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Background  In vertebrates, the inner ear is comprised of the cochlea and vestibular system, which develop from the otic vesicle. This process is regulated via inductive interactions from surrounding tissues.  Tbx1 , the gene responsible for velo-cardio-facial syndrome/DiGeorge syndrome in humans, is required for ear development in mice.  Tbx1  is expressed in the otic epithelium and adjacent periotic mesenchyme (POM), and both of these domains are required for inner ear formation. To study the function of  Tbx1  in the POM, we have conditionally inactivated  Tbx1  in the mesoderm while keeping expression in the otic vesicle intact.    Results  Conditional mutants ( TCre-KO ) displayed malformed inner ears, including a hypoplastic otic vesicle and a severely shortened cochlear duct, indicating that  Tbx1  expression in the POM is necessary for proper inner ear formation. Expression of the mesenchyme marker  Brn4  was also lost in the  TCre-KO .  Brn4  - ; Tbx1  +/- embryos displayed defects in growth of the distal cochlea. To identify a potential signal from the POM to the otic epithelium, expression of retinoic acid (RA) catabolizing genes was examined in both mutants.  Cyp26a1  expression was altered in the  TCre-KO , while  Cyp26c1  showed reduced expression in both  TCre-KO  and  Brn4  - ; Tbx1  +/-  embryos.    Conclusion  These results indicate that  Tbx1  expression in the POM regulates cochlear outgrowth potentially via control of local retinoic acid activity.

Tbx1 and Brn4regulate retinoic acid metabolic genes during cochlear morphogenesis