Some patients with congenital unilateral conductive hearing impairment had such good directional hearing and speech-in-noise scores in the unaided situation that no overall significant improvement occurred after BAHA fitting in our setup. Of the 18 patients with a complete data set, 6 did not show any significant improvement at all. However, compliance with BAHA use in this patient group was remarkably high. Observations of consistent use of the device are highly suggestive of patient benefit. Further research is recommended to get more insight into these findings.

No abstract

The BAHA was well accepted by most of the patients with congenital unilateral conductive hearing impairment. A preoperative trial of the BAHA system with the BAHA on a headband is part of the preoperative procedure. In children with unilateral conductive hearing loss, with regard to possible childs' development and communication difficulties, intervention with BAHA can be considered as an option.

Temporal processing, frequency discrimination and frequency resolution, three basic mechanisms involved in speech perception, were studied in 11 USH2a patients from 10 Dutch families. Measurements included loudness scaling, gap detection, determination of auditory filter shapes and difference limen for frequency. The results were compared to values obtained from listeners with normal hearing, DFNA8/12 patients, DFNA13 patients and unselected patients with presbyacusis. Overall, the results for USH2a were most similar to those obtained in patients with sensorineural hearing loss caused by hair cell defects. To our knowledge, this is the first study that presents results on audiometric measurements combined with psychophysical measurements in a group of USH2a patients.

In 1984 the Bone-Anchored Hearing Aid, or BAHA, system was introduced. Its transducer is coupled directly to the skull percutaneously to form a highly effective bone-conduction hearing device. Clinical studies on adults with conductive hearing loss have shown that the BAHA system outperforms conventional bone-conduction hearing aids. Therefore, the next step was to apply the BAHA system in children with congenital or acquired conductive hearing loss. Reviewed data showed that, on average, such children benefited significantly more from the BAHA than from reconstructive surgery. Thus, BAHA application appears to be the best option to achieve normal communication and speech and language development in children with bilateral conductive hearing loss. However, in children under the age of three to four years, a conventional solution must be applied, e.g. a bone conductor with a transcutaneous coupling, because they are too young to undergo BAHA implant surgery. In the case of unilateral congenital conductive hearing loss, there is no convincing evidence in the clinical literature for early intervention. In summary, the BAHA system can be considered a new, indispensable tool for children with bilateral conductive hearing loss.