Hearing recovery in sudden deafness with profound hearing loss

To more completely describe acromion morphology and its relationship to impingement syndrome, we performed three-dimensional magnetic resonance imaging (N = 111) or computed tomography (N = 27) on 132 symptomatic shoulders. The mean patient age was 46.2 years (range, 14 to 86). Four parameters were evaluated: the angle of anterior slope of the acromion in the midsagittal and lateral-sagittal planes, lateral acromial angulation in the coronal plane, and the presence or absence of medial encroachment in the acromioclavicular joint. Twenty-five asymptomatic age-matched shoulders were used as controls. All imaging data were combined because no significant differences existed between the two imaging techniques. The mean acromion angle was 19.4 degrees in the midsagittal plane and 20 degrees in the lateral-sagittal plane. In the coronal plane, 97 (73%) acromions were neutral and 35 (27%) were downward sloping. Medial encroachment was present in 31 (24%) shoulders. Age distribution from the 2nd to 8th decade demonstrated a consistent and gradual transition from a flat acromion in the younger decades to a more hooked acromion in the older decades that was significant in both the midsagittal and lateral-sagittal planes. Furthermore, a greater percentage of patients were found to have downward angulating acromions with increasing age. Ninety-eight patients (74%) had stage II or III impingement. Of these shoulders, 39 (40%) had type I acromions, 51 (52%) type II, and 8 (8%) type III. Twenty-eight of 33 acromions with coronal lateral downward sloping had impingement, and all 31 shoulders with medial encroachment had impingement.     

An implantable piezoelectric hearing aid transducer for inner ear deafness. II: Clinical implant]

A miniature, hermetically sealed implant was development and manufactured in several clinical and technical iteration steps based on the prototype of an implantable piezo-electric hearing-aid transducer described in Part 1 of the work presented here. The transducer is made of pure titanium (medical grade 2, ASTM F67) and designed to be implanted into the mastoid cavity. Transfer of mechanical oscillations to an ossicle in the middle ear is effected by a fixed directly coupling rod of pure titanium or via suitable coupling elements. The transducer is highly tuned with a resonance frequency in the range of 7-10 kHz, depending on the dynamic mass load. Below this resonance and down to low frequencies, the frequency response of elongation is smooth with a very small ripple of less than +/- 1 dB. Unlike the prototype, an increase in vibration amplitude of around 10 dB was achieved for a comparable power consumption. Vibration amplitude at low and middle frequencies is about 60 nm with a transducer voltage of 1 V, corresponding to an equivalent sound-pressure level of around 100 dB SPL at up to 1 kHz. At higher frequencies of up to 10 kHz, the output level increases to beyond 130 dB SPL. Nonlinear distortions at maximum volume (1 V) are extremely small (THD < 0.1%) throughout the whole transfer range. Due to an extremely short attack time (50 microseconds) and short release time (approximately 2 ms), the dynamic properties of the transducer allow good transmission of audio signals with fast changes in the time domain, i.e., plosives in speech signals. Electric power consumption at full volume and broadband signals is in the region of 1 microW. Unlike electromagnetic transducers described in the literature, the low power consumption of this piezoelectric transducer allows the realization of fully implantable hearing aids for rehabilitation of moderate to severe sensorineural hearing loss.     

Incidence of normal hearing in acoustic neuroma]

In a 4 1/2 year period fetal, echocardiographic studies were performed on 1600 fetuses. In 55 with arrhythmia, 44 had supraventricular ectopic beats, resolved in all, and none had heart disease. Sustained arrhythmias occurred in 11 fetuses. Atrial flutter was present in 3 all with heart disease (Ebstein disease, right atrial tumour and WPW diagnosed after birth). Another 3 fetuses had supraventricular tachycardia (SVT), all with a normal heart. In the bradycardia group, 2 had complete heart block (CHB) associated with AVSD; 2 sinus bradycardia and one had non conducted atrial ectopic beats. Digoxin was the first choice drug for tachyarrhythmia therapy; association with Verapamil, Flecainide, Quinidine and Procainamide was used in 4 of the 6. One fetus with CHB received Orciprenaline with no results. Atrial flutter resolved or improved; in SVT 2 fetuses converted to sinus rhythm and one died in utero. All fetuses with CHB died in cardiac failure. Mortality was 27% (3 cases) in utero and global 36%. In our experience most fetal arrhythmias (90%) were transitory ectopic beats or non lasting bradycardia in normal heart and did not trigger other kinds of arrhythmias. In sustained arrhythmias, heart failure and heart disease had a negative effect on prognosis.     

Prevalence and relevant background characteristics of deafness and severe hearing loss in the Netherlands

OBJECTIVE: To determine the prevalence of deafness and severe hardness of hearing among the Dutch population and of some relevant background characteristics. DESIGN: Study of documents. SETTING: Dutch schools for deaf and hard-of-hearing children. METHOD: The number of deaf and severely hard-of-hearing children of 6-12 years who received education or ambulatory supervision at schools for deaf and hard-of-hearing children was counted in the period November 1996-April 1997, and a number of relevant background characteristics were collected. By means of consultation with experts, this number was corrected for children who could not be retrieved by means of the inventory at these schools. To estimate the total number of deaf and severely hard-of-hearing people in the Dutch population, this adjusted number was extrapolated to older and younger year cohorts. RESULTS: The number of deaf and hard-of-hearing children of 6-12 years, after correction for missing groups, was 0.74 (95% confidence interval (95% CI): 0.69-0.78) per 1000 persons. In 9% the age of onset of the handicap was postlingual; they more often received ambulatory supervision and more often received education at a school for hard-of-hearing children than those with a prelingual auditive handicap. Of the children 29% had a non-Western origin; they less often received ambulatory supervision, and more often received education from a school for deaf children than indigenous pupils. Among the total Dutch population, the number of deaf and hard-of-hearing persons in 1996 was estimated to be 11,400 persons (95% CI: 10,690-12,110). CONCLUSION: The prevalence of deafness and severe hardness of hearing in the Netherlands (15,490,000 inhabitants in 1996) was 11,400 persons (95% CI: 10,690-12,110).     

Distortion products in normal hearing patients with tinnitus

Otoacoustic emissions are the result of cochlear active non-linear micromechanical mechanisms which probably originate within the OHC. OAE findings in patients with tinnitus are not univoque and there is no clear correlation between OAE modifications and tinnitus. We investigated distortion products in 20 normal hearing patients with tinnitus; all patients were selected with restrictive criteria (audiogram within 20 dB for all the frequencies, ABR and other tests normal, no history of ototoxic, nootropic drug intake, normal psychological evaluation, etc.). 12 patients out of 20 (60%) showed DP alterations. This finding is interpreted as an abnormality or a dysregulation of the efferent system (olivo-cochlear pathways) or of the other structures of the control loop which could modify outer hair cell activity in an otherwise normal cochlea with the development of tinnitus.     

Are normal hearing thresholds a sufficient condition for click-evoked otoacoustic emissions?

Transiently evolked otoacoustic emissions (TEOAE) have been reported in several studies as absent in a small minority of normal ears. Other studies have reported TEOAEs in all normal ears. Differences between studies may arise directly from criteria for TEOAE identification, criteria for selection of normals, or statistically due to limited sample sizes. In order to understand and model cochlear processes involved in TEOAE generation, it needs to be known whether the presence of normal hearing leads automatically to generation of TEOAEs. The present study set out to establish in a large sample if any ears could be found that lacked TEOAEs despite normal hearing threshold levels (HTL). A total of 397 ears from highly cooperative adult subjects were examined under laboratory conditions. Using cross correlation between replicate nonlinear waveforms as the criterion, TEOAEs were present in 99.2% of the sample (lower CI 98.1%). However, careful visual assessment of the recorded waveforms for the remaining ears did not unequivocally show absence of TEOAE characteristics in any ear with normal HTLs. While TEOAE strength varies widely among ears, no clear evidence was found to show that TEOAEs can be absent when HTLs are normal.     

Transient evoked otoacoustic emissions in patients with normal hearing and in patients with hearing loss

OBJECTIVES: 1) To evaluate transient evoked otoacoustic emission (TEOAE) test performance when measurements are made under routine clinical conditions. 2) To evaluate TEOAE test performance as a function of frequency and as a function of the magnitude of hearing loss. 3) To compare test performance using univariate and multivariate approaches to data analyses. 4) To provide a means of interpreting clinical TEOAE measurements. DESIGN: TEOAEs were measured in 452 ears of 246 patients. All measurements were made after acoustic immittance assessments, which were used to demonstrate that middle-ear function was normal at the time of the TEOAE test. TEOAE amplitudes and signal to noise ratios (SNRs), analyzed into octave bands centered at 1, 2, and 4 kHz, were compared with the pure-tone threshold at the same frequencies. Data were analyzed with clinical decision theory, cumulative distributions, discriminant analyses, and logistic regressions. RESULTS: Using univariate analysis techniques, TEOAEs accurately identified auditory status at 2 and 4 kHz but were less accurate at 1 kHz. Test performance was best when audiometric thresholds between 20 and 30 dB HL were used as the criteria for normal hearing. TEOAE SNR resulted in better test performance than did TEOAE amplitude alone; this effect decreased as frequency increased. Multivariate analysis methods resulted in better separation between normal and impaired ears than did univariate approaches, which relied on only TEOAE amplitude or SNR when test frequency band and audiometric frequency were the same. This improvement in test performance was greatest at 1 kHz, decreased as frequency increased, and was negligible at 4 kHz. CONCLUSIONS: TEOAEs can be used to identify hearing loss in children under routine clinical conditions. Univariate tests accurately identified auditory status at mid and high frequencies but performed more poorly at lower frequencies. The decrease in performance as frequency decreases may be a result of increased noise at lower frequencies but also may be due to properties of the measurement paradigm ("QuickScreen," high-pass filter at 0.8 kHz), which would not be ideal for recording energy around 1 kHz. The improvement in test performance when SNR was used and the interaction of this effect with frequency, however, would be consistent with the view that test performance in lower frequencies is at least partially influenced by the level of background noise. Multivariate analysis techniques improved test performance compared with the more traditional univariate approaches to data analysis. An approach is provided that allows one to assign measured TEOAE amplitudes, SNRs, or outputs from multivariate analyses to one of three categories: response properties consistent with normal hearing; results consistent with hearing loss; hearing status undetermined.     

Speech audiometry using the American Word Lists for Japanese subjects with normal hearing

The characteristics of listening to English words for Japanese people were studied in ten normal hearing subjects who had taken English classes to the level of college graduates and had opportunities to learn English continuously. Following pure tone audiometry, speech audiometry was performed using the Central Institute for the Deaf (CID) W-1 and W-22 word lists for English and the 67-S word lists for Japanese. The speech reception thresholds (SRTs) for the CID W-1 lists were significantly higher than average pure tone threshold (PTT), although the SRTs for the 67-S lists were equal to the average PTT. The difference in average SRT between the CID W-1 lists and the 67-S lists was about 15dB, which is statistically significant. The speech discrimination rate for the CID W-22 lists ranged from 78 to 100 percent with an average of 89.5 percent, while all subjects achieved the discrimination rate of 100 percent for the 67-S lists. Analysis with transient matrices of the perceived words demonstrated that the articulation rates were below 90 percent for the consonants /m/, /n/, /p/ and /delta/ . The observed variation in the speech discrimination score and the pattern of confusion among the subjects was assumed to be much more pronounced in noisy conditions.     

FO processing and the separation of competing speech signals by listeners with normal hearing and with hearing loss

Normal-hearing and hearing-impaired listeners were tested to determine F0 difference limens for synthetic tokens of 5 steady-state vowels. The same stimuli were then used in a concurrent-vowel labeling task with the F0 difference between concurrent vowels ranging between 0 and 4 semitones. Finally, speech recognition was tested for synthetic sentences in the presence of a competing synthetic voice with the same, a higher, or a lower F0. Normal-hearing listeners and hearing-impaired listeners with small F0-discrimination (deltaF0) thresholds showed improvements in vowel labeling when there were differences in F0 between vowels on the concurrent-vowel task. Impaired listeners with high deltaF0 thresholds did not benefit from F0 differences between vowels. At the group level, normal-hearing listeners benefited more than hearing-impaired listeners from F0 differences between competing signals on both the concurrent-vowel and sentence tasks. However, for individual listeners, deltaF0 thresholds and improvements in concurrent-vowel labeling based on F0 differences were only weakly associated with F0-based improvements in performance on the sentence task. For both the concurrent-vowel and sentence tasks, there was evidence that the ability to benefit from F0 differences between competing signals decreases with age.     

Identification of Caenorhabditis elegans genes required for neuronal differentiation and migration

To understand the mechanisms that guide migrating cells, we have been studying the embryonic migrations of the C. elegans canal-associated neurons (CANs). Here, we describe two screens used to identify genes involved in CAN migration. First, we screened for mutants that died as clear larvae (Clr) or had withered tails (Wit), phenotypes displayed by animals lacking normal CAN function. Second, we screened directly for mutants with missing or misplaced CANs. We isolated and characterized 30 mutants that defined 14 genes necessary for CAN migration. We found that one of the genes, ceh-10, specifies CAN fate. ceh-10 had been defined molecularly as encoding a homeodomain protein expressed in the CANs. Mutations that reduce ceh-10 function result in Wit animals with CANs that are partially defective in their migrations. Mutations that eliminate ceh-10 function result in Clr animals with CANs that fail to migrate or express CEH-23, a CAN differentiation marker. Null mutants also fail to express CEH-10, suggesting that CEH-10 regulates its own expression. Finally, we found that ceh-10 is necessary for the differentiation of AIY and RMED, two additional cells that express CEH-10.     

Identification of LytSR-regulated genes from Staphylococcus aureus

In this report, the characterization of a Staphylococcus aureus operon containing two LytSR-regulated genes, lrgA and lrgB, is described. Sequence and mutagenesis studies of these genes suggest that lrgA encodes a murein hydrolase exporter similar to bacteriophage holin proteins while lrgB may encode a protein having murein hydrolase activity.     

A behavioral measure of basilar-membrane nonlinearity in listeners with normal and impaired hearing

This paper examines the possibility of estimating basilar-membrane (BM) nonlinearity using a psychophysical technique. The level of a forward masker required to mask a brief signal was measured for conditions where the masker was either at, or one octave below, the signal frequency. The level of the forward masker at masked threshold provided an indirect measure of the BM response to the signal, as follows. Consistent with physiological studies, it was assumed that the BM responds linearly to frequencies well below the characteristic frequency (CF). Thus the ratio of the slopes of the masking functions between a masker at the signal frequency and a masker well below the signal frequency should provide an estimate of BM compression at CF. Results obtained from normally hearing listeners were in quantitative agreement with physiological estimates of BM compression. Furthermore, differences between normally hearing listeners and listeners with cochlear hearing impairment were consistent with the physiological effects of damage to the cochlea. The results support the hypothesis that BM nonlinearity governs the nonlinear growth of the upward spread of masking, and suggest that this technique provides a straightforward method for estimating BM nonlinearity in humans.     

Auditory brainstem response forward-masking recovery functions in older humans with normal hearing

We investigated the auditory brainstem response (ABR) recovery from forward masking using toneburst maskers and probes. Two subject groups matched for hearing thresholds were evaluated: normal-hearing young adults (21-40 years) and older subjects (63-77 years) with normal audiometric thresholds. Stimuli consisted of 1, 4 and 8 kHz tonebursts, with 2-4 cycle rise/fall time and no plateau. Forward maskers were tonebursts of the same frequency, with a 5 ms rise/fall time and a 20 ms plateau time. Probes were presented at 40 dB above threshold, and the forward masker was adjusted to a level that just eliminated the ABR to the 40 dB sensation level toneburst when the probe onset occurred at masker offset. Forward-masker intervals varied from 2 to 64 ms. ABR wave V latencies were similar for the young and old age groups regardless of toneburst frequency. Under forward-masking conditions, wave V latency was prolonged for the shorter intervals, and recovered to baseline latency by 64 ms. The forward-masker recovery functions were nearly identical for the two age groups for the 1 kHz toneburst. In contrast, there were clear differences in the recovery functions for the two age groups for the 4 and 8 kHz tonebursts. Specifically, the mean latency shift was greater for the aged group for forward-masker intervals of 16 ms or less. The two age groups showed identical latency shifts for longer forward-masker intervals. These data demonstrate prolonged recovery from forward masking in older human subjects. As these subjects had audiometric thresholds within normal limits, one plausible interpretation of this finding is that the prolonged recovery time is a manifestation of an aging effect on the central auditory nervous system rather than the periphery.     

Temporal analysis and stimulus fluctuation in listeners with normal and impaired hearing

The first experiment investigated the effects of mild to moderate sensorineural hearing impairment on temporal analysis for noise stimuli of varying bandwidth. Tasks of temporal gap detection, amplitude modulation (AM) detection, and AM discrimination were examined. Relatively high levels of stimulation were used in order to reduce the possibility that the results of the listeners with hearing impairment would be influenced strongly by audibility. A general summary of results was that there was relatively great interlistener variation among the listeners with hearing impairment, with most listeners showing normal performance and some showing degraded performance, regardless of the bandwidth of the stimulus carrying the temporal information. A second experiment investigated the hypothesis that listeners with sensorineural hearing impairment might have poor gap detection due to loudness recruitment. Here, gap markers were presented at levels where loudness growth was steeper for the listeners with hearing impairment than for the listeners with normal hearing. Although gap detection was sometimes poorer in listeners with hearing impairment than in listeners with normal hearing, there was no clear relation between gap detection performance and loudness recruitment in listeners with mild to moderate sensorineural hearing impairment.     

Identification of hearing loss after age 18 months is not early enough

Choriocarcinoma arising in the placenta, or intraplacental choriocarcinoma, has seldom been reported, particularly in the absence of maternal metastases. Reluctance to diagnose choriocarcinoma in the presence of chorionic villi can delay diagnosis; however, timely diagnosis of choriocarcinoma is prognostically important, both for the mother and infant. We report the clinicopathologic findings in five mothers and infants in whom choriocarcinoma was identified in the placenta. None of the mothers had a history of gestational trophoblastic disease in previous pregnancies. Three placentas were similar with a single small lesion grossly suggesting a small infarct; microscopically these consisted of infarcted areas surrounded by choriocarcinoma. These three mothers were unusual in that none had metastatic choriocarcinoma; two were treated with chemotherapy and remained disease-free; the third was lost to follow-up shortly following delivery. The remaining two mothers had known pulmonary metastases at time of delivery. One of these latter two placentas contained a large marginal lesion microscopically identified as choriocarcinoma. The fifth placenta had rare microscopic foci of choriocarcinoma, and sheets of necrotic choriocarcinoma were identified in "blood clot" submitted with the placenta. In four of the five cases the choriocarcinoma appeared to be arising from otherwise normal chorionic villi, and in no case was there invasion of the villous stroma. All of the infants survived, and none had evidence of choriocarcinoma. These cases support the concept that choriocarcinoma associated with otherwise normal pregnancy arises in the placenta and may be more common than reported.     

L-cone pigment genes expressed in normal colour vision

To directly test the hypothesis that only two pigment genes are expressed from the X-chromosome array, we examined expressed M and L pigment gene sequences from > 100 male eye donors. In this sample, there were eight men who expressed high levels of more than one L pigment gene in addition to M pigment genes. The fact that these eyes expressed both L and M pigment genes at significant levels suggests they were from men with normal colour vision. We reject the hypothesis that only two pigment genes from one X-chromosome array can be expressed.