Perilymphatic fistulas in children: rationale for therapy.

We report 26 consecutive patients (32 ears) who were identified in a 2 year period (July 1, 1985-June 30, 1987) with unexplained sudden, fluctuating, or progressive sensorineural hearing loss (SNHL). All patients underwent an exploratory tympanotomy and a perilymphatic fistula was identified in 13 patients (14 ears). The mean change of 14 +/- 27 dB in speech reception threshold before and after surgery was significant at p = 0.08 among children with fistula and ranged from -30 to 80 dB. In children with sudden, progressive or fluctuating SNHL and multiple sensory deficits, including blindness or contralateral SNHL, or prior head trauma, prompt surgical exploration is mandatory. Additionally, the aggressive management of otitis media with effusion is essential in such patients to minimize fluctuations in hearing caused by superimposed conductive hearing loss. Caution must be exercised to separate fluctuating hearing loss from fluctuations in audiologic testing.