What is a 'Mondini' and what difference does a name make?

The scientific report written in Latin by Carlo Mondini in 1791, titled The Anatomic Section of a Boy Born Deaf (1), has been often cited but seldom read. Recently, the American Journal of Otology published an English translation by Gordon J. Hartley of Rugby, England, with comments by Peter D. Phelps (2). Originals of Mondini’s publication known to exist in the United States are in the collections of the Library of Congress, Washington, D.C., Columbia University, New York City, and the Huntington Memorial Library, San Marino, California. It is clear from Hartley’s translation that the inner ear anomaly described by Mondini consisted of 1) a cochlea of one-and-one-half turns instead of the normal two-and-one-half turns, comprising a normal basal turn and a cystic apex in place of the distal one-and-one-half turns; 2) an enlarged vestibule with normal semicircular canals; and 3) an enlarged vestibular aqueduct containing a dilated endolymphatic sac. Thus, the cochlear anomaly was relatively mild, and would correspond to incomplete partition in the spectrum of congenital cochlear malformation because of an arrest of embryogenesis, as proposed by Jackler et al (3). Arrest at earlier stages in the spectrum include cochlear hypoplasia and common cavity and cochlear aplasia. Even earlier arrests result in otocyst and complete labyrinthine aplasia (Michel’s anomaly) (3). Mondini graphically supported his written description of the anatomic findings with drawings only (1, 2). Alexander is credited with first publishing, in 1904, a histologic section corresponding to the cochlear anomaly described by Mondini (4). In recent decades, multiple authors have published exquisite histologic sections firmly establishing the Mondini anomaly as an entity (5–8). Radiologically, the anomaly was recognized on polytomography by Jensen as early as 1969 (9). Subsequently, he also described a more severe type of deformity consisting of an amorphous cochlear sac continuous with a dilated vestibule, for which he suggested the term ‘‘dysplasia’’ (10). Unfortunately, the term ‘‘Mondini dysplasia’’ has come to mean virtually any congenital malformation of the osseous labyrinth detectable on radiographic examination (3). Inevitably, seemingly contradictory observations