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Alport's syndrome: can carriers be identified by audiometry?

Alport's syndrome is characterized by familial nephropathy with haematuria, sensorineural hearing loss and ocular abnormalities. It is transmitted as an X-linked dominant condition. In the affected males the symptoms are severe compared with females who show a more variable clinical picture due to the effect of lyonization of the X-chromosome. The diagnosis of carriers of genetic disease is extremely valuable for the purpose of counselling the patients as well as their relatives. A number of attempts have been made in the past to diagnose these carriers. Some studies have shown that it is possible to diagnose carriers of conditions where hearing loss is a symptom, by sensitive audiometric techniques. In this study we examined obligate and possible carriers of Alport's syndrome with pure-tone audiometry (PTA) and the audioscan method. We found that all obligate carriers and a predictable proportion of possible carriers had either a hearing loss on PTA or a mid frequency notch in the audioscan.

Adult↗

Feasibility studies of evoked response audiometry in the mentally subnormal.

Evoked response audiometry was attempted on a group of seventy-seven unsedated severly subnormal patients up to the age of twenty-two years. Data of some clinical value were obtained in fifty-seven cases. It is suggested that sedation need only be used if the initial attempt at E.R.A. is unsuccessful. Over twenty per cent of the subjects appeared to have significant hearing loss.

Acoustic Stimulation↗

Investigation of hearing loss in patients with acute brucellosis by standard and high-frequency audiometry.

This study aimed to determine the effect of acute brucellosis on the auditory system. Forty-two patients with acute brucellosis were evaluated clinically, and with serological and audiological tests, before and after treatment. Hearing threshold averages were calculated at 11 different frequencies (250-8000 Hz) of the auditory airway, and statistical analysis was performed. The average hearing thresholds were > 20 dB, with standard audiometry at 6000-8000 Hz, and < 20 dB at all other frequencies. After treatment, the average auditory threshold decreased to < 20 dB at 6000-8000 Hz (p < 0.0001). Pure-tone hearing thresholds were improved at all frequencies after treatment, with statistically significant differences at all frequencies except 12,000, 14,000 and 16 000 Hz (p < 0.05). There was no permanent hearing loss caused by acute brucellosis, and hearing thresholds were restored after treatment. It was concluded that acute brucellosis affects the auditory system, especially at high frequencies, and that patients with all forms of brucellosis should be evaluated for hearing loss.

Adolescent↗

Investigation of hearing impairment in Cavalier King Charles spaniels using auditory brainstem response audiometry.

Auditory brainstem response audiometry was used to investigate nine Cavalier King Charles spaniels with a history of hearing impairment. Successful recordings were made in all cases. In eight of the dogs, the hearing impairment was between 40 and 85 decibels re normal hearing level. In addition to confirming the degree of impairment in each ear, information was obtained concerning the site of the lesion. The auditory brainstem technique may have an important role to play in assessing treatment outcome. Other applications include screening animals used in breeding programmes as well as working dogs requiring good binaural hearing.

Animals↗

The effect of hemodialysis on hearing using pure-tone audiometry and distortion-product otoacoustic emissions.

Patients under treatment of hemodialysis (HD) frequently exhibit some degree of sensorineural hearing loss. Fifteen subjects and 10 controls were tested by using pure-tone audiometry (PTA) and distortion-product otoacoustic emissions (DPOAEs) before and after a HD treatment. Other parameters (blood pressure, body weight, blood chemistries) were also evaluated before and after HD: The purpose of this study was to determine the acute effect of HD on hearing level by measuring PTA and DPOAE before and after one HD session. The results from PTA and DPOAE testings showed that hearing was unaffected by HD. However, all 15 subjects revealed significantly poorer hearing, especially in the higher frequencies, compared to that of the controls (p < 0.0001). It was concluded then that HD is a safe treatment, and that the sensorineural hearing loss in these patients may be attributed to the preexisting renal disease.

Adult↗

Investigation of the 4,000-Hertz dip by detailed audiometry.

It is well known that a 4,000-Hz dip is often observed in the initial stage of noise-induced hearing loss and acoustic trauma, but it does not always occur as a result of these causes, and quite often the cause is unknown. Pure tone audiograms of 159 patients (76 cases of noise-induced hearing loss, 62 cases of hearing loss of unknown cause, 21 cases of familial hearing loss) with a 4,000-Hz dip were studied by detailed audiometry to clarify this configuration. The types of dip were classified into 11 groups; however, the basic types are the cone type, bowl type, and dish type, which show the smooth line of the hearing threshold. There were no clear differences in dip configuration among the 3 causes of hearing loss. It was thought that the dips displayed similar configurations regardless of the cause.

Adolescent↗

Auditory brainstem response audiometry in congenitally hypothyroid children under early replacement therapy.

Using auditory brainstem response audiometry, we evaluated 34 congenital hypothyroidism children under thyroid hormone therapy and 24 age- and sex-matched controls between 5 and 12 yr of age. Two main auditory brainstem response abnormalities were encountered: first, prolonged wave I latencies, secondary to a peripheral impairment, were found in seven congenital hypothyroidism children (20%): three of these showed signs of serious otitis media, unilaterally in two and bilaterally in the other, at the time of the evaluation. Second, shortened I-V interpeak latencies were observed in 10 children (29%). No correlation was found between the interpeak latencies and the L-thyroxine serum values at the time of the test or just prior to treatment initiation. Also, there was no correlation with estimated bone age at treatment initiation or with the Griffiths global mental development quotients assessed at 5 yr of age. These preliminary results suggest a significant incidence of auditory brainstem response abnormalities in treated hypothyroid children.

Audiometry↗

An innovation in insert visual reinforcement audiometry in children.

Visual reinforcement audiometry (VRA) is an important behavioural test of hearing in young children. The use of insert earphones during VRA provides a reliable method of obtaining ear- and frequency-specific data. Two testers are usually required for this procedure. The first tester operates the audiometer and other controls while the second tester distracts the child with various toys. The paper describes a technique of providing a visual cue to the second tester when a sound stimulus is introduced through the insert earphones.

Attention↗

New clinical finding in vestibular neuritis: high-frequency audiometry hearing loss in the affected ear.

Twenty-one patients with established vestibular neuronitis were examined otoneurologically and audiologically. High-frequency audiometry up to 20,000 Hz was performed on all the patients. In 17 cases a high-frequency hearing loss was greater in the ear with vestibular neuritis. The mean hearing loss difference varied from 14 dB to 24 depending on the frequency. Our results reveal that in most cases of vestibular neuritis there are no isolated vestibular lesions, but most cases also involve an auditory end-organ lesion. This finding is in unison with the anatomic conditions of the cochlea and the vestibular end-organ.

Adult↗

Brain stem electric response audiometry (BSERA).

Brain stem electric responses, recorded with external electrodes on vertex and ear lobes, are excellent for audiometry of young children. The vertex-positive wave with latency of 6 to 9 msec resembles closely the action potential of the auditory nerve, with the same high-intensity short-latency component and low-intensity long-latency component. Thresholds are reliable with filtered clicks at 1 000 Hz and higher. Practical advantages and theoretical limitations are summarized.

Acoustic Stimulation↗

Speech pure tone audiometry as a screen for exaggerated hearing loss in industrial claims.

We examined 596 patients referred with possible noise-induced hearing loss, by conventional and cortical evoked response audiometry. Discrepancies between the two tests identified 121 (20.3%) as exaggerating their hearing loss. We then studied the validity of simple conventional tests, which would be available in primary diagnostic facilities, in screening for the exaggrerators we had idenfied. By selecting those whose puretone average (for 0.5 and 1 kHz) was more than 10 dB different from the speech reception threshold, or whose initial puretone threshold at 0.5 kHz was 40 dB or greater, we identified 112 (93%) of the exaggerators, at the cost of additional examination of 209 (35% of the total) false-positive. However, this screening means that 46% of the claimants could be evaluated completely in a primary diagnostic facility, and only a small fraction of the exaggerator should be overlook.

Acoustic Stimulation↗

Reflex inhibition audiometry. A new objective technique.

The reflexive eyeblink, elicited by a tactile stimulus, is inhibited if an auditory stimulus precedes the eliciting stimulus by 100 msec. With adult subjects the threshold for this effect was found to be sufficiently low to suggest that reflex inhibition may be useful in objective audiometry. A preliminary investigation with infants and children showed that the inhibitory process is present, though variable, in children as young as 6 weeks olds.

Acoustic Stimulation↗

BSER-audiometry in difficult-to-test patients.

The results of BSER audiometry in a group of 38 difficult-to-test patients are reported, and the response thresholds compared with previous assessments by conventional audiometric techniques. The frequently severe behavioural disorders of these patients necessitated the use of general anaesthesia. Myringotomy revealed middle ear fluid in 20% of the ears, whilst 26% had significant ocular pathology.

Action Potentials↗

Classification of the severity of acoustic trauma based on pure tone threshold audiometry.

A simple-to-use classification system is presented for expressing the hearing loss resulting from acoustic trauma. They system is based upon the implications for social hearing as seen the pure tone air conduction hearing test, which is classified according to specifically segmented areas of the audiogram. A study of the audiometric results of 102 ex-army men with AcT and 25 controls with normal hearing included a detailed questionnaire to relate the patients' functional complaints to their audiograms, and the clinical picture was then completed by the administration of speech audiometry tests both in quiet backgrounds of various signal-to-noise ratios. Three main severity categories of hearing impairment were established. These are described and illustrated.

Adolescent↗

Behavioural tests: applications and limitations in comparison with brainstem response audiometry.

Conditioned behavioural hearing tests cannot be performed in children before the developmental age of 8 months. Informal tests cannot be used as threshold tests as the stimuli are unspecific in frequency, and the observed reactions are not equivalent to the threshold level. Brainstem response audiometry (BRA) was performed on 47 children (23 normal children and 24 children with mental retardation with or without behavioural disturbances) in whom informal test results were unclear. The BRA was followed up by behavioural tests 1 to 10 years later. In none of the cases did the BRA thresholds indicate better hearing function than the actual threshold noted later on at behavioural tests. High BRA thresholds (up to 75 dB nHL) were found in 5 normal-hearing children with serous otitis media at the time of BRA recording.

Age Factors↗

Possibilities of improving the diagnosis of noise-induced hearing damage by means of directional audiometry, the dichotic speech discrimination test, and the EEG1.

Several audiometric tests were made of 85 noise workers exposed to an intensive impulse noise during pressing and punching. These tests aimed at finding out noise-induced central hearing damages (directional audiometry, dichotic speech discrimination test according to Feldmann and checking of the induction of the impedance jump). In addition, a clinical EEG was taken from each subject. Having obtained the necessary norms with series of tests in subjects with normal hearing abilities for their respective ages, the following results have been obtained: (1) directional hearing has hardly been affected by noise-induced hearing damage. It behaves like age changes in the group compared with it; (2) the dichotic speech discrimination test shows increasingly pathological values with progressive noise-induced hearing damage; this was already obvious in the age group 41-50. The impedance jump is less and less produced with progressive noise-induced hearing damage and finally disappears at a noise intensity of up to 110 dB. Because of these findings a progressive degeneration of the spiral ganglion and hearing nerve in noise-induced hearing damage is discussed as a cause of a deteriorated speech discrimination and the absence of the impedance jump. The EEG findings show changes in the alpha-rhythm in about 50% of the cases at an exposure time of 10 years onwards which may be possibly due to damage to the reticular formation, once increasing noise-induced hearing damage no longer exists.

Adult↗

High-frequency audiometry above 8,000 Hz.

Human auditory sensitivity for the frequencies 8, 10, 12, 16, 18, and 20 kHz was examined in a group of 41 female university students ranging in age from 19 to 22 years. The purpose of this study was twofold: to determine the feasibility of high-frequency testing using procedures routinely employed in clinical audiometry and to collect normative data using this procedure on a young adult population. A modified Hughson Westlake procedure was employed and thresholds were obtained for both ears of each subject. It was noted that for the 20-kHz tone only 52% of the subjects tested were able to respond. Statistical analysis revealed no significant threshold differences between right or left ears for all subjects at any frequency. The approach described in the present study provided reliable thresholds and was found to be clinically feasible. Comparisons were made with similar previous studies and the results of the present study were found to be in general, overall agreement.

Acoustic Stimulation↗

Speech audiometry for children and subjective probability of Polish words.

Word lists used for speech audiometry should fulfill a number of conditions in order that the results of hearing tests be unambiguous and directly interpretable not only in terms of possible hearing loss but also in terms of the neurolinguistic decoding process. In order to construct a proper word list, it was decided to attempt to estimate the subjective probability of word occurrence in schoolchildren. Nine different lists of over 300 words each were prepared, containing words of all assumed degrees of familiarity. In the preliminary stage, the lists were presented to 600 children in primary and secondary schools. The children were asked to mark each word with a number indicating their relative familiarity with the word. The answers were examined for homogeneity.

Audiometry↗