Expressivity of hearing loss in cases with Usher syndrome type IIA.

Objective: The purpose of this study was to compare the genotype/phenotype relationship between siblings with identical USH2A pathologic mutations and the consequent audiologic phenotypes, in particular degree of hearing loss (HL). Decade audiograms were also compared among two groups of affected subjects with different mutations of USH2A.

Design: DNA samples from patients with Usher syndrome type II were analysed.

Hearing performance benefits of a programmable power baha® sound processor with a directional microphone for patients with a mixed hearing loss.

Objectives: New signal processing technologies have recently become available for Baha® sound processors. These technologies have led to an increase in power and to the implementation of directional microphones. For any new technology, it is important to evaluate the degree of benefit under different listening situations.

Baha solutions for patients with severe mixed hearing loss.

Patients with a mixed hearing loss present special challenges. The amplification demands of mixed hearing loss can drive powerful digital hearing aids to their limits and introduce distortion through saturation. Conversely, the Baha System effectively bypasses the conductive component and focuses on compensating for the sensorineural component of the hearing loss.