Dead regions in the cochlea: diagnosis, perceptual consequences, and implications for the fitting of hearing AIDS.

Hearing impairment is often associated with damage to the hair cells in the cochlea. Sometimes there may be complete loss of function of inner hair cells (IHCs) over a certain region of the cochlea; this is called a "dead region". The region can be defined in terms of the range of characteristic frequencies (CFs) of the IHCs and/or neurons immediately adjacent to the dead region. This paper reviews the following topics: the effect of dead regions on the audiogram; methods for the detection and delineation of dead regions based on psychophysical tuning curves (PTCs) and on the measurement of thresholds for pure tones in "threshold equalizing noise" (TEN); effects of dead regions on speech perception; effects of dead regions on the perception of tones; implications of dead regions for fitting hearing aids. The main conclusions are: (1) Dead regions may be relatively common in people with moderate-to-severe sensorineural hearing loss; (2) Dead regions cannot be reliably diagnosed from the audiogram; (3) PTCs provide a useful way of detecting dead regions and defining their boundaries. However, the determination of PTCs is probably too time-consuming to be used for routine diagnosis of dead regions in clinical practice; (4) The measurement of detection thresholds for pure tones in TEN provides a simple method for clinical diagnosis of dead regions; (5) Pure tones with frequencies falling in a dead region do not evoke clear pitch sensations (pitch matching is highly variable) and the perceived pitch is sometimes, but not always, different from "normal". However, ratings of pitch clarity cannot be used as a reliable indicator of a dead region; (6) Amplification of frequencies well inside a high-frequency dead region usually does not improve speech intelligibility, and may sometimes impair it. However, there may be some benefit in amplifying frequencies up to 50 to 100% above the estimated low-frequency edge of a high-frequency dead region; (7) The optimal form of amplification for people with low-frequency dead regions remains somewhat unclear. There may be some benefit from avoiding the amplification of frequencies well inside a dead region; (8) Patients with extensive dead regions are likely to get less benefit from hearing aids than patients without dead regions; (9) For patients with diagnosed dead regions at high frequencies, consideration should be given to use of a hearing aid incorporating frequency transposition and/or compression.