Perilymphatic fistulas in children: rationale for therapy
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Published on: February 29, 2020
C M Myer1, S M Farrer, A F Drake
1University of Cincinnati College of Medicine, Department of Otolaryngology and Maxillofacial Surgery, Ohio.
Insights
Sudden hearing loss may indicate a perilymphatic fistula, a condition found in half of the studied patients. Prompt surgical exploration is recommended for children with unexplained hearing loss and other sensory deficits.
Area of Science:
- Otolaryngology
- Audiology
- Neurosurgery
Background:
- Unexplained sensorineural hearing loss (SNHL) presents a diagnostic challenge.
- Sudden, fluctuating, or progressive SNHL requires thorough investigation.
Observation:
- A 2-year study identified 26 patients with unexplained SNHL.
- Exploratory tympanotomy revealed perilymphatic fistulas in 13 patients (14 ears).
Findings:
- Surgical repair of perilymphatic fistulas showed significant improvement in speech reception thresholds in pediatric patients.
- The mean change in speech reception threshold was 14 +/- 27 dB (p=0.08).
Implications:
- Prompt surgical exploration is crucial for children with SNHL and associated sensory deficits or head trauma.
- Aggressive management of otitis media with effusion is vital to prevent superimposed conductive hearing loss.
- Distinguishing true fluctuating hearing loss from audiologic testing variability is essential.
Abstract:
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.
