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PubMed2004

Middle ear and cochlear disorders result in different DPOAE growth behaviour: implications for the differentiation of sound conductive and cochlear hearing loss.

Input/output functions of distortion product otoacoustic emissions (DPOAE I/O-functions) give an insight into the compressive, non-linear sound processing of the cochlea. With an inner ear dysfunction a steeper I/O-function is observed. Due to the linear sound processing of the middle ear, one can assume that the DPOAE growth behaviour remains unaltered with a sound conduction dysfunction. If that is true, a differentiation between middle and inner ear dysfunction will be possible by using the slope of DPOAE I/O-functions as a means for assessing cochlear compression. In order to test that hypothesis, DPOAE I/O-functions were recorded in a wide primary tone level range at up to 8 f2 frequencies between 2.0 and 8.0 kHz (15 dB SPL < L2< 60 dB SPL; L1=0.46 L2 + 41 dB SPL; f2/f1=1.2) in guinea pigs in which middle (saline solution in the bulla) and inner ear (exposure to loud broadband noise) disorders were induced. Middle ear dysfunction resulted in a reduction of the DPOAE amplitude independent of the primary tone level. Consequently, DPOAE growth behaviour was not affected. In contrast to that, during cochlear impairment, steepened DPOAE I/O-functions were observed reflecting loss of compression of the cochlear amplifier. Accordingly, DPOAE I/O-functions allow a differentiation between middle and inner ear dysfunction. Further studies will have to show the usability of this method for clinical diagnostics, e.g. for detecting sound conduction disturbances in newborn hearing screening due to amniotic fluid or Eustachian tube dysfunctions during the early postnatal period.

Animals↗
PubMed2003

Characterization of the transmembrane channel-like (TMC) gene family: functional clues from hearing loss and epidermodysplasia verruciformis.

Mutations of TMC1 cause deafness in humans and mice. TMC1 and a related gene, TMC2, are the founding members of a novel gene family. Here we describe six additional TMC paralogs (TMC3 to TMC8) in humans and mice, as well as homologs in other species. cDNAs spanning the full length of the predicted open reading frames of the mammalian genes were cloned and sequenced. All are strongly predicted to encode proteins with 6 to 10 transmembrane domains and a novel conserved 120-amino-acid sequence that we termed the TMC domain. TMC1, TMC2, and TMC3 comprise a distinct subfamily expressed at low levels, whereas TMC4 to TMC8 are expressed at higher levels in multiple tissues. TMC6 and TMC8 are identical to the EVER1 and EVER2 genes implicated in epidermodysplasia verruciformis, a recessive disorder comprising susceptibility to cutaneous human papilloma virus infections and associated nonmelanoma skin cancers, providing additional genetic and tissue systems in which to study the TMC gene family.

Amino Acid Motifs↗
PubMed1995

Geriatric grand rounds: hearing impairment and functional independence.

Hearing loss is a commonly encountered impairment in the elderly. It can profoundly affect physical, psychosocial and cognitive function. In addition to the actual hearing loss, other factors contribute to the hearing handicap experienced by older persons. These include difficulty in understanding speech in noisy situations, difficulty in supplementing hearing with visual information, and the slowing of cognitive and psychologic processes. Many forms of amplification are available and can be very useful in reversing much of the functional impairment associated with hearing loss. A comprehensive audiological assessment should be recommended before selecting the type of hearing aid or assistive listening device. Such a referral also affords an opportunity for more extensive aural rehabilitation.

Aged↗
PubMed1992

Sudden loss of hearing and vestibular function, muscular weakness, and multiple white matter lesions in preschool children.

Sudden cochlear hearing loss, occurring successively or simultaneously in both ears, was observed in four unrelated preschool children. Vestibular testing could be carried out in three patients and showed complete bilateral loss of function. All patients had a mildly retarded motor development due to nonprogressive muscular weakness. On MR imaging all patients showed multiple periventricular and subcortical white matter lesions. These lesions were not clearly progressive in one patient examined repeatedly over 6 years. Virological, bacteriological, immunological, and metabolic examinations were normal in all patients. Muscle biopsy showed morphologically abnormal mitochondria in two and lipid storage in one patient. No indications have been found for a disturbed functioning of the muscle mitochondria. The identical pattern and course of the disease in these patients suggests a new nosological entity, the aetiology of which can only speculatively be attributed to a vascular process associated with a mitochondriopathy.

Biopsy↗
PubMed2003

Effects of otitis media with effusion (OME) on central auditory function.

Conductive hearing loss attenuates and delays sound passing through the middle ear. This impairs binaural hearing and other central auditory functions dependent on high fidelity sound transmission. Persistent conductive loss leads to central impairments that persist after the peripheral loss has resolved. For example, children who have had multiple episodes of otitis media with effusion (OME) in the first few years of life may have poor detection of sounds in noisy environments, evidenced by reduced binaural unmasking (BU). Recent research shows that a 'threshold' level of OME is required to produce impaired BU. Children who had OME in one or both ears for more than about 50% of the first 5 years had reduced BU. Animal research, using long-term ear plugging, suggests that total OME duration, rather than age at the time of having the disease, determines its effect on BU. Animals reared with bilateral (but not unilateral) ear plugs also have poor auditory temporal resolution, and reduced sensitivity to short tones in the presence of background noise, after plug removal. However, given time (6-24 months) and training, all animals regained normal temporal resolution.

Auditory Diseases, Central↗
PubMed2003

Diminished auditory mismatch fields in dyslexic adults.

Electroencephalographic studies have demonstrated smaller auditory responses to infrequent deviances of speech and nonspeech sounds in dyslexic than normal-reading subjects. We used a whole-scalp neuromagnetometer to study selectively reactivity of the auditory cortices to sound deviances in 8 dyslexic and 11 normal-reading adults. Within a monotonous sequence of 50-millisecond 1000 Hz binaural tones, tones of 920 and 1080 Hz occurred with 7% probability each. Magnetic mismatch fields, elicited by the stimulus deviances, were diminished in the left hemisphere of the dyslexic subjects. The results indicate deficient change detection in the left auditory cortex of right-handed dyslexic adults.

Adult↗
PubMed2026

Utility of hearing aid use on neurocognitive functions and auditory processing: A systematic review.

PURPOSE: Hearing loss not only affects the peripheral auditory system but also leads to changes in central auditory pathways and neurocognitive skills. The use of hearing aids has shown potential benefits in mitigating the adverse effects of hearing loss. Earlier literature showed various studies assessing the impact of hearing aid use on cognitive functions and auditory processing, a comprehensive review of the existing literature is warranted. Existing literature showed heterogeneous outcomes among studies investigated the effect of Hearing aid use in combating the adverse effects of hearing loss on neurocognitive functions and auditory processing. METHOD: Databases including PubMed, SciELO, Cochrane Library, PsycINFO, Web of Science, and Index Copernicus were searched (2005-2025) using English keywords on hearing aid effects on neurocognition and auditory processing in individuals with hearing loss. Studies on effect of hearing aid use on neurocognition and auditory processing were included, while those involving cochlear implant users or other neurological disorders unrelated to hearing loss were excluded. Titles, authors and publication years were examined, and duplicates were removed. RESULTS: Total of 61 research papers were found on specific area and were included in the present review. Findings of these 61 articles have been analysed and are reported in this article. The outcome of the present study showed usefulness of hearing aid use on neurocognitive functions and auditory processing. CONCLUSION: The present literature review emphasized the importance of early rehabilitation through hearing aid use to alleviate the adverse effects of hearing loss on neurocognitive function and auditory processing.

Humans↗
PubMed1998

Children with minimal sensorineural hearing loss: prevalence, educational performance, and functional status.

OBJECTIVE: This study was designed to determine the prevalence of minimal sensorineural hearing loss (MSHL) in school-age children and to assess the relationship of MSHL to educational performance and functional status. DESIGN: To determine prevalence, a single-staged sampling frame of all schools in the district was created for 3rd, 6th, and 9th grades. Schools were selected with probability proportional to size in each grade group. The final study sample was 1218 children. To assess the association of MSHL with educational performance, children identified with MSHL were assigned as cases into a subsequent case-control study. Scores of the Comprehensive Test of Basic Skills (4th Edition) (CTBS/4) then were compared between children with MSHL and children with normal hearing. School teachers completed the Screening Instrument for Targeting Education Risk (SIFTER) and the Revised Behavior Problem Checklist for a subsample of children with MSHL and their normally hearing counterparts. Finally, data on grade retention for a sample of children with MSHL were obtained from school records and compared with school district norm data. To assess the relationship between MSHL and functional status, test scores of all children with MSHL and all children with normal hearing in grades 6 and 9 were compared on the COOP Adolescent Chart Method (COOP), a screening tool for functional status. RESULTS: MSHL was exhibited by 5.4% of the study sample. The prevalence of all types of hearing impairment was 11.3%. Third grade children with MSHL exhibited significantly lower scores than normally hearing controls on a series of subtests of the CTBS/4; however, no differences were noted at the 6th and 9th grade levels. The SIFTER results revealed that children with MSHL scored poorer on the communication subtest than normal-hearing controls. Thirty-seven percent of the children with MSHL failed at least one grade. Finally, children with MSHL exhibited significantly greater dysfunction than children with normal hearing on several subtests of the COOP including behavior, energy, stress, social support, and self-esteem. CONCLUSIONS: The prevalence of hearing loss in the schools almost doubles when children with MSHL are included. This large, education-based study shows clinically important associations between MSHL and school behavior and performance. Children with MSHL experienced more difficulty than normally hearing children on a series of educational and functional test measures. Although additional research is necessary, results suggest the need for audiologists, speech-language pathologists, and educators to evaluate carefully our identification and management approaches with this population. Better efforts to manage these children could result in meaningful improvement in their educational progress and psychosocial well-being.

Achievement↗
PubMed1997

Gap detection as a function of stimulus loudness for listeners with and without hearing loss.

Temporal resolution, or the ability to process rapidly changing stimuli, has been purported to be reduced in some listeners with hearing loss while being described as normal in others. Ensuring stimulus audibility by increasing stimulus levels results in near-normal temporal resolution abilities for many listeners with hearing loss, but may also result in uncomfortably loud stimulus levels. The current study was conducted to describe temporal resolution abilities of listeners with and without hearing loss as a function of stimulus loudness. The gap detection abilities of 8 listeners with normal hearing were compared with those of 8 listeners with mild to moderate hearing losses over a wide range of intensities using a 650-Hz wide high-frequency noise marker. At low intensities, listeners with hearing loss show poor gap detection ability. As intensity increases, most listeners' performance improves and stabilizes near normal at high loudness and sensation levels. At comfortable loudness, gap detection abilities of listeners with hearing loss are less than at loud levels and are considerably poorer than normal.

Adolescent↗
PubMedDate not supplied

Revisiting loudness measures in children using a computer method of cross-modality matching (CMM).

The test efficiency and reliability of loudness assessment using a computer-controlled method of cross-modality matching (CMM) between line length and loudness was investigated in children 4 to 12 years with normal hearing or mild to severe degrees of sensorineural hearing loss. Adult listeners with normal hearing served as a comparison group. Computer-generated visual and acoustic stimuli were used to derive individual loudness data. Children and adults with normal hearing presented with similar loudness functions, while children with sensorineural hearing loss had steeper functions than their normal-hearing counterparts. Retest data supported reliability of the CMM method with children within the current study and between previous studies performed with a similar, but manual, method. The computer CMM approach proved more time efficient than the manual one, halving the test time. The CMM loudness task in a computerized version may have potential in a research or clinical setting, in particular for individualizing hearing aid fittings with children.

Acoustic Stimulation↗
PubMed1989

Eflornithine for the treatment of Pneumocystis carinii pneumonia in patients with the acquired immunodeficiency syndrome: a preliminary review.

Pneumocystis carinii pneumonia (PCP) is the most common opportunistic infection in patients with acquired immunodeficiency syndrome (AIDS). Eflornithine is an antiprotozoal agent active against P. carinii. It acts by inhibiting ornithine decarboxylase, an enzyme that is essential for cellular function. The drug is initially administered intravenously, followed by oral therapy. Eflornithine has been used on a compassionate basis in AIDS patients with PCP who were intolerant of or unresponsive to traditional agents. Overall, the response rate has been about 35%; however, conclusions are difficult to make since patients had different stages of disease and received treatment for varying periods of time. Side effects include depression of bone marrow function, diarrhea, hearing loss, seizures, alterations in liver function tests, and rash. While the need for safer and more efficacious antipneumocystis drugs grows, widespread use of seemingly promising agents should be based on well-conducted clinical trials.

Acquired Immunodeficiency Syndrome↗
PubMed1989

Hearing impairment as a determinant of function in the elderly.

This clinical, practice-based study explores the research question: Is there a relationship between hearing loss and functional disturbance in elderly patients? We analyzed the impact of hearing impairment on 153 patients over 65 years of age screened in primary care practice. Functional and psychosocial impairment were measured using the Sickness Impact Profile (SIP), a standardized measure for assessing sickness related dysfunction. Hearing level was determined with pure tone audiometry. Multiple linear regression was used to adjust for patient case-mix differences and other clinical variables. After adjustment, a 10 dB increase in hearing loss was found to result in a 2.8 point increase in Physical SIP scores (95% confidence interval = 1.8-3.8), a 2.0 point increase in psychosocial SIP scores (95% confidence interval = 0.8-3.2) and a 1.3 point increase in overall SIP scores (95% confidence interval = 0.1-2.5). Poor hearing was associated with higher SIP scores and increased dysfunction. Thus, hearing impairment is an important determinant of function in the elderly.

Activities of Daily Living↗
PubMed1983

The ear effect as a function of age and hearing loss.

Many studies have shown that the right ear statistically is slightly more sensitive than the left ear, particularly in the male adult population. In this study, we examined the lateral difference in hearing sensitivity, termed the ear effect here, in an industrial noise-exposed, nonshooting population, by sex, age, and hearing level. It was found that the male population had a larger ear effect (right ear being more sensitive) than the female population. The magnitude of the ear effect was found to be significantly related to the hearing threshold level. The ear effect was highest when the threshold was between 30- and 40-dB HL. Several possible causes for the ear effect are discussed.

Adult↗
PubMed2005

Targeted disruption of mouse Coch provides functional evidence that DFNA9 hearing loss is not a COCH haploinsufficiency disorder.

Dominant progressive hearing loss and vestibular dysfunction DFNA9 is caused by mutations of the human COCH gene. COCH encodes cochlin, a highly abundant secreted protein of unknown function in the inner ear. Cochlin has an N-terminal LCCL domain followed by two vWA domains, and all known DFNA9 mutations are either missense substitutions or an amino acid deletion in the LCCL domain. Here, we have characterized the auditory phenotype associated with a genomic deletion of mouse Coch downstream of the LCCL domain. Homozygous Coch (-/-) mice express no detectable cochlin in the inner ear. Auditory brainstem responses to click and pure-tone stimuli (8, 16, 32 kHz) were indistinguishable among wild type and homozygous Coch (-/-) mice. A Coch-LacZDeltaneo reporter allele detected Coch mRNA expression in nonsensory epithelial and stromal regions of the cochlea and vestibular labyrinth. These data provide functional evidence that DFNA9 is probably not caused by COCH haploinsufficiency, but via a dominant negative or gain-of-function effect, in nonsensory regions of the inner ear.

Animals↗