Auditory Dysfunction Among Individuals With Neurofibromatosis Type 1.

Importance: Neurofibromatosis type 1 (NF1) affects hearing through disruption of central auditory processing. The mechanisms, functional severity, and management implications are unclear. Objective: To investigate auditory neural dysfunction and its perceptual consequences in individuals with NF1. Design, Setting, and Participants: This case-control study included children and adults with NF1 and control participants matched on age, sex, and hearing level. Patients were recruited through specialist neurofibromatosis and neurogenetic outpatient clinics between April and September 2019. An evaluation of auditory neural activity, monaural/binaural processing, and functional hearing was conducted. Diffusion-weighted magnetic resonance imaging (MRI) data were collected from a subset of participants (10 children with NF1 and 10 matched control participants) and evaluated using a fixel-based analysis of apparent fiber density. Main Outcomes and Measures: Type and severity of auditory dysfunction evaluated via laboratory testing and questionnaire data. Results: A total of 44 participants (18 [41%] female individuals) with NF1 with a mean (SD) age of 16.9 (10.7) years and 44 control participants (18 [41%] female individuals) with a mean (SD) age of 17.2 (10.2) years were included in the study. Overall, 11 participants (25%) with NF1 presented with evidence of auditory neural dysfunction, including absent, delayed, or low amplitude electrophysiological responses from the auditory nerve and/or brainstem, compared with 1 participant (2%) in the control group (odds ratio [OR], 13.03; 95% CI, 1.59-106.95). Furthermore, 14 participants (32%) with NF1 showed clinically abnormal speech perception in background noise compared with 1 participant (2%) in the control group (OR, 20.07; 95% CI, 2.50-160.89). Analysis of diffusion-weighted MRI data of participants with NF1 showed significantly lower apparent fiber density within the ascending auditory brainstem pathways. The regions identified corresponded to the neural dysfunction measured using electrophysiological assessment. Conclusions and Relevance: The findings of this case-control study could represent new neurobiological and clinical features of NF1. Auditory dysfunction severe enough to impede developmental progress in children and restrict communication in older participants is a common neurobiological feature of the disorder.

Endoscopic Ossiculoplasty for the Management of Isolated Congenital Ossicular Chain Malformation: Surgical Results in 16 Ears.

OBJECTIVE: The aim of this study is to describe the clinical characteristics and intraoperative findings and further evaluate the efficacy of endoscopic ossiculoplasty for the management of isolated congenital ossicular chain malformation. METHODS: A retrospective study was performed on 16 ears (15 patients) with the isolated congenital ossicular chain malformation who underwent endoscopic ossiculoplasty in our department from May 2017 to January 2019. Endoscopic exploratory tympanotomy was conducted to check the ossicular chain; at the same time, endoscopic ossiculoplasty was performed depending on intraoperative findings. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps (ABGs) were measured before and after surgery, and the hearing outcome was assessed at 6 months postoperatively. RESULTS: The most common malformations of ossicular chain were the missing of the incus long process and stapes suprastructure. A serial assessment of the hearing status was conducted before and 6 months after surgery. It showed the mean postoperative pure-tone average (PTA) was significantly reduced, and the mean postoperative ABG was obviously closed, respectively (P < .001). The mean PTA gain was 36.3 ± 8.6 dB, and the ABG closure was 35.1 ± 8.3 dB; ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 14 cases (87.5%) and 5 cases (31.3%), respectively. No differences were observed in postoperative hearing outcome between type Ⅲ cases and type Ⅳ cases; however, cases with partial ossicular replacement prosthesis implantation showed a larger hearing gain (P = .049) and a higher proportion of postoperative ABG less than 10 dB (P = .021). No facial palsy and significant sensorineural hearing loss occurred; all patients completed the surgery without the need of canalplasty, and the chorda tympani nerve was preserved in all patients. CONCLUSIONS: This research showed endoscopic surgery was effective in the diagnosis and management of isolated congenital ossicular chain malformation; the endoscopic ossiculoplasty provides an alternative method to manage congenital ossicular chain malformation, with comfortable hearing outcome and the advantage of excellent vision and less invasion.

Impairment, disability, and handicap associated with hearing problems and voice disorders among Colombian teachers / Deficiencia, discapacidad y minusvalía asociadas a los problemas de voz y audición en profesores colombianos

Objectives: 1. define the occurrence of work-related hearing problems and voice disorders among teachers that have contacted the Colombian National Board of Disability Assessment (NBDA) for follow-up; 2. identify individual associated factors of hearing problems and voice disorders among teachers; 3. assess the limitations and restrictions due to hearing problems and voice disorders among these participants. Methods: Retrospective study. The National Database of the Colombian NBDA was reviewed. Information on distributions of occupation, individual characteristics, and diagnosis code (ICD-10) was analyzed. Results: Communication disorders among teachers that have contacted the Colom-bian NBDA for follow-up included voice disorders, with a prevalence of 51%, and hearing problems, with a prevalence of 7%. Female teachers who have contacted the Colombian NBDA for follow-up were 4 times more likely to be identified as having voice disorders compared with their male colleagues. Conclusions: While teachers that have contacted the Colombian NBDA for fol-low-up have a high occurrence of voice disorders, hearing problems are more likely to be stated as a debilitating condition. One possible explanation is that teachers who contacted the Colombian NBDA for follow-up continued working even when many voice symptoms were evident, while hearing problems would prevent a teacher from interacting with students, thereby affecting the teaching-learning process soon-er. Nevertheless, with both voice and hearing problems, work performance and social interaction is affected, and, therefore, quality of life is reduced.

Objetivos: 1. definir la ocurrencia de problemas de audición y de voz relacionados con el trabajo de docentes que contactaron la Junta Nacional de Evaluación de la Discapacidad de Colombia (NBDA) para su seguimiento; 2. identificar los factores individuales asociados a los problemas de audición y voz entre los profesores; 3. eva-luar las limitaciones y restricciones debidas a problemas de audición y voz asociados al trabajo entre los participantes.Métodos: estudio retrospectivo. Se revisó la Base de Datos Nacional de la NBDA colombiana. Se analizó información sobre distribuciones de ocupación, característi-cas individuales y código de diagnóstico (CIE-10).Resultados: Los trastornos de comunicación entre los docentes que han contacta-do a la NBDA colombiana para seguimiento incluyeron problemas de la voz, con una prevalencia del 51%, y problemas de audición, con una prevalencia del 7%. Las pro-fesoras que se han puesto en contacto con la NBDA colombiana para el seguimiento tenían 4 veces más probabilidades de ser identificadas con trastornos de la voz en comparación con sus colegas masculinos.Conclusiones: Si bien los maestros que contactaron a la NBDA colombiana para el seguimiento tienen una alta incidencia de trastornos de la voz, es más probable que los problemas de audición se consideren una condición debilitante. Una posible ex-plicación es que los maestros que se comunicaron con la NBDA colombiana para el seguimiento continuaron trabajando incluso cuando muchos síntomas vocales eran evidentes, mientras que los problemas de audición evitarían que un maestro inte-ractuara con los estudiantes, afectando así el proceso de enseñanza-aprendizaje. Sin embargo, tanto con los problemas de voz como de audición, el desempeño laboral y la interacción social se ven afectados y, por lo tanto, la calidad de vida se reduce.

Monitoramento eletrofisiológico do sistema auditivo central em crianças nascidas pequenas para a idade gestacional / Electrophysiological follow-up of the central auditory system in children born small for gestational age

RESUMO Objetivo Monitorar o sistema auditivo central de crianças nascidas pequenas para a idade gestacional, por meio da avaliação eletrofisiológica da audição, para verificar a ocorrência de eventuais disfunções neurais nesse sistema. Métodos Estudo longitudinal, cuja casuística foi composta por 23 crianças distribuídas em quatro grupos: 1) grupo de quatro crianças nascidas pequenas para a idade gestacional e a termo; 2) grupo de sete crianças nascidas pequenas para a idade gestacional e pré-termo; 3) grupo de quatro crianças nascidas com peso adequado para a idade gestacional e a termo; 4) grupo de oito crianças nascidas adequadas para a idade gestacional e pré-termo, cuja idade ao final da pesquisa foi de 3 anos (variação entre 34 e 39 meses). O critério de inclusão foi presença bilateral de emissões otoacústicas transientes. Todas as crianças foram submetidas ao potencial evocado auditivo de tronco encefálico ao nascimento, aos 6 meses e aos 3 anos de idade e à pesquisa do potencial evocado auditivo de longa latência aos 3 anos. Resultados crianças nascidas pequenas para a idade gestacional e a termo tiveram maior ocorrência de alterações, em relação aos demais grupos, com aumento da latência das ondas III e V e interpicos I-III e I-V. Todas apresentaram resultados normais no potencial evocado auditivo de longa latência. Conclusão Crianças nascidas pequenas para a idade gestacional e a termo apresentam disfunções na condução neural no tronco encefálico e devem ser consideradas de risco para alterações do desenvolvimento das habilidades auditivas necessárias para garantir qualidade de processamento da informação acústica.

ABSTRACT Purpose To follow up the central auditory system of children born small for gestational age, through electrophysiological evaluation of hearing, in order to verify the occurrence of possible neural dysfunctions in this system. Methods A longitudinal study was carried out with 23 children divided into four groups: Term-born group, subdivided into small for gestational age (four children) and four children born with appropriate weight for gestational age, whose age at the end of the research was three years old. Preterm group subdivided into small for gestational age (seven children), and appropriate for gestational age (eight children), whose corrected age, at the end of the research was three years old. All children were subjected to assessment of auditory brainstem auditory evoked potentials at birth, at six months and at three years of age, and Long-Latency Auditory Evoked Potential at three years. Results children born at term and small for gestational age had a higher occurrence of hearing alterations in relation to the other groups, with increased latency of waves III and V and interpeaks I-III and I-V. All children presented normal evaluation in the Long-Latency Auditory Evoked Potential. Conclusion Children born term and small for gestational age present dysfunctions in neural conduction in the brainstem and should be considered at risk for alterations in the development of the auditory skills that are necessary to guarantee quality of acoustic information processing.

Feasibility of early activation after cochlear implantation.

OBJECTIVES: The purpose of the study is to investigate feasibility of early activation after cochlear implantation by evaluating long-term impedance change and speech perception. DESIGN: Case-control study SETTING: Between July 2015 and December 2016, we prospectively enrolled 20 subjects for early activation (within 24 hours after cochlear implantation). On the other hand, from November 2013 to July 2015, 20 age- and sex-matched control subjects from the database of cochlear implantees treated with conventional activation schedule (4 weeks after surgery) were retrospectively enrolled. PARTICIPANT: Forty patients who underwent cochlear implantation surgeries. MAIN OUTCOME MEASURES: The series impedance and speech perception score of both groups were compared. RESULTS: No statistical difference in long-term follow-up between the two groups was found using GEEs and multivariate analysis. In the early activation group, impedance reached a steady level by the 2nd postoperative week, and the hearing perception ability significantly improved by the 4th postoperative week. CONCLUSION: This comparative study illustrated sequential impedance data during early activation (24 hours) and conventional activation (4 weeks) after CI surgery. There were no major complications in either group, and the safety of early activation with respect to impedance changes, postoperative residual hearing preservation and speech perception scores were non-inferior to that of the conventional group. Therefore, in this study, we established the feasibility of early activation 24 hours after cochlear implantation.

Estado actual de la atención sanitaria de personas con discapacidad auditiva y visual: una revisión breve / Health care of people with visual or hearing disabilities

Visual or hearing disabilities account for 20% of people reporting some sort of disability. We performed a literature review about the interaction that people with visual or hearing disabilities have with the health care system. We found that these people report a lower quality of life and have a higher frequency of physical and psychological ailments. They also have difficulties to obtain an adequate health care and report a paucity of trained professionals to take care of them.

The evaluation of the sense of hearing in patients with carotid artery stenosis within the extracranial segments.

Stenosis of arteries that supplies blood to the brain is one of the main causes of ischemic stroke which is the third most common cause of deaths in Europe. Atherosclerosis of carotid and vertebral arteries is responsible for 20% of the ischemic stroke cases. Stenosis may be either asymptomatic or manifested with typical neurological symptoms including motor and sensory disturbances as well as disturbances in vision and speech. However, discrete non-specific symptoms of ischemia, including headaches and vertigo, tinnitus and hearing loss, are also quite common. These symptoms may be indicative of a clinically significant stenosis of carotid and vertebral arteries, particularly within the internal carotid artery region, as well as of a risk of ischemic stroke. To date, research reports were unable to provide exact explanation of correlations between impaired hearing and the stenosis of carotid and vertebral arteries. Despite this, numerous articles list these symptoms as one of the first non-specific symptoms of this disorder. The ischemic mechanism within the inner ear region may lead to early symptoms of atherosclerosis of large vessels. However, no evidence of relationship and no explanation could be provided with this regard. The objective of the study was to assess the effect of carotid and vertebral artery stenosis on the function of the hearing and equilibrium organ on the basis of diagnostic audiological examinations including pure-tone threshold audiometry, impedance audiometry, otoacoustic emission tests and brainstem auditory evoked potential tests. The study was conducted in 63 patients (32 males, 31 females) aged 45-75 years, presenting with carotid and vertebral artery stenosis and treated at the Vascular Surgery Clinic of the University Clinical Hospital in Wroclaw. Patients were stratified into two subgroups according to their age (45-60 years, 61-75 years). Patients were also divided into subgroups according to the stenotic arteries and to the symptomatic/asymptomatic status of the disorder. All patients were homogeneous in terms of the degree of artery stenosis. The control group consisted of 32 healthy persons (14 males, 18 females) aged 48-75 years. Patients qualified to the control group reported no history of middle or inner ear disorders, disturbed hearing, vertigo and balance disorders, as well as cardiovascular diseases; they also presented with unremarkable ultrasound scans of the arteries. All patients were subjected to precise audiological examinations. Prior to being qualified for the study, patients were subjected to the assessment of arteries by means of Doppler ultrasonography. The hearing organ function was assessed by means of pure-tone threshold audiometry, impedance audiometry, otoacoustic emission tests and brainstem auditory evoked potential tests. Reduction of the flow through the carotid arteries causes problems in the organ of hearing; abnormalities are reported especially in tone threshold audiometry, examinations of the stapedius muscle reflexes and brainstem auditory evoked potentials, which prove the presence of receptive cochlear-extracochlear hearing damage. Disturbances of the organ of hearing have similar severity in stenosis of the internal carotid artery and vertebral artery. Abnormalities found in audiologic examinations in patients with carotid artery stenosis are not always explicitly clinically expressed in patients with hearing loss; we should consider diagnostics for carotid artery stenosis.

Negative hearing effects of a single course of IV aminoglycoside therapy in cystic fibrosis patients.

OBJECTIVE: Identify hearing effects of a single course of intravenous (IV) aminoglycoside antibiotics (AGs) therapy in adult cystic fibrosis (CF) patients. Determine whether the change is large enough to enable a proof-of-concept study of a new drug preventing AG-associated hearing loss. DESIGN: Retrospective case review of CF patients with sequential audiograms ± an intervening course of IV AG therapy. STUDY SAMPLE: 84 patients with no intervening IV AG treatment, 38 patients undergoing a single course of IV AGs. RESULTS: Using ASHA ototoxicity metrics, 45% of adult CF patients in the Single-IV group met the criteria for ototoxicity compared to 23% of the No-IV patients. Other hearing metrics including the average maximal threshold shift (TS) and average high frequency TS showed highly significant differences between groups. Testing only participants with mild or greater pre-therapy high frequency hearing loss further increased the differences between the two groups by every metric tested. CONCLUSION: Adult CF patients exposed to a single course of IV AGs have significantly greater TS than patients without IV AG exposure. Patients with mild to moderate hearing loss prior to AG-IVs are at increased risk of developing ototoxicity from subsequent parenteral AG therapy.

Hearing Disorders in Congenital Toxoplasmosis: A Literature Review

Abstract Introduction Several studies show correlations between congenital toxoplasmosis and hearing loss, with a broad diversity of levels of hearing loss and specifications of hearing disorders. Objective To describe the studies found in the literature regarding hearing disorders in congenital toxoplasmosis. Data Synthesis A literature review was conducted on the Lilacs, SciELO, PubMed and Scopus databases by combining the following keywords: congenital toxoplasmosis and hearing. Based on this search strategy, 152 papers were found, the majority published on the Scopus and PubMed databases from1958 to 2015. After theapplication of the inclusion criteria, 8 articles published between 1980 and 2015 were included in the present study. Conclusion This review showed a moderate evidence of the association between hearing disorders and congenital toxoplasmosis, which is characterized by sensorineural hearing loss. However, there are gaps in the description of the specific characteristics of the type and level of hearing loss, or of other possible disorders involved in the auditory processing.

Long-term hearing outcomes after gamma knife surgery in patients with vestibular schwannoma with hearing preservation: evaluation in 92 patients with serial audiograms.

The treatment strategy for patients with vestibular schwannoma (VS) is controversial, and data concerning the long-term hearing outcomes > 5 years after gamma knife surgery (GKS) are limited. The long-term hearing outcomes after GKS were evaluated in VS patients with hearing preservation. Ninety-two VS patients with a pure tone average (PTA) ≤ 50 dB were evaluated. The median age was 54 years; the median tumor volume was 1.5 cm3. The tumors were treated with a median margin dose of 12 Gy and a median mean cochlear dose of 4.0 Gy. At the time of GKS, 65 patients retained a PTA of 0-30 dB, and 27 had a PTA of 31-50 dB. The median follow-up period was 106 months. At the final follow-up, 2 (2%) developed tumor progression. During the median audiogram follow-up of 83 months, the PTA was ≤ 30 dB in 22 patients (24%) and 31-50 dB in 27 patients (29%); 43 patients (47%) worsened to a PTA > 50 dB. Hearing preservation rates were 66, 57, and 44% at 3, 5, and 10 years, respectively. In multivariate analysis, the mean cochlear dose (P < 0.001) and pre-GKS PTA (P = 0.045) were significant for hearing preservation. GKS was an effective treatment option for VS patients with a PTA ≤ 50 dB. As a lower cochlear dose and better pre-GKS PTA contributed to long-term hearing preservation, prophylactic GKS before hearing deterioration or tumor growth would be a treatment of choice if patients provided informed consent.

Sequential analysis as a tool for detection of amikacin ototoxicity in the treatment of multidrug-resistant tuberculosis / Análise sequencial como ferramenta na detecção da ototoxicidade da amicacina no tratamento da tuberculose multirresistente

ABSTRACT Objective: To investigate early detection of amikacin-induced ototoxicity in a population treated for multidrug-resistant tuberculosis (MDR-TB), by means of three different tests: pure-tone audiometry (PTA); high-frequency audiometry (HFA); and distortion-product otoacoustic emission (DPOAE) testing. Methods: This was a longitudinal prospective cohort study involving patients aged 18-69 years with a diagnosis of MDR-TB who had to receive amikacin for six months as part of their antituberculosis drug regimen for the first time. Hearing was assessed before treatment initiation and at two and six months after treatment initiation. Sequential statistics were used to analyze the results. Results: We included 61 patients, but the final population consisted of 10 patients (7 men and 3 women) because of sequential analysis. Comparison of the test results obtained at two and six months after treatment initiation with those obtained at baseline revealed that HFA at two months and PTA at six months detected hearing threshold shifts consistent with ototoxicity. However, DPOAE testing did not detect such shifts. Conclusions: The statistical method used in this study makes it possible to conclude that, over the six-month period, amikacin-associated hearing threshold shifts were detected by HFA and PTA, and that DPOAE testing was not efficient in detecting such shifts.

RESUMO Objetivo: Verificar a detecção precoce de ototoxicidade causada pelo uso de amicacina numa população tratada para tuberculose multirresistente (TBMR) por meio da realização de três testes distintos: audiometria tonal liminar (ATL), audiometria de altas frequências (AAF) e pesquisa de emissões otoacústicas por produto de distorção (EOAPD). Métodos: Estudo longitudinal de coorte prospectiva incluindo pacientes de ambos os sexos, com idade entre 18 e 69 anos, com diagnóstico de TBMR pulmonar e que necessitaram utilizar amicacina por seis meses em seu esquema medicamentoso antituberculose pela primeira vez. A avaliação auditiva foi realizada antes do início do tratamento e depois de dois e seis meses do início do tratamento. A análise dos resultados foi realizada por meio de análise estatística sequencial. Resultados: Foram incluídos 61 pacientes, mas a população final foi constituída de 10 pacientes (7 homens e 3 mulheres), em razão da análise sequencial. Ao se comparar os valores das respostas dos testes com aqueles encontrados na avaliação basal, foram verificadas mudanças nos limiares auditivos compatíveis com ototoxicidade após dois meses de tratamento através da AAF e após seis meses de tratamento através da ATL. Entretanto, essas mudanças não foram verificadas através da pesquisa de EOAPD. Conclusões: Ao se considerar o método estatístico utilizado nessa população, é possível concluir que mudanças nos limiares auditivos foram associadas ao uso da amicacina no período de seis meses por meio de AAF e ATL e que a pesquisa de EOAPD não se mostrou eficiente na identificação dessas mudanças.

Prospective evaluation of the early effects of radiation on the auditory system frequencies of patients with head and neck cancers and brain tumors after radiotherapy.

Patients with head and neck cancer after radiotherapy often suffer disability such as hearing disorders. In this study, the effect of radiotherapy (RT) on hearing function of patients with head and neck cancer after RT was determined according to the total dose delivered to specific parts of the auditory system. A total of 66 patients treated with primary or postoperative radiation therapy for various cancers in the head and neck region were selected. All patients had audiologic evaluation with pure tone audiometry for the frequencies of 250, 500, 1,000, 2,000, 3,000, 4,000, 6,000, and 8,000 Hz before and immediately after completion of treatment and again 3 months later. The cochlea dose volume histograms of the patients were derived from their computed-tomography-based treatment plans. At study's end, RT-induced auditory complications developed in 33% of patients. The greatest hearing loss (changes >15 dB) occurred immediately after RT at frequencies of 3,000 (14.5%), 4,000 (12.9%), 6,000 (13.6%), and 8,000 Hz (10.6%), and after 3 months of follow-up, at 3,000 (6.8%), 4,000 (7.7%), 6,000 (10.7%), and 8,000 Hz (12.1%). Univariate and multivariate analyses indicated a positive relationship between dose delivered to the cochlea and hearing loss (p < 0.001, r = 0.484). An increased risk of hearing loss was present for patients receiving ≥40 Gy as their mean dose compared with those receiving <30 Gy. We conclude that radiation dose has negative effects on the auditory system. This effect occurs more in high-frequency hearing. The received dose to the cochlea was the main cause of damage to hearing.

Profile Analysis of Psychological Symptoms Associated With Misophonia: A Community Sample.

Misophonia is characterized by extreme aversive reactions to certain classes of sounds. It has recently been recognized as a condition associated with significant disability. Research has begun to evaluate psychopathological correlates of misophonia. This study sought to identify profiles of psychopathology that characterize misophonia in a large community sample. A total of N = 628 adult participants completed a battery of measures assessing anxiety and anxiety sensitivity, depression, stress responses, anger, dissociative experiences, obsessive-compulsive symptoms and beliefs, distress tolerance, bodily perceptions, as well as misophonia severity. Profile Analysis via Multidimensional Scaling (PAMS) was employed to evaluate profiles associated with elevated misophonia and those without symptoms. Three profiles were extracted. The first two accounted for 70% total variance and did not show distinctions between groups. The third profile accounted for 11% total variance, and showed that misophonia is associated with lower obsessive-compulsive symptoms for neutralizing, obsessions generally, and washing compared to those not endorsing misophonia, and higher levels of obsessive-compulsive symptoms associated with ordering and harm avoidance. This third profile extracted also showed significant differences between those with and without misophonia on the scale assessing physical concerns (that is, sensitivity to interoceptive sensations) as assessed with the ASI-3. Further research is called for involving diagnostic interviewing and experimental methods to clarify these putative mechanisms associated with misophonia.

Hearing eyeball and/or eyelid movements on the side of a unilateral superior semicircular canal dehiscence.

Hearing of eyeball movements has been reported in superior semicircular canal dehiscence (SSCD), but not hearing of eyelid movements. Our main objective was to report the hearing of eyeball and/or eyelid movements in unilateral SSCD. Our secondary objective was to access its specificity to SSCD and discuss the underlying mechanism. Six patients with SSCD who could hear their eyeball and/or eyelid movements were retrospectively reviewed. With the aim of comparisons, eight patients with an enlarged vestibular aqueduct (EVA), who share the same mechanism of an abnormal third window, were questioned on their ability to hear their eyeball and/or eyelid movements. Three patients with SSCD could hear both their eyeball and eyelid movements as a soft low-pitch friction sound. Two patients with SSCD could hear only their eyelid movements, one of whom after the surgery of a traumatic chronic subdural hematoma. The latter remarked that every gently tapping on the skin covering the burr-hole was heard in his dehiscent ear as the sound produced when banging on a drum, in keeping with a direct transmission of the sound to the inner ear via the cerebrospinal fluid. One patient with SSCD, who could hear only his eyeball movements, had other disabling symptoms deserving operation through a middle fossa approach with an immediate relief of his symptoms. None of the eight patients with EVA could hear his/her eyeball or eyelid movements. Hearing of eyeball and/or eyelid movements is highly suggestive of a SSCD and do not seem to occur in EVA. In case of radiological SSCD, clinicians should search for hearing of eyeball and/or eyelid movements providing arguments for a symptomatic dehiscence. The underlying mechanism is discussed particularly the role of a cerebrospinal fluid transmission.

Speech Recognition Outcomes After Cochlear Reimplantation Surgery.

This study compares speech recognition outcomes before and after cochlear reimplantation surgery, in relation to clinical factors known before and at time of surgery. Between 2006 and 2015, 2,055 adult cochlear implant surgeries were conducted at this center, of which 87 were reimplantation surgeries (4.2%). Speech recognition scores (SRS) assessed before and after reimplantation were available for 54 adults. Overall, SRS measured after reimplantation were similar to the best SRS obtained by the patient and greater than the last SRS measured before surgery. Additional complications were noted in the clinical files of all patients for which reimplantation was considered unsuccessful (16%).

Advances in translational inner ear stem cell research.

Stem cell research is expanding our understanding of developmental biology as well as promising the development of new therapies for a range of different diseases. Within hearing research, the use of stem cells has focused mainly on cell replacement. Stem cells however have a broad range of other potential applications that are just beginning to be explored in the ear. Mesenchymal stem cells are an adult derived stem cell population that have been shown to produce growth factors, modulate the immune system and can differentiate into a wide variety of tissue types. Potential advantages of mesenchymal/adult stem cells are that they have no ethical constraints on their use. However, appropriate regulatory oversight seems necessary in order to protect patients from side effects. Disadvantages may be the lack of efficacy in many preclinical studies. But if proven safe and efficacious, they are easily translatable to clinical trials. The current review will focus on the potential application on mesenchymal stem cells for the treatment of inner ear disorders.

Parents are aware of the ototoxic effects of chemotherapy in paediatrics undergoing cancer treatment - Professional versus parental views: A pilot study.

BACKGROUND: The primary goal of chemotherapy is to cure cancer and its symptoms. Hence, in recent years, there has been an increase in cancer paediatric survival rate. However, there have also been adverse side effects such as ototoxic hearing loss because of chemotherapy. Therefore, this study aimed at exploring whether the parents of children undergoing chemotherapy are aware of ototoxic effects of chemotherapy. METHODS: A non-experimental quantitative study was conducted to collect data through questionnaires, one for paediatric oncologists and the other for parents. A convenience sampling strategy was employed to recruit 11 paediatric oncologists and 7 parents from two public hospitals in Gauteng. The questionnaires were analysed quantitatively, using descriptive statistics. RESULTS: About 55% of paediatric oncologists indicated informing parents about the ototoxic effects of chemotherapy. On the contrary, 71% of parents reported having been informed by paediatric oncologists about the possible hearing loss because of chemotherapy; however, 57% of the children are receiving a combination of cisplatin and cyclophosphamide despite being aware of their ototoxic nature. CONCLUSION: This study paves the way for qualitative studies to ascertain how parents are informed about the possible side effects such as hearing loss because of chemotherapy treatment. The mode in which parents are informed about the possible side effects related to chemotherapy is critical, considering that a high number of children are still receiving chemotherapeutic drugs that are directly linked to hearing loss.

Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy.

PURPOSE: We assessed whether patients with auditory auras have similar outcomes after epilepsy surgery as patients without auditory auras, and hypothesized that patients with non-dominant hemisphere foci might fare better after temporal lobe surgery than patients with dominant resections. METHODS: In this retrospective study, outcome after temporal resection was assessed for patients with drug-resistant epilepsy. Preoperative demographic data, clinical data, and surgical outcome were prospectively registered in a database from 1986 through 2016. Seizure outcome was classified as either seizure-free or relapsed. RESULTS: Data were available in 1186 patients. Forty five patients (3.8%) reported auditory auras; 42 patients (93%) had temporal lobe epilepsy (TLE), and three patients (7%) had extratemporal epilepsy. Since most patients with auditory auras had TLE and in order to have comparable groups, we selected 41 patients with auditory auras and compared them with patients without auditory auras who had temporal lobe resections (767 patients). There were no significant demographic or clinical differences between TLE patients with auditory auras and those without. Patients who had auditory auras were more likely to relapse after temporal lobe surgery than those without (p=0.03). Among patients who had auditory auras and temporal lobe surgery, side of surgery was not related to postoperative outcome (p=0.3). CONCLUSION: Auditory auras are rare among patients with drug-resistant TLE. The presence of an auditory aura in a patient with drug-resistant TLE carries a worse prognosis for a postoperative seizure free outcome and this is not related to the side of surgery.

Analysing use of the Chinese HHIE-S for hearing screening of elderly in a northeastern industrial area of China.

OBJECTIVE: The aim of the present study was to investigate the use of Chinese version of HHIE-S as a hearing screening tool for the elderly in an industrial area in northeast China. DESIGN: Prevalence, sensitivity and specificity of Chinese version of HHIE-S were calculated. Factors that had impact on HHIE-S were analysed. STUDY SAMPLE: Five hundred and seventy Mandarin speaking participants, aged from 50 to 85 years were included. They were tested with pure tone audiometry and Chinese version of HHIE-S. RESULTS: The prevalence of hearing handicap was 55.3%. The sensitivity and specificity of HHIE-S were 84.5% and 58.3% respectively when the pass/fail criteria were set at PTA0.5-4kHz >40 dBHL. In general, HHIE-S total and subscale scores were significantly associated with severity of hearing impairment. After stratified by severity of hearing impairment, both the prevalence of reported handicap and the scores of HHIE-S were not significantly associated with age. Male participants had significantly higher HHIE-S scores than female participants did. CONCLUSIONS: The Chinese version of HHIE-S contributes useful information to identifying hearing handicap and addressing the rehabilitative needs in the elderly in an industrial city in Mainland China.

O que sabemos das alterações auditivas centrais em crianças expostas ao álcool na gestação? Revisão sistemática / What we know of the central auditory disorders in children exposed to alcohol during pregnancy? Systematic review

RESUMO Objetivo Identificar os efeitos da ingestão de álcool na gestação sobre o sistema nervoso auditivo central em relação aos seus possíveis diagnósticos, Síndrome Fetal do Álcool, Síndrome Fetal do Álcool Parcial, Distúrbios ao Nascimento Relacionados ao Álcool e Distúrbio do Neurodesenvolvimento Relacionado ao Álcool, sua extensão e o método de avaliação auditiva. Estratégia de pesquisa Busca sistemática e integrativa nas bases de dados PubMed, LILACS e SciELO, com os termos em português e inglês “síndrome fetal do álcool”, “desordens relacionadas ao uso do álcool” associadas a “audição”. Critérios de seleção Dos 123 resumos identificados, foram seis selecionados, publicados até maio de 2015. Análise dos dados Foram elencados tópicos a serem respondidos, caracterização da casuística; o diagnóstico decorrente da exposição fetal nas crianças; método de avaliação auditiva; e resultados descritos. Resultados Entre as avaliações comportamentais, foram utilizados os testes dicóticos verbais com sílabas e com sentenças e o teste fala com ruído. Entre os testes eletrofisiológicos, no Potencial Evocado Auditivo de Tronco Encefálico, foi detectada alteração de sincronia neural, e no Potencial Evocado Auditivo de Longa Latência – P300, valores de latência precoces. Conclusão Existem evidências de que as crianças e adultos jovens expostos ao álcool na gestação apresentam sinais de comprometimento do sistema nervoso auditivo central, mas não foi possível caracterizar essas alterações nos diferentes subtipos diagnósticos do espectro. As vias auditivas corticais foram as mais investigadas e o método eletrofisiológico o mais utilizado, com um resultado inesperado em dois deles, a latência precoce da N2 e da P300.

ABSTRACT Purpose To identify the effects of alcohol intake during pregnancy on the central auditory nervous system in relation to their possible diagnosis, Fetal Alcohol Syndrome, partial Fetal Alcohol Syndrome, Alcohol-Related Birth Defects and Alcohol-Related Neurodevelopmental Disorder, his extension and the hearing assessment method. Research strategy Systematic and integrative review searched the databases PubMed, LILACS and SciELO, with terms in Portuguese and English “fetal alcohol syndrome”, “alcohol-related disorders” associated with “hearing”. Selection criteria: We identified 123 abstracts, six were selected and published until May 2015. Data analysis Were listed topics to be answered, characterization of the sample; the diagnosis result of fetal exposure; method of hearing assessment and described results. Results Among the behavioral assessments, Verbal Dichotic Tests with syllables and sentences and Speech in Noise Test, were used. Among the electrophysiological tests, the Brainstem Auditory Evoked Potential was detected change neural synchrony, and Long-Latency Auditory Evoked Potential – P300, early latency values. Conclusion There is evidence that children exposed to alcohol in utero present central auditory nervous system involvement signals, but it was not possible to identify the influence of different subtypes and their losses. Cortical auditory pathways were the most investigated and the electrophysiological method as used with an unexpected result in two of them, early N2 and P300 latency.