title: [Neurofibromatosis type 2 and its head and neck manifestations: literature review and population study in the Community of Cantabria and the province of Las Palmas].
abstract: OBJECTIVE
  Neurofibromatosis type 2 (NF2) is an infrequent autosomal dominant disease characterised by the appearance of viii nerve schwannomas, meningiomas and ocular abnormalities. Incidence of 1:25,000 and prevalence above 1:80,000 are estimated in general. The objectives of our study were to determine current prevalence of NF2 in the Community of Cantabria and the province of Las Palmas, and its head and neck manifestations.
  
  
  METHODS
  This was a population-based, retrospective study in 3 tertiary hospitals.
  
  
  RESULTS
  The study population showed prevalence of 1:600,000 in the Community of Cantabria and 1:280,000 in the province of Las Palmas. The most frequently diagnosed tumour was acoustic neuroma (n=15), followed by trigeminal neurinoma (n=2) and vagus (n=1).
  
  
  CONCLUSIONS
  Cases of NF2 are infrequent in Cantabria and Las Palmas, lower than that reported in the literature. The most frequently described head and neck tumour in the literature is acoustic neuroma, followed by schwannoma of cranial nerves v and x. Other tumours such as nasal, laryngeal, chorda tympani or cranial nerve vii schwannomas are also described. The most frequent ENT manifestation is hearing loss, especially unilateral, followed by cervical mass, tinnitus and headache. Early diagnosis and multidisciplinary management in specialised centres could improve life expectancy and quality of life for these patients.
authors.affiliation: Servicio de Otorrinolaringología, Complejo Hospitalario Insular Materno Infantil (CHUIMI), Las Palmas de Gran Canaria, España. Electronic address: gloriaguerraj@gmail.com.
authors.forename: Gloria
authors.initials: G
authors.lastname: Guerra-Jiménez
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario de Gran Canaria Dr. Negrín (HUGCDN), Las Palmas de Gran Canaria, España.
authors.forename: Pilar
authors.initials: P
authors.lastname: Camargo Camacho
authors.affiliation: Servicio de Otorrinolaringología, Complejo Hospitalario Insular Materno Infantil (CHUIMI), Las Palmas de Gran Canaria, España.
authors.forename: Ángel
authors.initials: Á
authors.lastname: Ramos-Macías
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario Marqués de Valdecilla (HUMV), Santander, España.
authors.forename: Carmelo
authors.initials: C
authors.lastname: Morales Angulo
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006258:Head and Neck Neoplasms / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D016518:Neurofibromatosis 2 / Q000150:complications*; D010038:Otorhinolaryngologic Diseases / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology
keywords: Cabeza y cuello; Ear, nose and throat manifestations; Head and neck; Manifestaciones otorrinolaringológicas; Neurofibromatosis tipo ii; Neurofibromatosis type 2