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Purpose: The purpose of this study was to clarify the prevalence, type, severity, and age-dependency of hearing loss in 22q11.2 deletion syndrome. Method: Extensive audiological measurements were conducted in 40 persons with proven 22q11.2 deletion (aged 6-36 years). Besides air and bone conduction thresholds in the frequency range between 0.125 and 8.000 kHz, high-frequency thresholds up to 16.000 kHz were determined and tympanometry, acoustic reflex (AR) measurement, and distortion product otoacoustic emission (DPOAE) testing were performed. Results: Hearing loss was identified in 59% of the tested ears and was mainly conductive in nature. In addition, a high-frequency sensorineural hearing loss with down-sloping curve was found in the majority of patients. Aberrant tympanometric results were recorded in 39% of the ears. In 85% of ears with a Type A or C tympanometric peak, ARs were absent. A DPOAe response in at least 6 frequencies was present in only 23% of the ears with a hearing threshold ≤ 30 dB HL. In patients above 14 years of age, there was a significantly lower percentage of measurable DPOAEs. Conclusion: Hearing loss in 22q11.2 deletion syndrome is highly prevalent and both conductive and high-frequency sensorineural in nature. The age-dependent absence of DPOAEs in 22q11.2 deletion syndrome suggests cochlear damage underlying the high-frequency hearing loss., The 22q11.2 deletion syndrome (22q11DS), also known as Shprintzen syndrome, velocardiofacial syndrome, or Di George syndrome, is one of the most common chromosomal microdeletion syndromes with an estimated incidence of [...]

Auditory processing problems in persons with dyslexia are still subject to debate, and one central issue concerns the specific nature of the deficit. In particular, it is questioned whether the deficit is specific to speech and/or specific to temporal processing. To resolve this issue, a categorical perception identification task was administered in thirteen 11-year old dyslexic readers and 25 matched normal readers using 4 sound continua: (1) a speech contrast exploiting temporal cues (/bA/-/dA/), (2) a speech contrast defined by nontemporal spectral cues (/u/-/y/), (3) a nonspeech temporal contrast (spectrally rotated/bA/-/da/), and (4) a nonspeech nontemporal contrast (spectrally rotated/u/-/y/). Results indicate that children with dyslexia are less consistent in classifying speech and nonspeech sounds on the basis of rapidly changing (i.e., temporal) information whereas they are unimpaired in steady-state speech and nonspeech sounds. The deficit is thus restricted to categorizing sounds on the basis of temporal cues and is independent of the speech status of the stimuli. The finding of a temporal-specific but not speech-specific deficit in children with dyslexia is in line with findings obtained in adults using the same paradigm (Vandermosten et al., 2010, "Proceedings of the National Academy of Sciences of the United States of America," 107: 10389-10394). Comparison of the child and adult data indicates that the consistency of categorization considerably improves between late childhood and adulthood, particularly for the continua with temporal cues. Dyslexic and normal readers show a similar developmental progress with the dyslexic readers lagging behind both in late childhood and in adulthood. (Contains 2 figures and 2 tables.)

The etiology of developmental dyslexia remains widely debated. An appealing theory postulates that the reading and spelling problems in individuals with dyslexia originate from reduced sensitivity to slow-rate dynamic auditory cues. This low-level auditory deficit is thought to provoke a cascade of effects, including inaccurate speech perception and eventually unspecified phoneme representations. The present study investigated sensitivity to frequency modulation and amplitude rise time, speech-in-noise perception and phonological awareness in 11-year-old children with dyslexia and a matched normal-reading control children. Group comparisons demonstrated that children with dyslexia were less sensitive than normal-reading children to slow-rate dynamic auditory processing, speech-in-noise perception, phonological awareness and literacy abilities. Correlations were found between slow-rate dynamic auditory processing and phonological awareness, and speech-in-noise perception and reading. Yet, no significant correlation between slow-rate dynamic auditory processing and speech-in-noise perception was obtained. Together, these results indicate that children with dyslexia have difficulties with slow-rate dynamic auditory processing and speech-in-noise perception and that these problems persist until sixth grade. (Contains 1 figure and 3 tables.)

Objective: Recently, the digit triplet test was shown to be a sensitive speech-in-noise test for early high-frequency hearing loss in noise-exposed workers. This study investigates if a further improvement is achieved when using a closed set of consonant-vowel-consonant (CVC) speech items with the same vowel, and/or a low-pass (LP) filtered version of the standard speech-shaped noise. Design: Speech reception thresholds in noise were gathered for the digit triplet, CVC, and CVC_LP test and compared to the high-frequency pure-tone average (PTA). Study sample: 118 noise-exposed workers showing a wide range of high-frequency hearing losses. Results: For the 84 Dutch-speaking participants, the CVC test showed an increased measurement error and a decreased between-subject variation, leading to a weaker correlation with the PTA2,3,4,6 (R = 0.64) and thus a lower sensitivity compared to the digit triplet test (R = 0.86). However, the use of LP-filtered noise resulted in a sensitivity improvement (R = 0.79 versus R = 0.64) due to the large increase in between-subject spread. Similar trends were found for the 34 French-speaking workers. Conclusions: Using CVC words with the same vowel could not increase the sensitivity to detect isolated high-frequency hearing loss. With LP-filtered noise, test sensitivity improved, but it did not surpass the original digit triplet test. [ABSTRACT FROM AUTHOR]

Objective: To compare results on the everyday sentence test 'FIST', the new closed-set sentence test 'FrMatrix', and the digit triplet screening test 'FrDigit3'. Design: First, the FrMatrix was developed and normative values were obtained. Subsequently, speech reception thresholds (SRTs) for the three types of tests were gathered at four study centers representing different geographic regions in Belgium and France. Study sample: Fifty-seven normal-hearing listeners took part in the normative study of the FrMatrix, and 118 subjects, with a wide range of hearing thresholds, participated in the comparative study. Results: Homogenizing the individual words of the FrMatrix with regard to their intelligibility resulted in a reference SRT of −6.0 (±0.6) dB SNR and slope at the SRT of 14.0 %/dB. The within-subject variability was only 0.4 dB. Comparison of the three tests showed high correlations between the SRTs mutually (>0.81). The FrMatrix had the highest discriminative power, both in stationary and in fluctuating noise. For all three tests, differences across the participating study centers were small and not significant. Conclusions: The FIST, the FrMatrix, and the FrDigit3 provide similar results and reliably evaluate speech recognition performance in noise both in normal-hearing and hearing-impaired listeners. [ABSTRACT FROM AUTHOR]

In Flanders, periodic hearing screening of school children is mandatory. Up to now, pure tone audiometry was routinely used for this purpose. However, the lack of optimal testing conditions, uniform test protocols and referral criteria prompt the need for alternative screening methodologies. Currently, the applicability of the Digit Triplet Test -- a quick, automated speech-in-noise adaptive self-test -- for large-scale hearing screening of 10 and 14 year old children is being evaluated. Pupils with an abnormal test result (based on initially set age-specific cut-off speech reception thresholds) are referred to an ENT specialist for expertise pure tone testing. The aim is to fine-tune the cut-off values for the purpose of a balanced referral and investigate test reliability. After 5 months, more than 6000 children have already been screened. According to current cut-offs, 5.5% (10y) and 5.1% (14y) were referred. Intrasubject measurement error was found to be very low in both groups (0.6 dB). Median SRTs were -9.9 dB SNR for the younger and -10.6 for the older children. Also, test duration shortens with age. ENT expertise audiograms from referrals will allow us to set uniform and accurate cut-off values in order to obtain the highest possible sensitivity to detect (minimal) hearing impairment. Due to the low measurement error, accurate follow-up is possible. Furthermore, the availability of an effective registration system for data storage will enable to link school age hearing screening results with data from previous screenings and provide insights into the prevalence of acquired hearing loss in school children. [ABSTRACT FROM AUTHOR]

Over the last three years, EFAS has made an overview of audiology education programs in Europe. With the inputs of many EFAS representatives and local coordinators of the teaching programs, detailed information was received about more than 130 teaching programs in more than 20 European countries. In a first step, these programs have been specified according to 17 dimensions (e.g. duration of the program, degree to be earned, contact details, website, profile and strengths of the program). The situation in the different European countries is very diverse. Some countries don't have any specific audiology education program available, others offer bachelor and master programs in Audiology (e.g. Sweden, Denmark, Belgium, Portugal, Germany), some focus on training in medical audiology for physicians (e.g. Finland and France), etc. In a next step, the bachelor and master programs have been characterized in more detail. The specific courses of the individual programs were classified in a number of components (e.g. basic & applied physics, anatomy & physiology, research methodology). This more detailed characterization of the inventory helps to detect similarities and differences between the various European programs. Some programs are very technically oriented, while others are more clinically oriented. Audiology is sometimes the main focus, while it is also quite often combined with other topics like speech and language disorders. The inventory and more detailed classification of audiology teaching programs may facilitate collaboration and exchange. Moreover, it may help to identify a minimum common denominator or a core program. [ABSTRACT FROM AUTHOR]

Adaptive speech-in-noise tests allow for the fast and/or precise assessment of supra-threshold speech perception. Screening versions like the digit triplet test (DTT) can be used for the quick detection of hearing problems or to raise awareness about noise-induced hearing loss. In this study, we discuss several variations of the currently used DTT procedure. 17 normal-hearing (NH) and 34 hearing-impaired subjects were evaluated with 5 variations of the current procedure: (1) The baseline test with broadband stimuli and a fixed step size of ±2dB per triplet, (2) a 15dB low-pass filtered version to increase sensitivity for high-frequency hearing loss, and (3-5) three digit-scoring versions with adaptive step sizes targeting the 24, 50 and 76% point of the psychometric curve. The low-pass version resulted in the same test-retest error, duration and correlation with pure-tone thresholds as the baseline version, with a 13dB decrease in speech reception thresholds (SRTs) for NH subjects. The digit-scoring version targeting the 76% point maintained the same SRT, duration and correlation, but also achieved significantly lower test-retest errors. While the low-pass DTT combines supra-threshold SRTs and puretone audiometric thresholds, no significant improved correlation with the latter could be found. Digit scoring results in lower test-retest errors than triplet scoring, allowing for a more precise or faster SRT determination. Depending on the used step sizes, different points on the psychometric curve can be targeted to quickly reconstruct the complete curve. [ABSTRACT FROM AUTHOR]