The current scientific literature reports on the incidence of hearing impairments due to HIV/AIDS, and the hearing changes can occur due to damage to the outer, middle or inner ear. Thus, it is important to study how these changes occur, the hearing loss and their associations with the HIV/AIDS infection.To identify the factors related to hearing loss in people with HIV/AIDS in the global scientific literature.Study carried out an Integrative Review of the Literature. The key words used were: hearing loss, hearing disorders and deafness, separately associated to the keyword HIV on PUBMED, ScIELO, LILACS and ISI databases. We used complete original papers, of free access, in English, Spanish, French and Portuguese. Thirteen quantitative studies from 1994-2010 were selected.We did not find any strong direct association between anti-retroviral therapy and hearing loss; however, there are indications of hearing loss in the population studied, and their associations and causes need to be better investigated. In recent years, there has been promising developments in world initiatives to approach the AIDS epidemics, including a greater access to efficient treatment and prevention programsConsidering such challenges, treatment with antiretroviral (ARV) medication is one of our greatest allies in controlling disease progression. These drugs started to be used worldwide by means of a mono-therapy with Zidovudine, in 1987, until we reached a point of having a combination of drugs, known as TARVTreatment with ARV medication has been the most important factor to control AIDS evolution, its chronicity and to guarantee the survival of those infected.Considering HIV's specificity, which affects the immune system, favoring the incidence of diseases which prey on low immunityStudies have proven that some ARV medications may have potential ototoxic effects and cause hearing loss, and they point to a possible association with hearing loss in the central hearing systemThe hearing loss may be described as any reduction in hearing and/or in the person's capacity to detect speech or ambient sounds, regardless of cause, type of degree. This may happen at different moments in life, during gestation or delivery, in childhood, adult life or old ageKnowledge about the hearing changes and early diagnosis concerning hearing loss helps in the prognosis, reduces the damages caused by disease development, reduces sensorial deprivation, improves inclusion in society and, it also contributes to improving quality of life. Thus, it is relevant to investigate how such hearing loss happened and its relationship with the HIV infection/AIDS in affected people.Therefore, we ask: how have the HIV-associated hearing changes been approached in the scientific literature? Which factors are associated with the hearing loss in patients with HIV/AIDS?It is worth stressing that there is a need for a hearing health policy encompassing the particularities associated with prevention, early diagnosis and treatment of hearing disorders in the Brazilian public healthcare system.In pursuing these answers, our study aimed at identifying the factors associated with hearing loss in people with HIV/AIDS in the literature.This is an Integrative Literature Review, which enables the abstracting of previous studies and establishing conclusions based on the critical review of the studies outlines, enabling one to summarize the evidence available about the theme being investigatedThe stages followed in order to operate this review involved: problem identification, creation of a research protocol, defining the information to be extracted from the papers selected, selection of papers, analysis, presentation and discussion of resultsISI Web of Knowledge (Institute for Scientific Information), PUBMED/MEDLINE , LILACS  and SCIELO (Scientific Electronic Library Online), using the following keywords listed in the Health Sciences Keywords: hearing loss, hearing disorders and deafness, individually associated to the HIV keyword.The papers were searched in four virtual databases, namely: The inclusion criteria were: original papers, open access papers, papers published in Portuguese, Spanish, English and French, without time constraints. The exclusion criteria were: repetitive papers, reviews, meeting proceedings, opinion papers, editorials, theses, dissertations, epidemiological bulletins, papers which do not directly approach the theme of this study.We found a total of 41 publications. After reading the title and summary, we took off repeated papers and those which did not meet the inclusion criteria. There were 22 papers left for complete text analysis, and of these we took nine papers off because they were not associated with the theme being studied. The sample was made up of 13 scientific papers, as per shown by In order to extract the data from the selected studies, we created a table with columns, bearing the following information: year of publication, authors, title, journal, country, type of study, study design, settings, target population, hearing loss-HIV association.The data was grouped according to the similarities and differences, making up a finding which was interpreted and supported by other pertaining papers. All the studies selected are listed on To analyze and discuss the occurrence of hearing disorders in HIV/AIDS patients and its associated factors in the selected papers, they were organized in topics. Since it was not possible to group the studies by methodological similarity, we chose to group the findings by adherence to theme, namely: “Virus actionIn 1995, authors had already found a high incidence of audiological changes in HIV-positive patientsIn a recent study involving adult patients treated and not treated by ARV therapy, the audiological manifestations  were more frequent than the vestibular changes in patients treated with ARV. There were thirty-nine treatment regimens utilized in the group of treated patients, and five of those treatment regimens were associated with otoneurological signs , and there were no significant correlation between the ototoxicity and the ARV treatmentHowever, adult HIV-positive individuals between 18 and 58 years of age, exposed to ARV treatment, had changes suggestive of peripheral auditory pathway involvement and also had high frequency hearing thresholds compromisedHIV/AIDS patients may have changes in their conventional audiological assessment, high-frequency audiometry, otoacoustic emissions, otoacoustic emission suppression and in auditory evoked potentials, suggesting involvement in the periphery auditory pathway as well as in the central auditory pathwayHigh-frequency audiometry assesses hearing in the range above 8,000 Hz - between 9,000 and 20,000 Hz - and does not have standardized results, such as in a conventional audiometry - between 250 and 8,000 Hz. Nevertheless, people with HIV/AIDS are the first to be affected with some ear disorders, showing an important element used in early diagnosisHigh-frequency audiometry is very useful to follow up patients exposed to intense noise: those who use ototoxic medication; as well as those exposed to degenerative etiological agents; nonetheless, some factors prevent this test to be part of the clinical routine, they are: we still lack a normality standard to test these frequencies, specificity in the equipment calibration patterns and the methodology employed, which may impact the results obtainedWe stress the study carried out with 23 children (ranging between 5 months and 16 years of age) diagnosed with HIV/AIDS, receiving highly active ARV therapy. Tonal audiometry was carried out in 12 children with more than 4 years of age, and 33% had hearing loss, two were of conduction type. The brainstem responses were measured in all 23 children, suggesting a conductive hearing loss in six and sensorineural in two. Concerning the brainstem auditory responses, they had changes at different levels of the auditory pathwaysThus, there are peripheral auditory pathway changes affecting people with HIV/AIDS associated with ototoxicity and to the high viral load or virus action. One may suppose that such fact is due to the morphological arrangement of the nervous fibers, since they have characteristics of the hair cells and/or of neurons, in responding in a specialized way to certain frequencies, which is called tonotopy. In tonotopy, the nervous fibers coming from the cochlear apex and forming the central region of the cochlear nerve are responsible for the transmission of low frequencies, while those fibers coming from the base and make up the periphery region of the nerve, are responsible for transmitting high frequencies, showing that, in current times it is not possible to be clear about the agent causing hearing loss in these peopleConcerning opportunistic diseases associated with hearing loss, we have the prevalence of central nervous system infections by cytomegalovirus (CMV), which must be considered as a cause for hearing loss in HIV-infected patientsCMV is a broadly distributed infectious agent in the general population, belonging to the herpes virus family, and it is a frequent cause of infections in human beings. This virus has periods of activation and latency, and once the person is infected, the virus remains indefinitely in the host's body, and it can be reactivated at any time, especially in the presence of immunosuppression agentsCMV incidence in the United States population is 1.6 infections for every 100 persons per year - and these are individuals with low family income. There are about 27,000 new CMV infections among serum-negative pregnant women every yearUpon studying 50 cases of patients infected with HIV aiming at characterizing the occurrence of ear diseases, we used a questionnaire, ear exam, audiological assessment and reviewing hospital charts. Among the otological manifestations we had: ear fullness (34%), tinnitus (26%), hearing loss (29%), otalgia (23%) and otorrhea (5%). Otitis media was also a frequent finding in these patients, and the sensorineural hearing loss was more severe in HIV patients, but it was not associated with the routine medication used to treat HIV/AIDS. The authors concluded that auditory disease affects up to 33% of HIV-infected patients, and sensorineural hearing loss is more severe in this specific groupWe know that otitis may cause temporary peripheral hearing loss, and it must be diagnosed as soon as possible in order to install proper medical treatment. According to the National Institute of Health of the USA, there are about 75% of adults with AIDS with some kind of hearing disorder arising from opportunistic infections, its treatment and ototoxic effectThere is a case report of a male patient infected with HIV who had hearing loss on the right side two months after secondary prophylaxis for cryptococcuc meningitis. The authors associate the unilateral hearing loss of adult patients with cryptococcus meningitis and the treatment of HIV-associated infectionsHIV-infected children were assessed in order to measure their audiological skills and vestibular disorders, considering that they were under highly active antiretroviral treatment. We included 23 patients with mean age of 4.5 years; audiometry was carried out in 12 children with more than 4 years of age. Four children had sensorineural hearing loss and two of them had conduction-type hearing loss. Brainstem responses were measured in all the patients, suggesting conductive-type hearing loss in six children and sensorineural hearing loss in two. The ones diagnosed with conductive hearing loss had a past of acute or chronic otitis media. These results suggest that children with HIV-1 must be audiologically assessed as soon as possible in order to reduce its impact on their psychosocial developmentTreponema pallidum happens in the tertiary syphilis. Initial symptoms may include uni or bilateral sensorineural hearing loss, usually of rapid progression and, often times sudden. Tinnitus, ear pressure and labyrinthine symptoms may also be present. The audiometric curve usually shows a drop in the low frequencies, suggesting endolymphatic hydropsOne epidemiological study carried out in order to study the frequency and clinical presentation of otosyphilis results in developed countries confirmed that it still is a complication of syphilis in these countries. Thus, we can state that this disease is a known cause of sensorineural hearing loss and has a higher prevalence among HIV patients. The otological involvement by Cerebromeningeal diseases may be associated with hearing loss. In a retrospective study carried out in Senegal, with the aim of establishing the prevalence of cerebromeningeal involvement in hospitalized patients and describe epidemiology, clinical signs and symptoms and disease etiology, there were 470 cases identified - 89 children and adults had HIV and numerous opportunistic disorders, such as meningeal syndrome, coma, seizure, focal neurological failure, cranial nerve dysfunction, cerebral malaria, purulent meningitis, cryptococcal meningitis, tuberculous meningitis, intracranial abscess, toxoplasmosis, cerebromeningeal hemorrhage. Of the 89 investigated, 22 survived and among these there were five with hearing loss. The study concluded that the labs must have greater technical capacity to diagnose the opportunistic infections presented by these patients, as well as involve the many experts to manage cerebromeningeal sequelaeThere are also two studies which did not reach conclusions as to the causes of the hearing loss. In the first one, with nine HIV-positive men, between 25 and 42 years of age, there were five with hearing lossKnowing that, every day, HIV/AIDS kills 6,000 people and infects some 8,200We conclude that there is still no consensus in the studies we analyzed, concerning a determining factor for hearing disorders and hearing loss in persons who live with HIV/AIDS, and these changes are related to multiple factors. Among them we stress a high viral load, direct virus action on the cochleovestibular system, the use of TARV and opportunistic infections - having cytomegalovirus, meningitis, otosyphilis and treatments, the ones most correlated to the hearing disorder and ototoxicity.Therefore, it was not possible to find a direct association between the ARV treatment and the hearing loss. Notwithstanding, we found hearing disorders in the population investigated, and their associations and causes are still not clear enough and concluding, which fosters the need for studies investigating the hearing loss in this population in a more in-depth manner.To those adult and children with HIV/AIDS, we recommend hearing monitoring in Hearing Reference Centers, minimizing the effects from hearing-loss-causing factors, thus reducing the psychosocial impact and providing a better quality of life to these people.Therefore, we require new studies, going more in-depth concerning the hearing disorders of patients with HIV/AIDS, which may become references for the initiatives of training and bringing awareness to healthcare professionals as to its problems and control.


To characterize the hearing loss after cancer treatment, according to the type of treatment, with identification of predictive factors.Two hundred patients who had cancer in childhood were prospectively evaluated. The mean age at diagnosis was 6 years, and at the audiometric assessment, 21 years. The treatment of the participants included chemotherapy without using platinum derivatives or head and neck radiotherapy in 51 patients; chemotherapy using cisplatin without radiotherapy in 64 patients; head and neck radiotherapy without cisplatin in 75 patients; and a combined treatment of head and neck radiotherapy and chemotherapy with cisplatin in ten patients. Patients underwent audiological assessment, including pure tone audiometry, speech audiometry, and immittancemetry.versus patients not treated with cisplatin . Children whose cancer diagnosis occurred after the age of 6 have shown an increased risk of hearing loss vs. children whose diagnosis occurred under 6 years of age (p = 0.02).The treatment involving chemotherapy with cisplatin caused 41.9% and 47.3% hearing loss in the right and left ear, respectively, with a 11.7-fold higher risk of hearing loss in the right ear and 17.6-fold higher in the left ear The auditory feature found after the cancer treatment was a symmetrical bilateral sensorineural hearing loss. Chemotherapy with cisplatin proved to be a risk factor, while head and neck radiotherapy was not critical for the occurrence of hearing loss. Over the last two decades, childhood cancer mortality has decreased significantly; however, it still represents the second leading cause of death in Brazil.The use of different treatment modalities  and the combination of these modalities contribute to improved results, both in controlling the disease and in improving survival rates.2.5Among the ototoxic drugs, cisplatin is an antineoplastic agent with proven anti-tumor activity, but which may have ototoxicity as a side effect; the dose related to risk has been described as being 400 mg/mHead and neck radiotherapy concomitantly employed with cisplatin (cis-diamminedichloroplatinum [CDDP]) increases the likelihood of severe hearing loss.9i.e., the effect represented by an injury to the peripheral organ of hearing, is characterized by an irreversible descending bilateral sensorineural hearing loss.13Ototoxicity, This study was conducted with the aim of characterizing the audiologic profile of patients who had cancer in childhood and were out of cancer treatment for at least eight years; to relate the hearing loss found with respect to the type of treatment and age; and to identify predictive factors for hearing loss.We prospectively evaluated children who had cancer treated between 2000 and 2004, and who had completed treatment for at least eight years, and who had been monitored in a group of pediatric studies on the late effects of cancer treatment. Patients with history of previous otologic disease or who had been submitted to a surgery involving the auditory system were excluded. The study was approved by the Research Ethics Committee of the institution under the protocol 549/03. Eligible patients or their legal guardians were consulted on the possibility of participating in the study, and were asked to sign the informed consent.Patients were interviewed at the Pediatric Outpatient Clinic in order to investigate the presence of hearing complaints and then were referred to a hearing evaluation in the institution's Audiology Service, regardless of the presence of hearing complaints. Otoscopy was conducted before the test and, if the patient had cerumen or any suspicion and/or obstruction that prevented the test, he/she was referred to the otorhinolaryngologist before evaluation.For hearing assessment, auditory quantification tests (pure tone audiometry and speech audiometry) and evaluation tests of the tympanic-ossicular system (immittancemetry) were performed. To this end, a Madsen Orbiter 922 audiometer and a Madsen Zodiac 901 immittancemeter were used.2. The clinical records of all patients who underwent head and neck radiotherapy were analyzed, taking into account the side on which radiotherapy was performed and whether the auditory system was included in the radiation field. The total dose and the estimated dose of radiation reaching the auditory system were calculated for each ear by a radiation oncologist, based on the planning form. The variable “radiation reaching the auditory system” was categorized as: no Rxt, Rxt ≤ 4000 cGy, and Rxt > 4000 cGy.14The dose of CDDP received by the participants was calculated and adjusted by the pediatric oncologist for a body surface area of 1 mPatients were studied according to the type of treatment performed, based on the use of chemotherapy with CDDP or head and neck radiation therapy.Hearing loss criteria were based on the Bureau International d’Audiophonologie – BIAP,To identify hearing loss predictors, a dichotomous variable (yes/no) was created, and hearing loss was diagnosed only in light of changes in the frequencies from 0.25 to 4 kHz. Hearing loss at 6 and 8 kHz was not included in the statistical analysis, due to the minor handicap that these losses cause in daily life.17The variable “age at diagnosis” was categorized as ≤6 years and >6 years, based on the median of the values found.α = 5% was established, i.e., the results were considered statistically significant at p < 0.05.Measures of central tendency and of dispersion for quantitative variables and absolute and relative frequencies for categorical variables were calculated. In order to verify the association among independent variables and hearing loss, the associative chi-squared test or Fisher's exact test (when at least one of the expected frequencies was <5) was used. To identify independent risk factors for occurrence of hearing loss, logistic regression  was used. For all statistical tests, an error vs. patients who received only Rxt (2996.8 ± 1427.8 cGy)  .Table 1DPatients who received CDDP or CDDP + Rxt were affected by a symmetrical bilateral sensorineural hearing loss at 4-, 6-, and 8-kHz frequencies. Hearing loss did not occur in cancer patients who did not receive a risk-to-hearing treatment or head and neck Rxt as a single treatment .Figure 1p = 0.025) and to the left (p = 0.020) side, a statistically significant difference was observed. It is not possible, however, to state that the Rxt factor has influenced the hearing loss because, the values for the percentage of hearing loss comparing the group that did not receive Rxt vs. those that received more than 4000 cGy are similar. Thus, it is believed that the statistical significance is due to the lower percentage of hearing loss in the group that received less than 4000 cGy, thereby differing from the other two groups, as can be seen in In the comparison among the three groups without Rxt, and with Rxt up to 4000 or above 4000 cGy, both to the right (p < 0.001 for both ears), compared to children who did not use CDDP. Diagnosis before the age of 6 years imparted a 2.7 times higher risk for hearing loss in the right ear (p = 0.02), compared with children aged ≤6 years , use of CDDP and age at the time of diagnosis were predictive factors for hearing loss. Children whose treatment included the use of CDDP showed an 11.7-fold increased risk for hearing loss in the right ear and a 17.6-fold increased risk in the left ear . In this series, the mean dose of CDDP in patients treated with chemotherapy was 650 mg/m2, and in individuals who received CDDP + Rxt, the dose was 670 mg/m2. Li et al.2 showing a higher risk for hearing loss. Studies using conventional frequencies (0.25–8 kHz) for hearing evaluation reveal a variation of 20–70% in the incidence of hearing loss.Patients treated with CDDP and CDDP + Rxt received high doses of CDDP – higher than the dose considered a risk for hearing loss  compared with children ≤6 years of age at diagnosis, but only for the right ear, with a tendency for the left ear . This finding may be due to this series of patients, who had their hearing loss concentrated in osteosarcoma diagnoses, more often established in adolescence ; but the same degree and type of hearing loss was found in patients who underwent chemotherapy with CDDP without Rxt. This finding demonstrates that, in this study, Rxt was not a risk factor for hearing loss, but CDDP was.In this study, the sample of patients undergoing a combined treatment (Rxt + CDDP) was small (Considering the impact of hearing loss, even if subclinical, on the linguistic, pedagogical, and cognitive development of children treated for cancer in childhood,The hearing loss identified in cancer patients, examined years after the completion of treatment was sensorineural, bilateral, and symmetrical, and predominantly affected the frequencies of 4, 6, and 8 kHz.Chemotherapy with CDDP was demonstrated to be a risk factor for acquisition of hearing loss, while head and neck radiation therapy was not decisive.The authors declare no conflicts of interest.


Scientific Reports 10.1038/s41598-022-11998-9, published online 16 May 2022Correction to: The original version of this Article contained repeated errors. Due to an error in the mean rearing temperature, all ATUs (Accumulated Temperature Units) were stated incorrectly.As a result, in the Results section, under the subheading ‘Eye morphology and migration’,“The youngest Atlantic halibut examined  and days from fertilization, 96) were bilaterally quasi-symmetrical with a compressed, oval shaped body and similar pigmentation on either side of the body  and days from fertilization, 96) were bilaterally quasi-symmetrical with a compressed, oval shaped body and similar pigmentation on either side of the body Fig. A,E.”And,“Migration of the eye proceeded throughout the study period Fig. A–D such now reads:“Migration of the eye proceeded throughout the study period Fig. A–D such Under the subheading ‘Photoreceptor types and mosaics’,“At 960 ATU, the retina had two main types of cone photoreceptors: single and double cones Fig. A,C,E,G.”now reads:“At 720 ATU, the retina had two main types of cone photoreceptors: single and double cones , the honeycomb mosaic was absent from the retina and other cone formations had emerged .”now reads:“By the time eye migration was complete (878 ATU), the honeycomb mosaic was absent from the retina and other cone formations had emerged .”“As per the trends in the dorsonasal retina, the ventral retina at 1170 ATU consisted of square mosaics with variable jitter in the lattice .”now reads:“As per the trends in the dorsonasal retina, the ventral retina at 878 ATU consisted of square mosaics with variable jitter in the lattice .”And,rh2 riboprobe along the inner segment myoids of cones  revealed profound retinal transformations  revealed profound retinal transformations  provided by tungsten-halogen tubes . Peak hatching occurred at 14 days post-fertilization . Hatched larvae had a prominent yolk sac which was fully absorbed around 60 days post-hatching (~ 740 ATUs).”“Atlantic halibut were reared from the fertilized egg in 100 L recirculating saltwater indoor tanks at a temperature of 10 ± 1.6 °C and maintained under artificial illumination  provided by tungsten-halogen tubes . Peak hatching occurred at 14 days post-fertilization . Hatched larvae had a prominent yolk sac which was fully absorbed around 50 days post-hatching (~ 320 ATUs).”“Atlantic halibut were reared from the fertilized egg in 100 L recirculating saltwater indoor tanks at a temperature of 7.5 ± 1.6 °C and maintained under artificial illumination  Cone mosaic from the centrodorsonasal retina at 1030 ATU (A); Nearest neighbour analysis of double cone centroids, illustrating the near neighbours of a single cone, including its nearest neighbour in red (B) and their frequency distribution (C) ; Voronoi tessellation of double cone domains (D) and their frequency distribution (E) . (F–J) Same presentation of data as per (A–E) but for the centroventronasal retina at 1170 ATU. (K–O) Same presentation of data as per (A–E) but for the centrodorsotemporal retina at 1170 ATU. (P–S) Same presentation of data as per (B–E) but for a random distribution of points matched for density with that in (K) and constrained by single cone soma size.”“Spatial analysis of single cone distributions from representative mosaics in the retina of Atlantic halibut undergoing metamorphosis. Single cones are marked with yellow dots. now reads:(A–E) Cone mosaic from the centrodorsonasal retina at 773 ATU (A); Nearest neighbour analysis of double cone centroids, illustrating the near neighbours of a single cone, including its nearest neighbour in red (B) and their frequency distribution (C) ; Voronoi tessellation of double cone domains (D) and their frequency distribution (E) . (F–J) Same presentation of data as per (A–E) but for the centroventronasal retina at 878 ATU. (K–O) Same presentation of data as per (A–E) but for the centrodorsotemporal retina at 878 ATU. (P–S) Same presentation of data as per (B–E) but for a random distribution of points matched for density with that in (K) and constrained by single cone soma size.”“Spatial analysis of single cone distributions from representative mosaics in the retina of Atlantic halibut undergoing metamorphosis. Single cones are marked with yellow dots. The original Article has been corrected.


Aim: This study examines speech recognition in sensorineural impaired hearing patients with and without cochlear dead regions at high frequencies. Methods: a clinical and experimental study was made of thirty patients with sensorineural hearing loss that were classified into two groups: group 1 - included 15 subjects with hearing loss and no dead regions; and group 2 - included 15 subjects with dead regions in the cochlea at high frequencies. Patients undertook word recognition score and speech reception threshold tests, with and without background noise. The speech tests were done with and without hearing aids in two situations: program 1 - broadband amplification (bandwidth 8000 Hz); and program 2 - amplification up to 2560 Hz, without high frequency gain. Results: For subjects with no dead regions in the cochlea (group 1) performance improved with program 1. For subjects with dead regions in the cochlea (group 2) performance improved with program 2. Conclusions: Subjects with no dead regions in the cochlea benefited from high-frequency information. Subjects with dead regions in the cochlea benefited from reduced gain at high frequencies.In patients with moderate to severe high-frequency hearing loss, cochlear damage may include “dead regions” where there are no functional inner hair cells and/or associated neurons.  In audiology, sloping auditory sensorineural deficiency is the most common type/configuration of hearing loss associated with difficulty in understanding speech in noisy environments. Although hearing aids may increase the available acoustic information, not always a satisfactory improvement in speech recognition is attained. Some patients enjoy little or not benefit from amplification, particularly in cases of sloping hearing loss where a severe grade occurs at high frequencies.An old concept is the relation between absence of benefit from hearing aids and functional reduction and complete loss of inner hair cells and/or neurons in certain regions of the cochlea. No clinical test, however, was done to identify the dead zones in the cochlea.In these regions information generated by vibration of the basilar membrane is not transmitted to the central nervous system. If sufficiently intense, however, a tone with a corresponding frequency to that of the dead zone may be detected through apical or basal transmission of the vibration pattern by other functional regions of the cochlea. The vibration amplitude of the basilar membrane at a distant site will be lower than the dead zone amplitude. Broad band noise may thus mask that tone much more effectively than expected, as noise needs only to eliminate the response coming from the remote site. If the threshold needed to detect a tone in the presence of broad band noise is higher than that of the normal threshold, this alteration may indicate lack of inner hair cells and/or adjacent neurons, with a typical frequency that corresponds to the tone frequency, in other words, a dead zone.A few studies have related the difficulty that hearing loss imposes on speech recognition to the need for hearing speech at high sound pressure levels; these levels, however, may reduce the analytical capability of the normal cochlea. As hearing loss increases, certain frequencies do not support or even reduce the available information at other frequencies. Less amplification should therefore be prescribed for frequencies at with auditory thresholds are increased.5Auditory resolution is the ability that inner ear structures and their associated neural systems have of generating patterns of neural activity that reflect spectral and time differences between sound information. The auditory nerve is organized, as is the basilar membrane: typically high frequency fibers originate from hair cells of the base of the cochlea, while low frequency fibers are at the apex of the cochlea. Fibers in the basal region respond synchronically to the presentation of a stimulus. Fibers in the apex are activated later (2 to 4ms later). Those that are activated simultaneously will contribute the most to the action potential of the whole nerve. Consequently this potential reflects mainly the response of high frequency fibers.Other authors4 have proposed a clinical test to identify dead zones of the cochlea; this test is the threshold equalizing noise (TEN), which compares auditory thresholds investigated with and without ipsilateral masking. The TEN noise level has a spectrum that was elaborated to obtain equally masked thresholds at all frequencies, (125 Hz to 15000 Hz), and is expressed as ERB , which refers to the bandwidth of the auditory filter. Normal listeners have a small variation (2 dB to 3 dB) between masked thresholds and noise levels. In patients with sensorineural hearing loss, dead zones of the cochlea are found when masked thresholds are at least 10 dB over absolute thresholds and 10 dB over the noise level. The results are confirmed by the measurement of psychophysical tuning curves. When the TEN test is positive, the peak of the tuning curve is displaced relative to the signal frequency. The authors emphasize that if dead zones are present, it may be useful to amplify a frequency range slightly above the dead zone; the reason being that amplification should aim at where hair cells can make use of it.The effectiveness of hearing for intelligibility is affected by the sound pressure level of the signal (distortion level), the degree of hearing loss, the frequencies at which it takes place, and the sound information processing capability.Based on these thoughts, the aim of this study was to verify the benefit of high frequency amplification for speech recognition in patients with sloping sensorineural hearing loss with or with no dead zones in the cochlea.The procedures used in this study were described to the Research Ethics Committee and approved under code number 0235/04. Participants signed a free informed consent form containing the necessary information before undergoing the tests. We assessed 30 subjects (14 women and 16 men) with sloping, bilateral, and symmetrical sensorineural hearing loss. Identification of dead zones of the cochlea was done using the TEN(NA) test,6 2004 version. Two groups were defined based on these results: group 1 - 15 subjects with no dead zones of the cochlea, and group 2 - 15 subjects with dead zones of the cochlea at high frequencies.The TEN test was applied using a two channel audiometer to control separately the stimulus (pure tone) and noise. The audiometer was coupled to a CD player. Auditory thresholds were measured in 2 dB intervals at 500, 750, 1000, 1500, 2000, 3000, and 4000 Hz in each ear separately, using TDH49 earphones, initially without masking, followed by an ipsilateral TEN noise at 70 dB NA/ERB. If this level was not sufficient to mask the absolute threshold, we would gradually increase the intensity up to 85 dB NA/ERB (maximum noise level tolerated by our patients). When the masked threshold was 10 dB or more over the absolute threshold and the noise level, we would give the result as suggesting dead zones for the tested frequency.We then proceeded with speech recognition tests in quite and with background noise, presenting the stimuli in an acoustic room, always using the same loudspeaker that the patient sat facing at a distance of 1 meter and azimuth 0º.We used a digital Siemens Signia HdO+ behind the-ear hearing aid with eight independently programmable channels for different frequency ranges to observe performance according to the amplified signal. Adaptation of hearing aids was binaural in all subjects. There were two programs: program 1 was amplification at a wide range of low frequencies (between 100 Hz and 8000 Hz), and program 2 had no gain at high frequencies (over 2560 Hz). The cut-off frequency for program 2 was approximately 2000 Hz, which was selected based on research that reports benefits from amplification of 1.7 times the dead zone limit frequency (roughly one octave higher).The speech material used for the Percentage Index of Speech Recognition (PISR) survey was a list of 25 phonetically balanced monosyllablesWe then applied the Abbreviated Profile of Hearing Aid Benefit (APHAB)We used non-parametric comparison tests to analyze our results statistically. Since the sample was relatively small, our significance level was set at 0.07 (7%).Masked thresholds were never over 10 dB above the noise level (from 70 to 85 dB NA/ERB) in all patients; the maximum difference between them was 6 dB NA. Generally only one noise level (70 dB NA/ERB) was enough for the TEN(NA) test. When the audiometric threshold was above 60 dB at a specific frequency, we used 10 dB over this threshold to define the minimum masking level.When masked auditory thresholds obtained by TEN(NA) testing of group 2 exceeded one or more of the absolute thresholds and the noise level by 10 dB, we considered the result as positive for dead zones of the cochlea at high frequencies. We used two or three noise levels due to the degree of hearing loss at these frequencies. Various patients had a 10 dB or more change compared to the absolute threshold when the noise level was below this threshold, where masking would theoretically be insufficient to change the threshold.In subjects with no dead zones of the cochlea we found that the PISR in quiet and in the presence of background noise improved significantly when using program 1 compared to the unaided and aided conditions of program 2.When dead zones of the cochlea were present we found that the PISR in quiet and in the presence of background noise improved significantly when using programs 1 and 2 in unaided conditions, and that program 2 showed significantly improved results compared to program 1.SRTQ was significantly improved in group 1 when using program 1 in unaided condition and to program 2. In the presence of background noise (SRTN) we can say that there was a trend towards a difference between both programs, as the statistical analysis revealed that the p-value was close to the acceptable limit. There were no significant differences between program 2 unaided and aided results.SRTQ and SRTN were significantly improved in group 2 when using program 2 and unaided conditions and when using program 1 and aided conditions.There was a significant difference between groups only in environments that favored communication; in this condition the most significant difficulty was found in group 2.The degree of high frequency hearing loss and the percentage index of speech recognition already suggested significant differences between groups 1 and 2 before we identified dead zones of the cochlea. We may say that a negative result for dead zones of the cochlea corresponded to the expected pattern in group 1, due to the audiometric sloping configuration but with no threshold differences over 50 dB obtained in successive octaves of tested frequencies, and the absence of thresholds over 90 dB NA at high frequencies .11 We foThe percentage index of speech recognition in group 1 showed a significant improvement when using hearing aids with program 1 (sound amplification in a wide frequency range from 100 Hz to 8000 Hz) compared to program 2 (restricted amplification from 100 Hz to 2560 Hz), both in quiet and in the presence of background noise . Thus, i14The percentage index of speech recognition in group 2 was significantly improved by using hearing aids with programs 1 and 2 compared to the unaided condition, both in quiet and in the presence of background noise , althoug15We may assume that patients with dead zones of the cochlea at high frequencies are used to a perception of filtered speech, as their hearing would operate as a low-pass filter. This could explain the benefit difference between each program for groups 1 and 2. Subjects with no dead zones, that effectively use high frequency information, are more affected by removal of these high frequencies.Comparing both programs, we observed that group 2 subjects reported increased clarity of sound and absence of hissing with program 2. We believe that the presence of dead zones at high frequencies reduce sound distortion by not amplifying those frequencies. Vibration generated in a dead zone is detected by another region; little useful information, therefore, is transmitted from the affected site. Furthermore, when the typical frequency of a region is different from that of the stimulus, detection of intense sound corresponding to these regions is altered.Group 1 subjects performed better in the sentence recognition threshold test in quiet and in the presence of background noise when using program 1 compared to program 2 . There wSentence recognition thresholds in quiet and in the presence of background noise  for grouThe APHAB questionnaire , used toOver half of group 2 subjects were going through hearing aid selection procedures just by taking part of this study, as previous adaptation attempts had been unsuccessful. In another paper the superior percentage index of speech recognition in patients with no dead zones of the cochlea was related to a higher acceptance rate of hearing aids (94.1%). In the presence of dead zones the acceptance rate was 21.4%. During the tests patients in group 2 reported improved sound quality with program 2, and that they wished to attempt adaptation once again.Observing the region in which auditory thresholds were preserved most in group 2, we could question whether amplification of sounds corresponding to this area could truly benefit these patients. We know that there is also loss of low frequency phase/synchronization components together with loss of high frequency information.1.In the absence of dead zones of the cochlea, improved speech recognition performance is reached with amplification over a wide frequency range.2.In the presence of dead zones of the cochlea at high frequencies, sound amplification of a restricted frequency range, avoiding gain at high frequencies, leads to the best speech recognition performance.Based on a critical analysis of our results, we reached the following conclusions:


Despite decades of research, there is much to be learned about the genetic landscape of sensorineural hearing loss. Novel genes for hearing loss remain to be identified while ‘secrets’ of the known genes need to be uncovered. These ‘secrets’ include regulatory mechanisms of gene activity and novel aspects of gene structure. To obtain a more complete picture of the genetics of hearing loss, the available experimental and bioinformatic tools need to be fully exploited. This is also true for data resources such as ENCODE. For the inner ear, however, such data resources and analytical tools need to be developed or extended. Collaborative studies provide opportunities to achieve this and to optimally use those tools and resources that are already available. This will accelerate the discoveries that are necessary for improving molecular genetic diagnostics and genetic counselling and for the development of therapeutic strategies. It is clear from the missing diagnoses in molecular genetic testing as well as from deafness loci for which the defective genes are still elusive that our knowledge of the genetic landscape of (nonsyndromic) sensorineural hearing loss is far from complete . As there is a positive correlation between the number of investigated genes and the diagnostic yield . An experimental strategy to obtain clues to potentially pathogenic effects of noncoding variation on either transcript splicing or gene regulation is to combine WGS and RNA sequencing data of affected subjects. However, this is only meaningful for those genes that are expressed at a sufficient level in accessibly cell types such as fibroblasts and peripheral blood cells. In the latter cell source, this is true for about 30% of the known genes for hearing loss (the Genotype-Tissue Expression (GTEx) project and K. Neveling, personal communication). Analysis pipelines for this strategy have been developed and successfully applied e.g. for mitochondriopathy patients  addressed and/or difficult to interpret. This is specifically the case for variants in the noncoding regions of genes and flanking regulatory regions including sequences that determine (tissue-specific) chromatin organisation. Tools to predict aberrant transcript splicing caused by (deep-)intronic variants are being used and predicted effects are often experimentally tested in mini-gene splice assays. Evaluation of variants in noncoding DNA for a potential deleterious effect on gene regulation is more challenging. Modelling of variants in conserved sequences in mouse can provide insights into a  pathogenic effect and mechanism . This is also illustrated for a small deletion in In the Deafness Variation Database, more than 79% of variants in genes implicated in hearing loss are classified as VUS, most of which are missense variants genetic modifiers is indicated part of which might have a gender-specific effect on disease penetrance.Last but not least, variants in known genes for hearing loss with an allele frequency higher than expected based on disease incidence or prevalence need to be considered for a pathogenic effect with incomplete penetrance. This might be specifically relevant for genetic types of hearing loss with a variable, mainly adult onset and/or variable severity. The occurrence of reduced penetrance of variants as well as the potential genetic complexity of a Mendelian disorder is exemplified in clinically and allelically highly heterogeneous All discussed aspects for the evaluation of variants in genes already known to be associated with hearing loss are also relevant for the identification of novel genes for hearing loss. Therefore, the knowledge and expertise gained by analysing the full range of genetic variation of these known genes will facilitate the identification of novel genes that are critical for hearing in humans. The analysis of known genes as well as the identification of novel genes for hearing loss can be importantly accelerated and supported by initiatives such as building population-specific databases for hearing loss and the development of hearing loss-specific multi-omics baseline data.Large WES and/or WGS datasets of genetically unexplained index cases as well as families will enable us to address non-Mendelian inheritance patterns of hearing loss, more specifically digenic or oligogenic inheritance. So far, there is no strong evidence of digenic inheritance of hearing loss. However, non-Mendelian inheritance might well significantly contribute to hearing loss in isolated cases for which the diagnostic yield is significantly lower than for familial cases. This is also true for adult-onset hearing loss even if this is familial. The genes involved might already be associated with hearing loss but could also be novel. The recently developed platform ORVAL (the Oligogenic Resource for Variant AnaLysis) aims to identify candidate pathogenic variant combinations in gene pairs  are already paving the way for global collaborative efforts.In conclusion, identification of novel genes for hearing loss is an ongoing process that is importantly facilitated by the developments in DNA and RNA sequencing technologies and in tools for bioinformatic and experimental analysis of genetic variation, as well as by the emerging resources of


Good hearing is essential to learning and rehabilitation of adolescent and young adults in juvenile correctional facilities. Hearing screening programme is not commonly in place for this incarcerated group.To evaluate hearing threshold among inmates of a juvenile correctional facility in Nigeria and compare pattern of hearing loss with a control group.A total of 135 inmates and equal number of age and sex matched control responded to interviewer-administered questionnaire followed by otoscopy and audiometry.Mean age of inmates was 19 years ±2.0, while that of control was 18yrs ± 2.5. . Four (3%) inmates had bleeding from the ear; otoscopy revealed traumatic tympanic membrane perforation in 2(1.5%) of them. Prevalence of hearing loss was 19.2% and for disabling hearing loss it was 1.4%. Conductive hearing loss was the most common 33(24.4%). Inmates had consistently worse mean hearing thresholds than controls across all frequencies tested in both ears .Hearing loss is prevalent among inmates of juvenile correctional facility. Rehabilitation programme should be balanced with detail attention to health needs of inmates; including pre-admission and periodic hearing screening. Youths in either conventional schools or rehabilitation homes need good hearing for formal learning12Although this experience is not peculiar to Nigeria, it is important to provide evidence-based data to guide government policy towards improving health care services in juvenile correctional facilities. This study evaluates hearing health of inmates with a focus on the prevalence, the type and degree of hearing loss among young offenders in one of the three juvenile correctional facilities in Nigeria.One hundred and thirty-five consenting inmates of the juvenile correctional facility were included in the study. Approval was obtained from the ethical review committee of University of Ilorin Teaching Hospital and The Nigerian Prison Service Authority. Security coverage was provided for all our weekly study visits with regulated access to only inmates signed-up for the week. All consented participants responded to interviewer-administered questionnaire. Information obtained included, biodata, socioeconomic background and otological symptoms. This was followed by otoscopy conducted by the author and pure tone audiometry in a quiet room (ambient noise level not exceeding 25 dB (A)) with constant checking of ambient noise level with CRL 412A Testo sound level meter. Pure tone audiometry was performed with a calibrated audiometer  according to recommended standard procedure by British Society of AudiologyEqual number of age and sex matched boarding students in a conventional Government Secondary School were used as control. Primary outcome measure was four-frequency Pure-Tone Average . Cut off for normal hearing threshold was ≤ 25dB. Level of impairment was classified according to the World Health Organization (WHO) gradingMean age of Inmates was 19yrs ±2.0, range 15 – 21 years; while that of Control was 18yrs ± 2.5, range 15 – 21years. There was no statistical difference in the ages of the two groups . Frequency of otological complaints was consistently higher among inmates, with a statistically significant difference recorded for hard of hearing (p<0.001), . AlthougCerumen (wax) was the most common content of the external auditory canal (EAC); seen in 18(13.3%) of inmates and 10(7.4%) of controls. Almost all participants in the control group, 134(99.3%) had normal TM integrity as against 124(92.0%) of the inmates with either a demonstrable TM perforation or neomembrane; with a significant statistical difference; , . Dull T.Conductive hearing loss was most common among inmates 33(24.4%) than control 13(9.4%) and it was the only type of hearing loss seen among controls. Sensorineural hearing loss (SNHL) and mixed hearing loss (MHL) constituted 3(2.2%) and 12(9.0%) respectively among inmates .Prevalence of hearing loss was 19.2% in the better and 34.8% in the worse ear of inmates. While the prevalence of disabling hearing, loss was 1.4% in the better and 6.6% in the worse ear of inmates. Hearing was normal in the better ears of controls, while the prevalence of hearing loss in the worse ear of control was 2.2% .Of the 26 (19.2%) inmates with suboptimal hearing in their better ears; 24(17.9%) had slight, 1(0.7%) each had moderate or profound impairment. . WhereasThere was a consistently higher mean hearing threshold in both ears of inmates than controls across all frequencies tested. .By reasons of incarceration, inmates in juvenile correctional facility are often deprived the benefits of contact with the larger society including access to medical careMore inmates were found with untreated impacted wax than controls . On the Otalgia was not a prominent symptom among inmates  and acutMean hearing threshold of inmate was worse than that of control across all frequencies . This isPrevalence of hearing loss was 19.2% in the better and 34.8% in the worse ear of inmates in this study. However, slight degree of hearing loss was the most common. This degree of hearing loss could go unnoticed by parent or teacher because it develops slowly over years that lip-reading could compensate for the deficit. Indig et al. in a baseline health survey of young people in custody in New South Wales quoted a prevalence of 18% for mild to moderate hearing loss in one ear or both ears with 32% having at least one ear having a degree of hearing lossHearing loss is prevalent among inmates of juvenile prison. It is often of mild degree, and progression may be preventable by early diagnosis and treatment. Rehabilitation programme in the juvenile correctional facilities should be balanced with detail attention to health needs of inmates; including baseline and periodic hearing screening and prompt access to hearing heath care.


Aim: to evaluate the effects of different signal-to-noise ratios on speech recognition obtained by the use of cochlear implant (CI); to compare the speech recognition in noise with different types of multichannel cochlear implants (CIs) and to evaluate the degree of difficulty for speech understanding in noise in daily life situations. Study design: cohort transversal. Material and Method: Forty adults with post-lingual hearing loss implanted with Nucleus 22, Nucleus 24, Combi 40, Combi 40+ and Clarion. We evaluated the recognition for CPA sentences in quiet and in S/N +15, +10 and +5 dB. We also applied the Social Hearing Handicap Index (SHHI) questionnaire for self-assessment in daily life. Results and Conclusion: All the implanted adults presented a significant reduction in the scores for sentences recognition as the S/N decreased. The medians´ curve for sentence recognition reached 50% in the signal-to-noise ratio of +10 dB. There was no statistically significant difference in sentences’ recognition scores and difficulty scores obtained with the SHHI, for all types of implants. The difficulties of implanted adults were rare in quiet and occasional in noisy situations according to SHHI questionnaire. Technological advance has allowed the improvement of strategies for speech signal codification in multichannel cochlear implants. However, the most frequent complaint of patients has been to recognize and understand the speech signal in noise Daily hearing conditions vary enormously concerning the ideal conditions and competitive environmental noise that is frequently found at home, workplace, school, leisure activities and other environments. Implanted patients mention the difficulties to understand in public places, such as in restaurants and parties, or even in a three-people group conversation, when everyone speaks at the same time The communication in noisy situations has been reported as extremely stressing and orofacial reading is essential in such conditions Explanations for the difficulty to understand speech in noise for patients with sensorineural hearing loss are: noise, that works as masking; loss of binaural integration, which increase the signal/noise ratio in 3dB or more; difficulties in temporal and frequency resolution; reduction of hearing dynamic field and masking effect of low frequency energy about the thresholds of medium and high frequencies, that is, low frequency speech sounds (vowels) are louder and interfere in the perception of high frequency segments (consonants)To subjects that use hearing aids, it transmits a processed and amplified acoustic signal to the damaged ear. The integrity of the system after the cochlea is a determining factor in the skills of the users to separate the desired signal from other signals and noise. To subjects that use the cochlear implant, the authors describe that a speech processor codifies the acoustically processed signal to an electrode stimulation pattern. In this case, integrity of the speech processor and its algorithm has been a determining factor for the users’ skills to separate the target signal from other signals The negative influence of noise in speech perception of cochlear implant users may be justified by the following factors: the speech processor codifies the signal to a stimulation pattern of electrodes in quiet that is different from it in noise The studies have been carried out to develop new strategies for speech codification The report of users of cochlear implant on their performance shows the need to assess speech understanding in competitive noise conditions, that is, in conditions that are close to the reality, in which we have exhibition of different variations in the signal/noise ratio along the day in each environment.The assessment allows the verification of reduction of cochlear implant user performance in quiet to the competitive noise condition and helps the clinician to indicate and choose technological and therapeutic resources that favor speech understanding of users of cochlear implant in noisy environments.In addition to analyzing the effectiveness of cochlear implant through the assessment of speech perception in competitive noise, it is necessary to check the level of difficulty of cochlear implant users, in noisy situations of daily life, through self-assessment questionnaires.The objective of the present study was to assess the effects of different signal/noise ratio, speech recognition with cochlear implant, to compare the recognition of speech in noise, with different types of multichannel cochlear implants; to assess the influence of duration of deafness, use of cochlear implant and progression of deafness in speech recognition with cochlear implant and to assess the level of difficulty that cochlear implant users have in situations with daily competitive noise.The present study was carried out at Centro de Pesquisas Audiológicas (CPA), Hospital de Reabilitação de Anomalias Craniofaciais (HRAC), University of Sao Paulo (USP) - Bauru, and approved by the Research Ethics Committee.We selected 40 adult patients with post-lingual hearing loss, experience of over 6 months with multichannel cochlear implant use and speech recognition in open-set condition. The subjects were divided into 5 groups .Table 1COut of 40 studied adults, 19 were male and 21 were female, mean age of 43 years and 6 months at the time of the assessment , mean time of deafness of 5 years and 10 months  and mean duration of cochlear implant use of 2 years .In We carried out CPA sentence recognition test To make self-assessment of the performance of the cochlear implant in daily situations, we used the questionnaire Social Hearing Handicap Index (SHHI)In the statistical assessment, to compare the groups (different types of cochlear implants) concerning index of CPA sentence recognition in the same hearing situation and scores of difficulty obtained in the same component of the questionnaire SHHI, we used non-parametric Kruskal-Wallis test for independent groups. In the comparison of hearing situations in the same group, we employed the non-parametric test of Friedman for repetitive measurements, and for comparison of scores of difficulty of hearing loss and selectivity components of SHHI questionnaire in the same group, we used non-parametric Wilcoxon test. In all tests, we considered significant result as p<0.05 (5%).To assess the influence of the characteristics of the subjects  in CPA sentence recognition index in each situation of hearing, we adjusted the logistic model by using Genmod procedure of statistical software SAS for Windows, version 6.12.In We can see in In the group comparison, Kruskal-Wallis test showed that there was no statistically significant difference (p>0.05) between medians of sentence recognition index obtained for different cochlear implants in all studied hearing situations.In the comparison between hearing situations in each group, Friedman test revealed that the median of sentence recognition index obtained in quiet was significantly better than that obtained in situations of noise; the median obtained for S/N +15 dB was statistically significant difference better than for the ratios S/N +10 and +5 dB and the median obtained in these S/N ratios was significantly better than in S/N +5 dB.In In Considering that there was no difference between types of implants and index of sentence recognition, the influence of the characteristics of subjects  in speech perception was independently analyzed from type of cochlear implant.The statistical analysis revealed that time of deafness had significantly influenced (p<0.05) the rates of sentence recognition indexes in hearing situations of S/N +5 dB, +10 dB and +15 dB, that is, in those situations, the longer the duration of deafness, the lower the CPA sentence recognition index.The duration of use of cochlear implant significantly influenced (p<0.05) the situations of S/N ratio of +15 dB and in quiet: the longer the use, the higher the rates of CPA sentence recognition in these situations.The progression of deafness significantly influenced (p<0.05) CPA sentence recognition index in all studied hearing situations. In silence, the subjects with progressive deafness have 1.3719 times more likelihood of getting it correct than sudden deafness cases. In S/N +15, +10 and +5, subjects had with progressive loss had respectively, 1.3356, 2.1876 and 1.2907 more likelihood of getting a correct answer than the subjects with sudden deafness.In We can see that in In the comparison between groups, Kruskal-Wallis test did not show any statistically significant difference between the median of scores of difficulty with SHHI questionnaire in the total and in components between the different types of cochlear implant.In the comparison of scores of difficulty in the components of SHHI questionnaire in each group, Wilcoxon test indicated that the median scores of difficulty in the components of selectivity were significantly higher (p<0.05) than the component of hearing loss in all types of cochlear implants.The results obtained in the CPA sentence recognition assessment in the situations of hearing in S/N ratio +5 dB, +10 dB and +15 dB and in quiet, revealed that there was no significant difference between cochlear implant and strategies of speech codification used in this study .Despite the fact that CIS strategy has high stimulation speed (813 pps per channel in implant Clarion and 1515 pps in implants Combi 40 and Combi 40+), the results of the assessment of speech perception with this strategy were equivalent to the results with strategy SPEAK (250 Hz in implant Nucleus 22) and strategy ACE (Nucleus 24). This last strategy brings together strategy SPEAK and skills of stimulation for strategy CIS, in high speed, emphasizing at the same time, spectral and temporal clues. These results agreed with the findings of previous studies that did not find a difference between sentence recognition in quiet and in noise with the implant Nucleus 22 (strategy SPEAK) and implant Ineraid (strategy CIS)Even though there was no statistically significant difference between cochlear implants, in the results of the speech perception assessment, medians of CPA sentence recognition indexes in S/N ratios of +5 dB, +10 dB and +15 dB, they were higher with cochlear implant Nucleus 24 (strategy ACE) . These rIn The introduction of competitive noise in the test room caused significant reduction of performance to all assessed hearing situations, even in situations considered to be more favorable, with S/N +15 dB, in which speech signal level was 15 dB above the noise level, as detected in previous studies We could detect a reduction in sentence recognition indexes as a result of increase in levels of noise , with thSubjects’ characteristics such as duration of deafness, duration of CI use and deafness progression, influenced significantly the CPA sentence recognition index. The longer the deafness, the lower the rates of CPA sentence recognition in ratios S/N +5 dB, +10 dB and +15 dB, evidencing that deafness time is one of the most important indicators of post-surgical performance The longer the duration of implant use, the higher the CPA sentence recognition index in quite and in S/N + 15 dB, because there is significant improvement in recognition and speech understanding as time goes by, especially during the first year The progression of deafness has significantly influenced CPA sentence recognition indexes, given that in all studied situations subjects with progressive hearing had higher chances of having a correct answer than sudden deafness subjects. It may be justified by the fact that profound sudden deafness causes a drastic change to auditory skills, whereas in progressive hearing loss, auditory skills gradually change, allowing an adaptation, plus a period of benefit with the hearing aid Medians of scores of difficulty obtained in SHHI questionnaire in total and in components of hearing loss and selectivity revealed that there was no statistically significant difference between the used cochlear implants , confirmMedian of scores of difficulty in selectivity component were significantly higher than in the component of hearing loss, in all types of cochlear implants and difficulties were rare in quiet and occasional in situations that involved environment noise . FindingThe main difficulty of cochlear implant users in daily life is to understand speech in noise, regardless of the type of multichannel cochlear implant used It is important to emphasize that high rates of speech recognition and daily performance of cochlear implant users in situations with competitive noise in the study reflected the strict criteria for indication of cochlear implant during pre-surgical assessment Clinical and subjective assessment of speech perception in competitive noise situations contributes to defining a real profile of the performance of cochlear implant in daily life and, based on this database, the speech therapist can guide patients to use noise suppression systems, available in speech processors, and to indicate technological resources, such as modulated frequency statistics and use of hearing aid in the non-implanted ear. Another possibility is bilateral cochlear implant, which has already proven to improve speech understanding in noise Cochlear implants have presented significant advances for the past decades relative to speech codification strategies as reported here, but current devices still do not restore normal perception of speech, especially in adverse situations such as presence of noise or many speakers at the same time. New perspectives concerning speech perception are expected based on studies about the combined use of electrical and acoustic stimulation, bilateral cochlear implant and strategies of speech codification that are more similar to the speech processing in the normal cochlea.To clinically work by directing to the optimization of the speech perception that each cochlear implant user has, it is essential to be familiarized with the scientific and technological knowledge available for the diagnosis of hearing loss, indication and programming of cochlear implant, hearing aids and modulated frequency systems, training of auditory skills, and the strategies of communication associated with knowledge of intervening variables in speech perception.•Users of cochlear implant presented significant reduction of CPA sentence recognition indexes as a result of reduction in S/N ratio;•The best CPA sentence recognition index with cochlear implant, in the presence of competitive noise, was S/N of +15 dB, and CPA sentence recognition reached 50% in S/N ratio of +10 dB;•There were no statistically significant differences in CPA sentence recognition with cochlear implants Nucleus 22 (strategy SPEAK), Nucleus 24 (strategy ACE), Combi 40, Combi 40+ and Clarion (strategy CIS), in quiet and in ratios of N/R +5 dB, +10 dB and +15 dB;•The longer the duration of deafness, the smaller the CPA sentence recognition index in ratios S/N + 5 dB, +10 dB and +15 dB;•The longer the use, the higher the CPA sentence recognition indexes in quiet and in S/N ratio + 15 dB;•The users of cochlear implant with progressive deafness had more chances of correct response than those with sudden deafness, in all situations of assessment;•The users of cochlear implant presented scores of difficulty that were higher than the component of selectivity (noise) than in the component of hearing loss (quiet) in SHHI questionnaire;•The difficulties in cochlear implant users were rare in situations of quiet and occasional in situations with daily competitive noise;•There were no statistically significant differences in scores of SHHI questionnaire with cochlear implants Nucleus 22 (strategy SPEAK), Nucleus 24 (strategy ACE), Combi 40, Combi 40+ and Clarion (strategy CIS).


Since hearing loss and cognitive decline often co-occur among older adults, a cognitive screening test suitable for hearing-impaired people is of high clinical relevance. We report the first evaluation of a German language version of the Montreal Cognitive Assessment—Hearing Impaired version (MoCA-HI).The aim of the present study was to compare cognitively healthy participants with and without hearing loss, to examine the impact of age, sex, educational level and degree of hearing impairment on the German MoCA-HI performance, and to develop normative data.The German MoCA-HI was tested in 94 participants with normal or mild hearing impairment (group 1: 4PTA ≤ 40 dB on the better hearing ear) and 81 participants with moderate to profound hearing loss (group 2: 4PTA > 40 dB on the better hearing ear). Additionally, all participants performed the standard MoCA (version 8.2).p = 0.05). In contrast, group 1 performed significantly better than group 2 on the standard MoCA (p < 0.001). There was no difference between the MoCA and the MoCA-HI performance in group 1 (p = 0.12), whereas individuals of group 2 performed significantly better on the MoCA-HI than on the standard MoCA (p < 0.001). Test-retest reliability of the MoCA-HI was high (p < 0.001). Higher age (p < 0.001), male sex (p = 0.009) and lower education (p < 0.001) were associated with a lower overall MoCA-HI score. Based on the demographic data normative data were developed by a regression-based approach.No significant group difference between group 1 and 2 was found in the MoCA-HI total score (The MoCA-HI is a cognitive screening test which is suitable for people with hearing impairment. Age-related hearing loss and dementia are among the most common chronic diseases in old age. Currently, approximately 430 million people live with disabling hearing loss , while 5There is a growing interest in neurocognitive testing in settings outside psychologic or psychiatric ones , 10, parNumerous screening tests are available to identify cases of cognitive impairment . HoweverSeveral attempts have been made to adapt cognitive screening tests for people with hearing loss , 22. Adawww.mocatest.org). This MoCA-HI is freely available from the MoCA website (www.mocatest.org) to appropriately trained persons after a short fee-based online training offered by the same website. In Dawes et al.'s version, the spoken items of the standard MoCA (version 8.1) were presented visually  or substituted with alternative visual tasks . These adaptations were designed to index the same cognitive domain as the standard items and to be of a similar level of difficulty.Dawes et al. developed a visual version of the Montreal Cognitive Assessment (MoCA)  for peopDawes et al.'s validation was carried out using an English version of the MoCA-HI . PoolingThe aim of the present study was (1) to compare performance of the German version of the MoCA-HI and the original MoCA in cognitively healthy participants with and without hearing loss, (2) to examine the impact of age, education, sex and level of hearing loss on performance and (3) to derive corresponding performance norms of the German MoCA-HI.Inclusion criteria were as follows: (1) age of 60 years or older, (2) native or excellent German speaker, (3) normal or corrected near visual acuity of ≤ 0.3 logMAR, (4) normal performance in the GPCOG   a score. Particit-test at an alpha level of 0.05 with a power (1-beta) of 0.90, two groups of at least 70 participants were required. In total, 175 participants were included . Descriptive statistics including mean (M) and standard deviation (SD) were used to describe sociodemographic, audiological and cognitive data. T-tests were performed to compare group 1 to group 2 with regard to age, education, MoCA-HI total-score and the individual cognitive subdomains and reported by mean difference (MD) and p-value. To compare the results of the two groups in the adapted tasks of the MoCA-HI and the corresponding tasks of the standard MoCA, the Mann-Whitney-U-test was used. To examine performance differences between the MoCA and the MoCA-HI within each group, the Wilcoxon signed rank test was applied. In order to analyze the impact of hearing impairment on the MoCA-HI-total-score and the cognitive subdomains, multiple regression analysis was carried out including the 4PTA as a continuous measure of hearing ability taking into account age, education and sex. Test-retest-reliability was determined by a Pearson-correlation of the MoCA-HI total-scores at both measurement time points.To achieve a medium effect size for a group comparison using a th response from a model that was estimated without this observation  was developed using the z-score formula z = (score–expected score)/residual standard deviation. Cutoff scores were developed based on the z-score-formula for the 10th percentile (z = −1.28) for men and women for each age (60–97 years) and all years of education (7–18). Statistics were calculated by the statistical program SPSS (Version 28) and normative data were calculated by Rstudio (2021.09.1). Confidence interval was set at 95% and statistical significance was defined as a www.mocatest.org). The study met the guidelines of the Declaration of Helsinki, and all participants gave their written consent. All examiners underwent training as required by www.mocatest.org.The study was registered on the MoCA homepage , 60 subjects between 72 and 83 years  and 15 subjects were aged 84 years or older . According to the WHO definition, 52 patients did not suffer from hearing loss  dB), 42 participants were classified as WHO 1 with a mean 4PTA of 30.63 (SD 3.43) dB, 41 were suffering from a hearing loss of 45.70 (SD 5.66) dB in mean (WHO 2). 40 subjects with a mean 4PTA of 88.75 (SD 22.05) belonged to WHO 3 and 4.One hundred seventy-five participants aged 60 to 97 years  were included in the present study. 100 subjects were aged between 60 and 71 years ] and had a lower educational level than group 1 (p < 0.001) (Study samples were divided into 2 groups. Group 1 (94 subjects) was normal hearing or only slightly hearing-impaired  with a mean 4PTA of 22.23 dB (SD 8.78) and group 2 (81 subjects) was moderate or profound hearing-impaired  with a mean 4PTA of 66.96 dB (SD 26.87). Audiometric data are shown in < 0.001) .p = 0.05). However, Welch test showed that group 1 performed significantly better than group 2 in the cognitive subdomain of visuospatial and executive functions . None of the other cognitive subdomains, such as naming , attention , language , abstraction , and recall , showed a significant difference between the two groups in the independent sample t-test. For the cognitive subdomain of orientation, Welch's test also showed no significant group difference .Mean scores of the MoCA-HI and the subdomains in group 1 and group 2 are shown in p < 0.001), male sex , and lower education  were associated with a lower MoCA-HI total score and together explained 41.7% (adjusted R2 = 0.41) of the total variance . Mean total scores in the MoCA-HI in the 4 different WHO groups are shown in p = 0.72). Further no significant effect of the level of hearing impairment on the different subscores was found .A multiple linear regression analysis revealed that older age . However, the MoCA-HI total score was higher in the retest than at baseline , due to a statistically significant improvement in the cognitive subdomain “recall” in the retest (p < 0.001). All other subtests remained stable after re-testing.A retest was performed in 115 participants. The mean retest interval was 60.38 (SD 18.08) days after the first administration with a minimum of 28 and a maximum of 112 days. Test-retest reliability was high with a Pearson correlation of 0.84 (p = 0.58). In contrast, individuals of group 1 (mean rank = 106.27) performed significantly better than subjects of group 2 (mean rank = 66.80) on the sum of the corresponding items of the standard MoCA (p < 0.001). Further, group 1 did not differ in the MoCA and MoCA-HI performance (p = 0.12), whereas group 2 performed significantly better on the MoCA-HI than on the standard MoCA (p < 0.001).There was no difference between group 1 (mean rank = 89.70) and group 2 (mean rank = 86.03) in the adapted items of the MoCA-HI (www.mocatest.org), showing that 35.5% of women and 39.4% of men scored below the original cutoff . This effect is strongest for education (t = 7.52), followed by sex (t = −2.65), age (t = −1.86), and the interaction of age and sex (t = −1.41), as indicated by the t-values. Based on the present data, the z-Score could be determined as follows: z = (Score–(22.86 + (−0.07 * age) + (0.53 * education) + (−1.11 * sex) + (−0.07 * (age−71.52) * sex)))/2.72. Sex was coded as 0 = female and 1 = male, age and education are inserted in years. The resulting cutoff scores for the 10th percentile are shown in In a first step, MoCA-HI scores were adjusted for education as suggested by Dawes  from the assessment and established new cutoff scores proportionally adjusted for the deleted items . Howevern = 103) vs. hearing loss (n = 49)  . Lerch a(n = 49) . A compa(n = 49) . Utoompr(n = 49) , 30 cognThe visual version of the MoCA reported in the current study has several advantages over previous versions of the MoCA adapted for people with hearing loss. First, rather than deleting spoken items, it replaces the standard spoken items by other items that tap into the same cognitive domain and are of a similar level of difficulty. Secondly, it was validated in a large cohort in a multi-centre study , 26. ThiGroup 2 (hearing loss of ≥ 41 dB) performed worse than group 1, which had no hearing loss or only a mild hearing loss, on the standard MoCA, but not on the three adapted tasks of the MoCA-HI. In line with that, there was no significant difference in the performance in the MoCA and MoCA-HI of group 1, while group 2 performed significantly worse in the standard MoCA than in the MoCA-HI. Thus, at least people with a severe hearing impairment may benefit from a visual version of the MoCA and the MoCA-HI may prevent false-positive diagnosis of dementia especially in case of a severe or profound hearing loss . HoweverThe MoCA-HI total score was best predicted by a regression model including age, education, sex, and the interaction of age and sex; age had a stronger effect on the total score in men than in women. This is in line with previous studies on the original MoCA, where regression models including age, education and sex had the best predictive power , 40, 41.To use the MoCA-HI in clinical practice, a re-test is necessary. In the present study participants performed slightly better in the re-test with less than 1 point more. Although this improvement was statistically significant, it did not make a difference to the clinical classification on the MoCA-HI. Practice effects cannot fully be ruled out in re-testing , althougOne limitation of our study is that we relied on the GPCOG to establish normal cognition criteria. The GPCOG is somewhat like the standard MoCA in including spoken items, so hearing status may have impacted categorization as normal cognition based on GPCOG performance. Some people with hearing loss might have been incorrectly excluded. However, we do not consider this to be a serious issue, since it was our aim to include only cognitive healthy individuals in this analysis.Previous research indicated that performance criteria to identify cognitive impairment developed in English may not be applicable to translations of the MoCA in other languages . Cut-poiThe German translation of the MoCA-HI is suitable in subjects with and without hearing loss and has high retest reliability. Performance criterion for identification of cognitive impairment should be developed, considering the impact of age, sex and educational level. A language-specific validation is required due to linguistic and cultural differences.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.The present study was approved by the Ethic Institution of the Ruhr-University Bochum (No. 20-7076). The patients/participants provided their written informed consent to participate in this study.CV, LG, and PD designed the study. HF collected the data and did the statistical analysis with critical feedback from FL and MT. CV and HF wrote the manuscript with contributions from PD and critical feedback from LG, ZN, and RW. All authors contributed to the article and approved the submitted version.


This study evaluated the determinants of disability and quality of life in elderly people who participated at the multi-centred RubiN project  in Germany.Baseline data of the subjects aged 70 years and older of the RubiN project were used and only subjects with complete data sets were considered for the ensuing analysis ).Disability was examined using the concepts of ADL (activities of daily living) and IADL . Subjects exhibiting one or more deficiencies in ADL respectively IADL were considered as ADL respectively IADL disabled. Quality of life was assessed using the WHOQOL-BREF and the WHOQOL-OLD. Applying multivariate analysis, sociodemographic factors, psychosocial characteristics as well as the functional, nutritional and cognitive status were explored as potential determinants of disability and quality of life in the elderly.One thousand three hundred seventy-five subjects from the RubiN project exhibited data completeness regarding baseline data. ADL and IADL disability were both associated with the respective other construct of disability, sex, a reduced cognitive and functional status as well as domains of the WHOQOL-BREF. Furthermore, ADL disability was related to social participation, while IADL disability was linked to age, education and social support. Sex, ADL and IADL disability, income, social support and social participation as well as the functional status were predictors of the domain ‘Physical Health’ (WHOQOL-BREF). The facet ‘Social Participation’ (WHOQOL-OLD) was affected by both ADL and IADL disability, income, social participation, the nutritional and also the functional status.Several potential determinants of disability and quality of life were identified and confirmed in this study. Attention should be drawn to prevention schemes as many of these determinants appear to be at least partly modifiable.The online version contains supplementary material available at 10.1186/s12877-022-03621-3. As life expectancy of the global population is increasing, the term Aging is of growing interest in scientific literature , 2. In GDue to the significance of disability and QOL research intensively investigated possible determinants regarding functional status and QOL, in order to promote prevention and rehabilitation schemes –58. DepeIn this paper we present novel insight about the determinants of disability and QOL in a real German ambulant healthcare setting. There are several reasons for this approach. First, to our best knowledge those determinants are usually assessed in mere cross-sectional or cohort studies, but have not been investigated in an actual care setting yet –98. SecoData used in this study were collected in the context of the RubiN project. This is a prospective controlled intervention study in an ambulant clinical setting  which was set up to assess the impact of case management for elderly patients . The EthThe outcome variables in this study were disability and QOL.Disability was examined applying the Barthel index (BI) for ADL according to the Hamburg Classification Manual  and the QOL was assessed using the WHOQOL-BREF and the WHOQOL-OLD. The WHOQOL-BREF is a valid and reliable short version of the WHOQOL-100 –115. It The independent variables were chosen based on a preceding literature review and availability in the RubiN project. Regarding sociodemographic determinants age and sex were recorded. Age was used as a continuous variable . The livThe statistical analysis was carried out using JMP Pro 15 .2-tests based on logistic regression for continuous variables and contingency analysis for categorial variables. Independent variables of QOL were assessed using bivariate correlation for continuous variables and two-sided pooled t-tests respectively ANOVAs for categorial variables. Third, multiple regression models were created in order to identify and confirm independent variables significantly influencing ADL, IADL and QOL (WHOQOL-BREF and -OLD) and to examine the relationship between the outcome variables. The dichotomous dependent variables ADL and IADL were examined using nominal logistic regression, whereas the continuous dependent variables of QOL  were assessed using mean least squares regression. Regression models were tested towards their Lack of Fit and significance, the explained variance R2 was computed. Independent variables within each regression model were analysed using Effect Likelihood Ratio Tests, and significant Parameter Estimates were identified. Odds ratios were calculated for the categorial dependent variables ADL and IADL. For all statistical tests a p-value < 0.05 was considered statistically significant.First, we examined the study participants’ descriptive characteristics regarding the frequencies, means and standard deviations of the dependent and independent variables. Descriptive analysis was performed for the complete study sample as well as for those subjects included and excluded for the subsequent analysis. Second, bivariate analysis was performed. Independent variables of ADL and IADL were tested using ChiN = 4116), thereof N = 1375 persons could be included in the analysis. N = 2741 persons had to be excluded due to missing data. Executed bivariate analysis indicates that there are several significant differences regarding the independent variables between the two latter groups. For example, excluded subjects were significantly older, exhibited larger proportions of ADL respectively IADL disabled persons, rated their quality of life significantly lower in most of the domains of the WHOQOL-BREF and the WHOQOL-OLD and performed worse on the Timed Up-and-Go test.Table N = 877, men: 498). 24.8% of the included study participants were ADL disabled, while 45.8% were IADL disabled. Sex did not exert a significant influence on ADL disability , while significantly more men than women were IADL disabled .The 1375 study participants included in this analysis had a mean age of 80.7 ± 5.6 years with a female percentage share of 64% , having social support and a reduced cognitive and functional status. Attainment of an intermediate education level served as a protective factor regarding IADL disability, while a higher education level did not exert a significant influence. Analogous to ADL disability, the most important determinant of IADL disability was a given ADL disability  and IADL disability  , 131. ThMultivariate analysis revealed that both ADL and IADL disability were significantly associated with the respective other type of disability: IADL disability was the strongest predictor for ADL disability (OR: 7.77) and vice versa (OR: 7.76). A sizeable intercorrelation between both concepts is in line with previous findings and emphasizes that independence in ADL/IADL serves as a prerequisite for the respective other one , 31. NevNonetheless, several results were in accordance with the idea of only IADL building mainly on psychosocial and cognitive resources. First, an intermediate education level exerted a significant protective effect only on IADL, but not on ADL . InteresThe domain ‘Physical Health’ overlapped mostly with ADL which was displayed in terms of its dependence on ADL disability and aligned associations with IADL disability, ‘Social Support’ and ‘Social Participation’, a reduced functional and nutritional status. However, in contrast to ADL, an appropriate income was associated with higher scores on ‘Physical Health’ which is in line with previous findings , 118. Fip value 0.5389). This would have been a plausible explanation considering the aforementioned outcome concerning age and IADL.Multivariate analysis revealed that both age and sex (partly) exert a significant influence on disability and physical QOL. First, an increased age was only significantly related to IADL disability, neither to ADL disability nor to the domain ‘Physical Health’. Especially the latter contradicts earlier findings , 80, 118If IADL disability generally precedes ADL disability, and men are more likely to be IADL disabled, men will be expected to be more likely ADL disabled as well. However, this was not the case in this sample. In fact, women had higher odds of being ADL disabled (OR: 1.72), and scored lower on the domain ‘Physical Health’. Previous research has suggested that men face higher death rates among the studied age groups, while women experience higher incidence rates of ADL disability , 91, 149This study has several limitations. One major limitation is its cross-sectional nature which does not allow assumptions about causality and generalisability to older populations  and whicThe study has various strengths as well. First, it is a multi-centre study in an actual care setting comprising eight certified practice networks including their GP practices. Despite the study’s cross-sectional entity, this fact enhances its representativity for older adults . Second,2 values of our 14 multiple regression models ranged between 0.05 and 0.39, future research should focus on significant independent variables not included in this study. These comprise health care use and chronic medical conditions such as vascular disease, impairment of vision and hearing, pain, history of falls, polypharmacy and depression as well as their level of severity [As the Rseverity , 60. Addseverity , 58.This study detected and approved several potential determinants of disability and quality of life in the elderly. Attention should be drawn to prevention schemes as many of these determinants like the nutritional and functional status, social participation and education appear to be at least partly modifiable.Additional file 1. Bivariate Analysis of ADL.Additional file 2. Bivariate Analysis of IADL.Additional file 3. Multivariate Analysis – Effect Likelihood Ratio Tests .Additional file 4. Bivariate Analysis of WHOQOL-BREF, WHOQOL-OLD.Additional file 5. One-way Analysis of Age by Sex.