onset to treatment ≤ 30 days). From August 2002 to March 2009, 174 patients (174 ears) received systemic steroid plus HBO therapy (HBO group), and from June 2015 to January 2018, 127 patients (128 ears) received systemic plus intratympanic steroid (IT group). Hearing outcomes were evaluated by 6 indices: cure rate, marked-recovery rate (percent of patients with hearing gain ≥ 30 dB), recovery rate (percent of patients with hearing gain ≥ 10 dB), hearing gain, hearing level after treatment, and percent hearing improvement compared to the unaffected contralateral ear. Results The recovery rate was significantly higher in the IT group than in the HBO group (80.5% vs. 68.4%, p = .019). The IT group showed a higher recovery rate than the HBO group in patients aged ≥ 60 years ( p = .010), patients with early (≤ 7 days from onset) treatment ( p = .005), patients with initial hearing levels ≥ 90 dB ( p = .037), and patients with vertigo/dizziness ( p = .040). The IT group also showed higher hearing gain and percent hearing improvement than the HBO group in patients with vertigo/dizziness ( p = .046 and p = .026, respectively). Conclusions Systemic plus intratympanic steroid is more effective for ISSNHL than systemic steroid plus HBO, particularly in patients with negative prognostic factors, such as old age, profound hearing loss, and/or presence of vertigo/dizziness.

Sensorineural hearing loss and cognitive impairments: Contributions of thalamus using multiparametric MRI.

BACKGROUND: The thalamus is an integrative hub conveying sensory information between cortical areas and related to cognition. However, alterations of the thalamus following partial hearing deprivation remains unknown. PURPOSE: To investigate the modifications of the thalamus and its seven subdivisions in terms of structure, function, and perfusion in subjects with sensorineural hearing loss (SNHL), as well as their associations with SNHL-induced cognitive impairments. STUDY TYPE: Cross-sectional study. SUBJECTS: Thirty-seven bilateral long-term SNHL patients and 38 well-matched controls. FIELD STRENGTH: 3 T/BOLD, T1 -weighted imaging, arterial spin labeling (ASL). ASSESSMENT: Quantitative measurements in the thalamus and subdivisions were obtained, including the relative volume, fractional amplitude of low-frequency fluctuation (fALFF) within slow 5 (0.01-0.027 Hz), slow 4 (0.027-0.073 Hz), and combined frequency (0.01-0.073 Hz), as well as the whole-brain functional connectivity. Twenty-five SNHL patients and 20 controls underwent ASL scanning. Then correlation analysis was computed between all significant changes and cognition tests. STATISTICAL TESTS: Continuous and categorical variables were compared by independent-sample t-test and chi-square test, respectively. Quantitative MRI measurement comparisons were corrected for multiple comparison, and functional connectivity (FC) analysis used two-sample t-test with false-discovery rate correction. Area under the curve (AUC) in receiver operating characteristic curve analysis was applied to evaluate the power of alterations in differentiating SNHL and controls. RESULTS: No significant difference in the relative volume and perfusion of seven thalamus subdivisions were observed, but a decrease in fALFF in SNHL. SNHL showed reduced thalamic connectivity with the cerebellum lobule VIII, ventral anterior cingulate cortex, insula, superior temporal gyrus, media temporal gyrus, medial frontal gyrus, Heschl's gyrus, and temporal pole. And some FC abnormalities exhibited positive correlations with cognitive tests and high discriminative power (0.8 < AUC < 1) in two groups. DATA CONCLUSION: SNHL led to decreased thalamic activity and widespread weakened connectivity with other brain areas. LEVEL OF EVIDENCE: 2 Technical Efficacy Stage: 3 J. Magn. Reson. Imaging 2019;50:787-797.

Hyperbaric Oxygen Therapy as Concurrent Treatment with Systemic Steroids for Idiopathic Sudden Sensorineural Hearing Loss: A Comparison of Three Different Steroid Treatments.

We analyzed 356 patients with idiopathic sudden sensorineural hearing loss treated with hyperbaric oxygen therapy and systemic steroids (n = 161), systemic steroids alone (n = 160), or intratympanic and systemic steroids (n = 35). The main outcome measure was the hearing recovery rate. The effect of other variables, including the initial averaged 5-frequency hearing level, patient age, interval between the onset of symptoms and treatment, presence of vertigo as a complication, presence of diabetes mellitus, smoking history, and presence of hypertension, on the hearing recovery rate was also evaluated. The overall hearing recovery rate was significantly higher for the patients treated with hyperbaric oxygen therapy and systemic steroids than for those treated with systemic steroids alone (p < 0.001) or systemic and intratympanic steroids (p < 0.001). The presence of vertigo negatively affected hearing recovery. Our findings suggest that hyperbaric oxygen therapy confers a significant additional therapeutic benefit when used in combination with steroid therapy for idiopathic sudden sensorineural hearing loss.

Pancytopenia in an adult patient with thiamine-responsive megaloblastic anaemia.

Thiamine-responsive megaloblastic anaemia (TRMA) is a syndrome associated with megaloblastic anaemia, diabetes mellitus and sensorineural deafness, due to mutations in the SLC19A2 gene, which codes for a thiamine carrier protein. Oral thiamine supplementation is the main treatment. We report the case of a 25-year-old woman known for TRMA, who presented with pancytopenia (haemoglobin 7.6 g/dL, leucocytes 2.9×109/L, thrombocytes 6×109/L) revealed by dyspnoea. Investigations excluded coagulopathy, a recent viral infection, vitamin and iron deficiencies, and a malignant process. We later found out that thiamine treatment had been discontinued 5 weeks before, due to prescription error. Parenteral thiamine administration resulted in the recovery of haematopoiesis within 3 weeks. Pancytopenia is uncommon in patients with TRMA. Pre-existing medullary impairment caused by the patient's daily antipsychotic medications or the natural course of the syndrome may explain the severity of the laboratory findings in our patient.

The effect of tinnitus specific intracochlear stimulation on speech perception in patients with unilateral or asymmetric hearing loss accompanied with tinnitus and the effect of formal auditory training.

OBJECTIVES: Previous studies show that intracochlear electrical stimulation independent of environmental sounds appears to suppress tinnitus, even long-term. In order to assess the viability of this potential treatment option it is essential to study the effects of this tinnitus specific electrical stimulation on speech perception. DESIGN: A randomised, prospective crossover design. STUDY SAMPLE: Ten patients with unilateral or asymmetric hearing loss and severe tinnitus complaints. RESULTS: The audiological effects of standard clinical CI, formal auditory training and tinnitus specific electrical stimulation were investigated. Results show that standard clinical CI in unilateral or asymmetric hearing loss is shown to be beneficial for speech perception in quiet, speech perception in noise and subjective hearing ability. Formal auditory training does not appear to improve speech perception performance. However, CI-related discomfort reduces significantly more rapidly during CI rehabilitation in subjects receiving formal auditory training. Furthermore, tinnitus specific electrical stimulation has neither positive nor negative effects on speech perception. CONCLUSIONS: In combination with the findings from previous studies on tinnitus suppression using intracochlear electrical stimulation independent of environmental sounds, the results of this study contribute to the viability of cochlear implantation based on tinnitus complaints.

Association of Sudden Sensorineural Hearing Loss With Risk of Cardiocerebrovascular Disease: A Study Using Data From the Korea National Health Insurance Service.

Importance: The interruption of vascular supply to the cochlea has been proposed as a major etiological factor for sudden sensorineural hearing loss (SSNHL), and several risk factors for cardiocerebrovascular disease (CCVD) are associated with SSNHL, including heavy smoking, alcohol consumption, and thromboembolic events. However, the link between SSNHL and CCVD has not been fully evaluated. Objective: To investigate the association between SSNHL and CCVD. Design, Setting, and Participants: A retrospective propensity score-matched cohort study was conducted using a nationwide representative sample from the National Sample Cohort 2002 through 2013 data from the Korea National Health Insurance Service. The SSNHL group (n = 154) included certain patients who were diagnosed with SSNHL between January 2003 and December 2005. The comparison group was selected (4 patients for every 1 patient with SSNHL; n = 616) using propensity score matching, according to sociodemographic factors and the year of enrollment. Each patient was monitored until 2013. Main Outcomes and Measures: Survival analysis, the log-rank test, and Cox proportional hazards regression models were used to calculate the incidence, survival rate, and hazard ratio of CCVD for each group. Results: Among the 770 patients, 385 (50.0%) were female and 370 (48.1%) were aged between 45 and 64 years. Of the total study population, 66 patients developed CCVD, such as stroke and acute myocardial infarction, during the 11-year follow-up period: 18 patients in the SSNHL group (incidence, 13.5 cases per 1000 person-years) and 48 from the comparison group (incidence, 7.5 cases per 1000 person-years). After adjustment for other factors, the hazard ratio of CCVD during the 11-year follow-up period was 2.18 times (95% CI, 1.20-3.96) greater for patients with SSNHL. An increased risk of stroke was associated with SSNHL (HR, 2.02; 95% CI, 1.16-3.51); however, there was no relation between SSNHL and risk of myocardial infarction (HR, 1.18; 95% CI, 0.25-5.50). Conclusions and Relevance: This observational study using nationwide data suggests that SSNHL is associated with an increased incidence of CCVD, specifically stroke. Therefore, patient surveillance for signs of CCVD should be considered for patients who receive a diagnosis of SSNHL.

Brain activity in patients with unilateral sensorineural hearing loss during auditory perception in noisy environments.

Patients with unilateral sensorineural hearing loss (UHL) often complain of hearing difficulties in noisy environments. To clarify this, we compared brain activation in patients with UHL with that of healthy participants during speech perception in a noisy environment, using functional magnetic resonance imaging (fMRI). A pure tone of 1 kHz, or 14 monosyllabic speech sounds at 65‒70 dB accompanied by MRI scan noise at 75 dB, were presented to both ears for 1 second each and participants were instructed to press a button when they could hear the pure tone or speech sound. Based on the activation areas of healthy participants, the primary auditory cortex, the anterior auditory association areas, and the posterior auditory association areas were set as regions of interest (ROI). In each of these regions, we compared brain activity between healthy participants and patients with UHL. The results revealed that patients with right-side UHL showed different brain activity in the right posterior auditory area during perception of pure tones versus monosyllables. Clinically, left-side and right-side UHL are not presently differentiated and are similarly diagnosed and treated; however, the results of this study suggest that a lateralityspecific treatment should be chosen.

Hyperbaric Oxygen Therapy as Adjuvant Treatment for Idiopathic Sudden Sensorineural Hearing Loss after Failure of Systemic Steroids.

We evaluated the outcomes of and prognostic factors for idiopathic sudden sensorineural hearing loss (ISSNHL) treated with adjuvant hyperbaric oxygen therapy (HBOT). A retrospective review of clinical data was performed for 167 patients with ISSNHL who failed to respond to systemic steroids and were treated by adjuvant HBOT at Shizuoka Saiseikai General Hospital. We analysed the clinical outcomes, the averaged 5-frequency hearing level after systemic steroids, patient age, the interval between post-steroids and pre-HBOT, vertigo as a complication, the presence of diabetes mellitus, smoking history, and hypertension. Overall, after HBOT, complete recovery occurred in 16 (9.6%) of the patients, with definite improvement in 16 (9.6%) and slight improvement in 45 (26.9%). The overall rate of hearing improvement was higher in the study group (77/167 cases, 46.1%) than in the control group (52/160 cases, 32.5%; p = 0.021). If performed appropriately, HBOT should be able to improve hearing in many cases unresponsive to initial therapy.

COQ6 Mutations in Children With Steroid-Resistant Focal Segmental Glomerulosclerosis and Sensorineural Hearing Loss.

The phenotypic combination of steroid-resistant focal segmental glomerulosclerosis (SR-FSGS) and sensorineural hearing loss has been mainly reported in patients with mitochondrial cytopathies, including primary coenzyme Q10 (CoQ10) deficiency. In this report of 10 children with SR-FSGS and sensorineural hearing loss, we found 6 patients with biallelic COQ6 mutations. Median age at the onset of nephrotic syndrome was 29 (range, 15-47) months. All patients progressed to end-stage renal disease within a median of 13 (range, 1-27) months after the onset. Kidney biopsy revealed abnormal mitochondrial proliferation in podocytes in all 6 patients. None of the 5 patients who underwent kidney transplantation developed recurrence of FSGS. Primary CoQ10 deficiency due to COQ6 mutations should be considered in children presenting with both SR-FSGS and sensorineural hearing loss. An early diagnosis of COQ6 mutations is essential because the condition is treatable when CoQ10 supplementation is started at the early stage. We recommend early kidney biopsy because detection of abnormal mitochondrial proliferation in podocytes might provide an earlier diagnostic clue.

Psycho acoustical Measures in Individuals with Congenital Visual Impairment.

INTRODUCTION: In congenital visual impaired individuals one modality is impaired (visual modality) this impairment is compensated by other sensory modalities. There is evidence that visual impaired performed better in different auditory task like localization, auditory memory, verbal memory, auditory attention, and other behavioural tasks when compare to normal sighted individuals. OBJECTIVE: The current study was aimed to compare the temporal resolution, frequency resolution and speech perception in noise ability in individuals with congenital visual impaired and normal sighted. METHODS: Temporal resolution, frequency resolution, and speech perception in noise were measured using MDT, GDT, DDT, SRDT, and SNR50 respectively. Twelve congenital visual impaired participants with age range of 18 to 40 years were taken and equal in number with normal sighted participants. All the participants had normal hearing sensitivity with normal middle ear functioning. RESULTS: Individual with visual impairment showed superior threshold in MDT, SRDT and SNR50 as compared to normal sighted individuals. This may be due to complexity of the tasks; MDT, SRDT and SNR50 are complex tasks than GDT and DDT. CONCLUSION: Visual impairment showed superior performance in auditory processing and speech perception with complex auditory perceptual tasks.

Effects of Various Extents of High-Frequency Hearing Loss on Speech Recognition and Gap Detection at Low Frequencies in Patients with Sensorineural Hearing Loss.

This study explored whether the time-compressed speech perception varied with the degree of hearing loss in high-frequency sensorineural hearing loss (HF SNHL) individuals. 65 HF SNHL individuals with different cutoff frequencies were recruited and further divided into mildly, moderately, and/or severely affected subgroups in terms of the averaged thresholds of all frequencies exhibiting hearing loss. Time-compressed speech recognition scores under both quiet and noisy conditions and gap detection thresholds within low frequencies that had normal thresholds were obtained from all patients and compared with data from 11 age-matched individuals with normal hearing threshold at all frequencies. Correlations of the time-compressed speech recognition scores with the extents of HF SNHL and with the 1 kHz gap detection thresholds were studied across all participants. We found that the time-compressed speech recognition scores were significantly affected by and correlated with the extents of HF SNHL. The time-compressed speech recognition scores also correlated with the 1 kHz gap detection thresholds except when the compression ratio of speech was 0.8 under quiet condition. Above all, the extents of HF SNHL were significantly correlated with the 1 kHz gap thresholds.

Evaluación epidemiológica de la exposición a ruido en trabajadores de policía local y bomberos / Epidemiological evaluation of noise exposure in workers local police and firefighters

La discusión acerca de la exclusión de determinados puestos de trabajo del ámbito de la aplicación de la Ley 31/1995, finalizó con la Sentencia del Tribunal de Justicia de las Comunidades Europeas (12//01/2006). Objetivos: Conocer el riesgo de presentar una audiometría anormal relacionada con el trabajo, en aras de poder establecer relación entre el puesto y la exposición. Material y Métodos: Estudio de casos y controles realizado durante el periodo 2006-2010 entre agentes de Policía Local, Bomberos y personal de Administración. De una población accesible de 881 trabajadores se reclutaron a 631 que cumplían criterios de inclusión. De forma aleatoria y estratificada por sexo y edad, la muestra seleccionada fue de 389 (207 casos y 182 controles). Se realizó un análisis estadístico mediante contraste de medias independientes y porcentajes y como medida de asociación se calculó la Odds Ratio (OR) para los grupos de estudio y análisis multivariante de regresión logística así como curvas ROC. Resultados: En el análisis univariante, edad, puesto de trabajo (POL) y umbrales OSHA presentaron diferencias significativas, con una OR de 2,8 (p<0.001) en el grupo de Policía Local. En el multivariante, edad, umbrales OSHA y puesto de Policía Local (OR 2,8), siguen siéndolo. El área bajo la curva ROC fue para OSHA (0,815). Conclusiones: El puesto de policía local muestra mayor riesgo de lesión neurosensorial que el de administrativo; y la evaluación epidemiológica de la salud se muestra como herramienta eficaz para la evaluación de los riesgos (AU)

The discussion about the exclusion of certain jobs in the field of application of Law 31/1995, ended with the judgment of the Court of Justice of the European Communities (12 / 01/2006). Objective: to know the risk of abnormal audiometry work-related, in order to be able to establish relationship between the position and exposure. Material and Methods: a case-control study conducted during the period 2006-2010 among local police officers, firefighters and personal Administration. An accessible population of 881 workers were recruited 631 who met inclusion criteria. Random and stratified by sex and age, the selected sample was 389 (207 cases and 182 controls). Statistical analysis was performed using contrast independent means and percentages and as a measure of association calculated the odds ratio (OR) for groups of multivariate analysis and logistic regression analysis and ROC curves. Results: in the univariate analysis, age, job (POL) and OSHA thresholds differ significantly, with an odds ratio of 2.8 (p<0.001) in the group of local police. In multivariate, age, OSHA thresholds and local police station (OR 2.8), remain so. The area under the ROC curve was to OSHA (0.815) Conclusions: the local police station shows higher risk of sensoneural injury than administrative staff, and the epidemiological assessment of health is shown as an effective tool for risk assessment (AU)

Effects of sound amplification in self-perception of tinnitus and hearing loss in the elderly / Efeitos da amplificação sonora na autopercepção do zumbido e da perda auditiva em idosos

ABSTRACT OBJECTIVE: To determine the effect of the use of hearing aids in self-perception of tinnitus and hearing loss in the elderly. METHODS: A total of 24 elderly patients between 60 and 70 years of age with moderate-grade sensorineural hearing loss were evaluated and divided into two groups according to the presence or absence of tinnitus. All volunteers were fitted with binaural micro-channel hearing aids of the same brand and model and submitted to tests, scales, and questionnaires relevant to this topic. The evaluations were performed before and after one and three months of effective use of these hearing aids. RESULTS: Acoustic stimulation through the effective use of hearing aids caused a reduction in the perception of tinnitus sound intensity (especially in evaluations with the prosthesis on) and in nuisance associated with this symptom and with hearing loss. In addition, all participants were satisfied with the use of hearing aids. CONCLUSION: The continuous use of hearing aids is beneficial for the treatment of tinnitus and hearing loss, bringing satisfaction to users.

Resumo Objetivo: Verificar o efeito do uso de próteses auditivas na autopercepção do zumbido e da perda auditiva em idosos. Método: Foram avaliados 24 idosos com perda auditiva neurossensorial de grau moderado e idades entre 60 e 70 anos divididos em dois grupos conforme a presença ou não de zumbido. Todos os voluntários foram adaptados binauralmente com próteses auditivas micro canais de mesmo fabricante e modelo e submetidos a testes, escalas e questionários pertinentes ao tema. As avaliações ocorreram pré, e após um e três meses de uso efetivo das próteses auditivas. Resultados: A estimulação acústica por meio do uso efetivo de próteses auditivas propiciou redução na autopercepção da intensidade sonora do zumbido (principalmente nas avaliações com as próteses) e no desconforto com este sintoma e com a perda auditiva. Além disso, todos os participantes mostraram-se satisfeitos com o uso dos dispositivos de amplificação. Conclusão: O uso contínuo das próteses auditivas é benéfico ao tratamento do zumbido e da perda auditiva, além de implicar na satisfação dos usuários.

Antioxidant therapy in the elderly with tinnitus / Efeito da terapia com antioxidantes sobre o zumbido em idosos

ABSTRACT INTRODUCTION: Several approaches have been tried for the treatment of tinnitus, from cognitive-behavioral therapies and sound enrichment to medication. In this context, antioxidants, widely used in numerous areas of medicine, appear to represent a promising approach for the control of this symptom, which often is poorly controlled. OBJECTIVE: To evaluate the effects of antioxidant therapy for tinnitus in a group of elderly patients. METHODS: Prospective, randomized, double-blinded, placebo-controlled clinical trial. The sample consisted of 58 subjects aged 60 years or older, with a complaint of tinnitus associated with sensorineural hearing loss. These individuals completed the Tinnitus Handicap Inventory (THI) questionnaire before and after six months of therapy. The treatment regimens were: Ginkgo biloba dry extract (120 mg/day), a-lipoic acid (60 mg/day) + vitamin C (600 mg/day), papaverine hydrochloride (100 mg/day) + vitamin E (400 mg/day), and placebo. RESULTS: There was no statistically significant difference between THI by degree (p = 0.441) and by score (p = 0.848) before and after treatment. CONCLUSION: There was no benefit from the use of antioxidant agents for tinnitus in this sample.

Resumo Introdução: Uma série de abordagens terapêuticas tem sido empregada no tratamento do zumbido, desde terapias cognitivo-comportamentais e de enriquecimento sonoro até terapias medicamentosas. Nesse contexto, os agentes antioxidantes, amplamente utilizados em diversas áreas da medicina, parecem representar uma perspectiva promissora para o controle desse sintoma, que muitas vezes tem um controle clínico insatisfatório. Objetivo: Avaliar os efeitos da terapia com agentes antioxidantes sobre o zumbido em um grupo de pacientes idosos. Método: Ensaio clínico prospectivo, randomizado, duplo-cego e controlado por placebo. A amostra composta de 58 indivíduos com 60 anos ou mais, com queixa clínica de zumbido associado à perda auditiva, do tipo neurossensorial, em graus variados. Esses indivíduos foram submetidos ao questionário THI (Tinnitus Handicap Inventory) antes e após 6 meses de uso da medicação. Os esquemas terapêuticos foram os seguintes: extrato seco de Ginkgo biloba(120 mg/dia), ácido a-lipóico (60 mg/dia) + vitamina C (600 mg/dia), cloridrato de papaverina(100 mg/dia) + vitamina E (400 mg/dia) e placebo. Resultados: O THI após o tratamento foi estatisticamente igual ao THI antes do tratamento, tanto em graus (p = 0,441) quanto em escores (p = 0,848). Conclusão: Não se verificou benefício estatisticamente significativo com o uso de agentes antioxidantes para o zumbido dos indivíduos avaliados.

Auditory neuropathy in Brown-Vialetto-Van Laere syndrome due to riboflavin transporter RFVT2 deficiency.

AIM: Mutations in the genes encoding the riboflavin transporters RFVT2 and RFVT3 have been identified in Brown-Vialetto-Van Laere syndrome, a neurodegenerative disorder characterized by hearing loss and pontobulbar palsy. Treatment with riboflavin has been shown to benefit individuals with the phenotype of RFVT2 deficiency. Understanding the characteristics of hearing loss in riboflavin transporter deficiency would enable early diagnosis and therapy. METHOD: We performed hearing assessments in seven children (from four families) with RFVT2 deficiency and reviewed results from previous assessments. Assessments were repeated after 12 months and 24 months of riboflavin therapy and after cochlear implantation in one individual. RESULTS: Hearing loss in these individuals was due to auditory neuropathy spectrum disorder (ANSD). Hearing loss was identified between 3 years and 8 years of age and progressed rapidly. Hearing aids were not beneficial. Riboflavin therapy resulted in improvement of hearing thresholds during the first year of treatment in those with recent-onset hearing loss. Cochlear implantation resulted in a significant improvement in speech perception in one individual. INTERPRETATION: Riboflavin transporter deficiency should be considered in all children presenting with an auditory neuropathy. Speech perception in children with ANSD due to RFVT2 deficiency may be significantly improved by cochlear implantation.

Dental Treatment Considerations for Children with Complex Medical Histories: A Case of Townes-Brock Syndrome.

It is common for oral health and dental care to be considered a lesser priority for children with complex medical histories than other aspects of their health care. Often, these patients are at a high risk for caries and infection due to poor oral health practices at home, special or restricted diets, and no early establishment of a dental home for routine dental care. Unfortunately, many of these patients present to their first dental visits with caries and require aggressive treatment, such as extractions instead of pulp therapy, or crowns instead of fillings, due to their high caries risk and the difficulty in safely managing them medically during treatment. A unique example of this occurred at the Children's Hospital of Michigan, where a patient with Townes-Brock syndrome (TBS) presented to the dental clinic with advanced caries. TBS is a rare autosomal dominant disorder characterized by major findings such as anomalies of the external ear, imperforate anus, renal malformations, and malformations of the hand. Like many medically complex cases, dental anomalies are not a direct consequence of TBS; however, due to the necessity of high calorie and high sugar feeding supplementation, many of these patients are at high risk for advanced dental caries. Due to this high caries risk, a more aggressive treatment plan is necessary to minimize the risk of recurrent decay and infection. It is critical to stress that even if the disease, syndrome, etc., of a patient does not have inherent dental consequences, it is imperative for regular dental care to be part of the comprehensive treatment plan for these patients. This includes the establishment of a dental home at a young age and proper oral health education of the patient's caregivers and their physicians. In the case of the patient with TBS, recommendations for daily brushing, especially after high sugar feedings was stressed, as well as the reduction of any other sweets within the diet.

Effects of sound amplification in self-perception of tinnitus and hearing loss in the elderly.

OBJECTIVE: To determine the effect of the use of hearing aids in self-perception of tinnitus and hearing loss in the elderly. METHODS: A total of 24 elderly patients between 60 and 70 years of age with moderate-grade sensorineural hearing loss were evaluated and divided into two groups according to the presence or absence of tinnitus. All volunteers were fitted with binaural micro-channel hearing aids of the same brand and model and submitted to tests, scales, and questionnaires relevant to this topic. The evaluations were performed before and after one and three months of effective use of these hearing aids. RESULTS: Acoustic stimulation through the effective use of hearing aids caused a reduction in the perception of tinnitus sound intensity (especially in evaluations with the prosthesis on) and in nuisance associated with this symptom and with hearing loss. In addition, all participants were satisfied with the use of hearing aids. CONCLUSION: The continuous use of hearing aids is beneficial for the treatment of tinnitus and hearing loss, bringing satisfaction to users.

Antioxidant therapy in the elderly with tinnitus.

INTRODUCTION: Several approaches have been tried for the treatment of tinnitus, from cognitive-behavioral therapies and sound enrichment to medication. In this context, antioxidants, widely used in numerous areas of medicine, appear to represent a promising approach for the control of this symptom, which often is poorly controlled. OBJECTIVE: To evaluate the effects of antioxidant therapy for tinnitus in a group of elderly patients. METHODS: Prospective, randomized, double-blinded, placebo-controlled clinical trial. The sample consisted of 58 subjects aged 60 years or older, with a complaint of tinnitus associated with sensorineural hearing loss. These individuals completed the Tinnitus Handicap Inventory (THI) questionnaire before and after six months of therapy. The treatment regimens were: Ginkgo biloba dry extract (120mg/day), α-lipoic acid (60mg/day)+vitamin C (600mg/day), papaverine hydrochloride (100mg/day)+vitamin E (400mg/day), and placebo. RESULTS: There was no statistically significant difference between THI by degree (p=0.441) and by score (p=0.848) before and after treatment. CONCLUSION: There was no benefit from the use of antioxidant agents for tinnitus in this sample.