Maltese children with a hearing impairment : analysis of the current situation and its impact on the quality of life of parents

Understanding the effect that a diagnosis of a childhood hearing loss has on parents would help professionals adopt an approach which diminishes parents’ possible negative feelings and concerns. A sample of parents of children with hearing impairment was interviewed to document demographic data related to hearing loss in Maltese children. The present study also attempted to analyse the effect of hearing loss on the parents’ wellbeing. Parents of 23 children with a hearing loss and parents of eight children without a hearing loss participated in the study. Structured face-to-face interviews were carried out with both groups of parents. A self-devised questionnaire was used with parents of children with hearing impairment to collect information about several factors related to the hearing loss. This included documentation of the different types, degrees and causes of hearing loss as well as the ages of suspicion, diagnosis and amplification of children with a hearing loss. The World Health Organisation Quality of Life-BREF (WHOQOL-BREF) questionnaire (World Health Organisation [WHO], 1998) was then used with both groups of parents to obtain a quality of life profile in four domains: physical health, psychological health, social relationships and environment. Slight quality of life differences, which were not statistically significant, were noticed between parents of children with a hearing loss and parents of children without a hearing loss. Quality of life scores were marginally higher for parents whose gap between the day of diagnosis and the interview date was more than 24 months, when compared to parents whose gap was 24 months or less. These findings extend the limited data on the effect of hearing loss on parents’ quality of life in the Maltese context. More intensive support may be indicated for parents of children with hearing impairment, particularly in the initial stages following a diagnosis of a hearing loss. Support would help parents better understand and accept their child’s hearing impairment.peer-reviewe

Impact of aging on the auditory system and related cognitive functions: A narrative review

Age-related hearing loss (ARHL), presbycusis, is a chronic health condition that affects approximately one-third of the world’s population. The peripheral and central hearing alterations associated with age-related hearing loss have a profound impact on perception of verbal and non-verbal auditory stimuli. The high prevalence of hearing loss in the older adults corresponds to the increased frequency of dementia in this population. Therefore, researchers have focused their attention on age-related central effects that occur independent of the peripheral hearing loss as well as central effects of peripheral hearing loss and its association with cognitive decline and dementia. Here we review the current evidence for the age-related changes of the peripheral and central auditory system and the relationship between hearing loss and pathological cognitive decline and dementia. Furthermore, there is a paucity of evidence on the relationship between ARHL and established biomarkers of Alzheimer’s disease, as the most common cause of dementia. Such studies are critical to be able to consider any causal relationship between dementia and ARHL. While this narrative review will examine the pathophysiological alterations in both the peripheral and central auditory system and its clinical implications, the question remains unanswered whether hearing loss causes cognitive impairment or vice versa

A large scale hearing loss screen reveals an extensive unexplored genetic landscape for auditory dysfunction

The developmental and physiological complexity of the auditory system is likely reflected in the underlying set of genes involved in auditory function. In humans, over 150 non-syndromic loci have been identified, and there are more than 400 human genetic syndromes with a hearing loss component. Over 100 non-syndromic hearing loss genes have been identified in mouse and human, but we remain ignorant of the full extent of the genetic landscape involved in auditory dysfunction. As part of the International Mouse Phenotyping Consortium, we undertook a hearing loss screen in a cohort of 3006 mouse knockout strains. In total, we identify 67 candidate hearing loss genes. We detect known hearing loss genes, but the vast majority, 52, of the candidate genes were novel. Our analysis reveals a large and unexplored genetic landscape involved with auditory function

Detection of cochlear hearing loss applying wavelet packets and support vector machines

The aim of this paper is to evaluate the application of the wavelet packet transform (WP) and support vector machines (SVM) to transient evoked otoacoustic emissions (TEOAE) in order to achieve a detection of frequency-specific hearing loss. We introduce a system to determine detection rates between groups of persons with normal hearing, high frequency hearing loss, and pantonal hearing loss. The validity and use of our approach is verified on a different patient group

Synchrony, complexity and directiveness in mothers\u27 interactions with infants pre- and post-cochlear implantation

This study investigated effects of profound hearing loss on mother–infant interactions before and after cochlear implantation with a focus on maternal synchrony, complexity, and directiveness. Participants included two groups of mother–infant dyads: 9 dyads of mothers and infants with normal hearing; and 9 dyads of hearing mothers and infants with profound hearing loss. Dyads were observed at two time points: Time 1, scheduled to occur before cochlear implantation for infants with profound hearing loss (mean age = 13.6 months); and Time 2 (mean age = 23.3 months), scheduled to occur approximately six months after cochlear implantation. Hearing infants were age-matched to infants with hearing loss at both time points. Dependent variables included the proportion of maternal utterances that overlapped infant vocalizations, maternal mean length of utterance, infant word use, and combined maternal directives and prohibitions. Results showed mothers’ utterances overlapped the vocalizations of infants with hearing loss more often before cochlear implantation than after, mothers used less complex utterances with infants with cochlear implants compared to hearing peers (Time 2), and mothers of infants with profound hearing loss used frequent directives and prohibitions both before and after cochlear implantation. Together, mothers and infants adapted relatively quickly to infants’ access to cochlear implants, showing improved interactional synchrony, increased infant word use, and levels of maternal language complexity compatible with infants’ word use, all within seven months of cochlear implant activation

Management options in the sudden hearing loss of a diabetic patient

The aim of our paper is to highlight the main therapeutic principles and the management options in the case of a diabetic patient who has had a sudden hearing loss. Mainly, the aim is to underline the sudden hearing loss treatment adjustment of the diabetic patient compared to the non-diabetic patient. By understanding the mechanism of sudden hearing loss in a diabetic patient, namely the impact of glycemic variations and their implication on the microvascular structures of the inner ear, we try to underline the treatment principles and management options of the previously mentioned combined pathologies. Thus, it is necessary to adapt the classes of drugs used in the case of sudden sensorineural hearing loss of the diabetic patient in comparison with the non-diabetic patient, in order not to aggravate or complicate the patient’s functional status. Therefore, the treatment will need to be adapted both by classes of medication and by the type of administration used. Adequate control of the progression, treatment and complications of diabetes mellitus ensures optimal treatment management in case of a sudden hearing loss and therefore interferes with the favorable functional hearing outcomes. The role of this paper is not only to state the therapeutic principles in the case of sudden hearing loss in a diabetic patient, but also to analyze the impact on the management of potential local and systemic risk factors

Hearing in young adults, part I: the effects of attitudes and beliefs toward noise, hearing loss and hearing protector devices

There is great concern regarding the development of noise-induced hearing loss (NIHL) in youth caused by high sound levels during various leisure activities. Health-orientated behavior of young adults might be linked to the beliefs and attitudes toward noise, hearing loss, and hearing protector devices (HPDs). The objective of the current study was to evaluate the effects of attitudes and beliefs toward noise, hearing loss, and HPDs on young adults hearing status. A questionnaire and an audiological test battery were completed by 163 subjects (aged 18-30 years). The questionnaire contained the Youth Attitude to Noise Scale (YANS) and Beliefs about Hearing Protection and Hearing Loss (BAHPHL). A more positive attitude or belief represented an attitude where noise or hearing loss is seen as unproblematic and attitudes and beliefs regarding HPDs is worse. Hearing was evaluated using (high frequency) pure tone audiometry (PTA), transient evoked and distortion product otoacoustic emissions. First, mean differences in hearing between the groups with different attitudes and beliefs were evaluated using one-way analysis of variance (ANOVA). Second, a (2) test was used to examine the usage of HPDs by the different groups with different attitudes and beliefs. Young adults with a positive attitude had significantly more deteriorated hearing and used HPDs less than the other subjects. Hearing conservation programs (HCPs) for young adults should provide information and knowledge regarding noise, hearing loss, and HPDs. Barriers wearing HPDs should especially be discussed. Further, those campaigns should focus on self-experienced hearing related symptoms that might serve as triggers for attitudinal and behavioral changes

Hearing impairment in Stickler syndrome: a systematic review

BACKGROUND: Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. METHODS: English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. RESULTS: 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). CONCLUSIONS: Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired

Discovery and Testimony of Unretained Experts: Creating a Clear and Equitable Standard to Govern Compliance With Subpoenas

Hearing impairment is known to be one of the most frequent sensory impairments. This condition is known to be a hidden disorder which is under recognised and under treated all around the world. The World Health Organisation (WHO) estimates suggest that there are over 275 million people with hearing impairment and 80% of them living in low and middle income countries. Moreover, the estimates suggest that incidence and prevalence of hearing loss and also the number of people with hearing loss accessing services varies considerably across countries. This rises the need for health promotion (or public awareness campaigns) directed to increase awareness and education of hearing loss and hearing healthcare. This paper provides brief discussion on ‘Stories and storytelling’, ‘Cross-culture and cross-cultural communication’ and ‘Health promotion and cultural sensitivity’. The central focus of this paper is to highlight the applications of storytelling in different cultural context in health promotion, particularly to hearing loss public awareness campaigns

Implementing a newborn hearing screening programme : a feasibility study : a thesis presented in partial fulfillment of the requirements for the degree of Master of Arts at Massey University

Aim. To determine the feasibility of implementing a universal newborn hearing screening programme at National Women's Hospital (NWH), Auckland, New Zealand. Method. This feasibility study evaluates the potential for success of a Universal Newborn Hearing Screening (UNHS) Programme in a tertiary hospital setting. A review of the present provision of care for infants with congenital hearing loss and a clear description of the current environment and resources at National Women's Hospital was undertaken. By utilising the four key determinants of a feasibility study as described by Whitten, Bently & Dittman (2001) I was able to provide a clear description of the current position and explore the alternative solutions, ensuring an accurate and comprehensive study approach was undertaken. Results. A detailed analysis of the environmental setting and population at NWH identified the support required for implementing a UNHS programme. Findings also identified the acceptance by both staff and consumers in providing improved congenital hearing loss detection and intervention early in the newborn period. The evidence supported recommendations for two possible hearing screening protocols that are both practical and feasible in the National Women's Hospital setting for the detection of congenital hearing loss in the newborn population. Conclusion. Overall findings indicated that the implementation of UNHS at National Women's Hospital is feasible. The current method of detecting hearing loss in the newborn population is inadequate with unacceptable delays for diagnosis and appropriate intervention to improve outcomes for those identified with a congenital hearing loss. The protocols supported by this study are based on the research findings and are unique to the NWH environment and target population. They will ensure the infants with congenital hearing loss are detected and referred early (soon after birth) so formal audiological diagnosis and strategies for intervention can occur with treatment implemented by six months of age. This will improve the child's communication and learning skills, improving their level of education and long term learning ability. Further and regular audit of the programme, screeners and outcomes will be required to ensure its efficiency as a screening service for congenital hearing loss