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Assessment and amelioration of hyperacusis in tinnitus patients.

CONCLUSIONS: Tinnitus can be bothersome even without hyperacusis. The good correlation found in this study between the multiple-activity scale for hyperacusis (MASH) score and the overall annoyance of hyperacusis score supports the reliability of self-rating of hyperacusis. Objectives. A prospective study was undertaken to investigate the relationships between hyperacusis and tinnitus and to determine whether hyperacusis can be rapidly controlled over time. MATERIAL AND METHODS: All tinnitus patients seen over a period of 16 months with surnames beginning with the letters A L (n = 249) were assessed during a structured interview using (i) a scale ranging from 0 to 10 for assessing the annoyance of tinnitus and the overall annoyance of hyperacusis and (ii) the newly introduced MASH. RESULTS: The annoyance of tinnitus was greater in females. The prevalence of hyperacusis was high (79%). The annoyance of hyperacusis varied. Patients were categorized into five groups according to the hyperacusis annoyance score, as follows: no hyperacusis; mild hyperacusis (< or = 3); moderate hyperacusis (3.1-5.0); substantial hyperacusis (5.1-7.0); and severe hyperacusis (> or = 7.1). Individuals with severe hyperacusis were younger than those in the other groups. The correlation between the annoyance of tinnitus and the annoyance of hyperacusis was poor (r = 0.35). No audiometric difference was found between categories, whereas self-rated hearing deficiency increased with the annoyance of hyperacusis. A good correlation (r = 0.89) was found between the overall annoyance of hyperacusis and the MASH score. Changes over time, assessed in 32 patients investigated at least 3 times, showed more frequent improvement in hyperacusis (63%) than tinnitus (47%).

Adult↗

Relation of hyperacusis in sensorineural tinnitus patients with normal audiological assessment.

Hyperacusis is mainly a consequence of the noise level in the twenty-first century, owing to dramatic changes in people's lifestyles. Of every 100 people with otological complaints (e.g., tinnitus and hyperacusis), 20 are affected by hyperacusis. Because of its high incidence, this symptom has long been the subject of investigation. Nine patients (eight female, one male) with sensorineural tinnitus voluntarily participated in this study. Among the evaluated patients, three were between 25 and 40 years of age and six were between 41 and 60 years. The patients did not report a history of acoustic trauma, use of drugs, or otological diseases. The duration of complaints at the time the study began varied from 3 months to 8 years. The patients answered a questionnaire, submitted to an ear, nose, and throat examination, and were evaluated by pure-tone and speech audiometry and otoacoustic emissions and laboratory tests. In all patients, ear, nose, and throat assessments and audiological tests were within the normal ranges. Of the nine subjects in our study, 100% had tinnitus, and 89% (n = 8) had hyperacusis as an associated symptom. Six subjects (67%) had severe tinnitus; among them, four had moderate hyperacusis, one had severe hyperacusis, and one was not affected by hyperacusis. In two subjects (22%) with moderate tinnitus, one had moderate hyperacusis and the other referred severe symptoms. Another patient (11%) reported mild tinnitus and mild hyperacusis. No patient classified his or her tinnitus as disabling. The findings of the present study led us to conclude that the most affected age range of patients with tinnitus and hyperacusis was 41-60 years and that women are affected significantly more often than men. Tinnitus preceded hyperacusis as a complaint in 78% of the subjects. Hyperacusis was present in eight (89%) of the patients. There was no direct correlation between the severity of tinnitus and of hyperacusis, although we noticed that the discomfort of tinnitus was generally perceived as equal to or worse than that of hyperacusis.

Adult↗

Is central hyperacusis a symptom of 5-hydroxytryptamine (5-HT) dysfunction?

The subjective symptom of hyperacusis is described. The terms 'hyperacusis' and 'phonophobia' are considered to be synonymous as there is no recognized distinction between these two descriptions. Peripheral auditory pathologies with associated hearing sensitivity are reviewed and the likely mechanisms underlying the hyperacusis are listed. The neurological conditions, which have been reported to occur with hyperacusis, are reviewed. A separate aetiology of central hyperacusis is therefore proposed, with a symptom profile distinct from the peripheral hyperacusis. A common factor to neurological conditions with hyperacusis, is disturbance of 5-HT (5-hydroxytryptamine) or serotonin function. The research literature on the role of 5-HT in sensory modulation (specifically auditory startle) in animals is presented. It is proposed that 5-HT dysfunction is a probable cause of increased auditory sensitivity manifested as central hyperacusis or phonophobia.

Acoustic Stimulation↗

Fears, hyperacusis and musicality in Williams syndrome.

The study investigated the prevalence of fear and hyperacusis and the possible connections between fear, hyperacusis and musicality in a Swedish sample of individuals with Williams syndrome (WS). The study included 38 individuals and a cross-sectional design, with no matched control group. Two persons, who knew the participant well, completed a questionnaire. On reported fears, 58% of the participants scored higher than +2S.D., compared to a psychometric study. Thirteen percent scored above the suggested cut-off for hyperacusis, compared to 2.5% in a psychometric study. Female participants generally had higher reported fears and hyperacusis compared to male participants. There were also startling findings of correlations between reported fears and hyperacusis. This preliminary report supports a hypothesis that fears and anxiety could be associated with hyperacusis in the WS population. A hypothesis that musicality could serve as a protective factor and prevent anxiety, received no or very limited support. A hypothesis that hyperacusis could be connected to a general, readily arousal, tendency in the sympathetic nervous system and could be seen as vulnerability for psychopathology is discussed.

Adolescent↗

Hyperacusis in Williams syndrome: characteristics and associated neuroaudiologic abnormalities.

BACKGROUND: Hyperacusis and phonophobia are common, debilitating symptoms in Williams syndrome (WS), yet little is known about their underlying audiologic and neurologic processes. METHODS: The mothers of 49 subjects with WS were asked to complete the Hyperacusis Screening Questionnaire. Subjects with reported hyperacusis and sufficient developmental capacity underwent comprehensive audiological and brain auditory evoked response (BAER) testing. Findings were compared with those from pair-matched typically developing control subjects. RESULTS: Forty-one of the 49 children with WS (84%) had hyperacusis of moderate to severe degree, which began in infancy. Of these, 21 (mean age 15.8 +/- 5.5 years) were quantitatively tested. Subjects with WS reported discomfort at sound intensities on average 20 dB lower than control subjects. Pure-tone audiometry and distortion products otoacoustic emission test revealed a high-frequency cochlear hearing loss. An absence of ipsilateral acoustic reflex responses to maximum stimulation was significantly more common in the subjects with WS than controls. On BAER testing, the WS group had a significant prolongation in wave I latency. CONCLUSIONS: Hyperacusis in Williams syndrome (WS) is associated with a high-frequency hearing loss resembling the configuration of noise-induced hearing loss. The hyperacusis and hearing loss in WS may stem from a deficiency in the acoustic reflex resulting from auditory nerve dysfunction. Additional mechanisms that may mediate hyperacusis in WS and should be evaluated in future studies include recruitment, malformation of the facial canal, and haploinsufficiency of the elastin gene.

Acoustic Stimulation↗

[Study of hyperacusis at a tinnitus unit].

OBJECTIVES: Description of the epidemiological and clinical characteristics of hyperacusis in a tinnitus clinic unit. STUDY DESIGN: Transversal descriptive. PATIENTS: Two hundred and fifty patients referred to our Tinnitus Unit a Teaching Hospital, between January 1999 and December 2000. OUTCOME MEASURES: Questionnaires to evaluate hyperacusis and tinnitus interference in life quality. Epidemiological and audiological data as well as tinnitus characteristics in hyperacustic patients are reported. RESULTS: Sixty three percent of the patients showed hyperacusis according to sound threshold level. Hearing loss was present in 83%. Discomfort with background noise was referred in 54% of cases and 52% avoided at least one daily activity because of this discomfort. Tinnitus in hyperacusis population showed a disability level of 47% using the Tinnitus Handicap Inventory (THI). CONCLUSIONS: A physiopathological relation between tinnitus and hyperacusis could be explained by the high prevalence of both symptoms in the same population. It is necessary to agree in the criteria to define hyperacusis as well as for the evaluation of its effect in quality of life. The tinnitus retraining therapy (TRT) is the more accepted hyperacusis treatment approach in the literature.

Adult↗

Tinnitus - Hyperacusis and the Loudness Discomfort Level Test - A Preliminary Report.

This paper is a preliminary report from the Tinnitus Center, HSCB-SUNY, of hyperacusis in a patient population who request consultation for tinnitus of the severe disabling type. Forty-two consecutive patients seen from January to August 1995 were reviewed for this preliminary report. There is a positive correlation between tinnitus and hyperacusis as well as a positive correlation between hyperacusis and the loudness discomfort level test (LDL). Hyperacusis is an increased sensitivity to sound that occurs with/without a hearing loss in individuals with tinnitus of the severe disabling type. The present method of assessment for hyperacusis includes pure tone audiometry, LDL's, Feldmann Masking Curves and the Metz test for recruitment. A classification system exists for hearing loss and a similar system is suggested for hyperacusis.

Journal Article↗

[Assessment of hyperacusis: the self-rating questionnaire on hypersensitivity to sound].

BACKGROUND: Hyperacusis is a decreased sound tolerance. The audiological examination includes the loudness discomfort level measurement and the handicap evaluation, so we introduce the Spanish validation of the german sound intolerance questionnaire. OBJECTIVES: To update the concept of hyperacusis and to evaluate its handicap through a Spanish validation of the Geräuschüberempfindlichkeit (GUF). PATIENTS: Forty patients referred to our Tinnitus and Hyperacusis Clinic in the University Hospital, between October 2004 and February 2005. OUTCOME MEASURES: The Spanish version of the GUF was performed after transla-tion and retro-translation. Internal consistency and reliability were established. RESULTS: Spanish adaptation of the GUF and its subscales (cognitive, somatic behaviour and emotional reaction) showed a high reliability and internal consistency (Cronbach's alfa: 0.9007). Higher GUF scores were statistically demonstrated in hyperacusis patients with hearing loss (p < 0.05) or tinnitus (p < 0.05). CONCLUSIONS: Spanish adaptation of the GUF is valid, reliable and can be used in a clinical setting to quantify the impact of hyperacusis on patient's quality of life.

Adult↗

[Therapy of hyperacusis in patients with tinnitus].

BACKGROUND: Patients with tinnitus very often suffer from hyperacusis also. METHODS AND PATIENTS: In the present study a possible treatment for patients with bilateral hyperacusis is introduced and the therapeutical results of 41 patients suffering from bilateral hyperacusis combined with a uni- or bilateral tinnitus are discussed. Twenty-one of them also had a cochlear hearing loss. By means of white noise generators or hearing aids and a specific acoustic training the auditory input to the central nervous system was increased. All patients were taught a method to reduce stress (Progressive muscle relaxation according to Jacobson). Uncomfortable loudness levels were analysed before as well as 3 weeks and 6 months after beginning of the treatment. RESULTS: By the time of 3 weeks the discomfort from ordinary sounds was distinctly reduced and the reduction progressed further after 6 months of treatment. In more than 50% of the participants the uncomfortable loudness levels could be raised to a normal range. Interestingly the improvement of hyperacusis was independent of an existing hearing loss. CONCLUSIONS: The described method of treatment seems to be helpful for patients with hyperacusis and will be compared to other therapeutical regimes.

Acoustics↗

Psychometric normalization of a hyperacusis questionnaire.

Clinical hyperacusis consists of a marked intolerance to ordinary environmental sounds, while hearing thresholds are quite often normal. Hyperacusis appears to be a subjective phenomenon, which is not easily defined or quantified by objective measurements. In order to create a tool suitable to quantify and evaluate various hyperacusis symptoms, a questionnaire screening several aspects of auditory symptomatology has been constructed. Two hundred and one subjects (who were either hyperacusic or not), randomly selected from the general population, were tested. A principal component analysis performed on the correlation matrix of the 14 items of the questionnaire isolated three dimensions: attentional, social, and emotional. The three dimensions had satisfactory internal consistency reliability. The mean +/- SD total score was 15 +/- 6.7 out of 42 (maximum of hyperacusis) and a score greater than 28 seems to represent a strong auditory hypersensitivity. This new psychometric tool should further be tested on hyperacusic patients to verify its relevance in pathology and define the involvement of the three dimensions statistically obtained on the hyperacusis symptomatology.

Adolescent↗

Hyperacusis in Williams syndrome: a sample survey study.

Williams syndrome is a true multiple congenital anomalies mental retardation syndrome affecting the vascular, connective tissue and the central nervous system. Affected individuals have a distinctive neuropsychological profile characterized by extremely poor visuospatial skills but relatively preserved verbal skills. A very striking characteristic is the hyperacusis or over-sensitivity to particular sounds. Klein et al. (4) found high rates (95%) of auditory over-sensitivity in a sample of Williams patients. The cause and mechanisms of auditory-over-sensitivity in Williams syndrome remain unclear. Some association has been suggested between hyperacusis and the occurrence of otitis media and also between hyperacusis and hyperactivity. The present study reports the results of an investigation into the occurrence of hyperacusis, otitis media and hyperactivity in a large group (N = 82) of Dutch speaking subjects with Williams syndrome from Belgium and The Netherlands. Prevalence and characteristics of hyperacusis and co-occurrence with otitis media and hyperactivity will be discussed and some management strategies are offered.

Adolescent↗

Treatment of hyperacusis in Williams syndrome with bilateral conductive hearing loss.

Williams syndrome (WS) is a rather rare congenital disorder characterised by a series of cardiovascular, maxillo-facial and skeletal abnormalities. It sometimes displays otorhinolaryngological symptoms because of the relatively high incidence of secretory otitis media and hyperacusis, which may be present in up to 95% of patients. The present paper describes a case of WS associated with bilateral conductive hearing loss which was not related to secretory otitis media. Hyperacusis was, moreover, present in spite of the conductive deafness. Surgical or prosthetic treatment of hearing loss was delayed because of hyperacusis. Treatment of the hyperacusis by acoustic training, instead, yielded excellent, long-lasting remission of the symptoms.

Acoustic Stimulation↗

[Mechanisms and management of hyperacusis (decreased sound tolerance)].

Hyperacusis is a decreased sound tolerance. Prevalence of the disease is described in 9-15% of the population, but this percentage increases among the tinnitus patients. Pathophysiological mechanisms involve some disruptions in the amplification and regulation processes of the external hair cells or affect the central sound processing at the subcortical level. The role of the serotonin, also involved in other diseases related with hyperacusis (migraine, depression), can be crucial in this disorder. Other theories confirm the effect of the endorphins that activate the excitatory function of the glutamate, the auditory neurotransmitter, increasing its toxicity. The activation of the limbic and autonomic nervous systems produces the emotional reaction of the hyperacusis (anxiety, fear and depression). Proposed treatments are based on acoustic stimulation by a progressive introduction of sound (tinnitus retraining therapy TRT). Noise generators and hearing aids can be fitted in severe cases. The role of some drugs involved in the metabolism of the serotonin open new approaches for the management of hyperacusis.

Humans↗

Celebrating a decade of evaluation and treatment: the University of Maryland Tinnitus & Hyperacusis Center.

The University of Maryland Tinnitus & Hyperacusis Center in Baltimore was the first center in the United States dedicated to the evaluation and treatment of tinnitus and hyperacusis patients implementing an habituation-based protocol that has become known internationally as Tinnitus Retraining Therapy (TRT). A crucial feature of the model is the postulate that a number of systems in the brain are involved in the emergence of tinnitus. The cochlea and auditory periphery play only a secondary role. To facilitate the goal of habituation of the tinnitus signal, TRT implements both directive counseling to neutralize the negative emotional associations toward the tinnitus, and sound therapy to interfere with the signal. As an outgrowth of the work with tinnitus, the evaluation and treatment of hyperacusis has emerged as an increasingly important part of our program. This report describes the unique facility, staff, and services of the Center as we celebrate a decade of research and clinical management dedicated to the scientific understanding of tinnitus and hyperacusis.

Acoustic Stimulation↗

Hyperacusis: review and clinical guidelines.

OBJECTIVE: To review the current known causes of hyperacusis and the different hypotheses concerning its etiology, and to suggest clinical guidelines. DATA SOURCE: A review of the literature with the aid of the MEDLINE database, using the following key words: hyperacusis, intolerance to sound, loudness discomfort level, and phonophobia. DATA EXTRACTION: The data collected included clinical studies, case reports and laboratory studies. CONCLUSION: Hyperacusis was shown to be caused by pathologic conditions of the peripheral auditory system, diseases of the central nervous system diseases, and hormonal and infectious diseases. In some cases there was no known cause. The pathophysiology of hyperacusis probably involves a central mechanism rather than a peripheral one. Suggested clinical guidelines and treatment are discussed.

Auditory Pathways↗

Hyperacusis in Williams syndrome.

OBJECTIVE: To define hyperacusis in audiologic parameters and to further elucidate central and peripheral auditory pathways. DESIGN AND SETTING: Theories surrounding hyperacusis have always been highly debated. A group of children with Williams syndrome universally complain of hyperacusis. They have highly reproducible behavioural responses to noise and are thus hampered in their social interactions. Loss of inhibitory modulation to efferent sensory input to the cochlea is thought to be a possible mechanism. METHODS: Nine patients with Williams syndrome received a complete audiologic work-up, including audiogram, speech reception thresholds, acoustic reflexes, impedance, and transient evoked otoacoustic emissions (TEOAEs). MAIN OUTCOME MEASURES: Assessment of the efferent system is done by measuring changes in TEOAEs following stimulation of the contralateral ear. RESULTS: Three patients had high-frequency sensorineural hearing loss (SNHL) and thus, as expected, absent TEOAEs, indicating cochlear damage. Two had normal hearing and normal TEOAEs. However, four patients had normal hearing with absent TEOAEs. CONCLUSIONS: These findings are suggestive of cochlear disease and may, in fact, support the hypothesis of outer hair cell modulation by the ipsilateral medial olivocochlear system. Behavioural aspects of the syndrome make audiologic testing difficult. Thus, the diagnosis of SNHL may be hampered if it truly exists. The data show a preponderance of SNHL in the older age groups of our study population. This either reflects previously missed diagnoses or underlying cochlear disease, which may manifest later in life. Thus, this finding blurs the boundary between loudness recruitment and hyperacusis.

Adolescent↗

Hypersensitivity to sound (hyperacusis): a prevalence study conducted via the Internet and post.

The present study explored the prevalence and descriptive characteristics of hyperacusis, defined as unusual intolerance of ordinary environmental sounds. Two ways of collecting data were used in the study: the first was a postal survey of a random sample, to which 589 responded (59.7% response rate); the second was the internet, and 595 self-recruited individuals responded to a call for participants via a banner on a web page (51.9% response rate). The point prevalence rates of hyperacusis were 9% in the internet group and 8% in the postal group. Exclusion of participants who reported hearing impairment resulted in point prevalence rates of 7.7% (n = 39) and 5.9% (n = 28) respectively. The data collection format did not result in any substantial differences. Hyperacusis was associated with concentration difficulties, use of ear protection, avoidance, tension, and sensitivity to light/colours. The present data suggest that hyperacusis is a common problem.

Adolescent↗

Hyperacusis and otitis media in individuals with Williams syndrome.

Williams syndrome is characterized by cardiac defects, varying degrees of physical and developmental delay, stellate eye pattern, possible elevated serum calcium level, and elfin/pixie facial features. A problem perhaps unique to these children is hyperacusis that can be severe enough to disrupt many routine daily activities. Parental questionnaires were used to determine the prevalence of hyperacusis and otitis media in individuals with Williams syndrome. Prevalences of 95% for hyperacusis and 61% for otitis media were found. This was significantly higher than in the general population. Despite the prevalence of hyperacusis, parents of these children were not counseled about management of the problem. The audiologist may become involved with Williams syndrome patients through hearing assessment and management, parental counseling, and research.

Adolescent↗