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|Year : 2003 | Volume
| Issue : 20 | Page : 55--62
Is there an association between noise exposure and King Kopetzky Syndrome?
D Stephens1, F Zhao2, V Kennedy3,
1 Welsh Hearing Institute, University Hospital of Wales, Cardiff, Wales; Department of Health Studies, University of Wales College Swansea, United Kingdom
2 Department of Health Studies, University of Wales College Swansea, United Kingdom
3 Welsh Hearing Institute, University Hospital of Wales, Cardiff, Wales, United Kingdom
Welsh Hearing Institute, University Hospital of Wales, Cardiff CF14 4XW, Wales
The condition in which individuals with normal pure tone audiograms complain of hearing difficulties, especially in the presence of background noise, (normal pure tone audiograms), has had a number of different names. The present term King-Kopetzky Syndrome was coined by Hinchcliffe in 1992. This is a common condition reported in 5 - 10% of those attending clinics complaining of hearing problems. A dominant genetic aetiology has been found in a proportion of cases.
It may be associated with minor peripheral or central auditory dysfunction, and frequently the individuals exhibit anxious or depressive personalities.
We found no relationship with noise exposure in a series of patients compared with matched controls. Here we review the evidence for and against such an influence and present fresh data in an attempt to define the role of noise, if any, in the causation of this condition. Our final conclusion is that there is no clear association between KKS and noise exposure
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Stephens D, Zhao F, Kennedy V. Is there an association between noise exposure and King Kopetzky Syndrome?.Noise Health 2003;5:55-62
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Stephens D, Zhao F, Kennedy V. Is there an association between noise exposure and King Kopetzky Syndrome?. Noise Health [serial online] 2003 [cited 2023 Feb 3 ];5:55-62
Available from: https://www.noiseandhealth.org/text.asp?2003/5/20/55/31688
King Kopetzky Syndrome (KKS) is the condition in which an individual complains of hearing difficulties, particularly in the presence of background noise, but has a normal pure tone audiogram. Hinchcliffe (1992) described it as an "auditory stress disorder" in the paper in which he coined the term King-Kopetzky Syndrome. He took the names of the individuals who first described the condition (Kopetzky, 1948) and who first discussed the aetiological factors behind it (King, 1954).
It has been called a by a variety of names by different authors the most common being Obscure Auditory Dysfunction - OAD (Saunders and Haggard, 1989) and Auditory Disability with Normal Hearing - ADN (Stephens and Rendell, 1988)
KKS is a relatively common condition. Rappaport et al (1993) reported it in 0.75% of patients complaining of hearing problems seen in an otolaryngological clinic. Higson et al (1994) reported a prevalence of 5% in patients complaining of hearing problems in an audiological clinic. In an investigation of a consecutive series of patients referred to the Audiological Medicine Clinic in the Welsh Hearing Institute with hearing difficulties, 8.4% of the patients seen were found to have the condition. Their age distribution is shown in [Figure 1] in comparison with that of the patients with significant hearing impairment. The median age of the patients seen with hearing impairment was 78 years, whereas that of the KKS group was 41years. It is notable that the prevalence of KKS in the patients below the age of 50 was 37%.
KKS is a heterogeneous condition and it origins have been discussed by Saunders and Haggard (1989), Hinchcliffe (1992) and by Zhao and Stephens (2000). These are summarised in [Table 1] and, generally speaking, a combination of minor cochlear dysfunction and stress related or psychological causes occur most frequently. In addition the high prevalence of a family history of hearing loss in the condition has been noted by Saunders and Haggard (1989), King and Stephens (1992) and by Zhao and Stephens (1999). Most recently we have argued (Stephens and Zhao, 2000), particularly on the basis of the mid-frequency notches found on sensitised audiometric testing (Audioscan - Meyer Bisch, 1996), that it may frequently represent the early manifestation of a late-onset dominant genetic disorder.
The role of noise exposure in the possible causation of KKS is more problematical and was first raised by the early work of Pick and Evans (1983).
Within the present investigation, in two studies, we shall consider further evidence for noise exposure in the aetiology of KKS in comparison with a control group of subjects and also seek to determine whether having a history of noise exposure influences the severity of the symptomatology reported in the condition.
In this study we compared a range of aetiological factors in a prospective study on a consecutive series of 110 patients with KKS and 70 controls, to determine whether those with KKS were more likely to have a history of noise exposure and to have notches on audioscan testing.
110 consecutive patients with KKS were tested. Their mean age was 33.4 years (SD 9.3 years) and their ages ranged from 13 to 55 years. 50 were males and 60 females. All had sought help for their hearing difficulties, especially hearing speech in a noisy environment. They all had hearing better than or equal to 20dB HL at each octave frequency between 500 an 4000 Hz with no hearing level from 250 - 8kHz exceeding 30 dBHL. They had no obvious cause for their hearing loss such as CNS pathology, ototoxic drug intake or overt middle ear disease.
70 control subjects were selected from students, staff and friends of the hospital staff. All had normal hearing by the same definitions. They comprised one male and one female of each year of age in years from 16 - 50. Their mean age was 33.0 years (SD 10.2 years). Their age and sex distribution did not differ significantly from the KKS patients.
The aetiological factors questionnaire shown in appendix 1 was completed by all the KKS patients and the control subjects.
This was performed using the Essilor Audioscan (Meyer Bisch, 1996; Zhao and Stephens, 1999).
The test parameters used were as follows: Frequency range 250 -8000 Hz; Starting level
-5dBHL; Sweep rate 30 s/octave; Start side
- right; Stimulus style - pulsed tone; Step size 5dB
Before starting the test, all subjects were trained to listen for the test tones in a run of the test procedure. This was done by stopping and restarting the test tones after the subject had responded, to ensure that s/he fully understood the procedure.
The definition of notches in the audiogram followed the procedure described by Laroche and Hetu (1997) with a notch depth of 15dB or greater compared with the surrounding frequencies taken as indication a significant notch.
[Table 2] shows the proportions of individuals in the KKS and control groups reporting a history of ear infections, noise exposure, having been knocked unconscious or a family history of hearing problems. It may be seen that while there was a significant difference in the proportions reporting a history of middle ear infection and of a family history of hearing problems, there was no significant difference in the proportion reporting a history of noise exposure (X 2 = 3.7; P > 0.05).
We next investigated the relationship between noise exposure and Audioscan notches in the frequency bands 500 - 3000 Hz and 3001 - 8000 Hz. This analysis showed that. 49% of patients with KKS had a notch in the 500 - 3000 Hz band and 34% had a notch in the 3001 - 8000 Hz band. Overall 26% of the KKS group had a history of noise exposure.
Of those with notches in the 500 - 3000 Hz band, 18% had a history of noise exposure. However, 56% of those with notches in the 3001 -8000 Hz band had a history of noise exposure. Furthermore, of those with no notches, only 11% had a history of noise exposure. Chi Square analysis showed that there was a significant difference between the high frequency notch and no notch groups, with there being a greater likelihood of a history of noise exposure in those with high frequency notches than in those without notches (x 2 =22.4; 1df, P 2 = 27; 1df; NS).
The analysis was next extended to the control group. 23% had notches in the 3001 - 8000 Hz band and 16% in the 500 - 3000 Hz band. Of those with high frequency notches, 50% had a history of noise exposure compared with 4% of those without notches in that band (x 2 = 21.6; 1df;p Study 2
In this study we examined the effect of a history of noise exposure on performance measures and Audioscan notches in a consecutive clinical series of 113 patients with KKS to determine whether such noise exposure influenced the symptoms and performance in such patients.
There were 44 (39%) males and 69 (61%) females in the present study. Their median age was 34 years, range 7 to 70 years, interquartile range 26 to 42 years. All had sought help, resulting in a hospital referral for their problems, which entailed primarily difficulties hearing speech in noisy environments. They all had hearing better than or equal to 20dBHL at each octave frequency between 500 and 4000 Hz with no hearing level from 250 - 8kHz exceeding 30 dBHL. They had no obvious cause for their hearing loss such as CNS pathology, ototoxic drug intake or conductive pathology.
Fifty nine subjects (52.2%) reported no significant noise exposure and fifty four (47.8%) some exposure to either social noise, occupational noise or gunfire, or combination of these.
Individuals' exposure to noise was assessed from their history in terms of occupational noise exposure, social noise exposure and acoustic trauma. Each of these was assessed on a scale from 0 - 3, equivalent to no exposure, mild, moderate or severe exposure, for each individual. The basis of the categorisation is shown on [Table 3].
As part of our clinical protocol, all patients were questioned specifically about their exposure to occupational noise, social noise and acoustic trauma. Those with any relevant exposure were graded on a 3-point scale for each. The gradings are shown in [Table 3]. A composite score was also obtained by summing the scores on the three scales.
In the interview, the subjects were asked to rate their Activity Limitations and Participation restrictions on a visual analogue scale from 0 -100, using the technique described by Stephens & Zhao (2002) based on that presented earlier by Habib and Hinchcliffe (1978). The wording was as follows:
"Please rate the difficulty you have with you hearing on a scale between 0 and 100. Someone with perfect hearing would have a score of 0 and someone unable to hear at all a score of 100".
"Please rate the effect of your hearing loss on your life. If it had no effect, the score would be 0, if it completely altered your life it would be 100".
Finally the subjects were asked to complete the Social hearing Handicap Index (Ewertsen & Birk Nielsen, 1973), a 21 question scale of speech hearing activity limitation, which we have found to be a useful measure in this group of patients (Zhao & Stephens, 1996).
The subjects were tested using a sentence in noise test (BKB sentences - Bench & Bamford, 1979) presented at two signal to noise ratios ( 0 and -5dB). A uniform level for the speech peaks of 60 dB SPL was used for all subjects. The test was carried out in a sound-treated room with prerecorded material using a GST 16 audiometer, Technics amplifier, Mission speaker and Sony Betamax Stereo Hi Fi Video player equipment.
The distribution of summed levels of noise exposure is shown in [Figure 3]. For each of the individual scales 21.2%, 23.0% and 15.9% reported significant exposure to Occupational Noise, Social Noise and Acoustic Trauma respectively.
The median rating of Activity Limitation was 20 (range 1-67; IQ range 10-30) and of Participation restriction 20 (range 0-90; IQ range10-40 ). The median score for the SHHI was 21 (range 2-39; IQ range 14-27).
The median BKB score at a signal to noise ratio of 0 was 74 (range 24-94; IQ range 64-81).
For the S/N ratio of -5 the median score was 54 (range 8 -92; IQ range 39-69).
Significant Notches in the Audioscan in the Right ear were found in 39.8% of the patients (11.6% up to and including 3 kHz and 28.2% over 3kHz). In the left ear, notches were found in 38.9% of subjects (13.5% up to and including 3kHz and 23.4% over 3kHz. Where notches were found in both ears, their frequencies correlated significantly with each other (r = 0.73, P Relationship between noise and performance measures
[Figure 4] shows the relationship between the presence or absence of noise exposure and the presence of low or high frequency notches in the right ear. It may be seen that low frequency notches are predominantly associated with no noise exposure, whereas high frequency noises are associated with noise exposure (x 2 = 6.2; 2 df; P3kHz) versus performance measures showed a significant relationship between notch type in the left ear and speech in noise recognition at a S/N ratio of -5 indicating that those with a notch at a frequency of up to 3kHz performed at less well than those with no notch (t = 2.56; P = 0.013) or with a high frequency notch (t = 3.00; P3 kHz). Our studies support other evidence (eg Anderson and Wedenberg, 1964: Stephens and Zhao, 2000) that the mid frequency notches are more likely to be related to genetic aetiologies, and those at the high frequencies more likely to be related to noise exposure.
The one area in which noise did appear to have some impact was on self rated participation restriction. It is interesting that such participation restriction correlated better with reported occupational noise exposure than with overall noise exposure, and did not correlate at all with social noise exposure nor acoustic trauma (tau = 0.007 and 0.005 respectively). This suggests that the impact of noise on this measure could be coloured by the individual's feeling of resentment of the effect of their workplace noise.
Overall we have found that KKS is a common condition and is likely to represent a stress related reaction to a minor deficit. This deficit could well be caused by noise exposure but that is only one of a number of possible causes and there is no clear relationship between the two conditions.
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