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Symposium on sensorineural hearing loss in children: early detection and intervention. Histopathology of sensorineural hearing loss in children.

It is important to determine clinically the etiology of any sensorineural hearing loss in a child, if this is at all possible. With continued examination of the pathologic changes in the temporal bone, one can correlate the clinical and histopathologic findings. By use of this combined knowledge, one can diagnose and evaluate more thoroughly a child with such a hearing loss and also better counsel the child's parents.

Child

Effects of signal processing on intelligibility of speech in noise for persons with sensorineural hearing loss.

A person with sensorineural hearing loss accompanied by recruitment has difficulty in understanding amplified speech, especially when speech is in the presence o competition. Two groups of subjects with sensorineural hearing loss with recruitment were tested at +6 and at O dB signal-to-noise (S/N) ratio at preferred levels for unprocessed speech and for speech processed through a two-band compression/equalization of circuit. In addition, unprocessed key word identification scores were compared to those from use of a hearing aid and of a one-band compression/equalization circuit. Processing a two-band compression/equalization circuit improved key word identification scores dramatically for both groups of subjects. The range of improvement for both groups was from 6 to 74%, mean 37.4%, for the S/N ratio of O dB. Subjects who achieved higher scores had high scores for unprocessed tests and had less recruitment present throughout the frequency range. Scores from one-band compression/equalization were compared to those from unprocessed scores; there were no significant differences in key word identification. Design characteristics and an excellent rationale for using two-band compression/equalization are generated by the data of this study.

Acoustic Stimulation

Heparin therapy for sudden sensorineural hearing loss.

Twenty-three patients with sudden unilateral sensorineural hearing loss were treated with a course of intravenous heparin sodium within one week of the onset of the hearing loss. Complete or good recovery developed in a total of 69.6% of these patients, but this is very similar to previously reported criteria of 66% spontaneous recovery. There was one complication in the series, ie. priapism.

Female

Asymptotic threshold shift in people with sensorineural hearing loss.

Twelve men with mild to moderate sensorineural hearing loss in the frequency range of 3 to 6 kHz were exposed to 24 hours of continuous noise. The noise was an octave band centered at 4 kHz at a level of 85 dB. Hearing thresholds were measured monaurally at 11 test frequencies ranging from 250 to 10,000 Hz prior to exposure and at selected intervals during and after exposure. Temporary threshold shift (TTS) development followed a similar time course to that observed in normal hearing subjects, asymptotic levels being reached between 8 and 12 hours of noise exposure. Maximum TTS occurred at 4 and 6 kHz. The amount of TTS was less for the subjects with sensorineural hearing loss than for people with normal hearing. However, the sound pressure level required to detect pure tone (shifted thresholds) following noise exposure was greater in the group with hearing loss than was measured in the normal hearing subjects. Within the limits of this experiment, a sensorineural hearing loss does seem to exert a significant effect on change in hearing sensitivity resulting from noise exposure.

Auditory Threshold

Temporary increase in sensorineural hearing loss with hearing aid use.

Two cases are presented exhibiting temporary increases in sensorineural hearing loss following hearing aid use. Data suggesting this correlation are shown. There were no contributing middle ear problems during the period surveyed. The most significant changes in hearing thresholds were at frequencies 1000 and 2000 Hz. The use of different hearing aids, with decreased maximum power outputs, was not found to have similar effects on hearing threshold levels. A scheduling regime is recommended for introducing any new hearing aid to a child.

Auditory Threshold

Effect of sensorineural hearing loss on acoustic stapedius reflex growth functions.

The growth function of the acoustic stapedius reflex was measured in subjects with normal hearing and sensorineural hearing loss of cochlear origin. The effects of age and magnitude of hearing loss were controlled. Activating stimuli were 500, 1000, and 2000 Hz tones and broadband noise. Stapedius muscle activity was inferred from acoustic impedance measures in the contralateral ear. The mean growth functions for tones were essentially linear in log-log plots with the rate of growth equal for the two groups. The mean growth function for the noise signal was curvilinear for the normal hearing groud had linear for the hearing loss group. Comparison of slope functions derived from the fitted data indicated that the rate of reflex growth for the noise signal, over a limited range above reflex threshold, is greater in ears with cochlear lesions than normal ears. For higher level noise signals, however, the rate of reflex growth is similar for normal and pathological ears. The effect of a cochlear lesion on the input-output function of the cochlea for both tonal and noise stimuli is to maintain the rate of reflex growth but shift the function along the intensity axis of a tonal signal and the response axis for a noise signal.

Acoustic Impedance Tests

Evaluation of relationship between hearing threshold and loudness discomfort level in sensorineural hearing loss.

The relationship between hearing level and loudness discomfort level (LDL) for narrow-band noise was evaluated in two groups of patients with sensorineural hearing loss. Group I had thresholds ranging from 25-60 dB SPL and Group II's thresholds ranged from 65-100 dB SPL. LDLs were determined for narrow bands of noise centered at 500, 1000, 2000, and 4000 Hz. The LDLs for Group II were greater than those for Group I and the differences were statistically significant. It is speculated that one reason for others not finding differences as a function of hearing level may be the absence of severe to profound hearing loss in the test populations.

Adolescent

Sympsium on hearing loss--the otolaryngologist's responsibility. Habilitation and rehabilitation of patients with sensorineural hearing losses.

A philosophy of the (re)habilitation of patients with sensorineural hearing losses from a communication point of view is presented. Pediatric and adult populations are discussed separately. Topics of consideration for pediatric patients include the appropriate use of amplification, team management of the hearing handicap, educational placement and the importance of parent counseling. The discussion of the management of adult patients reviews some problems in hearing aid fitting, patient counseling and appropriate case follow-up. The guiding principle behind these (re)habilitation processes is the integration of the hearing-impaired patient into a hearing world.

Adult

Electrocochleographic response patterns in a group of patients mainly with presbyacusis.

The latency, amplitude and wave-form of the action potential were studied in a group of patients, mainly presbyacusics and a few with noise-induced hearing loss, sensorineural hearing loss of unknown etiology and conductive loss. Electrocochleographic recruitment with short latency of the AP "threshold" and very rapid increase in amplitude with increasing click intensity was seen in 22%. Partial recruitment with long latency at AP "threshold" but steep amplitude-intensity curves was present in 33%. Only 17% of the subjects showed both electrocpchleographic recruitment (or partial recruitment) and recruitment in the acoustic reflex test. Subjects with conductive loss had normal amplitude and latency intensity curves but they were shifted to the right. The action potential wave-form was normal. Broad action potentials with multiple peaks were the most common in sensorineural hearing loss.

Action Potentials

[Prenatal causes of sensorineural hearing loss in children (author's transl)].

Prenatal causes of sensorineural hearing loss in children may be genetic or nongenetic, the deafness occurs alone or with other abnormalities. In addition, there are prenatal causes of a sensorineural hearing loss which is of delayed onset postnatally. In this review only the better known causes are discussed in which the otorhinolaryngologist should establish a diagnosis early, particularly when the possibility exists of preventing or even treating the hearing loss.

Aminoglycosides

Abrupt (sharp cut) type sensorineural hearing loss--a human temporal bone study--.

Histopathology of a case of bilateral sensorineural hearing loss of abrupt (sharp cut) type is reported. While there was a 45 dB gap in threshold between 1,000 Hz and 2,000 Hz bilaterally, the patient had a good hearing at 1,000 Hz and lower frequencies. The patient was suffering from Takayasu's arteritis. Major histopathological findings were as follows: Almost complete loss of the outer hair cells from the basal end to 12 mm area in the left cochlea (length: 30.5 mm) and 13 mm in the right (length: 31.5 mm). The inner hair cell of the same region was also missing in the left cochlea, and to a lesser degree in the right. There was a clear separation between the normal and the pathological organ of Corti. Marked loss of the cochlear neuron was noted in the same region. Blood vessels within the cochlea and the internal auditory meatus were normal. Bilateral abrupt (sharp cut) type sensorineural hearing loss with unknown etiology is a group of inner ear disease due to abiotrophy of the organ of Corti and cochlear neuron. Disposition or hereditary factor possibly plays an important role in the development of hearing loss.

Arteritis

Symposium on sensorineural hearing loss in children: early detection and intervention. Evaluation of the child with sensorineural hearing impairment.

When the physician is confronted with a child with sensorineural hearing impairment, he should do everything in his power to establish the cause of the hearing loss in order to diagnose those conditions that might be corrected or controlled and to counsel the parent concerning the child's future. Certain physical characteristic should alert the physician to the fact that there may be an accompanying sensorineural hearing loss. The family, gestational, and postnatal history may help establish this diagnosis. Finally, laboratory and x-ray examinations should be performed in an attempt to arrive at a definitive diagnosis.

Cerebral Palsy

Influence of amplification on the discrimination of dichotic consonant-vowel syllables in a population with sensorineural hearing loss.

This study was designed to investigate the premise that the long-term use of monaural amplification influences dichotic listening conditions in a population with sensorineural hearing losses. 30 subjects with moderate, bilateral sensorineural hearing losses and 10 normally-hearing adults were chosen for this study. 20 of the subjects with sensorineural hearing loss had worn amplification successfully for at least 1 year prior to testing. 10 of these subjects had worn amplification only on the right ear while the remaining 10 had worn amplification only on the left ear. All subjects received 60 monaural and dichotic consonant-vowel (CV) nonsense syllables presented at equal loudness levels using the most comfortable level (MCL) as the loudness criteria. While monaural scores revealed nonsignificant differences between ears for all subjects, using percentage of error, dichotic results produced a right-ear advantage for the right-ear-aided, unaided and normally-hearing subjects. A significant left-ear advantage was seen for the left-ear-aided subjects. Results of this study suggest that amplification introduces selective listening effects which alter reported dichotic test scores.

Audiometry, Pure-Tone

Sensorineural hearing loss and pili torti.

Twelve patients with pili torti and sensorineural hearing loss have been described since 1965. We present two new families with this combination of symptoms, and a restudy of a third family. The genetic aspects are discussed. Tentatively it is concluded that pili torti is an autosomal dominant with a low penetrance of a pleiotropic manifestation of sensorineural hearing loss.

Adult

Surgical treatment of long-term sensorineural hearing loss due to labyrinthine fistula.

Perilymphatic fistula, usually in the round or oval window, causes a variety of symptoms, including sensorineural hearing loss. Surgical repair of these fistulas has resulted in restoration of hearing in some cases. It has been suggested that surgery must be performed within 2 months of the trauma if improvement in hearing is to occur. This paper presents the case of a patient whose fistula and resulting hearing loss had persisted ten years before surgical repair. Restoration of normal hearing and discrimination of speech occurred in the ear which had previously presented a severe sensorineural hearing loss with no useful discrimination for speech. The authors usge audiologists and otolaryngologists to consider the possibility of fistula in cases of sudden hearing loss, even when years have elapsed since the trauma. Their experience suggests that surgery may be successful in restoring hearing after many years.

Adult

The use of high-pass amplification for broad-frequency sensorineural hearing loss.

Recent research suggests that persons with sensorineural hearing impairment should derive extra benefit from amplification that eliminates or greatly reduces low frequencies, i.e. frequencies below 1 500 or 2 000 Hz. Such amplification seems to reduce the detrimental effects of the upward spread of masking on speech intelligibility, especially when listening in noisy environments. Also, current research indicates that extended high-frequency amplification, between 4 000 and 6 500 Hz is especially beneficial for optimal speech intelligibility. 9 patients with a relatively flat, moderate to severe sensorineural hearing loss were evaluated in the clinic and for daily listening situations while wearing conventional broad-frequency hearing aids with an upper range of about 4 500 Hz. They were also evaluated under these same circumstances with a hearing aid that reduces low frequencies and extends the high frequencies to nearly 6 500 Hz. Results indicate that these patients performed better and perferred the hearing aid that extends the high and reduces the low frequencies, particularly in noisy places. As a group, they did not prefer this type of amplification in quite listening situations.

Aged

Sensorineural hearing loss in juvenile chronic arthritis.

An 18-year-old female with pauciarticular juvenile chronic arthritis is described who has developed a profound bilateral sensorineural hearing loss. The association of sensorineural hearing loss with juvenile chronic arthritis has not been reported before.

Adolescent

Viral causes of sensorineural hearing loss.

Many viruses, even the common cold, are capable of producing sensorineural hearing loss. Treatment so far available is ineffective in correcting these losses. However, as the technology of vaccination advances, it is hoped that these can be prevented. Poliomyelitis and smallpox have been virtually eliminated from this country. Measles, rubella, and mumps are coming under control. Perhaps in the future we will be able to completely control influenza and the common cold and thus prevent many of the sensorineural hearing losses that we see today.

Cochlea