Genetic analysis of presbycusis by arrayed primer extension.

Using the Hereditary Hearing Loss arrayed primer extension (APEX) array, which contains 198 mutations across 8 hearing loss-associated genes (GJB2, GJB6, GJB3, GJA1, SLC26A4, SLC26A5, 12S-rRNA, and tRNA Ser), we compared the frequency of sequence variants in 94 individuals with early presbycusis to 50 unaffected controls and aimed to identify possible genetic contributors. This cross-sectional study was performed at Stanford University with presbycusis samples from the California Ear Institute. The patients were between ages 20 and 65 yr, with adult-onset sensorineural hearing loss of unknown etiology, and carried a clinical diagnosis of early presbycusis.

Steady-state response audiometry in a group of patients with steeply sloping sensorineural hearing loss.

Objectives: The purpose of this study was to examine the predictive value of auditory steady-state response (ASSR) evoked potential thresholds and predicted behavioral thresholds in a group of children with steeply sloping sensorineural hearing loss (HL).

Study Design: Case series.

Methods: Twenty-nine children with sloping sensorineural HL underwent behavioral audiometric evaluation, impedance testing, distortion product otoacoustic emissions, and steady-state response testing.

Auditory steady-state response audiometry in profound SNHL: the impact of abnormal middle ear function.

Auditory steady-state response (ASSR) audiometry is a commercially available tool that is used to predict behavioral auditory threshold levels. Its particular value stems from the technology's ability to measure frequency-specific responses in the background electroencephalogram to auditory stimuli presented across a broad range of frequencies and sound pressure levels. It is clearly of benefit when used to assess threshold levels in infants and children with severe-to-profound hearing impairment (i.