title: Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review.
abstract: BACKGROUND
  Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I).
  
  
  METHODS
  We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease.
  
  
  RESULTS
  There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms.
  
  
  CONCLUSIONS
  The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis.
authors.affiliation: Servicio de ORL, Complejo Hospitalario Insular Materno Infantil, Las Palmas, Gran Canaria, España. Electronic address: gloriaguerraj@gmail.com.
authors.forename: Gloria
authors.initials: G
authors.lastname: Guerra Jiménez
authors.affiliation: Servicio de ORL, Hospital Universitario Marqués de Valdecilla, Santander, España.
authors.forename: Ángel
authors.initials: Á
authors.lastname: Mazón Gutiérrez
authors.affiliation: Servicio de Radiología, Hospital Universitario Marqués de Valdecilla, Santander, España.
authors.forename: Enrique
authors.initials: E
authors.lastname: Marco de Lucas
authors.affiliation: Servicio de Radiología, Hospital Universitario Marqués de Valdecilla, Santander, España.
authors.forename: Natalia
authors.initials: N
authors.lastname: Valle San Román
authors.affiliation: Servicio de Neurocirugía, Hospital Universitario Marqués de Valdecilla, Santander, España.
authors.forename: Rubén
authors.initials: R
authors.lastname: Martín Laez
authors.affiliation: Servicio de ORL, Hospital Universitario Marqués de Valdecilla, Santander, España.
authors.forename: Carmelo
authors.initials: C
authors.lastname: Morales Angulo
mesh_terms: D000328:Adult; D000368:Aged; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D006261:Headache / Q000209:etiology; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D059906:Neuroimaging; D009759:Nystagmus, Pathologic / Q000209:etiology; D063189:Symptom Assessment; D013595:Syringomyelia / Q000150:complications / Q000175:diagnosis; D014012:Tinnitus / Q000209:etiology; D014717:Vertigo / Q000209:etiology
keywords: Acúfeno; Aural fullness; Chiari type I malformation; Dizziness; Hearing loss; Hipoacusia; Inestabilidad; Malformación de Chiari tipo i; Mareo; Plenitud ótica; Tinnitus; Unsteadiness; Vertigo