title: Unilateral hearing loss as primary symptom of craniopharyngioma in a six-year-old girl.
abstract: OBJECTIVE
  We report a rare case of otological presentation of craniopharyngioma.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Otorhinolaryngology, Isala Klinieken Zwolle, Zwolle, The Netherlands. r.hofman@kno.umcg.nl
authors.forename: R
authors.initials: R
authors.lastname: Hofman
authors.forename: H J
authors.initials: HJ
authors.lastname: Rosingh
mesh_terms: D002648:Child; D003397:Craniopharyngioma / Q000150:complications*; D003399:Craniotomy / Q000379:methods; D005260:Female; D046088:Hearing Loss, Unilateral / Q000209:etiology*; D006801:Humans; D010911:Pituitary Neoplasms / Q000150:complications*; D001405:Reflex, Babinski / Q000188:drug therapy; D016896:Treatment Outcome