There is a very nice article on this very uncommon malformation in AJNR 2009; 30: 113. We have only seen it in patients being screened for congenital sensorineural hearing loss. In these patients, its severity varies. In the above mentioned article, 4 of 6 patients had cranial nerve VIII abnormalities. I would be interested in learning from other neuroradiologists who are familar with the malformation what their experience has been.
Regarding Pontine Tegmental Cap Dysplasia
Dear Dr Castillo,
We recently have reported a case of Pontine Tegmental Cap Dysplasia in a child developmental delay, cranial nerve neuropathy including sensorineural hearing loss. We performed a succinct review of the MRI depicted cranial nerve defects in all previously published cases. J Neuroimaging. 2015 Feb 17. doi: 10.1111/jon.12209. We would be happy to discuss this fascinating condition further.