Cochlear implantation in children with white matter lesions: Prediction of hearing outcomes by multiple regression analysis.

ABSTRACT: Brain magnetic resonance imaging (MRI) white matter lesions have been reported in some preoperative cochlear implant children. However, the role of white matter lesions in predicting the hearing outcome is yet unclear. The present study investigated the outcomes of cochlear implantation (CI) in 40 children with white matter lesions.The data from children with white matter lesions were reviewed in this retrospective study. Based on brain MRI, the patients were divided into 3 groups: mild, moderate, and severe. The children were treated with unilateral CI and monitored for a follow-up period of at least 3 years. The main outcome measures were category of auditory performance (CAP) and speech intelligibility rating (SIR). MRI white matter lesions, age at implant, gender, physical impairment, and cognitive impairment were obtained from a research database to assess the correlation with long-term CAP and SIR outcome by multiple regression analysis.The data of children with white matter lesions were reviewed (18 females and 23 males). The mean age at implantation was 31.6 months. Strikingly, all children obtained better CAP and SIR scores. The age at implantation, brain white matters lesions on MRI, and cognitive and physical disabilities were associated with CAP and SIR scores. Multiple regression established a weak correlation between the degree of white matter lesions on brain MRI and long-term CAP and SIR, while cognitive impairment strongly accounted for long-term CAP and SIR outcome.The majority of the children with brain white matter lesions obtained a satisfactory postoperative effect. The cognitive impairment before CI is a major factor, and such factor should be considered.

Applications of qualitative grounded theory methodology to investigate hearing loss: protocol for a qualitative systematic review.

INTRODUCTION: Hearing loss is a chronic condition affecting 12 million individuals in the UK. People with hearing loss regularly experience difficulties interacting in everyday conversations. These difficulties in communication can result in a person with hearing loss withdrawing from social situations and becoming isolated. While hearing loss research has largely deployed quantitative methods to investigate various aspects of the condition, qualitative research is becoming more widespread. Grounded theory is a specific qualitative methodology that has been used to establish novel theories on the experiences of living with hearing loss. METHOD AND ANALYSIS: The aim of this systematic review is to establish how grounded theory has been applied to investigate the psychosocial aspects of hearing loss. Methods are reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis Protocols 2015 checklist. Studies included in this review will have applied grounded theory as an overarching methodology or have grounded theory embedded among other methodologies. Studies included will have adult participants (≥18 years) who are either people with an acquired hearing loss, their family and friends (communication partners), or healthcare practitioners including audiologists, general practitioners, ear, nose and throat specialists and hearing therapists. The quality of application of grounded theory in each study will be assessed using the Guideline for Reporting and Evaluating Grounded Theory Research Studies. ETHICS AND DISSEMINATION: As only secondary data will be used in this systematic review, ethical approval is not required. No other ethical issues are foreseen. This review is registered with the International Prospective Register of Systematic Reviews (http://www.crd.york.ac.uk/PROSPERO). Findings will be disseminated via peer-reviewed publications and at relevant academic conferences. Findings may also be published in relevant professional and third sector newsletters and magazines as appropriate. Data will inform future research and guideline development. PROSPERO REGISTRATION NUMBER: CRD42019134197.

Field test of the Rapid Assessment of Hearing Loss survey protocol in Ntcheu district, Malawi.

Objective: (1) To test the feasibility of the Rapid Assessment of Hearing Loss (RAHL) survey protocol in Malawi (Ntcheu); (2) To estimate the prevalence and probable causes of hearing loss (adults 50+).Design: Cross-sectional population-based survey.Study sample: Clusters (n = 38) were selected using probability-proportionate-to-size-sampling. Within each cluster, 30 people aged 50+ were selected using compact-segment-sampling. All participants completed smartphone-based audiometry (hearTest). Prevalence was estimated using WHO definitions (PTA of thresholds 0.5, 1, 2, 4 kHz in the better ear of >25 dB HL (any) and >40 dB HL (≥moderate)). Otoscopy and questionnaire were used to assess probable causes. Participants with hearing loss and/or ear disease were asked about care-seeking and barriers.Results: Four teams completed the survey in 24 days. 1080 of 1153 (93.7%) participants were examined. The median time to complete the protocol was 24 min/participant. Prevalence of hearing loss was 35.9% (95% CI = 31.6-40.2) (any level); and 10.0% (95% CI = 7.9-12.5) (≥moderate). The majority was classified as probable sensorineural. Nearly one third of people (30.9%) needed diagnostic audiology services and possible hearing aid fitting. Hearing aid coverage was <1%. Lack of perceived need was a key barrier.Conclusion: The RAHL is simple, fast and provides information about the magnitude and probable causes of hearing loss to plan services.

A Novel Method for Classifying Hearing Impairment in Epidemiological Studies of Aging: The Wisconsin Age-Related Hearing Impairment Classification Scale.

Purpose Longitudinal population-based cohort data were used to develop a standardized classification system for age-related hearing impairment using thresholds for frequencies (0.5-8 kHz) typically measured in cohort studies. Method Audiometric testing data collected in the Epidemiology of Hearing Loss Study from participants (n = 1,369) with four visits (1993-1995, 1998-2000, 2003-2005, and 2009-2010) were included (10,952 audiograms). Cluster analyses (Wald's method) were used to identify audiometric patterns. Maximum allowable threshold values were defined for each cluster to create an ordered scale. Progression was defined as a two-step change. Results An eight-step scale was developed to capture audiogram shape and severity of hearing impairment. Of the 1,094 participants classified as having normal hearing based on a pure-tone average, only 25% (n = 277) were classified as Level 1 (all thresholds ≤ 20 dB HL) on the new scale, whereas 17% (n = 182) were Levels 4-6. During the 16-year follow-up, 64.9% of those at Level 1 progressed. There was little regression using this scale. Conclusions This is the first scale developed from population-based longitudinal cohort data to capture audiogram shape across time. This simple, standardized scale is easy to apply, reduces misclassification of normal hearing, and may be a useful method for identifying risk factors for early, preclinical, age-related changes in hearing.

Cochlear implant outcomes in the elderly: a uni- and multivariate analyses of prognostic factors.

PURPOSE: To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. METHODS: All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. RESULTS: No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale). CONCLUSIONS: A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.

Avances en la rehabilitación auditiva con células madre / Advances in hearing rehabilitation with stem cells

Según la Organización Mundial de la Salud en el mundo hay 360 millones de personas que sufren algún tipo de pérdida auditiva discapacitante. Actualmente los tratamientos más usados para la rehabilitación de hipoacusias neurosensoriales son los aparatos electrónicos como audífonos o dispositivos implantables, tanto cocleares como osteointegrados. La presente revisión resume los avances que se han producido en la rehabilitación auditiva con células madre, y cómo éstas contribuyen en la generación de nuevas células ciliadas y neuronas auditivas. También se revisa el uso combinado con implantes cocleares, y cómo este último ayuda a la creación de redes entre las células ciliadas y las neuronas auditivas. Si bien queda un largo camino por recorrer y poder resolver algunos problemas como la implantación en la cóclea o ganglio espiral de las células o neuronas creadas exógenamente, este tipo de terapia abre una solución para una rehabilitación auditiva que en muchos casos supondría la solución definitiva para la discapacidad auditiva

There are 360 million people who suffers some kind of Hearing loss disability according to the World Health Organization (WHO). Currently, the electronic devices such as hearing aids or implantable devices both cochlear and osseointegrated, are the most useful treatments to hearing loss rehabilitation. The following review summarized both the advances that have occurred in the auditory rehabilitation with stem cells, and how they have helped in the generation of new hair cells and auditory neurons. Besides, both the combined use with cochlear implants and how it helps to create networks between the hair cells and the auditory neuron was reviewed. Although, there is a long way to go and solving some problems, such as implantation in the wall or the spiral ganglion of cells or neurons created exogenously, this kind of therapy opens a solution for a rehabilitation which would be the solution definitive for hearing impairment

The Epidemiology of Deafness.

Hearing loss is the most common sensory deficit worldwide. It affects ∼5% of the world population, impacts people of all ages, and exacts a significant personal and societal cost. This review presents epidemiological data on hearing loss. We discuss hereditary hearing loss, complex hearing loss with genetic and environmental factors, and hearing loss that is more clearly related to environment. We also discuss the disparity in hearing loss across the world, with more economically developed countries having overall lower rates of hearing loss compared with developing countries, and the opportunity to improve diagnosis, prevention, and treatment of this disorder.

Prevalence and nature of hearing loss in a cohort of children with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) may cause injury to any organ, including the auditory system. Although the association of SCD and hearing loss has been described, the nature of this complication is unknown. We sought to establish the prevalence and nature of hearing loss in a referred cohort of children with SCD and to identify correlating disease- or treatment-associated factors. PROCEDURE: We conducted a retrospective review of patients with SCD < 22 years of age who had hearing evaluations between August 1990 and December 2014. Demographics, audiograms, and disease and treatment variables were analyzed. RESULTS: Two hundred and ten audiograms among 81 patients were reviewed, and 189 were evaluable. Seventy-two children constituted the referred cohort. Fourteen (19.4%) had hearing loss documented on at least one audiogram. Seven (9.7%) patients had only conductive hearing loss, and the loss persisted for up to 10.3 years. The median age of first identification was eight years. Six (8.3%) patients had hearing loss that was at least partially sensorineural. One patient's hearing loss was ambiguous. All sensorineural hearing losses were unilateral and 4/6 patients had prior documented normal hearing, indicating acquired loss. No correlations were identified. CONCLUSIONS: Both conductive and sensorineural hearing losses are more prevalent in our study population than those observed in the general pediatric population. In children with SCD, sensorineural hearing loss appears to be acquired and unilateral. Conductive hearing loss was identified in older children and can persist. Serial screening is needed for early detection and more prompt intervention in this population.

Rehabilitation for disabling hearing loss: evaluating the need relative to provision of hearing aids in the public health care system.

Purpose: To determine the demand relative to supply of hearing rehabilitation through hearing aids for those with disabling hearing loss in a public health care setting in South Africa. Methods: Retrospective cross-sectional survey of medical records of all patients were seen at a public hospital (Polokwane Provincial Academic) during 2012-2014, was conducted. Audiological data from 3894 medical folders were accessed and reviewed; thereafter, results were analyzed using descriptive statistics. Results: Of the 3894 medical folders reviewed, two-third (62%, n = 2402) were diagnosed with hearing loss, mostly bilaterally (81%). More than 30% of all patients diagnosed with hearing loss were ≤10 years old. Sensorineural (permanent) hearing loss was diagnosed most often (38%, n = 913) and 74% (n = 1778) of hearing losses diagnosed were of moderate or worse severity (i.e., disabling loss). Hearing aids were fitted to only 15% (n = 272) of those diagnosed with disabling hearing loss and most hearing aid fittings were to low-income adult patients (≥25 years old) with more severe-profound hearing losses. Conclusions: This study showed that the need for hearing aids to provide hearing rehabilitation far exceeds the supply. Therefore, a multi-pronged approach that includes increased budget allocation and exploring low-cost interventions for developing countries to meet the demand for hearing aids. Furthermore, study highlighted a high prevalence of hearing loss in those younger than 10 years of age, and thus highlights the need for early intervention as well as intensifying efforts to reduce preventable causes of hearing loss. Implications for Rehabilitation Audiologists need to advocate for an increase in budget allocation for hearing rehabilitation devices. Study indicates need to explore low-cost hearing devices/rehabilitation interventions for developing countries. Health professionals should consider preventative measures to reduce prevalence of preventable hearing loss.

Cribado de la hipoacusia (parte 1) / Hearing loss screening (part 1)

El proceso por el que aprendemos y desarrollamos el lenguaje de forma natural es a través de la audición. La hipoacusia en la primera infancia limitará los estímulos auditivos y afectará significativamente al desarrollo del lenguaje y el habla, además de restringir el vínculo con el mundo que nos rodea. En los lactantes y niños pequeños, la detección y el tratamiento precoces de la hipoacusia pueden mejorar los resultados lingüísticos y escolares. En ausencia de cribado, la edad media a la que se confirma el diagnóstico de hipoacusia congénita en niños sin factores de riesgo está en torno a los 2-3 años. Los programas de cribado auditivo neonatal universal se han extendido ampliamente a pesar de la falta de pruebas sólidas que avalen su eficacia y coste-efectividad. En el presente trabajo, que se ha dividido en dos partes, el autor expone el tema de forma exhaustiva, con sus controversias y claroscuros, para finalmente pronunciarse sobre recomendaciones que el grupo PrevInfad ha consensuado para la consulta de los pediatras en Atención Primaria

The process of learning and developing our language in a natural way is through hearing. Infancy hearing loss will limit auditory stimuli and will significantly harm language and speech development and will narrow the bonds with the world around. In infants and small children, early hearing loss detection and treatment can improve language and school performance. In the absence of screening, medium age of congenital hearing loss diagnosis in children without risk factors is around 2-3 years. Universal new-born hearing screening has spread widely despite the lack of solid evidence supporting its effectivity and cost- efficiency. In this paper, that has been divided in two parts, the author thoroughly describes the topic, with its controversy and nuances, and finally declares on the recommendations that PrevInfad group have agreed for primary care paediatricians

Data-Driven Approach for Auditory Profiling and Characterization of Individual Hearing Loss.

Pure-tone audiometry still represents the main measure to characterize individual hearing loss and the basis for hearing-aid fitting. However, the perceptual consequences of hearing loss are typically associated not only with a loss of sensitivity but also with a loss of clarity that is not captured by the audiogram. A detailed characterization of a hearing loss may be complex and needs to be simplified to efficiently explore the specific compensation needs of the individual listener. Here, it is hypothesized that any listener's hearing profile can be characterized along two dimensions of distortion: Type I and Type II. While Type I can be linked to factors affecting audibility, Type II reflects non-audibility-related distortions. To test this hypothesis, the individual performance data from two previous studies were reanalyzed using an unsupervised-learning technique to identify extreme patterns in the data, thus forming the basis for different auditory profiles. Next, a decision tree was determined to classify the listeners into one of the profiles. The analysis provides evidence for the existence of four profiles in the data. The most significant predictors for profile identification were related to binaural processing, auditory nonlinearity, and speech-in-noise perception. This approach could be valuable for analyzing other data sets to select the most relevant tests for auditory profiling and propose more efficient hearing-deficit compensation strategies.

Superior Canal Dehiscence Involving the Superior Petrosal Sinus: A Novel Classification Scheme.

OBJECTIVES: To highlight superior semicircular canal dehiscence (SSCD) involving the superior petrosal sinus (SPS), and to propose a novel classification system for SPS associated SSCD with potential surgical implications. STUDY DESIGN: Multicenter retrospective review. SETTING: Three tertiary referral centers. PATIENTS: All patients diagnosed with SPS associated SSCD (1/2000 to 8/2016). Radiographic findings and clinical symptoms were analyzed. INTERVENTION: Surgical repair or observation. MAIN OUTCOME MEASURE: Radiographic findings and clinical symptoms were analyzed. RESULTS: Thirty-three dehiscences (30 patients) involving the SPS were identified. The average age at the time of presentation was 52.5 years (median, 56.9; range, 4.9-75.3 yr), and 53.3% of patients were men. Three patients had bilateral SPS associated SSCD. The most common associated symptoms at presentation were episodic vertigo (63.6%), subjective hearing loss (60.6%), and aural fullness (57.6%). Four distinct types of dehiscence were identified: class Ia. SSCD involving a single dehiscence into an otherwise normal appearing SPS; class Ib. SSCD involving a single dehiscence into an apparent venous anomaly of the SPS; class IIa. SSCD involving two distinct dehiscences into the middle cranial fossa and the SPS; class IIb. SSCD involving a single confluent dehiscence into the middle cranial fossa and the SPS. CONCLUSIONS: SSCD involving the SPS represents a small but distinct subset of SSCD cases. This scenario can create a unique set of symptoms and surgical challenges when intervention is sought. Clinical findings and considerations for surgical intervention are provided to facilitate effective diagnosis and management.

Auditory Phenotype of Smith-Magenis Syndrome.

Purpose: The purpose of this study was to describe the auditory phenotype of a large cohort with Smith-Magenis syndrome (SMS), a rare disorder including physical anomalies, cognitive deficits, sleep disturbances, and a distinct behavioral phenotype. Method: Hearing-related data were collected for 133 individuals with SMS aged 1-49 years. Audiogram data (97 participants) were used for cross-sectional and longitudinal analyses. Caregivers completed a sound sensitivity survey for 98 individuals with SMS and a control group of 24 unaffected siblings. Results: Nearly 80% of participants with interpretable audiograms (n = 76) had hearing loss, which was typically slight to mild in degree. When hearing loss type could be determined (40 participants), sensorineural hearing loss (48.1%) occurred most often in participants aged 11-49 years. Conductive hearing loss (35.2%) was typically observed in children aged 1-10 years. A pattern of fluctuating and progressive hearing decline was documented. Hyperacusis was reported in 73.5% of participants with SMS compared with 12.5% of unaffected siblings. Conclusions: This study offers the most comprehensive characterization of the auditory phenotype of SMS to date. The auditory profile in SMS is multifaceted and can include a previously unreported manifestation of hyperacusis. Routine audiologic surveillance is recommended as part of standard clinical care.

Hipoacusia neurosensorial bilateral del prematuro (Guías Clínicas de Prematurez) / Bilateral sensorineural hearing loss of prematurity (Clinical Guidelines for Prematurity)

Objetivo general: Generar recomendaciones basadas en la mejor evidencia disponible acerca de prevención, tratamiento y rehabilitación de la hipoacusia bilateral neurosensorial en recién nacidos y niños menores de 4 años

Ototoxicity (cochleotoxicity) classifications: A review.

OBJECTIVE: Drug-mediated ototoxicity, specifically cochleotoxicity, is a concern for patients receiving medications for the treatment of serious illness. A number of classification schemes exist, most of which are based on pure-tone audiometry, in order to assist non-audiological/non-otological specialists in the identification and monitoring of iatrogenic hearing loss. This review identifies the primary classification systems used in cochleototoxicity monitoring. By bringing together classifications published in discipline-specific literature, the paper aims to increase awareness of their relative strengths and limitations in the assessment and monitoring of ototoxic hearing loss and to indicate how future classification systems may improve upon the status-quo. DESIGN: Literature review. STUDY SAMPLE: PubMed identified 4878 articles containing the search term ototox*. RESULTS: A systematic search identified 13 key classification systems. Cochleotoxicity classification systems can be divided into those which focus on hearing change from a baseline audiogram and those that focus on the functional impact of the hearing loss. CONCLUSIONS: Common weaknesses of these grading scales included a lack of sensitivity to small adverse changes in hearing thresholds, a lack of high-frequency audiometry (>8 kHz), and lack of indication of which changes are likely to be clinically significant for communication and quality of life.

Acquired Hearing Loss in Children.

Hearing loss is the most common congenital sensory impairment. According to National Health and Nutrition Examination Survey data from 2001 to 2008, 20.3% of subjects aged greater than or equal to 12 had unilateral or bilateral hearing loss. The World Health Organization notes that, worldwide, there are 360 million people with disabling hearing loss, with 50% preventable. Although many hearing losses are acquired, many others are manifestations of preexisting conditions. The purpose of a pediatric hearing evaluation is to identify the degree and type of hearing loss and etiology and to outline a comprehensive strategy that supports language and social development and communication.