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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 ...
However, some children with severe to profound hearing loss may not be able to hear enough sound, even with a hearing aid, to make speech audible. A behind-the-ear hearing aid is often recommended for young children because it is safer and more easily fitted and adjusted as the child grows as compared to one that fits within the ear.
The hearing loss typically worsens over the years, and progression can be step-wise and related to minor head trauma. In some cases, language development worsens after head injury , demonstrating that the inner ear is sensitive to trauma in Pendred syndrome; this is as a consequence of the widened vestibular aqueducts usual in this syndrome. [ 4 ]
Movement disorder that can involve excessive eye blinking (blepharospasm) with involuntary movements of the jaw muscles, lips, and tongue (oromandibular dystonia). Ménière's disease Inner ear disorder that can affect both hearing and balance. It can cause episodes of vertigo, hearing loss, tinnitus, and the sensation of fullness in the ear.
CHARGE syndrome (formerly known as CHARGE association) is a rare syndrome caused by a genetic disorder.First described in 1979, the acronym "CHARGE" came into use for newborn children with the congenital features of coloboma of the eye, heart defects, atresia of the nasal choanae, restricted growth or development, genital or urinary abnormalities, and ear abnormalities and deafness. [1]
The hearing loss is generally bilateral with a conductive loss of about 50–70 dB. Even in cases with normal auricles and open external auditory canals, the ossicular chain is often malformed. [41] Attempts to surgically reconstruct the external auditory canal and improve hearing in children with TCS have not yielded positive results. [42]
AIED is used to describe any disorder in which the inner ear is damaged as a result of an autoimmune response. [3] Some examples of autoimmune disorders that have presented with AIED are Cogan's syndrome, relapsing polychondritis, systemic lupus erythematosus, granulomatosis with polyangiitis, polyarteritis nodosa, Sjogren's syndrome, and Lyme ...
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.