Evaluación del muy bajo peso (≤ 1.500 g) al nacer como indicador de riesgo para la hipoacusia neurosensorial

Introduction and objectiveHearing plays an essential role in the acquisition, development and maintenance of the properties of the speech and language. Birth weight is an indicator of biological maturation of the newborn. Premature newborns with very low birth weight (VLBW < 1,500 g) constitute a group with the highest risk of sensorineural hearing loss. Our objective was to ascertain the degree of hearing loss, sensorineural hearing loss and presence of the association to other risk factors for hearing loss in VLBW infants included in the Universal Hearing Loss Screening Programme at the University Mother-Child Hospital of Gran Canaria (Spain) in the 2007-2010 period.Material and methodsThis was a retrospective study of 364 infants with VLBW, measured by transient evoked otoacoustic emissions and auditory brainstem response.ResultsThere were 112 newborn (30.8%) referred for auditory brainstem response. A diagnosis of hearing loss was given to 22 newborns (2.2%), 14 had conductive hearing loss and 8, sensorineural hearing loss (SNHL), of which 2 had bilateral profound hearing loss. The VLBW newborn presented the association to another risk factor in more than a quarter of the sample studied. All those diagnosed with SNHL were premature.ConclusionsThe percentage of VLBW newborns diagnosed with hearing loss is higher than expected in the general population. All those diagnosed with SNHL were premature and presented one or 2 hearing risk factors associated with VLBW.     

Results of hearing aids use dispensed by a publicly-funded health service

Hearing aid use and satisfaction can be used to analyze the effectiveness of hearing rehabilitation, an issue few explored in Brazilian users.ObjectiveEvaluate hearing aid use dispensed in a publicly-funded health service after one year, the interventions needed and users' satisfaction.MethodProspective observational cross-sectional study. Hearing aid users were invited by telephone to participate in an evaluation of hearing aid use.Results200 hearing aid users attended the evaluation (47% of sample loss); 76.5% of the subjects were using hearing aids bilaterally, 10.5% unilaterally and 13.0% none of them; 99.5% of users needed some kind of intervention. Users who kept wearing their hearing aids were considered “satisfied”.ConclusionActive search by telephone showed that hearing aid users who attend this publicly-funded health service have difficulties to attend follow-up appointments. Most of the users that came for evaluation kept using their hearing aids; non-use rate was 13%. Practically all hearing aid users needed some kind of intervention. Despite the high level of satisfaction, those findings did not include users who did not participate in evaluation, which could represent subjects less adhered to the treatment. Results highlighted the need of periodical follow-ups to ensure the effectiveness of auditory rehabilitation.     

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.     

Prevalence and risk factors of hearing loss among infants in Jordan: Initial results from universal neonatal screening

Abstract

Objective: To determine the association of reported risk factors with hearing loss, and explore the possible contribution of other risk factors that may improve identifying Jordanian children for whom interventions should be provided. Design: A cross-sectional study was performed in 37 hospitals and mother-child clinics from different health sectors in Jordan. The hearing screening protocol was performed in three stages. Data collected as risk factors for infant hearing loss included those on the 2007 Joint Committee on Infant Hearing list as well as other factors believed to be possibly significant. Study sample: All babies born in or attending any of these hospitals or clinics were screened for hearing loss, which included a total of 63 041. Results: Of the 63 042 infants, 966 (1.5%) were confirmed to have significant hearing loss. We identified additional risk factors that were associated with hearing loss in infants. Conclusion: These preliminary data add to the understanding of the epidemiology of hearing loss in Jordan. The presence of at least one JCIH risk factor, admission to NICU for > 5 days, age, birth weight, postnatal hypoxia, and mode of delivery were independently associated with hearing loss.     

An auditory health program for neonates in ICU and/or intermediate care settings

Auditory screening and early identification and management of patients with hearing loss improve the development prospects of infants.ObjectiveTo analyze the outcomes produced by an Auditory Health Program in neonates managed in an intensive care unit.MethodThis prospective cross-sectional study enrolled neonates referred to the neonatal care unit at hospital CAISM/Unicamp with stays lasting for 48 hours and more within a period of 13 months.Automated monitoring of brainstem auditory evoked potentials was used in the auditory screening of neonates at the time of discharge. Children with poor BAEPs were sent to undergo audiological, otorhinolaryngological, and genetic tests.ResultsAuditory screening was performed for 84.7% of the live births; 39.7% were screened at 30 days or more of age. Diagnostic tests revealed that 63.8% of the children had normal hearing. Incidence of hearing loss was 4%; sensorineural hearing loss was observed in 1.4% of the subjects; 0.24% had auditory neuropathy spectrum disorder; and 2.2% had conductive hearing loss.ConclusionNeonatal auditory screening was not offered universally, and nor was it carried out, in many cases, within the child's first month of life. Screening must be performed before neonates are discharged and in more than one stage. A high incidence of hearing loss was observed.     

Assessment of hearing in a municipal public school student population

Children need good hearing for adequate learning. Hearing screening in students is important because it allows cases that go unnoticed by the family may be detected.AimTo evaluate hearing in children aged 4-15 years - public school students from pre-first to the fifth grade in the city of Cabedelo, Paraíba state.Material and MethodsA prospective study of 98 students referred by teachers of 18 public schools in Cabedelo, from June 2007 to June 2010. Students were examined by an otolaryngologist and underwent audiometric testing.ResultsEighteen schools were enrolled in this study. The student's age ranged from 4 to 15 years; 62% were male and 38% were female. Otolaryngological tests were normal in 85% of cases, and abnormal in 15% of cases. The most frequent findings were ear wax and tympanic membrane retraction. Audiometry demonstrated normal hearing in 66% of children and impaired hearing in 34%.ConclusionHearing Screening in school children is an important method for diagnosing hearing loss, especially in low-income populations.     

Differences between acoustic trauma and other types of acute noise-induced hearing loss in terms of treatment and hearing prognosis

Abstract

Objectives: To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews.

Methods: The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group.

Results: Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p?=?.030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant.

Conclusions: Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects.     

Frontonasal dysplasia: clinical evaluation on audiological and brainstem electrophysiological profiles

Frontonasal dysplasia (FND) is a rare malformative complex affecting the frontal portion of the face, the eyes and the nose; it may occur singly or associated with other clinical signs. No systematic studies describing hearing in this condition were found.AimTo evaluate hearing sensitivity and sound stimulus conduction from cochlea to brainstem in patients with clinical signs of FND.Methods21 patients with isolated or syndromic FND were submitted to a clinical (otological/ vestibular antecedents and otoscopy) and instrumental (pure tone and speech audiometry, tympanometry and brainstem auditory evoked response) hearing evaluation.DesignA clinical, cross-sectional observational prospective study.ResultsHearing thresholds were normal in 15 (70%) patients, abnormal in 5 (25%), mostly with conductive hearing loss; one patient did not cooperate with testing. The tympanometric curve was type A in 30 (72%) ears, type C in 5 (12%), type As in 4 (9%) and type B in 3 (7%). The auditory brainstem response (ABR) showed no abnormalities.ConclusionPatients with FND showed no abnormalities in the auditory system from cochlea to brainstem in this study. Mild conductive hearing loss found in some is probably related to cleft palate. Further evaluation of hearing pathways at higher levels is recommended.     

Hearing disorder from music; a neglected dysfunction

Conclusion: Music-induced acute acoustic trauma is not inevitably linked to hearing dysfunction as validated by conventional pure tone audiometry. Tinnitus is often in combination with hyperacusis. Our results point at ‘silent hearing loss’ as the underlying pathology, having afferent nerve terminal damage rather than hair cell loss as the structural correlate.

Objectives: Exposure to loud music is one of the most common causes of acute acoustic trauma, which adolescents and teenagers experience by voluntary exposure to loud music of sound levels up to 110?dB(A).

Methods: The clinical and psychophysical data of 104 consecutive patients with music-induced hearing disorder (MIHD) were analyzed to construct individual hearing and tinnitus profiles. In all cases, tinnitus was the presenting symptom.

Results: Hearing abilities were normal in about two-thirds of the tinnitus patients. Tinnitus was experienced most often as a high-frequency tone (83%). The Tinnitus Handicap Inventory (THI) scores ranged from 0 to 94 with an average score of 43.1. Visual analog scales (VAS) were used to assess tinnitus loudness (average 42.4) and annoyance (average 54.2), and tinnitus awareness was estimated (average 60.3). All VAS values correlated strongly with the THI. Hyperacusis was present in 65% and 71% of the patients reported sleeping disorders.     

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.     

LittlEARS auditory questionnaire as an infant hearing screening in Germany after the newborn hearing screening

Objective: To investigate the feasibility of using the LittlEARS® Auditory Questionnaire (LEAQ®) as part of the infant hearing screening programme in Germany.

Design: LEAQ®s were distributed to 47 paediatric practices and were completed by the parents/guardians of the infants (aged between 9-14?months) involved in the study (= LEAQ^® screening). The infants who failed the LEAQ^® screening were invited to a LEAQ rescreening. Infants who failed the LEAQ^® rescreening were sent to a paediatric ENT specialist. After 3?years, a follow-up was performed on two groups: the first group comprised infants who failed the LEAQ screening; the second group (control group) comprised 200 infants who passed the LEAQ screening.

Study Sample: 5316 questionnaires were returned.

Results: Six infants with permanent hearing loss were identified using the LEAQ^® as a screening tool.

Conclusions: An infant hearing screening using the LEAQ^® is easily implementable in paediatric practices and may be a good alternative in countries where no objective screening instruments are available. The LEAQ^® was suitable for monitoring hearing development in infants in general and could help to identify a late-onset or progressive hearing loss in infants.     

Measuring psychological inflexibility in adult and child hearing loss

Abstract

Objective: Hearing loss is a chronic condition that impacts functioning among individuals with hearing loss and caregivers of children with hearing loss. Even though treatments for hearing loss can alleviate functional impairment, psychological factors like psychological inflexibility may interfere with treatment engagement and adherence, undermining the benefits of treatment. Measuring psychological inflexibility may inform care providers’ case conceptualisation, improving the quality and precision of audiological interventions. Thus, this study aimed to develop and validate measures of psychological inflexibility in hearing loss for adults and caregivers of children with hearing loss.

Design: Participants were invited to complete an online survey.

Study samples: Our sample comprised adults with hearing loss (N?=?264) and primary caregivers of children with hearing loss (N?=?275).

Results: The final versions of Acceptance and Action Questionnaire-Adult Hearing Loss (AAQ-AHL) and Acceptance and Action Questionnaire-Management of Child Hearing Loss (AAQ-MCHL) showed good to excellent internal reliability and concurrent and discriminant validity.

Conclusion: Although the AAQ-AHL and AAQ-MCHL showed acceptable psychometric properties, more tests are needed to further validate these measures and verify their utility in research and clinical settings.     

An investigation into hearing loss among patients of 50 years or older

Objects To investigate the extent of hearing loss in an elderly sample population to estimate hearing disorders among the age-equivalent population in China and to study primary clinical characteristic...     

Findings from multidisciplinary evaluation of children with permanent hearing loss

Objectives

To describe clinical findings from a multidisciplinary program for children with permanent hearing loss (PHL).

Methods

Retrospective chart review at a tertiary care children's hospital. Patients: Two hundred patients charts were selected from the population of 260 children with permanent hearing loss presenting between July 2005 and December 2006. Main outcome measures: PHL etiology; radiographic findings; clinical findings by genetics, ophthalmology, developmental pediatrics, speech pathology, and aural rehabilitation.

Results

Etiology of hearing loss was determined in 60% of subjects. Genetic causes of hearing loss were identified or presumed (positive history of first degree relative with hearing loss) in 27% of the children. Structural ear anomalies were found in 20% of children. Among the 36% of children with CNS imaging, abnormal findings were noted in 32%. There were a high rate of ophthalmological findings (53%) among children seen by ophthalmology (n = 105). Neurodevelopmental evaluations were completed in 58% of subjects and clinically significant findings were noted in 68%. Of the 61% of children who receiving received speech/language evaluations, 77% required intervention. Over half of the 40% of subjects who had an aural rehabilitation evaluation needed therapy. There were not significant differences in rates of findings for children with mild or unilateral hearing loss as compared to children with more severe degrees of hearing loss.

Conclusions

Interdisciplinary medical evaluation of children with PHL allows for the identification and treatment of clinically significant ophthalmologic, neurodevelopmental, genetic, and speech/language disorders. A high rate of CNS and temporal bone abnormalities were identified. These findings provide an understanding of the importance of considering thorough medical and developmental evaluations among children who are deaf/hard of hearing.     

Autosomal recessive non-syndromic hearing loss is caused by novel compound heterozygous mutations in TMC1 from a Tibetan Chinese family

ObjectivesHearing loss is the most common sensory disorder worldwide. Biallelic mutations in 42 different genes have been identified as associated with autosomal recessive non-syndromic hearing loss (ARNSHL). One of the common genes responsible for ARNSHL is TMC1. TMC1 mutations have been reported to cause non-syndromic hearing loss in a variety of populations. The current study is designed to investigate mutations prevalent among Chinese ethnic groups with ARNSHL.MethodsTargeted exome sequencing (TES) was employed to study the genetic causes of two siblings with ARNSHL in a Tibetan Chinese family. Variants identified by TES were further confirmed by Sanger sequencing.ResultsWe identified two distinct variants in the TMC1 gene in two deaf siblings of one Tibetan Chinese family using TES. Both siblings inherited a paternal allele containing a deletion of c.1396_1398AAC (p.Asn466del) and a maternal allele containing an insertion of c.2210_2211insCT (p.Glu737HisfsX2). The former disrupts a highly conserved residue in the large intracellular loop domain adjacent to the fourth transmembrane domain, and the latter causes a truncation of a portion of the C-terminal domain. These variants were compound heterzygous and segregated with the hearing impairment in this family.ConclusionThe novel compound heterozygous mutant alleles of TMC1 identified in this study were responsible for the ARNSHL in this Tibetan Chinese family. Although compound heterozygous mutations in TMC1 occurring in different TMC1 domains have been previously described in Han Chinese; this result suggests that the TMC1 variants contributing to hereditary deafness in Chinese populations may be more complex than initially assumed and that sequence-based diagnostics will be required for a comprehensive evaluation of ARNSHL.     

Cochleovestibular manifestations in Fabry disease: Importance of screening and systematic ENT evaluation

ObjectivesFabry disease (FD) is an X-linked inherited lysosomal storage disease. It is a multisystem pathology that can include ENT disorders. The aim of the present study was to investigate the cochleovestibular manifestations of FD, in order to show the importance of screening and systematic ENT evaluation.Material and methodsA single-center retrospective study included 14 male and 23 female FD patients. Hearing impairment was defined as hearing loss greater than the 90^th percentile for at least one frequency. Vestibular impairment was defined by lateral semicircular canal dysfunction. Age, ongoing enzyme replacement therapy (ERT) and organic (renal, cardiac and cerebrovascular) complications were used as severity markers.ResultsHearing impairment was found in 62.6% of cases, mostly at high frequencies, and was associated with age, ERT, and cardiac and cerebrovascular disorder. It affected 46.7% of asymptomatic adult patients.Vestibular impairment was found in 56% of cases, associated with age; it affected two-thirds of ERT patients, more than 60% of patients with organic complications, and 50% of asymptomatic adult patients.ConclusionsMore than half of patients had ENT involvement. All FD patients should undergo early ENT screening for diagnostic, prognostic and therapeutic purposes. Systematic complete ENT follow-up with auditory and vestibular evaluation should be performed regularly, even for heterozygous women.