0000000000000862
AUTHOR
Andrea Bohnert
showing 5 related works from this author
Intrafamilial variability of the deafness and goiter phenotype in Pendred syndrome caused by a T416P mutation in the SLC26A4 gene.
2004
AbstractPendred syndrome (PS) is the most common cause of syndromic deafness, accounting for more than 5% of all autosomal-recessive hearing loss cases. It is characterized by bilateral sensorineural hearing loss and by goiter with or without hypothyroidism. Mutations in the SLC26A4 gene cause both classical PS and deafness associated with an enlarged vestibular aqueduct without goiter.To investigate a possible genotype-phenotype correlation in PS, we performed a detailed clinical and genetic study in three adult German sibs with typical PS caused by a common homozygous SLC26A4 mutation, T416P. An audiological long-term follow-up of 23 yr showed that the mutation T416P is associated with a …
Auditory, speech and language development in young children with cochlear implants compared with children with normal hearing.
2010
Abstract Objective This study had two aims: (1) to document the auditory and lexical development of children who are deaf and received the first cochlear implant (CI) by the age of 16 months and the second CI by the age of 31 months and (2) to compare these children's results with those of children with normal hearing (NH). Methods This longitudinal study included five children with NH and five with sensorineural deafness. All children of the second group were observed for 36 months after the first fitting of the device (cochlear implant). The auditory development of the CI group was documented every 3 months up to the age of two years in hearing age and chronological age and for the NH gro…
Evaluation of auditory development in infants and toddlers who received cochlear implants under the age of 24 months with the LittlEARS® Auditory Que…
2010
Abstract Background and Aims Newborn hearing screening and early intervention for congenital hearing loss have created a need for tools assessing the hearing development of very young children. A multidisciplinary evaluation of children's development is now becoming standard in clinical practice, though not many reliable diagnostic instruments exist. For this reason, the LittlEARS ® Auditory Questionnaire (LEAQ) was created to assess the auditory skills of a growing population of infants and toddlers who receive hearing instruments. The LEAQ relies on parent report, which has been shown to be a reliable way of assessing child development. Results with this tool in a group of children who re…
The German hearing in noise test
2020
The aims of this study were to develop a German Hearing In Noise Test (HINT) using the same methodology as with previous HINT tests; to develop sentence lists for measuring speech reception thresholds (SRTs); and to determine test-retest reliability and norms for measures obtained under headphones.The following steps were followed: develop and record sentences, synthesise masking noise, determine the performance-intensity (PI) function, equalise sentence difficulty in the masking noise. Form sentence lists of equal difficulty. Measure SRTs for normal hearing individuals to determine practice/learning effects, test-retest reliability, and norms.Three groups of adults (median age = 25 years) …
The Effect of Adaptive Nonlinear Frequency Compression on Phoneme Perception
2017
Purpose This study implemented a fitting method, developed for use with frequency lowering hearing aids, across multiple testing sites, participants, and hearing aid conditions to evaluate speech perception with a novel type of frequency lowering. Method A total of 8 participants, including children and young adults, participated in real-world hearing aid trials. A blinded crossover design, including posttrial withdrawal testing, was used to assess aided phoneme perception. The hearing aid conditions included adaptive nonlinear frequency compression (NFC), static NFC, and conventional processing. Results Enabling either adaptive NFC or static NFC improved group-level detection and recognit…