Pepler, A., Munro, K., Lewis, K. & Kluk, K. (2014). Prevalence of Cochlear Dead Regions in New Referrals and Existing Adult Hearing Aid Users. Ear and Hearing 20(10), 1-11.
This editorial discusses the clinical implications of an independent research study and does not represent the opinions of the original authors.
Cochlear dead regions are areas in which, due to inner hair cell and/or nerve damage, responses to acoustic stimuli occur not at the area of peak basilar membrane stimulation but instead occur at adjacent regions in the cochlea. Professor Brian Moore defined dead regions as a total loss of inner hair cell function across a limited region of the basilar membrane (Moore, et al., 1999b). This hair cell loss does not result in an inability to perceive sound at a given frequency range, rather the sound is perceived via off-place or off-frequency listening, a spread of excitation to adjacent regions in the cochlea where inner hair cells are still functioning (Moore, 2004). Because the response is spread across a broad tonotopic area, individuals with cochlear dead regions may perceive pure tones as “clicks”, “buzzes” or “whooshes”.
Cochlear dead regions are identified and measured by a variety of masking techniques. The most accurate method is the calculation of psychophysical tuning curves (PTCs), originally developed to measure frequency selectivity (Moore & Alcantara 2001). A PTC plots the level required to mask a stimulus frequency as a function of the masker frequency. For a normally hearing ear, the PTC peak will align with the point at which the stimulus can be masked by the lowest level masker. In ears with dead regions, the tip of the PTC is shifted off of the signal frequency to indicate that the signal is being detected in an adjacent region. Though PTCs are an effective method of identifying and delineating the edges of cochlear dead regions, they are time consuming and ill-suited to clinical use.
The test used most frequently for clinical identification of cochlear dead regions is the Threshold Equalizing Test (TEN; Moore et al., 2000; 2004). The TEN test was developed with the idea that tones detected by off-frequency listening, in ears with dead regions, should be easier to mask with broadband noise than they would in ears without dead regions. With the TEN (HL) test, masked thresholds are measured across the range of 500Hz to 4000Hz, allowing the approximate identification of a cochlear dead region.
There are currently no standards for clinical management of cochlear dead regions. Some reports suggest that affect speech, pitch, loudness perception, and general sound quality (Vickers et al., 2001; Baer et al., 2002; Mackersie et al., 2004; Huss et al., 2005a; 2005b). Some researchers have specified amplification characteristics to be used with patients with diagnosed dead regions, but there is no consensus and different studies have arrived at conflicting recommendations. While some recommend limiting amplification to a range up to 1.7 times the edge frequency of the dead region (Vickers et al., 2001; Baer et al., 2002), others advise the use of prescribed settings and recommend against limiting high frequency amplification (Cox et al., 2012; see link for a review). Because of these conflicting recommendations, it remains unclear how clinicians should modify their treatment plans, if at all, for hearing aid patients with dead regions.
Previous research on the prevalence of dead regions has reported widely varying results, possibly due to differences in test methodology or subject characteristics. In a study of hearing aid candidates, Cox et al. (2011) reported a dead region prevalence of 31%, but their strict inclusion criteria likely missed individuals with milder hearing losses, so their prevalence estimate may be different from that of hearing aid candidates at large. Vinay and Moore (2007) reported higher prevalence of 57% in a study that did include individuals with thresholds down to 15dB HL at some frequencies, but the median hearing loss of their subjects was higher than that of the Cox et al. study, which likely impacted the higher prevalence estimate in their subject group.
In the study being reviewed, Pepler and her colleagues aimed to determine how prevalent cochlear dead regions are among a population of individuals who have or are being assessed for hearing aids. Because dead regions become more likely as hearing loss increases, and established hearing aid patients are more likely to have greater degrees of hearing loss, they also investigated whether established hearing aid patients would be more likely to have dead regions than newly referred individuals. Finally, they studied whether age, gender, hearing thresholds or slope of hearing loss could predict the presence of cochlear dead regions.
The researchers gathered data from a group of 376 patients selected from the database of a hospital audiology clinic in Manchester, UK. Of the original group, 343 individuals met inclusion criteria; 193 were new referrals and 150 were established patients and experienced hearing aid users. Of the new referrals, 161 individuals were offered and accepted hearing aids, 16 were offered and declined hearing aids and 16 were not offered hearing aids because their losses were of mild degree. The 161 individuals who were fitted with new hearing aids were referred to as “new” hearing aid users for the purposes of the study. All subjects had normal middle ear function and otoscopic examinations and on average had moderate sensorineural hearing losses.
When reported as a proportion of the total subjects in the study, Pepler and her colleagues found dead region prevalence of 36%. When reported as the proportion of ears with dead regions, the prevalence was 26% indicating that some subjects had dead regions in one ear only. Follow-up analysis on 64 patients with unilateral dead regions revealed that the ears with dead regions had significantly greater audiometric thresholds than the ears without dead regions. Only 3% of study participants had dead regions extending across at three or more consecutive test frequencies. Ears with contiguous dead regions had greater hearing loss than those without. Among new hearing aid users, 33% had dead regions while the prevalence was 43% among experienced hearing aid users. On average, the experienced hearing aid users had poorer audiometric thresholds on average than new users.
Pepler and colleagues excluded hearing losses above 85dB HL because effective TEN masking could not be achieved. Therefore, dead regions were most common in hearing losses from 50 to 85dB HL, though a few were measured below that range. There were no measurable dead regions for hearing thresholds below 40dB HL. Ears with greater audiometric slopes were more likely to have dead regions, but further analysis revealed that only 4 kHz thresholds had a significant predictive contribution and the slopes of high-frequency hearing loss only predicted dead regions because of the increased degree of hearing loss at 4 kHz.
Demographically, more men than women had dead regions in at least one ear, but their audiometric configurations were different: women had poorer low frequency thresholds whereas men had poorer high frequency thresholds. It appears that the gender effect actually due to the difference in audiometric configuration, specifically the men’s poorer high frequency thresholds. A similar result was reported for the analysis of age effects. Older subjects had a higher prevalence of dead regions but also had significantly poorer hearing thresholds. Though poorer hearing thresholds at 4kHz did slightly increase the likelihood of dead regions, regression analysis of the variables of age, gender and hearing thresholds found that none of these factors were significant predictors.
Pepler et al’s prevalence data agree with the 31% reported by Cox et al (2012), but are lower than that reported by Vinay and Moore (2007), possibly because the subjects in the latter study had greater average hearing loss than those in the other studies. But when Pepler and her colleagues used similar inclusion criteria to the Cox study, they found a prevalence of 59%, much higher than the report by Cox and her colleagues and likely due to the exclusion of subjects with normal low frequency hearing in the Cox study. The authors proposed that Cox’s exclusion of subjects with normal low frequency thresholds could have reduced the overall prevalence by increasing the proportion of subjects with metabolic presbyacusis and eliminating some subjects with sensory presbyacusis—sensory presbyacusis is often associated with steeply sloping hearing loss and involves atrophy of cochlear structures (Shuknecht, 1964).
In summary:
The study reported here shows that roughly a third of established and newly referred hearing aid patients are likely to have at least one cochlear dead region, in at least one ear. A very low proportion (3% reported here) of individuals are likely to have dead regions spanning multiple octaves. The only factor that predicted the presence of dead regions was hearing threshold at 4 kHz.
On the lack of clinical guidance:
As more information is gained about prevalence and risk factors, what remains missing are clinical guidelines for management of hearing aid users with diagnosed high-frequency dead regions. Conflicting recommendations have been proposed for either limiting high frequency amplification or preserving high frequency amplification and working within prescribed targets. The data available today suggest that prevalence of contiguous multi-octave dead regions is very low and a further subset of hearing aid users with contiguous dead regions experience any negative effects of high-frequency amplification. With consideration to these observations, it seems prudent that the prescription of high-frequency gain should adhere to the prescribed targets for all patients at the initial fitting. Any reduction to high-frequency gains should be managed as a result of subjective feedback from the patient after they have completed a trial period with their hearing aids.
On frequency lowering and dead regions:
Some clarity is required regarding the role of frequency lowering and the treatment of cochlear dead regions. Because acoustic information in speech extends out to 10 kHz and because most hearing aid frequency responses roll off significantly after 4-5 kHz, the mild prescription of frequency lowering can be beneficial to many hearing aid users. It must be noted that the benefits of this technology arise largely from the acoustic limitations of the device and not the presence or absence of a cochlear dead region. There are presently no recommendations for the selection of frequency lowering parameters in cases of cochlear dead regions. In the absence of these recommendations, the best practice for the prescription of frequency lowering would follow the same guidelines as any other patient with hearing loss; validation and verification should be performed to document benefit with the algorithm and identify appropriate selection of algorithm parameters.
On the low-frequency dead region:
The effects of low-frequency dead regions are not well studied and may have more significant impact on hearing aid performance. Hornsby (2011) reported potential negative effects of low frequency amplification if it extends into the range of low-frequency dead regions (Vinay et al., 2007; 2008). In some cases performance decrements reached 30%, so the authors recommended using low-frequency gain limits of 0.57 times the low-frequency edge of the dead region in order to preserve speech recognition ability. Though dead regions are less common in the low frequencies than in the high frequencies, more study on this topic is needed to determine clinical testing and treatment implications.
References
Baer, T., Moore, B. C. and Kluk, K. (2002). Effects of low pass filtering on the intelligibility of speech in noise for people with and without dead regions at high frequencies. Journal of the Acoustical Society of America 112(3 Pt 1), 1133-44.
Cox, R., Alexander, G., Johnson, J., Rivera, I. (2011). Cochlear dead regions in typical hearing aid candidates: Prevalence and implications for use of high-frequency speech cues. Ear and Hearing 32(3), 339 – 348.
Cox, R.M., Johnson, J.A. & Alexander, G.C. (2012). Implications of high-frequency cochlear dead regions for fitting hearing aids to adults with mild to moderately severe hearing loss. Ear and Hearing 33(5), 573-87.
Hornsby, B. (2011) Dead regions and hearing aid fitting. Ask the Experts, Audiology Online October 3, 2011.
Huss, M. & Moore, B. (2005a). Dead regions and pitch perception. Journal of the Acoustical Society of America 117, 3841-3852.
Huss, M. & Moore, B. (2005b). Dead regions and noisiness of pure tones. International Journal of Audiology 44, 599-611.
Mackersie, C. L., Crocker, T. L. and Davis, R. A. (2004). Limiting high-frequency hearing aid gain in listeners with and without suspected cochlear dead regions. Journal of the American Academy of Audiology 15(7), 498-507.
Moore, B., Huss, M. & Vickers, D. (2000). A test for the diagnosis of dead regions in the cochlea. British Journal of Audiology 34, 205-224.
Moore, B. (2004). Dead regions in the cochlea: Conceptual foundations, diagnosis and clinical applications. Ear and Hearing 25, 98-116.
Moore, B. & Alcantara, J. (2001). The use of psychophysical tuning curves to explore dead regions in the cochlea. Ear and Hearing 22, 268-278.
Moore, B.C., Glasberg, B. & Vickers, D.A. (1999b). Further evaluation of a model of loudness perception applied to cochlear hearing loss. Journal of the Acoustical Society of America 106, 898-907.
Pepler, A., Munro, K., Lewis, K. & Kluk, K. (2014). Prevalence of Cochlear Dead Regions in New Referrals and Existing Adult Hearing Aid Users. Ear and Hearing 20(10), 1-11.
Schuknecht HF. Further observations on the pathology of presbycusis. Archives of Otolaryngology 1964;80:369—382
Vickers, D., Moore, B. & Baer, , T. (2001). Effects of low-pass filtering on the intelligibility of speech in quiet for people with and without dead regions at high frequencies. Journal of the Acoustical Society of America 110, 1164-1175.
Vinay and Moore, B. C. (2007). Speech recognition as a function of high-pass filter cutoff frequency for people with and without low-frequency cochlear dead regions. Journal of the Acoustical Society of America 122(1), 542-53.
Vinay, Baer, T. and Moore, B. C. (2008). Speech recognition in noise as a function of high pass-filter cutoff frequency for people with and without low-frequency cochlear dead regions. Journal of the Acoustical Society of America 123(2), 606-9.