Her hearing pattern did not get worse along the years. She said she had not undergone any neonatal hearing screening test. She did not complain of tinnitus, vertigo, otalgia and otorrhea. R.N., 18 years, female, came to our service complaining of right side hearing loss, noticed about one year ago, of unknown onset. 4Īs treatment modalities, the cochlear implant offers very promising results, as well as hearing aids, which help patients recover their hearing. MRI can be used in order to see the structures that involve the VIII cranial nerve, which may be aplastic. 3Īmong complementary tests, Audiometry may show a sensorineural hearing loss that varies as to the degree of hearing loss (from mild to profound), depending on the level of nerve involvement.ĭiagnosis of certainty is made by CT scan, which shows a narrow acoustic meatus, thus pointing to the disorder. Otorhinolaryngological exam involves otoscopy, which is usually normal, and also tests that investigate the patency of the VIII cranial nerve's vestibular branch (Romberg, Untemberg, heat tests, nystagmus and others). The physician should investigate the patient's obstetric, natal and post-natal history in order to rule out malformations during this period. When there is facial nerve involvement, there may be paresthesia and even paralysis. 1Ĭlinical manifestations involve especially hypoacusis, there may also be tinnitus and vertigo in the side affected. 1, 2, 3Īn acquired bone disease may also result in stenosis of the internal acoustic meatus (osteomas, osteopetrosis, Paget's disease, and others). It is not uncommon to have a completely normal labyrinth, though. The labyrinth may be also involved, being aplastic or with a deformed or incomplete cavity. The embryologic events involved in fetal growth between the 4th and the 8th weeks are crucial for bone growth, and may cause such disease. The stenosis etiology may be explained as being secondary to an aplasia or hypoplasia of the vestibulocochlear nerve.
The major alteration happens due to a constriction caused by impaired bone growth, resulting in an abnormal internal acoustic meatus. In most cases, it happens as an isolated congenital manifestation, and other systemic abnormalities are rarely found. This loss may happen because of alterations in the VIII cranial nerve (vestibulococlear).
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