Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age

Abstract

Objective: To determine the influence of the presence of auditory neuropathy spectrum disorder (ANSD) on speech, language, and psycho-social development of children at three years of age. Design: A population-based, longitudinal study was performed on outcomes of children with hearing impairment (LOCHI) in Australia. The demographic characteristics of the children were described, and their developmental outcomes were evaluated at three years of age. Performance of children with ANSD was compared with that of children without ANSD in the LOCHI study. Study sample: There were 47 children with ANSD in the study sample. Results: Sixty-four percent of children with ANSD have hearing sensitivity loss ranging from mild to severe degree, and the remaining have profound hearing loss. At three years, 27 children used hearing aids, 19 used cochlear implants, and one child did not use any hearing device. Thirty percent of children have disabilities in addition to hearing loss. On average, there were no significant differences in performance level between children with and without ANSD. Also, the variability of scores was not significantly different between the two groups. Conclusions: There was no significant difference in performance levels or variability between children with and without ANSD, both for children who use hearing aids, and children who use cochlear implants.     

The case for earlier cochlear implantation in postlingually deaf adults

Abstract

Objective: This paper aimed to estimate the difference in speech perception outcomes that may occur due to timing of cochlear implantation in relation to the progression of hearing loss. Design: Data from a large population-based sample of adults with acquired hearing loss using cochlear implants (CIs) was used to estimate the effects of duration of hearing loss, age, and pre-implant auditory skills on outcomes for a hypothetical standard patient. Study sample: A total of 310 adults with acquired severe/profound bilateral hearing loss who received a CI in Melbourne, Australia between 1994 and 2006 provided the speech perception data and demographic information to derive regression equations for estimating CI outcomes. Results: For a hypothetical CI candidate with progressive sensorineural hearing loss, the estimates of speech perception scores following cochlear implantation are significantly better if implantation occurs relatively soon after onset of severe hearing loss and before the loss of all functional auditory skills. Conclusions: Improved CI outcomes and quality of life benefit may be achieved for adults with progressive severe hearing loss if they are implanted earlier in the progression of the pathology.     

Barriers to early cochlear implantation

Abstract

Objective: Identify variables associated with paediatric access to cochlear implants (CIs). Design: Part 1. Trends over time for age at CI surgery (N?=?802) and age at hearing aid (HA) fitting (n?=?487) were examined with regard to periods before, during, and after newborn hearing screening (NHS). Part 2. Demographic factors were explored for 417 children implanted under 3 years of age. Part 3. Pre-implant steps for the first 20 children to receive CIs under 12 months were examined. Results: Part 1. Age at HA fitting and CI surgery reduced over time, and were associated with NHS implementation. Part 2. For children implanted under 3 years, earlier age at HA fitting and higher family socio-economic status were associated with earlier CI. Progressive hearing loss was associated with later CIs. Children with a Connexin 26 diagnosis received CIs earlier than children with a premature / low birth weight history. Part 3. The longest pre-CI steps were Step 1: Birth to diagnosis/identification of hearing loss (mean 16.43 weeks), and Step 11: MRI scans to implant surgery (mean 15.05 weeks) for the first 20 infants with CIs under 12 months. Conclusion: NHS implementation was associated with reductions in age at device intervention in this cohort.     

Comparison between hearing screening-detected cases and sporadic cases of delayed-onset hearing loss in preschool-age children

Abstract

Objective: This study aimed to compare the diagnosis and ages of intervention for cases of delayed-onset hearing loss identified sporadically or via a preschool hearing screening program. Design: Retrospective study with the comparative analysis of two groups of children. Study sample: Cases identified from screening were selected from 34 321 preschool children who underwent screening for delayed-onset hearing loss between October 2009 and May 2011. Sporadic cases of delayed-onset hearing loss were selected from pediatric clinical records. Cases from the first group were excluded from the latter to avoid duplication. Two groups were given the same questionnaire to record risk indicators, diagnosis, and age at intervention. Results: The average age of 26 children at the time of diagnosis in the screening group (52.81 ± 13.23 months) was significantly earlier than in the 33 cases identified in the sporadic group (62.03 ± 12.86 months; p < 0.05). The age at intervention of children with bilateral moderate to severe hearing loss in the screening group (50.40 ± 10.76 months) was also earlier than in the sporadic group (62.73 ± 13.77 months; p < 0.05). Conclusions: Improved rates of early diagnosis could therefore be achieved with hearing screening for preschool children with no significant symptoms of delayed-onset hearing loss.     

Receptive language as a predictor of cochlear implant outcome for prelingually deaf adults

Abstract

Objective: This study investigated outcomes and predictive factors, specifically language skills, for a group of prelingually hearing-impaired adults who received a cochlear implant. Design: Speech perception data, demographic information, and other related variables such as communication mode, residual hearing, and receptive language abilities were explored. Pre- and post-implant speech perception scores were compared and multiple regression analysis was used to identify significant predictive relationships. Study sample: The study included 43 adults with a prelingual onset of hearing loss, who proceeded with cochlear implantation at the Royal Victorian Eye and Ear Hospital in Melbourne, Australia. Results: The majority of patients experienced benefit from their cochlear implants, with 88% demonstrating significant improvement in speech perception performance. Volunteers achieved better post-operative speech perception scores if they had a shorter duration of severe-to-profound hearing loss, better language skills, and used an exclusively oral communication mode. Conclusions: Although post-operative speech perception performance is significantly poorer for prelingually hearing-impaired adults compared to postlingually hearing-impaired patients, the study group demonstrated significant benefit from their cochlear implants. The variability in post-operative outcomes can be predicted to some extent from the hearing history and language abilities of the individual patient.     

Use of technological aids and interpretation services among children and adults with hearing loss

Objectives: The technological development of communication aids for people with hearing loss has progressed rapidly over the last decades. Quality has improved and the number of different types of aids has increased. However, few studies have examined the prevalence of technology use and interpreting services use among people with hearing loss as they relate to demographic characteristics of this population. Design: This study reports from national surveys of children and adults with hearing loss. Use of hearing aids, cochlear implants, other aids and interpreting services were analysed with regard to gender, age, degree of hearing loss, mode of communication, having an additional disability, level of educational achievement among adults, and whether or not children lived together with both of their parents. Study sample: 269 children (0–15 years of age) and 839 adults (16–65 years of age). Results: Differences in technology and service use were associated with age, degree of hearing loss, and mode of communication among children and adults, and gender and level of educational achievement among adults. Conclusion: Individual and social factors have an impact on technological hearing aid and interpreter use. More research about individual differences and clinical implications of support services is needed.     

The clinical features and prognosis of mumps-associated hearing loss: a retrospective,multi-institutional investigation in Japan

Abstract

Conclusions: The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss.

Objective: The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes.

Subjects and methods: The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016.

Results: Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.     

Ten years’ experience with bone conduction hearing aids in the Western Cape,South Africa

Untreated conductive and mixed hearing losses as a result of middle ear pathology or congenital ear malformations can lead to poor speech, language and academic outcomes in children. Lack of access to centralised hearing healthcare in resource-constrained environments limits opportunities for children with hearing loss. Red Cross War Memorial Children’s Hospital (RCWMCH) is one of only two dedicated paediatric hospitals in sub-Saharan Africa. Between 2016 and 2021, 29 children received implanted bone conduction hearing devices, and 104 children were fitted with bone conduction devices on softbands. The authors’ experience at RCWMCH suggests that bone-anchored hearing devices, either fitted on softbands or on implanted abutments, can provide solutions in settings where patients have limited access to hearing healthcare and optimal classroom environments. Hearing healthcare should be accessible and delivered at the appropriate level of care to mitigate the adverse effects of hearing loss in children.ContributionThis article describes strategies employed at RCWMCH such as fitting bone conduction hearing devices on a softband immediately after hearing loss diagnosis and conducting follow-up via remote technology to make hearing healthcare more accessible to vulnerable populations.     

Sex-specific predictors of hearing-aid use in older persons: The age,gene/environment susceptibility - Reykjavik study

Objective: We estimate the prevalence of hearing-aid use in Iceland and identify sex-specific factors associated with use. Design: Population-based cohort study. Study sample: A total of 5172 age, gene/environment susceptibility - Reykjavik study (AGES-RS) participants, aged 67 to 96 years (mean age 76.5 years), who completed air-conduction and pure-tone audiometry. Results: Hearing-aid use was reported by 23.0% of men and 15.9% of women in the cohort, although among participants with at least moderate hearing loss in the better ear (pure-tone average [PTA] of thresholds at 0.5, 1, 2, and 4 kHz ≥ 35 dB hearing level [HL]) it was 49.9% and did not differ by sex. Self-reported hearing loss was the strongest predictor of hearing-aid use in men [OR: 2.68 (95% CI: 1.77, 4.08)] and women [OR: 3.07 (95% CI: 1.94, 4.86)], followed by hearing loss severity based on audiometry. Having diabetes or osteoarthritis were significant positive predictors of use in men, whereas greater physical activity and unimpaired cognitive status were important in women. Conclusions: Hearing-aid use was comparable in Icelandic men and women with moderate or greater hearing loss. Self-recognition of hearing loss was the factor most predictive of hearing-aid use; other influential factors differed for men and women.     

Improving speech-in-noise recognition for children with hearing loss: Potential effects of language abilities,binaural summation,and head shadow

Abstract

Objective: This study examined speech recognition in noise for children with hearing loss, compared it to recognition for children with normal hearing, and examined mechanisms that might explain variance in children's abilities to recognize speech in noise. Design: Word recognition was measured in two levels of noise, both when the speech and noise were co-located in front and when the noise came separately from one side. Four mechanisms were examined as factors possibly explaining variance: vocabulary knowledge, sensitivity to phonological structure, binaural summation, and head shadow. Study sample: Participants were 113 eight-year-old children. Forty-eight had normal hearing (NH) and 65 had hearing loss: 18 with hearing aids (HAs), 19 with one cochlear implant (CI), and 28 with two CIs. Results: Phonological sensitivity explained a significant amount of between-groups variance in speech-in-noise recognition. Little evidence of binaural summation was found. Head shadow was similar in magnitude for children with NH and with CIs, regardless of whether they wore one or two CIs. Children with HAs showed reduced head shadow effects. Conclusion: These outcomes suggest that in order to improve speech-in-noise recognition for children with hearing loss, intervention needs to be comprehensive, focusing on both language abilities and auditory mechanisms.     

Hearing and ear status of Pacific children aged 11 years living in New Zealand: the Pacific Islands families hearing study

Objective: This study aimed to determine the prevalence of hearing loss and ear problems in Pacific children, and investigate current and past demographic, health and social factors potentially associated with hearing and ear problems.

Design: A cross-sectional observational study design nested within a birth cohort was employed.

Study sample: Nine-hundred-twenty Pacific children aged 11?years were audiologically assessed. Using average hearing thresholds at 500, 1k and 2k Hz, 162 (18%) right and 197 (21%) left ears had ≥20?dB hearing loss. Hearing loss was mild (20–39?dB) in most cases; 2% of ears had moderate to moderate-severe (40–69?dB) hearing loss. However, only 101 (11%) children had normal peripheral hearing defined by passing hearing threshold, tympanogram and distortion product otoacoustic emission assessments. Those with confirmed middle ear disease at age 2?years had significantly increased odds of a non-Type A tympanogram (adjusted odds ratio: 2.00; 95% confidence interval: 1.56, 2.50) when re-assessed at age 11?years.

Conclusions: Hearing loss, abnormal tympanograms, and auditory processing difficulties were present in many Pacific children. Interventions are also urgently needed to mitigate the effect of the longstanding ear disease likely to be present for many Pacific children.     

Evidence-based guidelines for recommending cochlear implantation for young children: Audiological criteria and optimizing age at implantation

Abstract

Objective: Establish up-to-date evidence-based guidelines for recommending cochlear implantation for young children. Design: Speech perception results for early-implanted children were compared to children using traditional amplification. Equivalent pure-tone average (PTA) hearing loss for cochlear implant (CI) users was established. Language of early-implanted children was assessed over six years and compared to hearing peers. Study sample: Seventy-eight children using CIs and 62 children using traditional amplification with hearing losses ranging 25–120?dB HL PTA (speech perception study). Thirty-two children who received a CI before 2.5 years of age (language study). Results: Speech perception outcomes suggested that children with a PTA greater than 60?dB HL have a 75% chance of benefit over traditional amplification. More conservative criteria applied to the data suggested that children with PTA greater than 82?dB HL have a 95% chance of benefit. Children implanted under 2.5 years with no significant cognitive deficits made normal language progress but retained a delay approximately equal to their age at implantation. Conclusions: Hearing-impaired children under three years of age may benefit from cochlear implantation if their PTA exceeds 60?dB HL bilaterally. Implantation as young as possible should minimize any language delay resulting from an initial period of auditory deprivation.     

Characteristics of children with unilateral hearing loss

Objective: The purpose of this study was to describe the clinical characteristics of children with unilateral hearing loss (UHL), examine deterioration in hearing, and explore amplification decisions. Design: Population-based data were collected prospectively from time of diagnosis. Serial audiograms and amplification details were retrospectively extracted from clinical charts to document the trajectory and management of hearing loss. Sample: The study included all children identified with UHL in one region of Canada over a 13-year period (2003–2015) after implementation of universal newborn hearing screening. Results: Of 537 children with permanent hearing loss, 20.1% (108) presented with UHL at diagnosis. They were identified at a median age of 13.9 months (IQR: 2.8, 49.0). Children with congenital loss were identified at 2.8 months (IQR: 2.0, 3.6) and made up 47.2% (n?=?51), reflecting that a substantial portion had late-onset, acquired or late-identified loss. A total of 42.4% (n?=?39) showed deterioration in hearing, including 16 (17.4%) who developed bilateral loss. By study end, 73.1% (79/108) of children had received amplification recommendations. Conclusions: Up to 20% of children with permanent HL are first diagnosed with UHL. About 40% are at risk for deterioration in hearing either in the impaired ear and/or in the normal hearing ear.     

Comparison of outcomes in children with hearing aids and cochlear implants

Abstract

Objectives

The purpose of this study was to document the performance of a group of children with moderately severe to severe hearing loss who use hearing aids on a range of speech recognition, speech–language, and literacy measures and to compare these results to children with severe to profound hearing loss, who have learned language through cochlear implants.

Methods

This study involved 41 children with bilateral sensorineural hearing impairment, aged 6–18 years. Twenty children had moderately severe/severe hearing loss and used hearing aids, and 21 had severe to profound hearing loss and used cochlear implants. Communication and academic skills were assessed using speech recognition tests and standardized measures of speech production, language, phonology, and literacy.

Results

The two groups did not differ in their open-set speech recognition abilities or speech production skills. However, children with hearing aids obtained higher scores than their peers with cochlear implants in the domains of receptive vocabulary, language, phonological memory, and reading comprehension. The findings also indicate that children with moderately severe or severe hearing loss can develop spoken language skills that are within the range expected for normal hearing children.

Conclusions

School-aged children with moderately severe and severe hearing loss performed better in several domains than their peers with profound hearing loss who received cochlear implants between age 2 and 5 years. Further research is required to evaluate the benefits of hearing aids and cochlear implants in children with hearing loss who are diagnosed and receive intervention within the first year of life.     

An Australian version of the acceptable noise level test and its predictive value for successful hearing aid use in an older population

Abstract

Objective: The acceptable noise level (ANL), a measure of noise tolerance, has been proposed as a predictor for successful hearing aid use. The aims of this study were to obtain normative data, and to evaluate the clinical feasibility and predictive value of an Australian version of the ANL test in an older population. Design: Repeated ANL measurements were presented diotically using earphones. All participants provided demographic information and hearing aid owners were asked about their aid use. Study sample: A total of 290 older adults were assessed; 166 participants had a hearing impairment and 96 owned a hearing aid. Results: The mean ANL was lower than previously reported. While age and gender had no effect on ANL, a significant, but weak, correlation was found between ANL and hearing loss. The test-retest reliability showed the results were clinically unreliable. In addition, the ANL did not predict hours or pattern of hearing aid use. Conclusions: While the Australian ANL test showed several similar characteristics to previous versions of the test, low test-retest reliability raised questions about its clinical value as a predictor for long-term hearing aid use.     

When expectation meets experience: Parents’ recollections of and experiences with a child diagnosed with hearing loss soon after birth

Abstract

Objective: To examine parents’ recollections of and their experiences with bringing up a child diagnosed with hearing loss at a very young age. Design: Based on the analysis of informal parent discussion groups, four open-ended questions were formulated to solicit information about parents’ expectations following diagnosis, as well as experiences and challenges when raising a child with a hearing loss. Study sample: Forty parents of children, aged between three to five years, who were diagnosed with hearing loss before the age of three years. Results: Parents’ responses revealed strong support for early intervention, high expectations for their child's development, and desire for information tailored to individual needs. Parents also reported anxiety relating to their perceptions of the significance of consistent device usage on their child's development. Further concerns arose from their observations of the difficulties experienced by their child in real-world environments despite consistent device usage, and their perception of their child's language delay despite early intervention. Conclusions: The findings point to a need to support parents to form realistic expectations based on current knowledge. Implications for clinicians to provide improved management of children with hearing loss are discussed.     

Neonatal Hearing Screening: failures,hearing loss and risk indicators

To check the rate of failure, hearing loss and its association with demographic variables and risk indicators for hearing loss in newborns submitted to the Newborn Hearing Screening in a secondary hospital.Materials and MethodsCross-sectional and retrospective study, involving 1,570 newborns submitted to the different stages of the Newborn Hearing Screening Program. Initially, we carried out otoacoustic emission tests (ILO Echocheck) and the cochlear-eyelid reflex. Afterwards, we analyzed the demographic and clinical characteristics of the newborns, screening rate of failure, hearing loss and its association with demographic variables and risk indicators.ResultsTwenty-six newborns had failures in the first stages of the Program (1.7%), who were then referred to diagnostic evaluation. Of these, 16 (61.5%) did not come, two (7.7%) had normal results and eight (30.8%) were diagnosed with hearing disorders. The screening failure rate was 1.7% and the frequency of hearing disorders was 0.5%.ConclusionsPre-term newborns of very low birth weights had higher rates of screening failures and a greater occurrence of hearing changes. The factors associated with screening failure and hearing changes were similar to the ones described in the literature.     

La audición en la enfermedad ósea de Paget

Introduction and objectivesPaget's disease of bone (PDB) may lead to hearing loss. The present study was conducted with the aim of measuring, characterizing and determining the risk factors for hearing loss in a group of subjects with PDB.MethodsAn observational, transversal, case-control study was conducted, a cohort of 76 subjects diagnosed with PDB in the case group and a control group of 134 subjects were included. Clinical, demographic and audiometric data were analysed.ResultsThe comparative analysis between the subjects in the PDB group and the control group found that the case group showed higher hearing thresholds (39,51 dB) compared with the control group (37.28 dB) (P=.069) and presented a greater rate of conductive hearing loss (22.76%) than the control group (12.05%) (P=.0062). The study of risk factors for hearing loss found that skull involvement in bone scintigraphy, age and high blood pressure were risk factors for higher impairment in PDB.ConclusionsThe subjects with PDB showed more profound and a higher proportion of conductive hearing loss than the control group. The patients with PDB and skull involvement presented a more severe hearing loss compared with the subjects without skull involvement. Skull involvement and age were found to be risk factors for hearing loss.