Central presbycusis: a review and evaluation of the evidence.

BACKGROUND: The authors reviewed the evidence regarding the existence of age-related declines in central auditory processes and the consequences of any such declines for everyday communication. PURPOSE: This report summarizes the review process and presents its findings. DATA COLLECTION AND ANALYSIS: The authors reviewed 165 articles germane to central presbycusis. Of the 165 articles, 132 articles with a focus on human behavioral measures for either speech or nonspeech stimuli were selected for further analysis. RESULTS: For 76 smaller-scale studies of speech understanding in older adults reviewed, the following findings emerged: (1) the three most commonly studied behavioral measures were speech in competition, temporally distorted speech, and binaural speech perception (especially dichotic listening); (2) for speech in competition and temporally degraded speech, hearing loss proved to have a significant negative effect on performance in most of the laboratory studies; (3) significant negative effects of age, unconfounded by hearing loss, were observed in most of the studies of speech in competing speech, time-compressed speech, and binaural speech perception; and (4) the influence of cognitive processing on speech understanding has been examined much less frequently, but when included, significant positive associations with speech understanding were observed. For 36 smaller-scale studies of the perception of nonspeech stimuli by older adults reviewed, the following findings emerged: (1) the three most frequently studied behavioral measures were gap detection, temporal discrimination, and temporal-order discrimination or identification; (2) hearing loss was seldom a significant factor; and (3) negative effects of age were almost always observed. For 18 studies reviewed that made use of test batteries and medium-to-large sample sizes, the following findings emerged: (1) all studies included speech-based measures of auditory processing; (2) 4 of the 18 studies included nonspeech stimuli; (3) for the speech-based measures, monaural speech in a competing-speech background, dichotic speech, and monaural time-compressed speech were investigated most frequently; (4) the most frequently used tests were the Synthetic Sentence Identification (SSI) test with Ipsilateral Competing Message (ICM), the Dichotic Sentence Identification (DSI) test, and time-compressed speech; (5) many of these studies using speech-based measures reported significant effects of age, but most of these studies were confounded by declines in hearing, cognition, or both; (6) for nonspeech auditory-processing measures, the focus was on measures of temporal processing in all four studies; (7) effects of cognition on nonspeech measures of auditory processing have been studied less frequently, with mixed results, whereas the effects of hearing loss on performance were minimal due to judicious selection of stimuli; and (8) there is a paucity of observational studies using test batteries and longitudinal designs. CONCLUSIONS: Based on this review of the scientific literature, there is insufficient evidence to confirm the existence of central presbycusis as an isolated entity. On the other hand, recent evidence has been accumulating in support of the existence of central presbycusis as a multifactorial condition that involves age- and/or disease-related changes in the auditory system and in the brain. Moreover, there is a clear need for additional research in this area.

Genetics of isolated auditory neuropathies.

Auditory neuropathies are disorders combining absent or abnormal auditory brainstem responses with preserved otoacoustic emissions and/or cochlear microphonics. These features indicate a normal function of cochlear outer hair cells. Thus, the primary lesion might be located in the inner hair cells, in the auditory nerve or in the intervening synapse. Auditory neuropathy is observed in up to 10 percent of deaf infants and children, either as part of some systemic neurodegenerative diseases or as an isolated entity. Research on the genetic causes of isolated auditory neuropathies has been remarkably successful in the last few years. Here we review the current knowledge on the structure, expression and function of the genes and proteins so far known to be involved in these disorders, as well as the clinical features that are associated with mutations in the different genes. This knowledge is permitting to classify isolated auditory neuropathies into etiologically homogeneous types, so providing clues for the better diagnosis, management and therapy of the affected subjects.

Hipoacusias centrales / Central hypoacusis

Se retoma el interes por el estudio del Sistema Nervioso Auditivo Central (SNAC) iniciado por Bocca en la decada de los 50, con el enfoque de autores contemporaneos quienes describen las funciónes de análisis del sonido, las habilidades auditivas centrales, la clasificación de los trastomos del SNAC y la aplicación de pruebas auditivas con sensibilidad diagnostica central