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Clinical guide for audiologic tinnitus management I: Assessment.

PURPOSE: This article is the first of 2 that present basic guidelines for audiologists to provide clinical management of tinnitus. The method, termed audiologic tinnitus management (ATM), was developed to incorporate management strategies that can be implemented most efficiently by audiologists. METHOD: Development of ATM has been drawn from the clinical and research experience of the authors and numerous audiologists. Certain elements of ATM are adapted from the methods of tinnitus masking and tinnitus retraining therapy. Procedures are described in the present article for performing the intake assessment, while the companion article (J. A. Henry, T. L. Zaugg, & M. A. Schechter, 2005) describes treatment methodology. RESULTS: Development of ATM has resulted in defined procedures to conduct a basic tinnitus assessment that includes written questionnaires, an intake interview, audiologic evaluation, and a psychoacoustic assessment of tinnitus perceptual characteristics. If patients report a sound tolerance problem (hyperacusis), loudness discomfort levels are measured at audiometric frequencies. There are special procedures for selecting hearing aids, ear-level noise generators, combination devices (noise generator and hearing aid combined), and personal listening devices (i.e., portable radios and tape, CD, and MP3 players). CONCLUSIONS: This article explains each of these assessment components in detail. Adoption of the ATM assessment protocol by audiologists can contribute to the establishment of uniform procedures for the clinical management of tinnitus patients.

Audiology↗

Clinical guide for audiologic tinnitus management II: Treatment.

PURPOSE: This article is the second of 2 that address the need for basic procedures that can be used commonly by audiologists to manage patients with clinically significant tinnitus, as well as hyperacusis. The method described is termed audiologic tinnitus management (ATM). METHOD: ATM was developed specifically for use by audiologists. Although certain procedural components were adapted from the methods of tinnitus masking and tinnitus retraining therapy, ATM is uniquely and specifically defined. A detailed description of the ATM assessment procedures is provided in the companion article (J. A. Henry, T. L. Zaugg, & M. A. Schechter, 2005). The present article describes a specific clinical protocol for providing treatment with ATM. RESULTS: The treatment method described for ATM includes structured informational counseling and an individualized program of sound enhancement that can include the use of hearing aids, ear-level noise generators, combination instruments (noise generator and hearing aid combined), personal listening devices (wearable CD, tape, and MP3 players), and augmentative sound devices (e.g., tabletop sound generators). Ongoing treatment appointments involve primarily the structured counseling, evaluation, and adjustment of the use of sound devices, and assessment of treatment outcomes. The informational counseling protocol and an interview form for determining treatment outcomes are each described in step-by-step detail for direct clinical application. CONCLUSION: This article can serve as a practical clinical guide for audiologists to provide treatment for tinnitus in a uniform manner.

Habituation, Psychophysiologic↗

Organization of tinnitus management in Poland.

Spontaneous idiopathic tinnitus is a significant interdisciplinary therapeutic problem. Based on different programs of tinnitus treatment, we organized a team of physicians, psychologists and engineers in order to establish the needs for the first Tinnitus Clinic in Poland. At the same time, together with number of clinical centres, scientific societies and non-governmental organizations, we carried out training and an information campaign throughout the country and initiated the first epidemiological studies survey about tinnitus in Poland. Over a period of 2 years we have provided care for almost 1000 patients, including them in a IS 24-month therapeutic program at the clinic. As a method of choice, Tinnitus Retraining Therapy (TRT) based on a neurophysiological model of tinnitus origin is used. We present here epidemiological data on tinnitus and hyperacusis in Poland.

Adult↗

A compelling desire for deafness.

A case is described of a patient who has a compelling and persistent desire to become deaf. She often kept cotton wool moistened with oil in her ears and was learning sign language. Living without sound appeared to be a severe form of avoidance behavior from hyperacusis and misophonia. She had a borderline personality disorder that was associated with a poor sense of self. Her desire to be deaf may be one aspect of gaining an identity for herself and to compensate for feeling like an alien and gaining acceptance in the Deaf community. Will a compelling desire for deafness ever become a recognized mental disorder one day for which hearing patients may be offered elective deafness after a period of assessment and living like a deaf person? Those working in the field of deafness should be aware that individuals may occasionally be seeking elective deafness or self-inflicting deafness to obtain a hearing aid.

Adult↗

The prevalence and onset of gaze modulation of tinnitus and increased sensitivity to noise after translabyrinthine vestibular schwannoma excision.

OBJECTIVE: To determine the prevalence and timing of onset of gaze-modulated tinnitus and increased sensitivity to noise in patients who had undergone translabyrinthine excision of a vestibular schwannoma. STUDY DESIGN: Retrospective questionnaire study. SETTING: University hospital departments of audiology and neurotology. PATIENTS: A cohort of 359 patients who had undergone translabyrinthine excision of a vestibular schwannoma in the period 1997 to 2003. INTERVENTION: Translabyrinthine excision of a unilateral sporadic vestibular schwannoma. MAIN OUTCOME MEASURES: Patient reports and visual analogue scale measures of the timing of onset of gaze-modulated tinnitus and the presence, timing of onset, and persistence of increased sensitivity to noise after surgery. RESULTS: Completed questionnaires were returned by 275 patients (77%), of whom 132 (48%) were men and 143 (52%) were women. Preoperative tinnitus was reported in 150 patients (55%). In 124 of these 150 (83%) the tinnitus persisted, and in 26 of 150 (17%) it abated. Of the 125 patients without preoperative tinnitus, 43 (34%) developed it postoperatively. In 167 (61%) patients of the total group of 275, postoperative tinnitus was reported. Gaze-modulated tinnitus was reported in 53 patients (19%). Somatic-evoked or -modulated tinnitus was reported in 38 patients (14%). In response to the question, "Did you notice being extra sensitive to noise after your operation?," 138 patients (50%) replied that they did. In 111 patients, this persisted. The onset of the modulation of tinnitus was distributed throughout the postoperative period, whereas the onset of increased sensitivity to noise was overwhelmingly in the first 2 months after surgery. CONCLUSION: Gaze modulation of tinnitus after vestibular schwannoma removal was identified in 19% of patients in this series. The onset data did not convincingly argue for any specific mechanism. The prevalence of increased sensitivity to noise is surprising and may represent central hyperacusis in response to unilateral deafferentation of the auditory system.

Adult↗

[Loudness discomfort level in normal hearing individuals].

BACKGROUND: Loudness Discomfort Level (LDL), a test used in the hearing aid fitting process, has also been recommended to evaluate patients with tinnitus and/or suspect of hyperacusis. AIM: To determine LDL reference values for normal hearing individuals and to correlate the LDL to the Acoustic Reflex Threshold (ART). METHOD: LDL was investigated in 64 normal hearing subjects, with ages between 18 and 25 years (53.1% female), in the frequency threshold of 0.5 to 8KHz and for speech (non-recorded, non-standardized connected discourse). Pulsate pure tones were presented for two seconds, with a one-second interval between each presentation. The initial stimulus intensity was at 50dB and was followed by ascending presentations, of 5dB each, until the subject referred initial discomfort with loudness. The testing procedure was performed separately in each ear, and was immediately repeated at the end of the test (test and retest situation). The choice of the ear that would start the testing procedure was alternated for each subject. After that contralateral acoustic reflexes ART were measured. The presence of the ART was indicated by a minimal needle deflection (larger than 0.05ml) on the emittance equipment. RESULTS: Median varied from 86 to 98dBHL, with no statistically significant differences between gender (p > 0.11), between ears (p > 0.36) and between the test-retest situation (p > 0.34). The determination coefficients (r2) of the linear regression model revealed absence of correlation between log(LDL) and log(ART). CONCLUSION: Normal hearing individuals have LDL between 86 and 98dBHL for all of the tested stimuli. Inter-subject differences and the good reproductivity suggest that the interpretation of the test should be cautious and analyzed considering the patient's history. The test can be an useful instrument go follow-up patients. No correlation was found between LDL and ART.

Acoustic Stimulation↗

Assessment of the influence of background noise on escape-maintained problem behavior and pain behavior in a child with Williams syndrome.

We examined the influence of background noise on levels of problem behavior and pain behavior under functional analysis conditions for a child with a diagnosis of Williams syndrome and hyperacusis. Background noise was associated with increases in escape-maintained problem behavior and increases in pain behavior such as clasping ears and crying. When the child was fitted with earplugs, there were substantial reductions in both problem and pain behavior under the background noise condition.

Child Behavior Disorders↗

Tinnitus: pharmacological topodiagnosis.

The difficulty of accurately localizing the source of subjective tinnitus is well-known. Anamnesis and traditional audiological tests can often suggest a source if its origin as peripheral or merely central (or both). Therefore, several authors, such as Risey, Denk, and Shulman, recently proposed identifying the source of subjective tinnitus through the evaluation of the responses reported by patients to adequate pharmacological treatments. Our study presents a useful plan to perform tinnitus topodiagnosis, which consists of specific audiological tests evaluating the characteristics of symptoms (annoyance, pitch, loudness, hyperacusis) and of several pharmacological tests carried out through the administration of particular drugs, the pharmacodynamic mechanisms and meaningful side effects of which are described. On the basis of pharmacological effects on tinnitometry, some drugs will be combined.

Amantadine↗

Disturbances of loudness perception.

This article reviews information on some auditory disorders that have in common a disturbance in loudness perception. The perceptual disturbances in these disorders have interchangeably been labeled "hyperacusis," "dysacusis," or "phonophobia." Our question concerns whether the loudness disturbances associated with these auditory disorders are sufficiently different as not to justify the equivalence implied by the labelling. Emphasis is placed on those articles that have given clear accounts of the phenomenology of the disturbed perceptual experience and have offered testable hypotheses about the mechanisms underlying it. Hypotheses about the origins of disturbed loudness perception are compared with independent experimental and clinical evidence on those mechanisms. The disturbances of loudness perception that occur in cochlear hearing loss, facial nerve paralysis and stapedectomy, and in more "central" disorders are phenomenologically different, have different underlying mechanisms, and merit different labels that most of them do not currently receive.

Facial Paralysis↗

Hearing symptoms in migrainous infarction.

BACKGROUND: In case reports, migraine headaches have been associated with fluctuating low-frequency hearing loss and sudden, unilateral hearing loss. Auditory symptoms associated with migrainous infarction have not previously been emphasized. OBJECTIVE: To describe migrainous infarction presenting with acute auditory symptoms. DESIGN: Case reports. SETTING: Tertiary care hospitals. PATIENTS: A 40-year-old man with a history of migraine suddenly developed bilateral hearing loss associated with severe, throbbing, occipital headache, tinnitus, vertigo, speech disturbance, and right hemiparesis. An early audiogram showed profound, down-sloping, sensorineural-type hearing loss bilaterally. Sixteen days later, a follow-up pure tone audiogram documented marked improvement in both sides to a pure tone average of 30 dB. Right hemiparesis and dysarthria also improved steadily for 2 months. A 25-year-old woman with a history of migraine with aura suddenly developed hyperacusis, unilateral hearing loss, and migraine headache early in migrainous infarction. Magnetic resonance imaging documented infarcts in the pons and cerebellum. CONCLUSIONS: In these patients, acute auditory symptoms are a part of the prodrome of migrainous infarction. We speculate that these symptoms may have resulted from migraine-associated vasospasm. Migrainous infarction should be considered in the differential diagnosis of acute auditory symptoms, including sudden, bilateral hearing loss.

Acute Disease↗

Patient-based outcomes in patients with primary tinnitus undergoing tinnitus retraining therapy.

OBJECTIVE: To determine whether the Tinnitus Handicap Inventory (THI), a validated patient-based outcomes measure, may improve our ability to quantify impact and assess therapy for patients with tinnitus. DESIGN: Nonrandomized, prospective analysis of 32 patients undergoing tinnitus retraining therapy (TRT). Assessment tools included comprehensive audiology, a subjective self-assessment survey of tinnitus characteristics, and the THI. Tinnitus Handicap Inventory scores were assessed at baseline and 6 months following TRT. RESULTS: Baseline analysis revealed significant correlation between the subjective presence of hyperacusis and higher total, emotional, and catastrophic THI scores. Tinnitus Handicap Inventory scores correlated with subjective perception of overall tinnitus effect (P<.001). Mean pure-tone threshold average was 17.4 dB, and mean speech discrimination was 97.0%. There were no consistent correlations between baseline audiologic parameters and THI scores. Following 6 months of TRT, the total, emotional, functional, and catastrophic THI scores significantly improved (P<.001). Loudness discomfort levels also significantly improved (P< or =.02). CONCLUSIONS: There is significant improvement in self-perceived disability following TRT as measured by the THI. The results confirm the utility of the THI as a patient-based outcomes measure for quantifying treatment status in patients with primary tinnitus.

Adolescent↗

Acoustic reflex and loudness discomfort in acute facial paralysis.

An extensive research protocol was used to evaluate the conditions of 48 consecutive patients with acute facial paralysis. The results indicated that after nerve excitability testing, the acoustic reflex was the most efficient indicator of impending nerve degeneration and predictor of recovery. The presence of increased sensitivity to intense acoustic stimuli (loudness discomfort level) indicated poor prognosis. The data demonstrate that dysacusis (hyperacusis) is not related to stapedial muscle paralysis and also question the validity of "topographic diagnosis" in determination of the site of the lesion in facial paralysis.

Adolescent↗

Child with De Novo t(1;6)(p22.1;p22.1) translocation and features of ectodermal dysplasia with hypodontia and developmental delay.

We report on a 6.5-year-old girl with a balanced translocation between the short arms of chromosomes 1 and 6. She was referred for genetics evaluation because of developmental speech delay and congenital absence of several deciduous and permanent teeth. She was very sensitive to noise (hyperacusis), had poor hair and nail growth, decreased sweating, and turned very red with high fever. She had microcephaly (head circumference at the second centile; weight and height were at 25th centile), short palpebral fissures, epicanthal folds, sparse eyelashes, large ears, partial anodontia, short finger and toenails, and dry skin. She had mild developmental delay. Family history was significant for learning problems in two paternal uncles, one paternal aunt, and several paternal cousins. Thyroid studies, calcium, phosphorus, and alkaline phosphatase levels were normal. Her karyotype was 46,XX,t(1;6)(p22.1;p22.2), and parental karyotypes were normal. This apparently balanced translocation may have resulted in either a submicroscopic loss or disruption of a gene or genes involved in ectodermal dysplasia. There are no reported cases of ectodermal dysplasia associated with this chromosome rearrangement.

Anodontia↗

Otological complications of herpes zoster.

Otological complications of varicella-zoster virus (Ramsay Hunt syndrome) include facial paralysis, tinnitus, hearing loss, hyperacusis (dysacousis), vertigo, dysgeusia, and decreased tearing. Cranial nerves V, IX, and X are often affected. Gadolinium-enhanced magnetic resonance imaging demonstrates enhancement of the geniculate ganglion and facial nerve. These manifestations are identical to Bell's palsy but are more severe and carry a graver prognosis. Eight percent of Bell's palsy patients eventually are diagnosed as "zoster sine herpete." A new case of Ramsay Hunt syndrome will occur every 52 minutes, compared to every 10 minutes for a new case of Bell's palsy.

Ear Diseases↗

Fine structure of degeneration in the cochlear nucleus of the chinchilla after acoustic overstimulation.

To study plastic changes in the cochlear nucleus after acoustic stimulation, adult chinchillas were exposed once to a 4-kHz octave-band noise at 108 dB SPL for 3 hr. After survival times of 1, 2, 4, 8, and 16 weeks, samples were taken for electron microscopy from a part of the cochlear nucleus, where cochlear nerve fibers degenerated after the noise exposure. Progressive changes in fine structure were characterized as early, intermediate, and late stages of degeneration. Freshly occurring synaptic degeneration appeared in each period from 1-16 weeks. Endings with large round vesicles, putative excitatory synapses of the cochlear nerve, displayed progressive increases in neurofilaments and enlarged synaptic vesicles. Compared to controls, synaptic vesicles seemed fewer, often in small clusters in the interior of endings, and smaller in the synaptic zone. These early changes progressed to mitochondrial disintegration and overt "watery" degeneration. Some surviving endings, however, were shrunken and displaced partially by enlarged spaces in the synaptic complex. Dense-cored vesicles gathered in these endings. In terminals with pleomorphic and flattened vesicles, presumed inhibitory endings, cytological changes appeared within 1 week and persisted for months. The synaptic endings darkened, some vesicles disintegrated, and many smaller flatter vesicles collapsed into heaps. Especially at the presynaptic membrane, vesicles were shriveled, but a few mitochondria were preserved. Without overt signs of synaptic degeneration, some of these cytological changes presumably reflect reduced synaptic activity in the inhibitory endings. These changes may contribute to a continuing process associated with abnormal auditory functions, including hyperacusis and tinnitus.

Acoustic Stimulation↗

Effects of cochlear ablation on muscarinic acetylcholine receptor binding in the rat cochlear nucleus.

Cholinergic synapses in the cochlear nucleus (CN) have been reported to modulate spontaneous activity via muscarinic acetylcholine receptors. In this study, muscarinic receptor binding was measured as specific binding of 1-[N-methyl-(3)H]scopolamine in CN regions of control rats and 7 days, 1 month, and 2 months after unilateral cochlear ablation. In control rats, the strongest binding was found in granular regions, followed in order by fusiform soma, molecular, and deep layers of the dorsal cochlear nucleus (DCN), with much lower binding in the anteroventral CN (AVCN) and posteroventral CN (PVCN). After unilateral cochlear ablation, binding in the AVCN, PVCN, and their associated granular regions on the lesion side became progressively greater than on the control side through 2 months after lesion. A significant asymmetry, with binding higher on the lesion side, was also found in the DCN fusiform soma layer at 7 days, and there and in the DCN deep layer at 1 and 2 months after lesion. There was also evidence of increased binding on the control side in most CN regions. By contrast, binding in the ipsilateral facial nucleus decreased, compared with the control side, by 7 days after the lesion and showed some recovery toward symmetry by 2 months after lesion, and there was no evidence for contralateral changes. These muscarinic receptor binding changes reflect receptor plasticity after loss of auditory nerve innervation. Such plasticity may underlie some of the central auditory functional changes that occur following peripheral lesions, such as tinnitus and hyperacusis.

Animals↗

A complete physical contig and partial transcript map of the Williams syndrome critical region.

Williams syndrome (WS) is a contiguous gene syndrome caused by hemizygosity for a chromosomal deletion at 7q11.23. The range of phenotypes includes mental retardation, dysmorphic facies, heart abnormalities, short stature, a specific cognitive profile, hyperacusis, and infantile hypercalcaemia. To identify all the deleted genes, we have constructed a detailed physical map and complete BAC/PAC contig of the critical region, extending a distance of approximately 2 Mb and delimited by the nondeleted markers D7S1816 and D7S489A. Somatic cell hybrids of WS patients were made and used to define the centromeric and telomeric deletion breakpoints, enabling the size of the WS deletion to be defined as approximately 1.4 Mb. Genes previously mapped to the region have been located on the contig, and we have isolated eight transcripts, two of which have been characterized as the genes CPETR1 and CPETR2. This contig and expressed sequence map will form the basis for the construction of a complete transcription map of the deleted region and will enable genotype-phenotype correlations to be attempted to identify the individual components of WS.

Chromosome Mapping↗

CAP amplitude after impulse noise exposure in guinea pigs.

In this study, 21 guinea pigs were submitted to a single high energy impulse noise (gun shot with blank projectiles). The auditory function was evaluated over a 7-day recovery period by recording the compound action potential (CAP) from the round window. The threshold shift and input/output function (CAP amplitude and delay function of the stimulus intensity) were studied at different frequencies. CAP amplitude fell after the noise trauma, especially at the lower sound level, resulting in a threshold shift. Latency was significantly increased. During recovery, whereas latency returned to its initial value, CAP amplitude gradually increased and, in half the animals, exceeded the control value for the higher levels of stimulus. This could have been because of progressive disinhibition or recruitment, and may correspond clinically to hyperacusis. These results are discussed referring to those obtained by other authors using other methods.

Acoustic Stimulation↗