Hereditary deafness in chilldren: Diagnosis and a family report

1979 ◽  
Vol 93 (5) ◽  
pp. 495-506 ◽  
Author(s):  
Reda A. A. Ibrahim ◽  
Fred H. Linthicum
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Hard Of Hearing ◽  
Speech Development ◽  
Sensorineural Hearing ◽  
Membranous Labyrinth ◽  
Hereditary Deafness ◽  
The Family

Synopsis abstractA FAMILY with hereditary deafness is presented. The mother and ten of her children suffer sensorineural hearing loss. The maternal grandparents and four uncles and aunts were all hard of hearing. Clinical examination revealed no accompanying gross anomalies. In one child, Mondini deformity is the cause of deafness. In the rest of the family isolated membranous labyrinth deformity is suggested.The methods of early diagnosis are discussed, among which polytomography proved the most reliable for diagnosing Mondini and Michel deformities. We demonstrated how early fitting of hearing aids is essential for language acquisition and speech development.Clinical ability to differentiate between subtypes of inner ear hereditary deafness not accompanied by gross anomalies is limited. This problem and the problem arising from late diagnosis are illustrated by a report on a family of ten children, nine of whom suffer sensorineural hearing loss.

Otologics Fully Implantable Hearing Device Phase II Results

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P57-P57
Author(s):  
Drew M Horlbeck ◽  
Herman A Jenkins ◽  
Ben J Balough ◽  
Michael E Hoffer
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Phase Ii ◽  
Hearing Aids ◽  
Repeated Measures ◽  
Pure Tone ◽  
Sound Field ◽  
Sensorineural Hearing ◽  
Significant Difference ◽  
Hearing Device

Objective The efficacy of the Otologics Fully Implantable Hearing Device (MET) was assessed in adult patients with bilateral moderate to severe sensorineural hearing loss. Methods Surgical insertion of this totally implanted system was identical to the Phase I study. A repeated-measures within-subjects design assessed aided sound field thresholds and speech performances with the subject's own, appropriately fit, walk-in hearing aid(s) and the Otologics Fully Implantable Hearing Device. Results Six- and 12-month Phase II data will be presented. Ten patients were implanted and activated as part Phase II clinical trial. Three patients were lost to long term follow-up due to two coil failures and one ossicular abnormality preventing proper device placement. No significant differences between preoperative (AC = 59 dB, BC = 55 dB) and postoperative (AC = 61 dB, BC = 54 dB) unaided pure tone averages were noted (p < 0.05). Pure tone average implant aided thresholds (41 dB) were equivalent to that of walk-in-aided (37 dB) condition with no significant difference (p < 0.05) between patients’ walk-in-aided individual frequency thresholds and implant-aided thresholds. Word recognition scores and hearing in noise scores were similar between the walk-in-aided and for the implant-aided condition. Patient benefit scales will be presented at all end points. Conclusions Results of the Otologics MET Fully Implantable Hearing Device Phase II trial provide evidence that this fully implantable device is a viable alternative to currently available hearing aids in patients with sensorineural hearing loss.

scholarly journals Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial (Preprint)

2017 ◽  
Author(s):  
Joanna Nkyekyer ◽  
Denny Meyer ◽  
Peter J Blamey ◽  
Andrew Pipingas ◽  
Sunil Bhar
Keyword(s):  
Hearing Loss ◽  
Depressive Symptoms ◽  
Social Interaction ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Hearing Aid ◽  
Sensorineural Hearing ◽  
Auditory Training ◽  
Face To Face ◽  
Hearing Aid Use

BACKGROUND Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training—formal listening activities designed to optimize speech perception—are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. OBJECTIVE This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. METHODS This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive hearing aid (intervention) for either the first 3 or last 3 months of the 6-month auditory training program. Each participant will be tested at baseline, 3, and 6 months using a neuropsychological battery of computer-based cognitive assessments, together with a depression symptom instrument and a social interaction measure. The primary outcome will be cognitive performance with regard to spatial working memory. Secondary outcome measures include other cognition performance measures, depressive symptoms, social interaction, and hearing satisfaction. RESULTS Data analysis is currently under way and the first results are expected to be submitted for publication in June 2018. CONCLUSIONS Results from the study will inform strategies for aural rehabilitation, hearing aid delivery, and future hearing loss intervention trials. CLINICALTRIAL ClinicalTrials.gov NCT03112850; https://clinicaltrials.gov/ct2/show/NCT03112850 (Archived by WebCite at http://www.webcitation.org/6xz12fD0B).

Exposure of weak magnetic fields on auditory nerve of patients with moderate to severe sensorineural hearing loss

2021 ◽  
Vol 20 (5) ◽  
pp. 63-67
Author(s):  
S. V. Surma ◽  
◽  
D. S. Klyachko ◽  
B. F. Shchegolev ◽  
E. A. Ogorodnikova ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Magnetic Fields ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Auditory Nerve ◽  
Health Hazard ◽  
Sensorineural Deafness ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Alternative Methods

The article shows that one of the alternative methods of treating moderate to severe sensorineural hearing loss can be the use of an external weak magnetic field. The proposed method is based on the coincidence of the effects caused by the exposure of an external electromagnetic field of certain parameters on the auditory nerve, and natural acoustic exposure. The similarity of reaction allows using of external magnetic fields as an artificial stimulator of the auditory system’s neural part. Induction of applied magnetic fields does not exceed 300 µT, which means that under the current legislation such fields are classified as posing no health hazard. This method was tested at Saint Petersburg Research Institute of Ear, Throat, Nose, and Speech based on relevant Ethics Committee approval and complied with informed consent standards of volunteers. 15 patients aged 18 to 45 with III and IV levels of sensorineural deafness without concomitant pathologies were tested. Threshold pure-tone audiometry was performed on each patient at 500, 1000, 2000, 3000, and 4000 Hz before and after the procedure. The results of the experiments showed that electromagnetic stimulation of auditory neurons allows increasing hearing sensitivity. The proposed procedure may not yield a tangible result in patients with auditory deprivation. Patients with hearing aids showed the most significant results. At the same time, the measurements have shown that the sound thresholds among them became 10 dB lower on the average. The non-invasiveness of the exposure provided additional comfort for the patient.

Prevalence and classification of asymmetric forms of sensorineural hearing loss in older age group

2021 ◽  
Vol 20 (5) ◽  
pp. 8-12
Author(s):  
T. Yu. Vladimirova ◽  
◽  
A. B. Martynova ◽  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Hearing Threshold ◽  
Older Age ◽  
Sensorineural Hearing ◽  
Age Group ◽  
The Difference ◽  
The Right

The significance of asymmetric sensorineural hearing loss (ASNHL) is due to a special approach to diagnosis, followed by the process of hearing aids and auditory rehabilitation. Currently, there is no standard audiometric criterion for determining the forms of asymmetry, which significantly affects the assessment of the prevalence of ASNHL. The study aimed to assess the prevalence and classification of ASNHL forms in the older age group using two methods of calculation: 1) the difference in the average hearing threshold at speech frequencies (in the range of 0,5–4 kHz) ≥15 dB was detected in 14,14% of cases; 2) the different degree of hearing loss, according to the International classification, in the right and left ear was 35,98%. In most cases, asymmetry was manifested by bilateral sensorineural hearing loss of varying severity, prevailing in the group of long-livers – 82,6%. Given the potentially high prevalence of asymmetry depending on the audiological criterion, the results of the work are a reason for further research in the development of a unified method for verifying a clinically significant form of ASNHL.

scholarly journals Halláscsökkenést okozó etiológiai tényezők cochlearis implantáción átesett gyermekekben

2019 ◽  
Vol 160 (21) ◽  
pp. 822-828
Author(s):  
Nóra Kecskeméti ◽  
Anita Gáborján ◽  
Magdolna Szőnyi ◽  
Marianna Küstel ◽  
Ildikó Baranyi ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Speech Development ◽  
Sensorineural Hearing ◽  
Diagnostic Process ◽  
Sufficient Information ◽  
Hearing Rehabilitation ◽  
Junction Protein ◽  
Newborn Hearing

Abstract: Introduction: Congenital sensorineural hearing loss is one of the most common sensory defects affecting 1–3 children per 1000 newborns. There are a lot of causes which result in congenital hearing loss, the most common is the genetic origin, but infection, cochlear malformation or other acquired causes can be reasons as well. Aim: The aim of this study was to establish the etiological factors of congenital profound sensorineural hearing loss in children who underwent cochlear implantation. Results: Our results show that the origin of the hearing loss was discovered in 62.9% of our patients. The most common etiological factor was the c.35delG mutation of the gap junction protein β-2 gene, the allele frequency was 38.7% in our cohort. Infection constituted to 10.1%, and meningitis and cytomegalovirus infection were the second most common cause. 79.9% of our patients received sufficient hearing rehabilitation before the end of the speech development’s period (6 years old), but 11.2% of our cases were still diagnosed late. Conclusions: Based on our data we can state that genetic evaluation is crucial in the diagnostic process of congenital profound sensorineural hearing loss. Sufficient hearing rehabilitation affects the whole life of the child, and by late cochlear implantation the speech development falls behind. We can decrease the ratio of the late implantation with the new protocol of newborn hearing screening, and with sufficient information provided to the colleagues, so the children may be referred to the proper center for rehabilitation without delay. Orv Hetil. 2019; 160(21): 822–828.

scholarly journals LONG TIME OBSERVATIONS OF CHILDREN WITH SENSORINEURAL HEARING LOSS USING HEARING AIDS

1979 ◽  
Vol 82 (12) ◽  
pp. 1480-1485
Author(s):  
MICHI YAGI ◽  
YOSHIAKI HIRASUGI ◽  
YUKO HORIBE ◽  
SACHIKO IBATA ◽  
TOMOHIRO YASUNO
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Sensorineural Hearing ◽  
Long Time

Digital hearing aids for high-frequency sensorineural hearing loss: Preliminary experience with the RetroX® device

2005 ◽  
Vol 125 (7) ◽  
pp. 693-696 ◽  
Author(s):  
Maurizio Barbara ◽  
Giorgio Bandiera ◽  
Bruno Serra ◽  
Vania Marrone ◽  
Silvia Tarentini ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
High Frequency ◽  
Sensorineural Hearing ◽  
Digital Hearing Aids

Evaluation of Hardwire Personal Assistive Listening Devices

1994 ◽  
Vol 3 (2) ◽  
pp. 71-77 ◽  
Author(s):  
James J. Dempsey ◽  
Mark Ross
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Hearing Aid ◽  
Low Frequency ◽  
Sensorineural Hearing ◽  
Clinical Implications ◽  
Frequency Responses ◽  
Subjective Evaluations ◽  
Potential Benefits

A large number of personal amplifiers have recently become available commercially. These devices have not been classified as hearing aids by the FDA and are therefore not subject to the FDA rules and regulations governing the sales of hearing aid devices. In this investigation, several of these personal amplifiers were evaluated to determine potential benefits and problems for each device. The devices were evaluated electroacoustically and, also, subjectively by a group of adults with sensorineural hearing loss. The results of the electroacoustic evaluation revealed very sharply peaked frequency responses. The subjective evaluations revealed tremendous variability, with some preferences for power and low-frequency amplification. Clinical implications of these results and suggestions for further research are provided.

Sign in / Sign up

Export Citation Format

Share Document