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Hyperacusis and Williams syndrome.

Abnormal sensitivity to environmental sounds is a short-term feature often observed following insertion of grommets. Here we describe a child with this symptom who was found to have Williams syndrome, a condition in which hyperacusis is observed in 95 per cent of patients.

Abnormalities, Multiple↗

Carbamazepine in the treatment of Lyme disease-induced hyperacusis.

Lyme disease-induced hyperacusis can be an intensely disabling, chronic condition that is accompanied by posttraumatic stress disorder-like psychobehavioral sequelae. The authors describe effective treatment of 2 patients with carbamazepine. Speculations regarding a mode of action are offered.

Adult↗

Hyperacusis.

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Persons with Disabilities↗

Tinnitus Retraining Therapy (TRT) as a method for treatment of tinnitus and hyperacusis patients.

The aim of this paper is to provide information about the neurophysiologic model of tinnitus and Tinnitus Retraining Therapy (TRT). With this overview of the model and therapy, professionals may discern with this basic foundation of knowledge whether they wish to pursue learning and subsequently implement TRT in their practice. This paper provides an overview only and is insufficient for the implementation of TRT.

Hair Cells, Auditory↗

Hyperacusis.

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Hearing Disorders↗

Effects of (-)-baclofen, clonazepam, and diazepam on tone exposure-induced hyperexcitability of the inferior colliculus in the rat: possible therapeutic implications for pharmacological management of tinnitus and hyperacusis.

Recent investigations in the authors' laboratory have shown that acute tone exposure (4 kHz continuous tone, 104 dB sound pressure level (SPL), 30-min duration) induces increases in the amplitude of click-evoked potentials in the inferior colliculus (IC). These increases have been attributed to a decrease in GABAA-mediated inhibition on IC neurons. In the present study, we examined the effects of three compounds (diazepam, clonazepam, and (-)-baclofen) that are known to enhance GABAergic inhibition on these tone exposure-induced increases and on changes in temporal integration in the IC. (-)-Baclofen was the only one of the three compounds tested that reversed in a dose-dependent manner the effects of tone exposure on both the amplitude of the click-evoked potentials recorded from the IC and on measures of the changes in temporal integration based on these potentials. Diazepam and clonazepam exhibited remarkably different effects on the click-evoked potentials recorded from the surface of the IC. Diazepam caused a dose-dependent decrease in one of the components of the IC potentials that reflects postsynaptic activity in the IC, whereas clonazepam caused a dose-dependent decrease in a peak that reflects input to the IC from the superior olivary complex (SOC). At dosages up to 40 mg/kg, neither diazepam nor clonazepam reversed the changes in temporal integration in the IC that were induced by the tone exposure; diazepam caused a small, but statistically significant, enhancement of the effects of tone exposure on this function. The results of this study show that (-)-baclofen is a potent modulator of both the excitability of neurons in the ascending auditory pathway and the processing of auditory information by IC neurons. The finding of the present study that two benzodiazepines (clonazepam and diazepam) have remarkably different effects on evoked potentials, which reflects both input to the IC and postsynaptic events in the IC neurons, suggests heterogenicity of the GABAA receptor from one structure to another in the ascending auditory pathway. We suggest that (-)-baclofen may be clinically useful in treating disorders of the auditory system that are caused by plasticity in the ascending auditory pathway.

Acoustic Stimulation↗

[Use of auditory field measurement in patients with otosclerosis].

BACKGROUND: Middle ear surgery sometimes leads to unpleasant auditory impressions such as distortion or hyperacusis that cannot be detected by conventional audiometric testing. METHOD: Sixty-one patients with conductive hearing loss caused by otosclerosis underwent audiological evaluation, which included a questionnaire followed by testing of the audiometric threshold, speech audiometry, and assessment and quantification of loudness perception with a commercial system (WESTRA). This investigation includes the postoperative measurement of hearing improvement and the patients' subjective impressions regarding hearing increase, distortion of speech, and hyperacusis. RESULTS: Hearing improved in 88% of the patients. A quantification of this hearing increase was possible with pure tone audiometry and the Freiburg speech discrimination test. Reduced hearing threshold and lack of improvement in speech discrimination was confirmed by conventional hearing measurements. However, the presence of hyperacusis and distortion of speech could be determined by conventional audiometry in only 50% of cases. It was interesting to note that the subjects who reported speech distortion and hyperacusis in the questionnaire were identified by their increased loudness perception using the categorical loudness scaling. CONCLUSION: Category loudness scaling appears to be a valuable additional clinical test to characterize postoperative phenomena as distortion of speech and hyperacusis in patients undergoing stapes surgery.

Adult↗

Assessment of hearing and hearing disorders in rock/jazz musicians.

The aim of this study was to assess hearing and hearing disorders among rock/jazz musicians. One hundred and thirty-nine (43 women and 96 men) musicians participated. The results are based on pure-tone audiometry and questionnaire responses. According to our definition of hearing loss, tinnitus, hyperacusis, distortion and/or diplacusis as hearing disorders, we found disorders in 74%, of the rock/jazz musicians studied. Hearing loss, tinnitus and hyperacusis were most common, and the latter two were found significantly more frequently than in different reference populations. The women showed bilateral, significantly better hearing thresholds at 3-6 kHz than the men. Hyperacusis, and the combination of both hyperacusis and tinnitus, were found to be significantly more frequent among women than among men. Hearing loss and tinnitus were significantly more common among men than among women. It is important to evaluate all kinds of hearing problems (other than hearing loss) in musicians, since they represent an occupational group especially dependent on optimal, functional hearing. On the basis of our results, we suggest that hearing problems such as tinnitus, hyperacusis, distortion and/or diplacusis should, in addition to hearing loss, be defined as hearing disorders.

Acoustic Stimulation↗

Hypersensitivity to sound--questionnaire data, audiometry and classification.

This study included consecutive case histories and audiometry of 100 patients with hypersensitivity to sounds. There are several different conditions with the symptom of hypersensitivity to sounds. Hyperacusis is one of those and is seldom described in the literature. The term hyperacusis is often used synonymously with hypersensitivity to sound. We propose that there is a specific condition that could be termed hyperacusis. Hyperacusis is often elicited by loud sounds or by a number of other traumata or diseases. It is not typical of occupational noise exposure (with the exception of exposure to music). The typical patient is relatively young, the mean age being approximately 10 years less than for a population of patients with tinnitus or noise-induced hearing loss. In addition to hypersensitivity to sound, the patients often suffer from tinnitus (86%). Sounds are frequently painful and exposure to loud sounds worsens the condition for some time. The patients often have headaches. Pure tone audiograms show normal hearing or a slight high tone loss. The uncomfortable loudness level is markedly decreased, mostly less than 90 dB HL. Patients with hyperacusis may also be divided into those hypersensitive to the loudness of sounds with a decreased pure tone uncomfortable loudness level and those hypersensitive to certain specific sounds irrespective of loudness showing relatively high pure tone uncomfortable loudness levels and decreased uncomfortable loudness levels to specific sounds. With a careful history other conditions with the symptom of hypersensitivity to sound can be excluded.

Adolescent↗