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PubMed2003

[Hyperacusis, phonophobia, and recruitment. Abnormal deviations of hearing associated with hypersensitivity to sound].

Hypersensitivity to sound is a common description of distinct nosological phenomena of peripheral and central hearing disorders, which are characterized by intense suffering from the acoustic environment. One can distinguish between recruitment accompanying inner ear hearing loss, hyperacusis with a general hypersensitivity to sound of any frequency, and phonophobia as an anxious sensitivity towards specific sound largely independent of its volume. While recruitment can be described as a peripheral reaction caused by a lack of outer hair cell moderation, hyperacusis and phonophobia represent disturbances of central auditory processing without peripheral pathology, often combined with psychosomatic reactions. Due to insufficient efferent inhibition, hyperacusis often follows psychovegetative exhaustion. In cases of phonophobia, peripheral and efferent hearing functions are usually intact, but certain learning (conditioning) processes lead to development of specific reactions and avoidance patterns to certain content-related acoustic stimuli. This article describes those different phenomena with regard to their clinical appearance, diagnostics, and possibilities for therapy.

Anxiety↗
PubMed2001

[Hyperacusis after tympanoplasty].

A case of hyperacusis after tympanoplasty is presented. A 62-year-old woman experienced unilateral conductive hearing loss for about 60 years after antrotomy in infancy. Neurological or otoneurological symptoms were not evident. Tympanotomy showed a missing incus with discontinuity of the chain. Reconstruction was performed by interposition of a partial ossicular replacement prosthesis (PORP). The postoperative audiogram revealed minimal perceptive hearing loss in the high-frequency field in the operated ear. Several weeks after middle ear surgery, the patient complained of hyperacusis on the operated side, which persisted for more than 2 years. To alleviate the unpleasant sensations, an ear plug was used by the patient. In spite of the asymmetric hearing loss, no late onset auditory deprivation could be diagnosed. The pathophysiological causes of the hyperacusis have not yet been clarified. Possible reasons are discussed.

Audiometry, Pure-Tone↗
PubMed2005

Aversion, awareness, and attraction: investigating claims of hyperacusis in the Williams syndrome phenotype.

BACKGROUND: Williams syndrome (WS), a neurodevelopmental disorder, is characterized by pervasive cognitive deficits alongside a relative sparing of auditory perception and cognition. A frequent characteristic of the phenotype is adverse reactions to, and/or fascination with, certain sounds. Previously published reports indicate that people with WS experience hyperacusis, yet careful examination reveals that the term 'hyperacusis' has been used indiscriminately in the literature to describe quite different auditory abnormalities. METHOD: In an effort to clarify and document the incidence of auditory abnormalities in and among people with WS we collected data from parents of people with WS (n = 118) and comparison groups of people with Down syndrome, autism, and normal controls. RESULTS: Our findings revealed four phenomenologically separate auditory abnormalities, all of which were significantly more prevalent in WS than the three comparison groups. Among people with WS, we found relatively few reports of true hyperacusis (lowered threshold for soft sounds) or auditory fascinations/fixations, whereas 80% reported fearfulness to idiosyncratically particular sounds, and 91% reported lowered uncomfortable loudness levels, or 'odynacusis.' CONCLUSIONS: Our results confirm anecdotal reports of an unusual auditory phenotype in WS, and provide an important foundation for understanding the nature of auditory experience and pathology in WS. We conclude by reviewing the ways in which the present findings extend and complement recent neuroanatomical and neurophysiological findings on auditory function in people with WS.

Awareness↗
PubMed2004

The relationship of tinnitus, hyperacusis, and hearing loss.

The triad of tinnitus, hyperacusis, and hearing loss remains an often-underdiagnosed combination of symptoms that causes physical, mental, and emotional distress for millions of patients. To the best of our knowledge, no review has heretofore been published in the literature regarding the possible relationship among these three entities. We believe that these symptoms may have a common pathophysiology. Specifically, improper function of cochlear hair cells may result in a hearing loss secondary to the failure of these cells to propagate proper signals through the auditory centers. In response to an incongruous neural message, higher auditory cortical centers may adapt and remodel transmitted sound. This neuroplasticity may lead to an increased perception of volume in the auditory cortex (hyperacusis) and to the perception of phantom sounds (tinnitus). Awareness of the potential relationship among tinnitus, hyperacusis, and hearing loss may contribute to improved diagnosis, treatment, and follow-up for patients with these conditions.

Hearing Disorders↗
PubMedDate not supplied

[Hyperacusis--an unexplored field. Cognitive behavior therapy can relieve problems in auditory intolerance, a condition with many questions].

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.

Biomedical Research↗
PubMed1987

Pathophysiology of tinnitus: a special case--hyperacusis and a proposed treatment.

The occurrence of hyperacusis is rare. In our Tinnitus Clinic, where more than 4,000 patients have been seen, hyperacusis has been seen only four times. Treatment of hyperacusis has been a combination of protection from external sounds along with desensitization to sound. Desensitization has been produced by very low intensity masking which is gradually increased every two to four weeks. The process of desensitization requires a great deal of time.

Adult↗
PubMed2001

Experiences in the treatment of patients with tinnitus and/or hyperacusis using the habituation method.

The purpose of this study was to summarize the results achieved by patients with tinnitus and/or hyperacusis during the Tinnitus Retraining Therapy. One hundred cases, out of 516 patients registered until January 1999 at the Tinnitus and Hyperacusis Management Clinic, Warsaw, Poland, were examined. The patients have been treated for at least 10 months but not longer than 1 year. A questionnaire specially prepared for this program was used to assess the results. In the group of 100 cases, the results after a minimum of 10 months' therapy are different in each category but they show a significant improvement in about 70% of cases.

Female↗
PubMed2005

Daily living with hyperacusis due to head injury 1 year after a treatment programme at the hearing clinic.

The aim of this study was to gain a deeper understanding of the quality of daily living of persons with hyperacusis after a traumatic head injury 1 year after these persons had participated in a treatment programme at a Swedish hearing clinic. The study group consisted of 21 patients with a mean age of 36 years. In-depth interviews, conducted 1 year after the treatment programme, were analysed using the grounded theory method. Five emergent categories were labelled moderating vulnerability, awareness of restrictions, conditioned participation, structuring daily life and controlling mood changes. The core category, moderating vulnerability, describes the necessary balancing act between activity and recovery. The informants were aware of their new restrictions and managed daily life by structuring and planning each day in detail to minimize exposure to sensory stimuli. They had learned to prioritize their activities and, thereby, rationed their time. The evaluated programme appears to have positive effects and facilitates patients' adjustment process to hyperacusis with relatively restricted costs for the society.

Adaptation, Psychological↗
PubMed2002

Central hyperacusis with phonophobia in multiple sclerosis.

Hearing disorders are a well-described symptom in patients with multiple sclerosis (MS). Unilateral or bilateral hyperacusis or deafness in patients with normal sound audiometry is often attributed to demyelinating lesions in the central auditory pathway. Less known in MS is a central phonophobia, whereby acoustic stimuli provoke unpleasant and painful paresthesia and lead to the corresponding avoidance behaviour. In our comparison collective, patient 1 described acute shooting pain attacks in his right cheek each time set off by the ringing of the telephone. Patient 2 complained of intensified, unbearable noise sensations when hearing nonlanguage acoustic stimuli. Patient 3 noticed hearing unpleasant echoes and disorders of the directional hearing. All patients had a clinical brainstem syndrome. ENT inspection, sound audiometry and stapedius reflex were normal. All three patients had pathologically changed auditory evoked potentials (AEPs) with indications of a brainstem lesion, and in magnetic resonance imaging (MRI) demyelinating lesions in the ipsilateral pons and in the central auditory pathway. The origin we presume in case 1 is an abnormal impulse conduction from the leminiscus lateralis to the central trigeminus pathway and, in the other cases, a disturbance in the central sensory modulation. All patients developed in the further course a clinically definite MS. Having excluded peripheral causes for a hyperacusis, such as, e.g., an idiopathic facial nerve palsy or myasthenia gravis, one should always consider the possibility of MS in a case of central phonophobia. Therapeutic possibilities include the giving of serotonin reuptake inhibitors or acoustic lenses for clearly definable disturbing frequencies.

Adult↗
PubMed2000

Evaluation and treatment of severe hyperacusis.

A 52-year-old male was evaluated by the authors after initially reporting fullness in his left ear while traveling on an airplane. A unique feature of the patient's complaint was the development of severe bilateral hyperacusis (loudness discomfort levels of between 20-34 dB HL) in spite of the fact that the hearing loss was initially reported in the left ear. To achieve loudness comfort, the patient was initially fit with ER-25 musician earplugs that proved to be unsuccessful. The patient next purchased earplugs and earmuffs from a gun shop in order to obtain relief from the pain and discomfort caused by his exposure to everyday environmental sounds. This paper describes the use of hearing devices that proved to be effective in providing attenuation sufficient that the patient rarely needs to rely on earplugs and earmuffs for relief from his hyperacusis.

Anti-Inflammatory Agents↗
PubMed2000

[Subjective ear noise and hyperacusis. Treatment with fluctuating currents].

The prevalence of hyperacusis, and subjective ear noise in particular, tends to increase in the general population. The data available in the literature show that physiotherapeutical procedures are the most effective treatment of this abnormality. The techniques and devices using fluctuating currents were employed to treat 40 patients. A "SLUKH-OTO-1" (HEARING-OTO) device is of use now. This procedure proved to be the most effective in case of hyperacusis and subjective ear noise without hearing disorder, there was also a high percentage of positive results in chronic neurosensory hypoacusis. Treatment of subjective ear noise by this procedure is not so effective in otosclerosis and chronic otitis media.

Combined Modality Therapy↗
PubMed2000

Hyperacusis: case studies and evaluation of electronic loudness suppression devices as a treatment approach.

Hyperacusis, as defined here, is a relatively rare condition in which the patient, with or without hearing loss, experiences severe loudness discomfort to everyday environmental sound levels. The case studies of 14 patients with severe hyperacusis are described; all wore passive attenuators (earplugs and/or earmuffs) in an attempt to alleviate their discomfort, frequently producing communication difficulties. These subjects were fitted binaurally with experimental electronic loudness suppression devices housed in in-the-ear casings. The devices supplied low-level amplification followed by an extreme form of amplitude compression for moderate or high-level inputs in an attempt to reduce loudness discomfort without reducing audibility. Many of the subjects were found to function with a wider dynamic range with the active devices compared with passive attenuators or the unoccluded ear, and most reported that they benefited from the devices in at least some listening situations.

Adult↗
PubMed1984

Exaggerated acoustic reflex response in a patient with transient facial palsy and hyperacusis.

An unusual acoustic reflex response was documented by immittance recording in a patient with acute facial palsy. The amplitude of the stapedial response to sustained stimulation was reduced when recorded from the affected side, suggesting the facial nerve was affected proximal to the stapedius muscle. In addition, the impedance change at onset of intense acoustic stimulation of the affected side was unusually large, regardless of which stapedius response was recorded. The large change was coincident with the reported hyperacusis in the stimulated ear. This suggested the cochlear nerve as site of involvement of hyperacusis in this case of facial palsy.

Adult↗
PubMed2001

[Central hyperacusis with phonophobia in multiple sclerosis].

Auditory disturbances are a well known symptom in patients with multiple sclerosis (MS). Uni- or bilateral hypacusis or deafness in patients with normal auditory testing is considered to be a result of lesions in the central auditory pathway. Only rarely described is a central phonophobia whereby acoustic stimuli induce unpleasant and painful perceptions, with consecutive avoidance of these factors. Our first patient described acute shooting pain in the right cheek, triggered only through the ringing of a telephone. The second patient had uncomfortable perception of nonverbal noise. For example the wrinkling of paper bags was unbearable for him. The third patient had difficulties localizing the source of sound and disturbing echos while listening to speech or music. Clinically, in all patients symptoms of a brainstem syndrome were found, whereas auditory testing including inspection, audiometry, and stapedius reflex was normal. We found pathological acoustic evoked potentials (AEP) in all three patients with a prolonged latency III-V and T2 lesions in the ipsilateral pons and central auditory pathway. In case one, we suppose a lateral spread between the lateral lemniscus and the central trigeminal pathway. In the other cases, a dysfunction of the central sensory modulation which controls the regulation of sensitivity of incoming acoustic stimuli seems to be the cause of hyperacusis. All our patients developed clinically confirmed MS in the further course after suffering from phonophobia as their first symptom.

Acoustic Stimulation↗
PubMed2002

[The assessment of subjective distress related to hyperacusis with a self-rating questionnaire on hypersensitivity to sound].

BACKGROUND: So far there has been no adequate measure to assess or illustrate, in terms of different levels, subjective distress related to hypersensitivity to sound. METHOD AND PATIENTS: The here presented work describes and discusses the construction of a questionnaire to assess subjective distress related to hypersensitivity to sound (GUF). Between May and September 2000 226 patients that experienced suffering from hypersensitivity to sound as well as from chronic tinnitus, completed a first version of the questionnaire on admittance to the hospital. Of these patients 27.9 % were out-patients and 72.1 % were in-patients. In addition, the in-patients completed the questionnaire again during their last week of treatment. The 27 items of the GUF were interpreted by factor analysis to explore and determine the structure of the questionnaire; the number of items was reduced under the aspects of consistency and reliability. Finally, the revised version of the GUF underwent a first validation. RESULTS: The factor analysis shows three factors explaining 50.65 % variance (factor 1 [KRH], cognitive reactions to hyperacusis; factor 2 [ASV], actional/somatic behaviour; factor 3 [ERG], emotional reaction to external noises). First attempts to validate the questionnaire are promising; it appears that the GUF is also sensitive to therapy effects. CONCLUSIONS: The here presented questionnaire is suitable for identifying distinct levels of subjective distress related to hypersensitivity to sound. Thus, for the first time, there is an adequate measure for assessment available. Furthermore, results of part of the sample show that the GUF is also suitable for therapy evaluation.

Adaptation, Psychological↗
PubMed2002

Middle cerebral artery aneurysm presenting as isolated hyperacusis.

We present the first case of a middle cerebral artery aneurysm presenting as isolated hyperacusis. The patient had a Guglielmi detachable coil (GDC) embolization of his aneurysm with complete resolution of his symptoms. The pathophysiological mechanism is discussed. We suggest further radiological investigation in young patients presenting with this symptom.

Adult↗
PubMed2005

Dysfunction of the auditory efferent system in patients with traumatic brain injuries with tinnitus and hyperacusis.

OBJECTIVES: Tinnitus, hyperacusis and difficulty listening in background noise are common symptoms reported by patients with traumatic brain injury (TBI). The aim of this study was to explore the function of the auditory system in TBI patients with and without auditory complaints but having normal pure-tone audiograms. METHODS: The study consisted of 24 TBI patients with and 10 TBI patients without auditory complaints. In addition, 15 normal controls were included in the study. The function of the auditory system was tested by recording transient otoacoustic emissions (TEOAE) during the presentation of increasing levels of white noise in the contralateral ear. RESULTS: Most of the TBI patients with auditory complaints (87%) showed absent or significantly reduced effect of the auditory efferent system as compared with the TBI patients without auditory complaints and to normal controls. However, the global amplitude of the TEOAE was significantly higher in TBI patients with auditory complaints compared to those without. CONCLUSIONS: Due to its role in peripheral and central auditory activity, dysfunction of the efferent system may be at least partially responsible for these auditory complaints. This study underscores the importance of testing and evaluating the functional integrity of the medial efferent system by an objective and non-invasive method in patients with TBI.

Acoustic Stimulation↗