Mental Fatigue in Patients with Hearing Loss and/or Tinnitus Undergoing Audiological Rehabilitation-A Pilot Study.

BACKGROUND: Both tinnitus and hearing loss are multidimensional. The purpose of this study was to identify and determine the degree of mental fatigue in patients with hearing loss and/or tinnitus participating in audiological rehabilitation, and to examine the self-reported mental fatigue scale (MFS) in this patient group. METHODS: Patients undergoing audiological rehabilitation at the Department of Audiology and Neurotology, Karolinska University Hospital, Sweden, between 2011 and 2017 who completed a self-reported MFS questionnaire were investigated. Data on 76 patients were analysed in this pilot study. Patients were also assessed using the Tinnitus Handicap Inventory (THI). RESULTS: The study population had an age range of 38-65 years, and most had normal hearing (37%) or mild to moderate hearing loss (46%). Only 17% had severe to profound hearing loss. A total of 56.5% had tinnitus, of whom 39.5% scored ≥57 on the THI, indicating severe tinnitus, whereas 43.5% reported no tinnitus. The MFS scores, ranging from 13 to 42.5 points, were divided into three severity levels for analysis: 10.5-15, 15.5-20, and ≥20.5. In total, 67% of the patients had MFS scores ≥ 20.5. Importantly, most of the participants (90%) with a THI score ≥ 57 belonged to that group. A significant positive correlation between a high MFS score and severe tinnitus was found. CONCLUSIONS: The study reveals that severe mental fatigue is more common in patients with severe tinnitus than sole hearing loss.

Congenital cytomegalovirus infection - a common cause of hearing loss of unknown aetiology.

AIM: The aim of this study was to investigate the role of congenital cytomegalovirus (CMV) infection as a cause of various types of sensorineural hearing loss (SNHL) in a group of nonsyndromic children with otherwise unknown aetiology of hearing loss. Furthermore, the occurrence of combined congenital CMV infection and connexin 26 (Cx26) mutations was investigated. METHODS: The dried blood spot (DBS) cards of 45 children with various degrees of hearing deficits and 46 children with severe/profound hearing loss were tested for CMV DNA with polymerase chain reaction (PCR) technique. The DBS cards of the 46 children with severe/profound hearing loss were also analysed for Cx26 mutations. RESULTS: Of the 45 children with various degrees of hearing loss, nine were positive for CMV DNA (20%). The nine children represented severe/profound, mild and unilateral hearing loss. From the 46 children with severe/profound hearing loss, nine of 46 (20%) were positive for CMV DNA. In addition, three of the CMV DNA-positive children were carriers of mutations of Cx26. CONCLUSION: Congenital CMV infection is a high risk factor in hearing impairment among children.

Rehabilitation of Severe-to-Profound Hearing Loss in Adults in Sweden.

Severe-to-profound hearing loss (STPHL) can affect a person negatively in many ways. Audiological rehabilitation is important for these patients. Patients receiving cochlear implants make up less than 10% of this group but have been studied extensively. In 2005, a national registry for adult patients with STPHL was introduced in Sweden. Its purpose was to evaluate and improve rehabilitation for all patients with STPHL. Data from the Swedish registry for adult patients with STPHL were used to evaluate variables affecting the audiological rehabilitation. Previous published data from the registry were reviewed, and new data from the follow-up questionnaire were presented. More than 90% of patients rehabilitated with hearing aids experienced a good or very good benefit of audiological rehabilitation. Tinnitus and vertigo affected quality of life negatively and were reported by many patients with STPHL (41% and 31%) at follow-up. To maintain the high number of patients who find audiological rehabilitation beneficial, individualized treatment plans and timely re-evaluations are crucial. Tinnitus and vertigo need to be addressed repeatedly in the rehabilitation process.

Prevalence of severe-to-Profound hearing loss in the adult Swedish population and comparison with cochlear implantation rate.

BACKGROUND: The prevalence of disabling hearing loss is increasing worldwide. However, previous studies on hearing loss prevalence have enrolled small populations or only provided estimates. AIM: To establish the prevalence of severe-to-profound hearing loss (STPHL) in the adult Swedish population and compare it with the cochlear implantation rate in Sweden. MATERIAL AND METHODS: We established a database containing over 15 million audiograms obtained from regions covering > 99% of the Swedish population by extracting audiogram data from the computer software application, Auditbase. We used this database to calculate the percentage of adult patients with bilateral hearing levels ≥ 70 dB. We collected data regarding cochlear implantations in Sweden from the National Board of Welfare and Health. RESULTS: The prevalence of STPHL in the adult Swedish population was 0.28%. There were regional variations in the prevalence and rate of cochlear implantation; however, there was no association between both parameters. CONCLUSIONS: This study presents an updated and reliable prevalence figure for STPHL in Sweden. SIGNIFICANCE: Patients with STPHL have extensive rehabilitation requirements; accordingly, it is important to determine the accurate prevalence of STPHL to inform the allocation of adequate resources.

Quality of life, psychosocial consequences, and audiological rehabilitation after sudden sensorineural hearing loss.

OBJECTIVE: Sudden sensorineural hearing loss (SSHL) is characterized by a rapid loss of hearing, most often of cochlear origin. Very little attention has been paid in the literature to quality of life (QoL), psychosocial consequences and audiological rehabilitation after SSHL. DESIGN: We studied how level of hearing loss, hearing recovery, tinnitus and vertigo affect QoL after SSHL and the psychosocial consequences of SSHL in terms of sick leave. Furthermore, the audiological rehabilitation given to patients in connection with SSHL and the benefit of the rehabilitation were studied. STUDY SAMPLE: Three hundred and sixty-nine (369) patients with SSHL were analysed in the present study. RESULTS: Annoying tinnitus and remaining vertigo after SSHL were the strongest predictors of negative effects on QoL. CONCLUSIONS: The study indicates that patients with SSHL require extended audiological rehabilitation including a multi-disciplinary rehabilitation approach (medical, social and psychological) to cope with the complex issues that can arise after SSHL.

Analysis of gene polymorphisms associated with K ion circulation in the inner ear of patients susceptible and resistant to noise-induced hearing loss.

Noise-induced hearing loss (NIHL) is one of the leading occupational health risks in industrialized countries. It results from an interaction between environmental and genetic factors, however the nature of the genetic factors contributing to NIHL has not yet been clarified. Here, we investigated whether genetic variations in 10 genes putatively involved in the potassium recycling pathway in the inner ear may influence susceptibility to noise. 99 SNPs were genotyped in Polish noise-exposed workers, categorized into susceptible and resistant subjects. The most interesting results were obtained for KCNE1 and KCNQ4 as we replicated associations that were previously reported in a Swedish sample set, hence confirming that they are NIHL susceptibility genes. Additionally we report significant associations in GJB1, GJB2, GJB4, KCNJ10 and KCNQ1, however due to the lack of replication in the Swedish sample set, these results should be seen as suggestive.

Severe-to-profound hearing impairment: demographic data, gender differences and benefits of audiological rehabilitation.

Purpose: The purpose of this study was to identify and report demographic data of patients with severe-to-profound hearing loss, assess participation in audiological rehabilitation and analyze the benefits of various rehabilitation methods.Materials and methods: Data on 4286 patients with severe-to-profound hearing impairments registered in the Swedish Quality Register of Otorhinolaryngology over a period from 2006-2015 were studied. Demographic data, gender differences, audiological rehabilitation and benefits of the rehabilitation were analyzed.Results: Group rehabilitation and visits to a hearing rehabilitation educator provided the most benefits in audiological rehabilitation. Only 40.5% of the patients received extended audiological rehabilitation, of which 54.5% were women. A total of 9.5% of patients participated in group rehabilitation, with 59.5% being women. Women also visited technicians, welfare officers, hearing rehabilitation educators, psychologists and physicians and received communication rehabilitation in a group and fit with cochlea implants significantly more often than did men.Conclusions: The study emphasizes the importance of being given the opportunity to participate in group rehabilitation and meet a hearing rehabilitation educator to experience the benefits of hearing rehabilitation. There is a need to offer extended audiological rehabilitation, especially in terms of gender differences, to provide the same impact for women and men.Implications for RehabilitationSignificantly more women than men with severe-to-profound hearing impairment receive audiological rehabilitation.Hearing impairment appears to have a significantly more negative impact on women's quality of life than men's.It is important to offer extended audiological rehabilitation to all patients with severe-to-profound hearing loss to obtain an equal hearing health care regardless of gender.

Rehabilitation of adult patients with severe-to-profound hearing impairment - why not cochlear implants?

Background: In Sweden, an estimated prevalence of adult patients with severe-to-profound hearing loss is 0.2%, which corresponds to roughly 20,000. We know little about the use of cochlear implants (CIs) in this population and why not most of them are not offered CI. Objectives: To investigate the reasons for no rehabilitation with CI among this patient group. Materials and methods: Data were collected from 1076 patients in the Swedish Quality Register of Otorhinolaryngology. A baseline questionnaire and the reason for no CI, was evaluated. Results: Only 14.5% of the patients started a CI investigation, and 8.5% were rehabilitated with CI. Significantly more women (56.5%) than men received CI. The most common reasons for not receiving CI, were hearing reason (30.5%), indicating satisfaction with technical equipment, and unknown reason (25%). The oldest patient group (81-100 years old) had the highest risk for unknown reasons. Patients receiving extended audiological rehabilitation (53.5%) had a significantly lower risk for unknown reasons. Conclusions: It is worrying that the oldest patient group (81-100 years old) seemed to have fewer chances to start a CI investigation. An extended audiological rehabilitation increased the chances that professionals would discuss CI. Significance: This study shows that surprisingly few patients are offered CI despite their severe-to-profound hearing loss.

Patients with severe-to-profound hearing impairment and simultaneous severe vision impairment: a quality-of-life study.

CONCLUSIONS: Patients with severe vision impairment in combination with severe-to-profound hearing loss seem to have a higher risk for effects on QoL, including: mobility, the ability to provide self-care and perform usual activities, and levels of anxiety and depression, compared with patients with only severe-to-profound hearing loss. OBJECTIVES: To study the quality-of-life (QoL) and audiological rehabilitation of the severely vision-impaired patient population among adults with severe-to-profound hearing loss in Sweden. METHOD: A study of data collected from 543 patients with severe-to-profound hearing loss combined with severe vision impairment among the total of 2319 persons registered in the Swedish Quality Register of Otorhinolaryngology. QoL was measured with the following instruments: EQ5D, PIRS, and HADS. Audiological rehabilitation was described and evaluated. RESULTS: The patients with dual sensory loss were younger, were more likely to live alone, and had a lower level of education than the control group. The QoL of the study group was significantly negatively affected. In total, 89% of the study group had been rehabilitated with hearing aids, while 8% had received rehabilitation with cochlear implants. A total of 32% of the study group had received extended audiological rehabilitation.

The contribution of genes involved in potassium-recycling in the inner ear to noise-induced hearing loss.

Noise-induced hearing loss (NIHL) is one of the most important occupational diseases and, after presbyacusis, the most frequent cause of hearing loss. NIHL is a complex disease caused by an interaction between environmental and genetic factors. The various environmental factors involved in NIHL have been relatively extensively studied. On the other hand, little research has been performed on the genetic factors responsible for NIHL. To test whether the variation in genes involved in coupling of cells and potassium recycling in the inner ear might partly explain the variability in susceptibility to noise, we performed a case-control association study using 35 SNPs selected in 10 candidate genes on a total of 218 samples selected from a population of 1,261 Swedish male noise-exposed workers. We have obtained significant differences between susceptible and resistant individuals for the allele, genotype, and haplotype frequencies for three SNPs of the KCNE1 gene, and for the allele frequencies for one SNP of KCNQ1 and one SNP of KCNQ4. Patch-clamp experiments in high K+-concentrations using a Chinese hamster ovary (CHO) cell model were performed to investigate the possibility that the KCNE1-p.85N variant (NT_011512.10:g.21483550G>A; NP_00210.2:p.Asp85Asn) was causative for high noise susceptibility. The normalized current density generated by KCNQ1/KCNE1-p.85N channels, thus containing the susceptibility variant, differed significantly from that from wild-type channels. Furthermore, the midpoint potential of KCNQ1/KCNE1-p.85N channels (i.e., the voltage at which 50% of the channels are open) differed from that of wild-type channels. Further genetic and physiological studies will be necessary to confirm these findings.

[Cogan syndrome ­ when several of our senses are affected]. / Cogans syndrom - när flera av våra sinnen spökar - Ögon och öron påverkas.

Cogans syndrome is a rare systemic inflammatory disease characterized by a combination of audiovestibular and ocular symptoms. In some cases, systemic complications occur with vascular inflammation. Aortitis and large vessel vasculitis are the most common forms, but medium-sized and small vessel involvement has also been described. The autoimmune reaction can lead to blindness, deafness and in worst case death, if these patients remain untreated or if treatment is delayed. There is no specific blood test or imaging method available and the diagnosis is clinical. It should be suspected in patients presenting with both inflammatory eye symptoms and audiovestibular dysfunction, when more common autoimmune and infectious diseases have been excluded. The treatment consists of high dose systemic steroids, topical steroids for the affected eye and in some cases addition of immunosuppressive drugs. Treatment is based on the severity of the symptoms and how well the patient responds to initial systemic corticosteroids. Here we present a case of suspected Cogan's syndrome where diagnosis was considered after exclusion of other possible autoimmune and infectious diseases.

The influence of genetic variation in oxidative stress genes on human noise susceptibility.

Noise induced hearing loss (NIHL) is a complex disease caused by an interaction between genetic and environmental factors. Damage in the cochlea as a result of noise exposure appears to be mediated by reactive oxygen species (ROS). To investigate whether genetic variation in the human protective antioxidant system is associated with high or low susceptibility to NIHL, genetic polymorphisms derived from genes involved in the oxidative stress response were analysed in the 10% most susceptible and 10% most resistant extremes of 1200 Swedish noise-exposed workers. The genetic polymorphisms included 2 deletion polymorphisms for the GSTM1 and GSTT1 gene, and 14 SNPs derived from the CAT, SOD, GPX, GSR and GSTP1 genes. No significant differences were found between susceptible and resistant groups, providing no support for a major role of genetic variation of antioxidant enzymes in the susceptibility to NIHL.

Severe to profound hearing impairment: quality of life, psychosocial consequences and audiological rehabilitation.

PURPOSE: To study the quality of life (QoL) and psychosocial consequences in terms of sick leave and audiological rehabilitation given to patients with severe to profound hearing impairment. METHOD: A retrospective study of data on 2319 patients with severe to profound hearing impairment in The Swedish Quality Register of Otorhinolaryngology, followed by a posted questionnaire including The Hospital Anxiety and Depression Scale (HADS). RESULTS: The results indicate greater levels of anxiety and depression among patients with severe or profound hearing impairment than in the general population, and annoying tinnitus and vertigo had strong negative effects on QoL. The proportion of sick leave differed between the studied dimensions in the study. The proportion of patients who received extended audiological rehabilitation was 38% in the present study. CONCLUSIONS: Treatment focused on anxiety, depression, tinnitus and vertigo must be given early in the rehabilitation process in patients with severe or profound hearing impairment. Because sick leave differs greatly within this group of patients, collaboration with the regional Social Insurance Agency is crucial part of the rehabilitation. The study also shows that presently, only a small proportion of patients in Sweden with severe to profound hearing impairment receive extended audiological rehabilitation. Implications for Rehabilitation Greater levels of anxiety and depression have been found among patients with severe or profound hearing impairment than in the general population, and annoying tinnitus and vertigo have strong negative effects on QoL in this group of patients. Only a small proportion of patients with severe to profound hearing impairment receive extended audiological rehabilitation today, including medical, technical and psychosocial efforts. Extended audiological rehabilitation focused on anxiety, depression, tinnitus and vertigo must be given, together with technical rehabilitation, early in the rehabilitation process in patients with severe or profound hearing impairment.

GJB2 (Connexin 26) gene mutations among hearing-impaired persons in a Swedish cohort.

CONCLUSION: The most common mutation in the Swedish population was Connexin 26 (C×26) 35delG, which indicates that the percentage of Swedish persons with C×26 mutations and polymorphisms in the GJB2 gene among non-syndromic hearing-impaired (HI) persons is comparable to the rest of Europe. The results strongly support a Swedish policy to offer all children with diagnosed hearing impairment genetic tests for the C×26 35delG mutation. OBJECTIVES: The aim of the present study was to search for mutations in the GBJ2 gene among Swedish persons with non-syndromic hearing impairment to further clarify how common these mutations are in Sweden, one of the northernmost countries in Europe. METHODS: Seventy-nine patients with non-syndromic hearing impairment participated in the study. For 87% of the participants, a pure tone audiogram showed a severe or profound hearing impairment. Dried blood spots on filter paper, taken at 3-5 days of age in the Swedish nationwide neonatal screening programme for congenital disorders and saved in a biobank, were used for the molecular genetic analyses. RESULTS: The total number of subjects with one or two pathologic mutations or a mutation of unknown consequence found in the GJB2 gene was 28 of 79 (35%). Nineteen (19) persons (24%) were homozygotes for the 35delG mutation.

Variations in HSP70 genes associated with noise-induced hearing loss in two independent populations.

Noise-induced hearing loss (NIHL) is one of the most important occupational health hazards. Millions of people worldwide are exposed daily to harmful levels of noise. NIHL is a complex disease resulting from an interaction between genetic and environmental factors. Although the environmental risk factors have been studied extensively, little is known about the genetic factors. Heat-shock proteins (HSPs) are induced after exposure to severe noise. When first induced by exposure to moderate sound levels, they can protect the ear from damage from excessive noise exposure. This protection is highly variable between individuals. An association of HSP70 genes with NIHL has been described by Yang et al (2006) in a Chinese sample set of noise-exposed workers. In this study, three polymorphisms (rs1043618, rs1061581 and rs2227956) in HSP70-1, HSP70-2 and HSP70-hom, respectively, were genotyped in 206 Swedish and 238 Polish DNA samples of noise-exposed subjects and analyzed. One SNP, rs2227956 in HSP70-hom, resulted in a significant association with NIHL in both sample sets. In addition, rs1043618 and rs1061581 were significant in the Swedish sample set. Analysis of the haplotypes composed of the three SNPs revealed significant associations between NIHL and haplotype GAC in both sample sets and with haplotype CGT in the Swedish sample set. In conclusion, this study replicated the association of HSP70 genes with NIHL in a second and third independent noise-exposed sample set, hereby adding to the evidence that HSP70 genes may be NIHL susceptibility genes.

Association between variations in CAT and noise-induced hearing loss in two independent noise-exposed populations.

Noise-induced hearing loss (NIHL) is an important occupational hazard that results from an interaction between genetic and environmental factors. Although the environmental risk factors have been studied quite extensively, little is known about the genetic factors. On the basis of multiple studies, it was proposed that oxidative stress plays an important role in the development of NIHL. Here, we investigated whether variations (single nucleotide polymorphisms; SNPs) in the catalase gene (CAT), one of the genes involved in oxidative stress, influence noise susceptibility. Audiometric data from 1261 Swedish and 4500 Polish noise-exposed labourers were analysed. DNA samples were collected from the 10% most susceptible and the 10% most resistant individuals. Twelve SNPs were selected and genotyped. Subsequently, the interaction between noise exposure and genotypes and their effect on NIHL were analysed using logistic regression. Significant interactions were observed between noise exposure levels and genotypes of two SNPs for the Swedish population and of five SNPs for the Polish population. Two of these SNPs were significant in both populations. The interaction between predictor haplotypes and tagSNP haplotypes and noise exposure levels and their effect on NIHL were also analysed, resulting in several significant associations. In conclusion, this study identified significant associations between catalase SNPs and haplotypes and susceptibility to development of NIHL. These results indicate that catalase is a NIHL susceptibility gene, but that the effect of CAT polymorphisms can only be detected when noise exposure levels are taken into account.

Marital status and birthrate of deaf people in two Swedish counties: the impact of social environment in terms of deaf community.

Deafness affects many social interactions. The impact of deafness depends on several factors, e.g., the type of social environment in terms of the particular Deaf community a person lives in. The authors recorded the birthrate and the proportions of married and divorced people among deaf people in two Swedish counties: Narke, which had a strong Deaf community, and Varmland, whose Deaf community was weak. In both counties, the authors found that deafness was associated with low marriage rates and low birthrates. Further, in Narke, 99% of the people in the sample were married to another deaf person; only 10% were in Varmland. In Narke, the divorce rate among deaf people was about the same as that of the reference population (i.e., the county's marriage-age population). In Varmland, deaf people had a relatively low divorce rate. The findings are discussed from medical and social perspectives.