Giant posterior fossa cystic craniopharyngiomas presenting with hearing loss. Report of three cases and review of the literature.

BACKGROUND: Autopsy studies and recent reviews report that 4% of craniopharyngiomas have posterior fossa extension at initial operation, and 12% subsequently develop this extension during their course. However, only two patients in the literature have been shown to present with deafness, which preceded the more typical suprasellar signs and symptoms of increased intracranial pressure, endocrine disturbance, altered mentation, and visual deterioration.
METHODS: The authors report three cases of giant cystic posterior fossa craniopharyngioma initially presenting in childhood with either unilateral or bilateral deafness. These cases are presented, the literature on posterior fossa craniopharyngioma is reviewed, and staged operative management is discussed.
RESULTS: Despite being rarely reported, 10% to 20% of giant cystic craniopharyngiomas with posterior fossa extension at presentation may have unilateral or bilateral deafness as their first symptom. Deafness as a presenting symptom is much less common in patients presenting with recurrent tumors in this location than with primary tumors. Pterional or bifrontal craniotomy is appropriate for management of the parasellar component and should be undertaken first when visual symptoms are present. Suboccipital craniectomy is appropriate for management of the posterior fossa component and should be undertaken first when brain stem compressive symptoms make it necessary. Skull-base techniques may have value in certain settings, but patient's morbidity when using these techniques must be carefully considered.
CONCLUSIONS: Our results indicate that staged operations with the goal of achieving gross total resection can yield excellent results. Adjuvant radiation is indicated for those with residual tumor seen on magnetic resonance imaging (MRI), but in young children without residual tumors by MRI, we prefer to follow carefully with serial scans. Since the loss of hearing in children can compromise language development, we suggest referral of these children to a specialist in language rehabilitation.