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Journal of Clinical Medicine May 2024: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. : Data from 14 consecutive subjects undergoing implantation between April...
: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. : Data from 14 consecutive subjects undergoing implantation between April 2022 and November 2023 were reviewed. Ten subjects had conductive hearing loss, three had mixed hearing loss and one had single-sided deafness (SSD). Warble tone thresholds, Pure Tone Average (PTA) and Speech Discrimination Score (SDS) in quiet and in noise were determined unaided and aided. The subjective outcome was determined from two standardized questionnaires: (1) International Outcome Inventory for Hearing Aids (IOI-HA) and (2) Speech, Spatial and Qualities of Hearing Scale 12 (SSQ12b). : Unexpected postoperative pain was found in four cases. The warble tone thresholds exhibited a consistent reduction across all frequencies, contributing to a mean decrease of 27 dB in the aided PTA. SDS demonstrated notable improvements, with a 57.3% increase at 50 dB and a 55.6% increase at 65 dB. In noise, SDS exhibited a 43.9% improvement. The mean IOI-HA Score was 3.8, and the mean overall score for SSQ12b was 6.6, with consistent findings across the subgroups. : The Osia device emerges as a promising recommendation for individuals with conductive or mixed hearing loss, possibly also for those with SSD. Its safety and efficacy profile aligns with the broader category of active transcutaneous devices, demonstrating a reduced risk of wound infection compared to percutaneous alternatives. Both audiological assessments and subjective evaluations revealed positive outcomes.
PubMed: 38892822
DOI: 10.3390/jcm13113111 -
Animals : An Open Access Journal From... May 2024The reported positive outcomes of animal-assisted services have led to an emerging interest in many different aspects of human-animal interactions. The influence of an...
The reported positive outcomes of animal-assisted services have led to an emerging interest in many different aspects of human-animal interactions. The influence of an assistance animal is thought to encompass several psychosocial domains in the life of a person with a significant health impairment. However, little is known about the mechanisms underlying the relationship between Hearing Dogs and their owners. A prospective study design using a written questionnaire method was utilized to survey 58 current and 23 prospective Australian Lions Hearing Dogs owners. The Pet Expectations Inventory (PEI) was used to investigate the anticipated role of Hearing Dogs in waitlisted persons with hearing loss/Deafness, whereas the Lexington Attachment to Pets Scale (LAPS) was completed by current owners to assess emotional attachment. The results revealed a high mean PEI score (M = 73.1, SD = 10.9, Mdn = 73.0, range: 55-91), with prospective owners strongly expecting the role of Hearing Dogs to include companionship/love and security. Furthermore, strong attachment features were evident in the owners' relationships with Hearing Dogs, as demonstrated by a high total LAPS score (M = 81.2, SD = 7.5, range: 63-91). Mean scores for statements within the "people substitution" category were highest (range = 3.6/4.00-3.9/4.00). In this demographically homogenous study cohort, it appeared that the high expectations of potential Hearing Dog owners for their animals to serve supportive roles beyond hearing assistance should be achievable, as evidenced by the strong attachment relationships displayed between Hearing Dogs and their owners.
PubMed: 38891573
DOI: 10.3390/ani14111527 -
Scientific Reports Jun 2024While the relationships between spectral resolution, temporal resolution, and speech recognition are well defined in adults with cochlear implants (CIs), they are not...
While the relationships between spectral resolution, temporal resolution, and speech recognition are well defined in adults with cochlear implants (CIs), they are not well defined for prelingually deafened children with CIs, for whom language development is ongoing. This cross-sectional study aimed to better characterize these relationships in a large cohort of prelingually deafened children with CIs (N = 47; mean age = 8.33 years) by comprehensively measuring spectral resolution thresholds (measured via spectral modulation detection), temporal resolution thresholds (measured via sinusoidal amplitude modulation detection), and speech recognition (measured via monosyllabic word recognition, vowel recognition, and sentence recognition in noise via both fixed signal-to-noise ratio (SNR) and adaptively varied SNR). Results indicated that neither spectral or temporal resolution were significantly correlated with speech recognition in quiet or noise for children with CIs. Both age and CI experience had a moderate effect on spectral resolution, with significant effects for spectral modulation detection at a modulation rate of 0.5 cyc/oct, suggesting spectral resolution may improve with maturation. Thus, it is possible we may see an emerging relationship between spectral resolution and speech perception over time for children with CIs. While further investigation into this relationship is warranted, these findings demonstrate the need for new investigations to uncover ways of improving spectral resolution for children with CIs.
Topics: Humans; Cochlear Implants; Speech Perception; Child; Male; Female; Cross-Sectional Studies; Deafness; Child, Preschool; Signal-To-Noise Ratio; Noise; Adolescent
PubMed: 38890428
DOI: 10.1038/s41598-024-63932-w -
Trends in Hearing 2024This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support...
This multi-center study examined the safety and effectiveness of cochlear implantation of children between 9 and 11 months of age. The intended impact was to support practice regarding candidacy assessment and prognostic counseling of pediatric cochlear implant candidates. Data in the clinical chart of children implanted at 9-11 months of age with Cochlear Ltd devices at five cochlear implant centers in the United States and Canada were included in analyses. The study included data from two cohorts implanted with one or two Nucleus devices during the periods of January 1, 2012-December 31, 2017 (Cohort 1, n = 83) or between January 1, 2018 and May 15, 2020 (Cohort 2, n = 50). Major adverse events (requiring another procedure/hospitalization) and minor adverse events (managed with medication alone or underwent an expected course of treatment that did not require surgery or hospitalization) out to 2 years post-implant were monitored and outcomes measured by audiometric thresholds and parent-reports on the IT-MAIS and LittlEARS questionnaires were collected. Results revealed 60 adverse events in 41 children and 227 ears implanted (26%) of which 14 major events occurred in 11 children; all were transitory and resolved. Improved hearing with cochlear implant use was shown in all outcome measures. Findings reveal that the procedure is safe for infants and that they show clear benefits of cochlear implantation including increased audibility and hearing development.
Topics: Humans; Infant; Cochlear Implantation; Male; Female; Cochlear Implants; Treatment Outcome; Canada; United States; Time Factors; Retrospective Studies; Auditory Threshold; Postoperative Complications
PubMed: 38887094
DOI: 10.1177/23312165241261480 -
Investigative Ophthalmology & Visual... Jun 2024We investigated the natural history of retinal dystrophy owing to variants in the MYO7A gene.
PURPOSE
We investigated the natural history of retinal dystrophy owing to variants in the MYO7A gene.
METHODS
Fifty-three patients (mean age, 33.6 ± 16.7 years) with Usher syndrome owing to biallelic, mostly pathogenic, variants in MYO7A underwent baseline and two annual follow-up visits. Best-corrected visual acuity (BCVA), semiautomatic kinetic visual field, full-field electroretinogram, color fundus imaging, microperimetry, spectral-domain optical coherence tomography, and fundus autofluorescence were assessed.
RESULTS
At baseline, all patients presented with decreased BCVA (66.4 ± 17.9 Early Treatment Diabetic Retinopathy score and 59.5 ± 21.7 Early Treatment Diabetic Retinopathy score, in the better- and worse-seeing eyes, respectively), restricted semiautomatic kinetic visual field (III4e area, 3365.8 ± 4142.1°2; 4176.4 ± 4400.3°2) and decreased macular sensitivity (9.7 ± 9.9 dB; 9.0 ± 10.2 dB). Spectral-domain optical coherence tomography revealed reduced central macular thickness (259.6 ± 63.0 µm; 250.7 ± 63.3 µm) and narrowed ellipsoid zone band width (2807.5 ± 2374.6 µm; 2615.5 ± 2370.4 µm). Longitudinal analyses (50 patients) showed a significant decrease of BCVA in better-seeing eyes, whereas no changes were observed in worse-seeing eyes for any parameter. BCVA, semiautomatic kinetic visual field (III4e and V4e) and macular sensitivity were related significantly to age at baseline. Hyperautofluorescent foveal patch (16 eyes [31.4%]) and abnormal central hypoautofluorescence (9 eyes [17.6%]) were significantly associated with worse morphological and functional read-outs compared with the hyperautofluorescent ring pattern (22 eyes [43.1%]).
CONCLUSIONS
Our European multicentric study offers the first prospective longitudinal analysis in one of the largest cohorts of MYO7A patients described to date, confirming the slow disease progression. More important, this study emphasizes the key role of fundus autofluorescence patterns in retinal impairment staging and advocates its adoption as an objective biomarker in patient selection for future gene therapy clinical trials.
Topics: Humans; Male; Female; Adult; Prospective Studies; Tomography, Optical Coherence; Visual Acuity; Middle Aged; Electroretinography; Myosin VIIa; Visual Fields; Young Adult; Adolescent; Usher Syndromes; Genetic Therapy; Child; Visual Field Tests; Europe; Fluorescein Angiography; Follow-Up Studies; Aged; Longitudinal Studies; Disease Progression; Myosins; Retina
PubMed: 38884554
DOI: 10.1167/iovs.65.6.25 -
Advanced Genetics (Hoboken, N.J.) Jun 2024The co-occurrence of sensorineural hearing loss and male infertility has been reported in several instances, suggesting potential shared genetic underpinnings. One such...
The co-occurrence of sensorineural hearing loss and male infertility has been reported in several instances, suggesting potential shared genetic underpinnings. One such example is the contiguous gene deletion of and genes, previously associated with deafness-infertility syndrome (DIS) in males. Fifteen males with both hearing loss and infertility from southern India after exclusion for the DIS contiguous gene deletion and the gene mutations are subjected to exome sequencing. This resolves the genetic etiology in four probands for both the phenotypes; In the remaining 11 probands, two each conclusively accounted for deafness and male infertility etiologies. Genetic heterogeneity is well reflected in both phenotypes. Four recessive () and one dominant () for the deafness; six recessive genes (, and ) for male infertility can be conclusively ascribed. and genes are implicated earlier only in mice models, while the gene is implicated in chronic destructive airway diseases due to primary ciliary dyskinesia. This study would be the first to document the role of these genes in the male infertility phenotype in humans. The result suggests that deafness and infertility are independent events and do not segregate together among the probands.
PubMed: 38884051
DOI: 10.1002/ggn2.202300206 -
Frontiers in Neurology 2024The pathogenesis of idiopathic sudden sensorineural hearing loss remains unclear, and no substantial breakthroughs have been achieved in its treatment. Therefore, we...
BACKGROUND
The pathogenesis of idiopathic sudden sensorineural hearing loss remains unclear, and no substantial breakthroughs have been achieved in its treatment. Therefore, we conducted this study with the aim to investigate the clinical features and prognostic factors of patients with idiopathic sudden sensorineural hearing loss and auditory nerve enhancement by using three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) magnetic resonance imaging (MRI) of the inner ear.
METHODS
We retrospectively analyzed the clinical data of adult patients, who experienced sudden unilateral deafness and were admitted to the Department of Otolaryngology, Shandong Provincial ENT Hospital, between December 2020 and July 2021. Patients were divided into an auditory nerve enhancement group and a normal inner ear group, according to 3D-FLAIR MRI findings. Differences in sex, age, side, disease course, underlying diseases, dizziness/vertigo, vestibular function, degree of deafness, hearing classification, and treatment efficacy were analyzed.
RESULTS
Of the 112 cases of sudden idiopathic deafness, 16.07% exhibited enhancement of the auditory nerve on inner-ear 3D-FLAIR MRI. Statistically significant differences in the degree and type of hearing loss were detected between the two groups ( < 0.05). The rates of abnormal results in the caloric, vestibular-evoked myogenic potential, and video head impulse tests were higher in the auditory nerve enhancement group. The cure rate (11.1%) in patients with auditory nerve enhancement was lower than that in patients with normal inner ear MRI findings (28.7%); however, the difference was not statistically significant.
CONCLUSION
Findings from 3D-FLAIR MRI scans of the inner ear indicated that patients with sudden deafness and auditory nerve enhancement experienced severe hearing loss, aggravated vestibular function injury, and a significantly decreased cure rate. Prompt treatment, ideally within 2 weeks of disease onset, can facilitate hearing recovery.
PubMed: 38882699
DOI: 10.3389/fneur.2024.1410516 -
Orphanet Journal of Rare Diseases Jun 2024Biallelic pathogenic variants in USH2A lead to Usher syndrome or non-syndromic retinitis pigmentosa, and shown to have geographical and ethnical distribution in previous...
BACKGROUND
Biallelic pathogenic variants in USH2A lead to Usher syndrome or non-syndromic retinitis pigmentosa, and shown to have geographical and ethnical distribution in previous studies. This study provided a deeper understanding of the detailed clinical features using multimodal imaging, genetic spectrum, and genotype-phenotype correlations of USH2A-related retinal dystrophies in Taiwan.
RESULTS
In our cohort, the mean age at first visit was 47.66 ± 13.54 years, and the mean age at symptom onset, which was referred to the onset of nyctalopia and/or visual field constriction, was 31.21 ± 15.24 years. Among the variants identified, 23 (50%) were missense, 10 (22%) were splicing variants, 8 (17%) were nonsense, and 5 (11%) were frameshift mutations. The most predominant variant was c.2802T>G, which accounted for 21% of patients, and was located in exon 13. Patients with truncated alleles had significantly earlier symptom onset and seemly poorer disease progression regarding visual acuity, ellipsoid zone line length, and hypofluorescent lesions in the macula than those who had the complete gene. However, the clinical presentation revealed similar progression between patients with and without the c.2802T>G variant. During long-term follow-up, the patients had different ellipsoid zone line progression rates and were almost evenly distributed in the fast, moderate, and slow progression subgroups. Although a younger onset age and a smaller baseline intact macular area was observed in the fast progression subgroup, the results showed no significant difference.
CONCLUSIONS
This is the first cohort study to provide detailed genetic and longitudinal clinical analyses of patients with USH2A-related retinal dystrophies in Taiwan. The mutated allele frequency in exon 13 was high in Taiwan due to the predominant c.2802T>G variant. Moreover, truncated variants greatly impacted disease progression and determined the length of therapeutic windows. These findings provide insight into the characteristics of candidates for future gene therapies.
Topics: Adult; Female; Humans; Male; Middle Aged; Young Adult; Exons; Extracellular Matrix Proteins; Prevalence; Retinal Dystrophies; Taiwan; Usher Syndromes
PubMed: 38879497
DOI: 10.1186/s13023-024-03238-2 -
The Journal of Biological Chemistry Jun 2024Hearing, the ability to sense sounds, and the processing of auditory information are important for perception of the world. Mice lacking expression of neuroplastin (Np),...
Hearing, the ability to sense sounds, and the processing of auditory information are important for perception of the world. Mice lacking expression of neuroplastin (Np), a type-1 transmembrane glycoprotein, display deafness, multiple cognitive deficiencies, and reduced expression of plasma membrane calcium (Ca) ATPases (PMCAs) in cochlear hair cells and brain neurons. In this study, we transferred the deafness causing missense mutations pitch (C315S) and audio-1 (I122N) into human Np (hNp) constructs and investigated their effects at the molecular and cellular level. Computational molecular dynamics show that loss of the disulfide bridge in hNp causes structural destabilization of immunoglobulin-like domain (Ig) III and that the novel asparagine in hNp results in steric constraints and an additional N-glycosylation site in IgII. Additional N-glycosylation of hNp was confirmed by PNGaseF treatment. In comparison to hNp, transfection of hNp and hNp into HEK293T cells resulted in normal mRNA levels but reduced the Np protein levels and their cell surface expression due to proteasomal/lysosomal degradation. Furthermore, hNp and hNp failed to promote exogenous PMCA levels in HEK293T cells. In hippocampal neurons, expression of additional hNp or hNp was less efficient than hNp to elevate endogenous PMCA levels and to accelerate the restoration of basal Ca levels after electrically-evoked Ca transients. We propose that mutations leading to pathological Np variants, as exemplified here by the deafness causing Np mutants, can affect Np-dependent Ca regulatory mechanisms and may potentially cause intellectual and cognitive deficits in humans.
PubMed: 38879011
DOI: 10.1016/j.jbc.2024.107474 -
Medicine Jun 2024To explore the effects of focused solution nursing combined with cognitive intervention combined with functional training on negative emotions, compliance and quality of...
Focus on the effect of nursing care combined with cognitive intervention and functional training on negative emotion, compliance and quality of life in elderly patients with sudden deafness: A retrospective study.
To explore the effects of focused solution nursing combined with cognitive intervention combined with functional training on negative emotions, compliance and quality of life in elderly patients with sudden deafness. A total of 160 patients with sudden deafness in the elderly who were treated in our hospital from January 2019 to May 2021 were selected as the objects of this retrospective study. Based on different treatment approaches, subjects were divided into a control group and an observation group. Due to reasons such as the COVID-19 pandemic and transfers, 10 cases were lost to follow-up. In total, 75 cases were ultimately lost from both the control and observation groups. The control group implements cognitive intervention and functional training, and the observation group adopts focused solution nursing care on the basis of the control group. Observe and compare the effects of negative emotions, psychological distress, air conduction threshold level, compliance and quality of life of the 2 groups of patients. The air conduction hearing threshold level of the 2 groups of patients after nursing was lower than that before nursing and the observation group was lower than the control group. The positive coping scores of the 2 groups were significantly increased, and the negative coping scores were both significantly reduced and the observation group was in the 2 indicators. The degree of change was greater than that of the control group (P < .05). After nursing, the self-rating anxiety scale (SAS) and self-rating depression scale (SDS) of the observation group were lower than those of the control group (P < .05). The mental vitality score, social interaction score, emotional restriction score, and mental status of the observation group were significantly higher than those of the control group. The observation group's psychological compliance, activity compliance, dietary compliance, and treatment protocol compliance were significantly higher than those of the control group (P < .05). Adopting the focused solution model of nursing care can provide a better nursing recovery for elderly patients with sudden deafness, significantly improve the patient quality of life and anxiety and depression, improve patient compliance with treatment, and provide a certain reference for the nursing of elderly patients with sudden deafness.
Topics: Humans; Quality of Life; Retrospective Studies; Male; Female; Aged; Hearing Loss, Sudden; Patient Compliance; COVID-19; Emotions; Cognitive Behavioral Therapy; Aged, 80 and over; Adaptation, Psychological
PubMed: 38875427
DOI: 10.1097/MD.0000000000038283