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Deafness
Auditory neuropathy/ auditory dys-syncrony
A form of deafness that has attracted interest of audiologists and clinicians more recently is Auditory Neuropathy/ Dys-synchrony. This is a condition where there is often a mis-match between objective tests of hearing and the behavioural tests. This group belongs to the “neural” component of snesorineural deafness and is estimated to be up to 10% of all SNHL. The dys-synchronous firing of the hearing nerve fibres can be due to a number of reasons including dysfunction of the (1) inner hair cells, (2) synapse (the junction between the IHC and the hearing nerve), (3) hearing nerve itself, (4) VIIIth nerve nucleus in the brainstem and (5) brainstem pathways themselves. The pathology / aetiology may vary from genetic (otoferlin mutation that leads to absent or malfunctioning IHC) and, jaundice and hypoxia at birth, to delayed maturation of the hearing pathway. Diagnosis of this condition entails specific testing and multidisciplinary assessments with management requiring an experienced team.
Some of the aetiological investigations need to be carried out within the first few months of life and others repeated later to maximise the potential for arriving at a diagnosis. Amongst the causes of congenital hearing loss are: maternal infections such as Rubella which is now very rare because of immunisation; cytomegalovirus which is probably the commonest infective aetiology that can cause a progressive hearing loss; prematurity; severe lack of oxygen to the fetus; severe jaundice; some drugs that are harmful to inner ears; and genetic inheritance.
Glue ear
| How is it diagnosed? 
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