In your case, the deafness and tinnitus are permanent, I suspect. I know this will sound like a cop-out, but if you will be patient, over time, your brain's hearing center will adapt to the tinnitus and you will no longer be bothered by it as you have been. These acids can build up in the bloodstream and cause weakness, lethargy, low blood sugar, feeding difficulties, convulsions and death within the first few weeks of life. As you mentioned, early diagnosis is essential. The disease is usually first suspected because of a maple-syrup odor to the urine.
Treatment consists of avoiding the three amino acids and taking niacin, a vitamin.
I agree that early blood and urine testing of all newborns would be valuable, such as is now done with another amino-acid disorder called phenylketonuria. My doctor has employed a physician's assistant to help out. I refuse to see her. If my doctor has so many patients that he can't see us all, he ought to cut back and not overbook. I think that you are judging the P.A. too harshly.