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Clinical Interventions for Hyperacusis in Adults: A Scoping Review to Assess the Current Position and Determine Priorities for Research.

1. Introduction

Hyperacusis is the perception of everyday environmental sound as being overwhelmingly loud or intense. Other terminology in use includes reduced, decreased, or collapsed sound tolerance. It differs from phonophobia which is an episodic sound intolerance experienced by some people during migraine attacks, sometimes associated with other sensory sensitivities, and which abates as the attack recedes [1]. It can also be differentiated from misophonia which is an acquired aversive reaction to specific human generated sounds such as eating sound or breathing, the response being characterised by anger and sometimes rage [2, 3].

As with other subjective symptoms, data on the prevalence Of hyperacusis is strongly influenced by how the enquiry about the symptom was worded. In children and adolescents, the variability in wording of the enquiry in prevalence studies has been found to be so great as to render comparison across studies to be meaningless [4]. In a study of 7096 11-yearold children in the UK, 3.7% answered affirmatively to the question "do you ever experience oversensitivity or distress to particular sounds?" [5]. In adults, variability of the questions asked across studies of hyperacusis also significantly impacts on the ability to identify prevalence figures. Using the question "Do you consider yourself sensitive to everyday sounds?," Andersson and colleagues [6] found that of 1,174 adults that answered the question via either the Internet or post, 8.6% (95% CI = 7.0-10.0) responded affirmatively. Another Swedish study [7] in adults asked 3406 participants, "Do you have a hard time tolerating everyday sounds that you believe most other people can tolerate?" and found 9.2% responded affirmatively, with 1.9% reporting that they had been diagnosed with hyperacusis by a physician.

There is an apparent association between hyperacusis and tinnitus, with 86% of adult patients with a primary complaint of hyperacusis experience tinnitus [8] and 40% of patients with a primary complaint of tinnitus experience hyperacusis as well [9]. Many people with troublesome hyperacusis have normal or age appropriate hearing thresholds, but cochlear hearing loss has also been reported [10]. Medical conditions associated with hyperacusis include closed head injury, depression, posttraumatic stress syndrome, Williams syndrome, and pain syndromes such as fibromyalgia [1]. The physiological mechanisms that underpin hyperacusis are not well understood, and there is no compelling animal model. A consensus is emerging in the auditory neuroscience literature that hyperacusis may be associated with a sustained and persistent increase in central auditory gain [11].

A framework with which to categorise patients with hyperacusis has been proposed [12]. The subtypes consisted of hyperacusis that is characterised by loudness, annoyance, fear, or pain. This schema was not based upon empirical data and may serve to illustrate the various characteristics that can define the lived experience of hyperacusis, rather than to support diagnosis or treatment. Regardless, hyperacusis is a complex symptom which can have negative effects on daily functioning such as hearing, sleep, concentration, and emotional well-being that can vary daily and between individuals. Management, therefore, can be complex with approaches taken including cognitive behaviour therapy, Tinnitus Retraining Therapy, or sound therapy [13]. Complex interventions such as these need to be developed and evaluated in a systematic way to have confidence in the effectiveness of the intervention for the given population [14].

The purpose of this scoping review is to identify research gaps in existing literature and elements that should underpin the design of any new studies. The aim of this scoping review is to consider the current position of research on hyperacusis in adults. Specifically, the objective here is to identify

(i) how hyperacusis is currently defined in research studies,

(ii) How it is currently measured (i.e., what measures are used for diagnosis and outcome and are they adequate?),

(iii) What the level of evidence is for current management options (i.e., what has been previously studied and to what extent?).

2. Materials and Methods

This review is reported according to the methodological framework developed by Arksey and O'Malley [15] using the six-stage process. In this process (1) the purpose and research questions were defined, (2) relevant studies were identified, (3) studies were selected using an iterative approach through title, abstract, and full-text screening by three members of team (KF, IP, and GSS), (4) data were extracted and charted by two members of the team (KF, IP), (5) the results were collated, summarised, and reported, and (6) two clinical experts, who were not involved in the data extraction or collating and summarising results, were consulted and reviewed the findings.

2.1. Eligibility Criteria. To be included, records were required to report studies in which adults ([greater than or equal to] 18 years old) reported hyperacusis as a primary complaint or secondary complaint or as part of a symptom set. Records were included where management strategies (i.e., interventions) were tested to address hyperacusis. Records were eligible from Randomised Controlled Trials (RCTs), nonrandomised control trials, cohort studies, case series, and case studies. Review articles including systematic reviews, epidemiology articles, and any sources reporting personal/expert opinions were excluded. No records were excluded based on controls used, outcomes reached, timing, setting, or study design. Records were excluded for studies reporting misophonia, phonophobia, and loudness recruitment.

All included records were published in the English language. Where multiple eligible unique records pertaining to a single trial were identified, the record that was published first was included and any secondary analyses of the data were excluded.

2.2. Search Strategy. The search strategy followed a recommended three-step approach [16, 17]. In Step 1, to test keywords and search terms, a limited search in PsycINFO and Embase was conducted, checking the availability of relevant titles and abstracts. This allowed us to develop search term combinations to use across a wider search in step two. The search strategy included hyperacusis and terms for identifying research studies, such as intervention, therapy, treatment, management, assessment, outcome, and diagnoses (diagnostic) with narrow terms such as measure and test (Table 1). In Step 2, to identify relevant research studies, electronic databases of peer reviewed journals were searched in the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; Embase; PsycINFO; Scopus; Cumulative Index to Nursing and Allied Health Literature (CINAHL); Web of Science; the International Standard Randomised Controlled Trial Number (ISRCTN) registry;; the International Clinical Trials Registry Platform (ICTRP); and Google Scholar. Specific search term strategies were applied in each search engine, searching article topics, titles, abstracts, and keywords. Where possible, filters were applied to retrieve articles in the English language and using human participants only. There was no restriction in the search period as we wanted to include all available research up until the present time.

In Step 3, to seek further eligible documents for inclusion, we performed manual searches of the reference lists of any relevant review articles which had hyperacusis in the title.

In addition, manual searches of the most common journals (determined using the interquartile rule for outliers) in which eligible records had been sourced were conducted. The final manual search was conducted in April 2017.

2.3. Study Selection. Articles identified through electronic and manual searches were exported with citations, title, and abstract into Endnote where duplicates were removed. Search records were screened independently by two researchers out of a team of three (KF, IP, and GSS), first screening by title and abstract and then by full text. When disagreements regarding the inclusion or exclusion of any given record arose, the two researchers discussed their rationale until agreement was reached or a third researcher (DH) was consulted to reach a majority decision.

2.4. Data Extraction. A data extraction form was developed and piloted on two included records and was subsequently modified following team discussions. Data from each article were extracted by two researchers (KF and IP). Data were extracted on study characteristics, definition of hyperacusis, assessment measures used, and interventions (Box 1).

3. Results

3.1. Study Selection. Figure 1 illustrates the flow of study records identified, screened, included, and excluded (with reasons for exclusion). Electronic searches yielded an initial set of 1708 records. Duplicates were removed and of the remaining 792 records, 710 were excluded because the title and abstract indicated that the article did not fit our eligibility criteria. Most commonly the studies excluded did not focus on hyperacusis or did not report treatment of hyperacusis. Manual searches of included records in review articles identified a further 27 potential articles which were subjected to full-text screening. Manual searches in the selected journals identified one additional eligible record. Sixty-seven records were excluded at the full-text screening stage. Commonly, this was because the record did not report on the treatment or management of hyperacusis or was based on expert opinion or a review of the literature. For 1 record the reference was incomplete and could not be found. Full-text records could not be located for a further 2 records. None of these records could be traced, regardless of support from the University of Nottingham librarian. The electronic and manual searches created a final list of 43 eligible full-text records for data collection.

3.2. Study Characteristics. Of the 43 included full-text articles, there were 34 journal articles, six conference papers, and three book chapters. Articles were published from 1984, with the majority published after 2001 and the most recent being published in 2016. Records were predominantly reporting studies from the USA, UK, Australia, India, South Korea, and European countries. Of the included records, 23 were case studies [19, 25-27, 34-38, 40-43, 49-58], seven were cohort studies [28, 29, 39, 44, 46, 59, 60], five were RCTs [18, 20, 21, 30, 47], four were Nonrandomised Controlled Trials [22, 23, 31, 48], and four were retrospective studies [24, 32, 33,45].

3.3. Population Characteristics. Of the 43 included records, 16 studies [18, 19, 21-25, 38, 39, 41-43, 55-57, 60] included patients reporting hyperacusis as a primary complaint. Of these studies, nine (53%) were case studies and only two (12%) were RCTs (Table 2). Equally, 16 studies [34-37, 40, 44, 45, 47, 49-54, 58, 59] included patients reporting hyperacusis as part of a set of symptoms, the most common of which were tinnitus, hearing loss, vertigo, or aural fullness. The remaining 11 studies included patients reporting hyperacusis as a secondary complaint to tinnitus [20, 26-28, 30-33, 48] and/or hearing loss [29, 46]. Duration of the participants hyperacusis was not reported in 29 studies [20-26,28-33, 38, 39, 42-48, 50-53, 56, 57, 59]. For the remaining 14 studies [18, 19, 27, 34-37, 40, 41, 49, 54, 55, 58, 60], the duration of hyperacusis reported ranged from 6 weeks in a surgical case study [55] to 27 years in an "acoustic training" case study [40].

3.4. Current Definitions for Hyperacusis. Fourteen studies [26-28, 31-33, 44, 45, 47, 48, 51, 52, 54, 57] did not provide a working definition of hyperacusis. Across the remaining studies, common terminologies were used to define hyperacusis, with four main themes identified (Figure 2).
Box 1: Data extraction fields.

First author surname
Year of publication
Study Title

Publication type: journal article, report, conference paper,
undergraduate dissertation, postgraduate dissertation, book
chapter, webpage, or other (please specify)
Country where study was conducted
Aim of study
Terminology used to describe hyperacusis

Study design: case report, case series, cohort study, retrospective
study, non-randomised control trial, randomised control trial, or
Study design (if other)

Methods: questionnaire, focus groups, interviews, audiometric
tests, clinical tests, medical/physical investigations, or
Sample size for primary analysis (not follow up)
Study population: patients, general public, students or combination

Hyperacusis complaint: primary, secondary or as part of symptom set
What are the comorbid conditions/syndrome/symptom set?
Hyperacusis-related symptoms reported
Average duration of hyperacusis in years (SD)

Measures used for screening of hyperacusis to the study
Assessment time points
Outcome measures used on patients with hyperacusis

Treatment primarily aimed at hyperacusis

Nature of therapeutic interventions reported (summarise
key components)
Treatment outcomes from questionnaires
Treatment outcomes from LDLs
Treatment outcomes--anecdotal reports
Study limitations
Further research priorities specified

One theme that emerged from the definitions focused on "reductions" in sound tolerance [24, 34, 38, 43, 50, 60]. Authors described hyperacusis as "a reduction of normal tolerance for everyday sounds" [38], "decreased sound tolerance" [24], "lowered threshold for sound tolerance" [50], or "the collapse of loudness tolerance" [43]. In contrast to this, other studies emphasised the "increased sensitivity" to sound [20, 29, 35, 39, 40, 42, 46, 53, 55]. This theme included descriptors such as "hypersensitivity," "oversensitivity," or "abnormal sensitivity" to define the degree of tolerance to sounds (Figure 2). One author used descriptors that indicated increased loudness, "a disproportionate growth in subjective loudness of sounds" [49]. Other definitions referred to "intolerance" to sounds that had been deemed as ordinary [18, 19,21,30,37,41,58,59]. Authors referred to "noise intolerance to ordinary sounds" [58], "unusual intolerance" [18, 19], or "intolerance to the loudness of sounds that most individuals deem to be tolerable" [21]. The last theme to emerge included definitions that referred to the physical or emotional reaction to the sound, such as "subject exhibits negative reactions" [25], "abnormal, generally painful perception of loudness" [23], or the "response or reaction from the auditory cortex" [22, 36].

Underlying these themes, throughout, was the common concept of the tolerance to sound being different from "normal." Definitions referred to sound tolerance of "normal listeners" [38], "a normal person" [40], "a typical person" [19], or "others" [43] in comparison to sound tolerance of patients with hyperacusis. For example, Formby et al. [30] described hyperacusis as a "general intolerance to the loudness of sounds that would not typically be bothersome for most individuals." In some cases, the response to "ordinary" or "everyday" environmental sounds was described as "unusual" [18, 19, 55] or "abnormal" [22, 23, 36, 42], and in one case the response was described as "exaggerated or inappropriate" [19].

Across the themes reductions, sensitivity, and intolerance, some authors noted within their working definitions the overt physical and emotional responses evoked by the perception of sounds. These authors highlighted the "bothersome" [30,39], "distressing" [20], or "disabling" [37] nature of the sounds, the "discomfort" [19, 40, 43, 49, 55] or "pain" [23, 49] caused by the perception of sound, or the negative reaction to exposure to sound [25]. In one case, Silverstein et al. [58] listed associated symptoms of "emotional, social and physical" reactions to hyperacusis within their working definition.

3.5. Assessment Tools Used for Diagnosis and Outcome. In six studies (14%), the assessment tools used to quantify or diagnosis hyperacusis were not stated [20, 21, 39, 43, 47, 59]. Sixteen studies used clinical interviews (history and examination) to assess patient symptoms [28,29,35,36,41,42,46,4957]. Most of these studies were case series and reports, with some implying that interviews were used but not explicitly stating this.

Of the remaining 21 studies [18, 19, 22-27, 30-34, 37, 38, 40, 44, 45, 48, 58, 60], audiometric measures (Loudness Discomfort Levels (LDLs), Maximum Comfort Levels (MCLs), and Dynamic Range (DR)) and patient self-reported measurement tools (Baltimore questionnaire (VAS) [61], Sound Hypersensitivity Questionnaire [62], Hyperacusis Questionnaire (HQ) [63], Hyperacusis test (12-item) [38], a non-validated Hyperacusis Questionnaire adapted from Gerauschuberempfindlichkeit (GUF [64]) [60], and Tinnitus Retraining Therapy (TRT) assessment interview [65]) were reported to assess hyperacusis. Most commonly reported were LDLs, the HQ and TRT assessment interview. The six studies [18,22,24, 30, 31, 37] that reported using LDLs varied in the dB values used to quantifyhyperacusis. Gold et al. [22] and Hazell et al. [24] specified that average LDLs should be below 100 dB HL for 1, 2, 3, and 8 kHz in both ears (pure tones), whilst Juris et al. [18] and Formby et al. [30] specified that LDLs using pure tones should be <90 dB HL in at least one ear at the frequencies of 0.5,1, and 2 kHz or from at least two frequencies in the range 0.5 kHz to 4 kHz, respectively. In contrast, McKinney et al. [31] specified less commonly used LDLs using pure tones of less than 88.39 dB SPL and 81.24 dB SPL to classify individuals as having hyperacusis with normal hearing and hearing loss, respectively. Ruth and Hamill-Ruth [37] did not specify any criteria for LDLs. A similar case was found across the four studies reporting the HQ [19,45,48, 58]. Only one study [19] specifically identified a value on the HQ that would diagnose hyperacusis (36 points). Seven studies used the TRT assessment interview to classify patients into one of five categories associated with tinnitus and hyperacusis [24-27, 32-34]. Of these studies, only Hazell et al. [24] explicitly referred to categories 3 and 4 (hyperacusis present).

The most commonly specified posttreatment outcome measures were LDLs and the HQ (Tables 2-4). Nineteen studies reported increases in LDLs after treatment as either a primary [18, 21-25, 30, 31, 33, 34, 42, 47, 60] or secondary outcome [19, 28, 36, 38, 39, 58]. Six studies reported changes in HQ scores as the primary outcome [19, 39, 45, 46, 48, 58] or secondary outcome measure [18]. Of the 43 included records, 13 case studies [26, 35-37, 41, 43, 49, 50, 52-57] and two cohort studies [29, 59] relied on patient self-reported change in hyperacusis through clinical assessment at follow-up. One study [20] did not state a hyperacusis-specific outcome measure, only reporting the Tinnitus Questionnaire [66] as an outcome measure. The remaining studies reported a variety of different outcome measures, ranging from single item visual analogue scales to multi-item hyperacusis-specific questionnaires such as Multiple Activity Scale for Hyperacusis (MASH) [67] and Sound Hypersensitivity Questionnaire [62].

There is reasonable body of evidence on the development and reliability of the HQ as a diagnostic tool [63, 68], but the validity and reliability of the HQ as an outcome measure are yet to be fully examined. Questions have been raised on the appropriateness of the items in the questionnaire and the need of validation in a population with a primary complaint of hyperacusis is known [68]. Evidence for the reliability of LDLs is variable [69, 70] with reliability depending on a number of factors including instructions given to patients [71] and choice of sound stimuli [8]. Importantly, there are inconsistencies in the relationship between LDLs and self-report sound tolerance, with LDLs (using pure tones or speech sounds) often failing to reliably relate to self-reports of tolerance sounds in daily life [72].

3.6. Current Management Options for Hyperacusis. All 43 included records reported potential treatment benefits for hyperacusis (Tables 2-4). However, only nineteen studies (44%) sought to evaluate interventions or management strategies specifically aimed at reducing hyperacusis [18, 19, 21-25, 35, 38-43, 49, 56-58, 60]. For the most part, the studies explored interventions that were primarily aimed at reducing tinnitus. Management strategies explored were Cognitive Behavioural Therapy (CBT), TRT, counselling, devices, pharmacological therapy, and surgery.

3.7. Cognitive Behavioural Therapy. Three studies explored the potential benefits of CBT [18-20] (Table 2). Juris et al. [18] reported an RCT investigating the benefits of CBT for patients experiencing hyperacusis. Treatment comprised general CBT principles that were aimed to educate, target overt emotional reactions to sounds though graded exposure to sounds, reduce stress though relaxation, and provide patients with the tools to manage more difficult situations and restart activities (behavioural activation). After treatment, patients in the CBT group showed a significant reduction in hyperacusis severity as assessed by the HQ and an increase in LDLs from baseline, compared to the waiting list group. Only small effects were observed for quality of life and depression, and symptoms of anxiety were unchanged. Fioretti et al. [19] reported a case study in which a patient with hyperacusis underwent a four-month course of pharmacological therapy with bilateral sound generators (graded sound exposure) and CBT to target fear of sounds. Reduced hyperacusis-related symptoms were reported. Following this, the patient suspended treatment and underwent chemotherapy for breast cancer. Eight months later, after cancer treatment, the patient reported worsening of hyperacusis symptoms. Serotonin reuptake inhibitors and a further 4 months of CBT were prescribed. Following treatment, the patient reported an improvement in mood (depression, hostility, and sadness) and sound tolerance. In this case, no details were provided on the components of CBT. Hiller and Haerkotter [20] reported an RCT investigating CBT and the possible additional effects of sound stimulation on improving severity of tinnitus. In this study, one group received treatment comprised of an educational component (tinnitus education) in which patients learned about and applied psychological concepts such as "vicious cycle" and "coping cycle" to their personal situations with tinnitus. The second group completed ten sessions of CBT, including education (avoiding silence), changing thought processes (relaxation), diverting attention, and identifying avoidant behaviours and short/long terms consequences of behaviour. Half of each group were supplied with sound stimulation through behind-the-ear broadband white noise generators for each ear, with volume controls for graded increases. Following treatment, greater improvements in tinnitus severity were reported for patients with hyperacusis compared to those without hyperacusis. Notably, hyperacusis was not measured after treatment in this study.

3.8. Tinnitus Retraining Therapy. Sixteen studies explored the use of TRT for patients with hyperacusis as a primary complaint [21-25] or a secondary complaint [26-33] or as part of a symptom set [34-36] (Table 2.).

Nine studies [24-29, 32, 34, 35] focused on a classic TRT protocol to elevate hyperacusis with/without tinnitus. These studies incorporated a component of counselling including educational training in which the Jastreboff neurophysiological model is described to explain treatment and demystify the patients' experience. Guidance is given about avoidance behaviour (e.g., use of earplugs, avoiding environment sounds, or avoiding quiet) and the application of desensitising sound and sound enrichment was discussed. The depth of counselling and sound components depends on the treatment category (0-4: presence of tinnitus, hearing loss, hyperacusis, or noise exposure) assigned; for categories 1-2, sound generators are recommended; for categories 3-4 aimed at hyperacusis, bilateral open-fitting sound generators are fitted with instructions to gradually increase the sound daily to be tolerable without difficulties. Sound enrichment techniques are also used, in which digitally produced nature sounds are slowly reintroduced [24-29, 32, 34, 35]. Eight of these studies reported improvements in hyperacusis [25, 27, 29, 32, 35], three of which reported increased LDLs [24, 28, 34] following treatment. Hazell et al. [24] reported that, after 2 years of treatment, LDLs were well within the normal range (>100 dB) in over 60% of patients. In a case series, Formby and Gold [34] found that individual patients were reporting "noticeable subjective improvement in sound tolerance," resolution of complaints of discomfort, and in one case "complete resolve of sound tolerance problems." In a case report, Hesse et al. [35] described the need for the patient to build up a good therapeutic relationship before starting the sound exposure and generator component of therapy. This led to hyperacusis only occurring on very rare occasions. Both Suchova [29] and Molini et al. [27] reported that only a small number of patients with hyperacusis showed improvement following TRT. Molini et al. [27] reported that only one patient in hyperacusis category 3 achieved therapeutic success (a decrease of 2 or less on the scale of symptoms, a Tinnitus Handicap Inventory Grade 1, and an awareness of tinnitus value of less than or equal to 10% of the patient's wakefulness). In contrast, Forti et al. [26] found that TRT led to patients reporting no differences in difficulties with activities (relaxation, concentration, sleep, social relations, and work) following the treatment.

Three studies evaluated the effectiveness of using sound generators alongside directive counselling with patients reporting a primary complaint of hyperacusis [21], tinnitus [31], or hearing loss [30]. All three studies reported that LDLs had significantly improved over the course of treatment. In an RCT, Formby et al. [21] showed a clear pattern of LDLs initially increasing and then plateauing at 6 months after the onset of full treatment (counselling and noise generators). All three studies also reported greater treatment success and improvements of hyperacusis in patients who used noise generators in addition to directive counselling than those using part of the treatments (counselling alone, counselling and placebo noise generator, or noise generator only). In another RCT, Formby et al. [30] found that changes in judgements of uncomfortable loudness for the full treatment group after treatment averaged 15 dB-10 dB, compared to the changes of 5 dB or less in the other groups.

Gold et al. [22] investigated functional auditory changes demonstrated by increases in LDLs and DR during TRT for patients with tinnitus and hyperacusis. Following treatment, both LDLs and DR increased from the initial assessment whilst hearing threshold did not change significantly. The authors concluded that the DR can be increased following TRT. Patients self-reported an increase in quality of life and a decrease in the number of daily activities affected by tolerance problems. Similarly, Wolk and Seefeld [23] reported that regular use of maskers (set at hearing threshold and slowly increased) improved LDLs and DR and reduced severity of hyperacusis to "no longer a problem." Formby and Keaser [33] explored the sound therapy component of TRT and potential treatment benefits for tinnitus patients using hearing aids (HA) versus audiometrically matched or LDL-matched tinnitus patients with hyperacusis using noise generators. Increases in LDLs and DR at follow-up were observed for both the audiometrically matched and LDL-matched groups. In the HA versus audiometrically matched noise generator condition, changes in hearing thresholds between groups were nominal, but patients using noise generators showed significant increments in LDLs compared to the HA group. In the HA versus LDL-matched noise generator condition, the changes in LDLs at 1kHz were greater for the LDL-matched group than the HA group; differences were negligible at other frequencies.

In a case study of a patient with posttraumatic stress disorder reporting hyperacusis as a symptom, Westcott [36] reported that, following an initial treatment of antidepressants (a selective serotonin uptake inhibitor/a serotonin and noradrenaline reuptake inhibitor) for sound intolerance attributed to anxiety and depression, a TRT desensitisation program was carried out to help increase tolerance to environmental sounds. The patient self-reported an improved ability to cope with loud sounds, reduced reaction to unexpected loud sounds, and the ability to actively pursue her own piano playing.

One case report followed some of the TRT principles without explicitly stating this method [37]. Ruth and Hamill-Ruth [37] applied counselling with tinnitus habituation therapy (bilateral fitted in-the-ear-noise generators) to improve daily activities, relaxation, and mood monitoring in a patient experiencing both tinnitus and hyperacusis. In addition to therapy, the patient was prescribed Baclofen for pain and sleep disturbance. After one year, the patient no longer experienced tinnitus or hyperacusis and reported an improved ability to sleep and socialise.

3.9. Counselling Alone. Attri and Nagarkar [38] reported a case study using hyperacusis-focused directive counselling to educate the patient on the auditory system and mechanisms of hyperacusis (Table 2). Guidance and advice were given on avoiding silence, overprotection of ears, and use of background sounds to desensitise hyperacusis. The patient reported a reduction in HQ scores from 23 points (moderate) to 15 points (close to normal) and improved tolerance to sounds, with difficulties tending to occur only during depressive episodes.

3.10. Devices Alone. Eleven studies reported the effects of using different devices on patients with hyperacusis as a primary complaint or secondary complaint or as part of a symptom set (Table 3). Two studies [39, 40] reported "acoustic training" for hyperacusis which notably improved symptoms in the long term, with symptoms remaining in remission over a year later. These studies slightly differed in methodology. The seven "acoustic training" sessions (administered every 5 days) reported by Miani et al. [40] consisted of six to ten stimulations in which a narrowband noise was sent to an acoustic free field, before a 60 dB HL pure tone (other types of sounds were also used in later sessions) was sent through headphones for about 3 minutes followed by a pause of 3 minutes. The intensity of the sound stimulus and duration were increased in 5 dB HL steps (up to a total increase of 35 dB HL) and up to 5 minutes of exposure, until the last session, when the sound in the headphones reached 95 dB HL in all frequencies. High frequencies used in the sessions led to reports of discomfort by patients when listening via headphones, but not in the acoustic free environment. These sounds were not reflective of normal environmental sounds, so authors were not concerned. In contrast, Norena and Chery-Croze [39] used passive exposure to an enriched acoustic environment (EAE). Sounds used were pure tones (based on the cut-off frequency of hearing loss) presented in random order for the duration of 100 ms with pauses between of 100 ms. Participants listened to the EAE through headphones for 1-3 hours a day at a just audible level. Auditory sensitivity significantly decreased at all frequency bands, from 2 weeks onwards. The impact of hyperacusis on activities and daily functioning was reduced, with all participants reporting significant decrease in both MASH and HQ scores over the course of the treatment.

Three studies reported desensitisation programs using broadband tinnitus maskers [41, 42] and pink noise tape cassettes [43]. To desensitise patients, sounds were presented in each ear through wearable tinnitus maskers or through headphones (for pink noise) and started at a threshold the patient could tolerate and gradually increased in loudness over the sessions. In the case series reported by Vernon [41], the patients wore ear plugs during the day and carried out the desensitisation program at night. All three studies reported improvements in hyperacusis, although some patients did not comply with the use of the tape cassettes as they were afraid to use them because they believed that it would aggravate their hyperacusis. One patient reported improved ability to manage life without hyperacusis limitations [42].

One RCT [47] evaluated the effectiveness of a continuous wave laser TinniTool versus a dummy laser device. A laser probe was inserted into a specifically designed headset which projects the laser beam (power output 5mW) onto the tympanic membrane though a 17-degree diverging lens (creating a spot size of 1 cm) for 20 minutes a day, resulting in an energy density of around 6 J at the membrane. The placebo dummy device, apart from activation of the laser beam, reproduced all aspects of laser therapy. Tinnitus patients in the laser therapy group reported decreased hyperacusis. Seven patients in the laser therapy group and eight patients in the placebo group reported LDLs lower than 80 dB at baseline; of these, LDLs improved in five patients in laser therapy group compared to two patients in placebo group following treatment.

Two studies [44, 45] reported the long term effects of cochlear implants (CI) treatment in patients with unilateral hearing loss and tinnitus. Mertens et al. [45] found that hyperacusis only significantly reduced for the single-sided deafness group and not the asymmetric hearing loss group. Significant differences were observed in HQ total and the attentional subscale scores between the CI-ON and the CIOFF condition for the single-sided deafness group. Ramos Macias et al. [44] reported that CI use resulted in improved sound tolerance for six out of seven patients, who showed a reduction in Sound Hypersensitivity Questionnaire scores [62]. Only one patient showed decreased sound tolerance problems. Saglier et al. [46] investigated the impact of family history of hearing impairment on rehabilitation using HAs and reported that hyperacusis scores were more improved in patients without a family history following fitting of HAs than those with a family history.

One study [48] reported that 21 sessions using a tinnitus progressive phase-shift treatment device ("Phase-out TM"), which presented a sound that resembles the frequency and amplitude of patients' own tinnitus but shifts 6[degrees] every 30 seconds, did not significantly change scores on the HQ or audiometric data between a pure tone tinnitus group and a narrow band noise tinnitus group. All measurements remained unchanged after therapy.

One case study [49] reported that diazide and methylprednisolone improved unilateral hearing loss but did not diminish hyperacusis. Following fitting of a custommade binaural full-concha, unvented in-the-ear compression device (Micro Tech[R] Refuge-hyperacusic), the patient reported that the devices were very helpful and provided a level of protection that was at least as good as what he achieved with the plugs and muffs, but reported that he still could not carry out social activities (listening to music, attending concerts, restaurants, movies, and events, or hearing others when eating).

3.11. Pharmacological Therapy. Pharmacological therapy was reported in five case studies [50-54] where hyperacusis was part of a set of symptoms (Table 4). One case study [52] reports a patient placed on a diet to address metabolic factors, bisphosphonate and calcium, for otosclerosis. The patient reported feeling better until the treatment regime was discontinued, when symptoms of dizziness and hyperacusis returned. The patient was prescribed bisphosphonate risedronate and reported reduced complaints of symptoms and feeling best when the diet regime was followed. In another case study, Brookler [54] reported a patient prescribed metformin, risedronate (30 mg twice weekly), calcium, vitamin D, and sodium monofluorophosphate who reported worsening of hyperacusis on days when they took risedronate. Alternating the regime with etidronate (400 mg/day for 2 weeks) and risedronate (30 mg twice weekly for 4 weeks) did not improve hyperacusis but increasing risedronate intake to 30 mg twice weekly for 6 weeks and then for 11 weeks improved hyperacusis to be "almost gone." Gopal et al. [51] reported differences in LDLs between conditions in which the patient was unmedicated and medicated with fluvoxamine (50 mg/day) and fluoxetine (20 mg/day), with improved LDLs above 100 dB in the medicated condition. Lee et al. [53] reported that antihypertensives and gabapentin (600 mg/day) reduced the intensity of both tinnitus and hyperacusis after 1 month. Nields et al. [50] reported that although six weeks of intravenous ceftriaxone led to a remission of "all symptoms," excluding mild arthralgia and fatigue, hyperacusis subsequently worsened after treatment. Intravenous cefotaxim slightly diminished hyperacusis, but it remained an impediment to performing activities. Clonazepam led to a short-term lessening of hyperacusis symptoms but increased emotional lability. Following carbamazepine (blood level of 6 [micro]g/ml.), the patient reported that sound tolerance thresholds had increased, fear and irritability had lessened, and the kindling-like effects (sounds "adding up") had diminished so she was able to recover more quickly.

3.12. Surgery. Six studies reported results from patients electing to undergo surgery specifically for hyperacusis [56, 58, 60], hearing loss and hyperacusis [57], intracranial aneurysms [55], or Meniere's disease [59] (Table 4). In a cohort study, J. Gavilan and C. Gavilan [59] performed a middle fossa vestibular neurectomy, which includes ablation of the vestibulofacial anastomosis, and removal of part of the nerve. They reported that hyperacusis symptoms had lessened or were no longer a problem. Nikkar-Esfahani et al. [56] reported that successful occlusion (permeatal blocking) of the round window surgery in two patients led to an improvement of conductive hyperacusis symptoms but caused a mild exacerbation of conductive hearing loss. Khalil et al. [55] reported a case study of a patient with hyperacusis whose magnetic resonance imaging (MRI) revealed a large aneurysm. A Guglielmi detachable coil (GDC) embolization of the aneurysm led to resolution of hyperacusis. Dang et al. [57] present a case of a patient with hearing loss and hyperacusis caused by bilateral superior semicircular canal dehiscence and posterior semicircular canal dehiscence. Surgery involved a right-sided transmastoid approach and temporalis fascia plugs of each defect. Hyperacusis was assessed as resolved at three months' follow-up. The patient did report residual imbalance and right ear fullness, however. Two of the most recent studies [58, 60] reported that transcranial round window niche and oval window reinforcement surgery led to improved hyperacusis, with increased LDLs and reduced Hyperacusis Questionnaire scores (author-developed questionnaire adapted from the GUF [64]), but with no changes to hearing. One patient reported sustained improvements of hyperacusis and quality of life four years after surgery [58]. Silverstein et al. [60] reported higher success with unilateral surgery than bilateral surgery.

3.13. Future Research Priorities Identified in the Literature. Various further research priorities were identified in the included records. Gabriels [42] urged audiologists to pool their patient information together in order to study the link between symptoms of hyperacusis combined with having a limited DR. Most authors suggest that larger studies (Tables 2-4) need to be conducted to verify the effectiveness of CBT [18], TRT [28, 32], and different surgical treatments [56, 58, 60]. Studies evaluating the treatment benefit of counselling combined with alternative sound therapies (e.g., enriched acoustic therapy [39]) compared to those usually used in TRT should be conducted [30]. Larger studies focused on testing the effect of maskers/noise generators and HAs specifically for hyperacusis as a primary complaint are indicated [20, 21, 42]. Other possible treatments for hyperacusis suggested for further research include laser therapy [47], desensitisation techniques [41], and further investigation of the pharmaceutical treatments for certain subgroups of hyperacusis specifically when hyperacusis is induced by Lyme disease [50] or when cooccurring with depression [38]. Berry et al. [28] also highlight the importance of developing patient-based assessments for hyperacusis.

4. Discussion

This scoping review collated clinical research focused on management strategies used for hyperacusis, the definitions of hyperacusis, tools used for assessment and evaluation, and future research priorities. We found that more than half of the research currently reported was based on individual case studies and therefore cannot be generalised. In addition to this, management strategies were typically evaluated in patients reporting hyperacusis as a secondary complaint or as part of a symptom set, and as such the outcomes reported only provided an indication of effectiveness for hyperacusis. There is a lack of sufficient evidence to identify effective management strategies. These findings highlight an urgent need for controlled trials to evaluate the effectiveness of these management strategies for patients experiencing hyperacusis.

The definitions characterised by researchers had common terminology, with descriptions mainly differing in the emphasis placed on the direction of the sound tolerance, either reduction in tolerance or oversensitivity. The underlying theme reflected how the sound tolerance in patients with hyperacusis is different from "normal." However, the appropriateness of "hypersensitivity" as a term for problems with sound tolerance has been questioned. Tyler et al. [12] argued that hypersensitivity reflects hearing thresholds that are better than normal and that hyperacusis is not usually associated with this and as such recommended avoiding using this terminology. The reference to "abnormal" could be upsetting to patients and lead to negative reactions to the experience. Although some studies referred to emotional reactions, such as distress and discomfort, and physical reactions of pain as characteristics of hyperacusis within their working definitions, the four distinct definitions (loudness, annoyance, fear, and pain) used in a recent narrative review [12] of hyperacusis were not readily used in the literature. The framework suggested by Tyler et al. [12] may be indicative of characteristics of hyperacusis rather than defining discrete subtypes that can be unambiguously differentiated. Consequently, there is a need for consensus through a systematic process involving professionals and patients to define hyperacusis and inform standards for assessment criteria.

To assess hyperacusis, most studies relied on self-report, with only some studies reporting use of a tool (LDLs, the HQ, or the TRT interview) to establish severity. Across the board, no consistent diagnostic tools or criteria were used, making it virtually impossible for comparisons across study populations. There is a need for established diagnostic criteria for hyperacusis and self-report measures appropriate for the population which have been evaluated for this purpose. The same can be said for outcome measures. The majority of studies did not report the outcome measures used. For case reports, the reliance was on clinical interview after treatment, which can be meaningful as the findings directly relate to patient experience. However, with the restricted information reported, we can only have limited confidence in the findings and the possible avenues for future research. Otherwise, a variety of outcome measures were reported, the most popular of which were LDLs and the HQ. Neither has been fully evaluated as outcome measure and in terms of the HQ was not designed to be used as an outcome measure. The three response options only provide coarsegrained categorical units of measurement and as such will not reliably detect small but potentially meaningful changes in hyperacusis [73, 74]. For LDLs, variability in the type of sounds used and the instructions given to patients' makes comparisons across studies relatively meaningless. There is an apparent need for clear guidance and consensus on LDLs use as outcome measures and for the development of self-report questionnaire specifically aimed at being responsive to changes in the impact of hyperacusis.

The most commonly reported management strategy was TRT, and most studies indicated that the treatment was beneficial to patients with hyperacusis. Complex interventions that include several interacting components require systematic evaluations [14]. Most included records reported here lacked rigorous methodology, raising concerns about the validity of the findings. Having said this, the RCTs using patients with a primary complaint [21] or secondary complaint of hyperacusis [30] did provide empirical evidence for the combination of the counselling and sound therapy components (full treatment) being of more benefit than the single components and as such highlighting an important principle of TRT. For the most part, there was a lack of rigor in reporting what was done in the name of TRT, particularly details of the counselling components used throughout the sessions or any differences when treating tinnitus and hyperacusis.

Despite the earliest research reported being published in the 1980s, there is an absence of research evidence on treatments directly aimed at treating hyperacusis. Most studies reported here were not focused on hyperacusis as the primary reason for management. This was especially true for the pharmacological treatments, all of which reported hyperacusis as part of a set of symptoms and were case studies; the effects observed may not be representative of the general hyperacusis population. The drugs reported were in general aimed at alleviating other symptoms and the reduction of hyperacusis may have been a byproduct of the reduction in those symptoms. Without the appropriate population, sample size, and systematic trial methodology, no strong conclusions can be reached.

Overall, only five of the 43 studies used a RCT design [18, 20, 21, 30, 47], two of which were solely aimed at patients experiencing hyperacusis [18, 21]. Whilst there are challenges in utilising a placebo control in interventions that involve sound therapy and/or counselling, waiting list controls designs could be implemented, as could a standard care versus specialist care design. The use of more robust trial designs in future hyperacusis studies would increase the quality and value of the evidence and support the development of optimised treatments.

5. Conclusions

Clear themes were identified from the definitions of hyperacusis reported, with an underlying theme that highlights the difference in sound tolerance from what is considered "normal." In order to enable diagnosis and assessment, there needs to be an agreement on the definition including the perspectives of patients with lived experience of hyperacusis. The majority of management strategies were evaluated in patients reporting hyperacusis as a secondary complaint or as part of a symptom set and as such the outcomes only highlight potential benefits and no strong conclusions can be made. Authors agree that research should prioritise evaluating interventions and management strategies on patients with hyperacusis as a primary complaint, in particular TRT, sound therapy interventions, and pharmacological interventions. Importantly, to establish the benefits within this population, there is a need for controlled trials and appropriate patient-based assessments specifically for measuring hyperacusis. To date, only two controlled trials primarily aimed at hyperacusis have been conducted and as yet there are no registered ongoing or planned trials for hyperacusis as a primary complaint. With such an open field, it is essential that these opportunities for new research highlighted here lead to new controlled trials that will have a greater impact on the field.


The views expressed in this publication are those of the authors and not necessarily those of the NHS, the National Institute for Health Research, the Department of Health.

Conflicts of Interest

The authors declare that they have no conflicts of interest.


This report is independent research by the National Institute for Health Research Biomedical Research Centre Funding Scheme.


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Kathryn Fackrell, (1,2) Iskra Potgieter, (1) Giriraj S. Shekhawat, (3,4) David M. Baguley, (1,2) Magdalena Sereda, (1,2) and Derek J. Hoare (1,2)

(1) NIHR Nottingham Biomedical Research Centre, Ropewalk House, Nottingham, UK

(2) Otology and Hearing Group, Division of Clinical Neuroscience, School of Medicine, University of Nottingham, Nottingham, UK

(3) Health Systems and Audiology, The University of Auckland, Auckland, New Zealand

(4) Tinnitus Research Initiative, Regensburg, Germany

Correspondence should be addressed to Kathryn Fackrell;

Received 31 May 2017; Accepted 16 August 2017; Published 9 October 2017

Academic Editor: Alberto Raggi

Caption: Figure 1: Flow chart of stages of study selection process.
Table 1: Search term strategies for hyperacusis interventions and
outcome measures. CENTRAL = Cochrane Central Register of Controlled
Trials; CINAHL = Cumulative Index to Nursing and Allied Health
Literature; ISRCTN = International Standard Randomised Controlled
Trial Number; ICTRP = the International Clinical Trials Registry

Search terms                     Search engine

Hyperacus* AND [assess* OR       Embase, PsycINFO, CENTRAL, Scopus,
measure* OR test* OR outcom*               Web of Science
OR Diagnos* OR defin* OR
treat* OR manag* OR therap* OR

Hyperacus* AND [(assess* OR        Web of Science, Scopus, PubMed,
measure* OR test* OR diagnos*           CENTRAL, CINAHL Plus
OR defin OR outcom* OR therap*
OR interv* OR treat* OR

Hyperacus*                       Cochrane ENT Disorders Group Trials

Hyperacusis             , ISRCTN, ICTRP

Hyperacusis AND [assessment OR             Google Scholar
measurement OR test OR Outcome
OR diagnosis OR definition OR
treatment OR Therapy OR

Table 2: Charting population and outcome data according to
management strategy: Cognitive Behavioural Therapy, Tinnitus
Retraining Therapy, and Counselling. RCT = Randomised Controlled
Trial; NRCT = Nonrandomised Controlled Trial; Retro = retrospective
study; LDLs = Loudness Discomfort Levels; DR = Dynamic Range; HQ =
Hyperacusis Questionnaire; TRT = Tinnitus Retraining Therapy; VAS =
Visual Analogue Scale; HADS = Hospital Anxiety and Depression
Scale; TSK = Tampa Scale of Kinesiophobia; QOLI = Quality of Life
Inventory; TQ = Tinnitus Questionnaire.

Intervention       Ref           Study        Sample      Hyperacusis
                                design         size        complaint

CBT            Juris et al.       RCT           60          Primary

CBT and        Fioretti et       Case            1          Primary
pharma           al. [19]       report

CBT and         Hiller and        RCT           136        Secondary
noise           Haerkotter
generators         [20]

TRT             Formby et         RCT           40          Primary
                 al. [21]

TRT            Gold et al.       NRCT       130 (ears)      Primary

TRT              Wolk and        NRCT           122         Primary

TRT             Hazell et        Retro          187         Primary
                 al. [24]

TRT                P.J.       Case series   201 (56 hy)    Primary/
                Jastreboff                                 secondary
                and M. M.

TRT            Forti et al.   Case series    40 (Shy)      Secondary

TRT             Molini et     Case series       81         Secondary
                 al. [27]

TRT            Berry et al.     Cohort          32         Secondary

TRT              Suchova        Cohort          331        Secondary

TRT             Formby et         RCT           36         Secondary
                 al. [30]

TRT              McKinney        NRCT           182        Secondary
               et al. [31]

TRT             Bartnik et      Retro        100 (40 hy)   Secondary
                 al. [32]

TRT               Formby        Retro        51 (18 hy)    Secondary
                and Keaser

TRT               Formby      Case series        5          Within
                 and Gold                                 symptom set

TRT            Hesse et al.      Case            1          Within
                   [35]         report                    symptom set

TRT and         Wes tcot t       Case            1          Within
pharma             [36]         report                    symptom set

Counselling      Ruth and        Case            1          Within
and tinnitus     Hamill-        report                    symptom set
habituation        Ruth
therapy            [37]

Directive       Attri and                        1          Primary
counselling      Nagarkar

Intervention    Aimed at     Outcome measures
               treating hy       Primary           Secondary

CBT                Yes             LDLs            HQ; HADS;
                                                   TSK; QOLI

CBT and            Yes              HQ            LDLs, tonal
pharma                                            audiometry

CBT and            No         Not stated, TQ      Not stated

TRT                Yes             LDLs           Not stated

TRT                Yes             LDLs            Effect of
                                                hyperacusis on
                                                 the patients
                                                quality of life
                                                   and daily

TRT                Yes          LDLs, DR,        Not specified

TRT                Yes          LDLs, TRT        Not specified

TRT                Yes          LDLs (not        Not specified

TRT                No          Self-report       Not specified

TRT                No         TRT interview      Not specified

TRT                No           Subjective           LDLs
                               presence or
                                absence of

TRT                No          Self-report        Not stated

TRT                No         LDLs for pure      Uncomfortably
                              tone, speech,        loudness
                                and white         judgements

TRT                No             LDLs            Not specified

TRT                No            Author-          Not specified

TRT                No            Author-          Not specified

TRT                No             LDLs            Not specified

TRT               Yes          Self-report         Not stated

TRT and            No          Self-report        LDLs (after
pharma                                             treatment

Counselling        No          Self-report         Not stated
and tinnitus

Directive         Yes          Hyperacusis           LDLs,
counselling                        test            Hamilton
                                                 rating scale

Intervention   Main findings

CBT            There were significant group effects for the
               treatment group (CBT) on all secondary
               outcome measures except for the anxiety
               subscale of the Hospital Anxiety and
               Depression Scale. There were both large
               significant between-group (treatment versus
               waiting list) and within-group effects for the
               CBT group on hyperacusis severity as
               measured by the HQ. LDLs were
               significantly reduced after treatment in CBT
               group compared to waiting list group for
               both ears.

CBT and        The patient reported improved mood and
pharma         tolerance to sound with HQ scores below
               cut-off following second treatment. LDLs
               showed some improvement but were still
               below normal sound tolerance.

CBT and        At the 6-month follow-up, tinnitus
noise          education was shown to be more favourable.
generators     Improvements on TQ scores were observed
               for hyperacusis patients compared to all
               patients that completed the sessions. In the
               CBT group, similar results were shown with
               hyperacusis patients reporting
               improvements on TQ. More patients with
               hyperacusis than without were classified as
               responders for tinnitus education and CBT.
               LDLs improved over the course of the
               intervention. These improvements were
               generally apparent within the first 4 months
               of the intervention, plateauing at around 6
               months after the onset of full treatment
               (counselling and noise generators).

TRT            Comfortable level speech scores improved
               from 50% to 80% after 6 months of full
               treatment. Treatment success rate was
               highest for the full treatment when
               compared to any of the partial treatments
               (counselling and placebo noise generator,
               noise generator only) or placebo noise
               generator group.

TRT            The patients self-reported an improved
               quality of life, with ability to comfortably
               participate in an increased number of
               activities after treatment. LDLs were
               significantly improved, at or near normal
               LDL thresholds for each frequency after
               treatment. Significant increases in DR at all
               test frequencies were also observed
               following treatment.

TRT            Patients reported that severity of
               hyperacusis was improved from a big
               problem to no longer a problem on the
               analogue scale. Regularly wearing maskers
               was associated with improved LDLs and DR,
               on average after treatment, indicating
               improved acceptance of ambient noise.
TRT            The number of life factors affected by
               hyperacusis was significantly reduced
               between the first and third visit after
               treatment. LDLs were significantly increased
               between each visit, with the majority of
               improvements experienced between the first
               and second visit. LDLs reached normal
               levels in 60% of patients by the fourth visit.
               LDL results were not presented. 56 patients
               reported hyperacusis (with or without
               misophonia); of these, 45 patients (80%)
TRT            showed significant improvement after
               treatment. Improvement was higher for the
               hyperacusis and concurrent misophonia
               group (33/39) than hyperacusis alone
               patients (13/17).

TRT            No differences in difficulties with activities
               (relaxation, concentration, sleep, social
               relations, and work) were found in patients
               with hyperacusis following treatment.
               64 patients (79%) from categories 0 and 4
               improved and achieved therapeutic success
               at the end of 18 months based on a decrease
TRT            in symptom scale score of 2 or less (focused
               on tinnitus). Only nine out of the ten
               achieved therapeutic success in category 3.
               One patient (out of 1) in category IV

TRT            In the nine patients that presented with
               hyperacusis and tinnitus, LDLs were
               significantly improved following treatment.

TRT            Only 8 patients of 53 patients with
               hyperacusis showed an improvement in
               All treatment groups showed improvements
               of LDLs (10 dB or more at two consecutive
               follow-ups). LDLs and loudness judgments
               in the full treatment group (counselling and
               sound therapy with binaural sound
               generators) were consistently greater
TRT            (averaged 15 and 10 dB) than those measured
               for the other groups ((2) counselling and
               placebo sound generators, (3) binaural
               sound generators only, and (4) placebo
               sound generators). 82% of individuals were
               classified as successfully treated following
               full treatment, 50% following the neutral
               control treatment, and 25% and 40%
               following partial treatment.

TRT            Incidence of hyperacusis in the treatment
               group was significantly lower and LDLs
               were significantly increased after 12 months.
               Improved LDLs were greater for those
               patients who used noise generators in
               addition to directive counselling or
               amplification. LDLs of patients who
               complained of specific sounds as being
               uncomfortable were not significantly
               different from the rest of the treatment

TRT            In category III, 55% of hyperacusis patients
               showed improvement (hyperacusis present,
               no prolonged noise exposure, and irrelevant
               or significant subjective hearing loss) and
               60% in category IV (hyperacusis present,
               prolonged noise exposure, and irrelevant or
               significant subjective hearing loss)
               No significant treatment changes for the
TRT            audiometric thresholds. LDLs were
               significantly increased at each frequency
               following treatment. LDLs were significantly
               increased after treatment in the
               audiometrically matched hyperacusis noise
               generator treatment group compared to the
               hearing aid treatment group. LDLs between
               Hearing aid group and LDL-matched
               hyperacusis noise generator treatment
               groups for the 1000-Hz condition were
               significantly different.

TRT            In all five cases, LDLs were increased at 6
               months, exceeding normal tolerance levels
               after a year of treatment. In one case, LDLs
               were improved as much as 25 dB after a
               3-week period. Self-reported noticeable
               improvement in sound tolerance and
               complaints of discomfort to loud sounds
               had resolved.

TRT            The patient self-reporting hyperacusis rarely
               occurred ater successful treatment.
               No LDLs were measured at the beginning of
               treatment. LDLs were markedly lower than
               normal levels (>100 dB) after desensitisation
TRT and        treatment. Despite this, the patient
pharma         self-reported that all sounds had become
               more tolerable, with an improved ability to
               tolerate environmental sound and to cope
               with loud voices and reduced reactions to
               impact of sound.

Counselling    The patient no longer experienced severe
and tinnitus   tinnitus or hyperacusis and reported
habituation    improved pain symptoms and ability to
therapy        sleep and socialise one year after treatment.

Directive      The patient reported less difficulty with
counselling    sounds. An improvement in hyperacusis test
               scores and LDLs indicating close to normal
               sound tolerance. The patient also
               self-reported sufering less depression and
               difficulty with sounds only occuring during
               depressive episodes.

Table 3: Charting population and outcome data according to
management strategy: devices. RCT = Randomised Controlled Trial;
NRCT = Nonrandomised Controlled Trial. Retro = retrospective study;
LDLs = Loudness Discomfort Levels; LGOB = loudness growth in
one-half octave band; MCL = Most Comfortable Loudness; HQ =
Hyperacusis Questionnaire; SHQ = Sound Hypersensitivity
Questionnaire; MASH = Multiple Activities Scale for Hyperacusis.

                                         Study      Sample
Intervention             Ref            design       size

Acoustic              Norena and        Cohort        8
training             Chery-Croze

Acoustic             Miani et al.        Case         1
training                 [40]           report

Desensitisation          Case            Case         3
program with            report          report
tinnitus maskers

Desensitisation     Gabriels [42]     Case series     2
program with
tinnitus maskers

Desensitisation      Hudson [43]      Case series     20
program with
tinnitus maskers

Cochlear                Ramos           Cohort        16
implant            Macias et al.

Cochlear            Mertens et al.      Retro         23
implant                  [45]

Cochlear            Saglier et al.      Cohort       177
implant                  [46]

Wave laser          Teggi et al .        RCT
TinniTool                [47]                         54

Phase-shit           Meeus et al.        NRCT         21
treatment device         [48]

Suppression        Valente et al .       Case         1
device and               [49]           report

Pharmacological     Nields et al.     Case report

Pharmacological      Gopal et al.     Case report     1

Pharmacological     Brookler [52]     Case report     1

Pharmacological     Lee et al. [53]   Case report     1

Surgery             Brookler [54]     Case report     1

Surgery             Khalil et al.     Case report     1

Surgery                Nikkar-        Case report     2
                   Esfahani et al.

Surgery              Dang et al.      Case report     1

Surgery             Silverstein et    Case series     2
                       al. [58]

Surgery               J. Gavilan        Cohort        59
                        and C.
                     Gavilan [59]

Surgery             Silverstein et      Cohort        6
                       al. [60]

                    Hyperacusis      Aimed at
Intervention         complaint      treating hy

Acoustic              Primary           Yes

Acoustic           Within symptom       Yes
training                set

Desensitisation       Primary           Yes
program with
tinnitus maskers

Desensitisation       Primary           Yes
program with
tinnitus maskers

Desensitisation       Primary           Yes
program with
tinnitus maskers

Cochlear           Within symptom       No
implant                 set

Cochlear           Within symptom       No
implant                 set

Cochlear             Secondary          No

Wave laser         Within symptom       No
TinniTool               set

Phase-shit           Secondary          Yes
treatment device

Suppression        Within symptom       Yes
device and              set

Pharmacological        Within           No
                    symptom set

Pharmacological        Within           No
                    symptom set

Pharmacological        Within           No
                    symptom set

Pharmacological        Within           No
                    symptom set

Surgery                Within           No
                    Symptom set

Surgery               Primary           No

Surgery               Primary           Yes

Surgery               Primary           Yes

Surgery                Within           Yes
                    Symptom set

Surgery                Within           No
                    Symptom set

Surgery               Primary           Yes

                         Outcome measures
Intervention                 Primary                 Secondary

Acoustic                     MASH, HQ,                 LDLs
training                       LGOB

Acoustic                    Discomfort             Not specified
training                      levels

Desensitisation             Self-report             Not stated
program with
tinnitus maskers

Desensitisation              MCLs and
program with                   LDLs
tinnitus maskers

Desensitisation             Self-report
program with
tinnitus maskers

Cochlear                        SHQ                Not specified

Cochlear                        HQ                 Not specified

Cochlear                        HQ                 Not specified

Wave laser                      HQ                 Not specified

Phase-shit                      HQ                 Not specified
treatment device

Suppression                Self--report             Not stated
device and

Pharmacological             Self-report             Not stated

Pharmacological       (1) pure-tone threshold
                     testing (250-8000Hz), (2)
                         impedance testing
                         (tympanograms and
                    acoustic relex thresholds),
                       (3) speech audiometry
                      (speech threshold, word
                   recognition score), (4) click
                      evoked ABR testing, (5)
                         transient evoked
                       otoacoustic emission
                       (TEOAE) testing, (6)
                      uncomfortable loudness
                    level (LDL) testing, (7) a
                    screening test for central
                        auditory processing
                         disorders (SCAN-A
                      testing), and (8) blood
                       serum serotonin level

Pharmacological             Self-report             Not stated

Pharmacological             Self-report             Not stated

Surgery                     Self-report             Not stated

Surgery                     Self-report             Not stated

Surgery                     Self-report             Not stated

Surgery                     Self-report             Not stated

Surgery                         HQ                  LDLs (only
                                                   postop data)

Surgery                     Self-report             Not stated

Surgery                     Hyperacusis            Not specified
                           naire adapted
                            from GUF
                             and LDLs


Acoustic           Main findings
training           All participants reported a significant
                   decrease in both MASH and HQ scores over
                   the course of the treatment. Following the
                   enriched acoustic environment treatment,
                   auditory sensitivity significantly decreased
                   at all frequency bands and all loudness
                   categories (sot to too loud).
                   he treatment produced notable
                   improvement in the symptoms. No
                   discomfort levels were registered in a free
Acoustic           acoustic environment; discomfort levels
training           were only observed with headphones at high
                   frequencies that are not normally present in
                   environmental noises. Clinical situation
                   remained stable after 1 year.
                   One patient was able to travel daily without
                   ear protectors after 2 years of treatment.
                   Another patient self-reported an
Desensitisation    improvement in condition after 3 months.
program with       Another reported improvement of at least
tinnitus maskers   50% after 6 months of treatment.
                   he patient self-reported noticeable
                   improvements in hyperacusis with improved
Desensitisation    ability to manage life without the limitations
program with       associated with hyperacusis. LDLs and
tinnitus maskers   MCLs improved after four months. The
                   patient reported ear plugs as being
                   considerably beneficial.
                   Patients self-reported improvements in
Desensitisation    hyperacusis after using tape. Some were
program with       afraid to use them.
tinnitus maskers

Cochlear           Cochlear implant surgery resulted in
implant            improved sound intolerance, with a
                   reduction in SHQ score for six patients (out
                   of 7).

Cochlear           Cochlear implants were found to
implant            significantly reduce hyperacusis for the
                   single-sided deafness group, with significant
                   differences observed in HQ total and
                   attentional subscale scores between the
                   cochlear implant ON and the cochlear
                   implant OFF condition.

Cochlear           Hyperacusis scores prior to fitting hardly
implant            differed between patients without a family
                   history of hearing impairment and patients
                   with family history. At 6 months after
                   hearing aid fitting, patients without family
                   history reported lower hyperacusis scores
                   than those with a family history.
                   Laser therapy led to a decrease in
                   hyperacusis severity. LDLs for five patients
                   (out of 7) improved in sot laser group,
Wave las er        whilst only two patients in the dummy
TinniTool          control group showed LDLs of more than
                   80 dB. Four patients presenting hyperacusis
                   in the sot laser group also suffered from
                   migraine compared to only 2 in the dummy

Phase-shit         No significant differences were found on
treatment device   hyperacusis questionnaire or audiometric
                   data between pure tone tinnitus group and
                   narrow band noise tinnitus group.
                   he patient reported that the Micro Tech
                   Refuge-hyperacusic hearing instruments are
                   very helpful and provided a level of
                   protection that was at least as good as what
Suppression        he achieved with the plugs and mufs. The
device and         patient still reported an inability to listen to
pharma             music, attend concerts, restaurants, movies,
                   and events, or hear others when eating.

Pharmacological    Intravenous cetriaxone led to a remission of
                   all symptoms, but hyperacusis worsened
                   afterwards. Intravenous cefotaxim
                   diminished hyperacusis but did not
                   completely abate it. Hyperacusis luctuated.
                   Clonazepam led to short-term attenuation
                   of hyperacusis but increased emotional
                   liability. Threshold for sound tolerance
                   increased and sounds no longer seemed to
                   "add up" following carbamazepine and
                   patient reported increased ability to recover
                   more quickly.

Pharmacological    No remarkable clinical differences were
                   reported by the patient between the
                   unmedicated and medicated (luvoxamine
                   and luoxetine) conditions for (1) pure-tone
                   thresholds, (2) speech thresholds, (3) word
                   recognition scores, or (4) tympanograms
                   and (5) acoustic relex thresholds. Only (6)
                   LDLs were different between the two
                   conditions, with the medicated condition
                   showing increased sound tolerance.

Pharmacological    Patient self-reported that following the diet
                   regime made her feel better.

Pharmacological    Hyperacusis and tinnitus persisted but
                   intensity reduced after 1 month of treatment.

Surgery            The patient initially reported that
                   hyperacusis was worse on risedronate.
                   Following changes to the regime to
                   risedronate, hyperacusis was less frequent
                   and almost gone, although tinnitus and
                   aural fullness remained present.
                   Hyperacusis completely resolved after
Surgery            procedure and had not returned after 10

Surgery            Patients reported postoperative
                   improvement in symptoms. Two years after
                   the procedure, symptoms had continued to
                   improve, with no significant problems.
                   he patient self-reported that right-sided
                   hyperacusis significantly improved at 1
Surgery            month postop and completely resolved at 3
                   months postop. The patient was able to
                   resume all choir-related activities.
                   he patient reported marked improvement
Surgery            of hyperacusis and quality of life, with a
                   decrease in hyperacusis survey scores and
                   LDLs above 90 dB

Surgery            In the majority of cases (63% = 37 cases),
                   hyperacusis improved or disappeared
                   following surgery. Only in two cases did
                   hyperacusis become more severe, ten years
                   after surgery.

Surgery            All unilateral patients and two of the three
                   bilateral patients reported subjective
                   improvement in sound hypersensitivity,
                   with reduced questionnaire scores and
                   improved LDLs following treatment. The
                   patients reported no change in hearing and
                   improved quality of life after the procedure;
                   one reported improved noise tolerance in
                   social situations.

Figure 2: The four themes and underlying theme to emerge from the
content of the working definitions of hyperacusis reported in the
included records.

Different from "normal"


"Decreased sound tolerance and difficulty
adjusting to sudden shifts in volume                       Pt 1st
which may not evoke any such reaction
in a normal listener" [38].

"Reduced sound tolerance" [34].                            Pt SS

"Lowered threshold for sound                               Pt SS
tolerance" [50].

"Decreased sound tolerance" [24].                          Pt 1st

"The collapse of loudness tolerance so
that almost all sounds produce loudness
discomfort even though the actual                          Pt 1st
sound intensity is well below that
judged to be uncomfortable by others"

"A reduction of normal tolerance for                       Pt 1st
everyday sounds" [60].


"Noise intolerance to ordinary sounds, patients
often present with emotional, social and physical          Pt SS
symptoms" [58].

"A potentially disabling intolerance of moderate           Pt SS
to loud sounds" [37].

"General intolerance to the loudness of sounds
that would not typically be bothersome for most            Pt/GPb 2nd
individuals" [30].

"Tolerance to loud sounds" [59].                           Pt SS

"Intolerance to the loudness of sounds that most           Pt 1st
individuals deem to be tolerable" [21].

"Unusual tolerance to ordinary environmental
sounds" or as "consistently exaggerated or inappropriate   Pt 1st
responses to sounds that are neither
threatening nor uncomfortably loud to typical
person" [19].

"Unusual intolerance to ordinary environmental             Pt 1st
sounds" [18].

"An unusual intolerance to ordinary environmental          Pt 1st
sounds" [41].


"A subject exhibits negative reactions
following exposure to sound that
would not evoke the same
response in an average listener                            Pt 1st/2nd

[...] when negative reactions to a
sound depend only on its physical
characteristics" [25].

"An abnormally strong response
within the auditory pathways                               Pt 1st
resulting from exposure to
moderate sound" [22].

"An abnormally strong reaction to
sound occurring within the auditory                        Pt SS
pathways" [36].

"An abnormal, generally painful
perception of loudness in relation                         Pt 1st
to all normal external noise" [23].


"Disproportionate growth in subjective loudness
of sounds. [... ] response on the part of an individual
that sounds judged "soft" or "comfortable" to a listener   Pt SS
with normal hearing are judged "uncomfortable" or
"painful" to a patient experiencing hyperacusis... [49].

"Clinically significant, often highly distressing          Pt 2nd
oversensitivity to common external sounds" [20].

"Auditory hypersensitivity" [46].                          Pt 2nd

"Bothersome loudness perception at moderate levels         GPb 1st
[... ] referred to as auditory hypersensitivity" [39].

"Unusual hypersensitivity or discomfort induced by         Pt 1st
exposure to sound" [55].

"Oversensitivity to sound" [35].                           Pt SS

"Hypersensitivity to loud sounds" [29].                    Pt 2nd

"Abnormal sensitivity to sound, extreme sensitivity
and instability to some sounds within 3 dB of              Pt 1st/SS
thresholds" [42].

"An increased sensitivity to auditory stimulation"         Pt SS

"Hypersensitivity and discomfort in the presence of
common environmental noises (i.e. telephone,               Pt SS
electric mixer) that would not trouble a normal
person" [40].

Study population

Pt = patients recruited through

GPb = general public


1st = hyperacusis primary

2nd = hyperacusis secondary

SS = hyperacusis within a
symptom set
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Author:Fackrell, Kathryn; Potgieter, Iskra; Shekhawat, Giriraj S.; Baguley, David M.; Sereda, Magdalena; Ho
Publication:BioMed Research International
Article Type:Report
Date:Jan 1, 2017
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