Mondini Deformity Clinical Presentation

Updated: Nov 24, 2021
  • Author: Adam E Singleton, MS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Most newborns with congenital sensorineural hearing loss have impaired hearing at birth and are usually identified with infant hearing screening. In persons with Mondini deformity, the variability of hearing acuity in individuals is wide, ranging from normal hearing to profound sensorineural hearing loss.

Of the 25 patients studied in their 1987 study, Jackler et al found that the average hearing level in patients with incomplete partition was 75 dB with a speech discrimination score of 44%. [6]


Physical Examination

In patients with congenital cochlear malformation, otoscopy findings are normal, with intact middle ear structures present behind the tympanic membrane.

Hearing evaluation

Using a 512-Hz tuning fork, the Weber test can be performed to assess gross hearing. In patients with Mondini deformity, sound should localize to the ear opposite to the congenitally malformed ear.