Letter to the Editor: “Rosai Dorfman: Rare Cause of Cervical Lymphadenopathy”

Abstract

We read with great interest the article by Kobayashi KE et al. published in 2023 entitled: “Presentation of external ear Rosai–Dorfman disease with laryngeal involvement” and wish to reconnoiter Rosai–Dorfman Disease’s (RDD) clinical entities and the possible implications from the perspective of otology.

Dear Editor,

RDD is self-limiting and tends to regress over time in 20% to 50% of the cases. ¹ That said, the management should be individualized by symptomatology. Additionally, RDD has a predilection for adolescents and young adults, potentially imparting a consequential quality of life in the long run. The authors illustrated the involvement across 2 vital systems in RDD elegantly: the auditory and respiratory organs. ² The patient complained of progressive hearing loss with subsequent dyspnea, and improved clinically after prolonged systemic clofarabine and glucocorticoids without surgical intervention. On the contrary, Doule DG et al. reported a case of a middle-aged lady who suffered from relentless bloody otorrhea and otalgia from RDD despite multiple surgical debulking. ³ Her hearing in the affected ear eventually succumbed to the disease process and surgeries, but she successfully achieved remission after receiving low-dose fractionated radiation (20 Gy) with adjuvant cladribine. ³

The manifestation of middle ear involvement is exceptionally unusual, with not more than 3 cases reported.^3,4 Scarce evidence suggested that the ear involvement was often long-standing and not life-threatening. Common complaints may include tinnitus, difficulty hearing, otorrhea, and otalgia. We align with Kobayashi KE et al. to recommend a more conservative approach for symptom alleviation, reserving surgery only for diagnostic purposes or recalcitrant focal disease. However, we would like to highlight the importance of hearing threshold evaluation, either subjectively through pure tone audiometry or objectively (automated brainstem response), which might have been overlooked by the respected authors.

Having acknowledged the rare entity of otological involvement in RDD, we feel the necessity to array the type, degree, and configuration of the hearing loss prior to and after an intervention. We also wish to highlight the nature of RDD, as reported by the esteemed authors, as a great mimicker. Therefore, it is paramount for the clinicians and the pathologists to have a high index of suspicion. An inadvertent mistake of only relying on a test for diagnosis should be averted, instead of developing an algorithm for evaluating the significance of the findings.