Unilateral hearing loss: benefits and satisfaction from the use of hearing aids

A unilateral hearing loss is characterized by reduced hearing in one ear. The problems caused by sensory deprivation can be minimized with the use of hearing aids (HA). Aim: To analyze the correlation between the prescribed grain and the insertion gain difference and with the results obtained regarding the benefit and satisfaction with the use of hearing aids in unilateral hearing impaired patients. Materials and Methods: Prospective study with 15 subjects, mean age of 41.6 years, of both genders, users of hearing aids effectively. We used the International Questionnaire Results for hearing aids (International Outcome Inventory for Hearing Aids - IOI-HA), measured with a probe microphone. Results: The mean values in the analyses of the IOI-HA per item were positive and higher than four points. In relation to the objective measures, the frequencies in which we obtained the gain values which were closer to the target were: 1K Hz, 2K Hz and 500 Hz, respectively. Conclusion: The satisfaction of individuals using hearing aid unilaterally is not completely correlated to the prescribed gain, because even if the target is not being reached in some frequencies, the individuals were pleased as to the use of their hearing aids.

Saethre-Chotzen Syndrome, Pro136His TWIST Mutation, Hearing Loss, and External and Middle Ear Structural Anomalies: Report on a Brazilian Family

Objective: To describe the clinical, speech, hearing, and imaging findings in three members of a Brazilian family with Saethre-Chotzen syndrome (SCS) who presented some unusual characteristics within the spectrum of the syndrome. Design: Clinical evaluation was performed by a multidisciplinary team. Direct sequencing of the polymerase chain reaction amplified coding region of the TWIST1 gene, routine and electrophysiological hearing evaluation, speech evaluation, and imaging studies through computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed. Results: TWIST1 gene analysis revealed a Pro136His mutation in all patients. Hearing evaluation showed peripherial and mixed hearing loss in two of the patients, one of them with severe unilateral microtia. Computed tomography scan showed structural middle ear anomalies, and MRI showed distortion of the skull contour as well as some of the brain structures. Conclusions: We report a previously undescribed TWIST1 gene mutation in patients with SCS. There is evidence that indicates hearing loss (conductive and mixed) can be related both with middle ear (microtia, high jugular bulb, and enlarged vestibules) as well as with brain stem anomalies. Here we discuss the relationship between the gene mutation and the clinical, imaging, speech, and hearing findings.

Conductive hearing loss in preterm infants with bronchopulmonary dysplasia

Objective: To determine the risk of conductive hearing loss in preterm infants with bronchopulmonary dysplasia (BPD) and preterm controls. Methodology: The study population consisted of 78 infants with BPD of 26-33 weeks gestation and 78 controls of similar gestational age matched for broad-based birthweight categories. An auditory brainstem response (ABR) audiology was performed shortly before hospital discharge. Visual reinforcement orientation audiometry (VROA) and impedance audiometry were performed at 8-12 months corrected for prematurity. Infants with persistent audiological abnormalities were referred for evaluation to paediatric ENT surgeons. Results: Infants with BPD had a significantly higher rate of ABR abnormalities (BPD: 22%, controls: 9%; P = 0.028). On VROA and impedance audiometry, the infants with BPD also had a higher rate of persistent abnormalities. Following ENT assessment, 22.1% of infants with BPD and 7.7% of controls had persistent conductive dysfunction requiring myringotomy and grommet tube insertion (P = 0.03). Most of these infants had normal ABR audiometry at hospital discharge. Conclusions: Preterm infants with BPD are at high risk of persistent conductive hearing loss late in the first year of life compared to controls. An ABR audiology conducted at the time of hospital discharge does not predict accurately later conductive hearing problems. Infants with BPD should have routine audiological evaluation toward the end of the first year of life.

Validity of self-reported hearing loss in adults: performance of three single questions

OBJECTIVE: To estimate the validity of three single questions used to assess self-reported hearing loss as compared to pure-tone audiometry in an adult population. METHODS: A validity study was performed with a random sub-sample of 188 subjects aged 30 to 65 years, drawn from the fourth wave of a population-based cohort study carried out in Salvador, Northeastern Brazil. Data were collected in household visits using questionnaires. Three questions were used to separately assess self-reported hearing loss: Q1, "Do you feel you have a hearing loss?"; Q2, "In general, would you say your hearing is 'excellent,' 'very good,' 'good,' 'fair,' 'poor'?"; Q3, "Currently, do you think you can hear 'the same as before', 'less than before only in the right ear', 'less than before only in the left ear', 'less than before in both ears'?". Measures of accuracy were estimated through seven measures including Youden index. Responses to each question were compared to the results of pure-tone audiometry to estimate accuracy measures. RESULTS: The estimated sensitivity and specificity were 79.6%, 77.4% for Q1; 66.9%, 85.1% for Q2; and 81.5%, 76.4% for Q3, respectively. The Youden index ranged from 51.9% (Q2) to 57.0% (Q1) and 57.9% (Q3). CONCLUSIONS: Each of all three questions provides responses accurate enough to support their use to assess self-reported hearing loss in epidemiological studies with adult populations when pure-tone audiometry is not feasible.

Analysis of the combined exposure to noise and ototoxic substances—pilot study

Abstract: Ototoxic substances have been associated to damage of the auditory system, and its effects are potentiated by noise exposure. The present study aims at analyzing auditory changes from combined exposure to noise and organic solvents, through a pilot study in the furniture industry sector. Audiological tests were performed on 44 workers, their levels of exposure to toluene, xylene and ethylbenzene were determined and the levels of noise exposure were evaluated. The results showed that workers are generally exposed to high noise levels and cabin priming filler and varnish sector workers have high levels of exposure to toluene. However, no hearing loss was registered among the workers. Workers exposed simultaneously to noise and ototoxic substances do not have a higher degree of hearing loss than those workers exposed only to noise. Thus, the results of this study did not show that the combined exposure to noise and the organic solvent is associated with hearing disorders.

Evaluation of the noise exposure of symphonic orchestra musicians

For musicians, the impact of noise exposure is not yet fully characterized. Some inconsistencies can be found in the methodology used to evaluate noise exposure. This study aims to analyze the noise exposure of musicians in a symphonic orchestra to understand their risk for hearing loss, applying the methodology proposed by ISO 9612:2009. Noise levels were monitored among musicians during the rehearsal of eight different repertoires. Test subjects were selected according to their instrument and position in the orchestra. Participants wore noise dosimeters throughout the rehearsals. A sound meter was used to analyze the exposure of the conductor. The results showed that musicians are exposed to high noise levels that can damage hearing. Brass, woodwind and percussion and timpani musicians were exposed to noise levels in excess of the upper exposure action level of 85 dB (A), while the other instrumental groups had a lower exposure action level of 80 dB (A). Percussion musicians were exposed to high peak noise levels of 135 dB (C). Sound levels varied by instrument, repertoire and position. Octave frequency analyses showed differences among musicians. This study suggests that musicians are at risk for hearing loss. There is a need for more effective guidelines applicable to all countries, which should define standardized procedures for determining musician noise exposure and should allow exposure level normalization to the year, including different repertoires.

Gender and other factors associated with the use of hearing protection devices at work

OBJECTIVE To analyze whether sociodemographic, occupational, and health-related data are associated with the use of hearing protection devices at work, according to gender. METHODS A cross-sectional study was conducted in 2006, using a random sample of 2,429 workers, aged between 18 and 65 years old, from residential sub-areas in Salvador, BA, Northeastern Brazil. Questionnaires were used to obtain sociodemographic, occupational, and health-related data. Workers who reported that they worked in places where they needed to shout in order to be heard were considered to be exposed to noise. Exposed workers were asked whether they used hearing protection devices, and if so, how frequently. Analyses were conducted according to gender, with estimates made about prevalence of the use of hearing protection devices, prevalence ratios, and their respective 95% confidence intervals. RESULTS Twelve percent (12.3%) of study subjects reported that they were exposed to noise while working. Prevalence of the use of hearing protection devices was 59.3% for men and 21.4% for women. Men from higher socioeconomic levels (PR = 1.47; 95%CI 1.14;1.90) and who had previous audiometric tests (PR = 1.47; 95%CI 1.15;1.88) were more likely to use hearing protection devices. For women, greater perceived safety was associated with the use of protection devices (PR = 2.92; 95%CI 1.34;6.34). This perception was specifically related to the presence of supervisors committed to safety (PR = 2.09; 95%CI 1.04;4.21), the existence of clear rules to prevent workplace injuries (PR = 2.81; 95%CI 1.41;5.59), and whether they were informed about workplace safety (PR = 2.42; 95%CI 1.23;4.76). CONCLUSIONS There is a gender bias regarding the use of hearing protection devices that is less favorable to women. The use of such devices among women is positively influenced by their perception of a safe workplace, suggesting that gender should be considered as a factor in hearing conservation programs.

Spectrum and Frequency of GJB2 Mutations in a Cohort of 264 Portuguese Nonsyndromic Sensorineural Hearing Loss Patients

OBJECTIVE: To assess the spectrum and prevalence of mutations in the GJB2 gene in Portuguese nonsyndromic sensorineural hearing loss (NSSHL) patients. DESIGN: Sequencing of the coding region, basal promoter, exon 1, and donor splice site of the GJB2 gene; screening for the presence of the two common GJB6 deletions. STUDY SAMPLE: A cohort of 264 Portuguese NSSHL patients. RESULTS: At least one out of 21 different GJB2 variants was identified in 80 (30.2%) of the 264 patients analysed. Two mutant alleles were found in 53 (20%) of these probands, of which 83% (44/53) harboured at least one c.35delG allele. Twenty-seven (10.2%) of the probands harboured only one mutant allele. Subsequent analysis revealed that the GJB6 deletion del(GJB6-D13S1854) was present in at least 7.4% (2/27) of the patients carrying only one mutant GJB2 allele. Overall, one in five (55/264) of the patients were diagnosed as having DFNB1-related NSSHL, of which the vast majority (53/55) harboured only GJB2 mutations. CONCLUSIONS: This study provides clear demonstration that mutations in the GJB2 gene are an important cause of NSSHL in Portugal, thus representing a valuable indicator as regards therapeutical and rehabilitation options, as well as genetic counseling of these patients and their families.

Sudden sensorineural hearing loss : evaluation of co-morbidities and potential clinical associations

RESUMO: A surdez súbita (SS) caracteriza-se por uma perda abrupta de audição, mais frequentemente unilateral e associada a sensação de preenchimento aural, acufenos e vertigem. Afecta 5-20/100.000pessoas/ano (sobretudo adultos em fase activa na década de 40), com grande impacto na qualidade de vida. Possíveis causas incluem doenças infecciosas, circulatórias, traumáticas, imunológicas, neoplásicas, neurológicas, tóxicas e cocleares. No entanto, a causa da SS permanece desconhecida na maioria dos casos (80%), o que origina tratamentos controversos e frequentemente ineficientes. Os tratamentos disponíveis variam desde corticosteróides a antivirais, vasodilatadores, anti-agregantes, anticoagulantes, vitaminas e oxigénio hiperbárico (OHB). Atendendo a falta de informação relativa à etiologia e fisiopatologia da SS, pretendemos avaliar a evolução clínica dos doentes com SS tratados com OHB no Centro de Medicina Subaquática e Hiperbárica (CMSH) de Lisboa entre 2000 e 2005, durante um período mínimo de 5 anos, na tentativa de identificar eventuais factores de risco ou noxas clínicas com a SS. O estudo retrospectivo proposto baseia-se na revisão de processos clínicos do CMSH e na aplicação telefónica de questionários médicos de “follow-up” confidenciais – tanto a doentes (grupo de estudo), como aos respectivos esposos/companheiros/membros próximos da família (grupo de controlo) –, com particular ênfase nos antecedentes médicos e história clínica actual. Um estudo preliminar de 20 pessoas (10 doentes e 10 controlos) foi efectuado para antecipar dificuldades e estimar as necessidades logísticas. As dificuldades identificadas foram: 1) selecção dos doentes com números de telefone válidos e processos clínicos completos (com audiograma inicial e final); 2) contacto telefónico com os participantes de ambos os grupos (de estudo e controlo); 3) recursos humanos requeridos. Dado que a SS não é uma doença em si, mas um sintoma de uma doença subjacente, acreditamos que este estudo epidemiológico seja importante e útil, capaz de gerar novas luzes sobre a fisiopatologia e mecanismos desta entidade clínica.-------------ABSTRACT:Sudden sensorineural hearing loss (SSHL) is characterized by abrupt, mostly unilateral loss of hearing, frequently associated to aural fullness, tinnitus and vertigo. It affects 5-20/100.000 people/year (particularly working adults in the 40ths), with huge impact on quality of life. Possible causes include infectious, circulatory, traumatic, immunologic, metabolic, neoplastic, neurologic, toxic and unidentified cochlear diseases. Nevertheless, SSHL’s etiology remains unknown in most cases (80%), giving rise to controversial (and frequently ineffective) treatments. Available therapies range from corticosteroids to antivirals, vasodilators, antiaggregants, anticoagulants, vitamins and hyperbaric oxygen (HBO). Given the lack of data concerning SSHL’s etiology and physiopathology, we intend to evaluate clinical evolution of such patients treated with HBO in the Underwater and Hyperbaric Medical Center (UHMC) at Lisbon from 2000 to 2005 during a minimum period of 5 years, in an attempt to identify eventual risk factors or clinical associations to SSHL. The intended retrospective study is based on the review of patients’ medical charts from UHMC and confidential follow-up questionnaires applied telephonically both to patients (study group) and patients’ spouse/partner/close family member (control group), focusing past and present medical history. A preliminary study of 20 subjects (10 of each group) was performed to anticipate difficulties and to estimate the required logistics. The identified difficulties were: 1) selection of subjects with valid phone numbers and complete medical charts (with initial and final audiograms); 2) telephonic contact with subjects from the study and control group; 3) human logistics required. As it is believed that SSHL is not a disease by itself but rather a symptom of an underlying disease, we believe that this epidemiologic study is important and will hopefully generate sound scientific knowledge concerning physiopathology and mechanism of disease of SSHL.

Myelopathy and adult T-cell leukemia associated with HTLV-1 in a young patient with hearing loss as the initial manifestation of disease

A young male developed hearing loss, vertigo, headache and facial palsy. Neurological examination did not show any abnormalities. Two years later, cervical lymphadenopathy, hepatosplenomegaly and atypical lymphocytes in peripheral blood revealed leukemia. At the same time, acquired ichthyosis was observed. Subsequently, neurological abnormalities revealed myelopathy associated with HTLV-1, due to vertical transmission.

Mitigating the impact of occupational noise exposure for elderly workers: Setting the functional requirements for an ANC system

Noise affects people in very different aspects and in almost every aspect of our daily life. The most prominent impact of noise exposure is hearing loss. However, it can also impair people at their work settings due to other effects rather than hearing loss. Older works tend to be more susceptible to noise exposure effects at work, firstly because most of them already have some ‘natural’ hearing loss, as a results of the ageing process, and secondly because they also tend to be more susceptible at an psychological level. The current study is an attempt to describe the potential problem and to make a survey to identify the available active noise cancelation systems, as well as to specific the main requirements of this type of systems to be applied in such contexts. Several aspects of characteristics of the ANC systems were identified and are presented in this study. From the obtained results it was possible to have a clearer idea about the potential of this technology, and to confirm that this type of solution can be extremely important as a component of an active ageing program, as the preservation of hearing will also impact on the social life of the exposed workers.

A New Large Deletion in the DFNB1 Locus Causes Nonsyndromic Hearing Loss.

Mutations in the GJB2 gene encoding the gap junction protein connexin 26 are responsible for up to 30% of all cases of autosomal recessive nonsyndromic hearing impairment (HI) with prelingual onset in most populations. The corresponding locus DFNB1, located on chromosome 13q11-q12, is also affected by three distinct deletions. These deletions extended distally to GJB2, which remains intact. We report a novel large deletion in DFNB1 observed in a patient presenting profound prelingual HI. This deletion was observed in trans to a GJB2 mutated allele carrying the p.Val84Met (V84M) mutation and was shown to be associated with hearing loss. The deletion caused a false homozygosity of V84M in the proband. Quantification of alleles by quantitative fluorescent multiplex PCR (QFM-PCR) enabled us to study the breakpoints of the deletion. The deleted segment extended through at least 920kb and removed the three connexin genes GJA3, GJB2 and GJB6. The distal breakpoint inside intron 2 of CRYL1 gene differed from the breakpoints of the known DFNB1 deletions. This case highlights the importance of screening for large deletions in molecular studies of GJB2.

An integrated genetic and functional analysis of the role of type II transmembrane serine proteases (TMPRSSs) in hearing loss.

Building on our discovery that mutations in the transmembrane serine protease, TMPRSS3, cause nonsyndromic deafness, we have investigated the contribution of other TMPRSS family members to the auditory function. To identify which of the 16 known TMPRSS genes had a strong likelihood of involvement in hearing function, three types of biological evidence were examined: 1) expression in inner ear tissues; 2) location in a genomic interval that contains a yet unidentified gene for deafness; and 3) evaluation of hearing status of any available Tmprss knockout mouse strains. This analysis demonstrated that, besides TMPRSS3, another TMPRSS gene was essential for hearing and, indeed, mice deficient for Hepsin (Hpn) also known as Tmprss1 exhibited profound hearing loss. In addition, TMPRSS2, TMPRSS5, and CORIN, also named TMPRSS10, showed strong likelihood of involvement based on their inner ear expression and mapping position within deafness loci PKSR7, DFNB24, and DFNB25, respectively. These four TMPRSS genes were then screened for mutations in affected members of the DFNB24 and DFNB25 deafness families, and in a cohort of 362 sporadic deaf cases. This large mutation screen revealed numerous novel sequence variations including three potential pathogenic mutations in the TMPRSS5 gene. The mutant forms of TMPRSS5 showed reduced or absent proteolytic activity. Subsequently, TMPRSS genes with evidence of involvement in deafness were further characterized, and their sites of expression were determined. Tmprss1, 3, and 5 proteins were detected in spiral ganglion neurons. Tmprss3 was also present in the organ of Corti. TMPRSS1 and 3 proteins appeared stably anchored to the endoplasmic reticulum membranes, whereas TMPRSS5 was also detected at the plasma membrane. Collectively, these results provide evidence that TMPRSS1 and TMPRSS3 play and TMPRSS5 may play important and specific roles in hearing.