[Delayed endolymphatic hydrops].

Idiopathic endolymphatic hydrops, commonly known as Ménière's disease, is a well-documented condition with distinct clinical features. It typically presents with vertigo, tinnitus, fluctuating hearing loss, and ear fullness. These symptoms usually appear in middle age and may affect one or both ears. While the condition is well understood, a less common variant known as Delayed Endolymphatic Hydrops (DEH) remains under-recognized. This gap motivated further investigation into DEH, which differs in its clinical progression and presentation. Prior research has shown that Ménière's disease rarely presents with severe sensorineural hearing loss. In contrast, DEH is characterized by a pre-existing hearing loss that precedes vertigo symptoms by years. No prior work had resolved the distinction between these two entities clearly. Histopathological differences have been noted in DEH cases, suggesting unique underlying mechanisms. That uncertainty drove the need for a focused review of DEH's clinical and pathological features.

The aim of this paper is to clarify the clinical and pathological distinctions between idiopathic endolymphatic hydrops and Delayed Endolymphatic Hydrops. The specific problem addressed is the misdiagnosis of DEH due to overlapping symptoms with Ménière's disease. The motivation for this study stems from the lack of consensus on diagnostic criteria and treatment approaches for DEH. The authors sought to summarize the clinical features and diagnostic criteria for DEH. They also aimed to report on histopathologic findings and pathogenetic hypotheses. This paper reviews the literature to provide a comprehensive overview of DEH. It highlights the importance of distinguishing DEH from idiopathic hydrops for accurate diagnosis and treatment planning. The study also discusses therapeutic approaches for both ipsilateral and contralateral variants of DEH.

The authors conducted a literature review to synthesize clinical and pathological data on Delayed Endolymphatic Hydrops. They analyzed existing case reports and studies that describe DEH's clinical features and diagnostic criteria. Histopathological findings from temporal bone studies were also reviewed. The review approach included examining the progression of symptoms in patients with pre-existing hearing loss. The authors compared ipsilateral and contralateral forms of DEH to identify differences in clinical presentation. They evaluated the effectiveness of various treatment strategies, including medical and surgical interventions. The review also considered pathogenetic hypotheses proposed for DEH. The findings were synthesized to provide a structured overview of the disease's characteristics and management.

The strongest finding is that DEH is distinct from idiopathic endolymphatic hydrops. Patients with DEH typically have a profound sensorineural hearing loss in one ear, often from early childhood. Vertigo symptoms appear years after the initial hearing loss. The ipsilateral variant involves vertigo from the deaf ear, while the contralateral variant involves the previously normal ear. Histopathological studies show changes in the deaf ear similar to viral labyrinthitis. The hearing ear in contralateral DEH resembles Ménière's disease pathology. Medical treatment has shown limited effectiveness in DEH cases. Surgical interventions, including conservative and radical procedures, have demonstrated better outcomes.

The authors conclude that Delayed Endolymphatic Hydrops is a distinct clinical entity from idiopathic endolymphatic hydrops. The condition is characterized by a pre-existing hearing loss followed by delayed vertigo symptoms. Histopathological findings support the distinction between ipsilateral and contralateral variants. Medical treatment has not proven effective for DEH, and surgical intervention remains the best approach. The authors propose that pharmacological labyrinthectomy could be a future therapy. They emphasize the importance of accurate diagnosis based on clinical and histopathological features. The study highlights the need for further research into the pathogenesis of DEH. The authors suggest that treatment strategies should be tailored to the specific variant of DEH.