title: Prevention and management of hearing loss in syndromic craniosynostosis: A case series.
abstract: OBJECTIVE
  To assess the audiological profile in a cohort of children affected by syndromic craniosynostosis.
  
  
  METHODS
  Eleven children with Apert syndrome (n=4), Saethre-Chotzen syndrome (n=3), Muenke syndrome (n=2), Crouzon syndrome (n=1) and Pfeiffer syndrome type 1 (n=1) were submitted to a complete audiologic evaluation including otoscopy, pure-tone audiometry, tympanometry and acoustic reflex testing, ABR, otoacustic emissions, temporal bone High Resolution CT (HRCT) scan. The main outcome measures were prevalence, type and severity of hearing loss, prevalence of chronic otitis media, correlation with the time of first surgical correction.
  
  
  RESULTS
  Seven of 11 patients (64%) presented hearing loss (HL), conductive in 3/7 patients (43%) and mixed in 4/7 (57%). No patients showed a purely sensorineural HL. All hearing impaired patients displayed middle ear disorders: the patients with conductive HL had otitis media with effusion (OME) and 3/4 patients with mixed HL showed tympanic alterations or cholesteatoma. A bilateral vestibular aqueduct enlargement was detected by HRCT scan in one normal hearing patient. The ABRs resulted normal in all cases.
  
  
  CONCLUSIONS
  Our study confirms the high prevalence of otologic diseases in such patients. In contrast with previous studies, middle ear disorders were responsible for the hearing impairment also in patients with mixed HL due to secondary inner ear damage. These findings restate the necessity of a close audiologic follow-up. We did not detect the specific ABR abnormalities previously reported, possibly because of an early correction of the cranial vault malformations.
authors.affiliation: Department of Pediatrics, University of Torino, Torino, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Biamino
authors.affiliation: ENT, Department of Clinical Physiopathology, University of Torino, Torino, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Canale
authors.affiliation: ENT, Department of Clinical Physiopathology, University of Torino, Torino, Italy.
authors.forename: Michelangelo
authors.initials: M
authors.lastname: Lacilla
authors.affiliation: Department of Pediatrics, University of Torino, Torino, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Marinosci
authors.affiliation: ENT, Department of Clinical Physiopathology, University of Torino, Torino, Italy. Electronic address: federico.dagna@gmail.com.
authors.forename: Federico
authors.initials: F
authors.lastname: Dagna
authors.affiliation: Pediatric Neurosurgery Unit, Regina Margherita Children's Hospital, Torino, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Genitori
authors.affiliation: Neurosurgery Unit, Meyer Hospital, Firenze, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Peretta
authors.affiliation: Department of Pediatrics, University of Torino, Torino, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Silengo
authors.affiliation: ENT, Department of Clinical Physiopathology, University of Torino, Torino, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Albera
authors.affiliation: Department of Pediatrics, University of Torino, Torino, Italy.
authors.forename: Giovanni Battista
authors.initials: GB
authors.lastname: Ferrero
mesh_terms: D000168:Acrocephalosyndactylia / Q000150:complications*; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D015331:Cohort Studies; D003394:Craniofacial Dysostosis / Q000150:complications*; D003398:Craniosynostoses / Q000150:complications*; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D006320:Hearing Tests; D006801:Humans; D008297:Male; D010033:Otitis Media / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D038781:Otoscopy; D015995:Prevalence; D014057:Tomography, X-Ray Computed
keywords: ABR; Apert syndrome; Chronic otitis media; Craniosynostosis; Hearing loss