Relationship of Pathology to Function in Congenital Hearing Loss: I. The Pathology of Congenital Hearing Loss

Abstract

New sources of information on the pathology of congenital hearing loss allow the otolaryngologist to evaluate more confidently the number of functional elements present or absent in a given hearing loss. Information is obtained from two sources: histo-pathological studies on temporal bones of deceased hearing-impaired children, and polytomogram studies on living children. These sources reveal that varieties and modifications must be added to the classic pathologies described by Mïchel, Mondini, Scheibe, and Alexander, necessitating changes in our estimates of the hearing function of hearing impaired children. Variations occur in recessive and dominant genetic hearing loss, in viral labyrinthitis, in chromosomal abnormalities, and in iatrogenic hearing loss. Temporal bone slides and polytomograms of these entities are shown and their functional elements described.

Key Words:

• Auditory genetics
• Recessive hereditary hearing loss
• Dominant hereditary hearing loss
• Viral labyrinthitis
• Chromosomal abnormalities
• Iatrogenic hearing loss