Abstract
A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and the middle ear cavity, mastoid, or intracranial cavity. A PLF most commonly forms when the integrity of the oval or round window is compromised, and it may be trauma-induced or may occur with no known cause (idiopathic). Controversy regarding the diagnosis of idiopathic PLF has persisted for decades, and the presenting symptoms may be vague. However, potential exists for this condition to be one of the few etiologies of dizziness, tinnitus, and hearing loss that can be treated surgically. The aim of this review is to provide an update on classification, diagnosis, and treatment of PLF. Particular attention will be paid to idiopathic PLF and conditions that may have a similar presentation, with subsequent information on how best to distinguish them. Novel diagnostic criteria for PLF and management strategy for PLF and PLF-like symptoms is presented.
Highlights
A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and outside the inner ear that can allow perilymph to leak from the cochlea or vestibule, most commonly through the round or oval window
We have proposed a set of diagnostic criteria for aid in the identification of definite and probable PLF (Table 2)
Individuals with surgically confirmed PLFs and found that oval window PLFs most commonly presented with pneumolabyrinth and disorientation of the stapedial footplate, while round window PLFs most commonly presented with effusion of the round window niche
Summary
A perilymphatic fistula (PLF) is an abnormal communication between the perilymph-filled inner ear and outside the inner ear that can allow perilymph to leak from the cochlea or vestibule, most commonly through the round or oval window. PLF commonly causes cochlear and vestibular symptoms. Connections between vestibular symptoms and compromise of the structural integrity of the inner ear have been drawn as early as 1909 [1]; vague symptoms, lack of a clear diagnostic test, and changes in the description and definition of a PLF have made even the existence of the condition a controversial subject for decades. In his work titled Perilymph Fistula: Fifty Years of Controversy, Hornibrook provides a detailed examination of the history of PLF and the sources of controversy surrounding the condition, including associated symptoms and terminology [2]. Difficulties in defining and diagnosing PLFs has led to a dearth of more robust epidemiological information. Part of this problem has been that most methods used to identify PLFs lacked the sensitivity and specificity to provide consistent diagnoses [5]. Improvements in imaging techniques and emerging technology in the form of biomarkers have shown promise as tools to help define and diagnose PLF [6]
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