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Hyperacusis is defined as unusual intolerance to ordinary environmental sounds, and is commonly reported in the general population and in association with a range of medical conditions. These include neurological deficits (e.g. migraine), psychiatric conditions (e.g. depression), and several ear, nose and throat diagnoses such as tinnitus, noise-induced hearing loss, and middle ear malfunctions. However, extreme sensitivity to noise has also been studied from a public health perspective, but with a focus on noise sensitivity in general. In this review a distinction is proposed between three different aspects of the experience of hyperacusis. The first is the sensitivity, with the pain sensations reported in association with sounds. The second is the annoyance, which can be unrelated to loudness, but still cause marked distress. The third aspect deals with the fear of being harmed by sounds, which promotes avoidance and the unmotivated use of ear protection. The natural course of hyperacusis is largely unknown and there are no published randomized outcome studies on the available treatment options for the condition. In this paper we propose that cognitive behavioral therapy, presented in a multidisciplinary setting, could be a useful treatment. This treatment includes relaxation methods, advice regarding sound, and gradual exposure to everyday sounds.