A months old baby present...

A 7 months old baby presented with the complaints of progressive abdominal distension and poor feeding for 2 months. Upon further enquiry and clinical examination, the following findings were noted :- History - First born child of non consanguineous marriage, term institutional delivery without any history of NICU admission. History of top feeding present. Earlier the baby was sucking better and could roll over but now the baby is unable to roll over. Developmental Milestones - Gross Motor - Unable to sit Fine Motor - Bidextrous reach Language - Coos No Coarse Facies • P/A - Firm Hepato-Splenomegaly ( 4cm,5cm respectively) • Resp - B/L air entry present, no added sounds • CNS - Decreased tone and power in all 4 limbs, AF @ Level • CVS - S1 + S2 present, no murmurs Workup - TORCH profile - Negative Ophthalmology - Cherry Red Spot Thyroid Profile - Normal ENT - Bilateral Severe to Profound hearing loss Kindly suggest an appropriate diagnosis and treatment.