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Auditory neuropathy spectrum disorder (ANSD) is a specific form of hearing loss defined by the presence of normal or near-normal otoacoustic emissions (OAEs) but the absence of normal middle ear reflexes and severely abnormal or completely absent auditory brainstem response (ABRs).
These SCDs induce SCD syndromes (SCDSs), which define specific sets of hearing and balance symptoms. [1] [2] This entry mainly deals with the superior SCDS. The superior semicircular canal dehiscence syndrome (SSCDS) is a set of hearing and balance symptoms that a rare disease/disorder of the inner ear's superior semicircular canal/duct induces.
Auditory neuropathy is sometimes difficult to catch right away, even with these precautions in place. Parental suspicion of a hearing loss is a trustworthy screening tool for hearing loss, too; if it is suspected, that is sufficient reason to seek a hearing evaluation from an audiologist. [10]
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.
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]
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.
Indications include unilateral hearing loss (85%), speech impediments, disequilibrium, tremors or other loss of motor control. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine angle that lies between the cerebellum and the pons .
Profound unilateral hearing loss is a specific type of hearing loss when one ear has no functional hearing ability (91 dB or greater hearing loss). People with profound unilateral hearing loss can only hear in monaural (mono). Profound unilateral hearing loss or single-sided deafness, SSD, makes hearing comprehension very difficult.