Medical literature is replete with articles about the benefits of BERA, not merely as an auditory test, but also an aid for
evaluating the CNS function represented by the brainstem activity in several clinical conditions, such as in comatose
patients, bipolar disorders, attention deficit disorder, synapse dysfunctions and hydrocephalus. Irreversible
sensorineural hearing loss (SNHL) in infants, identified by a failed BERA, in addition to its being one of major clinical
features of intrinsic ear diseases, is also a sign of inborn disorders of white matter. Thus, a new dimension is attached
unto the benefits of a failed-BERA. We will high lighten the way towards identifying the exact etiology of irreversible
SNHL of non-intrinsicear-disease, in condition that other clinical features appear in conjunction with SNHL. To achieve
this, we reviewed all the diseases, syndromes and triads, at which a failed-BERA is one of their major components of the
clinical picture. We brought here a case report of an infant suffering from SNHL. Irreversible SNHL in childhood is one of
the major features in certain neurologic disorders, but not necessarily the first to appear. It is a main marker for the
diagnosis of Leukodystrophies, as well as Multiple Sclerosis. Otolaryngologists and clinicians in general should be
conscious while following-up certain infants who have irreversible SNHL of non-intrinsic-ear-disease. The appearance of
pendular nystagmus, truncal hypotonia, or intentional tremor with a staccato speech, in parallel with hyperreflexia, and
spastic paraplegia is the core of our upgraded flow chart of SNHL clinical approach.
Follow the link for full article
https://medwinpublishers.com/OOAJ/OOAJ16000174.pdf
Follow the link for full article
https://medwinpublishers.com/OOAJ/OOAJ16000174.pdf
