When Hearing Loss Is More Than Meets the Ear

A 32-year-old man presents with Hearing Loss and balance problems. MRI reveals bilateral tumors. Explore the hereditary condition linked to these findings.

Neurofibromatosis type 2


A 32-year-old man presented to the neurosurgery clinic with mild balance difficulties and hearing loss in the left ear. Evaluation of the patient’s hearing revealed profound sensorineural loss on the left side and normal hearing on the right side. Magnetic resonance imaging of the brain performed after the administration of contrast material revealed tumors in both internal acoustic canals (33 by 26 by 31 mm on the left side and 32 by 28 by 30 mm on the right side), with extension in the cerebellopontine angles and brainstem compression, representing bilateral vestibular schwannomas. What hereditary condition is associated with this presentation?

What is the most likely diagnosis?

Multiple endocrine neoplasia type 1
von Hippel-Lindau syndrome
Neurofibromatosis type 1
Li-Fraumeni syndrome
Neurofibromatosis type 2

Autosomal Dominant, Cerebellopontine Angle, Hearing Loss, Neurofibromatosis type 2, Vestibular Schwannoma

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