scholarly journals Hearing problems in patients with hereditary gelsolin amyloidosis

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Tuuli Mustonen ◽  
Ville Sivonen ◽  
Sari Atula ◽  
Sari Kiuru-Enari ◽  
Saku T. Sinkkonen

Abstract Background Gelsolin amyloidosis (AGel amyloidosis) is a hereditary form of systemic amyloidosis featuring ophthalmological, neurological and cutaneous symptoms. Previous studies based mainly on patients’ self-reporting have indicated that hearing impairment might also be related to the disease, considering the progressive cranial neuropathy characteristic for AGel amyloidosis. In order to deepen the knowledge of possible AGel amyloidosis-related hearing problems, a clinical study consisting of the Speech, Spatial and Qualities of Hearing Scale (SSQ) questionnaire, clinical examination, automated pure-tone audiometry and a speech-in-noise test was designed. Results Of the total 46 patients included in the study, eighteen (39%) had self-reported hearing loss. The mean scores in the SSQ were 8.2, 8.3 and 8.6 for the Speech, Spatial and Qualities subscales, respectively. In audiometry, the mean pure tone average (PTA) was 17.1 (SD 12.2) and 17.1 (SD 12.3) dB HL for the right and left ears, respectively, with no difference to gender- and age-matched, otologically normal reference values. The average speech reception threshold in noise (SRT) was − 8.2 (SD 1.5) and − 8.0 (SD 1.7) dB SNR for the right and left ears, respectively, which did not differ from a control group with a comparable range in PTA thresholds. Conclusion Although a significant proportion of AGel amyloidosis patients experience subjective difficulties in hearing there seems to be no peripheral or central hearing impairment at least in patients up to the age of 60 years.

2020 ◽  
Vol 14 (2) ◽  
pp. 106-114
Author(s):  
Abdullahi Musa Kirfi ◽  
◽  
Mohammed Bello Fufore ◽  
Garba Mohammed Mainasara ◽  
Abdulrazak Ajiya ◽  
...  

Background: Prisoners, due to confinement are isolated from contact with the society and access to many of the facilities, including medical care. This study aimed to evaluate the hearing threshold of inmates of Kaduna convict prison. Method: It was a cross-sectional study of prison inmates at the Kaduna convict prison between April 2017 and February 2019. Ethical approvals were obtained from relevant bodies and all consented inmates aged 18 – 55years in the Kaduna convict prison were enrolled. Equal number of control matched for age and gender were enrolled from the communities in Kaduna North Local Government Area. Data were collated using a structured questionnaire. A diagnostic Pure Tone Audiometry was performed to assess their hearing threshold. Statistical Package for Social Sciences (SPSS) version 20.0 was used for analysis. Results: Four hundred and thirty inmates and equal number of control group were enrolled. The mean age for the inmates and controls were 30.2±7.5 and 30.4±8.02 years respectively. There were 383 males and 47 females in both groups. Among the 860 ears of the inmates, 238(27.7%) ears had hearing loss while in the control group, 95/ (11.1%) ears had impaired hearing. Conductive hearing loss was the commonest among the inmates 111(46.6%) while sensorineural was commoner among the controls 57(60.0%). The mean pure tone average among the inmates was 25.6±11.3dBHL and 26.1±11.2dBHL on the right and left ears respectively while in the control group, it was 18.4±7.8dBHL on the right and 17.9±7.9dBHL on the left. Conclusion: This study revealed that hearing loss was more prevalent among prison inmates than the general population. In majority of the inmates, the hearing loss was mild, conductive and mostly affecting all the frequencies.


2021 ◽  
Author(s):  
Abubakar O Aliyu ◽  
Waheed A Adedeji ◽  
Adekunle Daniel ◽  
Tajudeen Yusuf ◽  
Paul A Onakoya ◽  
...  

Abstract Introduction: Navy personnel are exposed to several risk factors that increase hearing thresholds and impair hearing. The aim of the study was to determine the prevalence of hearing impairment among Nigerian Navy personnel. Materials and Method: This was a cross sectional study of Nigerian Navy personnel working in various ships. Structured, self-administered questionnaire was used to obtain information on hearing. All the subjects had ear examination and pure tone audiometry of both ears. Results: The prevalence of hearing impairment was 22(14.7%) in the right and 17(11.3%) in the left ears. The Pure Tone Average for the right and left ear were 19.0±8.6dB and 17.4±6.7dB respectively. There was a statistically significant association between noisy work place and total hearing loss (p<.01), pure tone average in the left ear (p<.05) and frequencies of 3000 Hz (p<.05), 2000 Hz (p<.001) and 1000Hz (p<.05) in the left ear. The association between past history of ear infections and pure tone average in the left ear (p<.05) and the frequency 2000 Hz (p<.005) in the left ear were significant. The length of service years, occupational group, age, sex report of change in hearing, non-use of hearing protection and presence of tinnitus did not show significant association with pure tone averages nor on the frequencies tested. Conclusion: The significant unilateral hearing impairment on the right ear suggests the need for more research on hearing impairment in the Nigerian Navy as well as development of a comprehensive hearing conservation programme.


2011 ◽  
Vol 22 (08) ◽  
pp. 550-559 ◽  
Author(s):  
Samuli Hannula ◽  
Risto Bloigu ◽  
Kari Majamaa ◽  
Martti Sorri ◽  
Elina Mäki-Torkko

Background: There are not many population-based epidemiological studies on the association between self-reported hearing problems and measured hearing thresholds in older adults. Previous studies have shown that the relationship between self-reported hearing difficulties and measured hearing thresholds is unclear and, according to our knowledge, there are no previous population-based studies reporting hearing thresholds among subjects with hyperacusis. Purpose: The aim was to investigate the prevalence of self-reported hearing problems, that is, hearing difficulties, difficulties in following a conversation in noise, tinnitus, and hyperacusis, and to compare the results with measured hearing thresholds in older adults. Research Design: Cross-sectional, population-based, and unscreened. Study Sample: Random sample of subjects (n = 850) aged 54-66 yr living in the city of Oulu (Finland) and the surrounding areas. Data Collection and Analysis: Otological examination, pure tone audiometry, questionnaire survey Results: The prevalence of self-reported hearing problems was 37.1% for hearing difficulties, 43.3% for difficulties in following a conversation in noise, 29.2% for tinnitus, and 17.2% for hyperacusis. More than half of the subjects had no hearing impairment, or HI (BEHL[better ear hearing level]0.5–4 kHz < 20 dB HL) even though they reported hearing problems. Subjects with self-reported hearing problems, including tinnitus and hyperacusis, had significantly poorer hearing thresholds than those who did not report hearing problems. Self-reported hearing difficulties predicted hearing impairment in the pure-tone average at 4, 6, and 8 kHz, and at the single frequency of 4 kHz. Conclusions: The results indicate that self-reported hearing difficulties are more frequent than hearing impairment defined by audiometric measurement. Furthermore, self-reported hearing difficulties seem to predict hearing impairment at high frequencies (4–8 kHz) rather than at the frequencies of 0.5–4 kHz, which are commonly used to define the degree of hearing impairment in medical and legal issues.


2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Dimitris G. Balatsouras ◽  
George Koukoutsis ◽  
Panayotis Ganelis ◽  
George S. Korres ◽  
Andreas Aspris ◽  
...  

Introduction. Otitis media with effusion is a common pediatric disease whose diagnosis is based on pneumatic otoscopy, pure-tone audiometry, and tympanometry. The aim of this study was to evaluate transiently evoked otoacoustic emissions in the diagnosis of otitis media with effusion as compared to tympanometry.Patients and Methods. 38 children with bilateral otitis media with effusion were studied. 40 normal children of similar age and sex were used as controls. All subjects underwent pneumatic otoscopy, standard pure-tone audiometry, tympanometry, and transiently evoked otoacoustic emissions.Results. In the group of children with bilateral otitis media, transiently evoked otoacoustic emissions were absent in 51 ears (67%). In the remaining 25 ears (33%) the mean emission amplitude was reduced, as compared to the mean value of the control group.Conclusions. Transiently evoked otoacoustic emissions should be included in the diagnostic workup of otitis media with effusion because it is a fast, reliable, and objective test. Transiently evoked otoacoustic emissions should always be used in conjunction with tympanometry, because a more meaningful interpretation of transiently evoked otoacoustic emissions measures is possible.


2016 ◽  
Vol 27 (08) ◽  
pp. 677-682 ◽  
Author(s):  
Sofia Margarida Marques de Paiva ◽  
João Simões ◽  
António Paiva ◽  
Craig Newman ◽  
Francisco Castro e Sousa ◽  
...  

Background: The use of the Hearing Handicap Inventory for the Elderly (HHIE) questionnaire enables us to measure self-perceived psychosocial handicaps of hearing impairment in the elderly as a supplement to pure-tone audiometry. This screening instrument is widely used and it has been going through adaptations and validations for many languages; all of these versions have kept the validity and reliability of the original version. Purpose: To validate the HHIE questionnaire, translated into Portuguese of Portugal, on the Portuguese population. Research Design: This study is a descriptive correlational qualitative study. The authors performed the translation from English into Portuguese, the linguistic adaptation, and the counter translation. Study Sample: Two hundred and sixty patients from the Ear, Nose, and Throat (ENT) Department of Coimbra University Hospitals were divided into a case group (83 individuals) and a control group (177 individuals). Intervention: All of the 260 patients completed the 25 items in the questionnaire and the answers were reviewed for completeness. Data Collection and Analysis: The patients volunteered to answer the 25-item HHIE during an ENT appointment. Correlations between each individual item and the total score of the HHIE were tested, and demographic and clinical variables were correlated with the total score, as well. The instrument’s reproducibility was assessed using the internal consistency model (Cronbach’s alpha). Results: The questions were successfully understood by the participants. There was a significant difference in the HHIE-10 and HHIE-25 total scores between the two groups (p < 0.001). Positive correlations can be seen between the global question and HHIE-10 and HHIE-25. In the regression study, a relationship was observed between the pure-tone average and the HHIE-10 (p < 0.001). Reliability of the instrument was proven by a Cronbach alpha index of 0,79. Conclusions: The HHIE translation into Portuguese of Portugal maintained the validity of the original version and it is useful to assess the psychosocial handicap of hearing impairment in the elderly.


Author(s):  
Graziela Maria Martins-Moreira ◽  
Alessandra Spada Durante

Abstract Introduction Good hearing in pilots, including central auditory skills, is critical for flight safety and the prevention of aircraft accidents. Pure tone audiometry alone may not be enough to assess hearing in the members of this population who, in addition to high noise levels, routinely face speech recognition tasks in non-ideal conditions. Objective To characterize the frequency-following response (FFR) of a group of military pilots compared with a control group. Methods Twenty military pilots in the Study Group and 20 non-pilot military personnel, not exposed to noise in their work, in the Control Group, all with normal hearing, aged between 30 and 40 years old, completed a questionnaire to assess their hearing habits, and their FFRs were measured with a /da/ syllable (duration 40 milliseconds, speed 10.9/s), at 80 dB NA in the right ear. All procedures were approved by the ethical committee of the institution. Statistical analysis was performed using the t-Student or Mann-Whitney tests for quantitative variables, and the Fisher or chi-squared tests for qualitative variables, and a value of p < 0.05 was considered to be statistically significant. Results There was no significant difference between the groups regarding auditory habits. In the FFR, wave amplitudes A (p = 0.01) and C (p = 0.04) were significantly lower in the Study Group. Conclusion Working as a military pilot can be a crucial factor in determining an individual's typical FFR pattern, demonstrated in the present study by statistically significant reductions in the amplitudes of the A and C waves.


Author(s):  
Kapildev Mondal ◽  
Poulomi Saha

Hepatitis B has been documented to cause various extra hepatic manifestations along with known hepatic complications. It has been reported that hepatitis-B patients are more susceptible to inner ear damage and hearing loss. The aim of this study is to evaluate hearing loss among patients of   hepatitis B {all 6 categories Hepatitis B infection: chronic Hepatitis B  infection , hepatitis B cirrhosis ,Hepatitis B virus carriers , occult chronic Hepatitis B and Hepatitis B infection with poly arthritis nodosa, hepato cellular carcinoma with hepatitis B}compared with healthy subjects. METHOD: In this case control study 100 Hepatitis B positive patients and 100 age and gender-matched healthy individuals were included over the period of 5 years. All of them were known cases of chronic hepatitis B positive for   HBsAg at least for 18 months. All   patients were aged 18 to 50 years to exclude presence of presbycusis. After base line investigations, they were subjected for all cases and controls were subjected otoscopic examination and hearing assessment using standard pure tone audiometry. Descriptive statistical analysis has been carried out in this study. RESULT: In patients of Hepatitis B (94 patients,6 patients had of  natural death ) pure tone average (mean thresholds 250,500, 1000,2000,4000 &8000 Hz) was 28.4 dB in the right ear and 27.3 dB in the left (hearing loss).In the control group(96 patients,4 patients dropped out), PTA average was 9.9 dB in the right ear and 9.3dB in the left (normal hearing). In both groups, Speech Discrimination score (SDS) was100% in both ears. The percentage of hearing loss in the right and left ear over the total of six frequencies differed significantly in the two groups. Out of 94 patients of control group, 38 patients (40.4%) patients presented with Chronic Liver Disease (CLD), 14 patients (14.8%) patients presented with cirrhosis with Hepatitis B, 6 (6.3%) patients had Poly arthritis Nodosa with Hep-B, 18(19.1%) patients were diagnosed as carrier of Hepatitis-B , 11(11.7%) patients had occult Hepatitis-B and 7(7.4%) patients were diagnosed with hepato cellular carcinoma. Hearing loss was maximum in patients of   PAN with Hep-B. Second highest mean SNHL was seen in patients of Hep-B with cirrhosis .Third highest mean hearing loss was noted in patients with HCC .Forth highest mean hearing loss was noted in patients with occult Hep-B. Fifth highest mean hearing loss was noted in carriers of Hep-B.Lowest group with SNHL was chronic liver disease. CONCLUSION: Regular audiometric tests are recommended for patients with HBV infection to assess their hearing ability and enable the earlier detection of SNHL. We also suggest that HBV presenting with the sudden onset of hearing loss should be examined for the possibility of acute exacerbation of chronic HBV infection. KEYWORDS: Mean, Sensorineural, Hearing loss, Cirrhosis.


2021 ◽  
Vol 9 ◽  
Author(s):  
Abdoulaye Yalcouyé ◽  
Oumou Traoré ◽  
Abdoulaye Taméga ◽  
Alassane B. Maïga ◽  
Fousseyni Kané ◽  
...  

Objectives: To identify the etiologies of hearing impairment (HI) in schools for students who are deaf and to use a systematic review to summarize reports on the etiologies and clinical and genetic features of HI in Mali.Methods: We included individuals with HI that started before the age of 15 years old. Patients were carefully evaluated under standard practices, and pure-tone audiometry was performed where possible. We then searched for articles published on HI in the Malian population from the databases' inception to March 30, 2020.Results: A total of 117 individuals from two schools for the deaf were included, and a male predominance (sex ratio 1.3; 65/52) was noted. HI was pre-lingual in 82.2% (n = 117), and the median age at diagnosis was 12 years old. The etiologies were environmental in 59.4% (70/117), with meningitis being the leading cause (40%, 20/70), followed by cases with genetic suspicion (29.3%, 21/117). In 11.3% (8/117) of patients, no etiology was identified. Among cases with genetic suspicion, three were syndromic, including two cases of Waardenburg syndrome, while 15 individuals had non-syndromic HI. An autosomal recessive inheritance pattern was observed in 83.3% of families (15/18), and consanguinity was reported in 55.5% (10/18) of putative genetic cases.Conclusion: This study concludes that environmental factors are the leading causes of HI in Mali. However, genetic causes should be investigated, particularly in the context of a population with a high consanguinity rate.


Sains Medika ◽  
2015 ◽  
Vol 6 (1) ◽  
pp. 12
Author(s):  
Citta Arunika Risyudhanti ◽  
Agung Sulistyanto ◽  
Suparmi Suparmi

Introduction: Hearing impairment due to cochlear damage is one complication of Type 2 Diabetes Mellitus (DM2). Objective: This study aims to determine the relationship between duration of DM2 with hearing impairment.Method: The analytic observasional study with the cross sectional design using the medical record of DM2 outpatient in internal clinic of Islamic Sultan Agung Hospital Semarang and also pure tone audiometric examination’s record in Otorynolaryngology clinic of Islamic Sultan Agung Hospital Semarang.31 medical records of DM2 patients were meeting the inclusion and exclusion criteria. The data consist of 18 medical records of patients with less than6 years duration of DM2, and 13 medical records of patients with more than 6 years duration of DM2. The pure tone audiometry was used to classified the hearing impairment. The data analysis was conducted using SPSS 20.0. The correlation between the duration of DM2 and hearing impairment was analyzed using Chi Square test. The significance relation between the duration of DM type 2 and hearing impairment was analyzed using cooficient contigency, with significance of < 0.05. Result: hearing impairment’s distribution frequency on tested ears in Sultan agung Islamic Hospital Semarang were 27 ears having SNHL type (43,5%), 19 ears having CHL (30,6%) and 16 ears were normal (25,8%), p = 0,02 (p < 0,05). Conclusion: There is a weak correlation between the DM 2 duration and the incidence of hearing impairment in Sultan Agung Islamic Hospital Semarang.


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