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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↗
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↗
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↗
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↗
PubMed1978

Detection of syphilitic hearing loss.

Syphilis can cause sensorineural hearing loss that is potentially treatable. To determine the incidence of positive serological findings in patients with sensorineural hearing loss, the rapid plasma reagin card test (RPRCT) and the FTA-ABS test were performed in a study group composed of patients with sensorineural hearing loss of obscure origin. Of the 306 patients with sensorineural hearing loss, 20 (6.5%) demonstrated a positive FTA-ABS as compared with two of 100 (2%) of the control group. Considerable variation was found in the rate of the false-negative and the biological false-positive RPRCT as performed by our hospital and state laboratories when compared with the results of the FTA-ABS test. Syphilis should be considered in any patient with sensorineural hearing loss of obscure origin, despite a negative history for syphilis. The FTA-ABS is the preferred test to exclude hearing loss of syphilitic origin.

Adolescent↗
PubMed1978

Non-vertiginous fluctuating hearing loss.

Not all fluctuating hearing loss is due to Eustachian insufficiency. Not all sensorineural loss is untreatable. Fluctuating hearing loss due to endolymphatic hydrops is discussed, and the use of a low sodium diet, diuretics and vasodilators in the treatment of this disorder is described. To be effective, the treatment must be initiated early. Five typical cases are described, showing the typical presentation and response to treatment.

Adult↗
PubMed2026

Distinct cochlear cell types associated with genetic susceptibility to sensory and metabolic hearing loss in older adults.

Hearing loss is a heterogeneous condition that can be classified into different subtypes with diverse genetic and cellular components. To investigate the cochlear cell types underlying the genetic basis of sensory and metabolic components of age-related hearing loss (ARHL), we integrated human genome-wide association study data with mouse cochlear single-cell RNA sequencing data using the single-cell disease relevance score tool. These analyses revealed that genes associated with the sensory component of ARHL were most highly expressed in hair cells, while genes associated with the metabolic component of ARHL were most highly expressed in spiral ganglion neurons. We also investigated whether ARHL-associated gene expression patterns differed across subpopulations of the same cell type. Sensory hearing loss-associated genes showed differential expression across supporting cell subpopulations in younger mice, whereas metabolic hearing loss-associated genes exhibited differences across intermediate cell subpopulations of the stria vascularis in older mice. These findings provide evidence for the role of distinct genetic and cellular risk profiles for different ARHL subtypes, suggesting that prevention and therapeutic strategies may require targeting specific cell populations at different life stages.

ARHL↗
PubMed1978

Central auditory testing with peripheral hearing loss.

Seventy subjects with sensorineural hearing losses participated in this study. The goal of the study was to examine the effect of peripheral hearing loss on auditory tasks that are used to assess dysfunction within the central auditory pathways. Each subject received a central auditory processing (CAP) test battery that consisted of: (1) a dichotic sentence listening task; (2) a monosyllabic filtered word task; (3) a spondaic word binaural fusion task; and (4) a rapidly alternating speech task. All of these tasks were affected by certain degrees/configurations of sensorineural hearing loss, with the monosyllabic filtered word task being the most seriously affected. From the results of this study, it was concluded that while the CAP test battery can be administered to certain persons with sensorineural hearing losses, all results must be interpreted with caution and in view of the basic audiological assessment.

Adolescent↗
PubMed1979

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↗
PubMed1978

Serous otitis media associated with sensorineural hearing loss in children.

The effect of a conductive hearing loss secondary to serous otitis media in children with sensorineural hearing loss was seen to be an increase in threshold and in one case a decrease in speech discrimination. The diagnosis of the conductive component can be made by means of measurement of impedance, middle ear pressure, the recording of a tympanogram and otoscopy. It is likely that a conductive component can lead to delay in the diagnosis of an underlying sensorineural hearing loss in some children which could result in exacerbation of speech and language deficits. Correction of the conductive component can convert a profound hearing loss to a severe hearing loss, or a severe hearing loss to a moderate hearing loss, and it may also increase speech discrimination.

Adolescent↗