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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.
Mohr–Tranebjærg syndrome (MTS) is a rare X-linked recessive syndrome also known as deafness–dystonia syndrome and caused by mutation in the TIMM8A gene. It is characterized by clinical manifestations commencing with early childhood onset hearing loss, followed by adolescent onset progressive dystonia or ataxia, visual impairment from early adulthood onwards and dementia from the 4th ...
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 ...
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]
Hearing loss due to noise may be temporary, called a 'temporary threshold shift', a reduced sensitivity to sound over a wide frequency range resulting from exposure to a brief but very loud noise like a gunshot, firecracker, jet engine, jackhammer, etc. or exposure to loud sound over a few hours such as during a pop concert or nightclub session ...
Hearing loss completely recovers in around 35–39% of patients with SSNHL, usually within one to two weeks from onset. [34] Steroid treatment within seven days, a lower initial severity of hearing loss, the absence of vertigo, younger patient age, and a history of cardiovascular disease are all associated with complete hearing recovery. [35]
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Autoimmune inner ear disease (AIED) was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing. [2] The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system.