A 38-year-old male with hypertension and sensorineural hearing loss due to low visual acuity (VA).
In the family history, three deaf-mute male siblings and one with low vision were recorded.
Visual acuity (VA) without correction was 0.3 in the right eye (OD) and 0.2 in the left eye (LE).
An anterior lenticonus was found and in the eye fundus the existence of an MA was evidenced in both eyes, by a perimacular ring of white spots surrounded detected.
Optical coherence tomography (OCT) was performed with Stratus OCT-3 (Carl Zeiss Meditec, Dublin, CA), which verified the presence of AM, in stage IV, with basal diameter of 1347μm in dementia
After systemic study (including renal biopsy), the patient was diagnosed with AS, currently on hemodialysis for severe renal failure, maintaining the characteristics of the AM.
