Auditory Findings and Electrophysiologics in Individuals with G/BBB Syndrome

Overview

Journal Braz J Otorhinolaryngol

Specialty Otorhinolaryngology

Date 2011 Dec 21

PMID 22183284

Citations 1

Authors

Tatiana Vialogo Cassab

Sthella Zanchetta

Celia Maria Giacheti

Neivo Luiz Zorzetto

Antonio Richieri-Costa

Affiliations

Soon will be listed here.

Abstract

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.

Citing Articles

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