Question
Is there a difference between vestibular aqueduct syndrome and enlarged vestibular aqueduct syndrome? Please describe the medical and audiologic presentation that is most often related to this syndrome, diagnosis, and management/treatment options.
Answer
I believe the conditions you describe, ''vestibular aqueduct syndrome'' and ''enlarged vestibular aqueduct syndrome'', are one and the same. The syndrome is related to a congenital malformation of the inner ear (an enlarged channel from the endolymphatic sac - which is itself abnormally large - to the vestibular labyrinth). As such, presentation is usually in children and younger adults.
The typical clinical presentation of the enlarged vestibular aqueduct syndrome is progressive ''stepwise'' hearing loss, usually following a blow to the head. The blow need not be severe for the hearing loss to occur. I have seen bilateral presentations in children presenting with progressive hearing loss. Additionally, I recall a middle-aged woman with no prior history of hearing loss, who accidentally banged her head against her child's head during a snowball fight. She developed a unilateral hearing loss (she had a unilateral malformation) and upon radiographic analyis, an enlarged vestibular aqueduct syndrome was noted on the involved side.
Diagnosis is made by high resolution CT of the temporal bones.
There is no definitive medical/surgical treatment. The hearing loss may be treated with hearing aids, if appropriate. Additionally, I recommend avoidance of activities that may involve additional blows to the head. In the event of total hearing loss, cochlear implantation is an option.
BIO:
Eric W. Sargent, MD, FACS, is an otologist/neurotologist at the Michigan Ear Institute in Farmington Hills, Michigan. He is a member of the American Academy of Otolaryngology-Head & Neck Surgery, the American Neurotology Society and the North American Skull Base Society. He has authored over 20 peer-reviewed papers and several chapters on topics in hearing, balance, and the assessment and treatment of ear disorders.