Hearing Summary

Conductive hearing loss is a common occurrence in individuals with Kabuki syndrome, usually due to repeated ear infections during childhood. Cleft palatei, abnormal development of the Eustachian tube and immunity deficiencies can all be contributing factors. Hearing loss in Kabuki syndrome can be of three basic types: conductive, sensorineural or mixed

Conductive hearing loss occurs when sound is not conducted properly through the outer ear, middle ear, or both, such as in ear canal obstruction or in acute otitisi media (ear infection). It is generally a mild to moderate impairment because sound can still be detected by the inner ear. Generally, with pure conductive hearing loss, the quality of hearing (speech discrimination) is good, as long as the sound is amplified loud enough to be easily heard. This type of hearing impairment can often be medically or surgically treated.

Jackson_2Sensorineural hearing loss is due to the damage of the inner ear, the cochleai, or to the impairment of the auditory nerve. It can be mild, moderate, severe, or profound, to the point of total deafness. It is a permanent loss and it doesn’t only affect sound intensity such as the ability to hear faint sounds but also makes it more difficult for you to recognise complex sounds, to understand speech and to hear clearly.

Mixed hearing loss - In some cases, such as in complication of recurrent/chronic otitis media, a conductive hearing loss occurs in combination with damage of the inner ear or of the auditory nerve. When this occurs the hearing loss is referred to as a mixed hearing loss.

Conductive hearing loss, mainly due to recurrent otitis media, is reported with a frequency ranging from 24% to 82%. In fact chronic otitis media is extremely frequent in individuals with Kabuki syndrome during childhood. It is probably related either to cleft palate and abnormal development of the Eustachian tube or to immune deficiency. It has to be cured in order to limit permanent hearing loss sequelae (mixed hearing loss).

Sensorineural hearing loss is very rare in Kabuki syndrome. Only a few cases are reported in the literature and are mainly caused by anomalies of the inner ear, however this low prevalence could also be due to incomplete neuro-radiological investigations (CT brain) reported up to now in the medical literature.

Some children may utilize a personal or classroom soundfield FM system, either in conjunction with aids or without. The FM system enhances the distance to noise ratio, in the typical classroom, so that environmental/background noise is decreased while the voice of the speaker is amplified.

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