GIANT POSTERIOR-FOSSA CYSTIC CRANIOPHARYNGIOMAS PRESENTING WITH HEARING-LOSS - REPORT OF 3 CASES AND REVIEW OF THE LITERATURE

BACKGROUND Autopsy studies and recent reviews report that 4% of cranio pharyngiomas 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 de af ness, which preceded the more typical suprasellar signs and symptom s of increased intracranial pressure, endocrine disturbance, altered m entation, and visual deterioration. METHODS The authors report three c ases of giant cystic posterior fossa craniopharyngioma initially prese nting in childhood with either unilateral or bilateral deafness. These cases are presented, the literature on posterior fossa craniopharyngi oma is reviewed, and staged operative management is discussed. RESULTS Despite being rarely reported, 10% to 20% of giant cystic craniophary ngiomas with posterior fossa extension at presentation may have unilat eral or bilateral deafness as their first symptom. Deafness as a prese nting symptom is much less common in patients presenting with recurren t tumors in this location than with primary tumors. Pterional or bifro ntal craniotomy is appropriate for management of the parasellar compon ent and should be undertaken first when visual symptoms are present. S uboccipital craniectomy is appropriate for management of the posterior fossa component and should be undertaken first when brain stem compre ssive symptoms make it necessary. Skull-base techniques may have value in certain settings, but patients' morbidity when using these techniq ues must be carefully considered. CONCLUSIONS Our results indicate tha t staged operations with the goal of achieving gross total resection c an yield excellent results. Adjuvant radiation is indicated for those with residual tumor seen on magnetic resonance imaging (MRI), but in y oung children without residual tumors by MRI, we prefer to follow care fully with serial scans. Since the loss of hearing in children can com promise language develop ment, we suggest referral of these children t o a specialist in language rehabilitation. (C) 1997 by Elsevier Scienc e Inc.