Abstract
Children (28) with deafness or severe perceptive hearing impairment were examined. They were divided into 2 groups for symmetrical longitudinal planigraphy of the ear, which proved a labyrinthine aplasia. These findings were compared with the audiometric findings. A definite diagnosis was possible in small children. Histological findings on congenital anomalies of the inner ear with changes in the cochlea may also be divided into 2 groups: complete absence of development of inner ear with labyrinth aplasia; and greater or lesser aplasia in the bony part of the cochlea with 2 subgroups, a classic type after Mondini and a partial type after Mondini.

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