Unilateral hearing loss as primary symptom of craniopharyngioma in a six-year-old girl.

OBJECTIVE

We report a rare case of otological presentation of craniopharyngioma.

METHOD

Case report and review of world literature concerning presentations of craniopharyngioma.

RESULTS

A six-year-old girl was referred to our department with unilateral hearing loss. This appeared to be a complete sensorineural hearing loss. Otoacoustic emissions were reproducible on both sides. Magnetic resonance scanning revealed a massive, cystic craniopharyngioma exerting pressure on the patient's ventricular system and brainstem and also invading the internal acoustic canal. The mass was resected via a craniotomy. The patient's hearing loss completely recovered, and she experienced no neurological or endocrinological side effects of the treatment. Craniopharyngioma have a prevalence of 0.13-2:100,000.

CONCLUSION

Craniopharyngioma is a rare disease. First presentation with otological symptoms is extremely rare. Otoacoustic emissions can differentiate between cochlear and retrocochlear causes of sensorineural hearing loss.