Borderline Cochlear Implant Candidate with EVA
Alan was diagnosed at 2 years 8 months of age with an asymmetrical, mild dropping to a profound sensorineural hearing loss in his right ear, and a moderately-severe sloping to a profound sensorineural hearing loss in his left ear. The hearing loss was believed to be progressive, secondary to bilateral enlarged vestibular aqueducts (EVA), as observed in a CT scan.
Screening for genetic mutations indicated that Alan carries a single mutation of the SLC26A4 gene, the most common cause of EVA–associated with Pendred syndrome. Testing for Connexin 26 and 30 mutations was negative.
Alan passed newborn hearing screening at birth in the right ear, and was “referred” in the left ear. At one month of age, auditory brainstem response (ABR) testing showed the right ear was fine, but the left ear had a possible moderate hearing loss. A second ABR at 2 months of age found both ears to be within normal limits.