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Clinical Challenges
November 1998

Congenital Perilymphatic Fistula

Author Affiliations


Arch Otolaryngol Head Neck Surg. 1998;124(11):1279-1281. doi:10.1001/archotol.124.11.1279

A 5-year-old child with fluctuating sensorineural hearing loss (SNHL), normal computed tomographic findings, and no history of trauma or craniofacial abnormalities should undergo middle ear exploration for a perilymphatic fistula.

The evaluation and management of SNHL in a child, especially sudden or fluctuating hearing loss, remains a challenging and controversial task. The differential diagnosis in these situations must often be exhaustive. Depending on the age of the child, the clinician must consider etiologies such as viral labyrinthitis, autoimmune disease, hereditary hearing loss, Ménière disease, an acoustic neuroma, vascular disease, congenital syphilis, malingering, and a perilymphatic fistula (PLF).1-3 Based on the information provided, the primary concern is a PLF, defined as an abnormal connection between the inner ear and the middle ear cleft that allows the escape of perilymphatic fluid.4 Perilymphatic fistulae have been categorized into acquired and congenital types4,5: acquired PLFs are associated with a traumatic event or previous otologic surgery such as stapedectomy; congenital PLFs arise from a deformity or weakness of the temporal bone with no history of an antecedent event. A combined category is also possible when an underlying anatomical abnormality renders the ear unusually susceptible to developing a PLF with pressure changes. The current case focuses on the possibility of a congenital PLF, which is the most controversial of the 3 types.

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