title: Auditory findings and electrophysiologics in individuals with G/BBB syndrome.
abstract: The G/BBB syndrome is a rare condition characterized by hypertelorism, cleft lip and palate, and hypospadias. No studies were found on the hearing of individuals with this syndrome.
  
  
  OBJECTIVE
  To investigate the auditory function in patients with G/BBB syndrome, such as the occurrence of hearing loss, and central and peripheral auditory nerve conduction.
  
  
  METHODS
  Fourteen male patients aged 7-34 years with the G/BBB syndrome were assessed by otoscopy, audiometry, tympanometry and evoked auditory brainstem response (ABR).
  
  
  METHODS
  A retrospective clinical series study.
  
  
  RESULTS
  Audiometric thresholds were normal in 12 (66.7%) of the sample and altered in two (33.3%), one with conductive and one with sensorineural loss. ABR results were: all patients had normal absolute wave I latencies; absolute wave III and V latencies were increased in two and six patients, respectively; interpeak latencies I-III, IV and V interpeak latencies were increased in four, three and eight patients respectively.
  
  
  CONCLUSIONS
  Hearing loss can occur in the G/BBB syndrome. There is evidence of central auditory pathway involvement in the brainstem. Imaging studies are needed to clarify the clinical findings.
authors.affiliation: Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, São Paulo, Brasil.
authors.forename: Tatiana Vialôgo
authors.initials: TV
authors.lastname: Cassab
authors.forename: Sthella
authors.initials: S
authors.lastname: Zanchetta
authors.forename: Célia Maria
authors.initials: CM
authors.lastname: Giacheti
authors.forename: Neivo Luiz
authors.initials: NL
authors.lastname: Zorzetto
authors.forename: Antonio
authors.initials: A
authors.lastname: Richieri-Costa
mesh_terms: D000293:Adolescent; D000328:Adult; D001299:Audiometry; D002648:Child; D004947:Esophagus / Q000002:abnormalities / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006314:Hearing Loss, Conductive / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D006801:Humans; D006972:Hypertelorism / Q000150:complications* / Q000503:physiopathology; D007021:Hypospadias / Q000150:complications* / Q000503:physiopathology; D008297:Male; D038781:Otoscopy; D011446:Prospective Studies; D055815:Young Adult