Poor word discrimination is the fancy way to say she has trouble understanding speech even if the volume of the voice is loud enough. You may ask, “What good is the hearing aid if it can't help her understand me?" For her to be able to try and understand your speech she first has to be able to hear it. A hearing aid will help this. It cannot restore or repair a poor ability to understand as this usually has more to do with the way the sound travels through the hearing system and particularly up the nerve pathways to the brain than the amount of sound delivered at the eardrum. The brain can only try to understand if the ear hears it in the first place. It will take her brain a good six weeks to six months to learn to hear as well as it can through new hearing aids. Some people need a moment to process what was just said, so slowing down your speech and speaking to them in a quiet room will help. Try to reduce extraneous noises such as the television, radio or other conversations. You may also try gaining her attention like saying her name or tapping her on the shoulder before you start speaking. Some people can hear and understand in background noise better than others. There are speech in noise tests which can tell us who needs the most help in background noise. More general advice is to get hearing aids when your hearing loss is mild to moderate instead of waiting until the loss is severe as in general the earlier you obtain hearing aids the better. 

Auditory neuropathy was once thought to be rare but it may be quite common and account for up to 10% of childhood cases of hearing loss, and can also be seen in older children and adults. It is an unusual cause of hearing loss. The understanding of how it occurs is still changing and is a source of some controversy amongst hearing specialists, as is its diagnosis and treatment. The most common progressive and severe hearing losses involve hair cell failure within the cochlea while leaving the auditory nerve intact and working well. This is why a cochlear implant usually works so well for most profound hearing losses. It stimulates a nerve which is still functioning. In Auditory Neuropathy Spectrum Disorder (ANSD) some of the hair cells (or at least the outer hair cells) may be functioning well but inner hair cells and/or the junction with the auditory nerve (the synapse) along with the auditory nerve itself seems to have failed. ANSD seems to occur in several clusters of patients. One cluster involves infants who are born preterm and spend some time in the neonatal intensive care. They may have been affected by some degree of birth trauma (hypoxia), high levels of jaundice and exposure to a variety of medications during their treatment. A second group generally develops later onset symptoms during childhood, adolescence or early adulthood. This group (the minority) often have associated neurological disorders with neuropathy affecting other parts of the body. Disorders such as Charcot- Marie-Tooth disease fit this category. Genetic defects with failure of the otoferlin gene, which is required for normal inner hair cell function, may also be underlying factors. As one can imagine, the diagnosis of such a variable and complex condition requires several sophisticated hearing test. This involves an audiogram (and speech perception test in older children or adults), otoacoustic emission studies (a test of outer hair cell function) and an auditory brain stem response test (ABR). The classical test results in ANSD are a variable degree of hearing loss on the audiogram, but poor speech discrimination, particularly in background noise; poor or absent ABR wave forms and intact otoacoustic emission. The problem with ANSD is that the outcomes of children and adults with the disorder is highly variable. The long term outcomes range from normal hearing, speech and language development, to such disordered hearing that a cochlear implant is considered. The response to hearing aids and cochlear implantation is also highly variable. At this stage of our understanding, patients with disorders limited to the cochlea (hair cell failure) may have good outcomes, while those involving poor auditory nerve function have outcomes which are more varied and difficult to predict. Much work is being done in furthering the understanding and diagnosis of ANSD as well as more strongly predicting the outcomes of treatments. 

Universal newborn hearing screening (UNHS) is very successful in detecting sensory neural hearing loss at birth. However, some hearing losses can develop in the first few months or years of life. The success of 'pass' screening at birth can lead to a false sense of security with parents and professionals alike – it is important for parents to be vigilant and if they suspect their infant has developed hearing loss they should investigate with a professional. 

Otosclerosis is one of the most common causes of acquired hearing loss. The incidence of otosclerosis of 2.5% seems to be the same for most populations and with no difference between the sexes. However, the incidence of clinical otosclerosis (causing hearing impairment that requires stapedectomy surgery, a hearing aid or in very extreme cases, a cochlear implant) is much lower and differs between different ethnic groups, races and the genders. It is rare in Africans, Orientals and South Americans, and more frequent in populations of European origin where the prevalence is about 0.4%. It is more frequent in women with a female to male ratio of 2:1. In about 85% of people with clinical otosclerosis the hearing loss is bilateral with the remainder having hearing loss in only one ear. The familial nature of clinical otosclerosis was first reported in 1861. A twin study reported in 1966 found the presence of clinical otosclerosis in nearly all 40 pairs of identical twins, supporting the early hypotheses that otosclerosis had a genetic basis. Numerous studies on families with otosclerosis suggested that the pattern of inherence was autosomal dominant (meaning you only need to get the abnormal gene from one parent in order for you to inherit the condition). But with the proportion of individuals carrying the abnormal gene ranging from 25-40% the impression was given that hearing loss in otosclerotic families might skip a generation. Although a strong family history exists in many patients with otosclerotic hearing loss, about 40-50% of cases are sporadic with no family history. When all past and new data concerning the occurrence of clinical otosclerosis is examined, it seems like the genetic basis of the disease is complex and the expression of the associated hearing loss is dependent on multiple factors including those in the environment (oestrogens, fluoride, viral infections). Studies are underway to shed light on the genetic base diseases that are associated with hearing loss and the various forms of inheritance that lead to both histological and clinical otosclerosis. Hopefully this will allow individuals, families and their treating doctors to further understand the risk to both this generation and their children of developing otosclerotic hearing loss in one or both of their ears. 


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