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General hearing loss affects close to 10% of the global population. [42] In the United States alone, it is expected that 13.5 million Americans have sensorineural hearing loss. Of those with sensorineural hearing loss, approximately 50% are congenitally related.
Pendred syndrome is a genetic disorder leading to congenital bilateral (both sides) sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism (decreased thyroid gland function). There is no specific treatment, other than supportive measures for the hearing loss and thyroid hormone supplementation in case of hypothyroidism.
Low-frequency conductive hearing loss is present in many patients with SCDS and is explained by the dehiscence acting as a "third window." Vibrations entering the ear canal and middle ear are then abnormally diverted through the superior semicircular canal and up into the intracranial space where they become absorbed instead of being registered ...
A hearing test administered by a medical doctor, otolaryngologist (ENT) or audiologist including pure tone audiometry and speech recognition may be used to determine the extent and nature of hearing loss, and distinguish presbycusis from other kinds of hearing loss. Otoacoustic emissions and evoked response testing may be used to test for audio ...
Nonsyndromic deafness is hearing loss that is not associated with other signs and symptoms. In contrast, syndromic deafness involves hearing loss that occurs with abnormalities in other parts of the body. Nonsyndromic deafness constitutes 75% of all hearing loss cases, and an estimated 100 genes are thought to be linked to this condition.
Hearing loss is a partial or total inability to hear. [5] Hearing loss may be present at birth or acquired at any time afterwards. [6] [7] Hearing loss may occur in one or both ears. [2] In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. [8]
Published in 1994, this patient was monitored over the course of almost 20 years after exhibiting signs of hearing impairment as an infant. [4] Audiologic and related test results in concurrence with MRI confirmed bilateral absence of considerable portions of her temporal lobes resulting in cortical deafness.
In general, there are exceptional advantages to gain from an intervention to enable hearing in the microtic ear, especially in bilateral microtia. Children with untreated unilateral sensorineural hearing loss are more likely to have to repeat a grade in school and/or need supplemental services (e.g., FM system – see below) than their peers ...