Air-bone gap in patients with X-linked stapes gusher syndrome

It is often possible to elicit the stapedius reflex in patients suffering from recessive X-linked progressive mixed deafness syndrome with stapes gusher. The presence of an air-bone gap in the audiogram and the ability to elicit the stapedius reflex are conflicting. Measurements were performed on two patients who were suffering from this syndrome, to establish whether the hearing loss was of the mixed or purely sensorineural type. It was argued that, owing to congenital malformations, the audiovestibular system might act as a more than normally efficient transducer, to convert skull vibrations into inner ear fluid motions, leading to bone conduction thresholds that are better than expected. The results of tone and speech audiometry, stapedius reflex measurements, and brainstem evoked response audiometry in this study showed a pattern similar to that generally seen in patients with purely sensorineural hearing loss. This supports the hypothesis that the air-bone gap in the audiogram does not have the usual significance of a conductive hearing loss component.