The Incidence of Cranial Arteriovenous Shunts in Patients With Pulsatile Tinnitus: A Prospective Observational Study.

OBJECTIVES: Finding the underlying cause for pulsatile tinnitus can be challenging. We aimed to determine the incidence of arteriovenous shunts, i.e., arteriovenous malformations (AVMs) or dural arteriovenous fistulas (dAVFs), in patients referred for catheter angiography (digital subtraction angiography [DSA]). Furthermore, we assessed which clinical features were predictive for the presence of such a lesion. STUDY DESIGN AND METHODS: Fifty-one patients with pulsatile tinnitus, who were referred to us for DSA to exclude an arteriovenous shunt, were enrolled, prospectively. MAIN OUTCOME MEASURES: DSA determined the presence of a dAVF or AVM. Clinical characteristics were recorded systematically and all patients underwent a physical examination. RESULTS: Fifty patients were included in the final analyses. While no AVMs were found, a dAVF was found in 12 cases (24%). Three of these demonstrated cortical venous reflux, thus requiring treatment due to the risk of hemorrhage. In three cases (6%), DSA demonstrated a non-arteriovenous-shunt abnormality, likely causing the tinnitus. The odds of having a dAVF were significantly raised by unilaterality, objective bruit, and the ability to influence the tinnitus with compression. Unilaterality even had a negative predictive value of 1 and, if used as selection criterion, would have raised dAVF prevalence from 24 to 32%. CONCLUSION: In a tertiary care setting, the prevalence of dAVFs in patients with pulsatile tinnitus is not negligible. Thus, patients with unilateral pulsatile tinnitus should be offered dynamic vascular imaging to rule out a dAVF. Especially, since some of these patients are at risk of intracranial hemorrhage and treatment options exist.

Noise induced hearing loss and other hearing complaints among musicians of symphony orchestras.

OBJECTIVES: An investigation of the hearing status of musicians of professional symphony orchestras. Main questions are: (1) Should musicians be treated as a special group with regard to hearing, noise, and noise related hearing problems (2) Do patterns of hearing damage differ for different instrument types (3) Do OAE have an added value in the diagnosis of noise induced hearing loss (NIHL) in musicians. METHODS: 241 professional musicians, aged between 23-64 participated. A brief medical history and the subjective judgment of their hearing and hearing problems were assessed. Musicians were subjected to an extensive audiological test battery, which contained testing of audiometric thresholds, loudness perception, diplacusis, tinnitus, speech perception in noise, and otoacoustic emissions. RESULTS: Most musicians could be categorized as normal hearing, but their audiograms show notches at 6 kHz, a frequency that is associated with NIHL. Musicians often complained about tinnitus and hyperacusis, while diplacusis was generally not reported as a problem. Tinnitus was most often localized utmost left and this could not be related to the instrument. It was usually perceived in high frequency areas, associated with NIHL. In general, musicians scored very well on the speech-in-noise test. The results of the loudness perception test were within normal limits. Otoacoustic emissions were more intense with better pure-tone thresholds, but due to large individual differences it can still not be used as an objective test for early detection of NIHL. CONCLUSIONS: Musicians show more noise induced hearing loss than could be expected on the basis of age and gender. Other indicators, such as complaints and prevalence of tinnitus, complaints about hyperacusis and prevalence of diplacusis suggest that musicians' ears are at risk. Continuing education about the risks of intensive sound exposure to musicians, with the emphasis on the possible development of tinnitus and hyperacusis and the need for good hearing protection is warranted.

Hyperacusis in patients with complex regional pain syndrome related dystonia.

INTRODUCTION: In complex regional pain syndrome type 1 (CRPS-1), patients may have manifestations of central involvement, including allodynia, hyperalgesia or dystonia. We noted that more severely affected patients may experience hyperacusis, which may also reflect central involvement. The aim of this study was to evaluate the occurrence and characteristics of hyperacusis in patients with CRPS related dystonia. METHODS: The presence of hyperacusis, speech reception thresholds (SRT), pure-tone thresholds (PTT) and uncomfortable loudness (UCL) was evaluated in 40 patients with CRPS related dystonia. RESULTS: PTT and SRT were normal for all patients. 15 patients (38%) reported hyperacusis and this was associated with allodynia/hyperalgesia and with more affected extremities. UCLs of patients with hyperacusis were significantly lower than UCLs of patients without hyperacusis. CONCLUSION: Hyperacusis is common among severely affected patients with CRPS related dystonia and may indicate that the disease spreads beyond those circuits related to sensory-motor processing of extremities.

Homozygous and heterozygous expression of a novel insulin-like growth factor-I mutation.

IGF-I is a key factor in intrauterine development and postnatal growth and metabolism. The secretion of IGF-I in utero is not dependent on GH, whereas in childhood and adult life, IGF-I secretion seems to be mainly controlled by GH, as revealed from studies on patients with GHRH receptor and GH receptor mutations. In a 55-yr-old male, the first child of consanguineous parents, presenting with severe intrauterine and postnatal growth retardation, microcephaly, and sensorineural deafness, we found a homozygous G to A nucleotide substitution in the IGF-I gene changing valine 44 into methione. The inactivating nature of the mutation was proven by functional analysis demonstrating a 90-fold reduced affinity of recombinantly produced for the IGF-I receptor. Additional investigations revealed osteoporosis, a partial gonadal dysfunction, and a relatively well-preserved cardiac function. Nine of the 24 relatives studied carried the mutation. They had a significantly lower birth weight, final height, and head circumference than noncarriers. In conclusion, the phenotype of our patient consists of severe intrauterine growth retardation, deafness, and mental retardation, reflecting the GH-independent secretion of IGF-I in utero. The postnatal growth pattern, similar to growth of untreated GH-deficient or GH-insensitive children, is in agreement with the hypothesis that IGF-I secretion in childhood is mainly GH dependent. Remarkably, IGF-I deficiency is relatively well tolerated during the subsequent four decades of adulthood. IGF-I haploinsufficiency results in subtle inhibition of intrauterine and postnatal growth.

Auditory rehabilitation of older people from the general population--the Leiden 85-plus study.

BACKGROUND: Very few older people with severe hearing loss use hearing aids to reduce the negative consequences of reduced hearing in daily functioning. AIM: Assessment of a screening test and a standardised auditory rehabilitation programme for older people from the general population with untreated severe hearing loss. DESIGN OF STUDY: Intervention study and qualitative exploration. SETTING: Leiden 85-Plus Study, a prospective population-based study of 85-year-old inhabitants of Leiden, the Netherlands. METHOD: Hearing loss was measured by pure-tone audiometry in 454 subjects aged 85 years. Subjects with hearing loss above 35 dB at 1, 2, and 4 kHz who did not use hearing aids were invited to participate in a standardised programme for auditory rehabilitation. In-depth interviews were held with participants to explore arguments for participating in this programme. RESULTS: Of the 367 participants with severe hearing loss (prevalence = 81%), 66% (241/367) did not use a hearing aid. Three out of four of these participants (n = 185) declined participation in the auditory rehabilitation programme. The most common reason given for not participating was the subjects' feeling that their current hearing loss did not warrant the use of a hearing aid. Subjects who participated in the programme were found to suffer from more severe hearing loss and experienced more hearing disability. Those who did not participate in the programme felt they could cope with their disabilities and considered a hearing aid unnecessary. CONCLUSION: Untreated hearing loss is prevalent among older people from the general population. The majority of older people decline auditory rehabilitation. For these people the use of a hearing aid is not perceived as necessary in order to function on a daily basis. Older people who have expected benefits from a hearing aid have already obtained them, marginalising the benefits of a rehabilitation (and screening) programme.

Long-term audiometric follow-up of click-evoked auditory brainstem response in hearing-impaired infants.

Conventional pure-tone thresholds were collected as determined at ages between 4 and 8 years from a group of 163 infants, tested by auditory brainstem response (ABR) in the age range between 1 and 3 years old for objective hearing assessment. The subjects suffered from a variety of degrees and types of sensorineural hearing impairment. The prognostic value of the ABR peak V thresholds in response to 0.1 ms clicks with respect to the behavioural thresholds at octave frequencies from 125 to 8,000 Hz obtained later is evaluated. Correlation between ABR and behavioural thresholds is largest in the 1,000- to 8,000-Hz frequency range. Predicted pure-tone audiograms (mean and SD) were determined for each 10-dB class of ABR thresholds. SDs are in the order of 15 to 18 dB in the 500- to 4,000-Hz range and slightly higher at adjacent frequencies (i.e., somewhat larger than in comparable adult studies). Mean pure-tone thresholds in the 1,000- to 8,000-Hz frequency range are up to 20 dB worse than ABR thresholds, which is opposite to findings in normally-hearing subjects. Thus, with an increasing degree of sensorineural hearing impairment, pure-tone thresholds increase at a significantly higher rate than ABR thresholds. The observation is explained in terms of reduced temporal integration in cochlear hearing loss. ABR thresholds worse than 80 dB nHL are demonstrated to have very limited predictive value with respect to the amount of residual hearing, not only in the low- but also in the high-frequency range. The presence of otitis media during ABR testing is shown to make estimation errors increase to more than 25 dB (SD).

The prognostic value of electrocochleography in severely hearing-impaired infants.

This paper presents a longitudinal evaluation of electrocochleographic assessment in severely hearing-impaired infants. Electrophysiological data were obtained by transtympanic electrocochleography to tone-burst stimuli at octave frequencies of 500 to 8000 Hz at the age of 0-6 years in a group of 126 subjects. The results are compared with auditory thresholds determined at school age in the same children by means of pure-tone audiometry. Cochlear microphonics could be recorded in virtually all ears, although the majority of subjects had hearing losses of 90 dB and more. Compound action potentials (CAPs) showed waveforms varying from normal to a wide range of abnormalities. Audiometric thresholds correlated generally well with the compound action potential (CAP) thresholds obtained in infancy. The error in the predicted audiometric thresholds is between 15 and 20 dB, as compared with 11 dB reported for more moderate hearing losses. It is shown that, in spite of the high stimulus levels used, substantial frequency-specific threshold information is retained. Occasional large discrepancies in thresholds were often associated with markedly abnormal response waveforms. Among the many cases in which no ABR could be elicited, 68 per cent produced detectable electrocochleographic responses in the 1000-4000 Hz range. It is concluded that electrocochleography is a valuable method for the assessment of residual hearing in infants suspected of having a severe hearing impairment.

[Physical diagnostic procedures: whispered speech and tuning fork test]. / Fysische diagnostiek--fluisterspraak en stemvorkproeven.

Hearing impairment occurs in 10% of the Dutch population and may lead to major communication problems and even social isolation. A good method to detect hearing loss in a general practice is the screening audiometer. The treating physician may further use the whispered speech test and tuning fork tests which together give a good impression of the severity and the nature of the hearing loss if any. The whispered speech test is best performed in the standardized form according to the guideline 'Slechthorendheid' [hardness of hearing] of the Dutch College of General Practitioners (NHG), in which certain combinations of letters are recommended. The tuning fork tests according to Rinne and Weber indicate a difference in perception and conduction deafness, and are decisive for any hearing asymmetry. Use of the whispered speech test and of the tuning fork tests is recommended for adults and children from the age of 7.

The whispered voice: the best test for screening for hearing impairment in general practice?

Hearing loss is an important health problem in the elderly which sometimes leads to social isolation. In a study with 62 patients, the diagnostic value of four simple tests for screening for hearing loss in general practice was examined. When paying attention to the loudness of the whispering, the whispered voice test can be a valuable test for assessment of hearing loss in general practice.

Long-term effects of chemotherapy in patients with testicular cancer.

PURPOSE: Combination chemotherapy regimens that include cisplatin (CDDP) and bleomycin (BLE) result in the cure of the majority of patients with malignant germ cell tumors of the testis. We investigated the long-term damage of such chemotherapy to renal, pulmonary, and hearing function. PATIENTS AND METHODS: Forty-three patients with disseminated testicular carcinoma were studied 1.5 to 9.3 years (median, 4.1 years) after completion of chemotherapy. All 43 patients received CDDP; of these, 39 also received BLE, 27 vinblastine (VLB), and 27 etoposide (VP-16). Mean cumulative doses of individual cytotoxic drugs administered were CDDP 483 mg/m2 (range, 189 to 1,173 mg/m2), BLE 160 mg/m2 (range, 81 to 311 mg/m2), VLB 31 mg/m2 (range, 19 to 158 mg/m2), and VP-16 667 mg/m2 (range, 242 to 1,455 mg/m2). RESULTS: In the majority of cases, values of renal, pulmonary, and hearing function were within the normal range before treatment. An initial decrease in renal, pulmonary, and hearing function was observed, with recovery of pulmonary function at late follow-up. On average, a decrease of 15% in creatinine clearance rates was observed at late follow-up. Long-term effect on audiometric function was considerable, but frequencies affected were outside the range of conversational speech. With multivariate analysis, no overall relation between the cumulative doses of the individual drugs and the loss in organ function was found; the cumulative doses of CDDP and BLE only contributed approximately 30% to the loss in renal function and vital capacity, respectively. CONCLUSION: Chemotherapy-induced pulmonary toxicity is reversible, whereas nephrotoxicity and ototoxicity are not. However, the long-term effects of chemotherapy in testicular cancer patients were minor and not invalidating.

Hearing loss in facioscapulohumeral muscular dystrophy.

The coincidence of facioscapulohumeral muscular dystrophy (FSHD) with sensorineural hearing loss and retinal abnormalities might imply genetic heterogeneity of FSHD. We performed screening audiometry in 56 patients with autosomal dominant FSHD and in 72 healthy family members, and found that the difference in hearing level between 4,000 Hz and 6,000 Hz was significantly greater in FSHD patients than in controls, independently both for the left and right ear. We conclude that this change of hearing function is part of the disease and may lead to severe hearing loss in some patients. The fact that it was present in all families is another argument against genetic heterogeneity.

The effect of competing melodies on melody recognition by hearing-impaired and normal-hearing listeners.

For a group of 30 hearing-impaired subjects and a matched group of 15 normal-hearing subjects (age range 13-17) the following data were collected: the tone audiogram, the auditory bandwidth at 1000 Hz, and the recognition threshold of a short melody presented simultaneously with two other melodies, lower and higher in frequency, respectively. The threshold was defined as the frequency distance required to recognize the test melody. It was found that, whereas the mean recognition threshold for the normal-hearing subjects was five semitones, it was, on the average, 27 semitones for the hearing-impaired subjects. Although the interindividual spread for the latter group was large, it did not correlate with the subjects' auditory bandwidth, nor with their musical experience or education.