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 hyperacusisA 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

WDRC amplification sometimes led to better performance than linear amplification with peak clipping or output limiting, and it never led to poorer performance. Therefore, it appears to be safe to use well-designed WDRC for hearing-impaired children with severe or profound hearing loss.

ObjectiveTo determine research priorities in hyperacusis that key stakeholders agree are the most important.Design/settingA priority setting partnership using two international surveys, and a UK prioritisation workshop, adhering to the six-staged methodology outlined by the James Lind Alliance.ParticipantsPeople with lived experience of hyperacusis, parents/carers, family and friends, educational professionals and healthcare professionals who support and/or treat adults and children who experience hyperacusis, including but not limited to surgeons, audiologists, psychologists and hearing therapists.MethodsThe priority setting partnership was conducted from August 2017 to July 2018. An international identification survey asked respondents to submit any questions/uncertainties about hyperacusis. Uncertainties were categorised, refined and rephrased into representative indicative questions using thematic analysis techniques. These questions were verified as ‘unanswered’ through searches of current evidence. A second international survey asked respondents to vote for their top 10 priority questions. A shortlist of questions that represented votes from all stakeholder groups was prioritised into a top 10 at the final prioritisation workshop (UK).ResultsIn the identification survey, 312 respondents submitted 2730 uncertainties. Of those uncertainties, 593 were removed as out of scope, and the remaining were refined into 85 indicative questions. None of the indicative questions had already been answered in research. The second survey collected votes from 327 respondents, which resulted in a shortlist of 28 representative questions for the final workshop. Consensus was reached on the top 10 priorities for future research, including identifying causes and underlying mechanisms, effective management and training for healthcare professionals.ConclusionsThese priorities were identified and shaped by people with lived experience, parents/carers and healthcare professionals, and as such are an essential resource for directing future research in hyperacusis. Researchers and funders should focus on addressing these priorities.

We compared the effectiveness of three procedures for the initial fitting of hearing aids with multi-band compression: (1) CAMEQ, which aims to amplify speech so as to give equal loudness per critical band over the frequency range important for speech intelligibility, and to give similar overall loudness to 'normal': (2) CAMREST, which aims to amplify speech so as to restore 'normal' specific loudness patterns, over a wide range of speech levels; (3) DSL I/O, which aims to map the dynamic range of normally hearing people into the reduced dynamic range of hearing-impaired people, with 'full' restoration of audibility. Ten experienced hearing aid users with moderate sensorineural loss were fitted bilaterally with Danalogic 163D digital hearing aids, using each procedure in turn; the order was counterbalanced across subjects. The fitting required specification of gains for input levels of 55 and 80 dB SPL at six centre frequencies. Real-ear measurements were made to ensure that target gains were reached (+/-3 dB). Immediately after fitting with a given procedure, and one week after fitting, the gains were adjusted when required by the minimum amount necessary to achieve acceptable fittings. The amount of adjustment required provides one measure of the adequacy of the initial fitting. On average, the adjustments were smallest for the CAMEQ procedure. The gain changes were slightly larger for the CAMREST procedure and were largest of all for DSL I/O. For the latter, the gain changes were mostly negative, especially for high frequencies and the higher input level. This indicates that the DSL I/O procedure prescribes more high-frequency gain than is preferred by adult users. After these gain adjustments, users wore the aids for at least three weeks before filling out the APHAB questionnaire and taking part in laboratory measurements of the speech reception threshold (SRT) for sentences in quiet and in steady and fluctuating background noise at levels of 60 and 75 dB SPL. Following these tests, the hearing aids were re-fitted with the next procedure. The scores on the APHAB test and the SRTs did not differ significantly for the three procedures. We conclude that the CAMEQ and CAMREST procedures provide more appropriate initial fittings than DSL I/O.

We assessed whether gain requirements differ for experienced users and new users when fitted with multi-band compression hearing aids Three procedures for initial fitting were used: the Cambridge method for loudness equalization (CAMEQ), the Cambridge method for loudness restoration (CAMREST), and the desired sensation level input/output (DSL[i/o]) method. Twenty experienced hearing aid users and 20 new users with mild-to-severe sensorineural loss were fitted with Danalogic 163D digital hearing aids, using each procedure in turn in a counter-balanced order. The new users were given a pre-fitting with slightly reduced gains prior to the 'formal' fitting. Immediately after formal fitting with a given procedure, and 1 week after fitting, the gains were adjusted by the minimum amount necessary to achieve acceptable fittings. The amount of adjustment required provided the main measure of the adequacy of the initial fitting. On average, new users required decreases in gain for all procedures, the decreases being larger for DSL[i/o] than for CAMEQ or CAMREST. For experienced users, gain adjustments were small for CAMEQ and CAMREST, but were larger and mostly negative for DSL[i/o]. After these gain adjustments, users wore the aids for at least 3 weeks before filling out the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire and taking part in laboratory measurements of the speech reception threshold (SRT) for sentences in quiet and in steady and fluctuating background noise at levels of 60 and 75 dBSPL. The scores on the APHAB test and the SRTs did not differ significantly for the three procedures. We conclude that the CAMEQ and CAMREST procedures provide more appropriate initial fittings than DSL[i/o]. For inexperienced users, gains typically need to be reduced by about 3dB relative to those prescribed by CAMEQ or CAMREST, although the amount of reduction may depend on hearing loss. An analysis of gain adjustments as a function of order of testing provided some evidence for increased tolerance to high-frequency amplification with increasing experience during the 4-month course of the trial, but this effect did not differ for the experienced and new users.