Transient evoked otoacoustic emissions testing for screening of sensorineural deafness in puppies

<p><b>Background:</b> Transient evoked otoacoustic emissions (TEOAE) are widely used for human neonatal deafness screening, but have not been reported for clinical use in dogs.</p> <p><b>Hypothesis/Objectives:</b> To investigate the feasibility of TEOAE testing in conscious puppies and the ability of TEOAE testing to correctly identify deaf and hearing ears, as defined by brainstem auditory evoked response (BAER).</p> <p><b>Animals:</b> Forty puppies from 10 litters.</p> <p><b>Methods:</b> Prospective study on puppies presented for hearing assessment as part of a congenital deafness BAER screening program. Hearing status was determined using BAER. TEOAE testing was performed after the BAER assessment and the results of the TEOAE testing were compared with the hearing status for each ear. Parameters were tested for normality using the D'Agostino Pearson test and comparisons between the deaf and hearing ears were made using Mann-Whitney tests.</p> <p><b>Results:</b> TEOAE testing was readily performed in puppies presented for congenital deafness screening. Using analysis parameters based on those used in human neonatal hearing screening, TEOAE testing correctly identified all deaf ears, as defined by BAER testing, with a sensitivity of 100% (95% CI: 56-100%) for diagnosing deafness and specificity of 78% (95% CI: 66-87%).</p> <p><b>Conclusions and Clinical Importance:</b> TEOAE testing is an effective screening modality for identifying congenital sensorineural deafness in dogs. In light of the simpler and less expensive equipment, TEOAE testing has the potential to improve access to hearing screening and through this reduce the prevalence of congenital deafness in the dog.</p&gt

Molecular diagnostics for congenital hearing loss including 15 deafness genes using a next generation sequencing platform

Background: Hereditary hearing loss (HL) can originate from mutations in one of many genes involved in the complex process of hearing. Identification of the genetic defects in patients is currently labor intensive and expensive. While screening with Sanger sequencing for GJB2 mutations is common, this is not the case for the other known deafness genes (> 60). Next generation sequencing technology (NGS) has the potential to be much more cost efficient. Published methods mainly use hybridization based target enrichment procedures that are time saving and efficient, but lead to loss in sensitivity. In this study we used a semi-automated PCR amplification and NGS in order to combine high sensitivity, speed and cost efficiency. Results: In this proof of concept study, we screened 15 autosomal recessive deafness genes in 5 patients with congenital genetic deafness. 646 specific primer pairs for all exons and most of the UTR of the 15 selected genes were designed using primerXL. Using patient specific identifiers, all amplicons were pooled and analyzed using the Roche 454 NGS technology. Three of these patients are members of families in which a region of interest has previously been characterized by linkage studies. In these, we were able to identify two new mutations in CDH23 and OTOF. For another patient, the etiology of deafness was unclear, and no causal mutation was found. In a fifth patient, included as a positive control, we could confirm a known mutation in TMC1. Conclusions: We have developed an assay that holds great promise as a tool for screening patients with familial autosomal recessive nonsyndromal hearing loss (ARNSHL). For the first time, an efficient, reliable and cost effective genetic test, based on PCR enrichment, for newborns with undiagnosed deafness is available

The relationship between frontotemporal effective connectivity during picture naming, behavior, and preserved cortical tissue in chronic aphasia

NIH National Institute on Deafness and Other Communication Disorders (NIDCD) grant 1P50DC01228

A destressing "deafness" in French?

French is a language in which accent is mandatory on the last syllable of every content word. In contrast, Spanish uses accent to distinguish different lexical items (e.g., b'ebe vs beb'e). Two population of subjects were tested on the same materials to study whether such linguistic differences have an impact on the perceptual capacities of listeners. In Experiment 1, using an ABX paradigm, we find that French Subjects have a surprising deficit compared to Spanish Subjects in making accent distinctions. In Experiment 2, we find that Spanish subjects cannot ignore irrelevant differences in accent in a phoneme-based ABX task, whereas French Subjects have no difficulty at all. In Experiment 3, we replicate the basic French finding, and find that Spanish subjects benefit from redundant accent information even when phonemic information alone is sufficient to perform the task. In our final Experiment 4, we show that French subjects can hear the acoustic correlates of accent; their problem seem to arise at the level of short term memory. Implications for language-specific processing and acquisition are discussed

Brainstem auditory evoked responses in an equine patient population: part I--adult horses.

BackgroundBrainstem auditory evoked response has been an underused diagnostic modality in horses as evidenced by few reports on the subject.Hypothesis/objectivesTo describe BAER findings, common clinical signs, and causes of hearing loss in adult horses.AnimalsStudy group, 76 horses; control group, 8 horses.MethodsRetrospective. BAER records from the Clinical Neurophysiology Laboratory were reviewed from the years of 1982 to 2013. Peak latencies, amplitudes, and interpeak intervals were measured when visible. Horses were grouped under disease categories. Descriptive statistics and a posthoc Bonferroni test were performed.ResultsFifty-seven of 76 horses had BAER deficits. There was no breed or sex predisposition, with the exception of American Paint horses diagnosed with congenital sensorineural deafness. Eighty-six percent (n = 49/57) of the horses were younger than 16 years of age. The most common causes of BAER abnormalities were temporohyoid osteoarthropathy (THO, n = 20/20; abnormalities/total), congenital sensorineural deafness in Paint horses (17/17), multifocal brain disease (13/16), and otitis media/interna (4/4). Auditory loss was bilateral and unilateral in 74% (n = 42/57) and 26% (n = 15/57) of the horses, respectively. The most common causes of bilateral auditory loss were sensorineural deafness, THO, and multifocal brain disease whereas THO and otitis were the most common causes of unilateral deficits.Conclusions and clinical importanceAuditory deficits should be investigated in horses with altered behavior, THO, multifocal brain disease, otitis, and in horses with certain coat and eye color patterns. BAER testing is an objective and noninvasive diagnostic modality to assess auditory function in horses

How level and type of deafness affects user perception of multimedia video clips

Our research investigates the impact that hearing has on the perception of digital video clips, with and without captions, by discussing how hearing loss, captions and deafness type affects user QoP (Quality of Perception). QoP encompasses not only a user's satisfaction with the quality of a multimedia presentation, but also their ability to analyse, synthesise and assimilate informational content of multimedia . Results show that hearing has a significant effect on participants’ ability to assimilate information, independent of video type and use of captions. It is shown that captions do not necessarily provide deaf users with a ‘greater level of information’ from video, but cause a change in user QoP, depending on deafness type, which provides a ‘greater level of context of the video’. It is also shown that post-lingual mild and moderately deaf participants predict less accurately their level of information assimilation than post-lingual profoundly deaf participants, despite residual hearing. A positive correlation was identified between level of enjoyment (LOE) and self-predicted level of information assimilation (PIA), independent of hearing level or hearing type. When this is considered in a QoP quality framework, it puts into question how the user perceives certain factors, such as ‘informative’ and ‘quality’

Deafness-Related Materials: Collection Development and Information Retrieval

published or submitted for publicatio

Comparison of Academic Achievement between Students with Congenital and Acquired Deafness in a Nigerian College

Background:-Most students with deafness have some difficulty with academic achievement, especially with reading and mathematics. However, the range of intelligence levels of students with deafness does not differ from the range in their hearing counterparts. Academic performance must not be equated with intelligence. Most children who are deaf have normal intellectual capacity and it has been repeatedly demonstrated that their scores on non-verbal intelligence tests are approximately the same as those of the general population. Deafness imposes no limitations on the cognitive capabilities of individuals. The problems that deaf students often experience in academics and adjustment may be largely attributed to a bad fit between their perceptual abilities and the demands of spoken and written English. Several studies have suggested that one of the most potent predictors of academic achievement for the students with deafness is the amount of personalized and specialized attention they receive.           Objective:- The objective of this study was to investigate the presence of differences between the academic achievement of students with congenital and that of students with acquired deafness. Methods:- Fifty students with deafness (twenty-five students with congenital deafness and twenty-five students with acquired deafness) were studied ex-post facto.  All fifty students were of the same educational level at the Federal College of Education (Special) Oyo, Nigeria. The instruments used were teacher-made achievement test and subjects academic records on English and Mathematics. The teacher-made achievement test was tested valid with the use of Kuder-Richardson (KR-21), which indicated an alpha level of 0.62. Result:- There was no significant difference in the academic achievement (t = 2.00, p=0.973) in mathematics performance (t = 2.00, p=0.765) or in English Language performance (t= 2.00, p=0.680) between students with congenital deafness and those with acquired deafness. Conclusion: Academic achievement is comparable among non-mentally retarded deaf students who are undergoing proper rehabilitation irrespective of whether the deafness was congenital or acquired Keywords- Academic achievement, Congenital deafness, Acquired deafness, Nigerian colleg

Development of brainstem-evoked responses in congenital auditory deprivation

To compare the development of the auditory system in hearing and completely acoustically deprived animals, naive congenitally deaf white cats (CDCs) and hearing controls (HCs) were investigated at different developmental stages from birth till adulthood. The CDCs had no hearing experience before the acute experiment. In both groups of animals, responses to cochlear implant stimulation were acutely assessed. Electrically evoked auditory brainstem responses (E-ABRs) were recorded with monopolar stimulation at different current levels. CDCs demonstrated extensive development of E-ABRs, from first signs of responses at postnatal (p.n.) day 3 through appearance of all waves of brainstem response at day 8 p.n. to mature responses around day 90 p.n.. Wave I of E-ABRs could not be distinguished from the artifact in majority of CDCs, whereas in HCs, it was clearly separated from the stimulus artifact. Waves II, III, and IV demonstrated higher thresholds in CDCs, whereas this difference was not found for wave V. Amplitudes of wave III were significantly higher in HCs, whereas wave V amplitudes were significantly higher in CDCs. No differences in latencies were observed between the animal groups. These data demonstrate significant postnatal subcortical development in absence of hearing, and also divergent effects of deafness on early waves II–IV and wave V of the E-ABR