10 Serious Warning Signs Of A Biotinidase Deficiency

5. Various Skin Rashes

Various skin rashes may develop with biotinidase deficiency. In the early stages of this condition, patients may experience an eczematoid rash. These patches of eczema consist of red, itchy spots, and patients may have bumps that leak fluid and form a scab. Treatment for this type of rash normally involves moisturizers and steroid creams, and home remedies like oatmeal baths may help control itching. In addition to this rash, patients with biotinidase deficiency may develop fungal infections such as candidiasis, which often impacts the skin, nails, and mouth. Patients who notice any skin rash should be evaluated by a dermatologist to receive the most appropriate treatment.

6. Hearing Loss

A less commonly recognized but significant warning sign of Biotinidase Deficiency, especially in older children or those with partial deficiencies, is progressive hearing loss. This can range from mild to severe, often sensorineural, meaning it affects the inner ear or the nerve pathways to the brain. The precise mechanism linking biotin metabolism to hearing is still under research, but it underscores the vital role biotin plays in neurological function and cellular health. Early audiological testing is crucial for affected children, as prompt biotin supplementation can often prevent further hearing deterioration or even lead to some recovery.