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Cortical Deafness (Plus Other Central Hearing Disorders)
Published in Alexander R. Toftness, Incredible Consequences of Brain Injury, 2023
Pure word deafness is a disorder in which a person can still hear sounds, but they don't understand words. Instead, words start to sound a bit like a foreign language. They have the ability to hear, the ability to read, and the ability to speak, but not the ability to understand what other people are saying. Language sounds like nonsense to them. Despite this, a person with pure word deafness can understand other sounds. For example, they may hear and respond to music or knocks on the door (Mills, 1891). Pure word deafness may be a mild version of auditory agnosia, where the problem is with understanding short and quick speech sounds but they can still understand longer, repeated, and slower sounds of music or from nature (Phillips & Farmer, 1990). That is, musical and natural sounds may be easier for the brain to understand because they are simply more distinct, and more predictably repetitive, than language (Pinard et al., 2002).
Hearing Loss in Childhood
Published in Raymond W Clarke, Diseases of the Ear, Nose & Throat in Children, 2023
The question ‘why is my baby deaf’ is of great concern to parents. Deafness is a manifestation of one or more pathological processes rather than a final diagnosis, and it is important – but not always possible – to determine an exact aetiology. This helps to outline prognosis and may facilitate specific treatment strategies e.g. antiviral medication in some limited circumstances, and perhaps precise genetic interventions in the future. A definitive diagnosis may also enable referral to appropriate specialists e.g. endocrinologists for Pendred’s syndrome, and genetic counselling services.
Hunter disease/mucopolysaccharidosis type II/iduronate sulfatase deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
A variety of supportive measures are useful, especially in the milder forms of the disease, in which longer survival is associated with some painful complications. Shunting is important in the management of hydrocephalus. Hearing aids may aid in deafness. Physiotherapy is useful for the joint stiffness and the avoidance of contractures. Surgical decompression is carried out for carpal tunnel syndrome. Cardiac valvular status should be monitored by echocardiography. Tracheostomy or nasal continuous positive airway pressure may alleviate obstructive airway disease.
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
All the above studies have provided theoretical support and application basis for the clinical treatment of sensorineural deafness. Because one of the main clinical manifestations of sensorineural deafness is the lesion and structural destruction of the basement membrane, these mainly show themselves in the hardening of the basement membrane, local damage and deletion of the basement membrane structure, thickening of the structure, and changes in additional quality. Because of the deep part of the temporal bone and the fine structure of the internal tissue, it is difficult to diagnose cochlear lesions clinically. In view of this, this paper will establish a FE numerical model of the whole ear to computationally simulate the changes in the dynamic biological behavior of the middle ear structure caused by the lesions in the BM of the cochlea and the lesions in the inner cochlea can be diagnosed through the feedback of the specific abnormal information in the middle ear.
Post-lingual deaf adult cochlear implant users' speech and voice characteristics: cochlear implant turned-on versus turned-off
Published in Acta Oto-Laryngologica, 2021
Peyman Zamani, Arash Bayat, Nader Saki, Elahe Ataee, Hossein Bagheripour
Cochlear implants (CIs) provide the perception of sound through the conversion of auditory stimuli into electrical impulses, which are received by the auditory nerve and processed by the central auditory system. As a rising number of post-lingually deafened subjects receive CIs, there has been an increasing interest in the influence of CIs on speech characteristics [1]. Unlike pre-lingual deaf subjects, the post-lingual deaf individuals have been exposed to hearing experience and natural speech production. However, they may suffer from speech-related disorders due to the lack of control over provided by auditory feedback mechanisms. Actually, deafness could interfere with various aspects of verbal communication due to the lack of auditory feedback. It has been shown that auditory feedback cues have a crucial role for the CI users to monitor and fine-tune their speech articulation and to make purposeful adjustments in their voicing [2].
A narrative review of the logistic and economic feasibility of cochlear implants in lower-income countries
Published in Cochlear Implants International, 2021
Ellie Bodington, Shakeel R. Saeed, Michael C. F. Smith, Nigel G. Stocks, Robert P. Morse
One of the key difficulties in developing cochlear implant programmes is obtaining funding. In LMICs, most of the costs fall to the patient or their family, although sometimes health insurance and reimbursement schemes contribute to the costs (Jeyaraman, 2013; Liang and Mason, 2013). The potential to obtain additional funding from a government or charity is complicated by resource allocation: Many consider that the money required for cochlear implants could be better used for life-saving surgery, control of infectious diseases or health education (Fagan and Jacobs, 2009; Krishnamoorthy et al., 2014). For example, measles vaccination costs less than $1.50 per person (UNICEF, 2014). Similarly, the Haemophilus influenza B (Hib) vaccine can prevent some causes of meningitis, and costs less than $2.50 per person (Wolfson et al., 2008). Global use of such vaccination could therefore greatly reduce the incidence of the major causes of deafness. For a population with existing hearing loss, it can be argued that the provision of hearing aids, at a fraction of the cost of cochlear implants, provide greater quality-of-life benefits with limited funding. Additionally, some consider that use of cochlear implants diverts funds away from lower-cost resources that could potentially help more people with hearing loss, such as telecommunication devices (Power and Hyde, 1992).