Neural dissociation of the acoustic and cognitive representation of voice identity.

Recognising a speaker's identity by the sound of their voice is important for successful interaction. The skill depends on our ability to discriminate minute variations in the acoustics of the vocal signal. Performance on voice identity assessments varies widely across the population. The neural underpinnings of this ability and its individual differences, however, remain poorly understood. Here we provide critical tests of a theoretical framework for the neural processing stages of voice identity and address how individual differences in identity discrimination mediate activation in this neural network. We scanned 40 individuals on an fMRI adaptation task involving voices drawn from morphed continua between two personally familiar identities. Analyses dissociated neuronal effects induced by repetition of acoustically similar morphs from those induced by a switch in perceived identity. Activation in temporal voice-sensitive areas decreased with acoustic similarity between consecutive stimuli. This repetition suppression effect was mediated by the performance on an independent voice assessment and this result highlights an important functional role of adaptive coding in voice expertise. Bilateral anterior insulae and medial frontal gyri responded to a switch in perceived voice identity compared to an acoustically equidistant switch within identity. Our results support a multistep model of voice identity perception.

Auditory phenotype of Smith-Lemli-Opitz syndrome.

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive multiple congenital malformation and intellectual disability syndrome resulting from variants in DHCR7. Auditory characteristics of persons with SLOS have been described in limited case reports but have not been systematically evaluated. The objective of this study is to describe the auditory phenotype in SLOS. Age- and ability-appropriate hearing evaluations were conducted on 32 patients with SLOS. A subset of 21 had auditory brainstem response testing, from which an auditory neural phenotype is described. Peripheral or retrocochlear auditory dysfunction was observed in at least one ear of 65.6% (21) of the patients in our SLOS cohort. The audiometric phenotype was heterogeneous and included conductive, mixed, and sensorineural hearing loss. The most common presentation was a slight to mild conductive hearing loss, although profound sensorineural hearing loss was also observed. Abnormal auditory brainstem responses indicative of retrocochlear dysfunction were identified in 21.9% of the patients. Many were difficult to test behaviorally and required objective assessment methods to estimate hearing sensitivity. Individuals with SLOS are likely to have hearing loss that may impact communication, including speech and language development. Routine audiologic surveillance should be conducted to ensure prompt management of hearing loss.

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.

Age-Related Hearing Loss in C57BL/6J Mice Is Associated with Mitophagy Impairment in the Central Auditory System.

Aging is associated with functional and morphological changes in the sensory organs, including the auditory system. Mitophagy, a process that regulates the turnover of dysfunctional mitochondria, is impaired with aging. This study aimed to investigate the effect of aging on mitophagy in the central auditory system using an age-related hearing loss mouse model. C57BL/6J mice were divided into the following four groups based on age: 1-, 6-, 12-, and 18-month groups. The hearing ability was evaluated by measuring the auditory brainstem response (ABR) thresholds. The mitochondrial DNA damage level and the expression of mitophagy-related genes, and proteins were investigated by real-time polymerase chain reaction and Western blot analyses. The colocalization of mitophagosomes and lysosomes in the mouse auditory cortex and inferior colliculus was analyzed by immunofluorescence analysis. The expression of genes involved in mitophagy, such as PINK1, Parkin, and BNIP3 in the mouse auditory cortex and inferior colliculus, was investigated by immunohistochemical staining. The ABR threshold increased with aging. In addition to the mitochondrial DNA integrity, the mRNA levels of PINK1, Parkin, NIX, and BNIP3, as well as the protein levels of PINK1, Parkin, BNIP3, COX4, LC3B, mitochondrial oxidative phosphorylation (OXPHOS) subunits I-IV in the mouse auditory cortex significantly decreased with aging. The immunofluorescence analysis revealed that the colocalization of mitophagosomes and lysosomes in the mouse auditory cortex and inferior colliculus decreased with aging. The immunohistochemical analysis revealed that the expression of PINK1, Parkin, and BNIP3 decreased in the mouse auditory cortex and inferior colliculus with aging. These findings indicate that aging-associated impaired mitophagy may contribute to the cellular changes observed in an aged central auditory system, which result in age-related hearing loss. Thus, the induction of mitophagy can be a potential therapeutic strategy for age-related hearing loss.

Hipoacusia unilateral: bases neurobiológicas de la ambliaudia / Unilateral hearing loss: neurobiological basis of amblyaudia

Resumen La hipoacusia unilateral es una alteración prevalente e importante en la población infantil que puede llevar a un deterioro del procesamiento auditivo. Cualquier patología que cause hipoacusia asimétrica (conductiva o sensorioneural) durante ventanas críticas del desarrollo cerebral, puede dar lugar a déficits del procesamiento auditivo que podrían persistir incluso después de la rehabilitación audiológica. Este proceso fisiopatológico se ha denominado "ambliaudia" y los niños que la presentan tienen un mayor riesgo de sufrir retraso en el desarrollo del lenguaje, así como de padecer dificultades académicas, sociales y cognitivas. Por esto, la ambliaudia se presenta como un desafío en audiología, siendo necesario comprender los mecanismos neurobiológicos que la subyacen. Entender de mejor manera cuáles son las consecuencias que tiene la pérdida auditiva asimétrica sobre el desarrollo del sistema nervioso podría ser clave para guiar de mejor forma las intervenciones terapéuticas. En este artículo se revisan trabajos en humanos y en modelos animales que describen las consecuencias neurobiológicas de la deprivación auditiva unilateral durante el desarrollo del sistema nervioso y su posible aplicación a la práctica clínica.

Abstract Unilateral hearing loss is a prevalent and important disorder in children that can lead to an impairment of auditory processing. Any pathology that causes asymmetric (conductive or sensorineural) hearing loss during critical brain development windows can lead to hearing deficits that may persist even after audiological rehabilitation. This pathophysiological process has been referred to as amblyaudia, and children are at greater risk of experiencing language developmental delays as well as academic, social and cognitive impairments. For this reason, amblyaudia presents itself as a challenge in audiology, and it has become necessary to understand its neurobiological mechanisms. A better understanding of the consequences of asymmetric hearing loss on the development of the nervous system may be key to better guiding therapeutic interventions. This article reviews studies performed in humans and animal models that describe the neurobiological consequences of unilateral hearing deprivation during the development of the nervous system and its possible applications to clinical practice.

Neurodevelopmental Aspects and Cortical Auditory Maturation in Children with Cochlear Implants.

Background and objectives: The cochlear implant is not only meant to restore auditory function, but it also has a series of benefits on the psychomotor development and on the maturation of central auditory pathways. In this study, with the help of neuropsychological tests and cortical auditory potentials (CAEPs), we intend to identify a series of instruments that allow us to monitor children with a cochlear implant, and later on, to admit them into an individualized rehabilitation program. Materials and methods: This is a longitudinal study containing 17 subjects (6 boys and 11 girls) diagnosed with congenital sensorineural hearing loss. The average age for cochlear implantation in our cohort is 22 months old. Each child was tested before the cochlear implantation, tested again 3 months after the implant, and then 6 months after the implant. To test the general development, we used the Denver Developmental Screening Test (DDST II). CAEPs were recorded to assess the maturation of central auditory pathways. Results: The results showed there was progress in both general development and language development, with a significant statistical difference between the overall DQ (developmental quotient) and language DQ before the cochlear implantation and three and six months later, respectively. Similarly, CAEP measurements revealed a decrease of positive-going component (P1) latency after cochlear implantation. Conclusion: CAEPs and neuropsychological tests prove to be useful instruments for monitoring the progress in patients with cochlear implants during the rehabilitation process.

Systematic review protocol for assessing central auditory functions of Alzheimer's disease and its preclinical stages.

INTRODUCTION: A number of studies have reported an association between peripheral hearing impairment, central auditory processing and Alzheimer's disease (AD) and its preclinical stages. Both peripheral hearing impairment and central auditory processing disorders are observed many years prior to the clinical manifestation of AD symptoms, hence, providing a long window of opportunity to investigate potential interventions against neurodegenerative processes. This paper outlines the protocol for a systematic review of studies examining the central auditory processing functions in AD and its preclinical stages, investigated through behavioural (clinical assessments that require active participation) central auditory processing tests. METHODS AND ANALYSIS: We will use the keywords and Medical Subject Heading terms to search the following electronic databases: MEDLINE, PsychINFO, PubMed, Scopus, EMBASE and CINAHL Plus. Studies including assessments of central auditory function in adults diagnosed with dementia, AD and its preclinical stages that were published before 8 May 2019 will be reviewed. This review protocol will be reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Protocols guidelines. Data analysis and search results will be reported in the full review. This manuscript has designed the protocols for a systematic review that will identify the behavioural clinical central auditory processing measures that are sensitive to the changes in auditory function in adults with AD and its preclinical stages. Such assessments may subsequently help to design studies to examine the potential impact of hearing and communication rehabilitation of individuals at risk of AD. ETHICS AND DISSEMINATION: Ethical approval is not required as this manuscript only reports the protocols for conducting a systematic review as primary data will only be reviewed and not be collected. The results of this systematic review will be disseminated through publication and in scientific conferences. PROSPERO REGISTRATION NUMBER: CRD42017078272.

Evoked Potentials Reveal Noise Exposure-Related Central Auditory Changes Despite Normal Audiograms.

Purpose Complaints of auditory perceptual deficits, such as tinnitus and difficulty understanding speech in background noise, among individuals with clinically normal audiograms present a perplexing problem for audiologists. One potential explanation for these "hidden" auditory deficits is loss of the synaptic connections between the inner hair cells and their afferent auditory nerve fiber targets, a condition that has been termed cochlear synaptopathy. In animal models, cochlear synaptopathy can occur due to aging or exposure to noise or ototoxic drugs and is associated with reduced auditory brainstem response (ABR) wave I amplitudes. Decreased ABR wave I amplitudes have been demonstrated among young military Veterans and non-Veterans with a history of firearm use, suggesting that humans may also experience noise-induced synaptopathy. However, the downstream consequences of synaptopathy are unclear. Method To investigate how noise-induced reductions in wave I amplitude impact the central auditory system, the ABR, the middle latency response (MLR), and the late latency response (LLR) were measured in 65 young Veterans and non-Veterans with normal audiograms. Results In response to a click stimulus, the MLR was weaker for Veterans compared to non-Veterans, but the LLR was not reduced. In addition, low ABR wave I amplitudes were associated with a reduced MLR, but with an increased LLR. Notably, Veterans reporting tinnitus showed the largest mean LLRs. Conclusions These findings indicate that decreased peripheral auditory input leads to compensatory gain in the central auditory system, even among individuals with normal audiograms, and may impact auditory perception. This pattern of reduced MLR, but not LLR, was observed among Veterans even after statistical adjustment for sex and distortion product otoacoustic emission differences, suggesting that synaptic loss plays a role in the observed central gain. Supplemental Material https://doi.org/10.23641/asha.11977854.

Central auditory processing disorders after mild traumatic brain injury.

Auditory processing disorders are common following mild traumatic brain injury (mTBI), but the neurocircuitry involved is not well understood. The present study used functional MRI to examine auditory cortex activation patterns during a passive listening task in a normative population and mTBI patients with and without clinical central auditory processing deficits (APD) as defined by the SCAN-3:A clinical battery. Patients with mTBI had overall patterns of lower auditory cortex activation during the listening tasks as compared to normative controls. A significant lateralization pattern (pairwise t-test; p⟨0.05) was observed in normative controls and in those with mTBI and APD during single-side stimulation. Additionally, baseline connectivity between left and right auditory cortices was lower in mTBI patients than in controls (p=0.01) and significantly reduced in the mTBI with APD group (p=0.008). Correlation was also observed between bilateral task-related activation and competing words subscore of the SCAN-3:A. These findings suggest the passive listening task is well suited to probe auditory function in military personnel with an mTBI diagnosis. Further, the study supports the use of multiple approaches for detecting and assessing central auditory deficits to improve monitoring of short- and long-term outcomes.

Quantitative analysis tool for clinical functional MRI in mild traumatic brain injury.

Functional magnetic resonance imaging (fMRI) has been available commercially for clinical diagnostic use for many years. However, both clinical interpretation of fMRI by a neuroradiologist and quantitative analysis of fMRI data can require significant personnel resources that exceed reimbursement. In this report, a fully automated computer-based quantification methodology (Enumerated Auditory Response, EAR) has been developed to provide an auditory fMRI assessment of patients who have suffered a mild traumatic brain injury. Fifty-five study participants with interpretable auditory fMRI sequence data were assessed by EAR analysis, as well as both clinical radiologist fMRI interpretation and voxelwise general linear model (GLM) analysis. Comparison between the clinical interpretation and the two computer analysis methods resulted in 67% concordance (identical), 32% nearconcordance (one level difference), and 1% discordant. Comparison between the clinical computer-based quantification (EAR) and GLM analysis yielded significant correlations in right and left ear responses (p⟨0.05) for the full subject group. Automated fMRI quantification analysis equivalent to EAR might be appropriate for both future research projects with constrained resources, as well as possible routine clinical use.

Behavioral and Neural Correlates of Auditory n-Back Task in Adults With and Without Stuttering.

Purpose Adults with stuttering (AWS) exhibit compromised phonological working memory abilities, poor central auditory processing, and impaired auditory processing especially during overt speech production tasks. However, these tasks are sensitive to language disturbances already found in them. Thus, in this study, monosyllables were used ruling out the language effects, and auditory working memory ability was evaluated in AWS using the n-back task. In specific, the auditory sensory input of the working memory mechanism was evaluated. Method Thirty-two participants, 16 each of AWS and adults with no stuttering (AWNS), performed behavioral auditory 1-back and 2-back tasks. The long latency responses were also recorded during no-back and 2-back conditions from 64 electrode sites. Results Results revealed no significant differences between the groups in any of the behavioral parameters such as reaction time, accuracy, false alarm rate, or d'. N1 amplitude modulation was noted in AWNS, which was absent in AWS. The segmentation analysis showed a left hemisphere-oriented topographical distribution in the N2 region in AWS irrespective of conditions, whereas the scalp topography was right hemisphere-oriented with the involvement of parietal channels in AWNS. The timing differences existed between AWS and AWNS in the intervals that a topographical distribution lasted in all throughout the time window of analysis. Conclusion The results suggest altered neural pathway and hemispheric differences during auditory working memory tasks in AWS.

Nicotinic acetylcholine receptor subunit α7-knockout mice exhibit degraded auditory temporal processing.

The CHRNA7 gene that encodes the α7-subunit of the nicotinic acetylcholine receptor (α7-nAChR) has been associated with some autism spectrum disorders and other neurodevelopmental conditions characterized, in part, by auditory and language impairment. These conditions may include auditory processing disorders that represent impaired timing of neural activity, often accompanied by problems understanding speech. Here, we measure timing properties of sound-evoked activity via the auditory brainstem response (ABR) of α7-nAChR knockout mice of both sexes and wild-type colony controls. We find a significant timing delay in evoked ABR signals that represents midbrain activity in knockouts. We also examine spike-timing properties of neurons in the inferior colliculus, a midbrain nucleus that exhibits high levels of α7-nAChR during development. We find delays of evoked responses along with degraded spiking precision in knockout animals. We find similar timing deficits in responses of neurons in the superior paraolivary nucleus and ventral nucleus of the lateral lemniscus, which are brainstem nuclei thought to shape temporal precision in the midbrain. In addition, we find that other measures of temporal acuity including forward masking and gap detection are impaired for knockout animals. We conclude that altered temporal processing at the level of the brainstem in α7-nAChR-deficient mice may contribute to degraded spike timing in the midbrain, which may underlie the observed timing delay in the ABR signals. Our findings are consistent with a role for the α7-nAChR in types of neurodevelopmental and auditory processing disorders and we identify potential neural targets for intervention.NEW & NOTEWORTHY Disrupted signaling via the α7-nicotinic acetylcholine receptor (α7-nAChR) is associated with neurodevelopmental disorders that include impaired auditory processing. The underlying causes of dysfunction are not known but a common feature is abnormal timing of neural activity. We examined temporal processing of α7-nAChR knockout mice and wild-type controls. We found degraded spike timing of neurons in knockout animals, which manifests at the level of the auditory brainstem and midbrain.

Tinnitus: una patología cerebral / Tinnitus: a brain pathology

RESUMEN El tinnitus es un síntoma caracterizado por la percepción de un sonido en ausencia de un estímulo externo. Si bien su fisiopatología puede involucrar una alteración a nivel del funcionamiento del oído interno, la percepción de éste y el grado de molestias asociadas dependen de modificaciones de redes cerebrales cognitivas y emocionales. En la presente revisión, se abordan los cambios que existen a nivel coclear, de tronco encefálico, tálamo y la extensa red cerebral que dan cuenta del tinnitus, discutiendo como esta nueva conceptualización tiene importantes implicancias clínicas, permitiendo una mejor comprensión de los síntomas asociados al tinnitus, sus comorbilidades, y el desarrollo de nuevas estrategias terapéuticas.

ABSTRACT Tinnitus is a symptom characterized by the perception of a sound without an external stimulus. Although the pathophysiology of tinnitus initially involves an alteration of the inner ear function, the perception of it and the degree of distress associated with it depends on changes in cognitive and emotional brain networks. In this article, we review the changes that exist at the cochlea, brainstem, thalamus and a widespread cerebral networks that account for tinnitus, discussing how this new conceptualization has significant clinical implications and allows a better understanding of the symptoms associated with tinnitus, its co-morbidities, and how this view has allowed the development of new therapies.

Olfactory and other sensory impairments in Alzheimer disease.

The vast increase in Alzheimer disease (AD) worldwide has grave implications for individuals, family support systems and the health-care systems that will attempt to cope with the disease. Early markers of the disease are essential for efficient selection of clinical trial participants for drug development and for timely treatment once an intervention becomes available. There is avid interest in noninvasive, inexpensive markers that have the potential to identify prodromal AD. This Review considers sensory impairments that have the potential to serve as early indicators of AD, with a focus on olfaction, hearing and vision. Current evidence regarding the potential markers of AD in each modality is examined, with a particular emphasis on olfaction and current findings that olfactory function is associated with prodromal AD. Research suggests that olfactory impairment is associated with other markers that signal the emergence of prodromal AD. Auditory impairment is associated with dementia in epidemiological studies and visual system deficits have been reported in AD; however, the emergence of these deficits in prodromal AD is unclear. Further research is necessary to address the relative sensitivity and specificity of olfactory, auditory and visual measures for the detection of prodromal AD.

Peripheral auditory dysfunction secondary to traumatic brain injury: a systematic review of literature.

PRIMARY OBJECTIVE: To understand the effects of non-blast-related TBI on peripheral auditory function in adults, as measured through basic and advanced audiological assessments. BACKGROUND: Despite numerous studies demonstrating hearing loss post TBI there has been no systematic investigation of the prevalence, nature and severity of peripheral hearing loss. DATA IDENTIFICATION: An English-language systematic search using MEDLINE, CINAHL, PsychINFO, PubMed and hand-searching of reference lists was conducted from 1 January 1990 to 31 October 2016. STUDY SELECTION: After independent review by the authors, 20 of 281 originally identified articles were retained. DATA EXTRACTION: Audiological findings were extracted and synthesized across studies. RESULTS: Using the Oxford Centre for Evidence Based Medicine levels of evidence (2009), 3b was the highest level of evidence within the review. Sensorineural hearing loss was the most consistent auditory deficit reported post TBI. CONCLUSION: The range and frequency of auditory dysfunction in patients with TBI remain unclear. Future research should focus on understanding the nature, frequency and change of auditory deficits over time following TBI. Knowledge in this area will provide crucial information for clinicians and facilitate the development of diagnostic and best practice guidelines which currently are lacking for the management of this patient population.

The Middle Latency Response: A Review of Findings in Various Central Nervous System Lesions.

BACKGROUND: The middle latency response (MLR) first came to light as an auditory evoked potential in 1958. Since then, it has aroused substantial interest and investigation by clinicians and researchers alike. In recent history, its use and popularity have dwindled in tandem with various other auditory evoked potentials in audiology. One area for which MLR research and application has been overlooked is its potential value in measuring the neural integrity of the auditory thalamocortical pathway. In a broader sense, the MLR, when combined with the auditory brain stem response, can provide information concerning the status of much of the central auditory system pathways. This review is intended to provide information concerning the MLR as a measure of central auditory function for the reader to consider. PURPOSE: To review and synthesize the scientific literature regarding the potential value of the MLR in assessing the integrity of the central auditory system and to provide the reader an informed perspective on the value of the MLR in this regard. Information is also provided on the MLR generator sites and fundamental characteristics of this evoked potential essential to its clinical and or research application. RESEARCH DESIGN: A systematic review and synthesis of the literature focusing on the MLR and lesions of the central auditory system. STUDY SAMPLE: Studies and individual cases were reviewed and analyzed that evidenced documented lesions of the central auditory nervous system. DATA COLLECTION AND ANALYSIS: The authors searched and reviewed the literature (journal articles, book chapters, and books) pertaining to central auditory system lesion effects on the MLR. RESULTS: Although findings varied from study to study, overall, the MLR was reasonably sensitive and specific to neurological compromise of the central auditory system. This finding is consistent with the generator sites of this evoked potential. CONCLUSIONS: The MLR is a valuable tool for assessing the integrity of the central auditory system. It should be of interest to the clinician or researcher who focuses their attention on the function and dysfunction of the higher auditory system.

Síndrome de alport: reporte de un caso / Alport syndrome: Case report

El síndrome de Alport (SA) es un conjunto de enfermedades que se caracterizan por una afección hereditaria de la membrana basal glomerular con alteraciones en el colágeno tipo IV que la compone. Se presenta con hematuria micro- o macroscópica; además, suele asociarse a alteraciones auditivas y oculares, y es causa de alrededor de 0.3 a 3% de la enfermedad renal terminal en pediatría.Se reporta el caso de adolescente de 14 años, sexo masculino, que consultó por presentar fiebre, debilidad generalizada y palidez de tres días de evolución. Tenía historia de leucocoria en ojo derecho desde el nacimiento, antecedentes familiares de hematuria (padre y hermano), además hipoacusia en oído derecho. Esto sumado a la lesión ocular antes descrita y a insuficiencia renal crónica se sospechó SA, por lo que se le realizó una audiometría y un ultrasonido (USG) ocular los cuales reportaban una hipoacusia de oído derecho y condensaciones vítreas en ojo derecho respectivamente; dada la hematuria familiar y propia del paciente se realizó el diagnóstico de Síndrome de Alport según los criterios de Flinter...(AU)

Correlation between cognitive functions and central auditory processing in adolescents with non-cholesteatomatous chronic otitis media / Correlação entre funções cognitivas e processamento auditivo central em adolescentes com otite média crônica não colesteatomatosa

Abstract Auditory deprivation associated with early otitis media with effusion has been considered a risk factor for central auditory processing (CAP), as well as for the development of a number of cognitive functions. Objective: To study the neuropsychological functions of attention, working memory and executive function in adolescents with and without non-cholesteatomatous chronic otitis media (NCCOM) and analyze their interrelationships with the behavioral evaluation of CAP. Methods: Sixty-eight adolescents were recruited, 34 were diagnosed with NCCOM (study group - SG), and 34 had no otological history (control group - CG). The Neupsilin Brief Neuropsychological Assessment Instrument was used. CAP was assessed by: Masking Level Difference, Synthetic Sentence Identification, Random Gap Detection Test, Duration Pattern Sequence Test and Dichotic Digits Test. Results: The results of Neupsilin showed lower scores in the study group when compared to the control group on the following tests: digit sequence repetition, ascending digit ordering, auditory sentence span, and phonemic verbal fluency. An association was found between central auditory processing tests and Neupsilin subtests. Conclusion: The effects of NCCOM on attention, memory and executive function related to central auditory processing disorder in adolescents seem to be enhanced by the severity of the disease.

Resumo A privação auditiva associada à otite média precoce com efusão tem sido considerada um fator de risco para o processamento auditivo central (CAP), bem como para o desenvolvimento de diversas funções cognitivas. Objetivo: Estudar as funções neuropsicológicas da atenção, memória de trabalho e função executiva em adolescentes com e sem otite média crônica não-colesteatomatosa (NCCOM) e analisar suas inter-relações com a avaliação comportamental da PAC. Métodos: Sessenta e oito adolescentes foram recrutados, 34 foram diagnosticados com NCCOM (grupo de estudo - GE) e 34 não tinham história otológica (grupo controle - GC). Utilizou-se o Instrumento de Avaliação Neuropsicológica Breve de Neupsilin. O CAP foi avaliado por: Diferença de Nível de Mascaramento, Identificação de Frase Sintética, Teste de Detecção de Intervalo Aleatório, Teste de Sequência de Padrão de Duração e Teste de Dígitos Dicóticos. Resultados: Os resultados de Neupsilin mostraram menores escores no grupo de estudo quando comparados ao grupo controle nos seguintes testes: repetição de sequência de dígitos, ordenação de dígitos ascendentes, extensão de sentenças auditivas e fluência verbal fonêmica. Foi encontrada uma associação entre os testes do processamento auditivo central e os subtestes de Neupsilin. Conclusão: Os efeitos do NCCOM na atenção, memória e função executiva relacionados ao transtorno do processamento auditivo central em adolescentes parecem ser potencializados pela gravidade da doença.