THE USE OF GENETIC INFORMATION AND PSYCHOPHYSICAL CHARACTERISTICS TO DETERMINE HEARING AID AMPLIFICATION STRATEGY

Leijendeckers J, Bosman A, Pennings R, de Leenheer E, Plantinga R, Bom S, Snik A, Huygen P, Kunst H, Cremers C.

Department of Otorhinolaryngology / Audiology, University Medical Centre St Radboud, Nijmegen

This study reviews the phenotypes of several types of  hereditary hearing loss. By measuring psychophysical performance, cochlear functioning on a subtle scale can be investigated. Audiometric results further add to the phenotype information, which can be used to determine amplification parameters to relieve the hearing loss.

An overview will be given of results obtained from audiometric and psychophysical measurements in USH2a patients and DFNA2, DFNA8/12, DFNA9 and DFNA13 patients. Measurements include pure tone audiometry, speech recognition in quiet and in noise, loudness scaling, gap detection, determination of auditory filter shapes and difference limen for frequency.

Speech perception ability appears to be an important factor to distinguish between the consequences of sensorineural hearing loss in these patient groups. Overall results indicate that hearing qualities in USH2a patients closely resemble sensorineural hearing loss like presbyacusis. DFNA types 8/12 and 13 are mainly characterized by a marked performance of speech perception in noise and can be characterized as inner ear conductive hearing impairment. DFNA types 2 and 9, for example, are both characterized by high frequency sensorineural hearing impairment, but speech perception in DFNA2 is significantly better than in DFNA9 and also better than in presbyacusis.

E-mail: j.leijendeckers@kno.umcn.nl