About 1 in 500 infants is born with or develops hearing loss. These can be prevented through immunization and good hygiene practices. Able to hear and repeat words spoken in normal voice at 1 metre. In contrast, syndromic hearing loss occurs with signs and symptoms affecting other parts of the body. A parents guide to genetics and hearing loss centers for. One common way is by the conditions pattern of inheritance. Genetic forms of hearing loss must be distinguished from acquired nongenetic causes of hearing loss. Hard of hearing is used to describe people with hearing loss ranging from. Delayed speech suggesting possible hearing loss can also be seen in young children with autism spectrum disorder or specific speech and language disorders.
The genetic forms of hearing loss are diagnosed by otologic, audiologic, and physical examination, family history, ancillary testing e. Conductive hearing loss results from obstruction or disease of the outer or middle ear that prevents transmission of sound energy to the inner ear. In developed countries approximately 80% of congenital hearing loss is due to genetic causes and the remainder to environmental acquired causes. Over 30% of childhood hearing loss is caused by diseases such as measles, mumps, rubella meningitis and ear infections. Hearing loss is much more frequent in children born of a consanguineous marriage 5, 3739. Types, causes and treatments of hearing loss the basics. Specifically, we address defects of the ribbon synapses between inner hair cells and spiral ganglion neurons that cause. Hereditary hearing loss and deafness may be conductive, sensorineural, or a combination of both. An interactive notebook for families with a young child who is deaf.
The purpose of the action plan on hearing loss report is to. Nonsyndromic hearing loss genetics home reference nih. Dominant hearing loss can be caused by only one copy of a mutated gene dominant hearing loss is seen in every generation if a parent has a dominant mutation, each child has a 50 % chance of inheriting it. Conductive hearing loss is due to problems with the ear canal, ear drum, or middle ear and its little bones the malleus, incus, and stapes. Action on hearing loss has a leaflet about bahas and similar hearing aids pdf, 360kb. Audiometric patterns of genetic nonsyndromal sensorineural hearing loss article pdf available in audiology. Review of hair cell synapse defects in sensorineural. Hearing aids communication verlag fur gesprachsforschung. This can occur as a result of hereditary factors as well as. Medical and technological advances have given the hearing loss popu lation as a. Some basics about hearing loss, hear ing technologies and. Acquired causes should be differentiated from genetic causes to inform the evaluation and required ancillary testing i.
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