SNHL of Childhood New Diagnostic Criteria. An Upgraded Clinical Approach to a ‘Failed’ BERA Test

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.