Evaluation of some 200 endolymph proteins indicates that they are predomina
ntly derived from plasma. However, the profile of endolymph proteins is rem
arkably similar to that of perilymph and entirely different from that of pl
asma. This supports the current consensus that perilymph rather than plasma
is the (direct) source of endolymph. Although the levels of total protein
of endolymph is extremely low, a few plasma-derived proteins, such as apoli
poproteins J and D, are selectively enriched, conceivably for protection of
cell membranes bounding the endolymphatic space. A small number of endolym
ph proteins, mostly glycosylated ones, are continually secreted into the en
dolymph by specialized epithelial cells, primarily for the maintenance of t
he structural and functional integrity of the extracellular superstructures
comprising tectorial membrane, otoconial complex (membrane) and cupula. Th
ese complex macromolecules cannot be eliminated in the periphery of the com
partment, but are transported to the endolymphatic sac for elimination. Imp
aired clearance of these negatively charged macromolecules by a dysfunction
al endolymphatic sac will contribute to the chemical imbalance of endolymph
which accompanies long-standing endolymphatic hydrops, and may be one of t
he reasons for the observed loss of function.