Cochlear Nerve Deficiency and Brain Abnormalities in Pediatric Patients.

Area of Science:

• Pediatric neurology
• Neuroimaging
• Auditory disorders

Background:

• Cochlear nerve deficiency (CND) prevalence is rising with advanced MRI techniques.
• CND presents in varying degrees, from aplasia to hypoplasia, with unclear etiology.
• CND may be linked to other intracranial abnormalities, necessitating early detection for hearing loss management.

Purpose of the Study:

• To investigate intracranial abnormalities in pediatric CND patients.
• To identify associated cranial nerve anomalies.
• To characterize auditory abilities in children with CND.

Main Methods:

• Retrospective chart review of 56 pediatric patient ears with CND.
• Study period: August 2006 to November 2014.
• Data collected at a tertiary care pediatric hospital.

Main Results:

• 27.6% of children had cochlear abnormalities; 48.9% had vestibular anomalies.
• Five patients had absent/abnormal facial nerves; two had aplastic olfactory nerves.
• Of 27 ears with absent cochlear nerves tested, 29.6% retained partial hearing.

Conclusions:

• MRI is the primary imaging modality for identifying CND and brain anomalies in children with sensorineural hearing loss.
• Vestibular and cochlear abnormalities are common in pediatric CND.
• Balance testing is recommended for most CND patients due to high incidence of vestibular malformations.