Objective: This article provides an overview of relevant data supporting an
d refuting the existence of spontaneous perilymph fistula, as well as criti
cally reviewing the literature pertaining to their evaluation and managemen
t.
Data Sources: Sources used were relevant English language clinical and basi
c science publications.
Study Selection: A Medline search dating back to 1966 for articles concerni
ng perilymphatic fistula, including both human and animal data, was perform
ed Articles were included if they contained relevant data or were significa
nt reviews of the subject. A traditional bibliography search was then compl
eted to acquire articles missed by the computerized search, including works
published before 1966.
Data Extraction: The data from each publication were critically reviewed. E
mphasis on understanding the clinical features of surgically created perily
mph fistulas was used to more objectively assess the data regarding spontan
eous perilymph fistulas.
Data Synthesis: The data were not amenable to formal meta-analysis or valid
data summarization; however, when possible trends and contrasting data wer
e emphasized.
Conclusions: Spontaneous perilymph fistulas are very rare occurrences and t
he majority are likely incited by a pressure-altering event. Current method
ologies do not provide sufficient specificity and sensitivity to accurately
diagnose perilymph fistulas. The results of endoscopic studies of the midd
le ear in the evaluation of perilymphatic fistula suggest a low incidence c
ompared with the large number of fistulas reported in the literature. A hig
h index of suspicion must be maintained, and appropriate preoperative couns
eling should reflect the current controversies. Questions must continue to
be asked and further research pursued to help distinguish reality from myth
.