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Related Experiment Video

Updated: May 27, 2025

In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears

In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears

Published on: February 21, 2018

Norrie Disease: Cochlear Enhancement and Cerebellar Signal Abnormalities.

Kevin Ferenchak¹, Julie B Guerin², Asra Nayab³

¹From the Department of Ophthalmology (K.F, L.A.D., B.A.S), Mayo Clinic, Rochester, Minnesota.

AJNR. American Journal of Neuroradiology

|February 18, 2025

Summary

Norrie disease (ND) causes blindness and hearing loss. New MRI findings show brain and inner ear abnormalities in infants with ND, which can mimic infections but are not necessarily signs of central nervous system infection.

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Last Updated: May 27, 2025

In Vivo Morphometric Analysis of Human Cranial Nerves Using Magnetic Resonance Imaging in Menière's Disease Ears and Normal Hearing Ears

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Published on: February 21, 2018

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Published on: May 10, 2019

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

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Published on: January 25, 2016

Area of Science:

Genetics
Neurology
Ophthalmology

Background:

Norrie disease (ND) is a rare X-linked disorder causing childhood blindness and later sensorineural hearing loss.
Ocular manifestations include retinal detachments, anterior segment dysgenesis, buphthalmos, and microphthalmia.
Intracranial findings in ND have not been extensively documented.

Purpose of the Study:

To report novel MRI findings in two infants with genetically confirmed Norrie disease.
To highlight that these intracranial findings can mimic central nervous system infections.
To increase clinical awareness of Norrie disease in the differential diagnosis of such imaging abnormalities.

Main Methods:

Genetic confirmation of Norrie disease in two male infants.
Ophthalmic examination to document ocular abnormalities.
Cranial MRI to assess intracranial and inner ear structures.

Main Results:

Infants presented with characteristic ocular abnormalities of Norrie disease.
MRI revealed enhancement of cranial nerves, cochleae, and cerebellum with restricted diffusion in the cerebellum.
These intracranial findings mimicked meningitis, labyrinthitis, and cerebellitis, despite the absence of clinical infection.

Conclusions:

Labyrinthine and cerebellar signal abnormalities are previously unreported findings in Norrie disease.
Clinicians should consider Norrie disease in infants with these specific MRI findings.
The described intracranial features do not necessarily indicate central nervous system infection and warrant careful interpretation in the context of Norrie disease.