[The application of the screening questionnaires for the diagnostics of the central auditory processing disorders in the pediatric practice]. / Ispol'zovanie skriningovykh anket dlia vyiavleniia tsentral'nykh slukhovykh rasstroistv v pediatricheskoi praktike.

Identification and diagnostics of auditory processing disorders (APD) in the children continues to be a challenge. Screening tools help to decide the need for evaluation. However, none of them are used routinely in Russia, nor are there thus far auditory checklist questionnaires in the Russian language. The aim of his study was to approve Children's Auditory Performance Scale (CHAPS) and Fisher's Auditory Checklist in Russian for the children at the age of 5-10 years. The study included 52 children at the age from 5 to 11 years. All of them were native Russian speakers. Pure tone audiometry, immitancemetry, and the Simplified RuMatrix test were performed for all the children. All of them had normal hearing thresholds, speech and language development, and normal academic achievement at school. The parents filled in the CHAPS and Fisher's Auditory Checklist questionnaires in Russian. The mean value of Fisher's Auditory Checklist was 85.7 ± 9.3, the mean value of CHAPS questionnaire was equal to 0.08 ± 0.26. The results obtained with the use of the Russian language questionnaires are in excellent agreement with the test data yielded by the respective English language versions. It is recommended that the clinical assessment for APD should be performed for children with the result of the Fisher questionnaire less than 67 points and with the result of the CHAPS questionnaire less than -1 point. It is concluded that Fisher's questionnaire was more convenient for the parents to fill in.

Auditory dysfunction in patients with cerebrovascular disease.

Auditory dysfunction is a common clinical symptom that can induce profound effects on the quality of life of those affected. Cerebrovascular disease (CVD) is the most prevalent neurological disorder today, but it has generally been considered a rare cause of auditory dysfunction. However, a substantial proportion of patients with stroke might have auditory dysfunction that has been underestimated due to difficulties with evaluation. The present study reviews relationships between auditory dysfunction and types of CVD including cerebral infarction, intracerebral hemorrhage, subarachnoid hemorrhage, cerebrovascular malformation, moyamoya disease, and superficial siderosis. Recent advances in the etiology, anatomy, and strategies to diagnose and treat these conditions are described. The numbers of patients with CVD accompanied by auditory dysfunction will increase as the population ages. Cerebrovascular diseases often include the auditory system, resulting in various types of auditory dysfunctions, such as unilateral or bilateral deafness, cortical deafness, pure word deafness, auditory agnosia, and auditory hallucinations, some of which are subtle and can only be detected by precise psychoacoustic and electrophysiological testing. The contribution of CVD to auditory dysfunction needs to be understood because CVD can be fatal if overlooked.

Cochlear implantation in children with auditory neuropathy spectrum disorder.

OBJECTIVE: To report the patient's characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs). DESIGN: Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management protocol that included electrophysiologic and medical assessment, documentation of behavioral audiometric thresholds and subsequent fitting of amplification according to Desired Sensation Level targets, auditory-based intervention with careful monitoring of skills development and communication milestones, and finally implantation when progress with the use of acoustic amplification was insufficient. RESULTS: Of 140 children with ANSD, 52 (37%) received CIs in their affected ears (mean duration of use of 41 mos). Many of these children were born prematurely (42%) and impacted by a variety of medical comorbidities. More than one third (38%) had abnormal findings on preoperative magnetic resonance imaging of the brain and inner ear, and 81% had a greater than severe (>70 dB HL) degree of hearing loss before implantation. Although 50% of the implanted children with ANSD demonstrated open-set speech perception abilities after implantation, nearly 30% of them with >6 months of implant experience were unable to participate in this type of testing because of their young age or developmental delays. No child with cochlear nerve deficiency (CND) in their implanted ear achieved open-set speech perception abilities. In a subgroup of children, good open-set speech perception skills were associated with robust responses elicited on electrical-evoked intracochlear compound action potential testing when this assessment was possible. CONCLUSIONS: This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.

Central auditory impairment in unilateral diencephalic and telencephalic lesions.

The extent of perceptual impairment following unilateral lesions in the auditory cortex, its thalamic or callosal afferents was studied with psychoacoustic tests. Thresholds for the discrimination of signal frequency, intensity and duration were acquired under three different conditions of headphone stimulation ('monaural', 'interaural', and 'dichotic signal/noise tests') using the three-alternative forced-choice procedure. The different test alternatives generated distinct auditory percepts, which is in accordance with the assumption of specific signal processing at the level of the auditory brainstem and at thalamocortical auditory areas. Twenty-one patients from neurology were studied who suffered from unilateral lesions in the auditory cortex, the auditory thalamus, or the acoustic radiation. Location and extent of the lesions were assessed by magnetic resonance imaging. Monaural tests of either ear revealed no deficits in auditory performance. The patients showed impaired discrimination of signal frequency, intensity and duration in the dichotic signal/noise tests, when the signals were presented to the ear contralateral and the noise ipsilateral to the lesion. With inverted signal and noise stimulation, however, the thresholds were in the range of age-matched controls. All patients were able to master the interaural tests, which indicates the preserved ability to lateralize sound sources to the left and to the right with either one of the auditory cortices left intact. Another 24 patients were studied who had lesions mostly close to but sparing the before-mentioned auditory structures. All of them showed unimpaired performance in all test alternatives. The results indicate the specificity of the dichotic signal/noise tests for the identification of unilateral lesions in thalamocortical auditory structures. In addition, the results also point to the capacity of each telencephalic hemisphere to process the full range of auditory lateralization from left to right.

Auditory system dysfunction in Alzheimer disease and its prodromal states: A review.

Recent findings suggest that both peripheral and central auditory system dysfunction occur in the prodromal stages of Alzheimer Disease (AD), and therefore may represent early indicators of the disease. In addition, loss of auditory function itself leads to communication difficulties, social isolation and poor quality of life for both patients with AD and their caregivers. Developing a greater understanding of auditory dysfunction in early AD may shed light on the mechanisms of disease progression and carry diagnostic and therapeutic importance. Herein, we review the literature on hearing abilities in AD and its prodromal stages investigated through methods such as pure-tone audiometry, dichotic listening tasks, and evoked response potentials. We propose that screening for peripheral and central auditory dysfunction in at-risk populations is a low-cost and effective means to identify early AD pathology and provides an entry point for therapeutic interventions that enhance the quality of life of AD patients.

Brain abnormalities in language disorders and in autism.

It has been speculated that autism and specific language impairment share common underlying neural substrates because of the overlap in language impairment issues and evidence suggesting parallels in other domains and implying a possible shared genetic risk. Anatomically the two sets of disorders have generally been studied using different methodologies, but when identical methodologies have been used substantial similarities have been noted. Functionally there is a growing body of literature suggesting sensory perception abnormalities that have parallels in both conditions and that may be upstream of language abnormalities. Finding upstream mechanisms that impact language and non-language abnormalities in autism and specific language impairment would impact the orientation taken by translational attempts to use science to design treatments.

Tinnitus and neural plasticity of the brain.

OBJECTIVE: To describe the current ideas about the manifestations of neural plasticity in generating tinnitus. DATA SOURCES: Recently published source articles were identified using MEDLINE, PubMed, and Cochrane Library according to the key words mentioned below. STUDY SELECTION: Review articles and controlled trials were particularly selected. DATA EXTRACTION: Data were selected systematically, scaled on validity and comparability. CONCLUSION: An altered afferent input to the auditory pathway may be the initiator of a complex sequence of events, finally resulting in the generation of tinnitus at the central level of the auditory nervous system. The effects of neural plasticity can generally be divided into early modifications and modifications with a later onset. The unmasking of dormant synapses, diminishing of (surround) inhibition and initiation of generation of new connections through axonal sprouting are early manifestations of neural plasticity, resulting in lateral spread of neural activity and development of hyperexcitability regions in the central nervous system. The remodeling process of tonotopic receptive fields within auditory pathway structures (dorsal cochlear nucleus, inferior colliculus, and the auditory cortex) are late manifestations of neural plasticity. The modulation of tinnitus by stimulating somatosensory or visual systems in some people with tinnitus might be explained via the generation of tinnitus following the nonclassical pathway. The similarities between the pathophysiological processes of phantom pain sensations and tinnitus have stimulated the theory that chronic tinnitus is an auditory phantom perception.

A comparison of higher order auditory processes in younger and older adults.

The authors conducted 3 experiments investigating the effects of aging on higher order auditory processes. They compared younger and older adults with respect to (a) their auditory channel capacity, (b) the extent of their top-down control over auditory gain, and (c) their ability to focus attention on a narrow band of frequencies. To ensure that subclinical cochlear processing deficits in older adults (e.g., higher thresholds, poorer discrimination of frequency and intensity differences) did not limit performance, the authors used only stimuli that were perfectly discriminable by all participants. No age differences were found in any of these experiments, suggesting that some higher order auditory processes (e.g., top-down control over auditory gain, auditory attention) are preserved in normal aging, despite numerous age-related declines in peripheral auditory functionality.

Central auditory dysfunction may precede the onset of clinical dementia in people with probable Alzheimer's disease.

OBJECTIVES: To document the prognostic significance of a central auditory speech-processing deficit for the subsequent onset of probable Alzheimer's disease. DESIGN: Prospective cohort study. SETTING: Framingham Heart Study. PARTICIPANTS: Seven hundred forty dementia-free volunteers from the Framingham Heart Study cohort with symmetric hearing thresholds at biennial examination 15 (1983-1985). MEASUREMENTS: The diagnosis of probable Alzheimer's disease was made prospectively using the National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease Related Disorder Association criteria. A central auditory speech-processing deficit was defined as a score of 50% of less correct on the Synthetic Sentence Identification with Ipsilateral Competing Message test in at least one ear with normal word recognition ability in both ears. Cox proportional hazards regression assessed the relationship between a central auditory speech-processing deficit and the age at diagnosis of probable Alzheimer's disease. RESULTS: Forty subjects (5.4%) received a diagnosis of probable Alzheimer's disease during an average of 8.4 years (range 3-12) of follow-up; seven (17.5%) of these had a central auditory speech-processing deficit. The presence of a central auditory speech-processing deficit had an age-adjusted risk ratio for probable Alzheimer's disease of 10.8 (95% CI = 4.6-25.2), and the estimated risk ratio adjusted for age, gender, education level, apolipoprotein allele E4 presence, and hearing level was 23.3 (95% CI =6.6-82.7). A central auditory speech-processing deficit had a positive predictive value for subsequent probable Alzheimer's disease of 47% but the sensitivity was only 17.5%. CONCLUSION: Central auditory speech-processing deficits may be an early manifestation of probable Alzheimer's disease and may precede the onset of dementia diagnosis by many years.

Auditory neuropathy/auditory dys-synchrony in school children with hearing loss: frequency of occurrence.

OBJECTIVE: The frequency of occurrence of a recently identified hearing disorder, auditory neuropathy/auditory dys-synchrony (AN/AD), was investigated in children with hearing impairment in Hong Kong. METHODS: In this study, 56 students, aged 7-18 years, attending primary divisions in schools for the hearing impaired were screened using otoacoustic emission procedures. RESULTS: One student in the study group was found to have intact outer hair cell function. A detailed diagnostic assessment of this case strongly suggested AN/AD was involved. Combining the study results with those of a previously conducted survey in Hong Kong, the frequency of occurrence of AN/AD in children attending schools for the hearing impaired was estimated to be 2.44% (3 out of 123 children screened). Estimates of the frequency of occurrence of AN/AD in various other educational settings in Hong Kong were also derived. CONCLUSIONS: The study indicates that AN/AD is not an extremely rare disorder. Educational audiologists and other health care professionals need to be actively involved in the identification and management of AN/AD. Research should be directed at technological innovations that may help to overcome the limitations of present screening procedures, in order to more accurately identify the disorder.