Hearing loss in biotinidase deficiency: genotype-phenotype correlation.

Sivri, Hatice Serap Kalkanoğlu; Genç, Gülsüm Aydan; Tokatli, Ayşegül; Dursun, Ali; Coşkun, Turgay; Aydin, Halil Ibrahim; Sennaroğlu, Levent; Belgin, Erol et al. · J Pediatr · 2007

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Abstract

Children with symptoms of profound biotinidase deficiency with null mutations are more likely to have hearing loss develop than those with missense mutations, even if not treated for a period of time. Hearing loss appears to be preventable in children with null mutations if treatment is initiated soon after birth.

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