The Role of the Audiologist in Life Care Planning
Roger O. Weed, Debra E. Berens in Life Care Planning and Case Management Handbook, 2018
Hearing loss significant enough to affect the understanding of speech creates a potential handicap and adversely affects quality of life, regardless of age of onset. However, hearing loss present at birth or occurring during the newborn period has even greater implications. Normal hearing, particularly during the first 3 years of life, is essential to the development of speech and language. Children affected with congenital or newborn hearing loss may lose their ability to acquire fundamental speech, language, cognition, and social skills required for later schooling and success in society. Therefore, early identification and intervention for newborn hearing loss has been a priority for audiologists for decades. However, the fact that newborns show little behavioral responses to sound made early identification of hearing loss in this population particularly challenging.
Deafness
Angus Clarke, Alex Murray, Julian Sampson in Harper's Practical Genetic Counselling, 2019
Even without access to specialised audiological testing, it is often possible to assess the genetic situation accurately if the following points are borne in mind: What does the pattern of inheritance in the particular family suggest?Is the hearing loss severe congenital deafness, or some milder form?If hearing loss is milder, is it static or progressive?Is there an identifiable syndrome involving other systems?
Vision and hearing
Ajay Sharma, Helen Cockerill in From Birth to Five Years, 2021
Conductive hearing loss is common; at least half of pre-school children have one or more episodes of ‘glue ear' or otitis media with effusion (OME). Persistent OME, which may have adverse effects on children's language and behaviour, affects about 5–10 per cent of children. Parental smoking is a risk factor for children developing OME. Significant sensorineural hearing loss (SNHL), requiring a hearing aid, is present in about 16 per 10,000 children. Delayed identification of children with congenital or acquired hearing loss may result in deficits in speech and language development, low educational achievement, and behaviour difficulties.
Cost-effectiveness analysis on implementing newborn hearing screening programmes in a low- to middle-income country
Published in International Journal of Audiology, 2023
Pittayapon Pitathawatchai, Sitthichok Chaichulee, Wirawan Wannaro, Patchanok Pongprawat
Congenital hearing loss is one of the most common birth conditions in newborns. Approximately, 0.1–0.3% of all newborns suffer from this disability (Erenberg et al. 1999). To minimise adverse developmental effects from congenital hearing loss, early detection and intervention are crucial. A strategy to achieve this goal has been recommended by the Joint Committee of Infant Hearing (JCIH) which involves providing all newborns with universal newborn hearing screening (UNHS) (JCIH 2019). Although there have been no randomised controlled trials in regard to outcomes of UNHS programmes, a quasi-randomised control study reported that better results in speech and language development were evident in hearing-impaired infants who received early detection and intervention than those without early intervention (Korver et al. 2010).
Effects of basilar-membrane lesions on dynamic responses of the middle ear
Published in Acta Oto-Laryngologica, 2023
Junyi Liang, Wen Xie, Wenjuan Yao, Maoli Duan
Some disorders of metabolism in the cochlear such as mucopolysaccharidosis can cause basilar membrane damage or an increase the sensory cell weight. In addition, the mass of the spiral limbus cells, spiral process, and the spiral ligament increases notably [16]. All these structures are within the organ of Corti, which attaches to the basilar membrane. As a result, added mass of the basilar membrane increases. In addition, some genetic defects can lead to congenital deafness. The main characteristics embody in the hypertrophy of Sertoli cells in cortis device, leads to the increase of the basilar membrane mass. Added mass in the basilar membrane was adopted as the following situation: average thickness increased 0.15 mm, while the added mass is 36 × 10−9 kg. Under 90 dB SPL, compared with the normal human ear, the frequency-response curve of displacement and velocity were respectively obtained, as shown in Figure 7.
The applications of targeted delivery for gene therapies in hearing loss
Published in Journal of Drug Targeting, 2023
Melissa Jones, Bozica Kovacevic, Corina Mihaela Ionescu, Susbin Raj Wagle, Christina Quintas, Elaine Y. M. Wong, Momir Mikov, Armin Mooranian, Hani Al-Salami
It is also important to highlight the category of hereditary hearing loss, which may be one of the main benefactors in the development of an inner ear targeted gene therapy. Early onset forms of deafness, often presented as hereditary deafness, has been indicated to result from a wide array of genes, with an estimation that over 50% of congenital deafness presentations in newborns are hereditary in nature [33,34]. Classifications of genetic deafness are derived based upon the mutation inheritance mode, with ongoing novel discoveries of genes associated with hearing loss. Molecular genetic tests can be used to identify deafness, both syndromic and non-syndromic in nature. Genetic counselling is a key element in the diagnosis, with modes of inheritance listed as including autosomal recessive and dominant, in addition to X-linked Mendelian and mitochondrial inheritance from a maternal lineage. Syndromic hearing loss which is autosomal recessive in nature includes Usher syndrome and Pendred syndrome, whilst those which are autosomal dominant include Branchio-oto-renal syndrome and Waardenburg syndrome [35–37].
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