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Mutations of  SLC26A4  are among the most prevalent causes of hereditary deafness. Deafness in the corresponding mouse model,  Slc26a4 −/−  , results from an abnormally enlarged cochlear lumen. The goal of this study was to determine whether the cochlear enlargement originates with defective cochlear fluid transport or with a malfunction of fluid transport in the connected compartments, which are the vestibular labyrinth and the endolymphatic sac. Embryonic inner ears from  Slc26a4 +/−   and  Slc26a4 −/−   mice were examined by confocal microscopy  ex vivo  or after 2 days of organ culture. Culture allowed observations of intact, ligated or partially resected inner ears. Cochlear lumen formation was found to begin at the base of the cochlea between embryonic day (E) 13.5 and 14.5. Enlargement was immediately evident in  Slc26a4 −/−   compared to  Slc26a4 +/−   mice. In  Slc26a4 +/−   and  Slc26a4 −/−   mice, separation of the cochlea from the vestibular labyrinth by ligation at E14.5 resulted in a reduced cochlear lumen. Resection of the endolymphatic sacs at E14.5 led to an enlarged cochlear lumen in  Slc26a4 +/−   mice but caused no further enlargement of the already enlarged cochlear lumen in  Slc26a4 −/−   mice. Ligation or resection performed later, at E17.5, did not alter the cochlea lumen. In conclusion, the data suggest that cochlear lumen formation is initiated by fluid secretion in the vestibular labyrinth and temporarily controlled by fluid absorption in the endolymphatic sac. Failure of fluid absorption in the endolymphatic sac due to lack of  Slc26a4  expression appears to initiate cochlear enlargement in mice, and possibly humans, lacking functional  Slc26a4  expression.

Failure of Fluid Absorption in the Endolymphatic Sac Initiates Cochlear Enlargement that Leads to Deafness in Mice Lacking Pendrin Expression