Auditory findings and electrophysiologics in individuals with G/BBB syndrome.

Autor:	Cassab TV; Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru, São Paulo, Brasil., Zanchetta S, Giacheti CM, Zorzetto NL, Richieri-Costa A
Jazyk:	English; Portuguese
Zdroj:	Brazilian journal of otorhinolaryngology [Braz J Otorhinolaryngol] 2011 Nov-Dec; Vol. 77 (6), pp. 768-74.
Abstrakt:	Unlabelled: 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. Aim: 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). Model: 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. Conclusion: 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.
Databáze:	MEDLINE
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