Cochlear and Neural Diseases: Classification and Oto-Audiologic Correlations

Abstract

An extended clinical classification of cochlear and neural disease is presented along with an anatomical definition of corresponding cochlear and neural compartments. The clinical classification has three categories: prenatal, neonatal, and delayed, with each subdivided into genetic and acquired groups. The cochlear and neural compartments are arbitrarily divided at the habenula perforata with the unmyelinated dendrites considered part of the cochlear compartment. This allows a clinical reconciliation of cochlear and neural disease with oto-audiologic and pathologic findings. Patients are presented with Alport's syndrome, meningitis and obliterative labyrinthitis, mumps, diabetes and sickle cell disease to illustrate the clinical classification. Pathologic correlations are postulated in each of the patients.