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At least 19 recordsLinked to original sources
PubMed2025

Transcriptome sequencing reveals regulatory genes associated with neurogenic hearing loss.

Hearing loss is a prevalent condition with a significant impact on individuals' quality of life. However, comprehensive studies investigating the differential gene expression and regulatory mechanisms associated with hearing loss are lacking, particularly in the context of diverse patient samples. In this study, we integrated data from 10 patients across different regions, age groups, and genders, with their data retrieved from a public transcriptome database, to explore the molecular basis of hearing loss. These samples are mainly from fibroblasts and keratinocytes. Through differential gene expression analysis, we identified key genes, including ICAM1, SLC1A1, and CD24, which have already been shown to play important roles in neurogenic hearing loss. Furthermore, we predicted potential transcriptional regulatory factors that may modulate the expression of these genes. Enrichment analysis revealed biological processes and pathways associated with hearing loss, highlighting the involvement of circadian rhythm disruption and other neuro-related disorders. Although our study is limited by the sample size and the absence of larger-scale investigations, the identified genes and regulatory factors provide valuable insights into the molecular mechanisms underlying hearing loss. Further molecular and cellular experiments are necessary to validate these findings and elucidate the precise regulatory mechanisms involved. In conclusion, our study contributes to the understanding of hearing loss pathogenesis and offers potential targets for molecular diagnostics and gene-based therapies. This provides a foundation for further research into personalized approaches to diagnosing and treating hearing loss.

Humans↗
PubMedDate not supplied

Paget's disease and hearing loss.

Hearing loss, tinnitus, and vertigo have long been associated with Paget's disease of the bone. We reviewed the records of 463 patients with Paget's disease who were seen in the otolaryngology department. Hearing loss was common. It was our observation that mixed hearing losses were part of the disease process. Sensorineural hearing loss was the most frequent loss, but it usually was not part of the disease process. Tinnitus, vertigo, or both were seen in about 20% of these patients.

Adult↗
PubMedDate not supplied

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↗
PubMed1978

Symposium on hearing loss--the otolaryngologist's responsibility. Medical management of hearing loss.

Millions of Americans suffer hearing loss resulting in immense social and economic consequences. Hearing loss is merely a symptom or sign and the evaluation and management of afflicted individuals requires a thorough knowledge of etiologic factors and understanding of the underlying pathophysiology. Hearing loss is either conductive, sensorineural, or mixed. It may be congenital or delayed in onset, genetic or progressive or stable. Specific diagnosis should be sought in all cases with the objective being reversal of the hearing loss. Often definitive therapy is lacking yet prevention of progression, when possible; recognition of associated disorders, when present; compensation for disability, when applicable; epidemiologic study; genetic and psycho-social counseling; and habilitation and rehabilitation may still be initiated. A vigorous approach to the patient with hearing loss should be championed by the otolaryngologist.

Bone Conduction↗
PubMed1977

Luetic hearing loss.

Luetic hearing loss has been noted with increasing frequency in recent years. Four cases of luetic hearing loss seen at Walter Reed General Hospital from 1974 to 1975 are presented and discussed. Symptoms and pathology of luetic involvement of the inner ear are also presented. Serum FTA-ABS and TPI, in addition to VDRL, must be routinely obtained during the workup of hearing loss. CSF FTA-ABS should be obtained if serum FTA-ABS and TPI are positive. Reversibility may be dependent on early detection of luetic hearing loss and treatment with high doses of antibiotics and steroids.

Adult↗
PubMedDate not supplied

Audiologic and metabolic findings in 90 patients with fluctuant hearing loss.

Fluctuant hearing loss is a common occurrence. It is difficult to diagnose in its early stages when hearing thresholds are near normal and the only complaints the patient has are of fullness and tinnitus. Audiologic tests are helpful in confirming the diagnosis. Impedance measurements are an accurate assessment of middle ear status and can assist in localizing the fullness experienced by these patients. Site of lesion tests and discrimination scores at various sensation levels are sensitive indexes of disease activity. Observations during medical treatment of 90 patients with metabolic dysfunction (hyperlipoproteinemia: hypoglycemia; hypothyroidism) suggest that discrimination scores fluctuate more widely than do pure tone thresholds over a period of time. Thirty patients were given complete audiologic testing after dietary management and treatment. All reported relief from tinnitus and fullness, and 15 or 50% showed improved audiograms and discrimination scores. Any change in the energy reserve or metabolic rate of the inner ear by a systemic metabolic dysfunction can contribute to or cause sensorineural hearing loss. Energy flow from metabolic sources is needed to transduce the acoustic stimuli into neural excitation patterns. The presence of any systemic metabolic dysfunction can be expected to contribute to and cause fluctuant hearing loss.

Auditory Threshold↗
PubMed1975

Audiometry in fluctuant hearing loss.

Fluctuant hearing loss is a very complex matter, which involves not only neurological and physiological concepts but considerable acoustic factors as well. Fluctuant hearing loss is perhaps more visible with the use of pure tone audiometry because of the nature of its effect, the variation in the compliance properties of cochlear tissues. This visibility is due to impedance factors that bear on the inner ear as well as on the middle and external ear regions. It is suggested that changes in compliance of the cochlear tissues play a significant role in the existence of reduced sensitivity to low frequency pure tones. This indicates that sensorineural conditions with loss of hearing in the low frequency region may be a composite of conditions: sensory cell damage, which is only seen by high frequency response, and disturbance in the cochlear tissues, increasing their stiffness and resulting in loss of sensitivity for low frequency tones. When hearing damage is found in the inner ear exclusively because of permanent destruction of sensory cells, this theoretical model predicts that the fluctuating nature of certain types of hearing impairment ceases.

Aged↗
PubMed1978

Abnormal facial appearance and delayed diagnosis of congenital hearing loss.

Congenital hearing loss occurs in association with cranio-facial anomalies. Lay people and health professionals as well frequently regard individuals with cranio-facial anomalies as "stupid" or of lower than normal intelligence because of their odd appearance. Two case reports illustrate that this erroneous assumption will result in the delayed detection of significant hearing loss.

Adult↗
PubMed1978

[Noise-induced hearing loss and the sisi test. on the assessment of noise-induced hearing loss (author's transl)].

In the assessment of noise-induced hearing loss problems arise mainly in those cases where the middle frequencies are involved in the hearing defects at a proportionally high degree, because this strongly impairs the speech discrimination and because such a hearing defect cannot be regarded as a typical case of noise-induced hearing loss. A recruitment test must be carried out in the middle or lower frequency range in order to diagnose any possible multifactorial genesis of the hearing defect. As concerns the widely used SISI test the result is shown to depend not only on the test intensity but also on the test frequency. Since the hearing threshold in the middle under discussion is usually better in the middle and lower frequency ranges than in the higher ones and since, as a consequence, the test intensity in the SISI test is lower, frequently lower SISI values can be observed here than in the case of higher frequencies. Since the test of the lower frequencies results in lower SISI values than for higher frequencies also in those cases where the hearing threshold in the lower frequency range is the same or even worse, we must assume that the results of the SISI test depend on the frequency. In the examination of patients with a hearing defect where the hearing threshold curve flattens obliquely, intensity dependence and frequency dependence are added in the lower frequency range; negative SISI values are not infrequent. It is not admissible to conclude from that a retrocochlear defect in the middle and lower frequency ranges in cases of noise-induced hearing loss in the high-tone range.

Audiometry↗
PubMed1975

Diagnosis and treatment of fluctuant hearing loss.

Fluctuant hearing loss, a very real and common cause of sensorineural hearing loss, is probably due to cochlear hydrops resulting from an anatomically inadequate endolymphatic sac, poor circulation, and one or more metabolic disorders. In the early stages at least, it often responds to treatment.

Female↗
PubMed1975

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↗
PubMed1978

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↗
PubMed1976

Relationships between hearing loss and cognition in normally hearing aged persons.

The relationships between mild hearing losses and cognitive functioning were studied for two independently selected samples of aged subjects whose hearing was within normal limits. One group had 47 males of mean age 71.5 (SD 4.8) who were selected for their excellent health status. The second group consisted of 38 females of mean age 75.9 (SD 5.3), all of whom had some significant physical pathology. Hearing losses at various frequency levels (from 125 to 8000 cps) were correlated with performance on cognitive tests such as the WAIS, with age effects then being partialled out. The results reveal substantial associations between hearing losses and scores achieved on the intellectual measures for both samples. Verbal type tests show these relationships much more extensively than the performance tests. The findings imply that aged subjects may be more intellectually capable than their test performances suggest and that hearing is an important variable to be considered in the assessment of their cognitive functioning.

Aging↗
PubMedDate not supplied

Occupational hearing loss. The significance of asymmetrical hearing thresholds.

In 1873 consecutive patients referred for compensation assessment for presumed industrial hearing loss, 281 (15%) had an average difference in hearing threshold between the ears of 15 dB at 1/2, 1, 2 and 4 kHz. An unrelated cause was found in 115 (6%). It was due to trauma in 37 (2%), unexplained in 32 (1.7%) and attributed to noise exposure in 97 (5.2%). The possible mechanisms for noise as a cause are discussed and include shielding of one ear from noise, unequal recovery after severe noise exposure, and unequal sensitivity of the ears. After extensive investigation, including X-rays, vestibular tests and some myelograms, no treatable disorder was discovered.

Audiometry↗
PubMedDate not supplied

Longitudinal Study of hearing loss in childhood. Relationship between hearing impairment, poor learning and family background.

Of the Dutch school population 0.1% suffer from a hearing loss which makes it necessary for them to attend a special school. This permanent, binaural hearing impairment is of a moderate to severe degree (degrees II and III) and adversely affects the child's linguistic skills and school performance. Of the pupils attending ordinary schools 4-5% suffer from a hearing loss which, according to the Dutch audiologist Huizing, lies on the borderline of a slight to moderate hearing impairment (degrees I and II). This loss is characterised by its temporary nature, the fact that it is monaural in 71% of the cases and that there is no evidence for its clearly affecting the learning process. The risk of hearing impairment is predominantly determined by the child's family background.

Audiometry↗
PubMed1975

Definition of fluctuant hearing loss.

In summary, fluctuant hearing loss is defined as a disorder of the inner ear characterized by fullness, roaring tinnitus, and fluctuations in hearing. It is believed to be caused by an inadequate absorption of endolymph from the endolymphatic sac, with or without one or more metabolic disorders, that interferes with the delicate balance between the production and absorption of endolymph and thus produces cochlear hydrops. This triad of fullness, roaring tinnitus, and fluctuant hearing loss resulting from cochlear hydrops is much more common than the quadrad of true turning vertigo, fullness, roaring tinnitus, and fluctuant hearing loss due to vestibular and cochlear hydrops known as Meniere's disease. Although patients with fluctuant hearing loss only may eventually develop vertigo as the chief complaint and then be said to have Meniere's disease, it is remarkable how many patients continue to suffer mainly from cochlear symptoms at all times. It would appear, because of the greater frequency of fluctuant hearing loss than in Meniere's disease, that the cochlear labyrinth is more susceptible to hydrops than the vestibular labyrinth. For the purposes of diagnosis and treatment it is very useful to separate patients into those with fluctuant hearing loss and those with Meniere's disease.

Animals↗