Synopsis of key/new findings
- Inner ear malformations may range from mild to severe with variable
degrees of hearing loss. Severely malformed cochlea with a normal
hearing is a rarity.
- Our first case was a three years old boy whose hearing was normal as
per various audiological tests and he was diagnosed to have autism.
Hence, unresponsiveness to sounds and poor speech development could
have been attributed to autism. However, further evaluation was
carried out and radiology revealed cochlear hypoplasia type IV. Thus,
radiology as a diagnostic tool must not be overlooked.
- The second patient had an apparently normal hearing till adolescence
which is hard to explain in the presence of Mondini dysplasia. A
recent onset, progressive fluctuating bilateral hearing loss led to
the patient seeking medical attention and the radiologic diagnosis of
left sided Mondini dysplasia.
- The number of turns and morphology of the cochlea may not be the only
determinants of the final hearing status of individuals with cochlear
malformations.
- Radiology and psychological evaluation may be invaluable adjuncts
aiding in the final diagnosis and should be resorted to in such cases
as described above.