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PubMed1996

[Dysfunction of medial olivocochlear system and its audiological test].

The function of medial olivocochlear system (MOC) in patients with three kinds of auditory diseases was tested in order to investigate the relationship between MOC and retrocochlear impairment. Suppressive effect of evoked otoacoustic emissions (OAEs) by contralateral white noise was used as the indicator to evaluate the function of MOC in patients with: (1) sensorineural deafness with definite otoacoustic emissions, (2) hyperacusis and (3) tinnitus. Dysfunction of MOC was found in nine cases out of sixteen patients with sensorineural deafness and six cases out of fifteen patients with tinnitus. Two cases of hyperacusis also showed MOC dysfunction. It was concluded that MOC dysfunction may exist in some patients with sensorineural hearing loss and tinnitus, and in patients with hyperacusis as well. It seemed likely that dysfunction of MOC may played a role in causing tinnitus and hyperacusis in these patients. Testing the function of MOC may provide a helpful way for differential diagnosis of cochlear and retrocochlear impairment in auditory system.

Adolescent↗
PubMed2006

Tinnitus and Ménière's disease: characteristics and prognosis in a tinnitus clinic sample.

The aim of this study is to describe tinnitus epidemiological characteristics in Ménière's disease (MD), the prognosis and its interaction with other MD symptoms. It is a descriptive transversal study. One hundred two MD patients were referred to a tinnitus clinic. Tinnitus was evaluated according to psychoacoustical tinnitogram, visual analogue scale, and tinnitus handicap inventory (THI). All patients were diagnosed with definitive MD (following AAO-HNS' 95 criteria). Pitch was more commonly identified in low and medium frequencies. Psychoacoustical intensity was matched in 12.7 dB. The visual analogue scale showed a value of 7.1, while THI score reached an average of 49%. Compared with other aetiologies (acoustic trauma, otosclerosis), MD showed a statistical difference in tinnitus severity parameters (P<0.05). Tinnitus increased VAS and THI score as a function of duration and bilateral disease (P<0.01). We found a statistical association (P<0.05) between tinnitus intensity and worse hearing loss or hyperacusis, but it was not influenced by number of vertigo spells. Higher MD stage increased tinnitus intensity and handicap. Tinnitus in MD patients referred to our tinnitus clinic presents a high intensity and handicap levels and represents the most troublesome symptom. Severity is influenced by the longer duration of the disease, the bilateral affection, hearing impairment or hyperacusis and a higher MD stage.

Adolescent↗
PubMed2006

Acoustic shock injury (ASI).

CONCLUSION: The potential severity and persistence of ASI symptoms has significant clinical and medico-legal implications. With the rapid growth of call centres around the world, professionals providing tinnitus and hyperacusis therapy are increasingly likely to encounter some or all of the cluster of ASI symptoms in their clients. BACKGROUND: Acoustic shock injury (ASI), occurring as a result of exposure to a sudden unexpected loud sound, has been observed to cause a specific and consistent pattern of neurophysiological and psychological symptoms. These include aural pain, tinnitus, hyperacusis/phonophobia, vertigo and other unusual symptoms such as numbness or burning sensations around the ear. A range of emotional reactions including trauma, anxiety and depression can develop. Call centre staff using a telephone headset or handset are vulnerable to ASI because of the increased likelihood of exposure, close to their ear(s), of sudden unexpected loud sounds randomly transmitted via the telephone line. DISCUSSION: This paper presents an overview of a study of 103 people exposed to 123 acoustic incidents, and of the proposed neurophysiological mechanism of ASI, in particular tonic tensor tympani syndrome (TTTS). An understanding of TTTS has the potential to provide insight into the neurophysiological and psychological development of tinnitus and hyperacusis and the association with high levels of emotional trauma and anxiety. REHABILITATION: ASI rehabilitation is discussed.

Anxiety↗
PubMed2002

[Tinnitus Center at the Nofer Institute of Occupational Medicine--earliest experience].

Of the 150 patients admitted in 2001 to the Tinnitus Center located at the Nofer Institute of Occupational Medicine, Łódź, Poland, 80 were subjected to complex examinations consisted of standardized questionnaire on medical history, psychological tests and audiological assessment. The diagnostic procedure was completed for 52 patients (23 females and 29 males; mean age: 53 years). In this group, five patients were found to have conductive hearing loss due to chronic eustachtis or otosclerosis. They were excluded from further studies. Among the other 47 patients, 26 showed normal hearing threshold and 21 suffered from uni- or bilateral sensorineural hearing loss. Hyperacusis was diagnosed in 16 cases. The measurements of brainstem evoked potentials revealed V wave latency asymmetry in 7 cases, which implied the necessity to perform CT or MNR. In neither of cases did this diagnosis confirm the suspected tumor development (n. VIII neurinoma or pontocerebral angle tumor. The preliminary assessment of treatment efficacy for subjective tinnitus with use of retraining therapy yielded the following conclusions: 1. The application of hearing aid brings about an immediate improvement in the patient's self-assessment of hearing and a better tolerance towards tinnitus. 2. A systematic all-day wear of noise generators contributes to the patient's increased tolerance towards tinnitus, improved mental condition and alleviated hyperacusis. 3. The efficacy of the tinnitus retraining therapy, following Jastreboff, depends on providing the patient with detailed information on the causes and mechanisms of tinnitus development. 4. The negative diagnostics for tumor within the cranial cavity has not only a soothing effect on the patient as it relieves his/her stress, but it can also be a good starting point for the tinnitus retraining therapy.

Adult↗
PubMed2006

Factors influencing tinnitus loudness and annoyance.

OBJECTIVE: To evaluate the 2 major components of tinnitus severity, loudness and annoyance, and their degree of dependence on characteristics of tinnitus manifestation, history, and etiology. DESIGN: Cross-sectional survey performed during the first months of 2004. SETTING: Nonclinical population. PARTICIPANTS: A total of 4995 members of the German Tinnitus League. MAIN OUTCOME MEASURES: Comprehensive screening questionnaire, including the Klockhoff and Lindblom loudness grading system and the miniversion of the Tinnitus Questionnaire. RESULTS: A moderate correlation of 0.45 was found between tinnitus loudness and annoyance. Both factors were generally higher in men, those older than 50 years, those with binaural and centrally perceived tinnitus, those with increased noise sensitivity, and those who had continuous tinnitus without interruptions. Tinnitus that lasted 12 months or less had a stronger influence on annoyance (odds ratio [OR], 1.96) than on loudness (OR, 0.45), whereas the contrary was found for tinnitus of more than 5 years' duration (ORs, 0.72 and 2.11, respectively). Loudness and annoyance were increased in subjects with coexisting hearing loss, vertigo, and hyperacusis. The impact of hyperacusis on annoyance was clearly stronger than on loudness (ORs, 21.91 vs 9.47). CONCLUSIONS: Several clinical factors of tinnitus influence perceived loudness and annoyance. Both are distinguishable components of tinnitus severity.

Adolescent↗
PubMed1976

Audiotympanometric findings in myasthenia gravis.

Myasthenia gravis can be a difficult diagnostic and therapeutic problem. Our study on six patients consisted of pure-tone audiograms, tympanometry, and acoustic reflex tests. Positive findings in the myasthenia gravis patients prior to medication included hyperacusis and increase in the intensity of sound required to elicit an acoustic reflex. Following medication, the hyperacusis lessened and the intensity of sound required to elicit the acoustic reflex decreased. During follow-up of two of the patients we encountered cholinergic (overdosage) crisis in one patient and myasthenic (underdosage) crisis in the other patient. Muscle weakness can be a common factor in both conditions. The acoustic reflex test can differentiate between the two types of crises. We believe audiotympanometric tests can aid in diagnosis and treatment of myasthenia gravis patients.

Acoustic Stimulation↗
PubMed2001

Effects of tinnitus retraining therapy (TRT) for patients with tinnitus and subjective hearing loss versus tinnitus only.

The patients with tinnitus and/or hyperacusis undergoing an 18-24 month period of TRT are divided into five categories of treatment. Different types of counselling and sound therapy are used in each category. Selection of patients into a specific category depends on such factors as: hyperacusis, subjective hearing loss and long-lasting effect of noise on tinnitus. The 108 cases were evaluated After 1 year of treatment. The results of therapy of 40 patients with tinnitus and subjective hearing loss (category II) were compared with the results of therapy of patients with tinnitus only (categories 0 and I). A special questionnaire, answered before and during the treatment, was used to assess the results. Our data indicate significant improvement in about 70% of patients with tinnitus only and in about 90% of patients with tinnitus and subjective hearing loss after one year of therapy.

Female↗
PubMed2004

Pathophysiological changes of the central auditory pathway after blunt trauma of the head.

It is the aim of the present paper to correlate clinical symptoms of auditory dysfunction (tinnitus, hyperacusis, hearing loss) one year on average after a blunt trauma of the head with objective audiological test results (otoacoustic emission and auditory brainstem response testing, impedance audiometry) and to compare these findings to controls without history of head trauma. Thirty-one patients (24-56 years) were included. They were largely female (n = 26). The clinical and otolaryngological examination (including otoscopy) of all patients revealed no pathological abnormalities. Pure-tone audiograms were normal with one exception (pre-existing noise-induced hearing loss) as well as tympanograms. The main auditory symptoms were tinnitus (n = 9), hyperacusis (n = 2) and a reported transient hearing loss immediately after the trauma (n = 16) (which had improved at the time of examination). The results of testing the central auditory pathway showed that the transiently evoked otoacoustic emissions (otoemissions) revealed statistically significant differences between amplitude differences of all patients as well as patients with tinnitus and controls in the linear, but not in the non-linear stimulation mode. A complete loss of stapedial reflex responses was found in 12 of the patients and a partial (irregular) loss (in at least more than two frequencies) in four additional patients. Auditory brainstem responses (ABR) were normal in all patients, but 76% had lowered loudness discomfort levels (LDL). Blunt trauma of the head can lead to auditory dyfunction, probably as a result of diffuse axonal injury of the central auditory pathway. An initial sensorineural hearing loss after the trauma (as a result of the inner ear fluid concussion) was transiently reported only. Auditory symptoms play a minor role in the so-called "postconcussive syndrome," but should be considered and evaluated fully.

Adult↗
PubMed1992

GM1 gangliosidosis type 2 in two siblings.

A sister and brother, now aged 7 and 9 years, presented with developmental arrest, gait disturbance, dementia, and a progressive myoclonic epilepsy syndrome with hyperacusis in the second year of life. Then, spastic quadriparesis led to a decerebrate state. In the absence of macular or retinal degeneration, organomegaly, and somatic-facial features suggesting mucopolysaccharidosis, the presence of hyperacusis together with sea-blue histiocytes in bone marrow biopsies and deficient beta-galactosidase activity but normal glucosidase, hexosaminidase, and neuraminidase activity on lysosomal enzyme assays constitutes the clinical-pathologic-biochemical profile of GM1 gangliosidosis type 2. This is a rare, late infantile onset, progressive gray-matter disease in which beta-galactosidase deficiency is largely localized to the brain, though it can be demonstrated in leukocytes and cultured skin fibroblasts. It must be distinguished from the Jansky-Bielschowsky presentation of neuronal ceroid lipofuscinosis, mitochondrial encephalopathy, lactic acidosis, strokelike episodes (MELAS) and myoclonic epilepsy with ragged-red fibers (MERRF) syndromes, atypical presentations of GM2 gangliosidoses (Tay-Sachs and Sandhoff's diseases), primary sialidosis (neuraminidase deficiency), galactosialidosis, and Alpers' disease.

Biopsy↗
PubMed1984

Meniere's disease and fibrositis syndrome (psychogenic rheumatism). Relationship in audiometric and nystagmographic results.

A prospective neuro-otological study concerning 30 cases of fibrositis syndrome (psychogenic rheumatism, PR) and 30 age-matched normal controls was made and a retrospective study concerning 33 cases of Meniere's disease (MD) diagnosed and followed-up, examined between 1965 and 1982. Results showed: sensorineural hearing loss at low frequencies in all early stages of MD and in 10/30 of PR; hyperacusis (pain threshold below 100 dBHL bilaterally for all frequencies) without other sign of recruitment in 73.3% of PR and in 3/4 cases of MD where it was measured (discomfort or vertigo due to noise was noted retrospectively in 16/33 of early stages of MD); hyperreactivity of per-rotatory nystagmus in 53.3% of PR without neurological or peripheral vestibular lesions and, with or without vestibular unilateral lesions, in 39.9% of MD. None of the controls showed hyperacusis, hyperreactivity of per-rotatory nystagmus or deafness at low frequencies.

Adult↗
PubMed1999

Williams syndrome and the elastin gene in Thai patients.

Williams syndrome (WS) has long been known as a complex disorder of dysmorphic facial features, described as elfin face, mental retardation or learning disability, loquacious personality, and supravalvular aortic stenosis. The etiology is now known to be due to deletion of the elastin gene (ELN) on long arm of chromosome 7. Thai patients were previously reported by clinical diagnosis. This study reports the first two cases of WS with ELN deletion diagnosed by fluorescent in situ hybridization (FISH) technique. Clinically, hyperacusis is a common finding in WS associated with otitis media. Neither of the patients had hyperacusis, but one of them had bilateral sensorineural hearing loss, which to our knowledge, has never been reported.

Elastin↗
PubMed1999

Endogenous dynorphins: possible role in peripheral tinnitus.

Tinnitus has been defined as the perceptual correlate of altered spontaneous neural activity occurring without an external auditory stimulus. Hyperacusis, defined as a collapse of tolerance to sound, is present in 40-86% of those who suffer from disabling forms of tinnitus. Both phenomena often are induced or exacerbated by physical or psychological stress. Biological systems known to regulate the body's overall response to stress use and release endogenous neuroactive opioid peptides. These stress-related neuromodulators consist of products derived from three genetically distinct precursor hormones. Two of these precursor hormones are proenkephalin and prodynorphin. Enkephalin and dynorphin-related peptides exist within the efferent olivocochlear systems (lateral and medial) of several mammalian species, including humans. Prodynorphin derivatives, however, may be restricted exclusively to lateral efferent neurons. Descending lateral efferent axons terminate solely on primary (type I) auditory dendrites innervating cochlear inner hair cells in most species. This action indicates that they play an important role in modulating auditory nerve sensitivity and spontaneous discharge. In a fashion similar to that exhibited by the observed excitatory mechanism of action of dynorphins in the spinal cord, sodium salicylate (aspirin) recently was shown to facilitate the excitatory effects of glutamate in the cochlea. This article provides support for a neurochemical model in which endogenous dynorphins may induce hyperacusis and can contribute to the induction, maintenance, or exacerbation of tinnitus in the auditory periphery by altering auditory type I neural excitability to glutamate.

Cochlea↗
PubMed1992

Prediction of recovery of Bell's palsy from clinical manifestations.

Sixty patients of Bell's palsy aged between 8 and 72 years, comprising 31 males and 29 females, were studied clinically to find out a method of prediction of recovery in early stage. It was found that young patients with incomplete palsy, unaccompanied by postauricular pain, loss of taste sensation over anterior 2/3rds of tongue hyperacusis and dry eye and recovery beginning within 4 weeks of onset of palsy are likely to make complete recovery, while older patients with complete palsy accompanied by severe postauricular pain, loss of taste sensation, hyperacusis and dry eye and beginning of recovery after 4 weeks of onset of palsy are most likely to have incomplete recovery.

Adolescent↗
PubMed1996

Neurophysiological approach to tinnitus patients.

The principal postulate of the neurophysiological model of tinnitus is that all levels of the auditory pathways and several nonauditory systems play essential roles in each case of tinnitus, stressing the dominance of nonauditory systems in determining the level of tinnitus annoyance. Thus it has been proposed to treat tinnitus by inducing and facilitating habituation to the tinnitus signal. The goal is to reach the stage at which, although patients may perceive tinnitus as unchanged when they focus on it, they are otherwise not aware of tinnitus. Furthermore, even when perceived, tinnitus does not evoke annoyance. Habituation is achieved by directive counseling combined with low-level, broad-band noise generated by wearable generators, and environmental sounds, according to a specific protocol. For habituation to occur, it is imperative to avoid masking tinnitus by these sounds. Since 1991, > 500 tinnitus patients have been seen in our center. About 40% exhibited hyperacusis to varying degrees. A survey of > 100 patients revealed > 80% of significant improvement in groups of patients treated with the full protocol involving counseling and the use of noise generators. Notably, in patients who received counseling only, the success rate was < 20%. The improvement in hyperacusis was observed in approximately 90% of treated patients.

Counseling↗
PubMed2003

Neural correlates of auditory perception in Williams syndrome: an fMRI study.

Williams syndrome (WS), a neurogenetic developmental disorder, is characterized by a rare fractionation of higher cortical functioning: selective preservation of certain complex faculties (language, music, face processing, and sociability) in contrast to marked and severe deficits in nearly every other cognitive domain (reasoning, spatial ability, motor coordination, arithmetic, problem solving). WS people are also known to suffer from hyperacusis and to experience heightened emotional reactions to music and certain classes of noise. We used functional magnetic resonance imaging to examine the neural basis of auditory processing of music and noise in WS patients and age-matched controls and found strikingly different patterns of neural organization between the groups. Those regions supporting music and noise processing in normal subjects were found not to be consistently activated in the WS participants (e.g., superior temporal and middle temporal gyri). Instead, the WS participants showed significantly reduced activation in the temporal lobes coupled with significantly greater activation in the right amygdala. In addition, WS participants (but not controls) showed a widely distributed network of activation in cortical and subcortical structures, including the brain stem, during music processing. Taken together with previous ERP and cytoarchitectonic studies, this first published report of WS using fMRI provides additional evidence of a different neurofunctional organization in WS people than normal people, which may help to explain their atypical reactions to sound. These results constitute an important first step in drawing out the links between genes, brain, cognition, and behavior in Williams syndrome.

Adolescent↗
PubMed2004

Increased perception of loudness in autism.

Clinical reports on autism describe abnormal responses to auditory stimuli such as intolerance to sounds. The present study assessed subjective perception of loudness in subjects with autism compared to healthy controls, using two psychoacoustic tests. First, the auditory dynamic range was evaluated at six different tone frequencies. Secondly, loudness growth as a function of the intensity level of a 1 kHz tone was estimated. Verbal responses from a group of 11 children and adolescents with autism were compared to responses of 11 age- and gender- matched healthy controls. Smaller auditory dynamic ranges were found in the autistic group than in the control group, as well as increased perception of loudness, indicating hyperacusis in subjects with autism.

Acoustic Stimulation↗
PubMed2000

Serum NGF levels in children and adolescents with either Williams syndrome or Down syndrome.

The neurotrophin nerve growth factor (NGF) is a major regulator of peripheral and central nervous system development. Serum NGF was measured in normally developing control children (n=26) and in individuals affected by congenital syndromes associated with learning disability: either Williams syndrome (WS; n=12) or Down syndrome (DS; n=21). Participants were assessed at three distinct developmental stages: early childhood (2 to 6 years), childhood (8 to 12 years), and adolescence (14 to 20 years). A sample was taken only once from each individual. Serum NGF levels were markedly higher in participants with WS, than DS and control participants. In addition, different developmental profiles emerged in the three groups: while in normally developing individuals NGF levels were higher in early childhood than later on, children with WS showed constantly elevated NGF levels. When compared to control participants, those with DS showed lower NGF levels only during early childhood. Neuropsychological assessment confirmed previously reported differences among the three groups in the development of linguistic/cognitive abilities. Some features of individuals with WS, such as hyperacusis and hypertension, could be related to high-circulating NGF levels.

Adolescent↗
PubMed1999

Autism and hearing loss.

A group of 199 children and adolescents (153 boys, 46 girls) with autistic disorder was audiologically evaluated. Mild to moderate hearing loss was diagnosed in 7.9% and unilateral hearing loss in 1.6% of those who could be tested appropriately. Pronounced to profound bilateral hearing loss or deafness was diagnosed in 3.5% of all cases, representing a prevalence considerably above that in the general population and comparable to the prevalence found in populations with mental retardation. Hearing deficits in autism occurred at similar rates at all levels of intellectual functioning, so it does not appear that the covariation with intellectual impairment per se can account for all of the variance of hearing deficit in autism. Hyperacusis was common, affecting 18.0% of the autism group and 0% in an age-matched nonautism comparison group. In addition, the rate of serous otitis media (23.5%) and related conductive hearing loss (18.3%) appeared to be increased in autistic disorder. The study emphasizes the need for auditory evaluation of individuals with autism in order to refer those with pronounced to profound hearing loss for aural habilitation and to follow those with mild to moderate hearing loss because of the risk of deterioration.

Adolescent↗